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Risks of seroconversion of hepatitis B, hepatitis C and human immunodeficiency viruses in children with multitransfused thalassaemia major

Lee WS, Teh CM, Chan LL

J Paediatr Child Health, 2005 May-Jun;41(5-6):265-8.
PMID: 15953326 DOI: 10.1111/j.1440-1754.2005.00608.x

OBJECTIVES: To estimate the risks of seroconversion of hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency viruses (HIV) in children with multitransfused thalassaemia at a thalassaemic clinic in Kuala Lumpur, Malaysia.
METHODS: Seventy-two children (39 males, median age 11.3 years, 2.5th-97.5th centile: 1.4-19.2 years) with thalassaemia major were studied. The risks of seroconversion of HBV, HCV and HIV were estimated by comparing the seroprevalences of hepatitis B surface antigen (HBsAg), anti-HCV and anti-HIV between a defined starting point and an end point. The end point was the point when latest serological results were available while the starting point was when regular transfusion was commenced, or approximately 5 years before the end point when the duration of transfusion was longer.
RESULTS: The median duration of the study was 49 months (range 8-69 months, total 2953 patient-months). There were 2605 transfusion episodes and 4154 units of blood transfused (0.88 transfusion episode/patient per month, 1.41 units of blood transfused/patient per month). There were three new seroconversions for anti-HCV but none for HBsAg and anti-HIV. The risk of seroconversion for HCV was one in 1384 units of blood transfused (95% CI: 4000-472). The seroprevalence rates at the starting and end points were: HBsAg (1%, 1%), anti-HCV (10%, 13%) and anti-HIV (0%, 0%), respectively.
CONCLUSIONS: The estimated risk of acquiring HCV infection in children receiving multiple blood transfusions in this study is surprisingly higher than the generally accepted estimated risk. Other routes of transmission may be important. A prospective, multicentre study to estimate such risks more precisely is needed.
Neurologic manifestations and complications of pandemic influenza A H1N1 in Malaysian children: what have we learnt from the ordeal?

Muhammad Ismail HI, Teh CM, Lee YL, National Paediatric H1N1 Study Group

Brain Dev, 2015 Jan;37(1):120-9.
PMID: 24746706 DOI: 10.1016/j.braindev.2014.03.008

In 2009, pandemic influenza A H1N1 emerged in Mexico and subsequently spread worldwide. In Malaysia, there were more than a thousand of confirmed cases among children. The general clinical characteristics of these children have been well-published. However, the description of neurologic complications is scarce.
Congenital myasthenic syndrome with novel pathogenic variants in the COLQ gene associated with the presence of antibodies to acetylcholine receptors

Tay CG, Fong CY, Li L, Ganesan V, Teh CM, Gan CS, et al.

J Clin Neurosci, 2020 Feb;72:468-471.
PMID: 31831253 DOI: 10.1016/j.jocn.2019.12.007

Congenital myasthenic syndrome (CMS) is a heterogeneous group of inherited disorder which does not associate with anti-acetylcholine receptor (AChR) antibody. The presence of AChR autoantibody is pathogenic and highly sensitive and specific for autoimmune myasthenia gravis (MG). We describe 2 children from unrelated families who presented with hypotonia, ptosis and fatigability in early infancy with anti-AChR antibodies detected via ELISA on 2 separate occasions in the sera. Both were treated as refractory autoimmune MG due to poor clinical response to acetylcholinesterase inhibitor and immunotherapy. In view of the atypical clinical features, genetic studies of CMS were performed and both were confirmed to have novel pathogenic mutations in the COLQ gene. To the best of our knowledge, the presence of anti-AChR antibody in COLQ-related CMS has never been reported in the literature. The clinical presentation of early onset phenotype, and refractoriness to acetylcholinesterase inhibitor and immunotherapy should prompt CMS as a differential diagnosis.
Neurological outcome and health-related quality of life among Malaysian children with stroke

Fong CY, Kangatharan PASS, Lim WK, Ong LC, Li L, Azanan MS, et al.

