Oedema and nephrotic syndrome — branching viva

Branching viva from the puffy toddler at first presentation, through the KDIGO 2021 definition and the initial prednisolone regimen, the four-week response classification that separates steroid-sensitive from steroid-resistant disease, the move to steroid-sparing therapy in the frequently-relapsing child, to the unwell nephrotic child with abdominal pain who demands recognition of peritonitis and hypovolaemia.

branching clinical structured oral2 min readSource-verified ·

Prompt

You are the paediatric registrar on the ward and in the emergency department. The examiner asks you to assess three children: a three-year-old with new periorbital and ankle oedema and heavy proteinuria; a seven-year-old who has not cleared the proteinuria after four weeks of daily prednisolone; and a five-year-old with frequently-relapsing disease who arrives with abdominal pain and fever. The examiner releases information in stages.

Stem 1 — Three-year-old with new oedema and heavy proteinuria (5 minutes)

A three-year-old boy has four days of morning periorbital puffiness and ankle swelling. A first morning urine protein-to-creatinine ratio is 290 mg/mmol, his serum albumin is 20 g/L, his blood pressure is normal, his complement is normal, and there is no haematuria. ^[1]

Branch A (examiner): State the KDIGO 2021 diagnostic criteria, and explain why the first morning sample is essential rather than a random daytime dipstick. (2 marks) ^[1] ^[10]

Branch B (examiner): Outline the initial prednisolone regimen in full, including the daily dose, the maximum, the conversion to alternate-day dosing, and the total course duration. Why is the course kept within twelve weeks? (2 marks) ^[1] ^[3]

Branch C (examiner): What will you teach the family to do at home, and what are the three danger signs that should prompt them to seek urgent review? (1 mark) ^[11]

References12Show

[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