Chronic Kidney Disease (CKD)

in Children
Dr Saiel Al Sarhan MD, PhD
.Consultant & Assistant Professor of Pediatrics & Pediatric Nephrology
Faculty of Medicine / Hashemite University
5th yr medical students 2017/2018
 Outlines
Definition
Pathogenesis
Stages
Etiology
Clinical presentation
Laboratory findings
Treatment
RRT
 Definition
Kidney damage for ≥3 months, as defined by structural or
functional abnormalities of the kidney, with or without
decreased GFR or GFR < 60 ml/min/1,73 m2 for > 3 months
with or without kidney damage
.
The Kidney Disease Outcomes Quality Initiative (KDOQI) working group of the National
Kidney Foundation (NKF) defined chronic kidney disease as "evidence of structural or
functional kidney abnormalities (abnormal urinalysis, imaging studies, or histology)
that persist for at least 3 months, with or without a decreased glomerular filtration rate
.(GFR), as defined by a GFR of less than 60 ml/min /per 1.73 m2

CKD is defined as abnormalities of kidney structure or function,

.present for ≥3 months, with implications for health
!! A decade of efforts to define CKD

2012 2011 2010 2009 2008 2007 2006 2005 2004 2003 2002

K/DOQI CPG for CKD: Evaluation, classification & stratification. Am J Kidney Dis 2002; 39:S1
KDIGO ‘04 CC: Definition & classification of CKD. Kidney Int 2005;67:2089
KDIGO ‘06 CC: Definition & classification of CKD. Kidney Int 2007;72:247
KDIGO ‘09 CC: Definition & classification of CKD. Kidney Int 2010; 80:17
KDIGO CPG on CKD Kidney Int Suppl 2013; 3(1)
Criteria for CKD (Either of the Following Present for >3 Months)

Markers of kidney damage (one or more) .1

;Albuminuria (albumin excretion rate [AER] ≥30 mg/24 hours -
-)albumin-to-creatinine ratio [ACR] ≥30 mg/g [≥3 mg/mmol] -
Urine sediment abnormalities -
Electrolyte and other abnormalities due to tubular disorders -
Abnormalities detected by histology -
Structural abnormalities detected by imaging -
History of kidney transplantation -

Decreased glomerular filtration rate (GFR)GFR <60 .2

.ml/min/1.73 m2 (GFR categories G3a–G5)
 Prevalence
Globally, the prevalence of chronic kidney disease (CKD) stage II or
lower in children is reported to be approximately 18.5-58.3 per
million children
The frequency of chronic kidney disease increases with age and is
.much more common in adults than children
Among children, chronic kidney disease is more common in
.children older than 6 years than in those younger than 6 years
The percentages in the NAPRTCS cohort were 19% in children aged
0-1 years; 17% in those aged 6-12 years; 33% in children aged 2-5
 .years; and 31% in those older than 12 years
 Stages
The Kidney Disease Outcomes Quality Initiative (KDOQI) recommended the following
: classification of chronic renal disease by stage (2008-2009)

Stage I disease is defined by a normal glomerular filtration rate (GFR) (> 90 mL/min
per 1.73 m 2) and persistent albuminuria

Stage II disease is characterized by a GFR of 60-89 mL/min per 1.73 m 2 and persistent
albuminuria

Stage III disease is characterized by a GFR of 30-59 mL/min per 1.73 m 2

Stage IV disease is characterized by a GFR of 15-29 mL/min per 1.73 m 2

Stage V disease is characterized by a GFR of less than 15 mL/min per 1.73 m 2or end-
stage renal disease (ESRD)
 Categories - Stages
GFR Categories in CKD(ml/min/1.73 m2)
G1: ≥90 Normal or high
G2: 60–89 Mildly decreased
G3a: 45–59 Mildly to moderately decreased
G3b : 30–44 Moderately to severely decreased
G4: 15–29 Severely decreased
G5: <15 Kidney failure
 Schwartz equation for e-GFR

CrCl (ml/min/1.73m2)=  [length (cm) x k] / Scr

k = 0.45 for infants 1 to 52 weeks old

k = 0.55 for children 1 to 13 years old
k = 0.55 for adolescent females 13-18 years old
k = 0.7 for adolescent males 13-18 years old
 Pathogenesis
Hyperfiltration injury: possible final common pathway of glomerular
destruction, independent of the underlying cause of renal injury
As nephrons are lost, the remaining nephrons undergoo an increase in glomerular .
blood flow
This compensatory hyperfiltration temporarily preserves renal function, however it.
might cause damage to the surviving glomeruli by time

Proteinuria: direct toxic effect on tubular cells, recruitment of monocyte and

.macrophages : enhancement of glomerular sclerosis and tubulointerstitial fibrosis
.Hypertension: Arteriolar nephrosclerosis, increase hyperfiltration injury
Hperphosphatemia: Deposition of calcium phosphate in the renal tissue
and vessels
Hyperlipidemia: Glomerular dysfunction through oxidant-mediated injury
 Causes of CKD in Children
:Congenital abnormalities
Aplasia, Hypoplasia, Dysplasia-
Obstructive uropathy-
Reflux nephropathy -
:Herditary conditions
Polysystic kidney disease AR -
Hereditary nephritis -
Cystinosis -
Primary oxalosis -
Congenital NS -
Glomerulonephritis
Multisystem diseases
SLE -
HSP -
HUS-
Miscellaneous
Renal vascular disease -
Renal tumors
Unknown causes
 Clinical presentation
Depends on the severity of renal impairment and the underlying disorder

