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.

The Validity and Reliability of Malay Version Child Exposure to Domestic Violence Scale.

Background: Domestic violence has a significant impact on growing children. However, existing evidence is limited and often under reported. Consequently, the Child Exposure to Domestic Violence (CEDV) scale has been developed for global use. This study aims to provide a cross-cultural translation, adaptation and validation of the CEDV based on Malay language. Methods: CEDV scale was translated from the original English version to Malay. Content and face validity were examined before field-testing. The study comprised a cross sectional study using the Malay version of the CEDV (CEDV-M) scale conducted at a secondary school in Perlis, Malaysia and investigated 235 children aged 13 years old-16 years old using an online platform. The construct validity, internal consistency and stability of the tool were assessed. Results: The CEDV-M scale's content and face validity both yielded a value exceeding 0.80. Furthermore, the tool demonstrated has good stability reliability, using the intra-class correlation value for all items ranging from 0.659 to 1.00. The exploratory factor analysis of the 36 items of the CEDV scale revealed possibilities of five or six factor domains. However, the five factor domains were most conceptually equivalent. Consequently, this study found that the CEDV-M scale is reliable with a total Cronbach's alpha of 0.87. Conclusion: CEDV-M scale is a valid and reliable tool for measuring a child's experience of domestic violence. Future studies recommend confirmatory factor analysis and standard settings for scoring systems.

Purine Nucleoside Phosphorylase Deficient Severe Combined Immunodeficiencies: A Case Report and Systematic Review (1975-2022).

Purine nucleoside phosphorylase deficient severe combined immunodeficiency (PNP SCID) is one of the rare autosomal recessive primary immunodeficiency disease, and the data on epidemiology and outcome are limited. We report the successful management of a child with PNP SCID and present a systematic literature review of published case reports, case series, and cohort studies on PNP SCID listed in PubMed, Web of Science, and Scopus from 1975 until March 2022. Forty-one articles were included from the 2432 articles retrieved and included 100 PNP SCID patients worldwide. Most patients presented with recurrent infections, hypogammaglobulinaemia, autoimmune manifestations, and neurological deficits. There were six reported cases of associated malignancies, mainly lymphomas. Twenty-two patients had undergone allogeneic hematopoietic stem cell transplantation with full donor chimerism seen mainly in those receiving matched sibling donors and/or conditioning chemotherapy before the transplant. This research provides a contemporary, comprehensive overview on clinical manifestations, epidemiology, genotype mutations, and transplant outcome of PNP SCID. These data highlight the importance of screening for PNP SCID in cases presented with recurrent infections, hypogammaglobulinaemia, and neurological deficits.

Health-Related Quality of Life of Patients and Families with Primary Immunodeficiency in Malaysia: a Cross-Sectional Study.

PURPOSE: Primary immunodeficiency disease (PID) affects various aspects of a patient's life. However, the health-related quality of life (HRQOL) of PID among Malaysian patients is poorly described. This study aimed to determine the quality of life of PID patients and their respective parents. METHOD: This cross-sectional study was performed from August 2020 to November 2020. Patients with PID and their families were invited to answer the PedsQL Malay version (4.0) questionnaire, the tool used to assess the HRQOL. A total of 41 families and 33 patients with PID answered the questionnaire. A comparison was performed with the previously published value of healthy Malaysian children. RESULT: Parents of respondents recorded a lower mean of total score than the parents of healthy children (67.26 ± 16.73 vs. 79.51 ± 11.90, p-value = 0.001, respectively). PID patients reported lower mean total score to healthy children (73.68 ± 16.38 vs. 79.51 ± 11.90, p-value = 0.04), including the psychosocial domain (71.67 ± 16.82 vs. 77.58 ± 12.63, p-value = 0.05) and school functioning (63.94 ± 20.87 vs. 80.00 ± 14.40, p-value = 0.007). No significant difference of reported HRQOL when comparing between subgroup of PID on immunoglobulin replacement therapy and those without immunoglobulin replacement (56.96 ± 23.58 vs. 65.83 ± 23.82, p-value 0.28). Socioeconomic status was found to be predictive of the lower total score of PedsQL in both parent and children reports. CONCLUSION: Parents and children with PID, especially those from middle socioeconomic status, have lower HRQOL and school function impairment than healthy children.

