Chronic Kidney Disease

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CKD Basics - Tiny Kidneys, Big Trouble

  • Definition: Kidney damage OR GFR < 60 mL/min/1.73m² for ≥ 3 months.
  • Pediatric Focus:
    • CAKUT (Congenital Anomalies of Kidney and Urinary Tract) is the primary cause.
    • Growth failure & hypertension are key complications.
  • KDOQI Staging (GFR in mL/min/1.73m²):
    • Stage 1: GFR ≥ 90 (with kidney damage)
    • Stage 2: GFR 60-89 (mild ↓)
    • Stage 3: GFR 30-59 (moderate ↓)
    • Stage 4: GFR 15-29 (severe ↓)
    • Stage 5: GFR < 15 or dialysis (kidney failure)

⭐ The most common method for eGFR estimation in children is the Schwartz formula, which relates eGFR to height and serum creatinine.

  • Formula: $eGFR = k \times \frac{Height (cm)}{Serum Creatinine (mg/dL)}$.

  • Early Clues: Often subtle; e.g., polyuria, enuresis, poor growth.

CKD Causes - The Usual Suspects

Pediatric CKD Etiology Pie Chart

  • CAKUT (Congenital Anomalies of Kidney & Urinary Tract): Most common (~50% of peds CKD)
    • Renal dysplasia/hypoplasia
    • Obstructive uropathy (e.g., PUV, UPJ obstruction)
    • Reflux nephropathy (VUR)
  • Glomerulonephritis (GN): Significant group (~15-20%)
    • FSGS (common in SRNS)
    • Chronic GN (e.g., IgA nephropathy, MPGN)
    • Systemic: Lupus nephritis, HSP nephritis
  • Hereditary Nephropathies: Genetically determined (~10-15%)
    • Alport syndrome (COL4A defect)
    • ARPKD (Autosomal Recessive Polycystic Kidney Disease)
    • Nephronophthisis
    • Cystinosis
  • Other Causes: Diverse; includes:
    • HUS (Thrombotic microangiopathy)
    • Renal vascular (e.g., RVT, RAS)
    • Severe AKI sequelae (e.g., cortical necrosis)

⭐ Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) are the leading cause of pediatric CKD and ESRD in most countries.

CKD Signs & Symptoms - Spotting the Signals

  • Presentation often insidious; non-specific in early stages.
  • Early Clues:
    • Growth failure: frequently the first indicator.
    • Anorexia, nausea, vomiting.
    • Polyuria, nocturia, secondary enuresis (impaired concentrating ability).
    • Pallor (anemia from ↓ erythropoietin).
    • Unexplained fatigue, lethargy.
  • As GFR Declines:
    • Hypertension.
    • Edema (peripheral, periorbital).
    • Bone pain, deformities (renal osteodystrophy, rickets).
    • Pruritus.
    • Neurological: headache, poor concentration, seizures (uremic encephalopathy).
    • Electrolyte imbalances (e.g., hyperkalemia, acidosis).

⭐ Growth failure is often the earliest and most prominent clinical manifestation of CKD in children, significantly impacting quality of life.

CKD Management - The Balancing Act

  • Core Goals: Delay progression, manage complications, prepare for Renal Replacement Therapy (RRT).
  • Key Interventions (The Balance):
    • Blood Pressure: Target <90th percentile or <130/80 mmHg. ACEi/ARBs often used.
    • Anemia: Iron, Erythropoiesis-Stimulating Agents (ESAs). Target Hb 10-12 g/dL.
    • CKD-MBD: Phosphate binders, Vitamin D sterols. Control P, Ca, PTH.
    • Growth: Recombinant human Growth Hormone (rhGH) if GFR <50 ml/min/1.73m².
    • Acidosis: Oral alkali (e.g., NaHCO₃). Target serum bicarbonate >22 mEq/L.
    • Nutrition: Adequate calories; protein, Na⁺, K⁺, P, fluid restriction as per stage.
    • Immunizations: Crucial; follow specific schedules.
  • Renal Replacement Therapy (RRT):
    • Dialysis: Peritoneal (PD) often preferred in younger children; Hemodialysis (HD).
    • Transplantation: Optimal long-term solution.

⭐ Kidney transplantation offers the best long-term outcome and quality of life for children with ESRD, improving growth and neurocognitive development.

CKD and Anemia Pathophysiology

High‑Yield Points - ⚡ Biggest Takeaways

  • CAKUT (Congenital Anomalies of Kidney and Urinary Tract) is the leading cause of pediatric CKD.
  • Growth failure is a cardinal sign, requiring prompt intervention.
  • Anemia (due to ↓ erythropoietin) is frequent and significant.
  • CKD-MBD causes renal osteodystrophy, affecting growth and bone integrity.
  • Hypertension is common, accelerates CKD progression and cardiovascular risk.
  • Estimate GFR in children using the height-based Schwartz formula.
  • Proteinuria is a key marker of kidney damage and predicts progression.

Practice Questions: Chronic Kidney Disease

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Flashcards: Chronic Kidney Disease

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AR congenital nephrotic syndrome (Finnish type) is caused by a mutation in the _____ gene

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AR congenital nephrotic syndrome (Finnish type) is caused by a mutation in the _____ gene

nephrin (NPHS1)

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