METHOD: Retrospective study of children with ANE seen at University of Malaya Medical Centre from 2014 to 2019. All clinical details including ANE-severity score (ANE-SS), immunomodulation treatment and neurodevelopmental long-term outcome were collected.
RESULTS: Thirteen patients had ANE and brainstem death occurred in 5. In 10 patients (77%) viruses were isolated contributing to ANE: 8 influenza virus, 1 acute dengue infection, and 1 acute varicella zoster infection. The ANE-SS ranged 2-7: 9 were high risk and 4 were medium risk. Among the 8 survivors; 1 was lost to follow-up. Follow-up duration was 1-6 years (median 2.2). At follow-up among the 4 high-risk ANE-SS: 2 who were in a vegetative state, 1 remained unchanged and 1 improved to severe disability; the other 2 with severe disability improved to moderate and mild disability respectively. At follow-up all 3 medium-risk ANE-SS improved: 2 with severe disability improved to moderate and mild disability respectively, while 1 in a vegetative state improved to severe disability. Early treatment with immunomodulation did not affect outcome.
CONCLUSION: Our ANE series reiterates that ANE is a serious cause of encephalopathy with mortality of 38.5%. All survivors were in a vegetative state or had severe disability at discharge. Most of the survivors made a degree of recovery but good recovery was seen in 2. Follow-up of at least 12 months is recommended for accurate prognostication. Dengue virus infection needs to be considered in dengue endemic areas.
METHODS: We randomly selected 500 Nepali mother-infant pairs and measured maternal intake and infant and maternal vitamin B12 status using plasma cobalamin, total plasma homocysteine, and methylmalonic acid concentrations. We revisited available children when they were 5 years old and measured growth. The associations between intake and maternal and infant markers of vitamin B12 and growth were estimated in multiple linear regression models adjusting for relevant confounders (n = 331).
RESULTS: Maternal vitamin B12 intake and status and vitamin B12 status in infancy predicted linear growth at 5 years of age, but not during infancy. Each microgram increase in the vitamin B12 intake of the mother during infancy was associated with an increase in height of 0.4 (0.2, 0.6) height-for-age z-scores and 1.7 (0.7, 2.7) cm around the child's fifth birthday.
CONCLUSION: Vitamin B12 status and intake in early life is an important determinant for linear growth at school age. Our findings should be verified in randomized, placebo controlled trials before translated into public health recommendations.
METHOD: This study is a retrospective review of 116 pediatric patients presenting with sore throat. Group A comprised patients before the implementation of the McIsaac rule and Group B comprised patients after the implementation.
RESULTS: Unnecessary throat swab cultures were reduced by 40% (P = 0.003). Redundant antibiotic prescriptions were reduced by 26.5% (P = 0.003) and the overall use of antibiotics was reduced by 22.1% (P = 0.003). The pediatricians' compliance rate to McIsaac rule criteria was 45% before implementation of the McIsaac rule, but improved to 67.9% (P = 0.0005) after implementation.
DISCUSSION: The McIsaac rule is an effective tool for the management of sore throat in children in Malaysia.
METHODS: One hundred two study participants were identified through a network of collaborating clinicians and the Amy and Friends CS support groups. Families with a diagnosis of CS could also self-recruit. Comprehensive clinical information for analysis was obtained directly from families and their clinicians.
RESULTS AND CONCLUSION: We present the most complete evaluation of Cockayne syndrome to date, including detailed information on the prevalence and onset of clinical features, achievement of neurodevelopmental milestones, and patient management. We confirm that the most valuable prognostic factor in CS is the presence of early cataracts. Using this evidence, we have created simple guidelines for the care of individuals with CS. We aim to assist clinicians in the recognition, diagnosis, and management of this condition and to enable families to understand what problems they may encounter as CS progresses.Genet Med 18 5, 483-493.
