TiKa TaKa Pediatrics
TiKa TaKa Pediatrics
TiKa TaKa Pediatrics
______________
===================================================== Cardiology ===============
==============================================================
. Myocarditis:
______________
. Is an inflammation of the myocardium, Caused by: infections, toxins, and auto
immune diseases.
. Viral infection ( enteroviruses in particular) the most common cause of myoca
rditis in children.
. Classically occurring in spring and summer, myocarditis develops more often i
n infants and young children than in older children and adolescents.
. Leads to tissue necrosis, thereby worsening myocardial function, dilating the
heart, and increasing end diastolic volume with resultant pulmonary edema and C
HF.
. Myocarditis typically presents as a flulike illness: fever, anorexia, letharg
y, and irritability followed by respiratory distress from acute heart failure.
. A holosystolic murmur may be identified secondary to dilated cardiomyopathy a
nd the resulting functional mitral regurgitation.
. Hepatomegaly can develop due to passive congestion Other features of heart fa
ilure include fatigue, tachypnea, tachycardia, and decreased perfusion.
. Affected children should be monitored in the intensive care unit because of t
he risk of acute decompensation and fatal arrhythmias.
. The workup for suspected myocarditis includes a (CBC) with differential, ESR
and C-reactive protein, cardiac enzymes, and blood and viral cultures.
. A chest radiograph demonstrates cardiomegaly and pulmonary edema.
. Electrocardiography most commonly shows low voltage QRS and sinus tachycardia
.
. Echo.is the best means of evaluating myocardial function and often shows glob
al hypokinesis, left vent. hypertrophy, left vent. dysfunction & pericardial eff
usion.
. The gold standard for diagnosis is a myocardial biopsy, which also allows for
disease stage classification.
. Ventricular septal defect (VSD):
__________________________________
. The most common congenital heart malformation.
. VSDs range from small and asymptomatic to large with significant left-to-righ
t shunting.
. Indications of significant VSD is: left-to-right shunt First, he is symptomat
ic Second, he has the telltale murmur.
(Affected patients have pansystolic murmurs loudest at the left lower sternal
border.
. There maybe also a diastolic murmurs at the apex because of increased flow ac
ross the mitral valve ).
. This patient should have an echocardiogram with a bubble study to evaluate hi
s congenital heart defect.
. Most small ventricular septal defects close spontaneously and require no trea
tment, as long as there are no signs of pulmonary vascular disease.
. Reassurance and surveillance via EKG and echocardiography are needed in most
patients.
. Tetralogy of Fallot (TOF):
____________________________
. Is the most common cyanotic congenital disease in children less than 4 years.
. Patients often present with cyanotic spells.
. The classic tetrad includes:
(1) subpulmonary stenosis (single S2), (2) overriding aorta, (3) ventricularse
ptal defect (VSD) (pansystolic murmur), and (4) right ventricular hypertrophy.
. Because a large unrestrictive VSD is always present, the right ventricular pr
essure is the same as the left ventricular and aortic pressures.
. Pulmonary artery pressure and flow are inversely proportional to the degree o
f subpulmonary obstruction.
. The degree of cyanosis correlates precisely with the degree of pulmonary sten
osis.
. Untreated patients with TOF are at risk for "hypercyanotic" or "tet" spells.
. These spells are often precipitated by exertion (such as feeding) and are cha
racterized by irritability, cyanosis, and tachypnea.
. The exact etiology of these episodes is unclear, it may be predisposed by per
ipheral vasodilation, hyperventilation, and/or contractility of the Rt. ventricu
lar outflow tract.
. Regardless of the etiology, however, an increased right to left shunt across
the ventricular septal defect develops, causing a decrease in pulmonary blood fl
ow and
increased systemic venous return of deoxygenated blood leading to worsening c
yanosis.
. If untreated, "tet" spells can lead to syncope or death.
. The treatment of a "tet" spell is to place the child in a knee to chest posit
ion, which increases systemic vascular resistance resulting in
increased blood flow from the right ventricle to the pulmonary circulation.
. Morphine and an intravenous fluid bolus can also be given to increase pulmona
ry blood flow.
. Oxygen is of little benefit because the abnormality is decreased pulmonary bl
ood flow, not insufficient oxygenation
. Atrial septal defects:
________________________
. Are relatively common among children and may cause symptoms.
. On exam, there is a widely split and fixed S2, systolic ejection murmur in th
e left upper sternal border, and,
if there is a large shunt, a diastolic murmur at the left lower sternal borde
r.
. Transposition of the great vessels:
_____________________________________
. A cyanotic heart disease which presents with cyanosis in the first 24 hours o
f life.
. It is commonly seen in infants of diabetic mothers and in males.
. The aorta arises from the right ventricle, and the pulmonary artery from the
left ventricle.
. As a result, deoxygenated blood coming from the body goes to the right atrium
and ventricle and is cycled back to the body through the aorta.
. Oxygenated blood from the lungs is returned to the lungs by the left side of
the heart through the pulmonary artery.
. During intrauterine life, the fo ramen ovale and ductus arteriosus provide mi
xing of the deoxygenated and oxygenated blood,
resulting in an almost normal oxygenation of the fetal circulation.
. A patent foramen ovale or ductus arteriosus can maintain life after birth, as
seen in a patient with normal Apgar scores.
. The foramen ovale and ductus arteriosus begin to close after birth, leading t
o decreased mixing and poor oxygenation.
. As the aorta is located in front of the pulmonary artery, the S2 aortic compo
nent is better heard than the S2 pulmonic valve component and is audible as a si
ngle S2.
. There is usually no murmur because the foramen ovale and ductus arteriosus ha
ve closed.
. The neonate then presents with cyanosis and tachypnea within the first few ho
urs of life.
. Chest x-ray can be initially normal but show some evidence of increased pulmo
nary blood flow after 1-2 weeks.
. Echocardiography confirms the diagnosis.
. Prostaglandin E1 can be intravenously given to stabilize the neonate by maint
aining the patency of the ductus arteriosus, which is important for survival.
. Surgical treatment is definitive.
.N.B:
- Prostaglandin E1 is a vasodilator used to prevent ductus arteriosus closure i
n such infants,
to increase or maintain pulmonary blood flow and improve oxygenation until de
finitive surgery can be performed.
- Closure of the ductus arteriosus in such patients would cause progressive hyp
oxia and metabolic acidosis.
. Coarctation of the aorta (COA):
_________________________________
. A congenital defect characterized by stenosis of the aorta, usually near the
ductus arteriosus.
. present with hypertension in the upper part of the body (high BP in the arms)
and relative hypoperfusion in the lower part of the body (low BP in the legs).
. The prevalence of COA in patients with Turner's syndrome is approximately 7%.
. The clues to the diagnosis are occasional headaches and increased BP in both
arms, leg muscle fatigability while climbing stairs (due to lower body hypoperfu
sion).
. And a mild, continuous murmur heard all over the chest (due to the developmen
t of collaterals between the hypertensive and hypoperfused vessels).
. Rib notching caused by the dilatation of the collateral chest wall vessels is
specific for coarctation.
. Mitral stenosis:
__________________
. also causes a diastolic rumble but does not typically cause a pansystolic mur
mur at the left lower sternal border unless there is tricuspid regurgitation as
well.
. Other features to suggest rheumatic fever such as fever, arthritis, or skin l
esions.
. Tricuspid atresia:
____________________
. Suspect tricuspid atresia in a cyanotic infant with left axis deviation.
. This condition is characterized by an absent connection between the right hea
rt cavities, and a hypoplastic or absent right ventricle.
. Ventricular septal defect (holosystolic murmur on auscultation) occur in 90 %
of cases.
. Transposition of the great arteries occur in 30% of cases (which is not likel
y to be in a case where the chest x- ray does not demonstrate pulmonary overcirc
ulation).
. The associated heart defects, such as atrial septal defect (ASD), ventricular
septal defect (VSD), or patent ductus arteriosus (PDA), are necessary for survi
val.
. Venous blood passes through the ASD to reach the left cavities, and then thro
ugh the VSD back to the outlet portion of the right ventricle to reach the pulmo
nary circulation.
. Sometimes, there is an associated pulmonary stenosis, and the blood passes th
rough the PDA to reach the pulmonary circulation.
. Patients with normal arteries (without TGA) present in the first two weeks of
life with progressive cyanosis, tachypnea, easy fatigability, and poor feeding.
. EKG reveals left ventricular hypertrophy, which occurs since the left ventric
le handles both pulmonary and systemic venous returns.
. Echocardiography shows a fibromuscular membrane in the place of the tricuspid
valve, a variably small right ventricle, ventricular septal defect, and a large
left ventricle.
. The treatment generally includes PGE1 (to keep the ductus arteriosus open) an
d balloon atrial septostomy
(if the ASD is not large enough to allow an adequate flow from the right to l
eft atrium)
. postpericardiotomy syndrome:
______________________________
. It is reactive pericarditis with a pericardial effusion that present after su
rgery for congenital heart disease.
. Symptoms usually occur 1-6 weeks after surgery.
. Although the cause is not known, it is thought to be an autoimmune response,
possibly to a viral infection.
. Most children develop mild symptoms which are self limited. In infants, peric
ardial effusions can present with abdominal pain, vomiting, and decreased appeti
te.
. Older children may complain of chest pain Fever is often present On examinati
on, findings are consistent with pericardial and/or pleural inflammation,
including tachycardia, poor perfusion, pulses paradoxus, distant heart tones,
and jugular venous distention.
. If the effusion is large enough to affect the function of the heart , pericar
dial tamponade can occur.
N.B:
- Most cardiac murmurs in childhood are benign (innocent) and require only obse
rvation. Know the following features of a benign murmur:
systolic ejection quality.
varies with position;
Asymptomatic patient
Murmur intensity grade 2 or less
Normal S2
No audible clicks
Normal pulses
No other abnormalities.
- Pathological murmurs may indicate or lead to congenital heart disease, and ar
e very likely when the patient has any of the following:
symptomatic patient
Murmur intensity more than grade 2.
Pansystolic murmur
Murmur loudest at upper left sternal border
Absent or diminished femoral pulses
Murmur's quality is unchanged with position.
. Rheumatic fever:
__________________
. Suspect rheumatic fever in a child with sore throat, low-grade fever, pericar
ditis, arthritis, chorea and subcutaneous nodules.
. Other features include erythema marginatum, elevated ESR and prolonged PR int
erval on EKG.
. Rheumatic fever is caused by is group A streptococcus.
. Diagnosis is made if two major or one major and two minor criteria (Jones cri
. Diagnosis of acute bacterial sinusitis for children <6 years of age should be
based on clinical rather than radiographic criteria.
. The treatment is antibiotics, in uncomplicated sinusitis, the treatment of ch
oice is amoxicill in 45-50 mg/kg/day.
. Cholesteatomas:
_________________
. Cholesteatomas in children can either be congenital or acquired secondary to
chronic middle ear disease.
. Congenital cholesteatomas typically found in younger patients around the age
of five.
. New-onset hearing loss or chronic ear drainage despite antibiotic therapy are
typical presenting symptoms of acquire cholesteatomas, and
granulation tissue and skin debris may be seen within retraction pockets of t
he tympanic membrane on otoscopy.
. Complications of cholesteatomas include hearing loss, cranial nerve palsies,
vertigo, and life-threatening infections as brain abscesses o rmeningitis.
. Should be referred to an otolaryngologist for a dedicated otologic exam, poss
ibly accompanied by a CT and/or surgical visualization to confirm the diagnosis.
. Acute otitis media:
_____________________
. The most common causative organism is Streptococcus pneumoniae (40% of cases)
, followed by non-typable Haemophilus influenzae & Moraxella catarrhalis.
. Acute otitis media should be considered in any patient with symptoms of ear d
rainage and difficulty hearing.
. Ear pain is also common, but may be absent in young patients.
. Nonspecific systemic symptoms: fever, irritability, and diarrhea can sometime
s be present.
. The first-line treatment is 10 day course of amoxicillin.
. juvenile angiofibroma (JNA):
______________________________
. Any adolescent with a nasal obstruction, visible nasal mass, and frequent epi
staxis is considered to have a juvenile angiofibroma (JNA) unless proven otherwi
se.
. This is typically found in the back of the nose or upper throat (nasopharynx)
of adolescent boys.
. It is a benign growth, but is capable of eroding and locally invading.
. Removal is often difficult because the tumor is unencapsulated and may be dee
ply invasive.
. Recurrence of the tumor after surgical resection is common.
. Retropharyngeal abscesses:
____________________________
. Present with: fever, irritability, and fatigue along with a sore throat, dysp
hagia, trismus, a muffled or "hot potato" voice, and/or neck stiffness.
. On examination, unilateral cervical lymphadenopathy and decreased range of mo
tion of the neck are common.
. The oropharynx may be erythematous and a unilateral pharyngeal bulge may be p
resent if the child is able to open the mouth.
. Respiratory distress, if present, is concerning for airway obstruction.
. Diagnosis can be confirmed with either plain X-ray or a CT scan of the neck.
. Lateral neck X-ray often show widening of the soft tissue space between the t
rachea and the spine.
. Due to false +ve & -ve results of X-ray, so the diagnosis should be confirmed
by CT, which will delineate the abscess and help distinguish between an abscess
and cellulitis.
. N.B:
================================================================= Ophthalmology
==================================================================
. Neonatal Conjunctivitis:
__________________________
. Gonococcal conjunctivitis: A copious amount of purulent drainage in newborns
who are 2-5 days old is most consistent with gonococcal conjunctivitis.
