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Chronic kidney disease in children
Dr Ahmed Mohamed Adan
INTRODUCTION
• Chronic kidney disease (CKD) refers to a state of irreversible kidney
damage and/or reduction of kidney function that is associated with
progressive loss of function over time.
DEFINITIONS AND CLASSIFICATIONS
There are two pediatric exceptions:
(1) the criteria for duration longer than 3 months does not apply to
infants younger than 3 months, and
(2) the glomerular filtration rate (GFR) criteria of less than 60
mL/min/1.73 m2 cannot be used in children under 2 years of age since
developmental immaturity in this age group is associated with mean GFR
of less than this value. Thus, the diagnostic criteria for reduced GFR in
this particular population must be compared to age-matched normative
values.
• Modified Schwartz = 0.413 × length in cm
Serum creatinine in mg/d
The normal GFR in children is 90 - 130 ml/min/1.73m
2
CKD is divided into 5 stages according to GFR ( NKF KDOQI GIUDELINES )
GFR
mL/min/1.73 m2DescriptionStage
More than 90
Slight kidney damage with
normal or increased filtration
G 1
60-89
Kidney damage with Mild decrease in
GFR
2
30-59Moderate decrease in GFR3
15-29Severe decrease in GFR4
Less than 15 or in dailysis
Kidney failure; requiring
dialysis or transplantation
5
CKD is divided into 5 stages according to GFR:
GFR
mL/min/1.73 m2DescriptionStage
More than 90
Slight kidney damage with
normal or increased filtration
G 1
60-89
Kidney damage with Mild decrease in
GFR
2
30-59Moderate decrease in GFR3
15-29Severe decrease in GFR4
Less than 15 or in dailysis
Kidney failure; requiring
dialysis or transplantation
5
ETIOLOGY
 in children <5 yr old is most commonly a
result of congenital abnormalities of the
kidney and urinary (CAKUT) such as renal
hypoplasia, dysplasia, or obstructive
uropathy
Additional causes include :-
congenital nephrotic syndrome, prune belly
syndrome, cortical necrosis, focal segmental
glomerulosclerosis,
autosomal recessive PCKD , HUS.
renal vein thrombosis,
 After 5 yr of age, acquired diseases (various
forms of glomerulonephritis – FSGS -lupus
nephritis) and inherited disorders (familial
juvenile nephronophthisis, Alport
syndrome).
CKD
related to metabolic disorders
(cystinosis, hyperoxaluria)
Copyrights apply
Pathophysiology of CKD
MECHANISMSMANIFESTATION
Decrease in glomerular filtration rateAccumulation of nitrogenous waste
products
Impaired bicarbonate reabsorption
Impaired ammonia synthesis
Decreased net acid excretion
Metabolic Acidosis – Normal AG or
ed AG
Excessive renin production
Oliguria
Sodium retention
Solute diuresis
Tubular damage
Sodium wasting
Solute diuresis
Tubular damage
Urinary concentrating defect
MECHANISMSMANIFESTATION
Decrease in glomerular filtration rate
Metabolic acidosis- extracellular shift of K+
Excessive potassium intake
Hyporeninemic hypoaldosteronism
Hyperkalemia
Impaired renal production of 1,25-
dihydroxycholecalciferol
Hyperphosphatemia
Hypocalcemia
Secondary hyperparathyroidism
Renal osteodystrophy – high turnover
most common 2 to hyperparathyroidism
Inadequate caloric intake
Renal osteodystrophy
Metabolic acidosis
Anemia
Growth hormone resistance
Growth retardation
MECHANISMSMANIFESTATION
Decreased erythropoietin production- stage 3-4
Iron deficiency
Folate deficiency
Vitamin B12 deficiency
Decreased erythrocyte survival
Chronic blood loss from uremia-induced platelet
dysfunction .
Chronic inflammation
Secondary hyperparathyroidism- BM fibrosis
Accumulation of inhibitors of erythropoiesis
Anemia- normocytic normochromic anemia with
no reticulocyte response
Defective granulocyte function
Impaired cellular immune functions
Indwelling dialysis catheters
Infection
MECHANISMSMANIFESTATION
Gastroesophageal reflux
Decreased gastrointestinal motility
Serositis to uremia
Gastrointestinal symptoms (feeding intolerance,
abdominal pain)
Volume overload
Excessive renin production
Hypertension
Decreased plasma lipoprotein lipase activity-
uremia inhibits lipoprotein lipase and hepatic lipase leads to lack od
degradation of lipids .
