Amit Rawat1, Pandiarajan Vignesh2, Avinash Sharma2, Jitendra K Shandilya2, Madhubala Sharma2, Deepti Suri2, Anju Gupta2, Vikas Gautam3, Pallab Ray3, Shivaprakash M Rudramurthy3, Arunaloke Chakrabarti3, Kohsuke Imai4, Shigeaki Nonoyama4, Osamu Ohara5, Yu L Lau6, Surjit Singh2. 1. Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India. rawatamit@yahoo.com. 2. Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India. 3. Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India. 4. Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan. 5. Kazusa DNA Research Institute, Kisarazu, Chiba, Japan. 6. Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong Special Administrative Region, China.
Abstract
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed. RESULTS: Thirty-eight children were diagnosed to have CGD. Median follow-up of patients was 2 years (interquartile range 0.75, 6.0). Staphylococcus aureus and Pseudomonas spp. were the two most common causative bacteria isolated. Aspergillus was the most common fungus isolated. The most common organ involved was the lung (94.7%). Liver abscesses were identified in 5 patients (13.2%), and 20 (52.6%) patients had lymphadenitis. Infections with Pseudomonas spp. were high in our cohort (15.7%) compared to the other studies. Infections with some unusual organisms (e.g., Fusarium dimerium and Chryseobacterium gleum) were also seen in our cohort. Children with X-linked CGD presented earlier and also had a greater number of infections as compared to autosomal recessive CGD. CONCLUSIONS: Various socioeconomic factors coupled with the lack of awareness and paucity of readily available diagnostic facilities for primary immunodeficiencies accounted for a late clinical presentation with severe infections and increased mortality (28.9%) in our cohort. However, mortality was similar in X-linked and autosomal recessive CGD as was the number of fungal infections. The incidence of infections and mortality was significantly lower after initiation of antibacterial and antifungal prophylaxis.
PURPOSE:Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed. RESULTS: Thirty-eight children were diagnosed to have CGD. Median follow-up of patients was 2 years (interquartile range 0.75, 6.0). Staphylococcus aureus and Pseudomonas spp. were the two most common causative bacteria isolated. Aspergillus was the most common fungus isolated. The most common organ involved was the lung (94.7%). Liver abscesses were identified in 5 patients (13.2%), and 20 (52.6%) patients had lymphadenitis. Infections with Pseudomonas spp. were high in our cohort (15.7%) compared to the other studies. Infections with some unusual organisms (e.g., Fusarium dimerium and Chryseobacterium gleum) were also seen in our cohort. Children with X-linked CGD presented earlier and also had a greater number of infections as compared to autosomal recessive CGD. CONCLUSIONS: Various socioeconomic factors coupled with the lack of awareness and paucity of readily available diagnostic facilities for primary immunodeficiencies accounted for a late clinical presentation with severe infections and increased mortality (28.9%) in our cohort. However, mortality was similar in X-linked and autosomal recessive CGD as was the number of fungal infections. The incidence of infections and mortality was significantly lower after initiation of antibacterial and antifungal prophylaxis.
Entities:
Keywords:
Chronic granulomatous disease; North India; bacteria; fungi; infections; mortality
Authors: M de Boer; P M Hilarius-Stokman; J P Hossle; A J Verhoeven; N Graf; R T Kenney; R Seger; D Roos Journal: Blood Date: 1994-01-15 Impact factor: 22.113
Authors: Lizbeth Blancas-Galicia; Eros Santos-Chávez; Caroline Deswarte; Quentin Mignac; Isabel Medina-Vera; Ximena León-Lara; Manon Roynard; Selma C Scheffler-Mendoza; Ricardo Rioja-Valencia; Alexandra Alvirde-Ayala; Saul O Lugo Reyes; Tamara Staines-Boone; Jorge García-Campos; Omar J Saucedo-Ramírez; Blanca E Del-Río Navarro; Antonio Zamora-Chávez; Arturo López-Larios; Susana García-Pavón-Osorio; Eugenia Melgoza-Arcos; María R Canseco-Raymundo; Dolores Mogica-Martínez; Marco Venancio-Hernández; Daniel Pacheco-Rosas; Sigifredo Pedraza-Sánchez; Martha Guevara-Cruz; Federico Saracho-Weber; Berenise Gámez-González; Guillermo Wakida-Kuzunoki; Ana R Morán-Mendoza; Ana P Macías-Robles; Roselia Ramírez-Rivera; Eugenia Vargas-Camaño; Carmen Zarate-Hernández; Héctor Gómez-Tello; Emmanuel Ramírez-Sánchez; Fredy Ruíz-Hernández; Domingo Ramos-López; Héctor Acuña-Martínez; María L García-Cruz; María G Román-Jiménez; Marina G González-Villarreal; Aristóteles Álvarez-Cardona; Beatriz A Llamas-Guillén; Jennifer Cuellar-Rodríguez; Alberto Olaya-Vargas; Nideshda Ramírez-Uribe; Stéphanie Boisson-Dupuis; Jean-Laurent Casanova; Francisco J Espinosa-Rosales; Jeanet Serafín-López; Marco Yamazaki-Nakashimada; Sara Espinosa-Padilla; Jacinta Bustamante Journal: J Clin Immunol Date: 2020-02-10 Impact factor: 8.542