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Biomedical subjects

R Drachman

Publications and source records attributed to R Drachman.

13 recordsLinked to original sources
PubMed1989

The immune status of uraemic children/adolescents with chronic renal failure and renal replacement therapy.

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.

Adolescent↗
PubMed1989

Transient gall bladder dilatation associated with hypokalaemia in a patient with Bartter syndrome.

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.

Bartter Syndrome↗
PubMed1989

Vaccination against hepatitis B in children and adolescent patients on dialysis.

Twenty-one children and adolescent patients, 2-19 years of age, on renal replacement therapy were immunised at monthly intervals with three doses of 20 micrograms hepatitis B vaccine (Heptavax B, Merck Sharp & Dohme). In the absence of seroconversion, vaccination was continued with monthly doses of 40 micrograms hepatitis B vaccine until antibody to hepatitis B surface antigen became positive. The rate of seroconversion increased from 33.3% (7 of 21) to 76.1% (16 of 21) and 85.6% (18 of 21) with three, four and five vaccine injections respectively. Three patients had no immune response despite six to seven vaccine dosages; they had previously received immunosuppressive therapy. Antibody titres measured 1 year after seroconversion were found to be within the protective range (85-2500 mIU/ml). These results show that the impaired immune response to hepatitis B vaccination in young dialysis patients can be overcome by increasing the number of injections and the dose of the vaccine. Protective antibody titres are maintained for at least 1 year after vaccination. Immunosuppressive therapy may interfere with the vaccine response.

Adolescent↗
PubMed1989

Skin and mucosal lesions associated with cyclosporin A therapy: report of a case.

A 16-year-old boy developed bullous lesions on fingers, ears, tongue, and buccal mucosa as well as cauliflower-like growths on the epiglottis and vocal cords 1 1/2 years after a second renal transplantation and exposure to sunlight while receiving cyclosporin A (CsA). Withdrawal of CsA was followed by complete resolution of skin and mucosal lesions. A predisposition for adverse reactions to CsA is suggested, as additional side effects of CsA therapy were also present.

Adolescent↗
PubMed1988

Immunoregulation with levamisole in children with frequently relapsing steroid responsive nephrotic syndrome.

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 less than 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 less than 0.001), thus correcting the elevated pre-treatment OKT4+/OKT8+ ratio from 3.1 +/- 0.2 to 1.5 +/- 0.2; p less than 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.

Adolescent↗
PubMed1986

Cerebral atrophy and nephropathic cystinosis.

The management of end stage renal failure in cystinotic children is correlated with a longer survival, sometimes complicated with neurological abnormalities. Cranial computed tomography was performed in 10 patients and showed a significant atrophy; the pathogenesis of this damage remains unclear.

Adolescent↗
PubMed1986

Pulmonary calcifications in children on dialysis.

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 mumol/l (mean +/- SD) in group 1 as compared to 1.66 +/- 0.72 in group 2 (p less than 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 less than 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.

Adolescent↗
PubMedDate not supplied

Recurrence of oxalate deposition in a renal transplant during ciclosporin A therapy.

A 14.5-year-old boy with end-stage renal failure due to familial primary hyperoxaluria underwent cadaver donor renal transplantation. The graft function was stable for a period of 22 months, while he was on conventional immunosuppressive therapy (prednisolone and azathioprine) and additional oral pyridoxine, phosphorus, and magnesium supplementation. When ciclosporin A was introduced instead of azathioprine, the blood levels of oxalate rose, and oxalate deposition in the renal tubuli became evident. These observations suggest that ciclosporin A interferes with oxalate metabolism and, therefore, should be given with utmost caution in patients with primary hyperoxaluria.

Adolescent↗
PubMedDate not supplied

Modification of peritoneal ultrafiltration capacity in children undergoing peritoneal dialysis.

Eleven children (7 girls and 4 boys) 2 1/2 to 17 years and 8 months of age were treated with CAPD for periods ranging from 6 to 31 months. All children were treated with commercially available dialysate solutions containing lactate. Peritoneal ultrafiltration capacity (PUFC) decreased progressively in all children without accompanying decrease in peritoneal urea and creatinine clearances. Five children developed membrane failure with negative ultrafiltration. One episode of peritonitis occurred in one of these 5 children and in 4 of them only 1.5% glucose solutions had been used. After an initial period (ranging from 14 to 31 months) of CAPD, 2 children were treated with Intermittent Ambulatory Peritoneal Dialysis (IAPD) and two others with Intermittent Cycling Peritoneal Dialysis (ICPD). In these 4 children, PUFC increased within one month from -3.75 ml/kg/day to + 5 ml/kg/day. By providing a shorter dwell time, IAPD and ICPD may allow a reduction in net inward transport of glucose, the maintenance of osmolar gradient and preservation of ultrafiltration capacity. Furthermore, periods of rest may allow some recovery from the progressive deterioration of the peritoneum resulting from long-term irrigation of the peritoneal cavity. These results indicate that IAPD and CPD may be superior to CAPD to maintain the ultrafiltration capacity of the peritoneum.

Adolescent↗