. Chlamydia trachomatis conjunctivitis: caused by Chlamydia trachomatis is usua
lly milder and presents > 5 days after birth.
. Chemical conjunctivitis can result from prophylactic silver nitrate eye drops
, but it is usually fairly mild.
. viral conjunctivitis:
_______________________
. The conjunctiva is red with copious, watery discharge.
. Children are more commonly infected through contaminated swimming pools.
. It is mostly caused by adenovirus type 3.
. Chemical conjunctivitis:
__________________________
. It is the most common cause of a red eye that presents within the first 24 ho
urs of life.
. Approximately 80% of patients who receive silver nitrate prophylaxis (to prev
ent gonococcal conjunctivitis)
experience mild conjunctival irritation and tearing that generally resolve wi
thin 24 hours.
. Gonococcal conjunctivitis:
___________________________
. is acquired through contact with infected genital secretions, and occurs 2-5
days after birth.
. It presents as a hyperacute and highly purulent conjunctivitis with copious,
purulent eye discharge with swollen eyelids and chemosis.
. It is the most destructive neonatal eye infection, since it may result in cor
neal perforation and permanent blindness if left untreated.
. The treatment is ceftriaxone.
. Chlamydial conjunctivitis:
___________________________
. Develops a few days to several weeks after birth ( > 5 days after birth).
. Manifests with conjunctival congestion, edema, and mucoid or frank purulent d
ischarge.
. Pneumonia usually appears 3 to 19 weeks after birth, and symptoms include cou
gh, tachypnea, and rales; there is no fever, and wheezing is rare.
. The latter two features permit to differentiate chlamydial pneumonia from RSV
bronchiolitis.
. The treatment is oral erythromycin for 14 days, as topical agents are ineffec
tive.
. Trachoma:
___________
. caused by Chlamydia trachomatis serotype A-C. It is a major cause of blindnes
s worldwide.
. The active phase of the disease is characterized by follicular cionjunctiviti
s and pannus (neovascularization) formation in the cornea.
. conjunctivitis usually presents on the 5th to 14th day of life as congestion
and discharge that may be scant, mucoid or frankly purulent.
. Concurrent infection occurs in the nasopharynx, leading to nasal discharge.
. The diagnosis can be made by Giemsa stain examination of conjunctiva! scrapin
gs.
. Topical tetracycline or oral azithromycin should be started immediately.
. The treatment is erythromycin, which has to be administered systemically (ora
l) to decrease the risk of chlamydial pneumonia.
. Repeated infections can lead to scarring of the cornea.
. Retinal hemorrhages:
______________________
. Common findings in child abuse. Multiple retinal hemorrhages that differ in s
ize and color support the suspicion of child abuse.
. A precipitating factor (stepfather/mother, financial issue) is usually presen
t in the history In addition, there may be
bruises that usually have associated swelling and tenderness.
. In this case, nothing in the history and physical findings is suggestive of c
hild abuse; therefore, reporting to the authorities is not necessary.
. Herpes simplex keratitis:
___________________________
. Presents as pain, photophobia, and decreased vision.
. Dendritic ulcer is the most common presentation.
. There may also be minute clear vesicles in the corneal epithelium.
. Orbital cellulitis:
_____________________
. Is an infection that is posterior to the orbital septum which is unilateral a
========================================================================= Neurol
ogy ==========================================================
. Night Terrors:
________________
. Occurs in young children 2-12 years during Non-REM sleep.
. Characherized by fear, craying or screaming, decreased level of consciousness
.
. and amnesia of the event next morning.
N.B.:
- Todd's palsy is characterized by transient hemiplegia that occurs after a sei
zure.
. Migraine headaches:
_____________________
. are the most common cause of headaches in the pediatric population, occurring
in 50% of cases before the age of 20 years.
. The aura can be visual (e g , blurred vision, scotoma, photophobia, distortio
n of objects) or
sensory (e g perioral paresthesia, numbness of extremities, shoulder pain, ab
dominal pain, otalgia).
. Migraine headache is a clinical diagnosis, and further investigations are not
usually indicated.
. Neuroimaging is required only when there are behavioral changes, decline in s
chool performance, decline in growth parameters,
early morning headaches, seizures, orabnormal neurologic findings.
. In complicated migraines, neurologic signs (e g , basilar signs, ophthalmople
gia, or hemiparesis) may be present, although the work-up reveals nothing abnorm
al.
. These neurologic symptoms may last only during the headache or persist for se
veral days or weeks.
. Brain abscesses:
_________________
. can present with fever, headache, focal neurologic changes, seizure, spastici
ty, or signs of increased intracranial pressure.
. Congenital heart disease (particularly right-to-left shunts), head trauma, in
fections of the jaw or mouth, infections of the face or scalp, meningitis, and
cranial instrumentation increase the risk of developing a brain abscess.
. Absence (petit mal) seizures:
_______________________________
. Characterized by a sudden cessation, without warning, of ongoing mental activ
ity.
. Each episode rarely lasts longer than 30 seconds.
. A typical absence seizure is characterized by brief (typically lasting for a
few seconds) periods of impaired consciousness.
. Patients may also present with automatisms, but do not have a post- ictal sta
te.
. Hyperventilation during the EEG reveals a generalized 3Hz spike-and-wave patt
ern on a normal background.
. Minor motor symptoms are common, but complex automations and clonic activitie
s do not occur.
. Best diagnosed with EEG studies.
. An EEG with activation procedures (hyperventilation, photic stimulation, slee
p) helps in further diagnosis and classification of seizures.
. Ethosuximide is used almost exclusively for childhood absence seizures & valp
roic acid is the 2nd choice.
.N.B:
A) Type 1 neurofibromatosis:
---------------------------. Characterized by cafe-au-lait spots, axillary freckles, Lisch nodules of the
iris, neurofibromas, and bony lesions.
B) Type 2 neurofibromatosis:
---------------------------. Is associated with brain tumors, especially bilateral acoustic neuromas.
. Bilateral acoustic neuromas are diagnostic of neurofibromatosis.
. Patients with neurofibromatosis have a tendency to form tumors in the central
nervous system (CNS), peripheral nervous system, skin and viscera.
C) Sturge-Weber syndrome: A neurocutaneous syndrome
------------------------. Characterized by a congenital unilateral cavernous hemangioma along trigemina
l nerve distribution & intra-cranial calcifications that resemble a tramline in
X-ray.
. Also present with: focal or generalized seizures, mental retardation, and a p
ort wine stain or nevus flammeus along the branches of trigeminal nerve.
. Other pertinent findings are hemianopia, hemiparesis, hemisensory disturbance
, and ipsilateral glaucoma.
. Treatment is aimed at controlling the seizures and reducing intraocular press
ure Argon laser therapy is successful in removing the skin lesions.
D) Tuberous sclerosis:
---------------------. Ash leaf hypopigmentation, cardiac rhabdomyomas, kidney angioleiomyomas, men
tal retardation, seizures.
N.B.:
- The characteristic features of neurofibromatosis 1: cafe-au-la it spots, macr
ocephaly, feeding problems, short stature, and learning disabilities.
. Patients may later develop fibromas, neurofibromas or different tumors.
- The characteristic features of neurofibromatosis 2: are bilateral acoustic ne
uromas and cataracts.
. Strokes in children:
______________________
. are uncommon but When they occur, they are often the result of a congenital a
bnormality, infection, or systemic illness.
. Many childhood strokes are caused by sickle cell anemia, and suspected by (pa
tient's race, history of dactylitis, and prior pain crisis are all consistent wi
th sickle cell disease).
. The mechanism of strokes in children with sickle cell anemia is not fully und
erstood, but RBCs adherence to the endothelium, activation of van Willebrand's f
actor,
and hyperviscosity are all thought to contribute.
. Acute strocke syndrome:
_________________________
. Children who have trauma to the soft palate with a sharp object can either co
mpress the internal carotid artery (causing a thrombosis that embolizes
to the brain and causes stroke) or dissect the internal carotid artery (leadi
ng to ischemic stroke).
. The onset of symptoms can be delayed up to 24 hours after the traumatic episo
de.
. The diagnosis should be suspected clinically and can be confirmed with MRl/MR
A of the brain.
. Hydrocephalus:
________________
. Symptoms: Poor feeding, irritability, decreased activity, Vomiting.
. Physical examination: Tense and bulging fontanelle, Prominent scalp veins, wi
dely spaced cranial sutures, rapidly increasing head circumference.
. The best diagnostic approach in symptomatic children with rapidly increasing
head circumference is to perform diagnostic imaging.
. A CT scan of the brain is the best initial choice in an infant who is acutely
symptomatic & will reveal ventricular dilation as well as the infant's anatomy.
. In otherwise stable and asymptomatic infant, a sedated MRI could be considere
d to spare the child radiation exposure.
. Both CT and MRI provide greater detail than ultrasound, which requires a wide
ly open anterior fontanelle and is most useful in infants under 6 months.
. Treatment: a shunt that is placed from the ventricle to the peritoneum, pleur
a, or right atrium; which allows the excess CSF to drain.
. Guillain-Barre syndrome:
___________________________
. Suspect Guillain-Barre syndrome in a child who presents with an ascending pol
yneuropathy one week after an apparent viral infection (e.g. diarrhea).
. The underlying pathology involves mainly the peripheral motor nerves, althoug
h sensory and autonomic nerves may also be affected.
. Friedreich Ataxia:
_____________________
. an autosomal recessive condition and genetic counsel ing is recommended for p
renatal diagnosis for parents with one affected child.
. FA is associated with necrosis and degeneration of cardiac muscle fibers lead
ing to myocarditis, myocardial fibros is and cardiomyopathy.
. Cardiac arrhythmia and congestive heart failure contribute to a significant n
umber of deaths.
. Klumpke's paralysis:
_____________________
. is a brachial palsy that occurs in newborns following excessive traction on t
he arm.
. It consists of hand paralysis and ipsilateral Homer's syndrome (ptosis and mi
osis).
. It is secondary to injury to seventh and eighth cervical nerves and first tho
racic nerve.
. Confirmation MRI, which demonstrates nerve root avulsion or rupture.
. Treatment depends on the severity of the injury and, generally, it consists o
f partial immobilization and appropriate positioning to prevent contractures.
. Gentle massages and range-of-motion exercises can be started by 7-10 days of
age.
. If by 3-6 months there is no improvement, neuroplasty, neurolysis, end-to-end
anastomosis, and nerve grafting may be attempted.
. Erb-Duchenne palsy:
_____________________
. is another type of brachial palsy in which the injury involves the fifth and
sixth cervical nerves.
. It presents with: absent Moro reflex and intact grasp reflex of the affected
arm.
. Patients present with a characteristic position, which consist of adduction a
nd internal rotation of the arm with pronation of the forearm.
. Subarachnoid hemorrhage (SAH):
________________________________
. SAH can be caused by an intraventricular hemorrhage, which is common in prema
ture infants.
. Accumulation of the blood in the subarachnoid space may lead to destruction o
f the arachnoid villi and cisterns, thereby blocking the flow or decreasing the
absorption of CSF, and leading to communicating hydrocephalus.
. SAH is the most common cause of communicating hydrocephalus.
. CT scan: dilation of the entire ventricular system with distinct enlargement
of the subarachnoid space over the cerebral cortex is very suggestive of nonobst
ructive
or communicating hydrocephalus secondary to a subarachnoid hemorrhage (SAH).
.N.B:
- Dandy-Walker anomaly and Chiari malformation will both reveal CT findings con
sistent with obstructive or noncommunicating hydrocephalus.
. Dandy-Walker Anomaly will demonstrate a cystic expansion of the fourth ventri
cle, and Chiari malformation will reveal protrusion
of the structures of the posterior fossa through the foramen magnum.
===============================================================================
Hematology ===========================================================
. Acquired aplastic anemia:
___________________________
. present by: Normocytic or macrocytic anemia, Leukopenia, reticulocytopenia an
d thrombocytopenia.
. B.M. biopsy (essential for diagnosis) --> profound hypocellularity in all cel
ls with fatty infiltration.
. Result from B.M. supression due to acquired or congenital causes:
A) Acquired Causes of Aplastic Anemia:
-
Idiopathic.
Drugs (e.g, NSAIDs, sulfonamides, etc).
Toxic chemicals (e.g. benzene, glue, etc ).
Viral infections (e.g. HIV, EBV, etc ).
Immune disorders.
Thymoma.
r adenopathy.
. Depending on the extent and location of nodal and extranodal disease, there m
aybe symptoms and signs of airway obstruction, pleural or pericardial effusion,
hepatocellular dysfunction, or bone marrow infiltration.
. Although signs and symptoms are similar to ALL, presence of lymphoblasts make
s ALL most probable.
. Burkitt lymphoma:
___________________
. It is a neoplasm of mature B cells. It is associated with the Epstein-Barr vi
rus infection.
. Most patients present with either a mass involving the mandible or abdominal
viscera.
. High mitotic index is typical. Histological examination shows characteristic
"starry sky appearance".
. It is a very aggressive tumor but responds well to the high dose chemotherapy
.
. Myelodysplastic syndromes (MOS):
__________________________________
. They are clonal stem cell disorders which may progress to acute leukemias.
. These are usually seen in elderly patients, and are characterized by pancytop
enia.
. Henoch-Schonlein Purpura:
___________________________
. Is an lgA-mediated vasculitis of small vessels, affecting children more than
adults.
. Many cases follow an upper respiratory tract infection.
. The clinical manifestations: a classic tetrad of rash, arthralgias, abdominal
pain, and renal disease.