METHODS: This was a data review involving children aged
METHODS: One hundred osteosarcoma cases from 2003 to 2018 in Hospital Universiti Sains Malaysia were retrieved. The tissue samples were stained for RANKL, and the association with the clinicopathological characteristics was evaluated. Staining was interpreted in a semiquantitative scoring system and classified into negative and positive expressions. Results: Eighty-two cases had a positive expression of RANKL in which 56 and 26 patients were classified as low expression and high expression, respectively. The positive expression of RANKL did not show a significant association with clinicopathological characteristics. However, Kaplan Meier survival analysis showed a significant improvement in the disease-free survival patients who underwent limb salvage surgery (LSS) than amputated patients (p-value 0.002), whereas poorer survival was observed among conventional osteosarcomas compared to non-conventional osteosarcoma (p-value 0.01).
CONCLUSION: Limb salvage surgery had proven to improve osteosarcoma patients' survival compared to amputation, which could improve overall quality of life in osteosarcoma patients. However, our data did not show a significant association between positive RANKL immunohistochemistry with any of the clinicopathological characteristics and patients' final survival. Further studies may be acquired to understand the suitability of using RANKL immunohistochemistry as prognostication in the management of osteosarcoma patients.
METHODS: A cross sectional study on nationally representative sample deaths that occurred in Malaysia during 2013 was used. A VA questionnaire suitable for local use was developed. Trained field interviewers visited the family members of the deceased at their homes and conducted face to face interviews with the next of kin. Completed questionnaires were reviewed by trained physicians who assigned multiple and underlying causes. Reference diagnoses for validation were obtained from review of medical records (MR) available for a sample of the overall study deaths.
RESULTS: Corresponding MR diagnosis with matched sample of the VA diagnosis were available in 2172 cases for the validation study. Sensitivity scores were good (>75%) for transport accidents and certain cancers. Moderate sensitivity (50% - 75%) was obtained for ischaemic heart disease (64%) and cerebrovascular disease (72%). The validation sample for deaths due to major causes such as ischaemic heart disease, pneumonia, breast cancer and transport accidents show low cause-specific mortality fraction (CSMF) changes. The scores obtained for the top 10 leading site-specific cancers ranged from average to good.
CONCLUSION: We can conclude that VA is suitable for implementation for deaths outside the health facilities in Malaysia. This would reduce ill-defined mortality causes in vital registration data, and yield more accurate national mortality statistics.
DESIGN: Prospective observational study.
SETTING: PICU in a tertiary care pediatric hospital.
PATIENTS: All English-literate parents whose child was admitted to our PICU between February 2014 and February 2015 were eligible after informed consent was obtained. Parents included in this study in previous admission(s) were excluded.
INTERVENTION: Nil.
MEASUREMENTS AND MAIN RESULTS: We adapted Empowerment of Parent in the Intensive Care Questionnaire, a validated questionnaire survey specific for measuring parental satisfaction in PICUs. This adapted survey consisted of 31 questions (based on a scale of 1-6) examining five domains as follows: information giving, care and cure, parental participation, organization, and professional attitude. Reliability of Empowerment of Parent in the Intensive Care Questionnaire in our population was analyzed using Cronbach's alpha. We used ordinal logistic regression, controlling for socioeconomic status and educational level, to examine differences in parental perceptions of various ethnicities. We obtained a total of 206 responses (36.5%) from 543 admissions. There were 116 (56%) emergency and 90 (44%) elective admissions. The proportion of respondents were Chinese (126 [61%]), Malay (32 [16%]), Indian (23 [11%]), and "Others" (25 [12%]). Cronbach's alpha for domains of information giving (α = 0.80), care and cure (α = 0.93), parental participation (α = 0.84), organization (α = 0.79), and professional attitude (α = 0.88) were good. In all five domains, our median PICU scores were 6 (interquartile range, 5-6). Compared to other ethnic groups, Malay parents did perceive that domains of "care and cure," "parental participation," and "professional attitude" were less satisfactory.
CONCLUSIONS: Significant differences were found in satisfaction ratings between parents of different ethnicities. Further studies are needed to explore and determine reasons for these differences.