METHODS: We have conducted a survey of physicians to document the medical history of patients with MPS VII. The survey included anonymous information on patient demographics, family history, mode of diagnosis, age of onset, signs and symptoms, severity, management, clinical features and natural progression of the disease.
RESULTS: We collected information on 56 patients from 11 countries. Patients with MPS VII were classified based on their phenotype into three different groups: (1) neonatal non-immune hydrops fetalis (NIHF) (n=10), (2) Infantile or adolescent form with history of hydrops fetalis (n=13) and (3) Infantile or adolescent form without known hydrops fetalis (n=33). Thirteen patients with MPS VII who had the infantile form with history of hydrops fetalis and survived childhood, had a wide range of clinical manifestations from mild to severe. Five patients underwent bone marrow transplantation and one patient underwent enzyme replacement therapy with recombinant human GUS.
CONCLUSIONS: MPS VII is a pan-ethnic inherited lysosomal storage disease with considerable phenotypical heterogeneity. Most patients have short stature, skeletal dysplasia, hepatosplenomegaly, hernias, cardiac involvement, pulmonary insufficiency and cognitive impairment. In these respects it resembles MPS I and MPS II. In MPS VII, however, one unique and distinguishing clinical feature is the unexpectedly high proportion of patients (41%) that had a history of NIHF. Presence of NIHF does not, by itself, predict the eventual severity of the clinical course, if the patient survives infancy.
Methods: Forty histologically proven glioma patients underwent a standard MRI tumour protocol with the addition of IOP sequence. The regions of tumour (solid enhancing, solid non-enhancing, and cystic regions) were delineated using snake model (ITK-SNAP) with reference to structural and diffusion MRI images. The lipid distribution map was constructed based on signal loss ratio (SLR) obtained from the IOP imaging. The mean SLR values of the regions were computed and compared across the different glioma grades.
Results: The solid enhancing region of glioma had the highest SLR for both Grade II and III. The mean SLR of solid non-enhancing region of tumour demonstrated statistically significant difference between the WHO grades (grades II, III & IV) (mean SLRII = 0.04, mean SLRIII = 0.06, mean SLRIV = 0.08, & p
METHODS: This is a prospective cross-sectional study of all injured motorcyclists and pillion riders that were admitted to Hospital Sultanah Aminah and treated by the trauma surgery team from May 2011 to February 2015. Only injured motorcyclists and pillion riders were included in this study. Patient demography and predictors leading to mortality were identified. Significant predictors on univariate analysis were further analysed with multivariate analysis.
RESULTS: We included 1653 patients with a mean age of (35 ± 16.17) years that were treated for traumatic injuries due to motorcycle accidents. The mortality rate was 8.6% (142) with equal amount of motorcycle riders (788) and pillion riders (865) that were injured. Amongst the injured were male predominant (1 537) and majority of ethnic groups were the Malays (897) and Chinese (350). Severity of injury was reflected with a mean Revised Trauma Score (RTS) of 7.31 ± 1.29, New Injury Severity Score (NISS) of 19.84 ± 13.84 and Trauma and Injury Severity Score (TRISS) of 0.91 ± 0.15. Univariate and multivariate analysis revealed that age≥35, lower GCS, head injuries, chest injuries, liver injuries, and small bowel injuries were significant predictors of motorcycle trauma related deaths with p
OUTBREAK SITUATION: A stringent screening process at all airports in Malaysia was enforced after the first case outside China was reported in Thailand. Up to April 14, 2020, Malaysia had reported two waves of COVID-19 cases, with the first wave ending successfully within less than 2 months. In early March 2020, the second wave occurred, with worrying situations.
ACTIONS TAKEN: The Government of Malaysia enforced a Movement Control Order starting on March 18, 2020 to break the chain of COVID-19. The media actively spread the hashtag #stayhome. Non-governmental organizations, as well as prison inmates, started to produce personal protective equipment for frontliners. Various organizations hosted fundraising events to provide essentials mainly to hospitals. A provisional hospital was set up and collaborations with healthcare service providers were granted, while additional laboratories were assigned to enhance the capabilities of the Ministry of Health.
ECONOMIC DOWNTURN: An initial financial stimulus amounting to RM 20.0 billion was released in February 2020, before the highlighted PRIHATIN Package, amounting to RM 250 billion, was announced. The PRIHATIN Package has provided governmental support to society, covering people of various backgrounds from students and families to business owners.
METHODS: In the current study, 2074 students (706 males), filled out the Meaning in Life Questionnaire, with subscales of Search for Meaning (MLQ-S) and Presence of Meaning (MLQ-P); the Future Disposition Inventory-24 (FDI-24), with subscales of Positive Focus (PF), Suicide Orientation (SO), and Negative Focus (NF); and the Beck Hopelessness Scale (BHS). These scales measure protective and risk factors that are linked to suicidal behaviors; while suicidal behaviors were measured by the Suicidal Behaviors Questionnaire-Revised (SBQ-R). Mediation analyses were performed to test the models with both the MLQ-S and MLQ-P as the mediators between a) hopelessness, as measured by BHS and suicidal behaviors; and b) PF, SO, and NF, as measured by FDI-24, and suicidal behaviors.
RESULTS: We found that only MLQ-P mediated the relation between hopelessness and suicidal behaviors; while both MLQ-P and MLQ-S mediated PF, SO, and NF (as measured by FDI-24), and suicidal behaviors, respectively.
CONCLUSION: Meaning in life, including both the presence of meaning in life and search for meaning, can be good protective factors against suicidal behaviors.