. The rash is typically purpuric and distributed symmetrically over the lower l
egs, buttocks and arms.
. Arthralgias most commonly affect the knees and ankles, these symptoms are alw
ays transient, and there is no permanent damage to the joints.
. GIT symptoms most commonly colicky abdominal pain (mostly due to local vascul
itis) that is frequently associated with vomiting,
. Two common pathologies which should be ruled out emergently are GI bleeding a
nd intussusception ( a surgical emergency, and is treated with air/barium enema)
.
. Renal involvement relatively mild disease characterized by asymptomatic hemat
uria & proteinuria with a normal or slightly elevated plasma creatinine; however
,
. More marked findings may occur including the nephrotic syndrome, hypertension
, & acute renal failure (treatment includes steroids and monitoring of renal fun
ction).
. Other organs as the lung and central nervous system, may be involved.
. Confirmation of the diagnosis requires evidence of tissue deposition in the s
kin or kidney of lgA by immunofluorescence microscopy.
N.B.:
1- Minimal change disease: electron microscopy uasually shows Podocyte fusion i
n the glomerulous.
2- Membranous nephropathy: electron microscopy shows Glomerular basement thicke
ning & is considered the most common cause of adult nephrotic syndrome.
3- focal segmental glomerulosclerosis: electron microscopy shows Focal and segm
ental sclerosis.
4- Goodpasture's syndrome: electron microscopy shows Linear deposition of lgG o
n the basement membrane is seen.
. Lymphadenitis in children:
_____________________________
. Cervical lymphadenopathy is common in children.
. Lymphadenitis is diagnosed when the lymph node becomes tender and erythematou
s in addition to being enlarged.
. Lymphadenopathy maybe acute or subacute/chronic, and unilateral or bilateral.
. Acute, unilateral lymphadenitis in children is usually caused by bacterial in
fection.
. Staphylococcus aureus is the most common followed by group A streptococcus.
. Patients with bacterial lymphadenitis are usually less than 5 years old and n
ontoxic appearing.
. The affected lymph node is tender, warm, erythematous, and usually 3 to 6 cm
in size.
. In some cases, the infection can progress to induration and fluctuance.
N.B.: Other causes of unilateral lymphadenitis:
1- Tularemia: caused by Francisella tularensis, can present with acute unilater
al cervical lymphadenopathy Affected children.
. usually have fever, chills, headache, and malaise in addition to the lymphad
enopathy.
. In addition, tularemia is a zoonosis and presents after contact with an infe
cted animal (e g rabbits, hamsters, or blood-sucking arthropods).
2- Peptostreptococcus is an anaerobic bacteria that can cause acute, unilateral
lymphadenitis However,
it is usually seen in older children with a history of periodontal (gum) dis
ease.
3- Nontuberculous mycobacteria (most commonly Mycobacterium avium-intracellular
e)
. are one cause of unilateral subacute-chronic lymphadenopathy Affected childr
en are usually less than 5 years old and
. present with firm, nontender lymphadenopathy that is usually less than 4 cm
in size.
. The skin over the lymph node often thins and develops a violaceous color.
. Fever and tenderness are unusual with this infection.
4- Epstein-Barr virus usually presents with bilateral subacute-chronic lymphade
nopathy along with
systemic symptoms such as fever, pharyngitis, hepatosplenomegaly and lymphoc
ytosis.
. GeneticB-cell deficiencies:
_____________________________
. Patients with genetic B-cell deficiencies begin to develop recurrent infectio
ns after passing 6 months (when the newborn's levels of maternal antibodies drop
).
. The deficient humoral immune response in these patients impairs the body's ab
ility to destroy encapsulated organisms.
. Hence, recurrent sinopulmonary infections with H. influenzae and S. pneumonia
e are common.
. Lack of lgA also predisposes to Giardia infection.
. Severe combined immune deficiency (SCID):
__________________________________________
. is a life-threatening syndrome due to adenosine deaminase deficiency & is an
autosomal recessive.
. Patients typically present with recurrent sinopulmonary infections, oral cand
idiasis, persistent diarrhea, opportunistic infections and viral infections.
n.
. Purpura in children:
______________________
. The common causes of purpura in children: thrombocytopenia, clotting factor d
eficiencies, and vasculitis.
. Nearly 2.5% of the normal population have a platelet count of< 150,000/?L, an
d clinical manifestations of thrombocytopenia (e g petechiae, purpura) usually d
o
not occur unless the platelet count is < 100,000/?L.
. Common clotting factor deficiencies causing purpura in children: van Willebra
nd disease, hemophilia A (factor VIII deficiency) & hemophilia B (factor IX defi
ciency).
. These conditions do not usually cause abdominal pain and other renal findings
(proteinuria).
. If there is no thrombocytopenia (paltelets > 100.000), then vasculitis should
be high in suspecious, especially if there is no abdominal pain or renal affect
ion.
. Henoch Schonlein purpura (HSP): a small vessel vasculitis that usually presen
ts with the classic tetrad
. lower extremity palpable purpura, arthritis/arthralgias, abdominal pain, and
renal disease.
. HSP is the most common form of systemic vasculitis in children.
. 50% of patients develop HSP after upper respiratory infection & have other no
nspecific symptoms as subcutaneous edema, joint pains, bloody stools, and scrota
l edema.
. Small bowel intussusception is a common complication of HSP.
. Laboratory testing is usually normal (including a normal platelet count), alt
hough hematuria or proteinuria may be found.
. Diagnosis is usually based on clinical findings, and skin biopsy is rarely ne
cessary.
. Treatment is usually supportive, but NSAIDS are sometimes given to abdominal
pain.
.N.B: Purpura fulminans:
- seen with bacterial infections (e.g. N. meningitidis, S. pneumoniae) However
, patients are usually very ill with fever, hypotension, and evidence of DIC.
. Immune thrombocytopenia:
________________________
. usually occurs in children between 2 and 6 years of age.
. The pathogenesis involves antibodies that bind to platelets, and subsequent d
estruction of these complexes in the spleen.
. The condition is usually preceded by a viral infection and presents with purp
ura, petechiae, hematuria or GI bleeding & there is no adenopathy.
. Laboratory studies usually show no abnormalities, except thrombocytopenia.
. The course is self-l imited, and spontaneous recovery occurs in the majority
of patients.
. There is controversy on treatment, but corticosteroids are drugs of choice in
all age groups for thrombocytopenia less than 30,000/mm3, and/or for severe sym
ptoms.
. Patients with a platelet count of more than 30,000/mm3 usually have very few
symptoms and do not require treatment (Observation).
. Hemophilia:
_____________
. Presence of spontaneous hemarthrosis and soft tissue hematomas raise the susp
icion for hemophilia, for which factor VI 11 assay is diagnostic.
. Spontaneous hearthrosis is very unlikely with any other form of bleeding diso
rder, including Von Willebrand's disease.
. The best next step is a complete set of coagulation studies followed by facto
r VIII and IX levels.
. Prolonged PTI, normal prothrombin time, normal bleeding time, normal fibrinog
en level and low serum factor VIII activity are the typical lab findings.
. The standard treatment for hemophilia is to replace the factor VIII.
. However, mild hemophilia may be treated with desmopressin (DDAVP), which caus
es release of factor VIII from the endothelial cells.
. Leukocyte adhesion defect:
____________________________
. An immune deffeciency disease present with: recurrent bacterial infections an
d necrotic periodontal infection.
. Delayed separation of the umbilical cord (>3 weeks) is characteristic and pre
sents an important clue to the correct diagnosis.
. The leukocytes fail to express some adhesion molecules on their surface.
. The leukocyte number is increased, but the inter-leukocyte communication is d
efective; therefore, chemotaxis and cytotoxicity are impaired.
. Normal lymphocyte count and gamma globulin concentration help to differentiat
e this condition from a variety of cell and/or humoral immune defects.
. Hemolytic uremic syndrome:
Extremely high-yield question for the USMLE!!!
____________________________
. It is usually preceded by an acute diarrheal illness due to the pathogens Esc
herichia coli serotype 0157 H7, Shigella, Salmonella, Yersinia, and Campylobacte
r.
. It is less commonly preceded by an upper respiratory infection.
. GI bleeding is common, Physical examination frequently reveals purpura and hy
pertension.
. The hallmark finding is microangiopathic hemolytic anemia.
. Other typical features: acute renal failure, fever, oliguria (or anuria) and
thrombocytopenia
. The peripheral smear reveal schistocytes (which represent fragmented RBCs) an
d giant platelets.
. lntravascular hemolysis results in elevated levels of (LDH) and indirect bili
rubin, as well as reticulocyte count BUN and creatinine levels are markedly elev
ated.
. Moderate leukocytosis may be present.
. The urine contains hemoglobin, hemosiderin, albumin, RBCs, WBCs, and casts.
. Suspect HUS when: a diarrheal illness and presents with acute R.F., microangi
opathic hemolytic anemia, fever, thrombocytopenia and peripheral smear of schist
ocytes.
.N.B:
- Howell-Jolly bodies: are nuclear remnants of RBCs present in sickle cell atie
nts & are generally removed by a functioning spleen.
- Their presence in peripheral smear suggests functional asplenia in sickle cel
l patients (Sickle cell patients usually have infarcted spleens by the first 18
to 36 months of life).
- Heinz bodies: are aggregates of denatured hemoglobin & are commonly seen in p
atients with hemolysis due to G6PD deficiency and thalassemia
- Helmet cells are fragmented red blood cells, their presence is suggestive of
traumatic hemolytic conditions such as DIC, HUS and TIP.
- Basophilic stippling are ribosomal precipitates which appear as blue granules
& seen with thalassemias, as well as lead or heavy metal poisoning.
fficiency.
. Other possible presentations are meconium ileus at birth and infertility.
. Meconium ileus is virtually diagnostic for cystic fibrosis, an autosomal rece
ssive disorder.
. A mutation of a single gene localized on chromosome 7 is responsible for this
disorder.
. patient is suffering from an acute exacerbation of lung disease, and his pres
entation (shortness of breath, excessive use of accessory respiratory muscles, h
ypotension) suggestive of severe attack.
. The most common etiologic agent is Pseudomonas aeruginosa.
. The three most common organisms that cause pneumonia in patients with cystic
fibrosis are Haemophilus, Pseudomonas, and Staphylococcus.
. The treatment is rapid administration of empiric antibiotics and the usual ch
oice is a combination of two agents with coverage against Pseudomonas aeruginosa
,
(ceftazidime or a penicillin derivative such as ticarcillin + an aminoglycosid
e such as amikacin or gentamicin).
.N.B: It is essential to memorize the following for the USMLE
- Gram-positive diplococci - Streptococcus pneumoniae.
- Gram-positive cocci in clusters - Staphylococcus.
- Gram-negative cocci - Neisseria.
- Gram-positive rods - Listeria and Bacillus.
- Gram-negative rods - Pseudomonas, Haemophilus, Klebsiella, Legionella.
. Pertussis:
____________
. Caused by Bordetella pertussis infection.
. It is a highly contagious infection, but its incidence has dramatically decre
ased today because of immunization.
. It usually presents with severe bouts of coughing spells after an upper respi
ratory tract infection.
. The coughing spells can be so severe that they can cause rectal prolapse, epi
staxis and pneumothoraces.
. These spells may last from two to ten weeks
. In patients with apparent subcutaneous emphysema secondary to severe coughing
paroxysms, chest x-rays must be obtained first to rule out pneumothorax(same me
chanism).
. Presence of leukocytosis with predominant small and normal-appearing lymphocy
tes further supports the diagnosis.
. The recommended first line treatment is a macrolide antibiotic (erythromycin,
azithromycin, or clarithromycin), regardless of the age or the immunization sta
tus.
. Treatment is given whenever pertussis is suspected or confirmed, and regardle
ss of the stage of the disease.
. For pertussis prevention, all close contacts should be given erythromycin for
14 days, regardless of age, immunizations, or symptoms.
. Foreign body aspiration:
__________________________
. A sudden onset of respiratory distress may be caused by foreign body aspirati
on.
. Direct laryngoscopy & rigid bronchoscopy is the procedure of choice for both
diagnostic and therapeutic purposes.
. Flexible bronchoscopy is a useful diagnostic tool but not theraputic.
. Segmental lung resection is used in some cases where rigid bronchoscopy fails
to remove the foreign body.
. Maternal diabetes increases the incidence of RDS in several ways Most importa
ntly, maternal diabetes delays the maturation of surfactant production in the lu
ngs.
. The mechanism is that fetal hyperinsulinism antagonizes the actions of cortis
ol and may delay the lung maturation process.
. The decreased production and secretion of surfactant is the 1ry cause.
. In the absence of surfactant, atelectasis ensues and results in perfused but
non-ventilated alveoli, and consequently hypoxia.
. HMD presents as: tachypnea, prominent grunting, intercostal and subcostal ret
ractions, nasal flaring, and duskiness within a few minutes after birth.
. Breath sounds may be normal or diminished with a harsh tubular quality, & on
deep inspiration, fine rales may be heard, especially over the lung bases poster
iorly.
. The characteristic chest-x ray: fine reticular granularity of the lung parenc
hyma.
. Treatment includes early mechanical ventilation and surfactant administration
.
. Transient tachypnea:
_______________________
. usually follows an uneventful normal term vaginal delivery or cesarean delive
ry.
. It is characterized by the early onset of tachypnea, sometimes with retractio
ns or expiratory grunting, and cyanosis that is relieved by minimal oxygen.
