+nephrotic Syndrome

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Nephrotic Syndrome
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❑ Introduction
es ❖ Nephrotic syndrome is kidney disease with proteinuria, hypoalbuminemia,
is and edema. Nephrotic-range proteinuria is 3.5 grams per day or more.
❖ There are many specific causes of nephrotic syndrome. These include
kidney diseases such as minimal-change nephropathy, focal
glomerulosclerosis, and membranous nephropathy. Nephrotic syndrome
can also result from systemic diseases that affect other organs in addition
to the kidneys, such as diabetes, amyloidosis, and lupus erythematosus.
Nephrotic Syndrome
❖ Nephrotic syndrome may affect adults and children, of both sexes and of
any race. It may occur in typical form, or in association with nephritic
syndrome. The latter it can leads glomerular inflammation, with hematuria
and impaired kidney function.
Nephrotic Syndrome
❑Classification
❖Nephrotic syndrome can be primary, being a disease specific to the kidneys, or it can be
secondary, being a renal manifestation of a systemic general illness. In all cases, injury to
glomeruli is an essential feature.
❖Primary causes of nephrotic syndrome include the following, in approximate order of frequency:
⮚Minimal-change nephropathy (etiology is not known controlled/ mediated by T cells,
lymphokines is released mre and it increase the permeability of podocytes leading to
protein loss, occur in mostly children )
⮚Focal glomerulosclerosis (does not involve whole nephron like in segment , there is
scar formation leading to fibrosed area, and leads to protein loss through proteinuria
also haematuria can by rupture of small arterioles )
⮚Membranous nephropathy( due to complement activation ie C5-9 (C5b) it activates
MAC leads to release of protease and antioxidant leading to damage to nephron and
lead to nephrotic syndrome )/ immune complex deposition
⮚Hereditary nephropathies (
Nephrotic Syndrome
❖ Secondary causes include the following, again in order of approximate
frequency:
⮚ Diabetes mellitus (if for prolonged time , increased free sugar develop in
small arterial leading aneurysm and it can also rupture and leading to
protein leakage , can develop diabetic nephropathy)
⮚ Lupus erythematosus (the autoantibody can cause inflammatory changes,
ischemic necrosis at nephron)
⮚ Amyloidosis.(huge protein production leads to destruction of basement
membrane)
⮚ Viral infections (eg, hepatitis B, hepatitis C, human immunodeficiency
virus [HIV] ) (antigen antibody complex damage glomerular membrane )
Nephrotic Syndrome
❑Other Causes of nephrotic syndrome:
❖ Blood clot in a kidney vein. (blocking arteries leading to ischemia hence
necrosis membrane damages )
❖ Heart failure. (thromboembolism can occur blocking the arteries leading
to decreased blood perfusion there at nephron )
Nephrotic Syndrome
❑ Pathophysiology:
❖ NS is believed to have an immune pathogenesis. Studies have shown
abnormal regulation of T-cell subsets and expression of a circulating
glomerular permeability factor.
A circulating factor also play a role in the development of proteinuria such

