Recurrence of lipoprotein glomerulopathy after renal transplantation.

terized by abnormal lipoprotein deposition in glomerA 16-year-old man presented with a 7-day history of uli, usually with lipoprotein thrombi distending and headache. Direct questioning revealed a 1-month hisoccluding glomerular capillary lumina, and with a tory of tiredness, breathlessness on exertion, ankle variable degree of mesangial proliferation [1]. First oedema, and blurred vision. There was no relevant described and most frequently reported in Japan [1–5], past medical history. Family history was unremarkable it has been increasingly recognized in other racial apart from insulin-dependent diabetes mellitus and groups and in Europe over the past 10 years [6,7]. The mild hypertension diagnosed in his father when aged usual presentation is with proteinuria or nephrotic 36. His parents were from Jamaica, but he had lived syndrome together with moderate renal impairment, all his life in the United Kingdom. Examination often with hypertension, and progressing in many cases showed a fit, well muscled man. Blood pressure was to renal failure. 268/168 on repeated measurement. Fundoscopy The aetiology of the condition is uncertain, although showed bilateral papilloedema with haemorrhages. it has been associated with increased apolipoprotein There was bilateral pitting oedema to the knees, small levels, an increased prevalence of the apolipoprotein pleural effusions, and moderate left ventricular hyperE2/E3 phenotype, and with variable increases in serum trophy. Urinalysis showed 3+ protein and 1+ blood. cholesterol and triglycerides [1,3–5]. One report sugRemainder of examination was unremarkable. gests a familial predisposition [1]. It is clearly distinct Initial investigation showed sodium 134 mmol/l, from other causes of lipid accumulation within glomerpotassium 6.5 mmol/l, bicarbonate 16 mmol/l, urea uli, such as Gaucher’s disease and other hereditary 39.9 mmol/l, and creatinine 1859 mmol/l. Creatinine sphingolipoidoses. Immunohistochemical analysis indiphosphokinase and random glucose were not elevated. cates that the lipid deposits are composed mainly of He was appropriately anaemic with haemoglobin b/pre-b lipoprotein and that they represent accumula8.7 g/dl, and normochromic normocytic indices. Blood tion of VLDL and/or remnants of VLDL derived from films showed minor fragmentation but no evidence of the plasma [6 ]. The factors which determine abnormal haemolysis. White cell count was normal, platelets deposition/accumulation are, however, unknown. 92×109/l. ESR was 71 mm in the first hour, C reactive Most reports suggest that the pathogenesis of the protein <4 u (normal <10 u), uric acid 0.63 mmol/l. condition is likely to be humoral in origin, rather than Tests of liver function and coagulation were normal specific to the kidney, although no other organ systems apart from an albumin of 31 mmol/l. He was nephrotic have yet been shown to be affected. Given this, the with 6.4 g/24-h proteinuria. Chest and abdominal recurrence of lipoprotein deposits and renal disease radiographs were unremarkable, while ECG showed following renal transplantation would be of great concentric left ventricular hypertrophy, confirmed on interest. An abstract presented to the Japanese Society echocardiography. Renal ultrasound showed 9.0-cm of Nephrology in 1989 suggested that this might indeed unobstructed kidneys with increased cortical echogen-