Displaying publications 1 - 20 of 6616 in total

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  1. A Hamid MS, Sazlina SG
    PLoS One, 2019;14(1):e0209746.
    PMID: 30625165 DOI: 10.1371/journal.pone.0209746
    BACKGROUND: Childhood overweight and obesity has emerged as a major public health threat worldwide with challenges in its management. This review assessed the effectiveness of interventions for childhood overweight and obesity.

    METHODS: A systematic literature search was conducted using CINAHL, EMBASE, Ovid MEDLINE, PsycINFO and SPORTDiscus databases to retrieve articles published from 1st January 2000 to 31st December 2017. Randomised controlled trials (RCTs) and quasi-experimental studies comparing different strategies in managing overweight and obesity among schoolchildren (6 to 12 years of age) were included. The main outcomes of interest were reductions in weight related variables included anthropometry and body composition measurements. All variables were analysed using random effects meta-analyses.

    RESULTS: Fourteen studies were reviewed, 13 were RCTs and one was a quasi-experimental study. The risk of bias for randomisation was low risk for all of RCTs except for one, which was unclear. The risk of bias for randomisation was high for the quasi-experimental study. Most interventions incorporated lifestyle changes and behavioural strategies such as coping and problem solving skills with family involvement. The meta-analyses did not show significant effects of the intervention in reducing weight related outcomes when compared with controls.

    CONCLUSION: Meta-analyses of the selected studies did not show significant effects of the interventions on weight related outcomes among overweight and obese schoolchildren when compared with controls. The role of interdisciplinary team approaches with family involvement using behaviour and lifestyle strategies to curb obesity among schoolchildren is important.

    Matched MeSH terms: Child
  2. A Rahim NN, Chin YS, Sulaiman N
    Nutrients, 2019 Jan 11;11(1).
    PMID: 30641864 DOI: 10.3390/nu11010142
    Considering the double burden of malnutrition in Malaysia, data on malnourished children living in welfare homes are limited. This study aimed to determine the body weight status of children living in welfare homes and its associated factors. A total of 307 children aged 7⁻17 years old living in 15 selected welfare homes completed a standardized questionnaire, and their body weight and height were measured by trained researchers. There were 54.4% orphans, 23.8% abandoned children, and 21.8% children from problematic families. There were 51.5% boys and 48.5% girls; 52.4% were Malays, followed by 31.3% Indians, 12.7% Chinese, and 3.6% from other ethnic groups. The prevalence of overweight and obesity (23.1%) was higher than the prevalence of thinness (8.5%). In bivariate analyses, socio-demographic factors of age (p = 0.003), sex (p = 0.0001), ethnicity (p = 0.001), and welfare home enrollment status (p = 0.003), and psychological factors of self-esteem (p = 0.003), body shape dissatisfaction (p = 0.0001), and underestimation of body weight status (p = 0.002), were significantly associated with body mass index (BMI)-for-age. In the multiple linear regression analysis, children who were either Malays (β = 0.492) or Chinese (β = 0.678), with a status of being abandoned (β = 0.409), with body shape dissatisfaction (β = 0.457), and underestimated body weight status (β = 0.628) significantly explained 39.7% of the variances in higher BMI-for-age (F = 39.550; p < 0.05). Besides socio-demographic background, the current findings emphasized the importance of incorporating body image perception in an obesity prevention intervention program in welfare homes.
    Matched MeSH terms: Child
  3. AL-Kubaisy W, AL-Talib H, Al-khateeb A, Shanshal MM
    Trop Biomed, 2014 Sep;31(3):499-506.
    PMID: 25382477
    Parasitic diarrhea among children is a significant health problem worldwide. This cross sectional study described the burden of parasitic diarrhea among children. The objectives of this study were to evaluate the impact of risk factors on the parasitic diarrhea, and to determine the parasitic profile among children in Baghdad-Iraq, during the period extending from September 2003 to June 2004. A total number of 2033 cases were included in the study. The estimated prevalence rate of parasitic diarrhea was 22%. We identified the following major diarrhea determinants were large households size, residential location, water source, low socioeconomic status, and low parent education. Giardia lamblia was found to be the most prevalent parasite with an infection rate of 45.54% followed by Entamoeba histolytica 23.44%, Enterobius vermicularis 12.7%, Hymenolepis nana 9.82%, Trichuris trichiura 5.4%, and Ascaris lumbricoides 2.2%. In conclusion, this study demonstrates that poor sanitation, inadequate environmental conditions, and low socioeconomic status are the main determining factors that predispose children to parasitic diarrhea. Mass deworming programs are recommended for school children, as this population is easily accessible.
    Matched MeSH terms: Child; Child, Preschool
  4. Aamer Naseer, Humayun Iqbal Khan, Mian Jawad Ahmad, Rabia Tariq, Atif Amin Baig
    MyJurnal
    Objective: To compare the mean duration of diarrhea between children taking yogurt and with the group taking commercial probiotics, containing lactobacillus in acute watery diarrhea. Materials and

