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Medical Affairs Department of Pediatrics Clinical Practice Guideline

MANAGEMENT OF CHRONIC KIDNEY DISEASE IN CHILDREN

Document # Date CPG Formulated Next Review Date

PEDS-NEPRHO# 011 JULY 17, 2011 JULY 2013

0 ± 30.K is 0.7 2-12 years 133.8 ± 24.73 m2 CKD4— GFR between 15 and 29 mL/min per 1.55 in children and adolescent girls. GFR can be calculated as per the Schwartz formula: GFR = k X Height (cm) / sCreat .73 m2 or end-stage renal disease (ESRD) ** Kidney damage: (chronic) pathologic abnormalities in blood or urine tests or imaging studies related to the kidney.73 m2) with kidney damage** CKD2— GFR between 60 and 89 mL/min per 1.73m2 1 week 40.0 ± 27.0 13-21 years (females) 126. CKD Complications: Sodium and intravascular volume: .7 in adolescent boys. and 0.0 13-21 years (males) 140.45 for term infants <1 year. Classification: Is based on GFR for children older than 2 years as follows: . .MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 2 of 8 Definition of CKD: • Disorder that lasts ≥ 3 months with either kidney damage defined by structural or functional renal abnormalities.73 m2 CKD5 — GFR of less than 15 mL/min per 1. Identify and adequately prepare the child/family in whom renal replacement therapy will be required.0 GENERAL PRINCIPLES: Treat reversible kidney dysfunction. Prevent or slow the progression of kidney disease.73 m2 CKD3— GFR between 30 and 59 mL/min per 1.33 in premature infants and 0.In CKD 4/5 water and sodium retention may result in volume overload which needs sodium restriction. K is 0.8 >8 weeks 95. Treat the complications of CKD. Normal GFR in Children Mean GFR ± SD Age (Gender) mL/min/1.8 2-8 weeks 65.7 ± 21.CKD1 — Normal GFR (≥ 90 mL/min per 1.6 ± 22.6 ± 14.

Bone metabolism and bone disease: Subtle signs of renal osteodystrophy begin to be observed when the GFR decreases to 50% of normal (CKD3). It is associated with growth impairment. weakness. PTH level >21 pmol/L and ca <2. PTH <21 pmol/L and ca >2.Salt wasters (i.e. The aim is to maintain the serum bicarbonate level ≥ 22 mEq/L. Mixed osteodystrophy - Diagnosis: . Radiology is only helpful for monitoring severe cases. Sodium bicarbonate can be given to correct acidosis. and bony changes such as varus and valgus deformities of the long bones. b) administration of a loop diuretic. Management consists of: a) Low potassium diet. obstructive uropathy or dysplastic kidneys) require ongoing fluid and sodium replacement. Adynamic bone disease (low bone turnover) and is related to excessive suppression of the parathyroid gland.Bone biopsy is the gold standard. - . consider if persistent hypercalcemia with a serum intact PTH between 42 and 63 pmol/L. Physical findings may include muscle pain.. c) correction of metabolic acidosis and sodium polystyrene sulfonate (Kayexalate). Hyperkalemia: Hyperkalemia does not usually occur until the GFR is < 10%. Laboratory abnormalities of bone metabolism (e. Types: . Special attention should be paid to serum potassium in patients receiving ACEi/ARBs when approaching CKD5.Osteitis fibrosa cystica (high bone turnover) results from secondary hyperparathyroidism.5 mmol/L identified patients with adynamic bone lesions.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 3 of 8 .5 mmol/L identified patients with high turnover bone disease. elevated PTH) are common in CKD3 and require therapeutic interventions.g. Metabolic acidosis: Overt acidosis is characteristically present in CKD 4/5. as it may be normal even with moderate hyperparathyroidism.

4 pmol/L (normal range 0.5 mmol/L have either osteitis fibrosa or mixed osteodystrophy. Non-calcium phosphate binder (Sevelamer) should be added if hypercalcemia (s Ca >2.8 mmol/L in adolescents. CaCO3 can decrease absorption to 30-40 % if given before or during the meals.CKD2 — yearly. CKD3 — q 6 months CKD4/5 — q 3 months Phosphate: .55 mmol/L). The targeted goals: to avoid serum phosphorus below the target range because of the potential adverse effects of hypophosphatemia on linear growth.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 4 of 8 - Children on hemodialysis: all patients with a serum PTH concentration >13 pmol/L and a serum calcium value <2. Monitoring: For calcium and phosphorus the recommendations for measurements are at least: .5 to 6 pmol/L) CKD4: 7.3 to 1.In CKD4 hyperphosphatemia will usually occur unless appropriate therapy is given. - - . - Management: Level of PTH aimed for in CRF to maintain normal bone turnover are the following: . Tertiary HPT will be caused by a cycle of PTH stimulation as increased hydroxylation of Vit D induced by PTH stimulation will increase not only Ca but also PO4 absorption from the gut.4 to 11.CKD 2/3: 3.7 to 7.CKD 2 — yearly CKD3 — q 6 months CKD 4 — q 3 months CKD5 — monthly For PTH and alkaline phosphatase measurements: . CKD 5: The serum phosphorus should be maintained between 1.9 mmol/L in children 1 to 12 years of age and between 1.6 pmol/L (~up to 2x the upper NL) CKD 5: 21 to 32 pmol/L (or 3-5 times normal value) to allow normal growth.1 to 1.

