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

Noh LM 1 , Latiff AHA 2 , Ismail IH 3 , Noah RM 4 , Wahab AA 5 , Hamid IJA 6 Show all authors , Ripen AM 7 , Abdullah NB 8 , Razali KA 9 , Zainudin N 10 , Bakon F 11 , Kok LJ 12 , Ali A 13 , Aziz BBS 13 , Latif HA 13 , Mohamad SM 14 , Zainudeen ZT 6 , Hashim IF 6 , Sendut IH 15 , Nadarajaw T 16 , Jamil FM 17 , Ng DCE 18 , Abidin MAZ 3

Affiliations 

  • 1 Department of Paediatrics, Hospital Tunku Azizah, Ministry of Health Malaysia, Kuala Lumpur, Malaysia. lokman.m.noh@gmail.com
  • 2 Pantai Hospital, Kuala Lumpur, Malaysia
  • 3 Department of Paediatrics, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor, Malaysia
  • 4 Universiti Kuala Lumpur, Kuala Lumpur, Malaysia
  • 5 Department of Medical Microbiology and Immunology, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
  • 6 Primary Immunodeficiency Group, Cluster of Regenerative Medicine, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Kepala Batas, Penang, Malaysia
  • 7 Primary Immunodeficiency Unit, Institute for Medical Research, Kuala Lumpur, Malaysia
  • 8 Formerly At International Islamic University, Kuantan, Malaysia
  • 9 Al Islam Specialist Hospital, Previously At Institute of Pediatrics, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
  • 10 Sunway Medical Centre, Petaling Jaya, Selangor, Malaysia
  • 11 KPJ Kuching Specialist Hospital, Kuching, Sarawak, Malaysia
  • 12 Sarawak General Hospital, Kuching, Sarawak, Malaysia
  • 13 Department of Paediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
  • 14 Cluster of Life Sciences, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Kepala Batas, Penang, Malaysia
  • 15 Gleneagles Hospital, Kuala Lumpur, Malaysia
  • 16 Hospital Sultanah Bahiyah, Alor Setar, Kedah, Malaysia
  • 17 Hospital Serdang, Serdang, Selangor, Malaysia
  • 18 Hospital Tuanku Ja'afar, Seremban, Negeri Sembilan, Malaysia
Allergy Asthma Clin Immunol, 2021 May 17;17(1):50.
PMID: 34001231 DOI: 10.1186/s13223-021-00551-4

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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