Hyperlipidemia
Uremic factor(s)
Hypertension
Fluid overload
Pericarditis, cardiomyopathy
Tissue insulin resistanceGlucose intolerance
The goals of therapy in children with CKD are:
1. Treat reversible kidney disease
2. Slow progression to ESRD
3. Manage complications
4. Prepare for RRT- in whom renal replacement therapy will be required
MANAGEMENT
Multidisciplinary Services : Medical , Nursing , Social service Nutritional
Psychological
Slowing the progression of kidney dysfunction
1) Optimum Control of Hypertension
2) Control of proteinuria
3) Serum Phosphorus within normal range
4) Serum Calcium-Phosphorus Product < 5 mmol2
/l2
5) Prompt treatment of infectious complications and dehydration
6) Correction of anemia
7) Avoidance of cigarette smoking
8) Minimization of regular use of NSAIDs
9) Control of hyperlipidemia
10)Dietary Protein restriction
Dietary Management
Goals –
a) Reduce nitrogen intake
b) Maintain nitrogen balance
c) Cover essential amino acid requirement
d) Supply enough calories
Energy: Intake equivalent to the RDA of healthy children of the same age
• Infant : 100 – 120 kCal/kg/day
• Children : 80 – 100 kCal/kg/day
• 55 – 60% from carbohydrates
• 30% from fats
• 10% from proteins Proteins of high biological value or ‘good quality’ are preferred (are
metabolized primarily to usable aminoacids rather than to nitrogenous wastes) and are found
mainly in eggs, milk, meat, fish and poultry.
Nutrition
Anemia become manifested at a GFR less than 35 ml/min/1.73 m2
ESA- Recombinant Human Erythropoietin((Epogen, Procrit, Eprex) is
usually initiated when the patient's Hb concentration falls bellow 10 g/dL ,
at a dose of 50 – 100 unit/kg S.C. one to three times weekly.
An alternative option to rHuEPO is darbepoetin alfa (Aranesp), a longer-
acting agent administered at a dose of 0.45 μg/kg/wk. The chief advantage
of this agent is that it may be dosed once weekly to once monthly because
of its extended duration of action.
• Iron supplementation :- Oral or Intravenous.
oral : 3 – 5 mg/kg/day of elemental iron(max 150 – 300 mg/day)
ANAEMIA
• Control of levels of blood phosphate – most important factor in
prevention & treatment of secondary hyperparathyroidism .
• The target phosphorus level for adolescents is between 3.5 and 5.5 mg/ dL,
children 1-12 yr of age it is 4-6 mg/dL ,
Children and adolescents should follow a low-phosphorus diet .
The cornerstone of therapy for renal osteodystrophy is vitamin D administration.
– Therapy may be initiated with 0.01-0.05 µg/kg/24 hr of calcitriol.
– Phosphate binders: • Calcium salts [calcium carbonate & calcium acetate]
• Sevelamer hydrochloride
CKD - MBD
Hypertension
ACE inhibitors are the antihypertensive medication of choice in all children with proteinuric
renal disease.
Angiotensin-II blockers have been shown to be effective in controlling BP and in slowing
disease progression in patients with diabetic nephropathy.
–Sodium bicarbonate tablets ( 650mg =8meq ) is used to maintain the
serum bicarbonate level above 22 mEq/L.
– Growth problems (especially short stature) are very commen in CKD
children.
– CKD children have an apparent growth hormone resistant state with
elevated GH levels but decreased insuline-like growth factor-I (IGF-I).