Adverse Childhood Experiences and Health Risk Behaviours among the Undergraduate Health Campus Students.

Background: Adverse childhood experiences (ACEs) are linked with health risk behaviours (HRBs). The study aimed to evaluate ACEs in the undergraduate health campus of a public university located in the northeast of Malaysia and determine their association with HRBs. Methods: A cross-sectional study was performed by recruiting 973 undergraduate students at the health campus of a public university from December 2019 to June 2021. The World Health Organization (WHO) ACE-International Questionnaire and the Youth Risk Behaviour Surveillance System questionnaire were distributed using simple random sampling according to year of study and the selected batch of students. Descriptive statistics were used for demographic findings and logistic regression analyses were performed to determine the association between ACE and HRB. Results: The 973 participants (male [n = 245] and female [n = 728]) had a median age of 22 years old. The prevalence of child maltreatment among the study population was 30.2%, 29.2%, 28.7%, 9.1% and 6.1% for emotional abuse, emotional neglect, physical abuse, physical neglect and sexual abuse, respectively, among both sexes. The most commonly reported household dysfunctions were parental divorce/separation (5.5%). Community violence was 39.3% among the surveyed participants. The highest prevalence of HRBs among respondents was 54.5% from physical inactivity. The findings confirmed that those exposed to ACEs were at risk of HRBs and that a higher number of ACEs was associated with a higher number of HRBs. Conclusion: ACEs were highly prevalent among participating university students, ranging from 2.6%-39.3%. Hence, child maltreatment is an important public health problem in Malaysia.

Pericentric Inversion of Chromosome 9 in Twins With Cyclopia: A Rare Entity.

Pericentric inversion of chromosome 9 (inv(9)) is one of the most common variants seen in a normal human karyotype that occurs during meiosis. Despite being categorized as a normal variant, some studies using classical cytogenetics have recently shown that inv(9) could be associated with azoospermia, congenital anomalies, growth retardation, and rarely with abnormal karyotype. However, there is no reported association with cyclopia. Interestingly this genetic variant involves twin fetuses. A 36-year-old multiparous lady with dichorionic diamniotic twin pregnancy presented to the fetomaternal unit with fetal growth restriction at 34 weeks of gestation. An ultrasound scan revealed both have microcephaly, fisting hands, holoprosencephaly, and proboscis suspicious of Patau syndrome. Amniocentesis was not issued due to late pregnancy and guarded prognosis. The mother presented with pre-eclampsia at 35 weeks of gestation. The pregnancy managed to prolong up to 36 weeks after which caesarean section was performed due to the leading twin being in a transverse lie. Two baby twin girls were born 3 minutes apart with microcephaly and cyclops appearance. Chromosomal analysis of both twins revealed similar karyotypes of 46, XX, inv(9)(p11,q13). Pericentric inversion of chromosome 9 is regarded as a normal chromosomal variation in the general population, but in twins with cyclops is considered rare. Early referral to a tertiary hospital for twin management is highly required. It may identify fetuses with such abnormalities and counsel the parents with appropriate management.

Psychological distress and coping strategies among the caretakers of children with transfusion-dependent thalassemia.

