婴幼儿多发性大动脉炎14例临床特点分析

目的通过分析总结14例婴幼儿多发性大动脉炎(TA)的临床特点,并结合文献复习总结,以提高对该病的认识及诊疗水平。方法回顾性分析2016年7月至2019年5月在首都儿科研究所附属儿童医院住院的TA婴幼儿的临床资料及随访情况,并结合文献,分析总结该病的临床特点。结果14例患儿年龄为1个月23 d^28个月,男6例,女8例。临床表现中最常见的是发热[10例(71.4%)],高血压9例(64.3%),脉弱或无脉5例(35.7%)。按照病变血管部位的临床分型,广泛型11例(78.5%),头臂动脉型3例(21.4%),本组患儿无胸腹主动脉及单纯肺动脉型。14例TA患儿中,12例有颈总动脉、颈动脉、锁骨下动脉及冠状动脉及其分支(前降支、回旋支)受累(85.7%);11例肾动脉受累(78.6%);9例腋动脉受累(64.2%);8例腹主动脉受累(57.1%);6例降主动脉受累(42.9%);6例胸主动脉受累(42.9%);6例肠系膜上动脉受累(42.9%);5例股动脉受累(35.7%);5例肺动脉受累(35.7%);4例肱动脉受累(28.6%)。14例患儿中,误诊11例,诊断不清3例,误诊时间为18 d^2个月。误诊病例中,8例误诊为不典型川崎病。14例患儿中,7例治疗后大部分病变血管范围逐渐减少,受累较轻血管甚至可以完全恢复正常血管状态。4例患儿血管影像学检查较前无明显加重或好转。9例出现高血压患儿应用降压药血压能控制在正常范围,但不能停用降压药。5例查体发现脉弱或无脉患儿均未改善。14例患儿中7例生长发育同正常同龄儿,7例落后于正常同龄儿身高体质量第25百分位。14例患儿随访2~22个月,均规律治疗,未出现复发。结论3岁以内TA累及血管较多,病情严重,误诊率高,经过治疗病情很快能够控制,但容易遗留血管病变,部分患儿预后不佳。

Analysis of clinical features of 14 infants with Takayasu arteritis

Objective To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis(TA)by analyzing the clinical features of 14 pediatric patients and reviewing related articles.Methods The clinical and follow-up data of infants with TA who were admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles,the clinical features of this disease were summarized.Results The age of 14 patients(including 6 males and 8 females)were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases(71.4%),hypertension in 9 cases(64.3%),weak or no pulse in 5 cases(35.7%).According to the clinical type of lesion vessels,11 cases(78.5%)were generalized type,3 cases(21.4%)were brachiocephalic artery type,and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA,12 cases had common carotid artery,carotid artery,subclavian artery,coronary artery and its branches(anterior descending branch,circumflex branch)involved(85.7%);11 cases had renal artery involved(78.6%);9 cases had radial artery involved(64.2%);8 cases had abdominal aorta involved(57.1%);6 cases had descending aorta involved(42.9%);6 cases had thoracic aorta involved(42.9%);6 cases had superior mesenteric artery involved(42.9%);5 cases had femoral artery involved(35.7%);5 cases had pulmonary artery involved(35.7%);and 4 cases had brachial artery involved(28.6%).In those 14 patients,11 cases were misdiagnosed,and 3 cases had unclear diagnosis,with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases,8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases,the ranges of most lesions were gradually decreased,and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or improvement in 4 cases.Nine cases developed hypertension,the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients,7 cases showed normal development,while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.Conclusions TA patients aged less than 3 years tend to have more blood vessels involved,be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment,but vascular diseases may be developed easily.Some patients have a poor prognosis.