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儿童大动脉炎

Takayasu arteritis in children.

作者信息

Cakar Nilgun, Yalcinkaya Fatos, Duzova Ali, Caliskan Salim, Sirin Aydan, Oner Ayse, Baskin Esra, Bek Kenan, Soylu Alper, Fitoz Suat, Bayazit Aysun Karabay, Bircan Zelal, Ozen Seza, Uncu Nermin, Ekim Mesiha

机构信息

From the Department of Nephrology, Ministry of Health Diskapi Children's Hospital, Ankara, Turkey.

出版信息

J Rheumatol. 2008 May;35(5):913-9. Epub 2008 Mar 15.

Abstract

OBJECTIVE

To retrospectively evaluate the clinical features, angiographic findings, and outcomes of children with Takayasu arteritis (TA) in Turkey.

METHODS

Clinical, laboratory, and angiographic findings and outcomes of 19 children with TA were evaluated with a retrospective chart review. The criteria for inclusion were those proposed by the American College of Rheumatology.

RESULTS

Mean followup period was 35.89 +/- 40.75 months (range 1-168, median 30). There were 14 girls and 5 boys. The mean age at diagnosis was 12.84 +/- 2.69 years (range 8-17, median 13). The most common complaints on admission were headache (84%), abdominal pain (37%), claudication of extremities (32%), fever (26%), and weight loss (10%). One patient presented with visual loss. Examination on admission revealed hypertension (89%), absent pulses (58%), and bruits (42%). Angiography revealed type I in 13 patients (aortic arch, descending thoracic, and abdominal aorta), type II in 4 (descending thoracic aorta and abdominal aorta), and type IV in 2 (diffuse aortic and pulmonary artery). The most commonly involved vessels were the renal, subclavian, and carotid arteries. All patients received corticosteroid therapy, and further immunosuppressive therapy was added in 15 patients. Fourteen of the 17 hypertensive patients had renal artery stenosis and 9 underwent surgery or interventional therapy. Thoraco-abdominal bypass graft was performed in 2 patients who had abdominal aortic stenosis.

CONCLUSION

Hypertension is the most common clinical feature at presentation. Corticosteroid and immunosuppressive therapy was effective in the control of disease activity. Angioplasty or bypass grafting was successfully performed when needed.

摘要

目的

回顾性评估土耳其高安动脉炎(TA)患儿的临床特征、血管造影表现及预后。

方法

通过回顾性病历审查对19例TA患儿的临床、实验室、血管造影表现及预后进行评估。纳入标准采用美国风湿病学会提出的标准。

结果

平均随访期为35.89±40.75个月(范围1 - 168个月,中位数30个月)。有14名女孩和5名男孩。诊断时的平均年龄为12.84±2.69岁(范围8 - 17岁,中位数13岁)。入院时最常见的主诉为头痛(84%)、腹痛(37%)、肢体间歇性跛行(32%)、发热(26%)和体重减轻(10%)。1例患者出现视力丧失。入院检查发现高血压(89%)、脉搏消失(58%)和血管杂音(42%)。血管造影显示13例为I型(主动脉弓、胸降主动脉和腹主动脉),4例为II型(胸降主动脉和腹主动脉),2例为IV型(弥漫性主动脉和肺动脉)。最常受累血管为肾动脉、锁骨下动脉和颈动脉。所有患者均接受了糖皮质激素治疗,15例患者加用了进一步的免疫抑制治疗。17例高血压患者中有14例存在肾动脉狭窄,9例接受了手术或介入治疗。2例腹主动脉狭窄患者接受了胸腹旁路移植术。

结论

高血压是最常见的就诊临床特征。糖皮质激素和免疫抑制治疗对控制疾病活动有效。必要时成功进行了血管成形术或旁路移植术。

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