DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW
Background: Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs). Methods: A 15-year retrospective review was conducted in KK Women's and Children's Hospital in Singapore, from January 2003 to Octobe...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-08-01
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| Series: | International Journal of Infectious Diseases |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1201971220304240 |
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| author | Rina Yue Ling Ong Su-Wan Bianca Chan Siu Jun Chew Woei Kang Liew Koh Cheng Thoon Chia-Yin Chong Chee Fu Yung Li-Hwei Sng Ah Moy Tan Rajat Bhattacharyya Saumya Shekhar Jamuar Jiin Ying Lim Jiahui Li Karen Donceras Nadua Kai-qian Kam Natalie Woon-Hui Tan |
| author_facet | Rina Yue Ling Ong Su-Wan Bianca Chan Siu Jun Chew Woei Kang Liew Koh Cheng Thoon Chia-Yin Chong Chee Fu Yung Li-Hwei Sng Ah Moy Tan Rajat Bhattacharyya Saumya Shekhar Jamuar Jiin Ying Lim Jiahui Li Karen Donceras Nadua Kai-qian Kam Natalie Woon-Hui Tan |
| author_sort | Rina Yue Ling Ong |
| collection | DOAJ |
| description | Background: Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs). Methods: A 15-year retrospective review was conducted in KK Women's and Children's Hospital in Singapore, from January 2003 to October 2017. Results: Ten patients were identified, the majority male (60.0%). The median age at presentation of symptoms of BCG infections was 3.8 (0.8 – 7.4) months. All the patients had likely underlying PIDS – four with Severe Combined Immunodeficiency (SCID), three with Mendelian Susceptibility to Mycobacterial Diseases (MSMD), one with Anhidrotic Ectodermal Dysplasia with Primary Immunodeficiency (EDA-ID), one with combined immunodeficiency (CID), and one with STAT-1 gain-of-function mutation. Definitive BCGosis was confirmed in all patients by the identification of Mycobacterium bovis subsp BCG from microbiological cultures. The susceptibility profiles of Mycobacterium bovis subsp BCG are as follows: Rifampicin (88.9%), Isoniazid (44.47%), Ethambutol (100.0%), Streptomycin (100.0%), Kanamycin (100.0%), Ethionamide (25.0%), and Ofloxacin (100.0%). Four patients (40.0%) received a three-drug regimen. Five patients (50.0%) underwent hematopoietic stem cell transplant (HSCT), of which three (60%) have recovered. Overall mortality was 50.0%. Conclusion: Disseminated BCG disease (BCGosis) should prompt immunology evaluation to determine the diagnosis of the immune defect. A three-drug regimen is adequate for treatment if the patient undergoes early HSCT. |
| first_indexed | 2024-12-12T20:18:21Z |
| format | Article |
| id | doaj.art-a42d9819a7094fddbda0c6908ceaeb01 |
| institution | Directory Open Access Journal |
| issn | 1201-9712 |
| language | English |
| last_indexed | 2024-12-12T20:18:21Z |
| publishDate | 2020-08-01 |
| publisher | Elsevier |
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| series | International Journal of Infectious Diseases |
| spelling | doaj.art-a42d9819a7094fddbda0c6908ceaeb012022-12-22T00:13:20ZengElsevierInternational Journal of Infectious Diseases1201-97122020-08-0197117125DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEWRina Yue Ling Ong0Su-Wan Bianca Chan1Siu Jun Chew2Woei Kang Liew3Koh Cheng Thoon4Chia-Yin Chong5Chee Fu Yung6Li-Hwei Sng7Ah Moy Tan8Rajat Bhattacharyya9Saumya Shekhar Jamuar10Jiin Ying Lim11Jiahui Li12Karen Donceras Nadua13Kai-qian Kam14Natalie Woon-Hui Tan15Department of Pharmacy, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Corresponding author. Department of Pharmacy, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899. Tel.: +65 6394 8026; fax: +65 6394 2607.Rheumatology and Immunology Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, SingaporeDepartment of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeRheumatology and Immunology Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, 10 Medical Drive, Singapore 117597, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Lee Kong Chian School of Medicine, National Technological University, 59 Nanyang Drive, Experimental Medicine Building, Singapore 636921, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, 10 Medical Drive, Singapore 