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Paeds Casesnephrology-urology-fluids-and-electrolytes

Paeds Cases · nephrology-urology-fluids-and-electrolytes

Chronic kidney disease and progression: Case

Clinical long case of a seven-year-old boy with posterior urethral valves and progressive chronic kidney disease, covering KDIGO staging with the Schwartz estimate, the nephroprotective bundle of blood-pressure control and ACE inhibitor therapy, the management of growth failure, anaemia, acidosis and mineral-bone disease, and planning toward pre-emptive transplantation.

paediatric nephrology long case4 min readSource-verified ·

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 7-year-old boy was born with posterior urethral valves, ablated in the neonatal period. Over two years his height has fallen from the 50th to the 9th centile. His clinic blood pressure is 118 over 78 and his first-morning urine albumin-to-creatinine ratio is 85 mg per g. Serum creatinine is 120 micromoles per litre (1.36 mg per dL), height 128 cm, bicarbonate 18 mmol per litre, haemoglobin 96 g per litre, calcium 2.20 mmol per litre, phosphate 1.7 mmol per litre, and parathyroid hormone three times the upper limit of normal. The examiner asks how you would classify and stage his disease, slow its progression, and manage his complications.

This boy has chronic kidney disease from posterior urethral valves with associated reflux and dysplasia, and he is progressing with established complications. His estimated GFR by the bedside Schwartz equation is 0.413 multiplied by 128 divided by 1.36, which is about 39 mL per minute per 1.73 square metres, placing him in GFR category G3b. His albumin-to-creatinine ratio of 85 mg per g is category A2 (moderately increased), and albuminuria is the modifiable target most closely linked to his rate of decline. [3]

Clinical findings

The pattern is one of progressive CKD with multisystem complications. His growth has crossed centile lines downward, a cardinal feature of significant CKD in childhood driven by poor appetite, chronic acidosis, anaemia, mineral-bone disease and growth-hormone resistance. His blood pressure of 118 over 78 in a 7-year-old is above the 50th percentile and likely elevated, and ambulatory monitoring is needed to confirm whether his 24-hour mean exceeds the target that the ESCAPE trial showed slows progression. His albuminuria of 85 mg per g confirms significant protein leak that both reflects and drives glomerular damage. [2]

His biochemical disturbance shows the predictable complications of declining function. The bicarbonate of 18 mmol per litre reflects chronic metabolic acidosis, which dissolves bone and worsens growth failure. The haemoglobin of 96 g per litre reflects the anaemia of CKD from reduced erythropoietin production and relative iron deficiency. His parathyroid hormone at three times the upper limit of normal, with a phosphate of 1.7 mmol per litre, indicates secondary hyperparathyroidism as part of chronic kidney disease mineral and bone disorder. Each of these is treatable, and treating each one is central to letting him grow and reach transplantation in good condition. [1]

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References4ShowHide
  1. [1]Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group KDIGO 2024 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney Int, 2024.PMID 38490803
  2. [2]Wühl E, Trivelli A, Picca S, et al. Strict blood-pressure control and progression of renal failure in children. N Engl J Med, 2009.PMID 19846849
  3. [3]Schwartz GJ, Muñoz A, Schneider MF, Mak RH, et al New equations to estimate GFR in children with CKD. J Am Soc Nephrol, 2009.PMID 19158356
  4. [4]Schaefer B, Wühl E Educational paper: Progression in chronic kidney disease and prevention strategies. Eur J Pediatr, 2012.PMID 22968936
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