. The lungs are usually clear without rales or rhonchi & chest-x ray shows prom
inent pulmonary vascular markings, fluid lines in the fissures, overaeration,
a flat diaphragm, and occasionally, pleural fluid. Hypoxemia, hypercapnia, an
d acidosis are uncommon.
. The distinguishing feature from (HMD) is the x-ray finding
. Persistent pulmonary hypertension of the newborn (PPHN):
__________________________________________________
. Should be suspected in all term and post-term infants with cyanosis with or w
ithout fetal distress.
. Persistence of fetal circulatory pattern of right-to-left shunting through th
e PDA and foramen ovale after birth is due to very high pulmonary vascular resis
tance.
. The associated hypoxia is universal and unresponsive to 100% oxygen.
. The chest-x ray may be normal or may show parenchymal opacification in the ch
est, depending on the etiology.
. Although the clinical presentation of PPHN is indistinguishable from HMD, the
x-ray findings can differentiate.
. Meconium aspiration syndrome:
_______________________________
. Usually occurs in term or post-term infants, either in utero or more often wi
th the first breath.
. Thick & particulate meconium is aspirated into lungs, resulting in small air
way obstruction & consequent respiratory distress,that present within the 1st ho
ur of birth.
. Partial obstruction of some airways may lead to pneumothorax or pneumomediast
inum.
. Patchy infiltrates, coarse streaking of both lung fields, increased anteropos
terior diameter, and flattening of the diaphragm characterize the typical chestx ray
. Intracranial hemorrhage:
___________________________
. In neonates classically presents as periods of apnea, pallor or cyanosis, poo
r suckling, abnormal eye signs, high-pitched, shrill cry, muscular twitching,
. Aseptic necrosis of the femoral head: Extremely high yield question for USMLE!!
!
_______________________________________
. Osteonecrosis = A vascular necrosis.
. is a common complication of sickle cell disease.
. It involves occlusion of end arteries supplying the femoral head, bone necros
is, and eventual collapse of the periarticular bone and cartilage.
. The femoral head has two main sources of blood supply - ascending arteries an
d the foveal artery, which lie within the ligamentum teres.
. The foveal artery is patent early in life, but may become obliterated in olde
r patients.
. This explains why aseptic necrosis of the femoral head is uncommon in childre
n.
. Traction apophysitis (Osgood-Schlatter disease):
__________________________________________________
. is a common cause of knee pain, particularly in adolescent male athletes.
. During early adolescence (ages 13-14 for males, and ages 10-11 for females),
there are periods of rapid growth in which
the quadriceps tendon puts traction on the apophysis of the tibial tubercle w
here the patellar tendon inserts.
. This traction apophysitis is worsened by sports that involve repetitive runni
ng, jumping, or kneeling, and it improves with rest.
. Approximately 1/4 of affected individuals have bilateral disease.
. Physical examination, there is edema and tenderness over the tibial tubercle,
a firm mass can sometimes be felt due to heterotopic bone formation.
. Pain can be reproduced by extending the knee against resistance.
. Radiographic findings are nonspecific: anterior soft tissue swelling, lifting
of tubercle from the shaft, and irregularity or fragmentation of the tubercle.
. Treatment consists of activity restriction, stretching exercises, and non-ste
roidal anti-inflammatory medications.
. Clavicular fracture in neonat:
_________________________________
. Clavicular fracture presents with irregularity, crepitus, and fullness over t
he fracture site, and decreased movements of the arm.
. Predisposing factors are shoulder dystocia, traumatic delivery, and large siz
e of the infant.
. Generally, no specific treatment is required (reassurance).
. Acquired torticollis (wryneck):
_________________________________
. Refers to neck twisting usually due to asymmetric muscle activity.
. The most common causes of acquired torticollis are upper respiratory infectio
ns, minor trauma, and cervical lymphadenitis.
. More serious causes: retropharyngeal abscess and atlantoaxial subluxation.
. Cervical spine radiographs is needed in children with torticollis to ensure t
here is no cervical spine fracture or dislocation, which requires extreme cautio
n.
. Metatarsus adductus:
______________________
. A congenital foot deformity, which is most frequent in first-born infants & i
s attributed to the molding effect of the primigravid uterus.
. Approximately 10% of patients also have an associated acetabular dysplasia; h
ence, careful hip examination is required.
. Metatarsus adductus is subdivided into three types:
. The early symptoms are mild and are easily overlooked, arthritis is the hallm
ark of late disease.
. It tends to involve the knee in most patients Warmth, swelling from effusion
and restriction of movements differentiate it from arthralgias, which occur earl
y in the disease.
. The first episode occurs within six months of having erythema migrans, the ep
isodes gradually disappear in about ten years, if left untreated.
. In United States, Lyme disease is prevalent in: Connecticut, Rhode Island, Ne
w York, Pennsylvania, New Jersey, Delaware, Maryland and Wisconsin.
. Slipped capital femoral epiphysis (SCFE):
___________________________________________
. is a common hip disorder seen in adolescents.
. SCFE occurs when the capital femoral epiphysis displaces from the femoral nec
k.
. Boys > girls, and the usual age is 10 to 16 years old.
. Obesity is a significant risk factor, additional risk factors: hypothyroidism
, panhypopituitarism, and growth hormone deficiency or supplementation.
. Children with endocrine issues are more likely to have bilateral disease and
to present at an earlier age.
. Commonly present with an insidious onset of knee or hip pain and a limp, usua
lly without a history of trauma.
. Sometimes, minor trauma can increase the pain and bring the patient to medica
l attention.
. Patients tend to hold the hip in passive external rotation and exhibit decrea
sed internal rotation, abduction, and flexion.
. Diagnosis is made with plain radiographs of the hip (AP and frog leg lateral
views), which show the posteriorly and inferiorly displaced femoral head.
. Treatment: emergent orthopedic consultation and surgical fixation at the curr
ent degree of slippage to avoid the risk of avascular necrosis.
. Supracondylar fractures:
__________________________
. are the most common fractures in the pediatric population.
. The most common complication is entrapment of the brachial artery, resulting
in loss of the radial artery pulse.
. therefore, all patients with a supracondylar fracture must have their radial
pulse checked.
. After reduction of the fracture, the radial pulse must be reassessed.
. Duchenne muscular dystrophy:
______________________________
. should be suspected in a child under the age of 5 who presents with proximal
muscle weakness (difficulty climbing stairs),
Gower's sign (use his hands to "walk up" his legs in order to assume an uprig
ht position), diminished reflexes, and pseudohypertrophy of the calf muscles.
. Mental retardation.
. Cardiomyopathy.
. Serum CK (and aldolase)levels are used for screening the muscular dystrophies
.
. Muscle biopsy can confirm the diagnosis in most cases.
. The gold standard is genetic studies, which is required in atypical cases.
. Prognosis: most of patients are wheelchair bound by age of 12 years.
. Becker muscular dystrophy:
____________________________
. Similar to Duchenne muscular dystrophy but:
. After age of 5 years (usually around 12 y).
. Milder than Duchenne muscular dystrophy.
nsitive enteropathy.
. It is due to abnormal hypersensitivity to gluten and presents characteristica
lly at 12-15 months of age, when gluten-containing foods
such as wheat, rye or barley are introduced into the child's diet.
. Pyloric stenos:
_________________
. Is a common congenital anomally typical presentation is non-bilious projectil
e vomiting which gradually becomes more frequent and
forceful in a 4-8 week old infant Pertinent physical findings are a palpable
abdominal mass and visible peristaltic waves in the upper abdomen.
. Hypokalemic alkalosis occurs due to the frequent episodes of vomiting.
. Abdominal ultrasound is the most useful tool for confirming the diagnosis, an
d shows a thick hypoechogenic ring in the pyloric region.
. Additionally, the loss of large volumes of fluid via vomiting leads to contra
ction alkalosis due to the action of aldosterone.
. In order to compensate for the metabolic alkalosis, the respiratory system re
sponds via hypoventilation to create a secondary respiratory acidosis.
. i.e. have a primary metabolic alkalosis with respiratory compensation.
. This is characterized by a plasma pH greater than 7.45, PaC02 greater than 40
mm Hg and HC03- greater than 24 mEq/L.
. Pyloric stenosis is treated su rgically; however, surgery should be undertake
n only after the infant's hydration status and electrolyte levels have been stab
ilized.
. Meckel's diverticulum:
________________________
. Typically presents with painless melena in 2 to 3-year-old children.
. It is present in 2-3% of the population, and is the most common anomaly of th
e GIT.
. It results from the failure of the vitelline duct to obliterate during the fe
tal development.
. Heterotropic gastric tissue may be present in the diverticulum, which results
in ulcerations and bleeding.
. The diagnosis of a Meckel's diverticulum is best made with technetium-99m per
technetate scanning (uptake by heterotopic gastric mucosa).
.N.B.:
1- Peptic ulcer disease may present in preschool children with epigastric pain
related to meals with a positive family history.
2- Anal fissure is a painful condition associated with constipation it may occu
r in infancy, and presents with blood streaked stools.
3- lntussusception may present in an infant or a young child with irritability,
vomiting, red currant-jelly stools and abdominal mass.
4- Inflammatory bowel disease may present in preschool children with diarrhea,
abdominal pain, low-grade fever and malaise.
. A choledochal cyst:
_____________________
. a congenital abnormality of the biliary ducts characterized by the dilatation
of intra or extra-hepatic biliary ducts or both.
. It has a multifactorial origin, but most of the cases are related to an anoma
lous pancreaticobiliary junction, which
. leads to weakness and dilatation of the biliary wall due to the reflux of alk
aline pancreatic secretions into the biliary tree.
. Various types of choledochal cysts are:
- Type 1: Most common type, features the dilatation of the entire common hepati
c and common bile ducts or segments of each.
- Type 2: Relative isolated protrusions or diverticulae from the common bile du
ct
-
wall.
Type 3: Cyst found in the intraduodenal part of common bile duct
Type 4: Multiple dilatations in the intra and extra hepatic biliary tree.
Type 5: Isolated dilatation of intrahepatic bile ducts.
.N.B.:
- Caroli's syndrome is a congenital disorder characterized by intrahepatic dila
tation of bile ducts.
. Pseudo-pancreatic cyst:
________________________
. is a complication of acute or chronic pancreatitis characterized by retroperi
toneal/epigastric mass and high amylase.
. However, it will rarely cause a right upper quadrant mass and very mild jaund
ice by compressing the common bile duct.
. It is easily diagnosed by ultrasonogram.
. Intestinal atresia:
_____________________
. It can occur anywhere from the duodenum to the colon.
. Jejunal atresia is thought to occur due to an intrauterine vascular accident
that causes necrosis and resorption of a segment of bowel.
. The severity of the obstruction can vary from a membranous web to full atresi
a and loss of bowel length.
. Prenatal ultrasounds can diagnose intestinal atresia.
. If not discovered prenatally, the infant will develop vomiting and abdominal
distention along with feeding intolerance shortly after birth.
. Duodenal atresia appears as a "double bubble" on radiography.
. Jejunal atresia presents as a "triple bubble" on radiographs, indicating dila
tion of a larger amount of small bowel than that seen in duodenal atresia.
. Treatment should initially be focused on resuscitation and stabilization of t
he patient, followed by surgical correction.
. Duodenal atresia usually presents with bilious vomiting a few hours after the
first feeding.
. It is usually associated with other congenital anomalies, and can be a featur
e of Down's syndrome.
. Midgut volvulus:
___________________
. usually presents in a child less than one month old with bilious vomiting, ab
dominal distension and passage of bloodstained stools.
. Volvulus is associated with malrotation of the gut, and can be complicated by
perforation and peritonitis.
. lntussusception:
__________________
. is the most common cause of intestinal obstruction in the first two years of
life.
. The classic presentation: is an infant with colicky abdominal pain, vomiting,
and red currant jelly stools.
. The currant jelly stools are caused by bowel ischemia, which leads to bleedin
g and mucus production.
. Between episodes of abdominal pain, the child appears well.
. On physical examination, the abdomen is usually soft, nontender, and nondiste
nded, A sausage-shaped mass is often palpated.
. An abdominal radiograph may show air fluid levels due to obstruction.
. An ultrasound commonly shows a targetoid lesion in which one bowel segment is
nested inside another bowel segment.
. An air contrast enema is both diagnostic and therapeutic in the majority of c
ases.
pacing,
earlier return to pre-pregnancy weight, and decreased risk of breast and ovar
ian cancer.
B) contraindications to breastfeeding:
--------------------------------------. Infants with certain inborn errors of metabolism such as galactosemia, phenyl
ketonuria, and urea cycle defects.
. Other contraindications: maternal use of certain medications such as radioact
ive isotopes, antimetabolites, and chemotherapeutic agents.
. Mothers using drugs of abuse should be counseled to quit using these drugs an
d should not breastfeed if they are continuing to abuse drugs.
. Breastfeeding is also contraindicated with some maternal infections including
herpes simplex (if there are lesions on the breast), active tuberculosis, and H
IV.
. Tobacco smoking, hepatitis C infection, mastitis nor maternal alcohol use.
. Neonatal sepsis:
__________________
. Group B streptococcus is the most common cause of neonatal sepsis, it is part
of the normal vaginal flora of women and
is transmitted to infants during passage th rough the birth canal.
. In neonates, infection presents either as early-onset sepsis or late-onset se
psis.
. Early sepsis presents within the first week of life and usually involves seve
ral organs Symptoms include:
. Respiratory failure, meningitis, DIC, acute tubular necrosis and peripheral g
angrene.
. It is usually refractory to therapy and quickly evolves to shock, coma and de
ath.
