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Study of Takayasu's arteritis in children: clinical profile and management
M N Muranjan1, S B Bavdekar, V More
1Department of Paediatrics, Seth G. S. Medical College and K.E.M. Hospital Parel, Mumbai- 400 012, India. drsuji@vsnl.com
Insights
Pediatric Takayasu arteritis (TA) often presents with hypertension and cardiac issues. Treatment involves medications and angioplasty, with surgery reserved for severe cases.
Area of Science:
- Pediatric Rheumatology
- Cardiovascular Medicine
- Vascular Inflammation
Background:
- Takayasu arteritis (TA) is a rare, chronic inflammatory disease affecting large arteries.
- Understanding pediatric TA presentation and management is crucial for early diagnosis and intervention.
Purpose of the Study:
- To investigate the clinical features, angiographic patterns, and treatment outcomes of Takayasu arteritis in children.
- To identify common complications and mortality rates in pediatric TA.
Main Methods:
- Retrospective analysis of hospital records for pediatric patients diagnosed with TA over 4.5 years.
- Diagnosis based on Ishikawa's criteria, with angiographic assessment for disease pattern.
Main Results:
- 17 children (5-11 years) diagnosed with TA; common symptoms included hypertension (64.7%) and congestive heart failure (47%).
- Type II angiographic involvement was most frequent (52.9%). Medical management (antihypertensives, steroids) and angioplasty showed partial success.
- In-hospital mortality was 11.7%, with surgery reserved for non-responders.
Conclusions:
- Pediatric TA patients often present acutely with hypertension and cardiac complications.
- Type II vascular involvement is predominant, and a combination of medical therapy and angioplasty offers partial symptom control.
- Aggressive management strategies are necessary, with surgery as a last resort for refractory cases.
Aims:
To study clinical features, pattern of involvement and treatment modalities of Takayasu's arteritis (TA) in children.
Material And Methods:
Retrospective analysis of hospital records of children admitted over a period of 4.5 years.
Results:
17 patients in the age group of 5 to 11 years (M: F = 1.1: 1) were diagnosed to have TA on the basis of Ishikawa's criteria. One child was diagnosed post-mortem. The commonest presenting features were hypertension (64. 7%), congestive cardiac failure (47%), weak or absent peripheral pulses, cardiomyopathy (41.1% each) and cardiac valvular affection (35.2%). Retinopathy, hypertensive encephalopathy and abdominal bruits were uncommon. None presented in the prepulseless phase. No patient had an active tuberculous lesion, although Mantoux or BCG test was positive in 6 (35.2%). The predominant pattern of angiographic affection was Type II (52.9%). Nephrotic syndrome and portal cavernoma seen in one patient each were incidental associations. Anti-hypertensive drugs, oral steroids and drugs to control congestive heart failure were the mainstays of medical management. Antitubercular therapy was started in six patients. Angioplasty was attempted in 15 cases and proved to be partially beneficial in six. Three patients who failed to respond to medical management had to undergo surgical procedures, either bypass, nephrectomy or both. In-hospital mortality was 11.7%.
Conclusions:
Patients of TA in this study presented acutely in the pulseless phase, with hypertension and its complications. Type II involvement was the commonest pattern. Anti-hypertensive agents and steroids along with angioplasty were partially successful in controlling symptoms in 35.2%. Surgical procedures were reserved for a minority with poor response to drugs and angioplasty.