Liver Abscess in Chronic Granulomatous Disease-Two Decades of Experience from a Tertiary Care Centre in North-West India

被引:9
|
作者
Pilania, Rakesh Kumar [1 ]
Rawat, Amit [1 ]
Vignesh, Pandiarajan [1 ]
Guleria, Sandesh [1 ]
Jindal, Ankur Kumar [1 ]
Das, Gargi [1 ]
Suri, Deepti [1 ]
Gupta, Anju [1 ]
Gupta, Kirti [2 ]
Chan, Koon-Wing [3 ]
Lau, Yu-Lung [3 ]
Imai, Kohsuke [4 ]
Singh, Surjit [1 ]
机构
[1] Post Grad Inst Med Educ & Res PGIMER, Allergy Immunol Unit, Dept Pediat, Adv Pediat Ctr, Chandigarh 160012, India
[2] Post Grad Inst Med Educ & Res PGIMER, Dept Histopathol, Chandigarh 160012, India
[3] Univ Hong Kong, Queen Mary Hosp, Dept Paediat & Adolescent Med, Hong Kong, Peoples R China
[4] Tokyo Med & Dent Univ TMDU, Community Pediat Perinatal & Maternal Med, Grad Sch Med & Dent Sci, Tokyo, Japan
关键词
Chronic granulomatous disease; liver abscess; corticosteroids; primary immunodeficiency disorder; CORTICOSTEROID-THERAPY; HEPATIC-ABSCESS; INFECTIONS;
D O I
10.1007/s10875-020-00938-9
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Purpose Most of the literature on liver abscess in chronic granulomatous disease (CGD) emanates from developed countries. Data from developing countries are scarce. In this study, we report clinical features, microbiological profile, and treatment difficulties encountered while managing liver abscesses in patients with CGD at a tertiary care centre in North-West India. Methodology Case records of children with CGD and liver abscesses at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India were analyzed. Results Seven of 68 patients (10.29%) with CGD presented with hepatic abscess. One patient had 2 recurrences. All were males and age-range at presentation was 7 months-22 years. Mutation analysis was carried out in all patients-3 had defects in CYBB gene; 2 in NCF1; 2 in NCF2 gene. Staphylococcus aureus was isolated from 5 patients. Duration of antimicrobial treatment ranged from 3 weeks to 7 months. Open drainage was required in 1 patient, and 1 patient was treated with a prolonged course of prednisolone. Two children succumbed to the illness. Conclusions This is the largest reported experience of liver abscesses in patients with CGD from the developing world. Staphylococcus aureus was the commonest pathogen isolated. In our experience, prolonged courses of antimicrobials are usually necessary in these patients. Glucocorticoids can reduce inflammatory response and facilitate early resolution of abscesses in CGD.
引用
收藏
页码:552 / 564
页数:13
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