Deficiency of 25-hydroxyvitamin D is common in children with CKD.1 . Based on that the target BP goals are: The NHBPEP recommended a reduction in PB to below the 90th percentile based upon the age. o Alfacalcidol (1mo-12 yr): 15-30 ng/kg/d (max 500 ng or 0. and height of the patient.The prevalence of hypertension in children with CKD is high. the serum calcium level is <2.5 mcg/d) Once Calcitriol/Alfacalcidol is started. Hypotensive symptoms while taking antihypertensive medications. serum 25-hydroxyvitamin D concentrations should be measured yearly.55 mmol/L. Ambulatory blood pressure monitoring (ABPM) should be considered with the following indications: Suspected white coat hypertension. If the PTH remains elevated after having the phosphate within the normal range. serum PTH should be measured at least every three months. the recommended starting dose for Calcitriol is based on the body weight of the child: o Weight <10 kg: 0.0. the dose of calcium-based phosphate binders should be reduced and/or therapy changed to Sevelamer. - . If the total serum calcium value exceeds 2. Vitamin D therapy should also be discontinued until the serum calcium returns to the targeted range.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 5 of 8 Calcium: .05 mcg q 48 hrs. even when the GFR is only mildly reduced. The patients with CKD 2 to 4 and serum PTH values above the target range.25 mcg/d. Target ABPM is a mean BP <50th percentile (ESCAPE trial). Vitamin D: . Hypertension: . o Weight 10 . o Weight > 20 kg: 0. Aggressive blood pressure control slows the progression of CKD.15 mcg/d.The total serum calcium should be maintained within a normal range. Episodic hypertension and Autonomic dysfunction.20 kg: 0.37 mmol/L and the serum 25-hydroxyvitamin D is >75 nmol/L. gender. Resistant hypertension.

The target hemoglobin is 11 . Monitor the Hgb (Hct) every 1-2 weeks until a stable target Hgb (Hct) and then every 4 weeks. electrolyte.8% over a 2 .12 g/dL.73m2.Anemia due to reduced kidney erythropoietin production develops when the GFR is below 30 mL/min/ 1.75) mcg/kg/week.25 to 0. Growth: . The criteria for initiating recombinant human growth hormone (rHuGH) in children with CKD include all of the following: - . Echocardiograms should be performed in all patients: a) at the initiation of dialysis. Darbepoetin alfa is a long-acting erythropoietic agent can be used at a dose of 0.LVH is commonly seen in children with CKD especially patients on dialysis as a response to mechanical or hemodynamic overload.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 6 of 8 Left ventricular hypertrophy: . The initial rHuEPO is 80 to 120 u/kg per week in 2-3 divided doses (up to 300 u/kg per week for <5 years of age or children on dialysis). Treatment: .73 m2. Anemia: . The most important cause of poor growth is inadequate intake of calories and protein leading to malnutrition. - Monitoring: . and acid-base imbalance. and c) at 3-yearly intervals thereafter. and growth hormone resistance are also important. b) once patients have achieved dry weight (ideally within 1–3 months of dialysis initiation).The expected increase in Hgb (Hct) after the initiation of rHuEPO therapy or after a dose change is between 2 . renal osteodystrophy. Serum ferritin should be kept between 100 to 800 ug/l and TSAT≥ 20. Water.4 week period. All patients receiving therapy with erythropoiesis stimulating agent (ESA) require iron supplementation to prevent the development of iron deficiency.5 (range 0. In PD patient it can be given q 2 weeks and in CKD 4 it can be given once a month. The anemia of CKD is principally normocytic and normochromic.Growth retardation occurs in up to 50% of children with GFR<50ml/min/1.

3.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 7 of 8 1. - Prepared by: Dr Issam Abou Najab.uptodate. References: . Paul A. 2009. Evaluation should be performed at presentation. 1st edition 2007. Nicholas J.com.88 and/or a height velocity SDS <-2 persists beyond 3 months despite treatment of nutritional deficiencies and metabolic abnormalities. Growth potential that is documented by open epiphyses. as of July 2011 Oxford Handbook of Pediatric Nephrology by Lesley Rees. Brogan.kidney. Consultant Pediatric Nephrologist .org/professionals/kdoqi.www. 29 (2):136-42 (Changing the frequency of administration of darbepoetin alfa (from weekly to fortnightly) maintains the hemoglobin levels in patients undergoing peritoneal dialysis). Height SDS that is <-1. al. 2. Bajo MA et. as of July 2011). Immunization: Refer to separate CPG on TOL (in process). or c) receives a kidney transplant. The use of rHuGH is continued until the child a) reaches the 50th percentile for mid-parental height. There are no contraindications for rHuGH.Abnormal lipid metabolism is common in patients with CKD and adds to the risk for cardiovascular disease (CVD) in children with CKD. Nefrologia. 4. and annually thereafter or 2-3 months following a change in treatment or subsequent to the presentation of another condition known to cause dyslipidemia. Pediatric Nephro Specialist Dr Ibrahim Al Attrach. The National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF KDOQI) Clinical Practice Guidelines (http://www. Dyslipidemia: . Webb.A. b) achieves a final adult height with closed epiphyses. Nutrition: Refer to separate CPG on TOL (in process).

Issam Abou najab Tawam/JHopkins CMO Dr.MEDICAL CLINICAL PRACTICE GUIDELINE Document #: Page 8 of 8 Approval: Name Head of the Department: Dr. Walid Kaplan Reviewed By Johns Hopkins Member: Yes Johns Hopkins Member Signature Date No . Ibrahim Al Attrach Dr. Aiman Al Rahmani Other Involved Department Head/Manager: Division Chief Dr.