There is a lack of data describing the exposure of Malaysian schoolchildren to Secondhand Smoke (SHS). The aim of this study is to identify factors influencing schoolchildren's exposures to SHS in Malaysia.

Introduction: Mucopolysaccharidoses (MPS) are a group of inherited disorders caused by the deficiency of a specific lysosomal enzyme involved in glycosaminoglycans (GAGs) degradation. This enzyme deficiency leads to accumulation of GAGs in the lysosomes, resulting in organ dysfunction and enlargement. We aimed to detect cases of MPS in patients with suggestive signs and symptoms. Methods: This was a 2-year cross-sectional study conducted during June 2014 to May 2016. Urine and whole blood samples were taken from high-risk MPS patients. All urine samples were analysed for GAGs and characterised by high resolution electrophoresis (HRE). Whole blood was collected in ethylenediaminetetraacetic acid (EDTA) tube and analysed for specific enzymes based on the clinical history and HRE findings. Results: From the 60 samples tested, 15 were positive for MPS; (Type I=1), (Type II=4), (Type IIIA=3), (Type IVA=1), (Type VI=6). The overall prevalence of MPS among high-risk Malaysian patients was 26% (95% CI 14.72% to 37.86%). One patient had mucolipidosis. The mean age of patients when diagnosed was 5 years old. Patients with MPS were more likely to present with hepatosplenomegaly compared to other symptoms (OR=0.974, p<.05). Conclusion: One in 4 high-risk patients was diagnosed with MPS being MPS type VI the most common among Malaysian patients. Hepatosplenomegaly was the most common symptom. Patients with suspected MPS should be screened by urinary GAGs analysis and diagnosis confirmed by enzyme activity analysis.

Many studies have shown that healthcare workers are exposed to higher levels of stress than other occupations. However, little research has been conducted on the use of biomarker tools to assess chronic stress and assess the effectiveness of stress management programs for healthcare workers. The aim of this study was to measure the effectiveness of a Stress Management Program by measuring Depression Anxiety Stress Scale (DASS-21) scores and hair cortisol concentration (HCC) levels of healthcare workers in public healthcare facilities. This study was a three-group, quasi-experiment study with pre- and post-study assessment sessions. A total of 119 healthcare workers (28 from Group A, 21 from Group B, and 70 from Group C) were followed for 6 months. Significant favorable intervention effects on DASS-21 scores were found in Group A (Effect size =0.6) as compared to Group B (Effect size=0.2) and Group C (Effect size =02) at the end of the program. Time and group interaction effects were examined using the repeated measure ANOVA test in which there was a significant group *time interaction and effect size of 0.2 (p-value <0.01) across all the groups with a reduction in hair cortisol concentrations following the program. The study showed that stress management under healthy communities, building the nation (KOSPEN PLUS) program was successful in reducing stress levels, as seen through a decrease in both stress scores and hair cortisol levels. This is the first study in Malaysia to use hair cortisol as a biomarker for stress management, suggesting its reliability.

Background: Lysosomal storage disorders (LSD) are storage disorders involving malfunction of degradation enzymes in lysosome. More than 50 types of LSD have been discovered, which includes the group of mucopolysaccharidoses (MPS), sphingolipidoses, oligosaccharidoses, mucolipidoses, lipoprotein storage disorders, lysosomal transport defects and neuronal ceroid lipofuscinoses and others. The aims of this study were to calculate the birth prevalence and carrier frequency of LSDs in the Malaysian population; to compare our results with reported epidemiologic data from other populations, and to describe the mutation spectrum in Malaysia. From 2008 to 2017, 2.1% (92/4338) suspected patients were diagnosed with LSD. Results: The prevalence of LSD in Malaysia was 1/231,904 live births. The combined prevalence of MPS was 1/292,401 with its subtype of MPS II presented the highest calculated birth prevalence of 1/221,425. Within the group of sphingolipidoses, the combine prevalence was 1/770,777 with Fabry as the most common disorder with calculated prevalence of 1/193,203 followed by metachromatic leukodystrophy (MLD) (1/494,514). MLD is more common among people of Iban ethnicity with the prevalence of 1/6,981. Pompe and mucolipidoses type II are the less common subtypes of LSD with a prevalence of 1/1,694,634 and 1/2,229,516, respectively.Conclusion: Overall, although the prevalence of LSD in Malaysia may be underestimated, the prevalence of MPS is consistent with other reported in East Asian countries.

This article contains information related to a recent study "Selective screening for detection of mucopolysaccharidoses (MPS) in Malaysia; A Two-year Study" Affandi et al., 2019. Any patient registered under government healthcare facilities in Malaysia and fit at least two inclusion criteria were included in this selective screening. Urine and blood from these high risk patients were obtained and analysed for glycosaminoglycans (GAGs) level before characterization using high resolution electrophoresis (HRE). Thereafter, enzyme assay for different types of MPS based on result of HRE were determined using specific substrate. Demographic data as well as laboratory findings were tabulated and analysed. The data of this study demonstrate between clinical presentation and laboratory findings among high risk patients of MPS and can be employed to improve diagnosis of MPS.

Dalam penyakit kepincangan metabolisme terwaris (inborn errors of metabolism – IEM), terdapat tujuh jenis penyakit berpunca daripada kekurangan enzim yang terlibat dalam metabolisme homosisteina. Paras homosisteina jumlah adalah berbeza mengikut jenis penyakit akibat kekurangan enzim ini. Maka, terdapat keperluan bagi mewujudkan suatu julat rujukan untuk membezakan antara populasi normal dengan populasi berpenyakit. Kajian ini menerangkan penemuan kami berkenaan julat rujukan bagi homosisteina jumlah di kalangan kanak-kanak di Malaysia. Sebanyak 3 ml darah telah diambil daripada 86 individu normal (52 orang kanak-kanak lelaki dan 34 orang kanak-kanak perempuan) dan seterusnya diproses serta dianalisis menggunakan kaedah Kromatografi Cecair Berprestasi Tinggi jenis fasa penukar ion (HPLC-IEC). Hasil kajian mendapati min homosisteina jumlah bagi keseluruhan populasi rujukan ialah sebanyak 8.1 ± 3.89 µM (95% selang keyakinan 7.3-8.9 µM). Julat rujukan yang diwujudkan untuk populasi rujukan adalah sebanyak 2.5 – 16.2 µM dengan had pemutus terendah (lower cut-off) dan had pemutus teratas (upper cut-off) masing-masing adalah 1.0 µM dan 21.0 µM. Julat yang berjaya diwujudkan ini didapati agak tinggi berbanding dengan penyelidik luar. Penemuan ini dapat mengurangkan jumlah kadar positif palsu semasa proses diagnosis penyakit dilakukan. Secara kesimpulannya, kami telah berjaya mewujudkan suatu julat rujukan bagi homosisteina jumlah untuk populasi kanak-kanak di Malaysia. DOI : http://dx.doi.org/10.17576/JSKM-2021-1902-03