Clinical features of chronic granulomatous disease :analysis of 27 cases
10.3760/cma.j.issn.1000-6680.2018.06.005
- VernacularTitle:慢性肉芽肿病27例临床分析
- Author:
Yungu CHEN
1
;
Shan XING
;
Guangjie CHEN
Author Information
1. 200025,上海交通大学医学院免疫学与微生物学系 上海市免疫学研究所
- Keywords:
Granulomatous disease;
chronic;
Hematopoietic stem cell transplantation;
Infant;
newborn;
Infection
- From:
Chinese Journal of Infectious Diseases
2018;36(6):345-348
- CountryChina
- Language:Chinese
-
Abstract:
Objective To evaluate the clinical features and treatment of chronic granulomatous disease (CGD ) . Methods A total of 27 CGD patients diagnosed by neutrophil respiratory burst in Zhengzhou Children Hospital from January 2007 to June 2017 were selected .The clinical ,pathogenic and radiological characteristics were retrospectively analyzed ,and the treatment and prognosis were evaluated . Chi-square test was used for statistical analysis .Results Among the 27 cases ,all patients presented with pulmonary infection (27/27) and 55 .6％ had cutaneous infection (15/27) .Lymph node enlargement was presented in 48 .1％ (13/27) of patients .Eleven neonates and four infants had cutaneous infection ,which showed significant difference (χ2= 11 .408 , P < 0 .05 ) . The results of pathogenic detection revealed 66 .7％ (18/27) fungal infection ,25 .9％ (7/27) bacterial infection and 44 .4％ (12/27) mycobacterial infection .The incidence rate of fungal infection in neonates was significant higher than that of children (χ2 = 6 .075 , P < 0 .05) .Radiologic examination showed that 12 cases had calcification of subaxillary lymph nodes ,4 cases had pneumonia and thick-walled cavity ,13 cases had multiple nodules ,5 cases had massive high-density shadow and 4 cases had pulmonary abscess .The clinical pathogenic and radiologic features were similar between neonates and children .Treatment options included long-term combination therapy of bactericidal and fungicidal medicines .Conclusions Clinical manifestations of CGD are mainly pulmonary infection as well as cutaneous infection . Combination therapy with multiple medicines is effective .Hematopoietic stem cell transplatation plus retroviral vectors genetic therapy may be the trend of CGD treatment in the future .