Pts in the early stage of CKD may be asymptomatic unless there are signs/symptoms
:from the underlying disease or systemic disease with renal involvement

Tubulointerstitial disorder.. reduce concentrating ability –Polyuria -

Lupus Neph. Or Wegener’s..fever, rash, arthralgia, pulmonary symptoms -

Poor growth : as the CKD progress -

Signs & symptoms of uremia (severe renal impairment) : vomiting, loss of appetite, -
weakness, fatigue, anorexia, pericarditis, neurocognitive dysfunction
 Modes of presentation of CKD
Antenatal ultrasound scanning
Abdominal mass
Urinary tract infection
Enuresis
FTT
Short stature
Pallor & Lethargy
Hematuria
NS
Hypertension
Congestive heart failure
Seizures
Failure to recover from acute renal failure
Manifestations & mechanisms (pathophysiology) of CKD
Accumulation of waste products: decrease in GFR
Acidosis: impaired bicarbonate reabsrption, decreased net acid excretion
Na retention: excessive renin production, oliguria
Na wasting: tubular damage
Urinary concentrating defect: tubular damage
Hyperkalemia: decreased GFR, excessive intake, metabolic acidosis
Renal osteodystrophy : see next slide
Growth retardation: see next slide
Bleeding tendency: defective PLT function
Anemia: see next slide
Infection: granulocyte defect, impaired cellular immune function, indwelling dialysis catheter
Neuro-manifestations: uremia, aluminum toxicity, hypertension
Hypertension: overload, rennin production
Hyperlipidemia: decreased plasma lipoprotein, lipase activity
Pericarditis, cardiomyopathy: uremia, overload, hypertension
Glucose intolerance: glucose tissue resistance
Pathogenic Factors of the Anemia of CKD
Decreased erythropoiesis
Reduced availability of erythropoietin ●
*Inhibitor(s) of erythropoiesis ●
*Bone marrow fibrosis ●
*Shortened red blood cell survival
Hemolysis due to extracorpuscular factor(s) ●
Excessive blood losses
Deficiency states
Iron deficiency ● Folic acid deficiency ●
These factors seem to be exacerbated by hyperparathyroidism*
Clinical Features of Renal Osteodystrophy in Childhood
Clinical manifestations
Growth retardation ●
Bone pain ●
Myopathy ●
Skeletal deformities ●
Rickets signs in infants ●
Biochemical data
Increased serum AP activity ●
Elevated serum PTH concentrations ●
Lower serum 1,25-(OH)2-D3 ●
Radiologic abnormalities
Subperiosteal resorption ●
Epiphyseal slipping ●
Osteopenia ●
Pathologic findings
Osteitis fibrosa ●
Growth retardation in CKD ..Possible factors
Inadequate energy intake
Inappropriate protein intake
Disturbances in water and electrolytes balance
Renal osteodystrophy
Infections
Anemia
Corticosteroid therapy
Clinical evaluation
Hx of renal diseases or HTN
Growth Hx: poor linear growth
Polyuria, polydepsia, enuresis
Elevated BP
Recurrent uti’s
Antenatal diagnosed renal malformation
Unexplained anemia
Orthopedic or urological abnormalities
Seizures
Fluids and electrolytes disorders
Physical evaluation
Growth parameters
BP measurement
Assessment of pallor
Exam of extremities: Deformities (MBD), edema
Signs of hypervolemia: edema, rales, hepatic
enlargement, cardiac gallop
Cardiac auscultation: friction rub, diminished heart
sounds
 Laboratory test
CBC -
:Biochemistry -
Electrolytes -
KFT -
Protein & albumin -
Blood Ph & bicarbonate -
PTH -
Left hand & wrist x-ray -
CXR -
ECHO -
 Specific investigations - CKD
Renal tract USS -
MCUG -
Radio-isotope scans: DMSA, MAG3, DTPA -
IVU -
C3, C4, ANA, ANCA, Anti-GBM antibodies -
Renal biopsy -
White cell cystine level -
Oxalate excretion -
Treatment
:Goals

Slowing progression of kidney dysfunction **

Replacing absent or reduced kidney function **

1st goal .. Slowing progression
Optimum control of HTN .1
Control of proteinuria .2
Serum phosphorus within normal range .3
Serum ca-p within normal range .4
Prompt treatment of infections & dehydration .5
Correction of anemia .6
Minimization of regular use of NSAIDS .7
Control of hyperlipidemia .8
Fluids & electrolytes management
Sodium: as glomerular disease progress, stage4-5, salt
restriction may become necessary, as well as in case of
hypertension with edema and heart failure
Heavy electrolytes losers are those with tubulopathies, -
cystinosis : supplements
Infants on PD lose excessive Na and need supplements -
Remember … Salt depletion contributes to poor growth -

