中国实用儿科杂志2024，Vol.39Issue(11)：839-844,6.DOI:10.19538/j.ek2024110608
出生免疫缺陷合并肝占位病变单中心回顾性病例分析
A retrospective single-center analysis of inborn errors of immunity combined with liver occupation
邹亮燕 1孙碧君 1姚琼 2王文婕 1侯佳 1周钦华 1姚海丽 1孙金峤 1王晓川1
作者信息
- 1. 复旦大学附属儿科医院临床免疫与过敏科,上海 201102
- 2. 复旦大学附属儿科医院放射科,上海 201102
- 折叠
摘要
Abstract
Objective To analyze the clinical characteristics,treatment,and prognosis of inborn errors of immunity(IEI)combined with liver occupation,and to improve the diagnosis and management of this disease.Methods IEI patients who were hospitalized in the Department of Allergy and Clinical Immunology of the Children's Hospital of Fudan University and whose abdominal imaging suggested hepatic occupation were included.The clinical data of the patients were analyzed and they were grouped by prognosis for comparison.Results A total of 19 children with IEI combined with liver occupation were admitted to the hospital.The prevalence of IEI children with liver occupation was 2.8％(19/687),the median time of onset of disease was 2 months of age,and the age offindingliver occupation was 12 months of age.Elevated CRP level(94.7％,18/19),anemia(78.9％,15/19),fever(57.9％,11/19),and hypoalbuminemia(31.6％,6/19)were the main abnormalities.Detection rate of hepatic occupation on ultrasound was lower than that on CT(63％vs 100％).Hepatic occupation was with a maximum diameter of 19 mm(10 mm-50 mm)and multiple occupation(14/19).The classifications of liver occupations:liver abscess(6/19),hepatic tuberculosis(4/19),hepatic EBV infection(2/19),hepatic fungal infection(1/19),hepatic hemangioma(2/19),and unknown nature(4/19).Infectious lesions accounted for 68.4％(13/19).IEI disease categories included chronic granulomatous disease(8/19),severe combined immunodeficiency disease(5/19),susceptible disease to Mendelian mycobacteria(3/19),activated phosphatidylinositol 3-kinase delta syndrome(1/19),hyper IgE syndrome(1/19)and combined immunodeficiency disease(1/19).At a mean follow-up of 7 months,16 patients completed the follow-up(7 had good prognosis and 9 had poor prognosis).The mean duration of hospitalization was 39 days.The levels of CRP(19 vs.57 mg·/L,P=0.004)and white blood cell counts(9×109/L vs 12×109/L,P=0.04)were significantly improved after 4 weeks of treatment.Conclusion IEI combined with liver occupation is rare,and infection is the main cause.Liver abscess and hepatic tuberculosis account for the majority of cases,with a long duration of anti-infection treatment.Joint participation of physicians and surgeons is needed in therapeutic decision-making when necessary.关键词
原发性免疫缺陷病/肝脓肿/肝结核/人类疱疹病毒感染Key words
primary immunodeficiency disease/liver abscess/hepatic tuberculosis/Epstein-Barr virus infection分类
医药卫生引用本文复制引用
邹亮燕,孙碧君,姚琼,王文婕,侯佳,周钦华,姚海丽,孙金峤,王晓川..出生免疫缺陷合并肝占位病变单中心回顾性病例分析[J].中国实用儿科杂志,2024,39(11):839-844,6.