    Methods: This descriptive study was conducted in the Department of Pediatrics, Services Hospital, Lahore. The duration of this cross sectional study was 6 months, from June 2013 to November 2013. A total of 200 children suffering from acute watery diarrhea were involved in the study after the informed consent under the said hospital. Their demographic information was recorded and later divided in two groups of 100 each; group A received yogurt and group B was given commercial probiotic. The duration of diarrhea was observed in both groups. The results were compared by using independent sample t-test.

    Results: The average duration of diarrhea in group A, was 1.98 + 1.31 day while that in group B was 3.09 + 1.64 days. Student's t-test was applied and the difference between the two groups was found to be statistically significant (p< 0.05).

    Conclusions: Treatment with yogurt significantly decreases the mean duration of diarrhea as compared to probiotic among patients with acute watery diarrhea.
    Matched MeSH terms: Child
  5. Ab Ghaffar SF, Mohd Sidik S, Ibrahim N, Awang H, Gyanchand Rampal LR
    PMID: 31817328 DOI: 10.3390/ijerph16244913
    Anxiety is one of the most common mental health disorders in childhood, and children with anxiety have an increased risk of psychiatric disorders during adulthood. This study aimed to evaluate the effectiveness of a school-based anxiety prevention program for reducing anxiety among primary school students relative to a school-as-usual control group. Secondary to this, the current study aimed to examine the effect of a school-based prevention program on worry coping skills and self-esteem. A two-group parallel cluster randomized controlled trial of a single-blinded study was conducted to evaluate the effectiveness of the program, with schools as the unit of allocation and individual participants as the unit of analysis. The intervention program was conducted between May 2016 and December 2017. The primary outcome was anxiety, whereas the secondary outcomes were worry coping skills and self-esteem measured at three months post-intervention. Data were analyzed by using a generalized linear mixed model, accounting for the clustering effect. Subgroup analyses were performed for children with anxiety. A total of 461 students participated in this study. At baseline, there was no significant difference between groups for anxiety score, worry coping skills score, and self-esteem score (p > 0.05). The intervention was effective in reducing anxiety for the whole sample (p = 0.001) and the anxiety subgroup (p = 0.001). However, it was not effective in improving worry coping skills and self-esteem. These findings suggest that the program could be effective for reducing symptoms of anxiety when delivered in schools and provide some support for delivering this type of program in primary school settings.
    Matched MeSH terms: Child
  6. Ab Ghani N
    MyJurnal
    Accidental poisoning in children is a preventable condition. It is a problem mainly in children aged 1 to 4 years. This is a descriptive study of accidental poisoning in children below 12 years old admitted to Hospital Seremban from October 15, 1994 to October 14, 1995. A major cause of household accidental poisoning was kerosene ingestion (25%) followed by ingestion of medications (22%), liniment methyl salicylate (12 %), mothballs (12%) and hypochlorinate solution (9%). There was a higher incidence of poisoning in males (50.8%), among Indians (41.2%) and in children from lower social classes (67%). A greater proportion of the accident occurred when the child was taken care by the parents (53 %) and in the house (80%). Most of the poison was ingested from its original package (81%). The immediate action taken was sending the child to the hospital (75%) without carrying out other actions such as washing the child’s mouth. The delay of sending the child to hospital was due to husband not at home (57%). Most of the cases (72.1 %) were discharged well within 1-2 days following admission.
    Matched MeSH terms: Child
  7. Ab Hamid F, Che Azemin MZ, Salam A, Aminuddin A, Mohd Daud N, Zahari I
    Curr Eye Res, 2016 Jun;41(6):823-31.
    PMID: 26268475 DOI: 10.3109/02713683.2015.1056375
    PURPOSE: The goal of this study was to provide the empirical evidence of fractal dimension as an indirect measure of retinal vasculature density.

