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.

To study the characteristics of paediatric patients admitted to two non-COVID-19 teaching hospitals before and during the implementation of the Movement Control Order (MCO) in Malaysia. The retrospective study was performed in two phases (before MCO between February to March 2020, and during MCO lockdown between March to April 2020) in two teaching hospitals on the East Coast of Peninsular Malaysia. Hospitalized children <18 years were included and those coming for elective procedures or oncology treatment were excluded. The clinical data were retrieved from both hospitals’ admission records. There was a total of 496 and 191 admissions to two teaching hospitals on the East Coast of Peninsular Malaysia, respectively. A significant reduction in the number of non-COVID-19 hospital admissions was seen in both hospitals. For Hospital Universiti Sains Malaysia, the daily hospital admissions were reduced with a mean of 10 (before MCO) to 7 (during MCO) admissions/day (95% CI 1.54,7.54, p=0.001). In Sultan Ahmad Shah Medical Centre, a reduction in hospital admission was seen from 5 (before MCO) to 3 (during MCO) admissions/day (95% CI 0.61, 3.15, p=0.005). Our study observed a general drop in non-COVID-related respiratory illnesses and infectious disease cases during the MCO period. There were significant differences in neurological (p=0.029) and accident (p = 0.001) cases admissions observed between the two periods. Public health social distancing has indirectly reduced the non-COVID-19 related cases in the hospital settings with an increase in accidental injuries among the children during the MCO period.
COVID-19

Flow cytometer, a machine that utilizes the usage of light signals, has become one of the important machines or components in the studies of specific cell populations, cell surfaces, or even in detecting intracellular proteins. Due to its multiplex functionalities, while at the same time being very sensitive and specific, flow cytometer, or the technique of flow cytometry, has become a favourable and valuable tool for immunologists and clinicians for initial diagnosis of primary immunodeficiency diseases (PID), especially in studying cellular functional characteristics linked to PID. This paper will review key immunology tests of clinical flow cytometry, specifically in immunophenotyping, T cells classification and lymphocyte proliferation assays for their advantages and limitations in the field of PID. Importance of validation for the assays will be discussed. It is expected that development of validated immunology tests in clinical flow cytometry for PID diagnosis ensure standardized protocols, improve troubleshooting and disease interpretation, and improve the quality in patients’ diagnosis and management.
Flow cytometry

Despite having the highest prevalence of thalassemia in Malaysia, studies exploring factors that contribute to high thalassemia births in Sabah is still lacking. This study aimed to explore the reasons that may contribute to mothers in Sabah having multiple children with β-thalassemia major (β-TM). In-depth, semi-structured interview was conducted on 18 mothers with more than one β-TM child in two thalassemia treatment centres in Sabah. Thematic analysis was performed by two researchers and comparison of codes and sub-themes were made to verify the themes emerged. The thematic analysis revealed five core themes for reasons of mothers having multiple children with β-TM: 1) intention to have more children and acceptance towards child’s condition; 2) lack of understanding about thalassemia inheritance pattern; 3) barriers in prenatal screening; 4) barriers in abortion; and 5) ineffective family planning. Early identification of mothers with intention to have more children should be considered for active counselling. Providing continuous informational and financial support may increase uptake in prenatal screening, and involvement of local religious leaders to disseminate information regarding thalassemia prevention measures may improve effort to reduce thalassemia births in Sabah.
beta-Thalassemia