Oedema and nephrotic syndrome — branching viva

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]

Stem 2 — Seven-year-old not cleared after four weeks of steroids (5 minutes)

A seven-year-old boy started daily prednisolone 60 mg/m2/day four weeks ago for a first episode of nephrotic syndrome. His home dipsticks have shown persistent 3+ protein throughout, and he remains oedematous. His blood pressure is at the 95th percentile and his complement is normal. [5]

Branch A (examiner): What is the correct diagnostic label at this point, and how does it change the management pathway compared with the steroid-sensitive child? (2 marks) [1] [5]

Branch B (examiner): Outline the investigations you would now request, including the role of renal biopsy and genetic testing, and the first-line pharmacological agent class you would introduce. (2 marks) [5] [6]

Branch C (examiner): What is the prognostic significance of steroid resistance for this child's long-term kidney function, and how does the finding of a pathogenic genetic variant alter the counselling about treatment and transplant? (1 mark) [5] [6]

Stem 3 — Five-year-old with frequently-relapsing disease, abdominal pain and fever (5 minutes)

A five-year-old girl with frequently-relapsing steroid-sensitive nephrotic syndrome (four relapses in the last year) presents with a new relapse, now complicated by abdominal pain, fever of 38.8 degrees Celsius and tachycardia. Her capillary refill is 4 seconds and her serum albumin is 14 g/L. [11]

Branch A (examiner): What complication must you assume is present, what is the immediate investigation and empirical treatment, and why is she vulnerable to it? (2 marks) [11]

Branch B (examiner): She is also intravascularly depleted despite her oedema. State the resuscitation fluid, the dose and the accompanying drug, and explain why diuretics alone would be dangerous. (2 marks) [7]

Branch C (examiner): Once she is stabilised, what steroid-sparing options are now indicated for her frequently-relapsing course, and what is the evidence-based role of rituximab? (1 mark) [1] [4]