Increased incidence of glomerulonephritis following spleno-renal shunt surgery in non-cirrhotic portal fibrosis.

In a prospective study of 200 non-cirrhotic portal fibrosis (NCPF) patients, 7% had mild proteinuria and their renal biopsies showed mild mesangial proliferative glomerulonephritis (mes-PGN). The remaining 93% biopsies were normal. However, following the insertion of a spleno-renal shunt (SRS) for portal hypertension 32% of these patients developed nephrotic syndrome in five years. Renal histology revealed mesangiocapillary glomerulonephritis (MCGN) (18.5%), mes-PGN (9%), minimal change nephropathy (3%), and chronic sclerosing GN (1.5%). Immunofluorescence showed granular deposition of IgA and C3. IgA2 was the predominant form of Ig in the glomerular deposits, indicating that IgA in the immune complexes was derived from the gastrointestinal tract. Electron microscopy revealed electron dense deposits in the mesangium. In contrast to the NCPF patients who underwent a SRS for portal hypertension, the 200 patients in our study who underwent spleno-renal shunting because of extra hepatic portal obstruction did not have renal disease, nor did they develop renal disease during the five-year post-operative follow-up. Fifty percent of the glomerulonephritis (GN) in the NCPF group progressed to renal failure in five years; 46.6% continued to have proteinuria. Low serum complement, C3 (40%) and circulating immune complexes (14.8%) were detected in the glomerulonephritis group. Our study shows that: (i) there is a high rate of the occurrence of GN following SRS in NCPF patients, but not in those with normal livers; (ii) the type of GN is primarily IgA nephropathy; and (iii) the GN could be the result of defective hepatic reticuloendothelial function in the NCPF group that is worsened by the shunting procedure.

Treatment of acute rejection in live related renal allograft recipients: a comparison of three different protocols.

We present our experience on the comparison of three different modes of steroid therapy, oral prednisolone (OP), intravenous dexamethasone (IVDX) and intravenous methylprednisolone (IVMP) in the treatment of acute rejection (AR) in renal allograft recipients. Between January 1980 and January 1992, 206 patients underwent live related renal transplantation. Before 1990, all received prednisolone (PRED) and azathioprine (AZA) only. After 1990, patients were given PRED, AZA and cyclosporine (CsA). After 1 year, CsA was stopped and patients were converted to a two-drug regimen only. Of the 206 patients, 180 (87.4%) were male and mean age was 30.3+/-8.7 years (range 14-63). During the mean follow-up of 43.5 months, 178 episodes of AR were seen in 121 patients. Each episode was considered as a separate entrant in the study. Conventional immunosuppression was given in 151 episodes and 27 episodes were on triple-drug therapy. Diagnosis of AR was made by clinical, sonography, nuclear scan with or without graft biopsy evidence. Of the 178 AR, 110 (61.8%) were within 3 months, 36 (20.2%) were between 3 months and 1 year and 32 (18%) were after 1 year. OP was given in 11 cases while IVDX and IVMP were given in 48 and 119 cases respectively. Overall, 154 (86%) showed either a complete or partial response to antirejection therapy. Response to therapy was 91, 90 and 85% in OP, IVDX and IVMP groups respectively. There was no statistical difference in response rate in different groups. There was also no difference in side effects in three different groups. Our data suggest that it is the high dose of steroid rather than mode of therapy which is responsible for therapeutic benefit in treatment of AR.

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.

Clinical profile and course and outcome of late acute rejection episodes in living-related-donor renal allograft recipients.

