Renal cell carcinoma in a child: case report and brief review of literature.

Renal cell carcinoma is common malignancy in the older age but extremely uncommon in childhood. Here in is reported a case of renal cell carcinoma in a eight year old female because of its rarity in children.

Thrombotic microangiopathy with hypertension and acute renal failure in children (a typical hemolytic uremic syndrome).

Thus I would like to conclude by saying that an idiopathic form of obliterative renal arteriopathy account for the rare presentation of severe hypertension and progressive renal failure with or without overt hemolytic anemia and thrombocytopenia in children. It can be labelled as primary malignant nephrosclerosis (NScl) or atypical HUS, based on primary thrombotic angiopathy. This, essentially intimal changes, is seen in diverse conditions and appears to result from primary endothelial injury followed by intimal exudation, thrombosis, and repair by fibrosis. Persistent or recurrence of this process form the basis of progressive obliterative arteriopathy. The result is renal ischemia and renin-angiotensin mediated hypertension. Establishment of a vicious circle would further accelerate HT and lead to end stage renal failure. Early recognition and prompt therapeutic intervention might prove beneficial.

Falciparum malaria complicating cholestatic jaundice and acute renal failure.

Nine patients of acute falciparum malaria with severe hyperbilirubinemia developed acute renal failure (ARF). All of them had evidence of intrahepatic cholestasis and needed hemodialysis for several weeks; 7 survived and 2 died, one due to cerebral malaria, the other multiple organ failure. Interestingly, referal diagnosis did not include malaria as a cause of ARF in 8 out of 9 patients.

Anti-neutrophil cytoplasmic antibody (ANCA) in necrotising vasculitides with renal involvement.

Anti-neutrophil cytoplasmic antibody (ANCA) was detected in sera of 20 out of 32 patients with necrotising vasculitides involving the kidneys and other organs. It was of high titre and cytoplasmic type in Wegener's granulomatosis (8/9); and of low titre and cytoplasmic or perinuclear type in systemic polyarteritis (6/12), renal limited polyarteritis (5/9) and pulmonary renal syndrome (1/2). It fell to low or undetectable levels on clinical improvement or remission with cyclophosphamide therapy. Its detection was helpful in diagnosis and management of vasculitides with renal and extrarenal manifestations.

Outcome of patients with pre-existing hepatitis B virus infection following renal transplant.

Twenty four patients who were HBsAg positive prior to renal transplantation were studied with respect to their hepatic and renal outcome. The kidneys in all patients were obtained from living related donors. The standard immunosuppressive therapy consisted of azathioprine and prednisolone. Post-renal transplant follow-up varied from 32-86 months (mean 55 +/- 9). Renal functions, liver functions, HBsAg and HBsAb status was closely monitored during follow-up. One patient developed acute hepatitis four months after transplantation; this resolved in 10 weeks. Four patients showed transient elevations of liver enzymes. The results of liver biopsy in 16 cases revealed, normal histology (8), virus in hepatocytes (5), and acute hepatitis (3). None of the patients had evidence of chronic liver disease. Two patients died due to chronic rejection/chronic renal failure and two patients died due to septicaemia. Five living patients have mild to moderate chronic rejection. It was concluded that pre-existing HBsAg carrier state in transplant patients had no deteriorating effect on the liver. The mortality was related to chronic rejection and septicaemia.

Wegener's granulomatosis in northern India.

Till recently, Wegener's granulomatosis (WG) was considered a rare disease in India. Over the last 5 years we studied 13 proven cases of WG. This paper describes the details of the disease as seen in these patients, and response to corticosteroid and cyclophosphamide therapy, and compares these observations with a Western and an Indian study.

IgA associated glomerulonephritis.

Immunohistological analysis of 1146 renal biopsies revealed IgA associated glomerulonephritis (IgAGN) in 83 (7.24%) patients (33 children, 50 adults). Clinical features were unusually severe in a high proportion. Nephrotic syndrome (NS) responding poorly to prednisolone was found in 24%, hypertension (HT) in 39%, and azotemia in 34% of patients. NS was slightly more frequent in children than in adults, but HT and azotemia occurred twice as often in adults as in children. Histologically, extensive glomerular crescents and sclerosis were prominent. In addition, moderate arteriolitis and arteriolosclerosis and marked tubulointerstitial nephropathy were notable features. Thus, a low incidence and marked severity characterized IgAGN in this study.

Intermittent intravenous pulse cyclophosphamide treatment in systemic lupus erythematosus.

To determine the efficacy and safety of intermittent intravenous pulse cyclophosphamide in patients of severe systemic lupus erythematosus (SLE), 50 patients having severe/refractory lupus nephritis, vasculitis or neuropsychiatric manifestations were treated with 3 weekly pulses of cyclophosphamide for 6 such pulses. This treatment was found to be associated with significant and sustained improvement during a 2 yr follow up with respect to the mean renal activity score, individual renal parameters (proteinuria, erythrocyturia, and serum creatinine levels), focal neurological manifestations, vasculitic lesions, antinuclear antibody titers, complement component C3, anti-dsDNA antibodies levels and ESR. There was a sustained decrease in the overall mean disease activity score, and the mean daily dose of prednisolone (pretreatment 32.62 mg daily to 3.75 mg daily after 24 months). There was a significant decline in the percentage and absolute B cell count after 7, 14 and 21 days of this treatment. Effect on other lymphocyte subsets (CD3+, CD4+ and CD8+) was not marked. Pulse cyclophosphamide could therefore be an effective and less toxic form of treatment in patients with SLE having severe lupus nephritis, focal neurological lesions or vasculitis.

Henoch-Schonlein syndrome in northern Indian children.

In order to evaluate clinical features and renal pathological findings of Henoch-Schonlein syndrome (HSS) in northern Indian Children, we studied 47 such cases. The mean age at onset was 8.5 yr; sex ratio (M:F) 2.6:1. The clinical features were purpuric rash (96%), abdominal pain (64%), Henoch-Schonlein nephritis (51%) and arthralgias (47%). Patients younger than 6 yr also showed urticarial rash or edema of scalp and extremities. Henoch-Schonlein nephritis (HSN) and abdominal symptoms were more common in older cases. The manifestations of HSN were asymptomatic hematuria and/or proteinuria (n = 15), acute nephritic syndrome (n = 6), and nephrotic syndrome (n = 3). The severity of clinical manifestations correlated with the renal pathologic findings. On follow up, 29% cases showed renal impairment. The prognosis was poor in patients with the acute nephritic or nephrotic syndrome and crescents in more than 50% glomeruli. Combination of clinical data and renal biopsy findings are important in assessing the long-term outcome in cases with HSN.