    MATERIALS AND METHODS: Two hundred retinal samples of right eye [57.0% females (n = 114) and 43.0% males (n = 86)] were selected from baseline visit. A custom-written software was used for vessel segmentation. Vessel segmentation is the process of transforming two-dimensional color images into binary images (i.e. black and white pixels). The circular area of approximately 2.6 optic disc radii surrounding the center of optic disc was cropped. The non-vessels fragments were removed. FracLac was used to measure the fractal dimension and vessel density of retinal vessels.

    RESULTS: This study suggested that 14.1% of the region of interest (i.e. approximately 2.6 optic disk radii) comprised retinal vessel structure. Using correlation analysis, vessel density measurement and fractal dimension estimation are linearly and strongly correlated (R = 0.942, R(2) = 0.89, p 

    Matched MeSH terms: Child
  8. Ab Rahim SN, Omar J, Tuan Ismail TS
    Ann Pediatr Endocrinol Metab, 2020 Sep;25(3):152-155.
    PMID: 32871650 DOI: 10.6065/apem.2040004.002
    The gonadotropin-releasing hormone (GnRH) stimulation test is a valuable tool in diagnosing and differentiating causes of early pubertal occurrences. Utility of the test can be limited in some instances, however, including the early phases of pubertal hypothalamic-pituitary-gonadal axis activation, in girls showing commonly overlapping pictures, and in obese children due to excess circulating estrogen that suppresses luteinizing hormone (LH). A lack of consistent baseline and stimulated gonadotropin cutoffs observed in different studies also contributes to limitations in testing. Nevertheless, early detection of true pathological causes for pubertal disorders is needed to allow prompt treatment and better prognosis. While basal LH can be beneficial as a good screening tool for detecting pubertal disorder, it does not preclude the need for GnRH testing. The aim of this review was to highlight the role of GnRH stimulation tests and varying testing cutoffs in diagnosis of precocious puberty and its classification.
    Matched MeSH terms: Child
  9. Ab Rahman A, Ab Rahman R, Ibrahim MI, Salleh H, Ismail SB, Ali SH, et al.
    PMID: 21706952
    The objectives of this study were to describe the knowledge of sexual and reproductive health among adolescents attending school and to compare the levels of knowledge between males and females and between older and younger groups of adolescents. A cross-sectional study was conducted among 1,034 secondary school students using a self administered validated questionnaire. The items with the fewest correct responses included: whether one can get pregnant after a single act of sexual intercourse (30.4%), whether sexual intercourse causes sexually transmitted diseases (STDs) (12.4%) and whether washing the vagina after sexual intercourse prevents pregnancy (17.0%). Their main source of sexual information was friends (64.4%). An independent t-test revealed the mean knowledge score was significantly higher among females than males on items assessing whether the genitalia may be touched freely by family members, females having attained menarche may become pregnant if having sex, whether pregnancy will occur if there is penetration of the penis into the vagina, whether premarital sexual intercourse causes pregnancy and if there is a relationship between abandoned babies and premarital pregnancies. The mean knowledge score assessing whether pregnancy can be prevented using condoms was higher among males than females. The mean knowledge scores were significantly higher among form four and form five students than forms one, two and three students. Lack of knowledge regarding important aspects of sexual and reproductive health warrant the need to strengthen sexual and reproductive health education.
    Matched MeSH terms: Child, Abandoned
  10. Ab Rahman AF, Ibrahim MI, Ismail HI, Seng TB
    Pharm World Sci, 2005 Oct;27(5):403-6.
    PMID: 16341748
    OBJECTIVE: (1) To determine the effect of lamotrigine add-on therapy on the seizure frequency and cost in paediatric patients. (2) To determine the prescribing pattern of other antiepileptic drugs (AEDs).

