摘要
目的探讨新生儿期色素失禁症(IP)的临床特点、预后及其影响因素。方法收集2002年10月-2011年3月于北京儿童医院住院治疗并采用Landy-Donnai临床诊断标准确诊的10例新生儿期IP患儿的临床病例资料。患儿均为女婴,足月儿,中位出生体质量为3 350 g。截至2012年7月1日,除2例失访病例外,4例死亡,4例生存。4例生存病例的中位随访时间为43个月。对10例患儿的临床表现(包括皮肤表现、眼科损害、神经系统异常等方面)、辅助检查(外周血嗜酸性粒细胞计数、皮肤活检、颅脑CT等)及随访结果进行分析。采用SPSS 16.0统计软件进行分析。结果 10例患儿均有皮疹表现,包括红斑、丘疹、水泡、疣状增生、泼墨状色素沉着。6例患儿外周血嗜酸性粒细胞比例明显增高。2例患儿住院期间检查存在眼底异常。4例新生儿期内出现抽搐,抗惊厥治疗效果不佳,其中3例死亡;1例于新生儿期后随访期内出现抽搐,给予抗惊厥治疗后止痉。新生儿期是否发生抽搐与随访期内的生存率存在相关性(P=0.002 4)。结论新生儿期IP以皮肤改变为主,合并神经系统损害的患儿预后不佳。
Objective To investigate the clinical manifestations of incontinentia pigmenti （IP） in the neonatal period, as well as the diagnosis and prognosis. Methods The clinical manifestations and prognosis of 10 neonatal IP patients, hospitalized at Beijing Children＇ s Hospital from Oct. 2002 to Mar. 2011, were analyzed. All the patients were diagnosed according to Landy - Donnai criteria. All the patients were full - term baby girl,with median birth weight 3 350 g. There were 4 cases out of 10 patients survived with median following 43 months, till Jul. 1. 2012, except 4 cases died in the following period and 2 cases lost follow - up. The clinical manifestations including skin changes, eye abnormalities and nervous system damage, and assistant examinations, as well as their contributions to diagnosis and prognosis were analyzed. SPSS 16.0 software was used in this study for statistical analysis. Results Performance of 10 patients were skin rash,including erythema, papules, blisters, verrucous hyperplasia and ink - like pigmentation. Eosinophils in peripheral blood significantly increased in 6 patients. Two patients existed eye abnormalities during hospitalization. Four patients experienced convulsions in neonatal period, with poor antieonvulsant therapy effect and 3 cases died. One patient experienced convulsions after neonatal period, in the follow - up, with good anticonvulsant re-sponse. Further,whether experienced convulsions in neonatal period were related to the survival rate （P = 0. 002 4）. Conclusions Skin rash was the main change in neonatal patients suffered IP. Those patients who accompanied by nervous system damage especially had a poor prog-nosis.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2012年第20期1576-1578,共3页
Journal of Applied Clinical Pediatrics
关键词
色素失禁症
临床表现
抽搐
预后不良
婴儿
新生
incontinentia pigmenti
manifestations
convulsion
poor prognosis
infant, newborn