Acidosis
Growth problems
Long-term Follow Up
close monitoring of a patient's clinical and laboratory status
 Blood studies to be followed routinely include:
 serum electrolytes
 blood urea nitrogen (BUN)
 creatinine
 calcium
 phosphorus
 albumin
 alkaline phosphatase
 Hb and Htc
 Periodic measurement of intact parathyroid hormone (PTH) levels and
roentgenographic studies of bone may be of value in detecting early evidence of
renal osteodystrophy
 Echocardiography should be performed periodically to identify left ventricular
hypertrophy and cardiac dysfunction that can occur as a consequence of the
complications of CKD
• Nelson TEXTBOOK of PEDIATRICS EDITION 20
• Clinical Pediatric Tmephrology Third Edition
• Uptodate 22 version
References

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Chronic kidney disease in children

  • 1. Chronic kidney disease in children Dr Ahmed Mohamed Adan
  • 2. INTRODUCTION • Chronic kidney disease (CKD) refers to a state of irreversible kidney damage and/or reduction of kidney function that is associated with progressive loss of function over time. DEFINITIONS AND CLASSIFICATIONS
  • 3. There are two pediatric exceptions: (1) the criteria for duration longer than 3 months does not apply to infants younger than 3 months, and (2) the glomerular filtration rate (GFR) criteria of less than 60 mL/min/1.73 m2 cannot be used in children under 2 years of age since developmental immaturity in this age group is associated with mean GFR of less than this value. Thus, the diagnostic criteria for reduced GFR in this particular population must be compared to age-matched normative values. • Modified Schwartz = 0.413 × length in cm Serum creatinine in mg/d The normal GFR in children is 90 - 130 ml/min/1.73m 2
  • 4. CKD is divided into 5 stages according to GFR ( NKF KDOQI GIUDELINES ) GFR mL/min/1.73 m2DescriptionStage More than 90 Slight kidney damage with normal or increased filtration G 1 60-89 Kidney damage with Mild decrease in GFR 2 30-59Moderate decrease in GFR3 15-29Severe decrease in GFR4 Less than 15 or in dailysis Kidney failure; requiring dialysis or transplantation 5
  • 5. CKD is divided into 5 stages according to GFR: GFR mL/min/1.73 m2DescriptionStage More than 90 Slight kidney damage with normal or increased filtration G 1 60-89 Kidney damage with Mild decrease in GFR 2 30-59Moderate decrease in GFR3 15-29Severe decrease in GFR4 Less than 15 or in dailysis Kidney failure; requiring dialysis or transplantation 5
  • 6. ETIOLOGY  in children <5 yr old is most commonly a result of congenital abnormalities of the kidney and urinary (CAKUT) such as renal hypoplasia, dysplasia, or obstructive uropathy Additional causes include :- congenital nephrotic syndrome, prune belly syndrome, cortical necrosis, focal segmental glomerulosclerosis, autosomal recessive PCKD , HUS. renal vein thrombosis,  After 5 yr of age, acquired diseases (various forms of glomerulonephritis – FSGS -lupus nephritis) and inherited disorders (familial juvenile nephronophthisis, Alport syndrome). CKD related to metabolic disorders (cystinosis, hyperoxaluria)
  • 8.
  • 9. Pathophysiology of CKD MECHANISMSMANIFESTATION Decrease in glomerular filtration rateAccumulation of nitrogenous waste products Impaired bicarbonate reabsorption Impaired ammonia synthesis Decreased net acid excretion Metabolic Acidosis – Normal AG or ed AG Excessive renin production Oliguria Sodium retention Solute diuresis Tubular damage Sodium wasting Solute diuresis Tubular damage Urinary concentrating defect
  • 10. MECHANISMSMANIFESTATION Decrease in glomerular filtration rate Metabolic acidosis- extracellular shift of K+ Excessive potassium intake Hyporeninemic hypoaldosteronism Hyperkalemia Impaired renal production of 1,25- dihydroxycholecalciferol Hyperphosphatemia Hypocalcemia Secondary hyperparathyroidism Renal osteodystrophy – high turnover most common 2 to hyperparathyroidism Inadequate caloric intake Renal osteodystrophy Metabolic acidosis Anemia Growth hormone resistance Growth retardation
  • 11.
  • 12. MECHANISMSMANIFESTATION Decreased erythropoietin production- stage 3-4 Iron deficiency Folate deficiency Vitamin B12 deficiency Decreased erythrocyte survival Chronic blood loss from uremia-induced platelet dysfunction . Chronic inflammation Secondary hyperparathyroidism- BM fibrosis Accumulation of inhibitors of erythropoiesis Anemia- normocytic normochromic anemia with no reticulocyte response Defective granulocyte function Impaired cellular immune functions Indwelling dialysis catheters Infection
  • 13.