Introduction: Thalassemia is a chronic childhood disease that could result in psychological distress not only to the patients but also to their caretakers. Caretakers utilize different coping strategies to reduce stress and maintain a good quality of life. Objective: The study aims to measure the level of psychological distress among caretakers of transfusion-dependent thalassemia patients and identify coping strategies used by them, as well as examine factors related to both outcome measures. Methods: Sixty-eight (N = 68) caretakers of children with transfusion-dependent thalassemia agreed to participate in the study when they were approached during their visits to one of three major hospitals in Kelantan, Malaysia, for the children's medical treatment. They completed the Malay validated Depression Anxiety and Stress Scale 21 (DASS 21) and Brief-COPE self-report, in addition to a brief study proforma. Results: The majority of the participants reported feeling psychologically well, with no related scores in depression, anxiety, and stress sub-scales. The mean score for anxiety and stress sub-scales were 3.54 (SD = 3.54) and 4.25 (SD = 3.26) respectively. The median score for the depression sub-scale was 2.00 (IQR 4.00). The three mostly utilized coping strategies were religion, acceptance, and positive reframing. Those with depressed and anxious moods were found to engage more in negative coping strategies including substance abuse, denial, and behavioral disengagement. Being female, of younger age, employed, with higher educational level, and income status was found to significantly influence the adoption of positive reframing as a coping strategy. Conclusion: Psychological distress such as elevated anxiety and depression was found among a small portion of caretakers who have children with thalassemia whose treatment required blood transfusion. They were noted to apply more maladaptive coping strategies compared to their psychologically well counterparts.

A mixed method study on the impact of living with spinal muscular atrophy in Malaysia from patients' and caregivers' perspectives.

BACKGROUND: Spinal Muscular Atrophy (SMA) is a rare, recessively inherited neuromuscular disorder that causes progressive muscle weakness. There is a low degree of awareness about SMA amongst the public and healthcare providers, which may impact the perception of the disease and its proper management. To understand how this lack of awareness may have affected diagnosis, care and support for SMA patients and their caregivers, this study aims to investigate the impact of SMA on the lives and daily activities of SMA patients and their caregivers in Malaysia. METHODS: Nationwide recruitment was carried out via invitations coordinated by a local SMA advocacy organization. A mixed method cross-sectional study consisting of a self-administered questionnaire followed by in-depth interviews (IDIs) and focus group discussions (FGDs) was conducted. The interview sessions were audio-taped, and verbatim transcripts analyzed thematically. RESULTS: Participants reported feeling stressed, anxious and depressed. There were issues with delayed diagnosis, lack of information from healthcare professionals about the disease progression, and limited access to supportive services like physiotherapy. Participants expressed their concerns living with self-doubt and turmoil with having to modify their lifestyles, relationships with family and friends, and challenges with educational and career opportunities. Various themes of their hopes for the future touched on having access to treatment, clinical trials, holistic care for symptom management, as well as improving infrastructure for disability access. CONCLUSION: This study, to the best of our knowledge represents the first comprehensive study on SMA in South East Asia, highlights a plethora of issues and challenges experienced by persons with spinal muscular atrophy (PWSMA) and their caregivers in Malaysia, from the point of SMA diagnosis and throughout the management of care, in addition to the deep psychosocial impact of living with SMA. The significant findings of this study may contribute to a better understanding among stakeholders to make improvements in clinical practice, the education system, the work environment as well as holistic care support and society at large.

Impact of Primary Immunodeficiency Diseases on the Life Experiences of Patients in Malaysia From the Caregivers' Perspective: A Qualitative Study.

Introduction: Primary immunodeficiency diseases (PIDs) are chronic diseases that affect the various aspects of a patient's life. However, the impact of living with PIDs is poorly described. Objective: This study aimed to explore the living experience challenges among the Malaysian caregivers of the patients with PID who underwent a follow-up in the Universiti Sains Malaysia or those registered members of the Malaysian PIDs Society. Methodology: The study was conducted from March 1 to May 30, 2021. The parents of children with PIDs were invited to participate for a semi-structured in-depth interview at the PID clinics in the USM. The estimated time of each interview was 30 min. The semi-structured interview was performed via a telephone call because of COVID-19 pandemic restrictions. The audio recording of each interview was transcribed and translated from Malay to English. Subsequently, a thematic analysis utilizing the ATLAS.ti software was performed. Results: The thematic analysis revealed five main themes, which are living with fear and anxiety with four sub-themes (sickness, psychological issues, fear of infections and hereditary issues), PID healthcare support struggles with four sub-themes (PID health system, treatment, diagnosis and financial issues), knowledge with two sub-themes (educational issues and disease understanding), social constraint with two sub-themes (relationships and social isolations) and coping with three sub-themes (acceptance, child health improvement and emotional hygiene). Conclusion: Living with fear and anxiety is a major theme impacting the living experiences of Malaysian patients with PIDs. Improvements in healthcare delivery and disease education are needed to ensure optimal quality of life.

