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.

Pediatric renal transplantation in Johannesburg can be accomplished with low complication rates, but medium and long-term graft survival is poor when compared with contemporary results achieved in developed countries. The difficulties of undertaking such complex, multidisciplinary interventions in a developing nation are daunting, but we believe that renal transplantation should still be the treatment of choice for all children with ESRF. The poorer outcomes in black recipients can be addressed by increasing education in our communities and expanding the pool of appropriate donors. Better institutional support would allow for improved long-term patient care.

Background. Undifferentiated embryonal sarcoma of the liver (UESL) is a rare neoplasm, and the third-most common paediatric hepatic malignancy. However, no treatment guidelines exist. No randomised, controlled trials support specific combinations of therapy. Objective. To compare presentation and management of UESL with other series, review the literature, and formulate treatment guidelines. Methods. A retrospective chart review of all hepatic malignancies was conducted from 1996 to 2007 and 5 children with UESL were identified. Management and outcomes were documented. The literature regarding treatment modalities up to September 2012 was reviewed. Results. Over a period of 11 years, 5 patients presented. All underwent surgery and 4 received chemotherapy. One received radiotherapy at relapse. Three are disease-free with follow-up of 58 -184 months. One died after relapse, as did the patient whose family declined chemotherapy. Conclusion. The improved outcomes are consistent with the international experience and are probably related to combined treatment modalities and advances in supportive care. Pre-operative percutaneous biopsy provides no benefit if the lesion is resectable because it may not prove to be diagnostic, and may cause recurrence in the biopsy tract. If resectable, the recommended treatment is primary excision and adjuvant chemotherapy, with radiotherapy in selected cases. If unresectable, open biopsy is necessary to document histology, and neoadjuvant chemotherapy is given prior to resection. If deemed unresectable, liver transplantation is considered.