Bhuyan Un

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.

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.

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.

Recurrence of idiopathic membranous nephropathy in HLA-identical allograft.

Dr. S.C. Dash, Aditional Professor & Head, Department of Nephrology, AIIMS 110029 New Delhi (India) Dear Sir, We report a case of recurrent membranous nephropathy (rMGN) in an allograft, out of 200 live-related renal transplant (LRT) we have operated so far. S.S., a 49-year-old male first presented with idiopathic nephrotic syndrome (NS) in 1984: blood pressure was normal. He was empirically treated with prednisolone (1 mg/ kg/day) for an adequate period without any response. Renal biopsy done later in November, 1985, revealed MGN. Thereafter, he received only symptomatic treatment. He developed ESRD by the end of 1987 and underwent renal transplantation in March, 1988, the donor being his HLA-identical brother sharing A2, A10, B21 and B40 antigens. On the 17th postoperative day, he was discharged from the hospital with normal renal function and urinary protein 1.3 g/day. He remained asymptomatic till the 8th month, when he developed NS with a proteinuria of 8.5 g/day and creatinine clearance of 78 ml/min. Percutaneous graft biopsy done showed MGN. Since the first case report in 1975 [1], 28 cases of rMGN have been reported so far, including the present case. Details of 23 cases could be found in the literature. Of the remaining 5 cases, in 1981 Pirson et al. [2] and Blanc-Brunat et al. [3] reported 2 cases each. In the same year, Cosyn et al. [4] also reported a de novo MGN which recurred in the 2nd and 3rd transplant. Cases of rMGN have been published mostly as case reports. In three major series, First et al. [5] reported 1 of 14 cases, Montagnino [6] 3 of 9 cases and Mirzyeka [7] 4 of 7 cases of rMGN. These cases had usually an agressive primary disease; mean duration between diagnosis and ESRD being 4.0 years. Recurrent MGN is common in males and in LRT. Patients with LRT develop rMGN earlier than patients with cadaver renal transplants (CRT). Of 23 cases, 11 allografts were from LRT, 8 being 100% HLA-matched. In cases of rMGN from haploidentical donors, A2 antigen is common in all 4 cases where HLA of patients are known. Montagnino [6] reported 3 of 6 cases of rMGN while on ciclosporin and steroid for the first time in 1989. rMGN presents as early posttransplant variable proteinuria. Hypertension has been frequently found. Renal function at onset was normal. Prognosis of rMGN is relatively poor in LRT as compared to CRT. At 3-year follow-up, our case had heavy proteinuria (6.0-8.0 g/day) with abnormal renal function (serum creatinine 4.5 mg%). It appears that a group of idiopathic MGN patients have a rapid progressive course leading to ESRD in the short period. It may be reasonable to

Spectrum of Rapidly Progressive (Crescentic) Glomerulonephritis in Northern India

36 consecutive patients whose biopsies showed significant extracapillary proliferation in the face of rapidly declining renal function were reviewed between 1967 and 1979. About 30% of the patients belonged to the pediatric age group with a male:female ratio of 2.5:1. Oliguria/anuria, hematuria, and progressive renal insufficiency were present in all cases. There was evidence of 9 cases being poststreptococcal, 2 SLE, 1 Henoch-Schönlein purpura, 2 possibly viral and 1 staphylococcal. Histologically, 29 cases had more than 60% crescents, 5 between 50 and 60% and 2 cases a little less than 50%. 28 cases were fatal in less than 10 months. 2 were lost of follow-up. 6 survivors with reversal of renal functions had 3 common factors to note, namely an antecedent disease, less crescents both in number and size, accompanied by fewer interstitial changes and early treatment. Rebiopsy in 2 survivors showed regression of histologic severity.