Knowledge and practice of childhood immunisation among parents in Kelantan, Malaysia: A cross-sectional study.

Introduction: Parents are key decision-makers in the immunisation practice and compliance of children. This study aimed to determine the knowledge and practice of immunisation among parents in Kelantan, Malaysia, and their associated factors. Methods: A cross-sectional study was conducted using a validated online questionnaire from May to June 2021. An invitation was distributed to parents attending a university hospital and extended families of staff through online platforms. A total of 311 parents participated in the study. The questionnaire consisted of 10 questions each on knowledge and practice and three questions on vaccination status. Descriptive analysis was performed. The associations between the sociodemographic characteristics and knowledge and practice scores were determined using the chi-square test, and predictive factors were identified using logistic regression analysis. Results: Most respondents were Malay (94.2%), Muslim (94.5%), women (79.7%) and married (96.1%). The median score for immunisation knowledge and practice was 8 (interquartile range [IQR]=2) and 7 (IQR=3), respectively. Multiple logistic regression revealed that parents who were unmarried or single, less educated, and had lower incomes were predicted to have poor knowledge of childhood vaccination (P<0.05). Conversely, those living outside Kota Bharu, less educated, and younger parents were predicted to have poor vaccination practice of childhood vaccination (P<0.05). Most respondents (97.8%) indicated completing their children's vaccination schedule. Conclusion: Parental education and household income are associated with immunisation knowledge and practice. Improving access to information about childhood vaccination among targeted groups may further boost immunization coverage.

Mutations in the tail domain of MYH3 contributes to atrial septal defect.

Atrial septal defect (ASD) is one of the most common congenital heart defects diagnosed in children. Sarcomeric genes has been attributed to ASD and knockdown of MYH3 functionally homologues gene in chick models indicated abnormal atrial septal development. Here, we report for the first time, a case-control study investigating the role of MYH3 among non-syndromic ASD patients in contributing to septal development. Four amplicons which will amplifies the 40 kb MYH3 were designed and amplified using long range-PCR. The amplicons were then sequenced using indexed paired-end libraries on the MiSeq platform. The STREGA guidelines were applied for planning and reporting. The non-synonymous c. 3574G>A (p.Ala1192Thr) [p = 0.001, OR = 2.30 (1.36-3.87)] located within the tail domain indicated a highly conserved protein region. The mutant model of c. 3574G>A (p.Ala1192Thr) showed high root mean square deviation (RMSD) values compared to the wild model. To our knowledge, this is the first study to provide compelling evidence on the pathogenesis of MYH3 variants towards ASD hence, suggesting the crucial role of non-synonymous variants in the tail domain of MYH3 towards atrial septal development. It is hoped that this gene can be used as panel for diagnosis of ASD in future.

Humanitarian Mission in Pediatric Cardiothoracic Surgery: A Recipient's Perspective.

Introduction: Pediatric cardiac surgical mission programs are deemed as common practice, especially in developing nations funded by international non-governmental organizations (NGOs). This article presents and discusses the results and strategies implemented by this partnership, aiming at achieving the autonomy of the local center by this collaboration. Materials and Methods: A retrospective review was conducted on patients with congenital heart disease who underwent surgical intervention from the beginning of the NGO collaboration (September 2015) until November 2018 in an existing cardiac center. In between those visits, any congenital heart disease patient with Risk Adjustment Congenital Heart Surgery (RACHS)-1 Category 1-3 would be discussed in a local multi-disciplinary meeting with regards to the feasibility of the surgery being performed by the local members. Results: A total of 60 operations were performed during the trips. Throughout the visit, 46% (28) of the operations were performed by the local surgeon, with or without assistance from the visiting surgeon. Between September 2015 and November 2018, 27 cases were also performed by the local team independently. For the 27 cases performed by the local team independently, the median age of the patient was 42 days (ranging from 14 days to 20 years old), with median body weight of 3.2 kg (ranging from 2.8 to 64 kg). Conclusion: Humanitarian pediatric cardiac surgical missions are safe to be done for the population in need. In order to achieve autonomy, continuous efforts by both teams are crucial, as the cooperation by the two parties ensures that the objectives are achieved.

Central Retinal Artery Occlusion with Sparing of Cilioretinal Artery Post Pulmonary Artery Stenting.

Central retinal arterial occlusion is an ocular emergency. Central retinal artery occlusion following cardiac procedures have been described in adults. We describe a pediatric patient who developed central retinal artery occlusion following pulmonary artery stenting. It is important to highlight this potential risk to ensure early diagnosis and prompt treatment.

Beating the odds: a rare case of supracardiac total anomalous pulmonary venous return (TAPVR) in an adult patient.

Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage. Cardiac CT confirmed supracardiac TAPVR, whereby all the pulmonary veins drain into the anomalous vein and finally to the superior vena cava. She remained asymptomatic and underwent a successful surgical repair.

Screening of SMAD7 in Malay patients with ventricular septal defect.

Ventricular septal defect (VSD) is the most common form of cardiac malformations accounting approximately 20% of all congenital heart defects. SMAD7 is an inhibitory protein that antagonizes the signalling of TGF-ß family member and has been found in the development and function of mouse heart models. This study aims to screen and identify the polymorphisms of SMAD7 exonic regions in Malay population with VSD. Peripheral blood samples were collected and extracted from 30 clinically diagnosed VSD patients. PCR amplification was performed using 12 sets of designed primers encompassing seven exons of SMAD7. Re-sequencing was conducted to characterize the polymorphisms of SMAD7. Observed polymorphisms were then genotyped in 30 healthy individuals using both re-sequencing and allele-specific PCR techniques. A total of 10 variants were identified in the patient population located in the upstream (rs7236774), exonic (rs368427729, rs145686330, rs3764482, rs3809922, rs780863704 and rs3809923), intronic (rs3736242) and 3'UTR regions (rs375444823 and rs16950113). No significant difference of genotype and allele frequency was observed among these SNPs. Two synonymous variants (rs3809922 and rs3809923) were found in complete linkage disequilibrium (r2=1.0) with each other indicate a strong correlation of these SNPs. The identification of these SNPs provides a new perspective of the VSD causation.