Peripheral blood lymphocyte subsets in idiopathic nephrotic syndrome of childhood.

Lymphocyte subsets in the peripheral blood were examined in 34 patients with nephrotic syndrome [27 with minimal change nephrotic syndrome (MCNS), 7 with significant lesions] and 10 normal children. The investigations were done at the onset of disease before administering corticosteroids (n = 34), during the subsequent remission (n = 27), and the first relapse (n = 12). The proportions of T cell subsets (CD3, CD4 and CD8) were determined by immuno-alkaline phosphatase staining; interleukin-2 receptor (IL-2 R) positive cells, natural killer (NK) cells and B cells were studied by indirect immunofluorescence. No significant differences were found in the proportion of CD3, CD4, CD8 and IL-2 R positive cells in patients with MCNS as compared to controls. An increased proportion of NK and B cells was not specific to MCNS and was detected even in those with significant renal lesions. Our findings suggest that T lymphocyte subsets in MCNS are similar to those in normal children.

Long-term, low-dose prednisolone therapy in frequently relapsing nephrotic syndrome.

The efficacy of daily administration of a small dose of prednisolone was examined in 21 patients with corticosteroid-responsive, frequently relapsing nephrotic syndrome (FRNS). After induction of remission of a third or subsequent relapse with a 6-week course of prednisolone (standard therapy with prednisolone, STP), this drug was continued in a single daily dose of 0.25 mg/kg body weight (low-dose prednisolone, LDP) for 18 months. Relapses occurring during this period were treated with STP, following which LDP therapy was resumed. The historical controls comprised 14 patients with FRNS in whom relapses were treated with STP and who were observed over a minimum period of 30 months. The two groups were comparable for age at the onset of nephrotic syndrome and sex. Twenty patients completed LDP therapy, during which 12 had no relapse, 6 had infrequent and 2 frequent relapses (1 patient became steroid dependent and was taken off LDP). Twelve patients were followed for 12-42 months after stoppage of LDP; during this period 7 had no relapse, 4 had infrequent relapses and 1 showed steroid dependence. The number of relapses during LDP therapy (0.5/patient per year) was significantly less (P less than 0.001) than in the preceding 12 months (3.62/patient per year), and continued to remain low during the following 12 months (0.6/patient per year). Whereas the frequency of relapses in the LDP group was similar to that in the historical control group in the 1st year of comparison, it was significantly less during LDP therapy (0.5/patient per year versus 2.25/patient per year). No side effects were observed in patients on the LDP regimen, at the end of which the height percentiles improved in 6 patients and remained unchanged in 14. Our observations indicate that long-term therapy with a small daily dose of prednisolone can significantly reduce the number of relapses in patients with FRNS, and that the beneficial effect may continue even after its stoppage.

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.

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.

Efficacy of mung bean (lentil) and pop rice based rehydration solutions in comparison with the standard glucose electrolyte solution.

Children with acute diarrhea and moderate dehydration between 3 months and 5 years of age were randomly assigned to receive treatment with standard WHO oral rehydration solution (ORS) (n = 33) and two other solutions in which the 20 g/L glucose was substituted by 50 g/L of pop rice (n = 31) and 60 g/L of mung bean (lentil) powder (n = 29). Satisfactory oral rehydration, as assessed clinically and by changes in PCV and total serum solids (TSS), was achieved in 90.9% with WHO ORS, 96.8% with pop rice, and 96.6% in the mung bean ORS treated group (p greater than 0.05). The purging rates (ml/kg/h) until recovery were 2.49 +/- 1.5 (pop rice); 2.91 +/- 2.0 (WHO), and 3.41 +/- 1.7 in the mung bean group (p greater than 0.05). The percentage of patients recovering from diarrhea within the 72 h study period was 58.0 (pop rice), 48.4 (WHO), and 44.8 for mung bean group (p greater than 0.05). Though differences in stool volumes and duration in the three groups were not statistically different, there was a trend toward improvement in efficacy with the pop rice ORS in several parameters: greater weight gain, higher percentage decline in TSS, higher urine output despite lower ORS intake, and lower purging rates. The intake of semisolids in the 24-72 h study period was also higher in the pop rice group as compared to the other two groups (p less than 0.05). The number of breast feeds and intake of artificial milk was however similar in all groups (p greater than 0.05).