The patient presenting with proteinuria and a history of brain tumour in childhood: is there a quietly lurking nephrological nemesis?

weeks. A neurosurgical consultant refused to accept Case report our recommendation of shunt removal as he was not convinced of the diagnosis. A week after the initiation In August 1995, an 18-year-old youth was initially of antibiotic therapy, 3 and 4 levels increased to 205 referred for the evaluation of microscopic haematuria and 11 mg/dl, respectively. However, a fortnight foldiscovered on a routine army pre-recruitment urinlowing the cessation of antibiotics, levels decreased to alysis. At the age of 8, he had undergone radiotherapy pretreatment values. A repeat CSF culture was sterile. for a brain tumour. Hydrocephalus was managed by The above antibiotic regimen was recommended folthe insertion of a ventriculoatrial (VA) shunt. On lowing which, a month later, our neurosurgeons were, presentation, physical examination was unremarkable finally, persuaded into removing the VA shunt, replawith a body temperature of 37.1°C and blood pressure cing it with a ventriculoperitoneal (VP) shunt. Culture 122/72 mmHg. At this stage, the only notable laboratof the tip of the removed shunt produced a growth of ory data were a mild leucocytosis (WBC 13 200/mm3), coagulase negative staphylococci, sensitive to both a decreased C4 level of 11 mg/dl (normal>16 mg/dl) vancomycin and rifampicin. Two weeks after shunt and urinalysis that showed numerous RBC/HPF with replacement C3 and C4 levels were 230 and 37 mg/dl, no casts or proteinuria. C3 level was 130 (normal> respectively, and have since remained in the normal 90 mg/dl ). Antistreptolysin titre, rheumatoid factor range. Currently (1 month post-surgery), microscopic and cryoglobulins were all negative. Repeated blood haematuria and proteinuria persist. cultures yielded no growth. Ultrasound showed two kidneys of normal size and echogenicity. In April 1996, proteinuria ranging from 1.4–2.8 g/day developed. 3 Comment level had decreased to 54 mg/dl, and 4 to 6 mg/dl. Serum creatinine was unchanged at 1.0 mg/dl while The introduction of shunting procedures for the treathaemoglobin had dropped from 13.4 to 10.8 g/dl. ment of hydrocephalus has led to the wide application Clinically, the patient felt well, was afebrile with no of this device in clinical practice. Shunt infection has, elevation of blood pressure. however, proved to be a troublesome complication. It Percutaneous renal biopsy was performed. On light usually presents with fever, almost a universal occurmicroscopy, accentuated lobular structure of the glomrence, often with accompanying signs of raised intraeruli with mesangial cell proliferation and thickening cranial pressure [1]. In general, either blood or CSF of the glomerular basement membrane (GBM ) was cultures are positive. Whereas, the incidence of shunt shown (Fig. 1). Immunoperoxidase demonstrated infection may range as high as 27% [1], the incidence intense staining for IgM, IgG and 3 in a granular of glomerulonephritis associated with shunt infection pattern along the GBM. Electron microscopy revealed approximates only 3% of cases [2]. This last entity, the presence of subendothelial and mesangial electron now known as shunt nephritis, was first described in dense deposits with duplication of the GBM (Fig. 2). 1965 by Black et al. [3 ] who reported two cases of the A spinal tap yielded a normal, culture negative cerebronephrotic syndrome and macroscopic haematuria assospinal fluid (CSF). Blood cultures were persistently ciated with Staphylococcus albus bacteraemia after sterile. Despite this, presenting a working diagnosis of shunt insertion. Since then, more than 80 cases have shunt nephritis, intravenous vancomycin (2 g/day) and been reported in the literature. The mean interval oral rifampicin (600 mg/day) were administered for 6 between shunt insertion and nephritis is 4.4 years, but, as in our case, much longer intervals of 10 to 14 years Correspondence and o print requests to: J. Bernheim, Department of Nephrology, Meir Hospital, Kfar-Saba, Israel. have been documented [2].