Paeds SAQs · nephrology-urology-fluids-and-electrolytes
Oedema and nephrotic syndrome — formative SAQs
Two formative SAQs on childhood nephrotic syndrome: a four-year-old with a first presentation of periorbital and ankle oedema and heavy proteinuria, testing the KDIGO 2021 definition, the initial prednisolone 60 mg/m2/day regimen and the four-week response classification; and a six-year-old with frequently-relapsing steroid-sensitive nephrotic syndrome who develops abdominal pain and fever, testing the recognition and management of spontaneous bacterial peritonitis and hypovolaemia.
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SAQ 1 — Four-year-old with first presentation of oedema (10 marks, 15 minutes)
A four-year-old boy is brought to the emergency department by his parents, who describe three days of increasing puffiness around his eyes in the morning and swelling of his ankles by the evening. He had a cold the previous week. He is afebrile, his blood pressure is 95/60 mmHg, and he has periorbital and ankle oedema with a slightly distended abdomen. A random urine dipstick shows 4+ protein. His first morning urine protein-to-creatinine ratio is 320 mg/mmol, his serum albumin is 18 g/L, his creatinine is 42 micromoles per litre, and his complement C3 is normal. [1]
a) State the KDIGO 2021 diagnostic criteria for nephrotic syndrome in children, and explain why the first morning urine protein-to-creatinine ratio, rather than a random daytime dipstick, is the correct confirmation test. (3 marks) [1] [10]
b) Assuming minimal change disease, outline the initial prednisolone regimen you would prescribe, including the dose, the maximum daily dose, the point at which you convert to alternate-day dosing, and the total course duration. State the finding at four weeks that would define the steroid response. (4 marks) [1] [3]
c) Name the three life-threatening complications of nephrotic syndrome, and explain the physiological basis for the increased risk of pneumococcal peritonitis in this child. (3 marks) [11] [10]
You have read the opening of this SAQ. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.
References12Show ledgerHide ledger
- [1]Rovin BH; Adler SG; Barratt J; et al Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int, 2021.PMID 34556300
- [2]Rovin BH; Caster DJ; Cattran DC; et al Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int, 2019.PMID 30665569
- [3]Teeninga N; Kist-van Holthe JE; van Rijswijk N; et al Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome. J Am Soc Nephrol, 2013.PMID 23274956
- [4]Iijima K; Sako M; Nozu K; et al Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial. Lancet, 2014.PMID 24965823
- [5]Trautmann A; Vivarelli M; Samuel S; et al IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol, 2020.PMID 32382828
- [6]Tullus K; Webb H; Bagga A; et al Management of steroid-resistant nephrotic syndrome in children and adolescents. Lancet Child Adolesc Health, 2018.PMID 30342869
- [7]Rheault MN; Zhang L; Selewski DT; et al AKI in Children Hospitalized with Nephrotic Syndrome. Clin J Am Soc Nephrol, 2015.PMID 26450933
- [8]Kerlin BA; Haworth K; Smoyer WE; et al Venous thromboembolism in pediatric nephrotic syndrome. Pediatr Nephrol, 2014.PMID 23812352
- [9]Boyer O; Schaefer F; Haffner D; et al Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group. Nat Rev Nephrol, 2021.PMID 33514942
- [10]Bagga A; Mantan M Nephrotic syndrome in children. Indian J Med Res, 2005.PMID 16106086
- [11]Gipson DS; Massengill SF; Yao L; et al Management of childhood onset nephrotic syndrome. Pediatrics, 2009.PMID 19651590
- [12]Zhu H; Qi J; Schoepf J; et al Prevalence and Associated Risk Factors of Pulmonary Embolism in Children and Young Adults With Nephrotic Syndrome: A Chinese Large Cohort Study. J Thorac Imaging, 2021.PMID 34269751