. Late-onset GBS infection is usually a focal infection, most frequently mening
itis (75%),
although arthritis, osteomyelitis, cellulitis and urinary tract infection may
also occur.
. Meningitis presents with fever, lethargy, poor feeding, hypotonia, seizures a
nd a bulging fontanel.
. The diagnosis is confirmed by positive blood or cerebrospinal fluid cultures,
although all newborns with fever and sepsis should have full workup for sepsi
s, including urine cultures.
. If the mother has already received antibiotics, culture results may be negati
ve and a latex agglutination test may be more helpful.
N.B.
1- Escherichia coli meningitis is less frequent than GBS meningitis It is the s
econd most common cause of neonatal meningitis.
2- Listeria is the third most common cause of neonatal meningitis It also tends
to result in multiple abscesses and pneumonia, in addition to meningitis.
3- Congenital toxoplasmosis classically presents with microcephaly, microphthal
mia, hepatosplenomegaly and chorioretinitis.
4- Herpes simplex virus encephalitis usually presents with focal neurologic sig
ns. Herpes involves temporal lobes, so patients may present with seizures.
. Neonatal sepsis:
__________________
. Sepsis in the neonate often presents with fever or hypothermia, jaundice, let
hargy, and poor feeding.
. Infants with a serious bacterial infection such as meningitis rarely present
with classic findings such as neck stiffness or Kernig's or Brudzinski's signs.
. As a result, all infants with a suspected diagnosis of sepsis should be evalu
ated with blood cultures and a lumbar puncture
. Neonatal tetanus:
___________________
. Is generally seen in infants born to unimmunized mothers, frequently followin
g umbilical stump infection.
. Affected infants initially present in the first two weeks of life with poor s
uckling and fatigue, followed by rigidity, spasms and opisthotonus.
. Mongolian spot:
_________________
. The classic description (ie , well-demarcated, flat blue/gray lesion on the s
acral or presacral area) of a Mongolian spot.
. This lesion is seen more commonly in dark skinned children.
. It is caused by entrapment of melanin-containing melanocytes during their mig
ration from the neural crest into the epidermis in fetal development.
. and usually disappears in the first few years of life, most cases do not requ
ire any treatment.
. Mostly located at the base of the spine, lower back, and buttocks, it can als
o be seen in other parts such as the shoulders, arms, wrists, legs, ankles or ab
domen.
. The face, palms and soles are usually spared
N.B.:
- Cafe-au-lait spots are well-demarcated, tan or light brown flat lesions that
can vary in number, size, and distribution.
. This finding may indicate neurofibromatosis if the diameter is larger than 0.
5 mm and if more than 5 lesions are found.
- Cutis Marmorata appears as a lace-like pattern on the skin in response to col
d or stress.
. It can persist in some diseases such as Down's syndrome and trisomy 18.
- A salmon patch is a flat salmon-colored lesion commonly seen over the glabell
a, eyelids, and neck.
. It is a vascular lesion that usually disappears in early childhood.
. Indications of the evaluation of neonatal jaundice include:
_____________________________________________________________
1. Conjugated hyperbilirubinemia (> 2 mg/dL)
2. Jaundice that appears in the first 24 - 36 hours of life.
2. Serum bilirubin rising at a rate faster than 5 mg/dl/24 hours.
3. Serum bilirubin greater than 12 mg/dl in full-term (especially in the absence
of risk factors) or 10 - 14 mg/dl in preterm infants.
4. Jaundice persists after 10 -14 days of life.
5. The presence of signs or symptoms.
N.B.: Conjugated hyperbilirubinemia (> 2 mg/dL):
- Association with light colored stools, hepatomegaly, and direct (conjugated)
hyperbilirubinemia.
- Is indicative of neonatal cholestasis and impaired hepatic excretion of bilir
ubin, either by extrahepatic obstruction or liver cell injury.
- With conjugated neonatal hyperbilirubinemia, priority should be given to cond
itions that require prompt diagnosis and treatment, such as
sepsis, endocrinopathy (hypothyroidism). and nutritional hepatotoxicity cause
d by metabolic diseases (galactosemia, tyrosinemia).
- After these diagnoses are ruled out, the final step is to differentiate bilia
=========================================================================== Endo
crinology & Genetics ===========================================================
. Precocious pubarche (Puberty):
_________________________________
. It is very important to differentiate between precocious puberty that is caus
ed by premature activation of the hypothalamus-pituitary-gonad (HPG) axis,
and precocious pseudo-puberty that is caused by a gonadotropin-independent pr
ocess, typically an excess of sex steroids.
. In case of precocious pseudo-puberty: there is signs of severe androgen exces
s (i. e. severe cystic acne, significant growth acceleration) which suggests pre
cocious
ism.
. Turner syndrome is characterized by short stature, webbed neck, hypogonadism
(streak ovaries), lymphedema, high-arched palate,
congenitally bicuspid aortic valves, and coarctation of the aorta.
. Most commonly, ovarian failure in Turner syndrome manifests with delayed pube
rty, though at least 1/5 of patients will have a normal puberty followed by earl
y menopause.
. The decreased femoral pulses may indicate aortic coarctation, which occurs in
approximately 10% of patients with Turner syndrome.
. This clinical diagnosis should be confirmed by karyotype analysis.
.N.B.:
- A progesterone challenge: is used in the diagnostic evaluation of amenorrhea
in patients with both a uterus and normally developed breasts.
- Hyperprolactinemia can cause secondary amenorrhea as prolactin decreases GnRH
production and release.
- 17-hydroxyprogesterone measurement is indicated when congenital adrenal hyper
plasia is suspected as a possible cause of primary amenorrhea.
. Serum 17-0H progesterone is elevated in 21- and 11-hydroxylase deficiencies a
nd is decreased in 17-hydroxylase deficiency
. McCune-Albright syndrome:
___________________________
. is a rare condition characterized by precocious puberty, cafe au lait spots a
nd multiple bone defects (polyostotic fibrous dysplasia).
. It is responsible for 5% of the cases of female precocious puberty, and may b
e associated with other endocrine disorders,
such as hyperthyroidism, prolactin- or GH-secreting pituitary adenomas, and a
drenal hypercortisolism.
. McCune-Albright syndrome is sporadic and has been recently attributed to a de
fect in the G-protein cAMP-kinase function in the affected tissue, thereby resul
ting
in autonomous activity of that tissue.
. Remember the 3 P's of McCune-Albright syndrome precocious puberty, pigmentati
on (cafe au lait spots) and polyostotic fibrous dysplasia.
.N.B.: Adrenal tumors result most commonly in heterosexual precocious puberty (i
.e , premature development of male secondary sexual characteristics in a female)
.
. Cri-du-chat syndrome:
_______________________
. Is due to 5p deletion and presents as a cat-like cry.
. Also hypotonia, short stature, microcephaly with protruding metopic suture, m
oonlike face, hypertelorism, bilateral epicanthal folds,
high arched palate, wide and flat nasal bridge, and mental retardation.
. Gaucher's disease:
____________________
. Is due to the deficient activity of the lysosomal enzyme, acid beta-glucosida
se.
. The typical patient is an Ashkenazi Jewish adolescent with chronic fatigue,ea
sy bruisability, bone pain, and pathological fractures.
. Diagnosis is confirmed with radiologic (Erlenmeyer flask deformity of the dis
tal femur) and bone marrow studies (Gaucher cells with wrinkled paper appearance
).
. Niemann-pick disease:
_______________________
. Types A and B result from the deficient activity of sphingomyelinase.
. this is a fatal disorder of infancy Clinical manifestations include failure t
o thrive, hepatosplenomegaly, and
. a rapidly progressive neurodegenerative course that eventually leads to death
by age 2-3 years.
. Beckwith-Wiedemann syndrome:
______________________________
. The exact cause is unknown, but sometimes associated with duplication of chro
mosome 11 p.
. This region contains the gene encoding for IGF-2, which may explain the macro
somia.
. Present with macrosomia, macroglossia, visceromegaly, omphalocele, hypoglycem
ia, hyperinsulinemia, prominent eyes, prominent occiput, ear creases & pancreati
c hyperplasia.
. It is usually sporadic, but occasional cases have familial inheritance.
. Sometimes, the hypoglycemia may be severe and intractable, and subtotal pancr
eatectomy may be needed.
. Patients have an increased risk of neoplasms such as Wilms' tumor, hepatoblas
toma, and gonadoblastoma
. Lesch-Nyhan syndrome:
_______________________
. Is secondary to a deficiency in hypoxanthine-guanine phosphoribosyl transfera
se (HPRT).
. Symptoms: self-mutilation (self-injury, especially biting of the upper extrem
ities), neurologic features (mental retardation, dystonia, choreoathetosis, spas
ticity).
. Gouty arthritis, and tophus formation.
. Gout is usually seen in patients above 50 years of age; therefore, suspect Le
sch-Nyhan syndrome if you see a boy with gout.
. All victims of Lesch-Nyhan syndrome are male.
. Prader-Willi syndrome (PWS):
______________________________
. Is a sporadic disorder, in 50-70 % of cases, there is a deletion in the long
arm of chromosome 15.
. PWS displays a particular type of genetic transmission called genomic imprint
ing, wherein the phenotype expression depends on whether
the genetic defect is inherited from the mother or the father.
. presentation: severe hypotonia at birth or infancy, hyperphagia, obesity, sho
rt stature and mental retardation.
. The typical craniofacial features are narrow bifrontal diameter, diamond-shap
ed eyes and a small, down-turned mouth.
. Hypothalamic dysfunctions (GH deficiency and hypogonadotropic hypogonadism) m
ay be present.
. The associated short stature, obesity and hypotonia usually respond to GH adm
inistration; however,
the implication of GH deficiency accompanying PWS in the development of these
features is still controversial.
.N.B:
- If you see a vignette with a child that is >2-years-old, it is unlikely that
the diagnosis is Patau's or Edward's syndrome.
. Macrosomia secondary to maternal diabetes:
________________________________________
. The most common cause is thyroid dysgenesis (ie , aplasia, hypoplasia, or ect
opic gland).
. Other causes: inborn errors of thyroxin synthesis, and transplacental materna
l thyrotropin- receptor blocking antibodies.
. Infants initially appear normal at birth, but gradually develop apathy, weakn
ess, hypotonia, large tongue, sluggish movement, abdominal bloating & umbilical
hernia.
. Other signs: pathologic jaundice, difficult breathing, noisy respiration, hyp
othermia, and refractory macrocytic anemia.
. Infants initially appear normal due to the presence of moderate amounts of ma
ternal hormones in the infant's circulation.
. So, screening is mandated in all states at birth to allow for the early detec
tion, treatment, and consequent improvement of the prognosis.
. Screening is done by measuring serum T4 and TSH levels.
. The treatment is levothyroxine.
. Fragile X syndrome:
_____________________
. Male infant with a characteristic appearance (la rge head, long face, promine
nt forehead and chin, protruding ears), joint laxity, and large testes.
. Behavioral abnormalities: hyperactivity, short attention span, and autism, ar
e common.
. It results from a full mutation in the FMR1 gene caused by an increased numbe
r of CGG trinucleotide repeats accompanied by aberrant methylation of the FMR1 g
ene.
. Turner syndrome:
__________________
. Presentation: short stature, short webbed neck and broad chest with widely sp
aced nipples and 45 XO karyotype.
. Other features: a low posterior hairline, gonadal dysgenesis, infertility and
primary amenorrhea, which may occur as the child grows.
. A higher incidence of cardiac anomaly: coarctation of the aorta (COA) and a b
icuspid aortic valve.
. Physical findings of COA include upper extremity hypertension and a delay in
the radial-femoral pulse.
. Congestive heart failure may be seen in infants with severe cases.
. The diagnosis is made with an echocardiogram and the treatment is surgery.
. There is higher risk of renal abnormalities, particularly horseshoe kidney, a
nd should have a screening ultrasound after the diagnosis is made.
. Swollen hands and feet due to congenital lymphedema, which is common in patie
nts with Turner syndrome due to abnormal development of the lymphatic network.
. Edema that is due to lymphedema is generally nonpitting on physical exam.
. Patients are prone to osteoporosis which increases the risk of bone fracture,
this is due to low estrogen levels from gonadal dysgenesis.
. Edward's syndrome:
____________________
. trisomy 18.
. The features: micrognathia, microcephaly, rocker bottom feet, overlapping fin
gers and absent palmar creases.
. Closed fists with index finger overlapping the 3rd digit and the 5th digit ov
erlapping the 4th, and rocker bottom feet.
. Congenital heart disease occurs in greater than 50 % of affected patients; ve
ntricular septal defect is the most common one.
. 80% of affected children die in the first month, 90% die by 1 year and remain
ing 5-10% are mentally retarded.
. Osteomalacia caused by vitamin D deficiency:
_______________________________________________
. Vitamin D deficiency leads to decreased intestinal calcium absorption and hyp
ocalcemia.
. Hypocalcemia stimulates parathyroid glands & secondary hyperparathyroidism re
sults, which brings serum calcium to normal or near normal, especially in early
stages.
. Secondary hyperparathyroidism causes hypophosphatemia by increasing its urina
ry excretion.
. Therefore, vitamin D deficiency causes more marked hypophosphatemia than hypo
calcemia, especially in early stages.
. Labs show low or low-normal serum calcium, low serum phosphate, increased ser
um pTH, low plasma 25-OH vitamin D & normal, low or elevated 1,25 dihydroxy vita
min D (calcitriol).
. In patients with Pseudohypoparathyroidism, labs show low serum calcium, high
serum phosphate and high serum PTH.
