Endocrine Abstracts

A Multicystic dysplastic kidney (MCDK) is a congenital, renal, cystic transformation usually diagnosed perinatally with 1:1000-4,000 incidence¹. The natural history of MCDK is disputed with involution¹, enlargement and development of hypertension², infection and malignant transformation reported in the literature. We describe the incidental detection of An occult MCDK was detected in a 25-year-old chef who presented with a 4 month history of diarrhoeal episodes and left flank discomfort. He had noted a sensation of fullness in the flank for a number of years but had not sought medical investigation. It had increased in size and discomfort with the onset of diarrhoeal episodes. He had a 13 year history of Type I Diabetes Mellitus Medications included Novorapid 8iu/10iu/8iu, glargine 22iu nocte and lisinopril 2 mg daily. Blood pressure was 160/103 mmHg, and bilateral pre-proliferative retinopathy with neovascularisation. Abdominal palpation revealed a large left flank mass, confirmed on CT Abdomen and a non-functioning left kidney on DMSA scan. Laboratory studies revealed striking polycythaemia (Hb 21 g/dL), elevated erythropoietin level 36 mIU/ml (normal range 6–25), HbA1c 12.2%, diabetic proteinuria (0.16 grams/24 hours) and glomerular hyperfiltration GFR 130 mls/min/1.73 m². Preoperatively laser treatment and repeated venesection was required to manage worsening diabetic retinopathy and secondary polycythaemia. Following nephrectomy, stabilisation of retinopathy, normalization of haemoglobin and an improvement in hypertension control was observed. This case strengthens the arguement² for removal of all MCDKs in childhood to prevent complications in adulthood.

1. Rabelo EAS et al. Conservative management of multicystic dysplastic kidney: clinical course and ultrasound outcome. Jornal de Pediatria 2005 81 400–404.

2. Webb NJA et al. Unilateral multicystic dysplastic kidney: the case for nephrectomy. Archives of Diseases of Childhood 1997 76 31–34.