Aortitis syndrome in children: Clinical observation of 35 cases in Japan
Aortitis syndrome in children: Clinical observation of 35 cases in Japan
复制标题
儿童主动脉炎综合征：日本35例临床观察
DOI：
10.1111/j.1442-200x.1997.tb03600.x
复制
发表时间：
1997
影响因子：
1.4
通讯作者：
S. Fujikawa
中科院分区：
文献类型：
--
作者：
Motoharu Maeda;Munemitsu Kobayashi;S. Okamoto;Tomoko Fuse;T. Matsuyama;N. Watanabe;S. Fujikawa
本文报告了日本全国范围内调查的35例儿童大动脉炎综合征(AS)的临床观察结果。男女比例为1：2.5，估计发病年龄平均为10.2岁，从估计发病年龄到确诊时间平均为15个月。在人类白细胞抗原检测中，A24、BW52、Cw7和DR2较为常见。动脉病变倾向于广泛累及主动脉弓及其分支。发热是最常见的临床症状，其次是腹部、关节和肌肉疼痛。体检结果依次为上肢循环障碍、心脏和血管杂音、高血压、脑循环障碍、视力障碍和肺动脉循环障碍。杂音不仅见于胸壁，还见于颈部和腹部。66%的患者上肢无搏动。除3例外，其余均行经皮逆行主动脉造影和/或静脉数字减影血管造影确诊。实验室数据未见任何特定异常征象。激素治疗34例，免疫抑制剂治疗8例。5例行经皮肾动脉腔内成形术作为介入治疗。腹痛的高频率被认为是儿童AS的特征之一。在这份报告中，上肢无搏动以及心脏和血管杂音的高频率被认为对儿童AS的诊断具有重要意义。
The results of clinical observation of 35 patients with aortitis syndrome (AS) in childhood, obtained by a nationwide survey in Japan, are reported. The male to female ratio was 1:2.5, the estimated age of onset averaged 10.2 years, and the duration from the estimated age of onset to the diagnosis averaged 15 months. In HLA examination A24, Bw52, Cw7 and DR2 were relatively common. Arterial lesions tended to extensively involve the aortic arch and its branches. Fever was the most frequently noted clinical symptom, followed by abdomen, joint and muscle pain. The physical findings in order of frequency were impaired circulation of the upper extremities, cardiac and vascular murmurs, hypertension, impaired cerebral circulation, visual disorder and impaired circulation of the pulmonary artery. The murmurs were found not only over the chest wall but also over the cervical area and abdomen. Pulselessness of the upper extremities occurred in 66% of patients. Percutaneous retrograde aortography and/or intravenous digital subtraction angiography to make the final diagnosis was employed except for three cases. There were not any specific abnormal signs in laboratory data. Steroid hormones were administered in 34 cases, and immunosuppressive agents in 8 cases. Five cases had percutaneous transluminal angioplasty to the right renal artery as an interventional treatment. The high frequency of abdominal pain is considered to be one of the characteristics of AS in childhood. The high frequency of pulselessness of the upper extremities and cardiac and vascular murmurs in this report is considered significant for the diagnosis of AS in childhood.