Lokman Mohd Noh1,2, Amir Hamzah Abdul Latiff3, Intan Hakimah Ismail4, Rahim Md Noah5, Asrul Abdul Wahab6, Intan Juliana Abd Hamid7, Adiratna Mat Ripen8, Nasuruddin B Abdullah9,10, Kamarul Azhar Razali11, Norzila Zainudin12,13, Florence Bakon14, Long Juan Kok15, Adli Ali16, Bilkis Banu SAbd Aziz16, Hasniah Abdul Latif16, Siti Mardhiana Mohamad17, Zarina Thasneem Zainudeen7, Ilie Fadzilah Hashim7, Iean Hamzah Sendut18,19, Thiyagar Nadarajaw20, Faizah Mohamed Jamil21, David C E Ng22,15, Mohd Azri Zainal Abidin4. 1. Department of Paediatrics, Hospital Tunku Azizah, Ministry of Health Malaysia, Kuala Lumpur, Malaysia. lokman.m.noh@gmail.com. 2. Department of Paediatrics, UKM, Kuala Lumpur, Malaysia. lokman.m.noh@gmail.com. 3. Pantai Hospital, Kuala Lumpur, Malaysia. 4. Department of Paediatrics, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor, Malaysia. 5. Universiti Kuala Lumpur, Kuala Lumpur, Malaysia. 6. Department of Medical Microbiology and Immunology, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia. 7. Primary Immunodeficiency Group, Cluster of Regenerative Medicine, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Kepala Batas, Penang, Malaysia. 8. Primary Immunodeficiency Unit, Institute for Medical Research, Kuala Lumpur, Malaysia. 9. Formerly At International Islamic University, Kuantan, Malaysia. 10. Institute for Medical Research, Kuala Lumpur, Malaysia. 11. Al Islam Specialist Hospital, Previously At Institute of Pediatrics, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia. 12. Sunway Medical Centre, Petaling Jaya, Selangor, Malaysia. 13. Institute of Pediatrics, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia. 14. KPJ Kuching Specialist Hospital, Kuching, Sarawak, Malaysia. 15. Sarawak General Hospital, Kuching, Sarawak, Malaysia. 16. Department of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia. 17. Cluster of Life Sciences, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Kepala Batas, Penang, Malaysia. 18. Gleneagles Hospital, Kuala Lumpur, Malaysia. 19. Faculty of Medicine, University Malaya, Kuala Lumpur, Malaysia. 20. Hospital Sultanah Bahiyah, Alor Setar, Kedah, Malaysia. 21. Hospital Serdang, Serdang, Selangor, Malaysia. 22. Hospital Tuanku Ja'afar, Seremban, Negeri Sembilan, Malaysia.
Abstract
BACKGROUND: A retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia. An analysis of the available database would establish clinical characteristics, diagnoses and outcome including microbiologic pattern. Studying the demography allows us to document the occurrence of CGD amongst multiethnic groups and its geographical distribution for Malaysia. METHODS: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed. RESULTS: Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia. CONCLUSION: This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.
BACKGROUND: A retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia. An analysis of the available database would establish clinical characteristics, diagnoses and outcome including microbiologic pattern. Studying the demography allows us to document the occurrence of CGD amongst multiethnic groups and its geographical distribution for Malaysia. METHODS: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed. RESULTS: Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGDpatients with C. violaceuminfection displayed CD4 + (T helper cells) lymphopenia. CONCLUSION: This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceuminfection is associated with a higher mortality in CGDpatients in Malaysia. All the CGDpatients with C. violaceuminfection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.
Authors: L M Noh; B A Nasuruddin; A H Abdul Latiff; R M Noah; M R Kamarul Azahar; M Z Norzila; I Zulkifli; H K Gill; N I Nik Zainal; A W M Suhaimi; N Y Nik Khairulddin; H Maraina; H I Intan; I Mat; M Shahnaz; Azizi B H O Journal: Med J Malaysia Date: 2013
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
Authors: Intan Juliana Abd Hamid; Nur Adila Azman; Andrew R Gennery; Ernest Mangantig; Ilie Fadzilah Hashim; Zarina Thasneem Zainudeen Journal: Front Immunol Date: 2020-08-26 Impact factor: 7.561
Authors: Julia Nordin; Leire Solís; Johan Prévot; Nizar Mahlaoui; Helen Chapel; Silvia Sánchez-Ramón; Adli Ali; John W Seymour; Martine Pergent Journal: Front Immunol Date: 2021-11-18 Impact factor: 7.561