. Serum alkaline phosphatase and vitamin D levels are within normal limit.
. Patients with renal failure will have low serum calcium, high serum phosphate
, high serum PTH (due to secondary hyperparathyroidism) and low serum 1,25 dihyd
roxy vitamin D.
. Patients with X-linked hypophosphatemic rickets have low serum phosphate due
to renal phosphate wasting.
. They have normal serum calcium, normal serum PTH and normal serum alkaline ph
osphatase and normal levels of serum 25-hydroxy vitamin D.
. There may also be a functional defect in the activity of 1-hydroxylase and as
a result serum levels of calcitriol may below.
. In type II vitamin D dependent rickets, there is mutation of vitamin D recept
or.
. Therefore, these patients have normal serum levels of calcitriol but it is in
effective and as a result osteomalacia occurs.
. Pubertal gynecomastia: Extremely high yield question for USMLE!!!
________________________
. is seen in approximately one-half of adolescent boys, at an average age of 14
years.
. It is often asymmetric or transiently unilateral, and frequently tender.
. In prepubertal males the testicular size is normally 2 cm in length and 3 ml
in volume.
. The initial management involves reassurance and watchful waiting/observation.
. Down syndrome:
________________
. Patients with Down syndrome may have a wide range in severity of manifestatio
ns.
. The most common congenital heart defect is an endocardial cushion defect of t
he atrioventricular (AV) canal.
. Other congenital heart defects: PDA & ventricular septal defect (condenital h
eart defects are the most common cause of death in childhood).
. AV canal defects are diagnosed with ECho.
. The left to right shunt due to the (AV) defect can develop into pulmonary hyp
ertension,that manifested as a loud P2 on auscultation & has a high morbidity.
. AV canal defects may be partial or complete, but both need early surgical cor
rection to prevent pulmonary hypertension.
. Patients with Down's syndrome have a high predilection for duodenal atresia.
. Other GIT anomalies: Hirschsprung's disease, Esophageal atresia, Pylo ric ste
nosis, Malrotation of the bowel.
. Atlantoaxial instability is commonly seen in Down syndrome, and most commonly
occurs due to excessive laxity in the posterior transverse ligament, which caus
es
increased mobility between the atlas (C1) and the axis (C2).
. Symptoms of atlantoaxial instability: behavioral changes, torticollis, urinar
y incontinence, and vertebrobasilar symptoms as dizziness, vertigo, and diplopia
.
. Also upper motor neuron symptoms such as leg spasticity, hyperreflexia, a pos
itive Babinski sign, and clonus.
. Patients with Down syndrome are normally hypotonic, and they may remain hypot
onic or have increased tone due to atlantoaxial instability.
. It is diagnosed by lateral radiographs of cervical spine in flexion, extensio
n & in a neutral position Open mouth radiographs can also visualize the odontoid
.
. Treatment: surgical fusion of the first cervical vertebrae (C1) to the second
(C2).
. Marfan syndrome:
__________________
. MSF is an autosomal dominant disorder that results from the mutations of the
fibrill in-1 (FBN1) gene.
. It presents with tall stature, long and emaciated extremities, arachnodactyly
, hypermobility of the joints, upward lens dislocation, and aortic root dilation
.
. Homocystinuria:
_________________
. An autosomal recessive disease caused by cystathionine synthase deficiency.
. Her features are characteristic of Marfan's syndrome (tall stature, long emac
iated extremities, arachnodactyly, hyperlaxity of the skin and joints).
. But her clinical presentation of a stroke is highly indicative of homocystinu
ria.
. Remember: Marfan's features + thromboembolic events = classic homocystinuria.
. Thromboembolic events at any age, due to the pathologic changes in the vessel
walls and increased adhesiveness of the platelets (commonly cerebral vessels).
. Lens dislocation (ectopia lentis): In Marfan's syndrome, the lens is dislocat
ed upward, and in homocystinuria, it is dislocated downward.
. Elevation of both homocysteine and methionine in body fluids confirm the diag
nosis.
. The initial treatment: high doses of Vitamin B6.
. Restriction of methionine along with supplementation of cysteine is used for
patients not responsive to Vitamin B6 therapy.
. Glucose-6-phosphatase deficiency:
___________________________________
. is also known as type I glycogen storage disease and Von-Gierkes' disease.
. As its name suggests, this condition is caused by deficient glucose-6-phospha
tase in the liver, kidneys, and intestinal mucosa.
. The typical patient, is 3-4 months of age with hypoglycemia, lactic acidosis,
hyperuricemia, and hyperlipidemia.
. Hypoglycemic seizures may occur.
. The characteristic features: a doll-like face (fat cheeks), thin extremities,
short stature, and a protuberant abdomen (due to the enlarged liver and kidneys
).
. The spleen and heart are normal.
=========================================================================== Neph
rology =================================================================
. Systemic Lupus erythematosus:
_________________________________
. Positive anti-Smith antibodies and/or anti-double stranded DNA antibodies is
specific and confirmatory for the diagnosis of systemic lupus erythematosus
. Transient proteinuria:
________________________
. A urine dipstick can be positive in up to 10% of school-aged children. Protei
nuria in children can be transient (intermittent), orthostatic, or persistent.
. Transient proteinuria is the most common cause of proteinuria and can be caus
ed by fever, exercise, seizures, stress, or volume depletion.
. Orthostatic proteinuria is very common in adolescent boys and is defined as i
ncreased protein when the patient is in an upright position
that returns to normal when the patient is recumbent.
. If the urinalysis shows no hematuria and is otherwise normal, the urine dipst
ick should be repeated on at least two additional specimens.
. If these subsequent tests are negative for protein, the diagnosis is transien
t proteinuria that are usually benign conditions that require no further evaluat
ion.
. Posterior urethral valves:
____________________________
. are the most common congenital obstructive urethral lesion in males.
. There is midline lower abdominal mass probably represents a distended bladder
.
. The finding of a distended bladder indicates that the obstructive lesion is d
istal to the bladder neck.
. Posterior urethral valves are abnormal folds in the posterior urethral wall (
distal prostatic urethra) that obstruct urine flow out of the bladder.
. Affected infants may develop hydronephrosis, azotemia, and failure to thrive.
. A voiding cystourethrogram (VCUG) is the diagnostic test of choice.
. Bladder exstrophy:
____________________
. Results from a ventral defect of the urogenital sinus, which causes separatio
n of the pubic rami, external rotation of the hips,
and separation of the rectus abdominis muscles.
. The resulting ventral midline defect allows protrusion of a malformed bladder
.
. This condition is associated with epispadias, recurrent urinary tract infecti
ons, and urinary incontinence.
. Hypospadias:
______________
. is a congenital abnormality where the penile urethra opens on the ventral sur
face of the penis rather than at the tip.
. It results from incomplete fusion of the urethral folds on the ventral penis.
. urachus:
__________
. is a tubular extension of the allantois that extends from the bladder to the
umbilicus.
. It is normally obliterated during fetal development A patent urachus can resu
lt in an urachal fistula, cyst or sinus.
. None of these conditions would interfere with urine drainage from the bladder
.
_____________________
. Due to a developmental disorder or maturational lag in bladder control while
asleep.
. The condition is more common in boys and in those with a positive family hist
ory.
. More than 20% of the cases spontaneously resolve before the patients are scho
ol age.
. Reassurance of the parents is usually the first step in management, although
some physicians recommend bladder exercises and scheduled toileting.
. Conditioning with "wet" alarms and waking the child in the middle of the nigh
t have also helped a moderate number of children.
. For patients with persistent nocturnal enuresis, the drug of choice is DDAVP
(desmopressin) .
. The second line medication is imipramine.
N.B.:
- Bedwetting is considered normal before the age of 5 years and no investigatio
ns or medical treatment should be prescribed for it.
. Fibromuscular dysplasia:
_________________________
. The most common cause of secondary hypertension in children is fibromuscular
dysplasia.
. It is responsible for approximately 20% of all cases of renal hypertension.
. Aside from children, fibromuscular dysplasia is also generally seen in premen
opausal women (or women less than 50 years old).
. Physical examination reveals a hum or bruit in the costovertebral angle due t
o well-developed collaterals.
. The right renal artery is more affected than the left.
. Angiography typically shows a "string of beads" pattern to the renal artery.
. Vesicoureteral reflux (VUR):
______________________________
. Is the retrograde flow of urine from the bladder to the ureter and renal pelv
is.
. Reflux is a risk factor for UTI & Repeated attacks of UTI can lead to progres
sive renal scarring,
which is the major cause of end stage renal disease and hypertension in child
ren.
. It is a risk factor for UTI as it facilitates the transport of bacteria from
bladder to the upper urinary tract,
. this explains why VUR is the most likely cause of acute UTI in children.
. VUR is present in 35-40 o/o of children with UTI.
. The diagnosis is made with voiding cystourethrogram (VCUG) followed by renal
imaging.
. Diagnosis of VUR is best made with a voiding cystourethrogram or a radionucli
de cystogram (RNC).
. Renal ultrasonography is less sensitive in detecting reflux nephropathy.
. The American Academy of Pediatrics recommends that all children aged 2 to 24
months with a first UTI should undergo a VCUG or RNC to detect the presence VUR.
. N.B:
- Chronic pyelonephritis is characterized by focal parenchymal scarring and blu
nting of calices on IVP.
- Hydronephrosis is seen on IVP as dilation of the collecting system including
the calyces, pelvis and ureter, depending on the level of obstruction.
- Ureteropelvic obstruction would also lead to hydronephrotic changes in the ki
dney with IVP showing the level of obstruction as a constriction in the flow of
dye.
. Acute pyelonephritis:
_______________________
. usually present with fever, chills, nausea, vomiting and flank or suprapubic
pain.
. Physical examination shows costovertebral angle tenderness.
. Urinalysis shows bacteriuria and pyuria.
. Therapy should be started with empiric antibiotics after withdrawal blood & u
rine for culture and sensitivity and before its results are available.
. Severe disease with systemic manifestations warrants intravenous (IV) antibio
tics.
. If patient is vomiting and hypotensive; therefore, IV antibiotics should be a
dministered.
========================================================================= Infect
ion =====================================================================
. Measles (rubeola):
____________________
. It is caused by Paramyxovirus.
. Characterized by a prodrome of non-productive cough, coryza, non-purulent con
junctivitis, followed by
. Koplik's spots (pathognomonic): red spots with bluish specks over the buccal
mucosa, opposite the premolar tooth & sometimes on the inner conjunctivae & vagi
nal mucosa
. And maculopapular rash initially appearing on the face then spread to involve
the entire body.
. Lab. findings: leukopenia, lymphopenia and Proteinuria may be seen.
. Diagnosis is mostly clinical and can be supported by a fourfold rise in hemag
glutination inhibition antibody titer.
. Leukopenia (T-cell cytopenia) and thrombocytopenia can be seen with measles i
nfection.
. Vitamin A has been shown to reduce the morbidity and mortality rates of patie
nts with measles through immune enhancement.
. It also helps the gastrointestinal and respiratory epithelium to regenerate.
. N.B.: - Atypical measles:
- Occurs in persons who have previously received inactivated measles vaccine (
was available in the 1960's), (the measles vaccine available now is a live one).
- This form is potentially life-threatening, and characterized by atypical rash
(not maculopapular), the absence of Koplik spots, arthritis,
hepatitis and lung involvement Edema of the hands and feet may occur.
. Rubella:
__________
. characterized by low-grade fever, lymphadenopathy (sub-occipital and posterio
r auricular) and rash.
. The rash is erythematous, maculopapular and classically begins on the face, s
preading subsequently down the body.
. congenital rubella infection:
_______________________________
. Transmitted via the placenta, the causative organism is Toga virus.
. Maternal infection manifests after an incubation period of 14 to 21 days with
mild symptoms such as rash, arthralgias and generalized lymphadenopathy.
. Since rubella infection confers permanent immunity, only primary infection in
the pregnant woman carries the risk of fetal disease.
. The severity of fetal disease depends on the time of pregnancy when the trans
mission took place.
. Infections of early pregnancy are the most severe, and the associated adverse
outcomes include spontaneous abortion and congenital rubella syndrome (CRS).
. If transmission occurs in the first 4 weeks of pregnancy, the risk of develop
ing CRS is 50%, drops to 1 % if transmission occurs in the third trimester.
. Features of CRS: IUGR, deafness, cardiac malformations (e g , patent ductus a
rteriosus, atrial septal defects), microphthalmia,
cataract, retinopathy, hepatosplenomegaly, thrombocytopenia, and CNS involvem
ent.
. Chronic infection may result in growth retardation, radiolucent bone disease,
jaundice, hepatosplenomegaly, thrombocytopenia and purple skin lesions
("blueberry muffin spots").
. Maternal infection is confirmed by the presence of lgM to rubella virus, or a
fourfold increase in lgG on two serum samples obtained two weeks apart.
. In the infant, the diagnosis is confirmed by the presence of lgM or persisten
ce of lgG beyond the age of 6 months.
. Prevention of congenital rubella is primarily achieved by administration of r
ubella vaccine to all females of childbearing age.
. If the immunologic status of a pregnant woman is unknown, rubella titers shou
ld be obtained in the first trimester.
. Immunization should not be performed in pregnancy because of a theoretical ri
sk for the fetus, and such women should be advised to avoid anyone with possible
rubella infection.
. classic triad of congenital rubella syndrome (CRS) - sensorineural deafness,
cardiac malformations (e g , PDA and ASD), and cataracts.