117597, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Lee Kong Chian School of Medicine, National Technological University, 59 Nanyang Drive, Experimental Medicine Building, Singapore 636921, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Lee Kong Chian School of Medicine, National Technological University, 59 Nanyang Drive, Experimental Medicine Building, Singapore 636921, SingaporeDuke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Division of Pathology, Department of Microbiology, Singapore General HospitalYong Loo Lin School of Medicine, National University of Singapore, 10 Medical Drive, Singapore 117597, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Haematology/Oncology Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeDuke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Haematology/Oncology Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeGenetics Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, SingaporeGenetics Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, SingaporeInfectious Disease Service, Department of Pediatric Medicine, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, 10 Medical Drive, Singapore 117597, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore; Lee Kong Chian School of Medicine, National Technological University, 59 Nanyang Drive, Experimental Medicine Building, Singapore 636921, SingaporeBackground: Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs). Methods: A 15-year retrospective review was conducted in KK Women's and Children's Hospital in Singapore, from January 2003 to October 2017. Results: Ten patients were identified, the majority male (60.0%). The median age at presentation of symptoms of BCG infections was 3.8 (0.8 – 7.4) months. All the patients had likely underlying PIDS – four with Severe Combined Immunodeficiency (SCID), three with Mendelian Susceptibility to Mycobacterial Diseases (MSMD), one with Anhidrotic Ectodermal Dysplasia with Primary Immunodeficiency (EDA-ID), one with combined immunodeficiency (CID), and one with STAT-1 gain-of-function mutation. Definitive BCGosis was confirmed in all patients by the identification of Mycobacterium bovis subsp BCG from microbiological cultures. The susceptibility profiles of Mycobacterium bovis subsp BCG are as follows: Rifampicin (88.9%), Isoniazid (44.47%), Ethambutol (100.0%), Streptomycin (100.0%), Kanamycin (100.0%), Ethionamide (25.0%), and Ofloxacin (100.0%). Four patients (40.0%) received a three-drug regimen. Five patients (50.0%) underwent hematopoietic stem cell transplant (HSCT), of which three (60%) have recovered. Overall mortality was 50.0%. Conclusion: Disseminated BCG disease (BCGosis) should prompt immunology evaluation to determine the diagnosis of the immune defect. A three-drug regimen is adequate for treatment if the patient undergoes early HSCT.http://www.sciencedirect.com/science/article/pii/S1201971220304240DisseminatedBacillus Calmette-GuérinBCGVaccineBCGosisMycobacterium bovis subsp BCG |
| spellingShingle | Rina Yue Ling Ong Su-Wan Bianca Chan Siu Jun Chew Woei Kang Liew Koh Cheng Thoon Chia-Yin Chong Chee Fu Yung Li-Hwei Sng Ah Moy Tan Rajat Bhattacharyya Saumya Shekhar Jamuar Jiin Ying Lim Jiahui Li Karen Donceras Nadua Kai-qian Kam Natalie Woon-Hui Tan DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW International Journal of Infectious Diseases Disseminated Bacillus Calmette-Guérin BCG Vaccine BCGosis Mycobacterium bovis subsp BCG |
| title | DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW |
| title_full | DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW |
| title_fullStr | DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW |
| title_full_unstemmed | DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW |
| title_short | DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER 15-YEAR RETROSPECTIVE REVIEW |
| title_sort | disseminated bacillus calmette guerin infections and primary immunodeficiency disorders in singapore a single center 15 year retrospective review |
| topic | Disseminated Bacillus Calmette-Guérin BCG Vaccine BCGosis Mycobacterium bovis subsp BCG |
| url | http://www.sciencedirect.com/science/article/pii/S1201971220304240 |
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