慢性肉芽肿病儿童肝脾超声特点  

Sonographic Appearance of Liver/spleen in Children with Chronic Granulomatous Disease

作　　者：宋红艳[1] 贺建新[1] 付利民[1] 钱素云[1] 张锡沛[1] 

机构地区：[1]首都医科大学附属北京儿童医院

出　　处：《中国病案》2011年第4期4-6,共3页Chinese Medical Record

摘　　要：目的观察慢性肉芽肿病(chronic granulomatous disease,CGD)儿童肝脾超声特点,分析临床意义,利于选择适宜的治疗方法,以期获得最佳疗效,改善预后。方法来自于2007年7月-2010年4月经基因突变分析和功能诊断的31例CGD患儿的肝脾超声记录。结果 28例中国男性儿童通过CYBB基因突变分析被诊断为X连锁隐性CGD(X-linked recessive CGD,XLR-CGD),1例常染色体隐性CGD(autosomal recessive CGD,AR-CGD),2例可能为AR-CGD。31例患儿中,28例有肝和/或脾肿大,9例(9/31,29%)有脾脏低回声,多发为主,平均最大直径约0.6cm。1例诊断脾脓肿,1例怀疑脾脓肿。2例有肝脏低回声,其中1例最大直径为3.2cm,明确诊断肝脾脓肿,行肝穿刺抽脓,辅以抗生素治疗,渐好转。大部分患儿经单纯抗生素治疗后既往低回声影像明显好转,无新病灶出现。结论 90%CGD患儿有肝和/或脾肿大,提示反复微生物病原刺激存在。本组患儿以脾脏超声低回声病变为主,与国外报道的肝脓肿为主不同。26%(8/31)患儿脾脏病灶直径小于1cm,早期抗生素治疗效果明显。3%患儿有类似于散发肝脓肿的超声表现,治疗困难,必要时需外科开腹引流或清创。Objective To describe the sonographic findings of liver/spleen in children with chronic granulomatous disease and analyze clinical implication in order to select appropriate treatment with best effect and prognosis.Methods Between 2007.7-2010.4,31 CGD cases had sonographic records.Results All except one were male cases,of which there were 28 X-linked recessive CGD cases diagnosed by CYBB mutation analysis,1CYBA case genetically diagnosed,2 cases suspected with autosomal recessive CGD.There was hepatomegaly in 28 cases,spleenmegaly in 20 cases.There were multiple hypoechoic lesions in spleen in 9 cases,with mean diameter 0.6cm.One case was diagnosed with spleen abscess,one suspected with spleen abscess.There were multiple hypoechoic lesions in liver in 2 cases,in one case with max diameter 3.2cm and diagnosis with liver/spleen abscess.Lesions improved slowly through the treatment of percutaneous aspiration and intravenous sensitive antibiotics.For most of cases, single antibiotics are useful in resolutions of lesions.Conclusion 90% cases had heptomegaly/spleenmegaly,indicating the presence of recurrent pathogens,which is different from the presence of liver abscess reported abroad.Small spleen hypoechoic lesions（26%） were predominant in these cases on which early single antibiotics had effect.3% cases had overlap with sporadic liver abscess,for which surgical open abdomen discharge or debridement may be helpful.

关 键 词：慢性肉芽肿病 肝脾超声 低密度 脓肿 儿童 

分 类 号：R445.1[医药卫生—影像医学与核医学] R726.5[医药卫生—诊断学]