Rakesh Kumar Pilania1, Amit Rawat2, Pandiarajan Vignesh1, Sandesh Guleria1, Ankur Kumar Jindal1, Gargi Das1, Deepti Suri1, Anju Gupta1, Kirti Gupta3, Koon-Wing Chan4, Yu-Lung Lau4, Kohsuke Imai5, Surjit Singh1. 1. Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India. 2. Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India. rawatamit@yahoo.com. 3. Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India. 4. Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong. 5. Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
Abstract
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.
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.
Authors: J Merlijn van den Berg; Elsbeth van Koppen; Anders Ahlin; Bernd H Belohradsky; Ewa Bernatowska; Lucien Corbeel; Teresa Español; Alain Fischer; Magdalena Kurenko-Deptuch; Richard Mouy; Theoni Petropoulou; Joachim Roesler; Reinhard Seger; Marie-José Stasia; Niels H Valerius; Ron S Weening; Baruch Wolach; Dirk Roos; Taco W Kuijpers Journal: PLoS One Date: 2009-04-21 Impact factor: 3.240