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

Published online by Cambridge University Press:  06 July 2018

Antoinette M. Cilliers*
Affiliation:
Division of Paediatric Cardiology, C.H. Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa
Paul E. Adams
Affiliation:
Division of Paediatric Cardiology, C.H. Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa
Hopewell Ntsinjana
Affiliation:
Division of Paediatric Cardiology, C.H. Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa
Udai Kala
Affiliation:
Division of Nephrology, C.H. Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa
*
Author for correspondence: Dr A. M. Cilliers, Paediatric Cardiology, C.H. Baragwanath Academic Hospital, P.O. Bertsham, 2013 Johannesburg, South Africa. Tel: +27 11833435803; Fax: +27 119389074; E-mail: antoinette.cilliers@wits.ac.za

Abstract

Introduction

Takayasu’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.

Type
Original Article
Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Cilliers AM, Adams PE, Ntsinjana H, Kala U. (2018) Review of children with Takayasu’s arteritis at a southern African tertiary care centre. Cardiology in the Young28: 1129–1135. doi: 10.1017/S1047951118000938

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