Review of children with Takayasu's arteritis at a Southern African tertiary care centre.

IntroductionTakayasu's arteritis is a rare idiopathic arteritis causing stenosis or aneurysms of the aorta, pulmonary arteries, and their branches. It usually occurs in women, but has been described in children. OBJECTIVE: The objective of this study was to determine the clinical presentation, demographic profile, vascular involvement, origins, management, and outcome of children diagnosed with Takayasu's arteritis at a Southern African tertiary care centre between 1993 and 2015. METHODS: This is a retrospective analysis of all children with Takayasu's arteritis captured on a computerised electronic database during the study period. RESULTS: A total of 55 children were identified. The female:male ratio was 3.2:1, and the mean age was 9.7±3.04 years. Most originated outside the provincial borders of the study centre. The majority presented with hypertension and heart failure. In all, 37 (67%) patients had a cardiomyopathy with a mean fractional shortening of 15±5%. A positive purified protein derivative test was documented in 73%. Abdominal aorta and renal artery stenosis were the predominant angiographic lesions. A total of 23 patients underwent 30 percutaneous interventions of the aorta, pulmonary, and renal arteries: eight stents, 22 balloon angioplasties, and seven had nephrectomies. All patients received empiric tuberculosis treatment, immunosuppressive therapy, and anti-hypertensive agents as required. Overall, there was a significant reduction in systolic blood pressure and improvement in fractional shortening (p<0.05) with all treatments. CONCLUSION: Takayasu's arteritis is more common in girls and frequently manifests with hypertension and heart failure. The abdominal aorta and renal arteries are mostly affected. Immunosuppressive, anti-hypertensive, and vascular intervention therapies improve blood pressure control and cardiac function.

Impact of Highly Active Antiretroviral Therapy on paediatric Human Immunodeficiency Virus-associated left ventricular dysfunction within the Johannesburg teaching hospital complex.

OBJECTIVE: To analyse the outcome of children with left ventricular dysfunction placed on Highly Active Antiretroviral Therapy. METHOD: This study is a retrospective review of records of Human Immunodeficiency Virus-positive children with left ventricular dysfunction. Demographic data were collected. Left ventricular fractional shortening, CD4 percentage, viral load, and nutritional status were compared before and during antiretroviral therapy. RESULTS: We reviewed the records of 34 Human Immunodeficiency Virus-positive children with left ventricular dysfunction. In all, 18 patients received antiretroviral therapy (group one) and 16 were antiretroviral therapy naive (group two). The median age of group one at initial visit was 94 months, with a male-to-female ratio of 1:1. Of those, 17 children showed improved left ventricular function on treatment, with an increase in fractional shortening (median: 17-33.5%; p less than 0.0001). There was no significant statistical difference between the groups regarding initial fractional shortening. In group one, the CD4 percentage improved (median: 12% to 30.5%; p less than 0.0001), with viral load suppression (median: 24,900 copies per millilitre to less than 25 copies per millilitre; p less than 0.0001). There was weight gain in group one (median z-score: -1.70 to -1.32; p equal to 0.0083). Proper statistical analysis in group two was not possible because of poor follow-up of patients. CONCLUSION: The findings are in keeping with other reports that have shown improvement in left ventricular function in patients with Human Immunodeficiency Virus-associated cardiomyopathy treated with Highly Active Antiretroviral Therapy. Recovery of myocardial function is associated with improvement in immunological and nutritional statuses.

Improving Access to Pediatric Cardiac Care in the Developing World: the South African Perspective.

Purpose of Review: The paper outlines the current status of health care and pediatric cardiac services in South Africa and the challenges faced in providing pediatric cardiac care in the country. Recent Findings: As infant and child mortality rates in South Africa and most of Sub-Saharan Africa continue to decline, establishing and improving the infrastructure to manage congenital heart disease increases in importance. Summary: South Africa has well-established pediatric cardiac units in most major centers in the country. These have been able to train sufficient numbers of pediatric cardiologists to double the number in the country in just over a decade as well as train fellows from surrounding countries. A significant proportion of funding for this training comes from non-government sources. The number of pediatric cardiologists is however still far less than required with services spread unevenly throughout the country. Pediatric cardiac surgical services remain severely constrained with an urgent need to train more pediatric cardiac surgeons. Further progress depends not only on focussing resources on cardiac disease but also improvements in the health care systems and socioeconomic conditions in general.

Early presentation of endomyocardial fibrosis in a 22-month-old child: a case report.

We present an unusual, biopsy-proven case of endomyocardial fibrosis in a 22-month-old male child, which progressed rapidly resulting in death. The patient was born to a father originating from Mozambique, where the disease is endemic but who had not himself travelled there, suggesting a genetic link. Other remarkable features were the presence of a right ventricular diverticulum, and a positive Mycoplasma pneumoniae immunoglobulin M enzyme-linked immunosorbent assay test.