    METHOD: A retrospective study of medical records was carried out from October 2000 to June 2001 at the paediatric clinic, Hospital Pulau Pinang.

    MAIN OUTCOME MEASURE: Seizure frequency, cost of drug and types of AED prescribed.

    RESULTS: A total of 209 medical records were retrieved during the study period. Lamotrigine (LTG) was prescribed in 29 patients as add-on therapy. In 18 patients, there was a significant reduction in seizure frequency after the addition of LTG. Approximately 70% experienced a reduction in seizure frequency of more than 50%. Side effects of LTG were considered mild and manageable. However, drug cost after the addition of LTG increased by 103%. In the remaining 180 patients, the most common AED prescribed was sodium valproate (VPA). Only 15% of the patients received combination therapy. Mean monthly cost of monotherapy was found to be RM 24.4 while monthly cost of combination therapy was RM 45.4 (1 Euro-RM 5.00).

    CONCLUSION: The majority of paediatric patients in the study are on AED monotherapy and only a small percentage was prescribed lamotrigine. The use of lamotrigine is associated with better seizure control but with an increase in drug cost.

    Study site: paediatric clinic, Hospital Pulau Pinang.
    Matched MeSH terms: Child; Child, Preschool
  11. Ab Rahman AF
    Hum Exp Toxicol, 2002 Jul;21(7):377-81.
    PMID: 12269700
    A retrospective review of medical records was carried out to determine the pattern of drug and chemical poisoning cases admitted to a teaching hospital in Malaysia. Medical records of patients admitted during the period January 1987 to December 1995 were reviewed. They accounted for 0.2% of total admissions during the period. While all ages were represented, there was predominance of children, which showed little change throughout the nine-year period. Of all cases of poisoning, 77.8% were unintentional, 12.6% intentional and 9.6% were undetermined. Kerosene, pesticides and medicinal substances remained the common agents associated with poisoning. A significant number of patients sought treatment elsewhere before being admitted to the hospital. Of those who came directly to the hospital, many were presented late in the course of their poisoning. Only seven patients died and none were children. This study supports the need for greater emphasis on prevention of poisoning in children and among farmers in the state.
    Matched MeSH terms: Child; Child, Preschool
  12. Ab Rahman N, Sivasampu S, Mohamad Noh K, Khoo EM
    BMC Health Serv Res, 2016 06 14;16:197.
    PMID: 27301972 DOI: 10.1186/s12913-016-1444-0
    BACKGROUND: The world population has become more globalised with increasing number of people residing in another country for work or other reasons. Little is known about the health profiles of foreign population in Malaysia. The aim of this study was to provide a detailed description of the health problems presented by foreigners attending primary care clinics in Malaysia.

    METHODS: Data were derived from the 2012 National Medical Care Survey (NMCS), a cross sectional survey of primary care encounters from public and private primary care clinics sampled from five regions in Malaysia. Patients with foreign nationality were identified and analysed for demographic profiles, reasons for encounter (RFEs), diagnosis, and provision of care.

    RESULTS: Foreigners accounted for 7.7 % (10,830) of all patient encounters from NMCS. Most encounters were from private clinics (90.2 %). Median age was 28 years (IQR: 24.0, 34.8) and 69.9 % were male. Most visits to the primary care clinics were for symptom-based complaints (69.5 %), followed by procedures (23.0 %) and follow-up visit (7.4 %). The commonest diagnosis in public clinics was antenatal care (21.8 %), followed by high risk pregnancies (7.5 %) and upper respiratory tract infection (URTI) (6.8 %). Private clinics had more cases for general medical examination (13.5 %), URTI (13.1 %) and fever (3.9 %). Medications were prescribed to 76.5 % of these encounters.