:Potassium
,restriction of oral intake
medications, kayaxalate
Acidosis: Sodium bicarbonate is used to maintain the
bicarbonate level above 22meq/l

Anemia: erythropoiesis stimulating agents are effective

:in improving the anemia of CKD
Erythropoietin , Darbepoitin -
Aim for Hb 10-12gr/dl -
Iron: iv or oral is required -
Hypertension
Hypertensive children due to fluid overload should -
follow restricted salt diet (2-3gr/day), and may be
benefit from diuretic therapy
Thiazide diuretic may be used in mild renal -
insufficiency, and loop diuretic as GFR falls
.significantly
ACE inhibitors are a good antihypertensive drug in -
proteinuric renal disease
Ca channel blockers and B blockers may also be used -
Renal osteodystrophy
Goal is to prevent bone deformity and to normalize
growth velocity using both dietary and pharmacologic
intervention
Low phosphorous diet & in infants low phosphorous -
formula
;Phosphate binders: enhance fecal excretion -
calcium carbonate -
non-calcium based binders -
Vitamin D supplementation -
Growth
Growth retardation occurs in up to 50% of children with
moderate –severe CKD (GFR< 50ml/min)
Dietary supplements orally or enterally
Gastrostomy ! Feeding
Salt supplementation
Correct acidosis, hyperparathyroidism
Give recombinant growth hormone (rhGH)
Nutrition
Ensuring adequate nutrition is one of the most important
aspects of care of the child with CKD (Pediatric renal
dietician involvement is crucial)
Energy -
Protein -
Vitamins and minerals -
see guidelines for energy intake & protein requirements(
)in patients with CKD
Vaccination
Children with CKD should receive and complete all 
routine childhood vaccines

Exception: hold live vaccine if the patient is on steroids or

other immunosuppressant… (GN!)

Administer live virus vaccines before kidney transplant

All CKD children should receive a yearly influenza vaccine

 ESRF & RRT
ESRD represents the state in which a patient’s renal
dysfunction has progressed to the point at which
homeostasis and survival can no longer be sustained
with native kidney function and maximal medical
.management

At this point Renal Replacement therapy

.RRT(Dialysis or Transplantation) becomes necessary
Treatment Options for Renal Replacement Therapy

ESRD Comfort Care

Hemodialysis Peritoneal Dialysis

Kidney Transplant
 Dialysis Options

Dialysis

Hemodialysis Peritoneal Dialysis

In-Center HD (3 x week) Manual (CAPD)

Home HD (short daily, nocturnal) Hom
Cycler (CCPD) e
 Dialysis
PERITONEAL DIALYSIS VERSUS HEMODIALYSIS
There have not been any comparative studies of peritoneal 
dialysis (PD) and hemodialysis (HD) outcomes in children with
end-stage renal disease (ESRD) to suggest superiority of one
procedure versus the other. The choice of dialysis modality is
most often based on patient and family preference, center
philosophy, and availability of the desired modality

Chronic peritoneal dialysis (CPD) is the most common dialysis   

treatment modality used to treat pediatric patients with end-
stage renal disease (ESRD), particularly in children less than
five years of age
 Peritoneal dialysis
:Principles
Solute moves down the concentration gradient across the
peritoneal membrane by diffusion and water by osmosis
(ultrafiltration, UF)
Ultrafiltration causes movement of solutes by convection

:The efficiency of PD is affected by

The peritoneal membrane -
The peritoneal microcirculation -
The dialysis compatment(type and volume of solution) -
PD Treatment

Abdominal cavity is lined by a vascular peritoneal membrane which acts as a •

semi-permeable membrane
Diffusion of solutes (urea, creatinine, …) from blood into the dialysate •
contained in the abdominal cavity
Removal of excess water (ultrafiltration) due to osmotic gradient generated by •
glucose in dialysate
Principle of PD Treatment
PD
Advantages
Ability to perform PD at home -
.Technically easier than hemo
Freedom to attend school and activities
Less restrictive diet
.Less expensive than hemo

Disadvantages
Catheter malfunction
Catheter related infections
Reduced appetite
Negative body image
Hemodialysis (The machine, dialysate and Water)
:Principles
A semi-permeable membrane allows the passage of water
and small molecular weight molecules

Solute transfer occurs by diffusion and convection

Water is removed by ultrafiltration

Principle of Hemodialysis
Vein

Artery
Hemodialysis Filter (Dialyzer)
 Kidney transplantation
The general principle is that transplantation should be
the ultimate aim for the majority of children with CKD
.stage 5
Kidney Transplantation

Iliac Fossa
 Kidney Transplantation
Kidney transplantation is the most cost-effective
modality of renal replacement
Transplanted patients have a longer life and better
quality of life
Early transplantation (before [pre-emptive] or within 1
year of dialysis initiation) yields the best results
Living donor kidney outcomes are superior to deceased
donor kidney outcomes
Early transplantation is more likely to occur in patients
that are referred early to nephrologists
Refer for transplant evaluation when eGFR < 20
mL/min/1.73m2
 Thank you

?? ..… Questions