  • 14. MECHANISMSMANIFESTATION Gastroesophageal reflux Decreased gastrointestinal motility Serositis to uremia Gastrointestinal symptoms (feeding intolerance, abdominal pain) Volume overload Excessive renin production Hypertension Decreased plasma lipoprotein lipase activity- uremia inhibits lipoprotein lipase and hepatic lipase leads to lack od degradation of lipids . Hyperlipidemia Uremic factor(s) Hypertension Fluid overload Pericarditis, cardiomyopathy Tissue insulin resistanceGlucose intolerance
  • 15. The goals of therapy in children with CKD are: 1. Treat reversible kidney disease 2. Slow progression to ESRD 3. Manage complications 4. Prepare for RRT- in whom renal replacement therapy will be required MANAGEMENT Multidisciplinary Services : Medical , Nursing , Social service Nutritional Psychological
  • 16. Slowing the progression of kidney dysfunction 1) Optimum Control of Hypertension 2) Control of proteinuria 3) Serum Phosphorus within normal range 4) Serum Calcium-Phosphorus Product < 5 mmol2 /l2 5) Prompt treatment of infectious complications and dehydration 6) Correction of anemia 7) Avoidance of cigarette smoking 8) Minimization of regular use of NSAIDs 9) Control of hyperlipidemia 10)Dietary Protein restriction
  • 17. Dietary Management Goals – a) Reduce nitrogen intake b) Maintain nitrogen balance c) Cover essential amino acid requirement d) Supply enough calories Energy: Intake equivalent to the RDA of healthy children of the same age • Infant : 100 – 120 kCal/kg/day • Children : 80 – 100 kCal/kg/day • 55 – 60% from carbohydrates • 30% from fats • 10% from proteins Proteins of high biological value or ‘good quality’ are preferred (are metabolized primarily to usable aminoacids rather than to nitrogenous wastes) and are found mainly in eggs, milk, meat, fish and poultry. Nutrition
  • 18.
  • 19.
  • 20. Anemia become manifested at a GFR less than 35 ml/min/1.73 m2 ESA- Recombinant Human Erythropoietin((Epogen, Procrit, Eprex) is usually initiated when the patient's Hb concentration falls bellow 10 g/dL , at a dose of 50 – 100 unit/kg S.C. one to three times weekly. An alternative option to rHuEPO is darbepoetin alfa (Aranesp), a longer- acting agent administered at a dose of 0.45 μg/kg/wk. The chief advantage of this agent is that it may be dosed once weekly to once monthly because of its extended duration of action. • Iron supplementation :- Oral or Intravenous. oral : 3 – 5 mg/kg/day of elemental iron(max 150 – 300 mg/day) ANAEMIA
  • 21. • Control of levels of blood phosphate – most important factor in prevention & treatment of secondary hyperparathyroidism . • The target phosphorus level for adolescents is between 3.5 and 5.5 mg/ dL, children 1-12 yr of age it is 4-6 mg/dL , Children and adolescents should follow a low-phosphorus diet . The cornerstone of therapy for renal osteodystrophy is vitamin D administration. – Therapy may be initiated with 0.01-0.05 µg/kg/24 hr of calcitriol. – Phosphate binders: • Calcium salts [calcium carbonate & calcium acetate] • Sevelamer hydrochloride CKD - MBD
  • 22. Hypertension ACE inhibitors are the antihypertensive medication of choice in all children with proteinuric renal disease. Angiotensin-II blockers have been shown to be effective in controlling BP and in slowing disease progression in patients with diabetic nephropathy.
  • 23. –Sodium bicarbonate tablets ( 650mg =8meq ) is used to maintain the serum bicarbonate level above 22 mEq/L. – Growth problems (especially short stature) are very commen in CKD children. – CKD children have an apparent growth hormone resistant state with elevated GH levels but decreased insuline-like growth factor-I (IGF-I). Acidosis Growth problems
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  • 25.
  • 26. Long-term Follow Up close monitoring of a patient's clinical and laboratory status  Blood studies to be followed routinely include:  serum electrolytes  blood urea nitrogen (BUN)  creatinine  calcium  phosphorus  albumin  alkaline phosphatase  Hb and Htc  Periodic measurement of intact parathyroid hormone (PTH) levels and roentgenographic studies of bone may be of value in detecting early evidence of renal osteodystrophy  Echocardiography should be performed periodically to identify left ventricular hypertrophy and cardiac dysfunction that can occur as a consequence of the complications of CKD
  • 27.
  • 28. • Nelson TEXTBOOK of PEDIATRICS EDITION 20 • Clinical Pediatric Tmephrology Third Edition • Uptodate 22 version References