Probiotic Bifidobacterium lactis Probio-M8 treated and prevented acute RTI, reduced antibiotic use and hospital stay in hospitalized young children: a randomized, double-blind, placebo-controlled study.

PURPOSE: The development of probiotics has seen tremendous growth over the years, with health benefits ranging from gut health to respiratory. We thus aimed to investigate the effects of probiotic Bifidobacterium lactis Probio-M8 (2 × 1010 log CFU/day) against acute respiratory tract infections (RTI), use of antibiotics, hospitalization period and elucidate the possible mechanisms of action in hospitalized young children. METHOD: A prospective, randomized, double-blind and placebo-controlled study was performed in RTI-hospitalized children. Patients were randomized to either the probiotic (n = 60, mean age 13.81 ± 0.90 months) or placebo (n = 60, mean age 12.11 ± 0.73 months) which were administered upon admission, continued during hospitalization and 4-week post-discharged. RTI and gut health parameters were assessed at these time points using validated questionnaires while concentrations of inflammatory cytokines were assessed via oral swabs. RESULTS: Probio-M8 reduced the duration of nasal, pharyngeal and general flu-like symptoms compared to the placebo during the hospitalization period and 4-week post-discharged (P < 0.05) as compared to the placebo, with a more prevalent effect against lower respiratory tract infections (LRTI). Probio-M8 reduced prescription of antibiotic (P = 0.037), prevented new prescription of antibiotic in non-prescribed patients (P = 0.024) and reduced hospitalization period in antibiotic-prescribed patients (P = 0.004) as compared to the placebo. Oral cytokine levels of TNF-α decreased in the Probio-M8 group (P = 0.001) accompanied by increased in IL-10 (P = 0.018) over 4-week post-discharged, while the placebo group did not exhibit such an effect. Increased IL-10 in the Probio-M8 group was correlated with decreased body ache (r = - 0.296, P = 0.001), headache (r = - 0.295, P = 0.001) and pain during swallow (r = - 0.235, P = 0.010). CONCLUSION: Data from our present study show that B. lactis Probio-M8 could be a potential natural and non-drug strategy for the management of RTI in young children in a safe manner. CLINICAL TRIAL REGISTRATION: Clinical studies (Approval No. USM/JEPeM/19030177) were registered at ClinicalTrials.gov (Identifier No. NCT04122495) on September 30, 2019.

The Challenges, Coping Mechanisms, and the Needs of the Inhospital Parents Caring for Children with Life-limiting Neurological Disorders: A Qualitative Study.