. Erythema infectiosum or Fifth disease:
________________________________________
. caused by human parvovirus 819.
. Children have different presentations and develop a rash with a "slapped chee
ks" appearance.
. Fever is not present or very mild in this disease.
. Roseola infantum:
___________________
. Caused by herpes virus 6.
. Characterized by the abrupt onset of high-grade fever, which is then followed
by a maculopapular rash, appearing on the trunk and then spreading peripherally
.
. The patient is no longer febrile when the rash develops.
. There are no positive physical signs such as sore throat or lymphadenopathy d
uring the febrile stage.
. chickenpox (Varicella virus):
_______________________________
. The clinical manifestations of chickenpox in healthy children generally devel
op within fifteen days after the exposure.
. Include a prodrome of fever, malaise, or pharyngitis, followed by the develop
ment of a generalized vesicular rash, usually within 24 hours.
. The lesions are commonly pruritic and appear as successive crops of vesicles
over a three to four day period.
. Patients typically have lesions in different stages of development on the fac
e, trunk and extremities.
. New lesion formation generally stops within four days, and most lesions are f
ully crusted by the 6th day in normal hosts.
. Impetigo :
____________
. Is a superficial skin infection with multiple vesiculopustules on the exposed
sms.
. Scarlet fever:
________________
. Caused by strains of Group A streptococcus that produce erythrogenic exotoxin
s.
. Has the same mode of transmission and age of distribution as streptococcal ph
aryngitis.
. The illness may follow a streptococcal pharyngitis, wound infections, burns,
or streptococcal skin infection.
. It begins acutely after an incubation period of 1 to 7 days.
. Initial symptoms include fever, chills, toxicity, abdominal pain, and pharyng
itis.
. The rash initially appears on the neck, axillae, and groin within 12 to 48 ho
urs, and subsequently generalizes within 24 hours.
. The rash characteristically has a punctate or finely papular texture which is
sometimes readily palpable; hence, the "sandpaper-like" description.
. The pharynx is typically erythematous, swollen and possibly covered with gray
-white exudates, strawberry tongue may be present as in cases of Kawasaki diseas
e.
. The area around the mouth appears pale in comparison with the extremely red c
heeks, giving the appearance of "circumoral pallor.
. Towards the end of the first week, desquamation begins in the face, progresse
s down the trunk, and finally extends to the hands and feet.
. The most common condition with which Kawasaki's disease is confused is scarle
t fever; however, scarlet fever will have
a positive streptococcal throat test, and normal-appearing lips, as opposed t
o negative streptococcal throat test and inflamed lips in Kawasaki's disease.
. Latex agglutination test is more rapid but less accurate in the diagnosis of
scarlet fever.
. The treatment of choice for scarlet fever is a ten-day course oif penicillin
V; however, in penicill in-allergic patients, erythromycin or clindamycin can be
used.
. Treatment of scarlet fever with penicillin doesn't prevent post-streptococcal
glomerulonephritis.
. Staphylococcal scalded skin:
______________________________
. Caused by exfoliative strains of S. aureus, and is characterized by the devel
opment of superficial flaccid bullae followed by an extensive exfoliation of the
skin.
. It is most common in infancy, and rarely occurs beyond five years of age.
. Herpangina:
_____________
. is a throat infection caused by enteroviruses, especially Coxsackie A.
. It is characterized by a high fever and a severe sore throat that may result
in a complete inability to swallow, sometimes necessitating IV hydration.
. Ulcerative lesions are found on the palate, tonsils, and pharynx Sometimes,
. these lesions appear on the palms and soles & is called (hand-foot-mouth dise
ase).
. It generally does not produce a rash after taking an antibiotic.
. Infectious mononucleosis:
____________________________
. caused by the Epstein-Barr virus.
. Sometimes detected only when the patient develops a characteristic polymorpho
us rash after taking ampicillin or amoxicillin for an apparent URT infection.
. A prodrome of malaise, fever and adenopathy, the virus is spread via the sali
va, and the condition usually resolves after 2-3 weeks.
. can also present with exudative pharyngitis and lymphadenopathy.
.N.B.:
- Group A beta-hemolytic streptococci (not Group B) is responsible for 20-25% c
ases of pharyngitis in children.
. Group B streptococcus infections in the adult are serious. these are common i
n diabetics and those with peripheral vascular disease.
. Complications include endocarditis, arthritis, pneumonia, empyema and meningi
tis.
. It does not present with a rash after taking amoxicillin.
. Toxic epidermal necrolysis:
_____________________________
. is another form of cutaneous hypersensitivity that is sometimes considered to
be a variant of Stevens-Johnson syndrome.
. Most cases are secondary to medications, such as sulfa drugs, anticonvulsants
, and NSAIDs.
. Similar to staphylococcal-scalded syndrome, it presents with extensive erythe
ma, tenderness, and blister formation, followed by denudation of the epidermis.
. Mucous membranes are severely affected, and shedding of the nails may occur.
. Stevens-Johnson syndrome:
__________________________
. A severe variant of erythema multiforme.
. Characterized by skin lesions of erythema multiforme (target lesions), follow
ed by inflammatory bullae of two or more mucous membranes.
. Sometimes, the GI, respiratory, or GU tracts may also be involved.
. Rabies:
_________
. Fatal disease that is transmitted to humans by contact with saliva or nervou
s tissue from an infected animal through a bite, open wound, or mucus membrane c
ontact.
. Bats are a major reservoir for the rabies virus, Bat bites are often so quick
and minor that the victim is not even aware.
. For this reason, any direct contact between bats and humans requires post-exp
osure rabies prophylaxis.
. People bitten by domestic animals suspected of being rabid or not available f
or observation, or
by wild carnivores like raccoons, skunks, and foxes should also receive proph
ylaxis.
. Rabbits and small rodents like squirrels, chipmunks, and rats are ra rely inf
ected with rabies,so Post-exposure prophylaxis following bites is not routine.
. Viral meningitis:
___________________
. usually self-limited inflammation of the leptomeninges caused by a viral infe
ction.
. 90% of cases are caused by non-polio enteroviruses, such as echovirus and cox
sackievirus.
. The incidence of viral meningitis decreases with increasing age Infants are m
ost commonly affected, and disease morbidity and mortality is highest in this gr
oup.
. Present with a viral prodrome of constitutional and upper respiratory symptom
(if given within 1 hour of initial antibiotic therapy) to reduce the incidence
of hearing loss with bacterial meningitis.
. N.B.:
- CSF in tuberculous meningitis also shows a mildly elevated white cell count w
ith lymphocyte predominance; the protein will be very high and glucose will be l
ow.
However, the presentation tends to be subacute rather than acute.
- The long-term neurologic sequelae associated with bacterial meningitis are:
1. hearing loss
2. loss of cognitive functions (due to the neuronal loss in the dentate gyrus
of the hippocampus)
3. seizures
4. mental retardation
5. spasticity or paresis
. TORCHs infections:
___________________
. Toxoplasmosis, rubella, CMV, HSV and syphilis.
. cause a syndrome characterized by microcephaly, hepatosplenomegaly, deafness,
chorioretinitis, and thrombocytopenia.
. Standard prenatal care for high risk women (immegrants) in USA includes:
screening for infection with syphilis, chlamydia, gonorrhea, and HIV, as well
as rubella and hepatitis B immunity.
. N.B.:
1- Folic acid supplementation prior to conception is important to prevent neur
al tube defects such as spina bifida and anencephaly.
2- Zidovudine treatment early in pregnancy in women with confirmed HIV infecti
on can reduce the risk of vertical transmission of HIV to the fetus.
Also Caesarian delivery also reduces vertical transmission.
3- Malaria during pregnancy, causeS maternal anemia, also increases the risks
of fetal loss and IUGR. Microcephaly is not associated with malaria during pregn
ancy.
4- Smoking during pregnancy carries a risk of intrauterine growth retardation,
as well as neurologic and developmental defects. Microcephaly is not a feature.
. Congenital syphilis:
______________________
. Presents early on with hepatosplenomegaly, cutaneous lesions, jaundice, anemi
a, and rhinorrhea.
. Metaphyseal dystrophy and periostitis may be seen on radiography.
. Late manifestations: frontal bossing, high arched palate, Hutchinson teeth, i
nterstitial keratitis, saddle nose, and perioral fissures.
. These late manifestations can be prevented with early treatment of the infant
. Serologic testing: initial screening (VDRL), rapid plasma reagin (RPR), (EIA)
; confirmatory testing is with treponemal tests as the (FTA-ABS) or (TPPA).
. Parenteral penicillin G is the treatment of choice for syphilis.
. Congenital toxoplasmosis:
___________________________
. Remember the classic triad of congenital toxoplasmosis: chorioretinitis, hydr
ocephalus, and intracranial calcifications.
. Microphthalmia, microcephaly, hepatomegaly, diffuse lymphadenopathy, jaundice
and diffuse petechiae may be seen.
. Mumps:
________
. Parotitis secondary to mumps.
. Orchitis is one of the most frequent complications of mumps, developing in ab
out 20% of cases.
. It is most common in postpubertal young men, ages 15-29. Because less than 15
% of orchitis cases of are bilateral, infertility is a rare complication.
. Treatment for mumps is supportive, with application of cold compresses to the
parotid area or testes.
. Other common complications of mumps are aseptic meningitis and encephalitis.
. Eczema herpeticum:
____________________
. is a form of primary herpes simplex virus infection that is usually superimpo
sed on healing atopic dermatitis lesions after exposure to herpes simplex virus.
. Numerous umbilicated vesicles over the area of healing atopic dermatitis are
typical.
. It is frequently accompanied with fever and adenopathy.
. In infants, the infection may be life-threatening, and acyclovir treatment sh
ould be initiated as soon as possible.
. Erythema toxicum:
___________________
. The healthy appearance of the neonate, the evanescent nature of the rash, and
the distinctive red halo surrounding the lesions support the diagnosis of eryth
ema toxicum.
. The presence of numerous eosinophils in the pustules is diagnostic.
. Erythema toxicum is a benign, self-limited condition usually found in newborn
s after the first 2 days of birth.
. Treatment is not necessary.
.N.B.:
- Milia are small pearly white cysts, and are distinct from the rash of erythem
a toxicum.
- Sebaceous hyperplasia presents as little yellowish papules and are commonly f
ound on the face.
- Vaccination against hepatitis B decreases the incidence of hepatocellular car
cinoma, especially,
in regions with high levels of hepatitis B infection such as Asia (china) and
Africa.
- The vaccination schedule for preterm infants should be conformed to the child
's chronologic age, not the gestational age.
- Because prematurity does not markedly change the immune response to vaccines,
and the risk of infection and complications in preterm infants is greater than
term.
- The exception is that children should be 2 kg prior to receiving the first he
patitis B vaccine (which is at birth).
- Small for gestational age infants have a weight under the 10th percentile for
gestational age at birth
- May have complications such as hypoxia, polycythemia, hypoglycemia, hypotherm
ia, and hypocalcemia.
.N.B.:
- Albendazole or mebendazole is the first-line treatment for Enterobius vermicu
Laris infection, Pyrantel pamoate is an alternative.
- Urinalysis is a preliminary investigation that should be performed first in a
. After birth, excretion of this excess water accounts for the majority of the
weight loss.
. The birth weight should be regained by 10 days of life.
. Signs of Neonate/Infant Dehydration:
--------------------------------------- Dry oral mucosa, lips, and tongue.
- Decreased number of wet diapers.
- No tears present when crying.
- Delayed capillary refill time (>2 seconds).
- Decreased skin turgor.
Managment of dehydration:
------------------------. The initial step in managing children with dehydration is to determine its se
verity.
. The ideal method of assessing dehydration is to determine the measured change
in weight because 1 kg of acute weight loss equals 1 L of fluid loss.
. A child's weight, however, changes constantly, making it difficult to obtain
an accurate recent "well" weight.
. So, the degree of dehydration often has to be determined by the clinical hist
ory and physical examination & can be divided into:
1. Mild dehydration: presents with a history of decreased intake or increased f
luid loss with minimal or no clinical symptoms.
2. Moderate dehydration; decreased skin turgor, dry mucus membranes, tachycardi
a, irritability, a delayed capillary refill (2-3 seconds),& decreased urine outp
ut.
3. Severe dehydration: cool, clammy skin, a delayed capillary refill (>3 second
s), cracked lips, dry mucous membranes, sunken eyes, sunken fontanelle (if still
present),
tachycardia, lethargy, and minimal or no urine output, Patients can present
with hypotension and signs of shock when severely dehydrated.
- Oral rehydration therapy should be the initial treatment in children with mil
d to moderate dehydration.
- Children with moderate to severe dehydration should be immediately resuscitat
ed with intravenous fluids to restore perfusion and prevent end organ damage.
- Isotonic crystalloid is the only crystalloid solution recommended for intrave
nous fluid resuscitation in children.
. Rehydration:
______________
. The signs and symptoms of hypernatremia are mainly neurologic and include let
hargy, altered mental status, irritability, and seizures.
. Hypernatremia can also cause muscle cramps, muscle weakness, and decreased de
ep tendon reflexes.
. Hypovolemic hypernatremia develops secondary to renal losses (eg, diuretic us
e, glycosuria) or extrarenal losses (eg, gastrointestinal upset, excessive sweat
ing).
. Hypervolemic hypernatremia occurs due to exogenous sodium intake or mineraloc
orticoid excess (eg, hyperaldosteronism).
. When treating a patient with hypernatremia, the sodium must be slowly returne
d to normal.
