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Biomedical subjects

U N Bhuyan

Publications and source records attributed to U N Bhuyan.

At least 19 recordsLinked to original sources

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.

Acidosis, Renal Tubular

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.

Anticoagulants

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.

Adult

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.

Adolescent

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.

Adult

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.

Abdominal Pain

Nephronophthisis with bronchiectasis.

A boy with nephronophthisis and bronchiectasis, a previously unrecognised association is described. Electron-microscopic examination of a renal biopsy from an asymptomatic sibling revealed marked thickening and lamination of the tubular basement membranes.

Biopsy

Cryptogenic fibrosing alveolitis in children.

Cryptogenic fibrosing alveolitis (CFA) in children is supposedly a rare diffuse lung disease characterized clinically by tachypnoea, cough, poor weight gain, and cyanosis. Histologically, it is characterized by thickening of alveolar walls and infiltration of the alveolar septae with mononuclear cells and fibrous tissue. The condition is more acute in children, with fulminant course. Ten children were diagnosed as having CFA out of three hundred children seen over a period of 3 years. Diagnosis was established by characteristic clinical findings and laboratory evaluation. All the children had tachypnoea at rest and bilateral crepitations in the absence of fever. Diffuse bilateral persistent irregular shadows were noted on the chest X-ray of these children. Pulmonary function studies revealed a restrictive type of abnormality with low transfer factor for carbon monoxide in six children. Open lung biopsy was done in eight children. The biopsy material was graded according to nine morphologic variables. Administration of steroids early in the course of illness resulted in amelioration of symptoms in 60% of the patients.

Adolescent

Systemic lupus erythematosus in North Indian Asians. A prospective analysis of clinical and immunological features.

One hundred and one patients with systemic lupus erythematosus (SLE) from North Indian stock are presented. The clinical manifestations, laboratory parameters, causes of death, and survival are compared and contrasted with the other major reported series. SLE of North Indian Asians has several features comparable to those reported from the West, but other features are more similar to the SLE seen in Mongoloid races.

Antibodies, Antinuclear