Muranjan M N, Bavdekar S B, More V, Deshmukh H, Tripathi M, Vaswani R
Department of Paediatrics, Seth G. S. Medical College and K.E.M. Hospital Parel, Mumbai- 400 012, India.
J Postgrad Med. 2000 Jan-Mar;46(1):3-8.
To study clinical features, pattern of involvement and treatment modalities of Takayasu's arteritis (TA) in children.
Retrospective analysis of hospital records of children admitted over a period of 4.5 years.
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%.
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.
研究儿童大动脉炎（TA）的临床特征、受累模式及治疗方式。
对4.5年间收治儿童的医院记录进行回顾性分析。
根据石川标准，17例年龄在5至11岁的患者（男∶女 = 1.1∶1）被诊断为TA。1例患儿死后确诊。最常见的临床表现为高血压（64.7%）、充血性心力衰竭（47%）、外周脉搏减弱或消失、心肌病（各41.1%）及心脏瓣膜病变（35.2%）。视网膜病变、高血压脑病及腹部血管杂音较少见。无患者处于无脉前期。虽6例（35.2%）结核菌素试验或卡介苗接种试验呈阳性，但均无活动性结核病灶。血管造影主要受累模式为Ⅱ型（52.9%）。肾病综合征和门静脉海绵样变性各见于1例患者，为偶然关联。抗高血压药物、口服类固醇及控制充血性心力衰竭的药物是药物治疗的主要手段。6例患者开始抗结核治疗。15例尝试血管成形术，其中6例证明部分有效。3例药物治疗无效的患者不得不接受手术，包括搭桥术、肾切除术或两者均做。住院死亡率为11.7%。
本研究中TA患者在无脉期急性发病，伴有高血压及其并发症。Ⅱ型受累是最常见的模式。抗高血压药物、类固醇及血管成形术在35.2%的患者中部分成功控制了症状。手术仅用于少数对药物及血管成形术反应不佳的患者。
