Thirty children with frequently relapsing idiopathic nephrotic syndrome (INS) were treated with levamisole (2.5 mg/kg BW) twice a week for a mean period of 9.9 months. A beneficial effect was observed in 16 children in whom corticosteroids could be significantly decreased without relapse. Levamisole was ineffective in 14 patients. There was no difference between the two groups in the duration of INS, the number of relapses and the duration of treatment with levamisole. The mean age at onset of INS was higher in the group of patients where levamisole was effective (5.8 years versus 2.8 years). In 7 patients who responded to levamisole neutrophils decreased below 4 X 10(9)/l. Transient granulocytopenia was observed in 3. It is concluded that levamisole may be effective in frequently relapsing INS with minimal side effects.
In adults with chronic renal failure (CRF) and/or renal replacement therapy (RRT) various immunological abnormalities have been described, but few data are available for the paediatric age group. We performed basic in vitro immunological studies in 26 patients 10 months-19 years of age with advanced renal failure, 11 with CRF (creatinine clearance 16.8 +/- 5.2 ml/min per 1.73 m2), 15 on RRT with haemodialysis (HD; n = 9) and continuous ambulatory peritoneal dialysis (CAPD; n = 6) as well as in 16 healthy controls. None had clinical evidence of deranged immune function. No significant differences were found in the percentages of B- and T-cells, T-cell subsets CD3, CD4, CD8 and mitogenic responses to phytohaemagglutinin and concanavalin A (Con A) between RRT patients (HD = CAPD) and control children. Most parameters in CRF patients were also normal, although they had a low percentage of B-cells (12.1 +/- 4.1; RRT: 19.7 +/- 6.5; controls: 18.5 +/- 7.1; P less than 0.01), relatively low levels of serum immunoglobulin G (948.4 +/- 209.4 mg/dl; HD: 1374.7 +/- 235.2 mg/dl; P less than 0.01; CAPD: 966.3 +/- 430.2 mg/dl, NS) and a high normal response to Con A (34.3 +/- 13.6 cpm x 10(-3); RRT: 24.5 +/- 11.3 cpm x 10(-3); controls: 23.4 +/- 9.9 cpm x 10(-3), P less than 0.01). All these values were, however, well within the normal accepted range. These data indicate that children/adolescents with CRF and/or RRT have no significant basic in vitro immunological defects. This study did not test the functional immune status of the young uraemic patients.
ABSTRACT. The immunological and clinical effects of levamisole were studied in 10 children with frequently relapsing steroid responsive nephrotic syndrome (SRNS). The efficacy of the drug was tested during remission of the disease with all patients on alternate day steroid therapy. The lymphocyte proliferative response to phytohemagglutinin (PHA), concanavalin‐A (Con‐A) and pokeweed mitogen (PWM) were normal. The Con‐A induced suppressor T‐lymphocyte activity of 7 patients was low before treatment with levamisole 8±3.7% and increased to normal values during therapy 34±6%; p<0.001 (control 32±5%). In these 7 children prednisolone dosage could be decreased significantly or discontinued altogether (44.1±5.3%). Patients without immunoregulatory abnormalities did not respond to levamisole. In 3 out of 4 children tested the percentage of OKT8+ cells rose during levamisole therapy from 19.7±2.1 to 37±2.3 (p<0.001), thus correcting the elevated pre‐treatment OKT4+/OKT8+ ratio from 3.1±0.2 to 1.5±0.2; p<0.001 (control 1.47±0.2). These data support the hypothesis that abnormal immunoregulation may play a role in the pathogenesis of SRNS. Treatment with levamisole can be useful in some patients with the frequently relapsing form of the disease.
Pulmonary calcifications are known to occur in patients with chronic renal failure. Recently, scintigrams with bone-seeking radionuclides have been used to detect subclinical pulmonary calcium deposits. We studied 18 children on maintenance dialysis without evidence of pulmonary calcification on chest X-ray. Four children (22.2%) had a positive technetium 99m hydroxymethylene diphosphate scan (group 1), and 14 children had a negative scan (group 2). Mean serum aluminum levels were 2.68 ± 0.30 μmol/l (mean ± SD) in group 1 as compared to 1.66 ± 0.72 in group 2 (p < 0.01). No significant difference was found between the groups with respect to serum levels of calcium, phosphorus, bicarbonate, magnesium and the calcium-phosphorus product as well as parathyroid hormone and vitamin D levels. The patients with pulmonary calcifications were on dialysis a significantly longer time than those of group 2 (62 ± 15 versus 35.7 ± 23 months; p < 0.01). These data show that pulmonary calcification occurs with high frequency in children undergoing long-term dialysis. They seem to be related to high serum aluminum levels. We propose that pulmonary scintigrams with bone-seeking radionuclides be used routinely for the diagnosis and follow-up of uremic pulmonary calcification.
A 5.5-month-old male infant with hypokalaemia and gall bladder dilatation is reported. The child was shown to have Bartter syndrome. After oral treatment with potassium and indomethacin, serum potassium levels became low normal and the gall bladder enlargement resolved. This entity should be included in the clinical spectrum of neuromuscular disturbances resulting from hypokalaemia and should be considered in a hypokalaemic infant with a right-sided abdominal mass.
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