Association of type I (insulin-dependent) diabetes mellitus, autoimmunity, antinuclear antibody, and membranoproliferative glomerulonephritis.

In a large series of patients with insulin-dependent diabetes mellitus who were screened for autoantibodies, two patients were positive for antinuclear antibodies. Both of these patients developed severe renal disease with the renal biopsy findings of membranoproliferative glomerulonephritis. Multiple autoantibodies and circulating immune complexes were demonstrated in their sera. There was evidence suggesting complement consumption. This article illustrates that immune complex glomerulonephritis can occur in some patients with IDDM, particularly in those with antinuclear antibody and polyendocrine involvement, and that renal biopsy in such cases may have prognostic and therapeutic importance.

Reversible cerebellocerebral disorder in primary hemochromatosis.

Reversible cerebellar dysfunction is described in a patient with primary hemochromatosis. The electroencephalographic abnormalities initially recorded became normal after repeated phlebotomies. Both cerebellar dysfunction and electroencephalographic changes were present without clinical signs of hepatic failure, but may be related to the altered hepatic function tests.

Induction of abortion by intrauterine administration of prostaglandin F2 alpha and histopathological studies of gestational sac.

PIP: 124 patients were selected for termination of pregnancy by (PGF) prostaglandin F2alpha administration. PG was administered by the extraamniotic route in 81 patients, whose length of gestation ranged from 8-16 weeks. PG was administered intraamniotically in 43 patients whose length of gestation ranged from 14-20 weeks. In the extraamniotic group, abortion occurred in 89% of the cases within 36 hours with a mean induction-abortion interval of 18 hours. In the intraamniotic group, abortion occurred in 86% of the cases within 48 hours with a mean induction-abortion interval of 20 hours. Histopathological studies on gestational sacs were performed in 3 groups of patients: in group 1 abortions were induced by PGF2alpha administered extraamniotically; in group 2 abortions were induced by PGF2alpha administered intraamniotically; and in group 3 specimens were obtained by hysterotomy. Edema of villi was more often observed in group 1 and increased vascularization in group 2.^ieng

Prognostic significance of renal angiitis in systemic lupus erythematosus (SLE).

Analysis of 80 cases of SLE with renal involvement revealed the following. Angiitis involving arterioles and interlobular arteries was observed in 17 out of 42 cases with diffuse proliferative lupus GN. This was not observed in other types of lupus GN. Out of 17 cases of angiitis, 7 were complicated by thrombosis. Irregular deposits of immunoglobulins and complement were demonstrated in the vessel walls and in luminal thrombi and suggested an immune-complex origin. Fibrinogen was also demonstrable frequently. The vascular lesions were associated with severe glomerular and tubulointerstitial injury and a poor prognosis. A relatively favorable outcome was observed in SLE without renal angiitis.

Immunopathology, extent and course of glomerulonephritis with crescent formation.

Ten out of 66 cases of glomerulonephritis (GN) with variable crescent formation appeared to be of post-streptococcal origin. Immunofluorescent studies of biopsies from 42 of the patients showed findings suggestive of immune complex glomerular injury in 86% whilst there was no evidence of immunologic or nonimmunologic injury in the remainder. Anti-GBM GN was not demonstrable, although it can not be certain that there were no cases amongst the patients whose biopsies were not examined by immunofluorescence. Conservative measures were adequate for the treatment of cases with less than 50% crescents. In the others, with greater than or equal to 50% crescents, appreciable improvement was observed with anticoagulation, immunosuppression and dialysis. The mortality was 30% in cases with 50-80% and 65% in cases with greater than 80% crescents. No appreciable difference in survival was observed between post-streptococcal and idiopathic cases. The single most important factor in prognosis, regardless of etiology, appeared to be the extent of crescent formation. Tubulo-interstitial damage was contributory. Necrotizing angiitis, present in 4 cases, affected the course adversely.

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.

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.

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.

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.

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.

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.

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.

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.