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Biomedical subjects

A Moudgil

Publications and source records attributed to A Moudgil.

At least 37 records · Page 2Linked to original sources

Treatment of antineutrophil cytoplasmic autoantibody-positive systemic vasculitis and glomerulonephritis with pooled intravenous gammaglobulin.

Antineutrophil cytoplasmic autoantibody (ANCA) is considered a serological marker for disease activity in patients with ANCA(+) systemic vasculitis. Recently, ANCA has been implicated as a pathogenic antibody that may be associated with neutrophil degranulation and release of lytic enzymes. Since intravenous gammaglobulin (IVIG) is known to contain antiidiotypic antibodies to ANCA, which could decrease the activity of the later, we chose to treat two patients with symptomatic ANCA(+) systemic vasculitis and glomerulonephritis with high-dose IVIG. The first patient, a 66-year-old man, developed rapidly progressive renal failure despite treatment with intravenous (IV) cyclophosphamide. The second patient, a 14-year-old boy, had relapsed 3 months after cessation of treatment with prednisone and cyclophosphamide. Both patients improved dramatically after treatment with IVIG, with the former recovering renal function within 11 days of therapy. In both patients, a concomitant reduction in serum ANCA titers was also observed. The second patient is currently in a sustained remission 14 months after his last IVIG dose on no other medication. These cases provide clinical evidence that IVIG has therapeutic benefit in modifying the immune-mediated injury associated with ANCA(+) systemic vasculitis and glomerulonephritis. In addition, IVIG may provide an additional safe therapeutic option to clinicians treating patient's with ANCA(+) vasculitis and glomerulonephritis who are not responsive to or are experiencing toxicity from conventional therapy.

Adolescent↗

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↗

Hemolytic uremic syndrome in children in northern India.

We observed 73 patients with the hemolytic uremic syndrome (HUS) in 9 years (1980-1988), comprising 34% of patients with acute renal failure treated over the same period. There were 53 boys and 20 girls; 59% were below the age of 2 years and 33% between 2 and 5 years. Acute, usually severe dysentery, responding poorly to various antibiotics, was the prodromal illness in 80%, whereas 12% had watery diarrhea. Most patients had severe renal involvement with anuria in 56% and oliguria in 30%. A polymorphonuclear leukocytosis was present in 85% of cases, but had no correlation with the highest levels of blood urea. Coagulation abnormalities suggesting consumption coagulopathy were found in 24 of 30 cases. The results of stool culture showed Shigella species in 7 cases and nontyphoidal Salmonella in 9. Escherichia coli were isolated in 11 cases, but were not further characterized. Renal biopsy showed total or patchy cortical necrosis in 20 of 50 cases. The patients were managed with supportive care, including transfusion of fresh blood or plasma and dialysis as required. The mortality was 60%, being chiefly related to the duration of renal failure and presence of renal cortical necrosis, whereas persistent dysentery and infections were complicating factors. The presence of convulsions and coagulation defects had no relation to the outcome. Our observations indicate that HUS in children in northern India is mostly related to dysentery, likely to be shigellosis, and is usually associated with severe renal damage and a high death rate.

Acute Kidney Injury↗

Type O renal glucosuria.

We observed a 7-year-old boy with virtual absence of renal tubular glucose reabsorption (type O renal glucosuria). Glucose titration studies in his family revealed severe type A renal glucosuria in a younger brother, a mild type A defect in the mother and normal glucose reabsorption in the father; thus a spectrum of renal glucose transport defects was observed in members of the same family.

Biological Transport↗

Acute renal failure in north Indian children.

The spectrum and outcome of acute renal failure (ARF) were studied in 205 children aged between 1 month and 12 yr. There were 145 boys and 60 girls; 23 per cent were below 1 yr and 49 per cent between 1 and 4 yr. The main causes of ARF were haemolytic uraemic syndrome (HUS) in 36 per cent, serious infections in 19 per cent, acute gastroenteritis and dysentery in 17 per cent, glomerulonephritis (GN) in 13 per cent and intravascular haemolysis (IVH) in 6 per cent. Most patients with HUS, serious infections and gastroenteritis were below 5 yr, whereas GN and IVH occurred in older children. HUS was mostly associated with dysentery; Shigella and several other pathogens were isolated from stools in 35 per cent. In most patients with HUS disseminated intravascular coagulation and renal cortical necrosis were present, with a high mortality. The outcome was also poor in infants with serious infections. IVH occurred in patients with G-6-PD deficiency. In such patients and in those with post-streptococcal GN the prognosis was good. Crescentic GN had a poor outcome. Our observations highlight the common and serious nature of ARF in India. However, most of the underlying causes are preventable.

Acute Kidney Injury↗

Normal ionized calcium, parathyroid hypersecretion, and elevated osteocalcin in a family with fluorosis.

Sera from five patients with skeletal fluorosis were investigated for total calcium, ionized calcium, phosphate, alkaline phosphatase, 25 hydroxyvitamin D (25 OHD), 1,25 dihydroxyvitamin D (1,25[OH]2D), parathyroid hormone, and osteocalcin concentrations. Total and ionized calcium concentrations were normal in four and subnormal in one, but PTH concentration was elevated in all five. The patient with a subnormal calcium concentration also had subnormal 25 OHD and 1,25(OH)2D concentrations and a supranormal PTH concentration. The remaining four had supranormal PTH concentrations despite normal total and ionized calcium concentration, and normal 25 OHD and 1,25(OH)2D levels. Osteocalcin concentration was markedly elevated in all patients, as was alkaline phosphatase activity. These observations show for the first time that patients with fluorosis have markedly elevated osteocalcin, a marker of osteoblastic activity, and that they may have significantly elevated PTH concentrations in the presence of normal total and ionized calcium concentrations.

1-Carboxyglutamic Acid↗

Neonatal lupus erythematosus, late onset hypocalcaemia, and recurrent seizures.

A 6 day old infant had neonatal lupus erythematosus manifested by rash, haemolytic anaemia, and hepatosplenomegaly. His mother was asymptomatic until eight months of pregnancy. Between 7 and 10 weeks he had recurrent seizures with hypocalcaemia. Other causes of convulsions were excluded. By 14 weeks various abnormalities had largely disappeared.

Female↗