. In caseof hypernatremia and dehydration, the initial goal is to stabilize the
infant with fluid resuscitation as needed.
. When giving intravenous fluid boluses, only isotonic solutions such as normal
saline or lactated Ringer's should be used.
.N.B:
- Half normal saline (45%) and 5% dextrose are hypotonic solutions, they should
never be used for initial resuscitation.
. because they quickly exit the intravascular system and lower the sodium too r
apidly Precipitating drop in sodium levels can cause cerebral edema.
- Multiple studies have demonstrated that the expensive colloid solutions are n
o better than crystalloids at fluid resuscitation.
. Developemental processes:
___________________________
- Age: 12 months:
---------------. Fine Motor: Tow finger pincer grasp & turns several pages of a book at a time
.
. Gross Motor: Walks without assistance & - Waves bye & Climbs up on furniture.
. Language: Says 2-3 words & - Says "mama" and "dada" games (can identify each
parent).
. Social: Imitates actions & - Plays reciprocal games (peek-a-boo) & indicates
wants.
- Age: 2 year:
-------------. Fine Motor: Builds a twer of 6 cubes.
. Gross Motor: Walks up & down stairs
. Language: 200 word vocabulary & uses 2-word phrases
. Social: Follow 2 step commands.
- Age: 3 years:
---------------. Fine Motor: Copies a circle uses utenisles to feed self & stacks 9 blocks.
. Gross Motor: climbs stairs with alternating feet, rides a tricycle & kicks a
ball.
. Language: uses 3 word sentences, stats first name & 3/4 (75%) of speech is in
telligible.
. Social: wash/dry hands, helps with simple household tasks & group play.
- Age: 4 years:
---------------. Fine Motor: Copies a cross, draw a person, begins to use scissors & holds a c
rayon with a tripod grasp.
. Gross Motor: Hops on one foot with out losing balance & jumps over objects.
. Language: Counts to 10, tells stories & use lurals and prepositions.
. Social: Cooperative play, has imaginary friends & imitate adults role.
--------------------------------. Language development:
----------------------- Social smile 2 months
- Babbles 6 months
- 2 words, obeys 1-step command 1 year
- 2-3 word phrases, obeys 2-step command 2 years
. Gross motor development:
-------------------------- Holds head 3 months
- Rolls back to front and front to back 4 months
- Sits well unsupported 6 months
- Walks alone 12 months
- Walks up and down stairs without help 24 months
. Fine motor development:
------------------------- Raking grasp 6 months
- Throws object 12 months
- Builds tower of 2 blocks 15 months
- Builds tower of 6 blocks/turn pages of books 24 months
. Social development:
--------------------- Recognizes parents 2 months
- Recognizes strangers (stranger anxiety) 6 months
- Imitates action/comes when called 12 months
- Plays with other children 18 months
- Parallel play 24 months
N.B:
- Developmentally, a 12 month old should be able to walk holding onto a hand or
object. He should have a neat pincer grasp that allows him to grab small object
s.
- Language development at 1 year old consists of mama and dada said specificall
y to his parents, and at least one other word.
- Socially, a 12 month old can roll a ball and makes postural adjustments when
being dressed
. Toilet training:
__________________
. usually occurs between the ages of 2-4 years old.
. Parents should be counseled that several milestones must be met by the child
to become potty trained.
. Children must be aware of bladder filling, be able to consciously tighten the
external sphincter, have normal bladder growth, and be motivated to stay dry.
. They must also have met motor milestones to be able to walk to the toilet, si
t upright on the toilet, and the language to indicate the need to use the bathro
om.
. The child will demonstrate readiness to begin toilet training when they commu
nicate the need to urinate or defecate prior to passing urine or stool,
and when they can withhold the urine or stool for a brief period of time.
. When a child is ready, toilet training consists of placing the child on the t
oilet at regular times and using positive reinfo rcement as a reward.
. Girls are usually toilet trained faster than boys, but 90-95% of children wil
l achieve daytime continence by 5 years old.
. When a child is not ready for toilet training, forcing him to sit on the toil
et will make him more reluctant and can make potty training very difficult.
. The best step in the management of a reluctant child is to stop toilet traini
ng for several months and allow the child to become interested on his own.
. Vitamin D deficiency rickets:
_______________________________
. This condition usually presents after several months of being Vitamin D-defic
ient.
. The typical pathology is - defective mineralization of growing bone or osteoi
d tissue
. The typical patients are low-birth weight infants, unsupplemented dark-skinne
d infants, infants with inadequate sun exposure, and solely breastfed infants.
. Early manifestations: craniotabes (ping-pong ball sensation over the occiput
or posterior parietal bones), rachitic rosary, and thickening of the wrists and
ankles.
. Other findings: Harrison groove and a large anterior fontanelle.
. Diagnosis is confirmed by obtaining the serum calcifediol level (decreased),
alkaline phosphatase level (increased), and
observing the characteristic radiologic changes(cupping and fraying of the di
stal ends of long bones, and double contour along the lateral outline of the rad
ius).
. Treatment includes oral Vitamin D administration and adequate sunlight (or ar
tificial light) exposure.
. The current recommendation to prevent rickets is oral Vitamin D supplementati
on, beginning during the first 2 months of life and continuing until early adole
scence.
. Sexual developement:
______________________
. Normal sexual behaviors for young children (ages 2-5) include:
. Talking about genitals or reproduction, occasional masturbation, enjoying bei
ng naked, and curiosity about their own and other people's body parts.
. They may play "doctor" with each other or compare their bodies with other chi
ldren's bodies.
. In addition, transient cross-dressing is often common in preschool boys.
. Extensive sexual knowledge as well as simulating foreplay or intercourse are
concerning behaviors in a young child and could be indicators of sexual abuse.
. Conduct disorder:
___________________
. Characterized by disruptive behavioral patterns that violate basic social nor
ms for at least one year in patients less than 18 years old.
. It includes aggressive behavior towards others or animals, behaviors resultin
g in property damage, theft, or violations of societal rules.
. Constitutional growth delay:
_______________________________
. Constitutional growth delay is the most common cause of short stature and pub
ertal delay in adolescents.
. Affected individuals have a normal birth weight and height, but between 6 mon
ths to 3 years of age, the height growth velocity slows, and they drops percenti
les on their growth curve.
. Around 3 years of age, the child regains a normal growth velocity and follows
the growth curve at the 5th to 10th percentile.
. Puberty and the adolescent growth spurt are delayed, but eventually occur.
. The child will have a normal growth spurt and reach a normal adult height.
. Bone age radiographs show a bone age that is delayed compared to the chronolo
gical age.
. Separation anxiety:
_____________________
. Separation anxiety is a normal behavior in young children between 9-18 months
.
. usually manifests when parents leave the child or at night when the child is
put to bed.
. The child responds by crying, clinging to the parent, and becoming upset Sepa
ration anxiety usually lasts 2-4 months and gradually resolves as the child begi
ns to
understand that his parents will return.
.The initial step in the management of children with speech delay is an audiolo
gy evaluation especially when there is history of recurrent otitis media.
===================================================================== Miscellane
ous ==============================================
. Autism:
_________
. patient is usually less than 3 years old and presents with impairment in soci
al interactions and communication, delayed language development,
repetitive activities, and stereotypical behaviors.
. Treatment include special education and behavioral modification techniques, N
o pharmacological agent has been found to be useful in autism.
. Asperger syndrome:
____________________
. These kids are usually more socially aware and communicative. Although patien
ts may have features of stereotypical movements and
self-injurious behavior, their language development is normal.
. Attention-deficit hyperactivity disorder (ADHD):
__________________________________________________
. Children with ADHD have poor impulse control, inattentiveness, and motor over
activity.
. Multiple factors contribute to development of ADHD: genetics, pregnancy or bi
rth complications, maternal drug use, abnormal brain development or traumatic br
ain injury,
and psychological stressors.
. There are three subtypes of ADHD - predominantly inattentive, predominantly h
yperactive-impulsive, or combined.
. To meet the diagnostic criteria for ADHD, a child must exhibit six or more sy
mptoms of inattention or hyperactivity- impulsivity before age 7 years, with the
symptoms present for at least 6 months.
. The symptoms must cause significant impairment in functioning and must occur
in at least two different settings
. Many children with ADHD have a family history of ADHD. However, this is not n
ecessary for the diagnosis.
. Educational testing may be appropriate, as many children with ADHD have coexi
stent learning disabilities however.
. Teacher evaluations must be obtained first to establish the diagnosis of ADHD
.
. Children with ADHD can have sleep problems, which should be addressed if the
diagnosis of ADHD is confirmed by teacher evaluations.
Inattention Symptoms
Hyperactivity-lmpulsivity Symptoms
____________________
. Guidelines recommend active immunization with varicella vaccine for healthy a
dults & children exposed to varicella, ideally within the first 3- 5 days of exp
osure.
. because efficacy is decreased beyond this period.
. Because this is a live virus, the vaccine is only recommended for immunocompe
tent individuals.
. Postexposure prophylaxis with varicella-zoster immune globulin product is ind
icated in susceptible high-risk persons (lmmunocompromised patients, pregnant wo
men)
exposed to varicella within 96 hours (preferably 72 hours) of exposure, it do
es not prevent infection but effectively reduces disease severity.
. administration of any of these vaccines after these periods it will ineffecti
ve.
. Cat bites:
_____________
. Are of significant concern because these often result in deep puncture wounds
, also infection of such wounds with Pasteurella muftocida tends to develop quic
kly.
. And is associated with considerable pain, erythema, and swelling Localized ce
llulitis can develop subacutely.
. In some cases, systemic effects (e g , fever and lymphadenopathy) may arise.
. Prophylactic treatment is thus recommended for such cases. For treatment of m
inor cat bite wounds, amoxicillin/clavulanate for five days is recommended.
. Anabolic steroids:
____________________
. Are used to improve physique and athletic performance.
. Adverse effects, including acne, baldness, gynecomastia, hepatic dysfunction,
altered lipid profiles, virilization, testicular failure, and mood and behavior
changes.
. Informed consent:
___________________
. When planning to perform a procedure on a patient, informed consent must be o
btained.
. It should include an explanation of the following: the suspected diagnosis, t
he type of procedure or treatment planned,
the risks and possible complications of the procedure, and the alternative tr
eatments available.
. When a procedure needs to be performed on a minor, the minor's parent must pr
ovide informed consent.
. If the parent is a minor as well, which causes a dilemma because minors (<18
years old) are generally considered to be incapable of providing consent However
,
most states have exceptions to this rule that allow the following groups of m
inors to provide consent:
military personnel, those who are married, pregnant minors, or those who are
a parent, among others.
. In these instances, minors are considered free from their parents.
. Fetal alcohol syndrome:
_________________________
. which is caused by moderate or excessive maternal alcohol intake during pregn
ancy.
. It affects fetal growth and morphogenesis, resulting in midfacial abnormaliti
es (short palpebral fissures, epicanthal folds, long philtrum, thin upper lip bo
rder),
. cardiac defects (atrial or ventricular septal defect, persistence of arterial
canal), multiple joint anomalies, prematurity, growth retardation & mental reta
rdation.
. It is sometimes clinically difficult to differentiate it from Down's syndrome
(trisomy 21 ) since similarities include facial dysmorphism and heart malformat
ions;
however, the normal karyotype in this case rules out the latter.
. Fetal alcohol syndrome is the most common cause of mental retardation in chil
dren.
. Withdrawal symptoms are usually not seen unless the mother was drinking just
prior to delivery.
. Neonatal abstinence syndrome (NAS):
____________________________________
. The opiates that infants are most commonly exposed to are heroin and methadon
e.
. Methadone is prescribed for pregnant women who are addicted to heroin to prev
ent uncontrolled withdrawal in the fetus.
. Heroin and methadone are not associated with dysmorphic features or congenita
l anomalies in the fetus, but
prenatal exposure can lead to increased risk of intrauterine growth retardati
on, macrocephaly, sudden infant death syndrome, and neonatal abstinence syndrome
(NAS).
. NAS: presents in the 1st few days of life & characterized by irritability, a
high-pitched cry, poor sleeping, tremors, seizures,
sweating, sneezing, tachypnea, poor feeding, vomiting,and diarrhea.
. Withdrawal usually presents within 48 hours after birth for heroin withdrawal
and between 48 and 72 hours for methadone.
. The treatment for NAS: symptomatic care to calm the infant & help the infant
sleep, such as swaddling, providing small frequent feeds, &
keeping the infant in a low stimulation environment.
. Pharmacologic treatment used when supportive treatment does not control it; M
orphine can be administered & systematically weaned to help control opiate withd
rawal.
.N.B.:
- Prenatal exposure to cocaine can result in jitteriness, excessive sucking, an
d a hyperactive Moro reflex.
- Withdrawal symptoms are usually not as severe as with opiates; Long-term effe
cts on behavior, attention level, and intelligence may be seen.
. Serum sickness-like reaction:
_______________________________
. It most commonly occurs in young children following the treatment of viral in
fections with antibiotic.
. May occur 1-2 weeks after administration of certain drugs, such as penicillin
, amoxicillin or cefaclor in the setting of a viral illness.
. Prominent symptoms: fever, urticaria! rash, polyarthralgia and lymphadenopath
y.
. It derives its name from its similarities to true serum sickness, an immune-c
omplex mediated hypersensitivity reaction to non-human proteins.
. Serum-sickness like reaction is a clinical diagnosis, and should resolve with
withdrawal of the offending agent; it does not represent a true drug allergy.
. Deficiency of niacin (vitamin B3):
____________________________________
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=========================================================