OBJECTIVES: Parents who have children with complex lifelong and life-limiting neurological conditions experienced many stresses and anxieties throughout their lives as caregivers. However, this information is still very limited. The study aimed to explore the challenges faced by parents with children who have complex neurological conditions, their coping strategies, needs, and expectations. MATERIALS AND METHODS: A semi-structured, in-depth interviews were conducted in the either Malay or English language among the parents of children with complex lifelong neurological conditions and have been on long-term in-patient hospital care. The interviews were audio-recorded and transcribed for thematic analysis. The qualitative study was conducted from October to November 2016 at the Paediatric Institute of Hospital Kuala Lumpur. Grounded theory was used to examine the qualitative data with inductive and deductive types of coding. The transcripts were read repeatedly to allow familiarity to the themes presented by the participants. Further discussions were conducted among the researchers to triangulate the information. RESULTS: A total of 11 parents were interviewed for this study. The thematic analysis resulted in 8 challenges: Physical wellbeing, Environment, Relationship, Financial, Occupational, Rational, Mental, and Spiritual. Coping strategies comprised problem focused issues related to the key challenges in the caregivers' context. Similar to the needs and expectations, the key themes were derived from the key understandings of the challenges and looking at the palliative care impacts for these children. CONCLUSION: There are various challenges faced by parents of children with life-limiting neurological disorders. Physical, Environment, Relationship, Financial, Occupational, Rational, Mental, and Spiritual Wellbeing can be a platform for the assessment of the caregivers' needs and the planning for palliative care support.

A Malay Version of the International Society for the Prevention of Child Abuse and Neglect Screening Tool for Children: A Study of Its Validity and Reliability.

BACKGROUND: Childhood maltreatment is a global problem, for which the International Society for the Prevention of Child Abuse and Neglect (ISPCAN) has developed the Child Abuse Screening Tool-Child, Home Version (ICAST-CH) to obtain data concerning childhood maltreatment. The study aimed to translate the English version of the ICAST-CH into the Malay language and to assess its reliability and validity. METHODS: The original English version of the ICAST-CH was first translated into the Malay language. Its content and face validity were tested among five independent individuals. A cross-sectional study using the Malay version (ICAST-CH-M) was then conducted with 255 students in a secondary school in Kota Bharu, Kelantan, Malaysia. The data collected was used to examine the instrument's internal consistency and construct validity. The best ICAST-CH-M model was achieved after varimax rotation application. RESULTS: The analysis showed that the Malay version of the ICAST-CH had satisfactory internal consistency, with Cronbach's alpha ranging from 0.59-0.77. The exploratory factor analysis confirmed the validity of the underlying constructs into five domains in the Malay version, but they had to be re-classified as 'physical and psychological abuse', 'neglect', 'sexual abuse', 'exposure to domestic violence' and 'exposure to community violence'. CONCLUSION: This study demonstrated that the ICAST-CH-M is satisfactorily reliable and valid for measuring child maltreatment in Malaysia.

Comparison of antimicrobial resistance in neonatal and adult intensive care units in a tertiary teaching hospital.

Intrahospital variations in antimicrobial profiles may be related to many factors. This study compared causative agents of nosocomial bloodstream infections between a neonatal intensive care unit (NICU) that adopted a ward-tailored antibiotic policy and adult intensive care units (ICUs). Data on organisms from blood cultures obtained from the respective wards between 2005 and 2009 were analyzed. Compared with the adult ICUs, the NICU had a higher frequency of Enterobacteriacae and lower frequencies of typical hospital-acquired pathogens (eg, Klebsiella pneumoniae, 17.4% vs 10.0% [P < .001]; Acinetobacter baumannii, 3.9% vs 11.6% [P < .001]). Antibiotic resistance of gram-negative organisms was also significantly lower in the NICU, including resistance to imipenem (5.7% vs 32.1%; P < .001), amikacin (8.8% vs 30.3%), and ceftriaxone (36.1% vs 74.6%; P < .001). This could possibly be due to the ward-tailored antibiotic policy adopted by the NICU but not by the other ICUs.

Infantile fibrosarcoma of the penis in a 2-year-old boy.

Fibrosarcoma is rare in the pediatric age group. It generally involves the extremities and the trunk but rarely involves the genital area. We report a case of a large fungating infantile fibrosarcoma of the penis in a 2-year-old Malay boy. Partial recovery of the penile structure was achieved after chemotherapy. The difficulty in managing the social and surgical aspect of this case is discussed in our report. To the best of our knowledge, this is the first case report of infantile fibrosarcoma involving the penis in an Asian region.