ACHROMOBACTER XYLOSOXIDANS BACTEREMIA IN A CHILD WITH X-LINKED AGAMMAGLOBULINEMIA.

Mohammad Qazreen AHMAD SHAWALUDIN1, Mariana DAUD2, Fazila MAT ARIFIN2, Ilie Fadzilah HASHIM3, Zarina Thasneem ZAINUDEEN3, Adiratna MAT RIPEN4,  Fahisham TAIB1,  Intan Juliana ABD HAMID3.

1Department of Pediatric, Hospital Universiti Sains Malaysia, 16150, Kubang Kerian, Kelantan, Malaysia.

2Department of Pediatric, Hospital Raja Perempuan Zainab II, Kota Bharu, Kelantan, Malaysia.

3Primary Immunodeficiency Diseases Group, Department of Clinical Medicine, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Bertam 13200 Kepala Batas, Pulau Pinang, Malaysia.

4Primary Immunodeficiency Unit, Allergy and Immunology Research Centre, Institute for Medical Research, National Institutes of Health, Ministry of Health, Malaysia.

 

ABSTRACT

X-linked agammaglobulinaemia (XLA) is caused by mutation of the BTK gene. Early immunological screening is important in children with recurrent infection to avoid delay in diagnosis and treatment. A 9-year-old boy with delayed XLA diagnosis presented with a history of recurrent sinopulmonary infection since the age of 1 year old. He had a severe Achromobacter xylosoxidans bacteraemia at the age of 8 years that was successfully treated with intravenous antibiotics. Further immunological screening and genetic testing confirmed the diagnosis of XLA and he was treated with intravenous immunoglobulin replacement therapy. This case provides a valuable information on the diagnosis, treatment and management of XLA, emphasising the importance of early immunological investigations to avoid diagnostic delay.

Keyword: X-linked agammaglobulinemia, Brutonís Disease, Achromobacter xylosoxidans, Immunological screening.

Corresponding Author: Intan Juliana Abd Hamid, Primary Immunodeficiency Diseases Group, Cluster of Regenerative Medicine, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Bertam 13200 Kepala Batas, Pulau Pinang, Malaysia.

Email: intanj@usm.my; Phone number  +6045622532

Brunei Int Med J.2023;19:26-30

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