as IL-4, IL-12, IL-13, IL-15, IL-18, interferon-γ, tumor growth factor
(TGF)-β, vascular permeability factor and tumor necrosis factor (TNF)-α.
rect
min
attack
Nephrotic Syndrome
oedema: Urinary protein
is losses exceed synthetic
capacity of liver leads to
Urinary protein losses
exceed synthetic capacity of
❑ Clinical Presentation liver hence then reduced
oncotic pressure and edema
❖ Symptoms
⮚ In children, facial swelling is a common presenting feature,
with periorbital oedema often being the first evidence that something is
wrong; oedema may progress to involve the whole body.
⮚ Adults tend to present with peripheral oedema affecting the ankles and Hyperc
legs, which may progress to involve the whole body. loss of in
(antithro
⮚ Some times frothiness of urine. and incre
⮚ Hypercoagulability( because of lossof clotting factorse.g thrombin and procoagu
Venous t
increase in hepatic production of fibrinogen) may manifest as venous or
arterial thrombosis, eg deep vein thrombosis( due to edema or
hypercoagulability ), myocardial infarction.
Nephrotic Syndrome
❖ Sign
⮚ Oedema (oedema of dependent parts or generalised oedema are the main
clinical findings): periorbital oedema (facial oedema may be found in
children), lower- limb oedema, oedema of the genitals, ascites.
⮚ Tiredness.
⮚ Breathlessness: pleural effusion (occasionally, severely hypoalbuminaemic
cases may have pleural effusions or ascites), fluid overload (high jugular
venous pressure), acute kidney injury (acute renal failure).
⮚ Breathlessness with chest pain: pulmonary embolism, myocardial
infarction
Nephrotic Syndrome
❑ Physical Examination
❖ Edema is the predominant feature of nephrotic syndrome and initially develops
around the eyes and legs. With time, the edema becomes generalized and may
be associated with an increase in weight, the development of ascites, or pleural
effusions.
❖ Hematuria (hematuria pt can get anemia which can lead to lethargy bc in
advance case glomerulosclerosos can occur then micro or macro hematuria
develops )and hypertension manifest in a minority of patients.
❖ Additional features on exam will vary according to cause and as a result of
whether or not renal function impairment exists. Thus, in the case of
longstanding diabetes, there may be diabetic retinopathy, which correlates
closely with diabetic nephropathy. If the kidney function is reduced, there may
be hypertension and/or anemia.
Nephrotic Syndrome
❑ Differential Diagnoses
❖ AGN
❖ IgA Nephropathy
❖ Diabetic Nephropathy
❖ Heart failure
❖ Liver failure
❖ Acute fluid overload
❖ Metastatic cancer
Nephrotic Syndrome
❑Investigation
⮚ CBC with ESR and CRP
⮚ Urine tests – 24 hrs deposite urine (measure protein amnt)
⮚ FBS
⮚ Renal function test
⮚ LFT with lipid profile( due to hyperlipidemia)
⮚ CXR P/A view (if pt is suffering from pleural edema or pleural effusion)
⮚ Ultrasound KUB region (exclude kidney stone cause it leads to
hematuria )
⮚ CT or MRI
Nephrotic Syndrome
❑ Management principles
❖ Diet and fluids:
⮚ Reduce salt intake in the diet (avoid processed foods and adding salt to
food).
⮚ Give a diet with adequate calorific intake and sufficient protein content (1-
2 g/kg daily). (check creatinin level cause nephrotic syndrome lead to
acute renal failure )
⮚ Fluid restriction is not usually necessary (if severe enough to need this
then the patient may need admission)
Nephrotic Syndrome
❖Oedema:
▫ Oedema is treated through diuretic therapy with furosemide (~1
mg/kg/day) ±spironolactone(~2 mg/kg/day).
▫ Check weight regularly to assess response to diuretics and ensure fluid
retention is not worsening, or that the patient is over-diuresed.
Nephrotic Syndrome
❖ Cholesterol-reducing drug-atorvastatin
⮚ Initial dose: 10, 20 or 40 mg orally once a day. The 40 mg starting dose is
recommended for patients who require a reduction in LDL-cholesterol of
more than 45%.
Dose adjustments should be made at intervals of 2 to 4 weeks.
Maintenance dose: 10 to 80 mg orally once a day.

Nephrotic Syndrome
❖ Steroid therapy like Prednisolone (t0 reduce systemic inflammation)
60mg/m2 body surface area/day in 4 divided doses for 4 wks followed by
40mg/m2/day every alternative day for next 4 wks.
⮚ For relapse repeat above course.
⮚ For frequent relapse 3mg/kg body weight/day for 8 wks.
Nephrotic Syndrome
❖ Blood pressure medications(anti hypertensive drug)
❖ Antibiotics. Antibiotics can help control infections caused by bacteria.
Some antibiotics like Tab.Cefuroxime 500mg 12 hourly for 7 to 14 days.
❖ For venous thrombosis Heparin 20 IU/kg/hour i.v sos.
Nephrotic Syndrome
❑Complications
❖ Thromboembolism.
❖ High blood cholesterol and elevated blood triglycerides.
❖ HTN.
❖ Acute kidney failure or Chronic kidney failure.
❖ Infections.

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DR.

A circulating factor also play a role in the development of proteinuria such

Dose adjustments should be made at intervals of 2 to 4 weeks.

Maintenance dose: 10 to 80 mg orally once a day.

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