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Clinical and demographic pattern of chronic granulomatous disease (CGD) from a multicenter perspective: Malaysia's experience over 26 years.

Authors :
Noh LM
Latiff AHA
Ismail IH
Noah RM
Wahab AA
Hamid IJA
Ripen AM
Abdullah NB
Razali KA
Zainudin N
Bakon F
Kok LJ
Ali A
Aziz BBS
Latif HA
Mohamad SM
Zainudeen ZT
Hashim IF
Sendut IH
Nadarajaw T
Jamil FM
Ng DCE
Abidin MAZ
Source :
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology [Allergy Asthma Clin Immunol] 2021 May 17; Vol. 17 (1), pp. 50. Date of Electronic Publication: 2021 May 17.
Publication Year :
2021

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.<br />Methods: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.<br />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.<br />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.

Details

Language :
English
ISSN :
1710-1484
Volume :
17
Issue :
1
Database :
MEDLINE
Journal :
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology
Publication Type :
Academic Journal
Accession number :
34001231
Full Text :
https://doi.org/10.1186/s13223-021-00551-4