We prospectively monitored clinical data and renal function at monthly intervals in 165 patients who had received living-related-donor renal allografts in our institution between January 1981 and December 1991 and had a functioning allograft for 1 year or longer. During a mean follow-up period of 47.2 (range 13-155) months, 32 patients (17.2%) developed late acute rejections, of which 14 (43.7%) were asymptomatic. Amongst the symptomatic late acute rejections, worsening of hypertension was the commonest finding, being present in 11 (61.1%) patients, followed by oliguria in 8 (44.4%) and weight gain in 7 (38.8%) patients. Of these 32 late acute rejections, as many as 28 (87.5%) showed a response to antirejection therapy with high-dose steroids: 5 (15.6%) a complete response and 23 (71.9%) a partial response. The response rate was 100% if it was the first acute rejection (20% complete and 80% partial), 78.6% if it was the second (14.3% complete and 64.3% partial), and no or only a partial response to treatment if it was the third acute rejection episode. On long-term follow-up, patients who had responded to to antirejection treatment had a significantly better graft survival as compared with nonresponding patients: 76 and 27%, respectively. Our observations suggest that routine monitoring of the renal function at frequent intervals is essential for early diagnosis and treatment of acute rejections, even during the late posttransplant period. The chances of a response to antirejection therapy are higher during the first episode of late acute transplant rejection as compared with second or a third late rejection event.

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.

Primary repair of colorenocutaneous fistula in patients with genitourinary tuberculosis.

Two cases of colorenocutaneous fistula due to genitourinary tuberculosis are presented. They were successfully managed by single-stage surgery (nephroureterectomy, fistulectomy, and primary repair of the colon) and antitubercular treatment. The literature is briefly reviewed.

Acute renal failure and severe hypertension in children with renal thrombotic microangiopathy.

We observed 12 boys and 1 girl, 5-15 years old, presenting with acute renal failure (ARF) and severe hypertension. They constituted 4.9% of all patients with ARF. There was no preceding diarrheal or respiratory prodrome. Clinical and laboratory findings were indicative of hemolytic uremic syndrome (HUS) in 4, but obscure in 9 others. Renal biopsies revealed variably severe occlusive thrombotic microangiopathy (TMA) affecting predominantly interlobular arteries in 8 and both arteries and glomeruli in 5 cases. Glomerular crescents and cortical necrosis were not seen. Following supportive therapy, 8 progressed to or died of uremia; 2 showed persistent proteinuria, 1 moderate hypertension and 2 complete recovery. Our observations indicate that renal TMA without a prodromal illness and typical features of HUS may present with ARF, proteinuria and severe hypertension, and is associated with high mortality.

Renal tubular acidosis preceding systemic lupus erythematosus.

A 10-year-old girl with distal renal tubular acidosis (RTA) for 4 years (adequately treated for 3 years) developed clinical features suggesting systemic lupus erythematosus (SLE) with supportive laboratory evidence. She had heavy proteinuria and a decreased creatinine clearance (CCr). Renal biopsy showed diffuse proliferative and sclerosing glomerulonephritis with severe tubulointerstitial changes. Following treatment with corticosteroids and cyclophosphamide, she had a clinical remission, an increase in CCr and recovery from systemic acidosis. It is likely that distal RTA in this patient was a manifestation of SLE.

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.

Idiopathic adult focal segmental glomerulosclerosis: a clinicopathological study and response to steroid.

A total of 65 adult cases (53 males, 12 females) with biopsy-proven focal segmental glomerulosclerosis (FSGS) were studied. Hypertension, ascites and haematuria were seen in 13, 12 and 24 cases, respectively. Decreased creatinine clearance at presentation was found in 9 cases. Mean proteinuria per day, serum cholesterol and total protein were 7.5 +/- 4.3 g, 388.95 +/- 213.4 mg% (10.11 +/- 5.55 mmol/l) and 5.27 +/- 1.1 g% (0.527 +/- 0.11 milligram), respectively. Mesangial proliferation was seen in 13 cases and hilar sclerosis in 5. Fifty percent showed positive immunofluorescence; IgM in 10, C3 in 8, and IgG in 2. Forty-two cases could be followed (mean 32 months), out of which 38 had nephrotic syndrome and were treated with prednisolone; 58% showed response (31% complete remission and 27% partial remission). One patient in each group of responders and nonresponders had renal failure at the end of follow-up. Hypertension, degree of proteinuria, mesangial proliferation, degree of tubular atrophy and immunofluorescence findings did not significantly affect the response to steroids. We conclude that a group of patients with idiopathic adult FSGS has a favourable response to steroids, which cannot be predicted clinically.

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.

Crescentic glomerulonephritis in children: a review of 43 cases.

Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 +/- 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in greater than 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocellular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis.