Chronic granulomatous disease in Singapore

Author:

Chong, KW; Liew, WK; Thoon, KC

Author address:

KK Women’s and Children’s Hospital, Paediatric Medicine, Singapore, Singapore

Full conference title:

European Academy of Allergy and Clinical Immunology Congress 2015

Date: 5 August 2020

Abstract:

Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder of phagocytes.

Method: This paper describes the clinical course of 6 Singaporean patients diagnosed with CGD in our institution from year 2002 to 2012. Results: The median age at diagnosis was 2.1 years (range, 0.1–12.6 years). All 6 patients are of Chinese race. 5 patients suffered from X-linked recessive (XLR)-CGD and 1 from autosomal recessive (AR)- CGD. Pneumonia was the most common infection (11/35 infection episodes, 31%). Septicaemia was seen in 7/35 episodes. The rest consisted of 4 peri-anal abscesses, 4 lymphadenitis, 3 urinary tract infections, 2 splenic abscesses, 1 liver abscess, 1 skin abscess, 1 enteritis and 1 line sepsis. Burkholderia species was the most common bacteria isolated (6/41 organisms, 5 B.pseudomallei, 1 B.cepacia). Klebsiella pneumoniae was the second most common (4/41). There were only 2 Staphylococcal infections isolated. Aspergillosis was the most prevalent fungal infection seen (3/41), with the rest being Candida albicans (2/41) and yeast (1/41). 2 patients had confirmed Mycobacterium lymphadenitis (Bacillus Calmette-Guerin, BCG). 3 of the 6 patients received stem cell transplantation (SCT), 2 of whom fully engrafted and are well. 1 failed engraftment and died of septic shock secondary to B.cepacia pneumonia almost 2 years post-transplant. The only patient with AR-CGD has the mildest disease course and is alive and well. 2 patients died at presentation from overwhelming melioidosis sepsis (of which 1 was contributed by aspergillosis). All of the XLR-CGD patients received antibacterial and antifungal prophylaxis as well as immunomodulatory treatment (interferon-gamma).

Conclusion: CGD is associated with severe, life-threatening infections. Early diagnosis of CGD and rapid treatment of infections are critical. These data provide important insight in the clinical course of CGD in Singaporean population and can hopefully help to increase awareness and optimise treatment of these patients.

Abstract Number: 1086

Link to conference website:

Link Conference abstract: 

EAACI 2015

Conference abstracts, posters & presentations

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