AIDS presenting as focal segmental membranous glomerulopathy.

The case of a young, heterosexual man who was investigated for proteinuria is reported. A renal biopsy specimen showed a focal and segmental membranous glomerulopathy. He was later found to be HIV positive and died from cerebral infarction associated with HIV vasculitis 16 months after his initial presentation. Unusual forms of immune complex mediated glomerulopathies should alert the pathologist to the possibility of HIV associated disease.

Renal disease associated with circulating antineutrophil cytoplasm activity.

We report our detailed observations on a group of 30 consecutive patients with renal disease, histologically demonstrated glomerulitis or necrotizing vasculitis, and circulating antineutrophil cytoplasm activity (ANCA). The annual incidence of ANCA-related renal disease was seven cases per million population. The sensitivity of serum ANCA for histologically proved glomerular vasculitis was 79 per cent, with a specificity of 87 per cent. Most patients responded to treatment with cyclophosphamide and steroids but complications of therapy occurred in just over half the patients and were serious in 20 per cent. Actuarial survival at 1 year was 60 per cent. Age and dialysis requirement did not influence outcome and the only identified adverse prognostic factor was hypoxic lung disease. We conclude that the association of ANCA with renal disease is not rare and that positive serology accurately identifies a homogeneous group of patients with similar clinical, histological, and prognostic features. Separation of these patients into those with the disease entities of Wegener's granulomatosis and microscopic polyarteritis is not straightforward on clinical and histological criteria, and such a distinction does not yield useful therapeutic or prognostic information. Simple urinalysis should always be carried out in patients with undiagnosed systemic illness in order to identify renal disease. ANCA-related renal disease can be treated successfully with cyclophosphamide and steroids and elderly patients should not be excluded from treatment, including dialysis if necessary. The ANCA test is simple and quick to perform and, in the appropriate clinical setting, accurately identifies patients who may benefit from immunosuppressive treatment before a histological diagnosis can be established.

Autosomal dominant polycystic kidney disease complicated by glomerulonephritis.

Two patients with autosomal dominant polycystic kidney disease (ADPKD) and concurrent glomerulonephritis are described. Both developed nephrotic-range proteinuria and one showed a concomitant acceleration in the rate of decline of renal function. Subsequent open renal biopsy revealed membrano-proliferative type-1 and mesangio-proliferative glomerulonephritis, respectively. Nephrotic-range proteinuria in the presence of ADPKD, with or without an accompanying decline in renal function, should prompt further investigation to exclude coexisting glomerular disease.

Myoglobinuria: the importance of reaching a firm diagnosis--a patient with defective fatty acid oxidation.

A 52 year old man presented with myoglobinuria-induced acute renal failure requiring dialysis. Despite renal biopsy, the cause of the myoglobinuria was not established until he re-presented a year later with a milder episode. At this stage investigations, including a muscle biopsy, demonstrated a defect in fatty acid oxidation amenable to dietary and lifestyle advice. This report emphasizes the importance of reaching a definitive diagnosis in myoglobinuria.

Fatal cardiac tamponade complicating anticoagulant therapy in rheumatoid arthritis.

Haemopericardium with tamponade is a rare complication of anticoagulant therapy. A case of fatal haemopericardium is described in a patient with rheumatoid pericarditis in whom the precipitating factor appeared to be intravenous anticoagulant therapy.

Raynaud's syndrome and carpal tunnel syndrome.

We report three cases of Raynaud's syndrome with digital ischaemic ulceration, in association with carpal tunnel syndrome. In all cases, the aetiology of the Raynaud's syndrome was probably unrelated to the nerve compression. However, symptoms were worse on the side of the median nerve lesion in two patients and worse on the side with the most severe nerve dysfunction in the third; symptoms were relieved by carpal tunnel decompression in two patients. We suggest that carpal tunnel syndrome may exacerbate Raynaud's syndrome and should be considered particularly in patients with asymmetrical digital lesions.

Infective endocarditis with glomerulonephritis associated with cat chlamydia (C. psittaci) infection.

A patient with glomerulonephritis and endocarditis is described who had evidence of feline Chlamydia psittaci infection. Treatment with antichlamydial drugs resulted in resolution of the glomerulonephritis and the endocarditis. It is recommended that screening for chlamydia is included in the investigation of patients with suspected or obscure endocarditis.

The loin-pain/haematuria syndrome.

Nine patients with the loin-pain/haematuria syndrome are described. The previously reported clinical features of severe recurrent loin pain and tenderness in young women, the relationship of symptoms to use of oral contraceptives, and the demonstration of intrarenal vascular abnormalities by angiography are confirmed. Histology revealed minor non-specific abnormalities, and electron microscopy showed fibrin in the afferent arteriole and in glomerular capillaries of one patient only. No diagnostic changes were found on renography. In all patients except one the heparin-thrombin clotting-time was abnormal, suggesting increased platelet activity or release and providing further evidence of a vascular disorder.

A comparison of once and twice daily atenolol in hypertension.

The hypertensive action of atenolol has been studied in a randomized double-blind crossover comparison. Twelve patients showed a highly significant reduction in average supine systolic and diastolic blood pressures from pre-treatment values of 196.3/115.9 to 159.1/89.2 mmHg (26.1/15.4 kPa to 22.2/11.9 kPa) after 2 weeks on once daily atenolol. No dose-related reduction in blood pressure was seen and the single 100 mg daily dose was as effective as 100 mg twice daily or 50 mg twice daily. Blood pressures recorded after 2 weeks' atenolol were lower than those obtained at 7 days irrespective of dose.

Hypertension and renal artery stenosis with aneurysm formation in a solitary kidney in a patient with neurofibromatosis.

A case of hypertension in a patient with neurofibromatosis and renal artery stenosis with aneurysm formation in a solitary kidney is described. Studies of plasma renin activity and body sodium content are presented and have been related to findings in patients and experimental animals with renovascular hypertension. A reconstructive operative procedure is described in which the kidney is perfused in situ.

Glomerulonephritis associated with Coxiella burnetii endocarditis.

A patient with endocarditis associated with chronic Coxiella burnetii infection is described in whom glomerulonephritis developed with granular deposits containing immunoglobulins and complement in the glomeruli. The serum was notable for the variety of circulating antibodies detected, which included antibodies directed against native DNA.

Nephrotic syndrome in chronic lymphocytic leukaemia.

Two patients with chronic lymphocytic leukaemia and the nephrotic syndrome are described in whom deposits were shown in renal glomerular basement membranes in a pattern suggesting immune-complex glomerulonephritis. This renal lesion has been described in one case of squamous carcinoma of the bronchus, in one case of Burkitt's lymphoma, and in three cases of Hodgkin's disease though not previously in chronic lymphocytic leukaemia. Immune-complex glomerulonephritis is, however, a recognized finding in mice infected with leukaemogenic viruses