Brain Dev, 2025 Apr;47(2):104339.
PMID: 39999621 DOI: 10.1016/j.braindev.2025.104339

AIM: Describe the neurological and functional outcomes, and health-related quality of life (HRQL) of Malaysian children with stroke; and explore factors associated with poor outcomes and lower HRQL.
METHOD: Cross-sectional study of Malaysian stroke children who were followed-up for at least 2 years. Paediatric Stroke Outcome Measure (PSOM) and modified Rankin scale (mRS) were utilised to assess neurological and functional outcomes. HRQL was assessed using PedsQL (Paediatric Quality of Life inventory) 4.0. Multiple regression analysis was used to determine factors associated with poor PSOM, poor mRS, and lower HRQL.
RESULTS: Seventy-six children were recruited. Majority have good mRS (72.4 %) and good PSOM (75 %). PedsQL total, physical health summary and psychosocial health summary mean scores were 74.2(SD 21.57), 74.2(SD 28.16) and 76.13(SD 18.66) respectively, which were significantly lower compared with healthy Malaysian children (p ≤ 0.001). Learning disability post-stroke was associated with poor PSOM and poor mRS; while cardiac surgery was associated with poor mRS. Learning disability post-stroke was associated with lower total, physical health summary and psychosocial health summary PedsQL scores. Poor mRS was associated with lower total PedsQL scores while poor PSOM was associated with lower total and physical health summary PedsQL.
CONCLUSION: Although majority of Malaysian children with stroke had good long-term neurological and functional outcomes, they had lower HRQL compared to healthy Malaysian children. Optimizing care for cardiac surgery to reduce the risk of perioperative stroke may lead to improved neurological outcome. There is a need to address the issues of learning disability post-stroke and poor neurological outcome to reduce their impact on HRQL.
Quality of life of children with tuberous sclerosis complex

Fong CY, Ng K, Kong AN, Ong LC, Rithauddin MA, Thong MK, et al.

Arch Dis Child, 2019 10;104(10):972-978.
PMID: 31122923 DOI: 10.1136/archdischild-2018-316394

AIM: Evaluation of impaired quality of life (QOL) of Malaysian children with tuberous sclerosis complex (TSC) and its possible risk factors.
METHOD: Cross-sectional study on 68 parents of Malaysian children aged 2-18 years with TSC. QOL was assessed using proxy-report Paediatric Quality of Life Inventory (PedsQL) V.4.0, and scores compared with those from a previous cohort of healthy children. Parents also completed questionnaires on child behaviour (child behaviour checklist (CBCL)) and parenting stress (parenting stress index-short form). Multiple regression analysis was used to determine sociodemographic, medical, parenting stress and behavioural factors that impacted on QOL.
RESULTS: The mean proxy-report PedsQL V.4.0 total scale score, physical health summary score and psychosocial health summary score of the patients were 60.6 (SD 20.11), 65.9 (SD 28.05) and 57.8 (SD 19.48), respectively. Compared with healthy children, TSC patients had significantly lower mean PedsQL V.4.0 total scale, physical health and psychosocial health summary scores (mean difference (95% CI): 24 (18-29), 20 (12-27) and 26 (21-31) respectively). Lower total scale scores were associated with clinically significant CBCL internalising behaviour scores, age 8-18 years and Chinese ethnicity. Lower psychosocial health summary scale scores were associated with clinically significant CBCL internalising behaviour scores, Chinese ethnicity or >1 antiepileptic drug (AED).
CONCLUSION: Parents of children with TSC reported lower PedsQL V.4.0 QOL scores in all domains, with psychosocial health most affected. Older children, those with internalising behaviour problems, of Chinese ethnicity or on >1 AED was at higher risk of lower QOL. Clinicians need to be vigilant of QOL needs among children with TSC particularly with these additional risk factors.
Factors associated with outcomes of severe acute necrotizing encephalopathy: A multicentre experience in Malaysia

Lee VWM, Khoo TB, Teh CM, Heng HS, Li L, Yusof YLM, et al.

Dev Med Child Neurol, 2023 Sep;65(9):1256-1263.
PMID: 36748407 DOI: 10.1111/dmcn.15536

This case series compared clinical variables and various combinations of immunotherapy received with outcomes of patients with severe acute necrotizing encephalopathy (ANE). We performed a retrospective review of clinical variables, immunotherapy received, and outcomes (based on the modified Rankin Scale) in Malaysia between February 2019 and January 2020. Twenty-seven children (12 male), aged 7 months to 14 years (mean 4 years) at diagnosis were included. Of these, 23 had an ANE severity score of 5 to 9 out of 9 (high risk). Eleven patients received tocilizumab (four in combination with methylprednisolone [MTP], seven with MTP + intravenous immunoglobulin [IVIG]) and 16 did not (two received MTP alone, 14 received MTP + IVIG). Nine died. Among the survivors, six had good outcomes (modified Rankin Score 0-2) at 6 months follow-up. All patients who received tocilizumab in combination with MTP + IVIG survived. Twenty children received first immunotherapy within 48 hours of admission. No significant association was found between the timing of first immunotherapy with outcomes. Those with brainstem dysfunction (p = 0.016) were observed to have poorer outcomes. This study showed a trend towards better survival when those with severe ANE were treated with tocilizumab in combination with MTP + IVIG. However, larger studies will be needed to determine the effect of this regime on the long-term outcomes.