METHODS: The methodological and cross-sectional study design was used to translate, culturally adapt it, and validate PSPSQ 2.0 in Nepalese. The Nepalese version of PSPSQ 2.0 went through the full linguistic validation process and was evaluated in 300 patients visiting different community pharmacies in Kathmandu district, Nepal. Exploratory factor analysis was carried out using principal component analysis with varimax rotation, and Cronbach's alpha was used to evaluate the reliability.
RESULTS: Three-hundred patients were recruited in this study. Participants ranged in age from 21 to 83 years; mean age was 53.93 years (SD: 15.21). 62% were females, and 34% educational level was above 12 and university level. Only 7% of the participants were illiterate. Kaiser-Meyer-Olkinwas found to be 0.696, and Bartlett's test of sphericity was significant with a chi-square test value of 3695.415. A principal axis factor analysis conducted on the 20 items with orthogonal rotation (varimax). PSPSQ 2.0 Nepalese version (20 items) had a good internal consistency (Cronbach's alpha = 0.758). Item-total correlations were reviewed for the items in each of the three domains of PSPSQ 2.0.
CONCLUSION: The PSPSQ 2.0 Nepalese version demonstrated acceptable validity and reliability, which can be used in the Nepalese population for evaluating the satisfaction of patients with pharmacist services in both community pharmacy and research.
Aims: The primary objective was to evaluate whether addiction-specific cues compared with neutral cues, i.e., negative emotional valence cues vs. positive emotional valence cues, would elicit activation of the dopaminergic reward network (i.e., precuneus, nucleus accumbens, and amygdala) and consecutive deactivation of the executive control network [i.e., medial prefrontal cortex (mPFC) and dorsolateral prefrontal cortex (dlPFC)], in the PIGU subjects.
Method: An fMRI cue-induced reactivity study was performed using negative emotional valence, positive emotional valence, and truly neutral cues, using Instagram themes. Thirty subjects were divided into PIGU and healthy control (HC) groups, based on a set of diagnostic criteria using behavioral tests, including the Modified Instagram Addiction Test (IGAT), to assess the severity of PIGU. In-scanner recordings of the subjects' responses to the images and regional activity of the neural addiction pathways were recorded.
Results: Negative emotional valence > positive emotional valence cues elicited increased activations in the precuneus in the PIGU group. A negative and moderate correlation was observed between PSC at the right mPFC with the IGAT scores of the PIGU subjects when corrected for multiple comparisons [r = -0.777, (p < 0.004, two-tailed)].
Conclusion: Addiction-specific Instagram-themed cues identify the neurobiological underpinnings of Instagram addiction. Activations of the dopaminergic reward system and deactivation of the executive control network indicate converging neuropathological pathways between Instagram addiction and other types of addictions.
METHODS: We derived the data from the TECMA study, which used a cross-sectional study design and multi-stage sampling method to obtain a representative sample of school-going adolescents aged 11-19 years in Malaysia in 2016. Data were collected through a self-administered approach using a pre-validated standard questionnaire. Descriptive and multivariate analyses were used to analyze the data, and results are presented as adjusted odds ratio (AOR) with 95% confidence interval (95% CI).
RESULTS: SHS exposure for the past seven days was higher outside the home (51.2%; 95% CI: 49.2-53.2) compared to at home (37.8%; 95% CI: 35.8-39.9) while 27.3% (95% CI: 25.1-29.5) of school-going adolescents reported exposure to SHS inside the school in the past one month. In the regression analyses, older adolescents, those of Malay and Bumiputra Sarawak ethnicities, adolescents from rural areas and current smokers had higher likelihood of exposure to SHS at home, outside home and inside the school. Our study also found that adolescents who were current smokers had higher odds of being exposed to SHS at home (AOR=2.87; 95% CI: 2.57-3.21), outside the home (AOR=3.46; 95% CI: 3.05-3.92) and in the school (AOR=2.25; 95% CI: 2.01-2.51).
CONCLUSIONS: Health promotion measures should target parents/guardians and household members to reduce SHS exposure among adolescents. In addition, smoke-free regulation should be fully enforced in school. Furthermore, more public places should be designated non-smoking areas to reduce SHS exposure and denormalize smoking behavior.
Material and Methods: Patients with ACL re-injury to either knee after successful primary ACLR were included in Group I and those with no further re-injury were included in Group II. Variables including age, gender, side, body mass index (BMI), thigh atrophy, anterior knee laxity difference between both knees measured by KT-1000 arthrometer, mean time of return to sports (RTS), graft type, type of game, mode of injury, Tegner Activity Score, hormone levels, femoral tunnel length (FTL), posterior tibial slope (PTS) and notch width index (NWI) were studied. Binary logistic regression was used to measure the relative association.
Results: A total of 128 athletes were included with 64 in each group. Mean age in Group I and II were 24.90 and 26.47 years respectively. Mean follow-up of Group I and Group II were 24.5 and 20.11 months respectively. Significant correlation was present between ACL re-injury and following risk factors; PTS of >10º, KT difference of >3.0mm, thigh atrophy of >2.50cm and time to RTS <9.50 months P value <0.05). No correlation was found with age, sex, BMI, type of game, Tegner Activity Score, mode of injury, NWI, size of graft, FTL and hormone levels.
Conclusion: Possible risk factors include PTS of ≥ 10º, KT difference of ≥ 3.0mm at 1 year follow-up, thigh atrophy of ≥ 2.50cm at 1 year follow-up and RTS <9.5 months after primary ACLR.