Full blown nephrotic syndrome due to unilateral obstruction of the uretero‐pelvic junction

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A 45‐year‐old woman was referred to the nephrology outpatient clinic with bilateral ankle swelling. She was normotensive and physical examination was unremarkable except for bilateral pitting pedal oedema.
Blood results showed: Serum creatinine 72.49 mmol/L; albumin 29 g/L, total cholesterol (TC) 7.36 mmol/L, LDL‐C 5 mmol/L, tyrigliseride 0.95 mmol/L, and 24 h urine protein excretion 5.5 g/day. Urine sediment revealed 10–12 erythrocytes/hpf, complement levels were normal, hepatic serology and anti‐HIV were negative. Abdominal CT demonstrated gross right‐sided hydronephrosis due to obstruction of the uretero‐pelvic junction. MAG3 and 99 m‐Tc scans confirmed the obstruction in the right kidney due to UPJ narrowing with split renal functions of the right and left kidney being 45% and 55% respectively. Biopsy of the left kidney showed 25 glomeruli with four of them being globally sclerotic. There were mild thickening of the glomerular basement membranes. Direct immunofluorescence examination and congo‐red staining were negative. As minimal change disease could not be ruled out, the patient was started on oral methyl prednisolone therapy. After six weeks of treatment, proteinuria increased to 7.6 g/day and steroid therapy was slowly tapered and stopped. She was scheduled for cystoscopy and selective ureteric urine sampling to determine if the urine protein excretion was selective for one kidney. The protein leak was significantly higher in the right kidney compared to the left kidney (+++ versus +, respectively). 2 months after the placement of a double J stent, proteinuria significantly decreased. A month later proteinuria increased to 4.3 g/day because of the displaced double J stent. A new stent was inserted, followed by a progressive decline in proteinuria to 354 mg/day. The patient has undergone a successful laparoscopic pyeloplasty and is still free of proteinuria (Fig. 1).
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