Oedema and nephrotic syndrome — structured clinical encounter

Structured encounter testing the approach to a four-year-old presenting with new periorbital and ankle oedema and heavy proteinuria: the KDIGO 2021 confirmation, the assumption of minimal change disease and the initial prednisolone regimen, the education of the family in home urine testing, and the vigilance for the three complications of peritonitis, thromboembolism and acute kidney injury.

structured clinical encounter

Prompt

A four-year-old boy is referred to your general paediatric clinic with three days of morning puffiness around the eyes and ankle swelling by the evening. He had an upper respiratory tract infection the previous week. He is afebrile and well in himself. His blood pressure is 95/60 mmHg, he has periorbital and ankle oedema, and his abdomen is slightly distended with shifting dullness. A random urine dipstick shows 4+ protein. His first morning urine protein-to-creatinine ratio is 340 mg/mmol, his serum albumin is 17 g/L, his creatinine is 40 micromoles per litre, and his complement C3 is normal. There is no haematuria.

Task 1 — Confirm the diagnosis and assume the cause (3 minutes)

State the KDIGO 2021 diagnostic criteria for nephrotic syndrome, and confirm that this child meets them using his first morning urine protein-to-creatinine ratio and serum albumin. Explain why minimal change disease is the assumed cause in a child of this age with this picture, and why a renal biopsy is not part of the first-line work-up here. [1] [10]

Task 2 — The initial prednisolone regimen (4 minutes)

Outline the initial prednisolone regimen you would prescribe, including the daily dose, the maximum daily dose, the trigger for converting to alternate-day dosing, and the total course duration. Explain to the examiner why the course is kept within twelve weeks and what the Teeninga trial contributed to that decision. [1] [3]

Task 3 — The family conversation and home monitoring (3 minutes)

Describe the education you would give the parents about daily home urine dipstick testing, salt restriction, and the three danger signs that should prompt urgent review — abdominal pain with fever, breathing difficulty or leg swelling with tenderness, and reduced urine output with drowsiness. Explain in language the parents can use what nephrotic syndrome is and what the expected course over the coming months will be. [11]

Task 4 — Vigilance for the complications (5 minutes)

The child returns six weeks later with a relapse and now has abdominal pain, fever and tachycardia. Outline the immediate assessment and management of suspected spontaneous bacterial peritonitis, the recognition and resuscitation of intravascular hypovolaemia with 20 per cent albumin and furosemide, and the features that would raise the suspicion of a thromboembolic event. Explain to the parents why their child is vulnerable to these complications despite looking oedematous. [11] [7] [8]

References

[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

Oedema and nephrotic syndrome — structured clinical encounter

structured clinical encounter

Task 1 — Confirm the diagnosis and assume the cause (3 minutes)

Task 2 — The initial prednisolone regimen (4 minutes)

Task 3 — The family conversation and home monitoring (3 minutes)

Task 4 — Vigilance for the complications (5 minutes)

References

[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