We have reviewed 31 patients with Takayasu's arteritis followed at two pediatric nephrology units in Gauteng, South Africa over a 15-year period. There were 25 black patients, 4 white, and 2 of mixed race. The mean age at diagnosis was 8.42+/-3.59 (range 2.4-14.5, median 8) years. The most common presenting sign was hypertension, followed by cardiac failure, bruits, and absent pulses. The Mantoux test was strongly positive in 27 patients (90%, control population 5%). Markers of activity included a raised erythrocyte sedimentation rate (23 patients) or Gallium single photon emission tomography (positive in 12 of 16 patients). Angiography revealed type II (abdominal aorta) and III (arch plus abdominal aorta) lesions to be most common (11 in each group). All patients received antituberculous therapy and most low-dose aspirin for its antithromboxane effect. Corticosteroids and further immunosuppression were used to control disease activity. We added total lymphoid irradiation (TLI) or cyclophosphamide. Twenty-six patients in all received further immunosuppression, with 13 patients in each group. Results were similar in the two groups, with similar pre- and posttherapy systolic blood pressures and creatinine clearances. Two patients in each group relapsed, 3 died in the TLI group and 2 in the cyclophosphamide group. Surgical intervention, usually in the quiescent phase, consisted mainly of renal autotransplantation. Because of the problems with TLI and 2 patients with papillary carcinoma of the thyroid with long-term follow-up, we no longer use TLI. We have shown that with active medical and surgical intervention the aggressive course of this disease in children can be modified.

This study assesses the perceptions, knowledge, and attitudes of patients and parents attending the Pediatric Transplant Unit at the Johannesburg Hospital. Fifty-six children with renal transplants accompanied by their parents were assessed by means of a questionnaire. The children's ages were 2.53 to 20.85 years. Eleven of twelve in the noncompliant group were male and nine were Black. The ages of the two groups of patients and distance traveled to the transplant center were similar. The noncompliant group of patients more often missed clinic visits (50% vs. 14%), P=0.0201; forgot to take their medications (50% vs. 23%); and took more medications (10.3 +/- 3.0 vs. 7.5 +/- 2.0) and remembered fewer of their names (4.0 +/- 3.5 vs. 6.5 +/- 2.5), P=0.0001 than the compliant group. The noncompliant patients knew less about their disease (50% vs. 8.3%) P=0.0141, allograft (53% vs. 33%), and immunosuppression (66% vs. 200%) P=0.0217, than the compliant patients. A total of 9% of the patients (5/56) were concerned about immunosuppressive side-effects, and indicated that this affected their compliance. Families wanted additional information, both pretransplant (52%) and posttransplant (45.5%). In addition, 85% wanted ongoing in-house education concerning transplantation and medications. Pediatric renal transplant patients and their families require ongoing education, support, medication evaluation, and compliance surveillance. Patients at high risk of noncompliance require directed additional intervention.

The aim of this study was to quantify and where possible objectively confirm the magnitude of non-compliance (NC) in our paediatric renal transplant recipients. A total of 94 paediatric transplants were performed between 1984 and 1989; 17 were excluded due to graft loss (2), death (3), oxalosis (2) and transfer to the adult unit (10). NC was assessed as missed clinic visits plus medication shortages or actual admission of NC. NC was found in 22% (17/77) of transplanted patients. NC showed no correlation with parental marital status, sex, distance lived from the hospital, pre-emptive transplant status or total lymphoid irradiation. Most NC was peripubertal with a smaller NC in the late teenager group. Social class correlated positively with NC; 82.3% of NC was from social classes III and IV, who formed 52.4% of the patients. NC in social class II (3/26) was significantly different from social class IV (12/24) (P = 0.01); 91% of black patients with NC were from social class IV. Race, corrected for social class, failed to reach significance (P > 0.05). Confirmation of compliance was sought from retrospective cyclosporin A (CsA) trough levels (twice daily dosage). Concomitant phenytoin therapy and CsA given as a daily dosage were excluded as significant confounding variables. The CsA dosage was not significantly different between the compliant (C) and patients with NC. Patients with NC were 8 times more likely to have a CsA level < 10 ng/ml (P = 0.0026) than C patients.(ABSTRACT TRUNCATED AT 250 WORDS)