    CONCLUSIONS: More foreigners were seeking primary medical care from private clinics and the encounters were for general medical examinations and acute minor ailments. Those who sought care from public clinics were for obstetric problems and chronic diseases. Medications were prescribed to two-thirds of the encounters while other interventions: laboratory investigations, medical procedures and follow-up appointment had lower rates in private clinics. Foreigners are generally of young working group and are expected to have mandatory medical checks. The preponderance of obstetrics seen in public clinics suggests a need for improved access to maternal care and pregnancy related care. This has implication on policy and health care provision and access for foreigners and future studies are needed to look into strategies to solve these problems.
    Matched MeSH terms: Child; Child, Preschool
  13. Abai G, Henry J, Lian CB, Wee ASF, Bili H, Ratu I
    Int J Public Health Res, 2011;1(2):92-99.
    MyJurnal
    Introduction Neonatal jaundice occurs in about 60% of newborns. If not managed properly, it can progress to severe neonatal jaundice (SNNJ) leading to death or permanent disability. The incidence of SNNJ in Kuching District increased from 119.3 per 100,000 live births in 2005 to 123.3 per 100,000 live births in 2008, which was above the Standard National QAP Indicator of 100 per 10,000 live births. SNNJ can be prevented by early detection and proper management of neonatal jaundice. The objective is to increase the knowledge and practise of early detection of neonatal jaundice by nurses in Kuching District. Methods This was an interventional study covering a period of six months. The sample comprised 113 nurses of all categories working in urban and rural maternal and child health clinics in Kuching District. Tools used in the study were self-administered questionnaires in English and Bahasa Malaysia. The preintervention survey started in July 2009 while the post-intervention survey was done in January 2010. The interventions were done through Continuing Nursing Education sessions and included new nursing formats and new reporting procedures. New vehicles were also provided for home nursing. Data was collected and analyzed using MS Excel program. Results The pre-intervention survey on nurses showed that only 56.6% were able to identify the risk of factors causing jaundice; 94.6% able to define jaundice; 41.5% able to detect jaundice while 70.8% knew sign of Kernicterus. In term of recommended post natal nursing schedule only 40.7% able to practice the schedule while only 69.0% able to give advice on management of jaundice. Post intervention; 63.2% of nurses were able to identify the risk factors causing jaundice; 97.2% able to define jaundice while 97.2% were able to detect jaundice and 88.6% know sign of Kernicterus. On recommended post natal nursing schedule, 49.9 % practice the recommended schedule while 92.0% were able to give advice to mother on management of jaundice. The incident of jaundice of Severe Neonatal Jaundice dropped to 78 per 100,000 live births in 2010.
    Conclusions The study shows that the interventions taken helped to improve the knowledge and practice of recommended measures to detect neonatal jaundice early. Stronger emphasis must be placed on using the new reporting procedures and new nursing sheets. Continuous monitoring through regular nursing audits by clinic supervisors is also essential to reduce the incidence of SNNJ. Provision of vehicles for all busy maternal and child health clinics for home nursing care is highly recommended.
    Matched MeSH terms: Child Health; Child
  14. Abbasi H, Saqib M, Jouhar R, Lal A, Ahmed N, Ahmed MA, et al.
    Biomed Res Int, 2021;2021:1119710.
    PMID: 34124238 DOI: 10.1155/2021/1119710
    Introduction: Dental anxiety is a common occurrence in patients undergoing dental treatments, especially in children. The success in paedriatric dental treatments and patient comfort depends on controlling the level of patient's anxiety in clinical settings. This study is aimed at evaluating the efficacy of different techniques applied for the reduction of dental anxiety in paediatric patients. Material and Methods. One hundred and sixty participants were divided into 4 groups; each group having 40 patients as follows: group I: mobile application "little lovely dentist," group II: YouTube® "dental video songs," group III "tell-show-do," and group IV "control." Dental prophylaxis treatments were provided to all the participants. Initial anxiety levels were noted during the patient's education phase by measuring heart rate with pulse oximeter and distress level with facial image scale, at the same time in each group, respectively. The postoperative anxiety was noted later with the same methods, after the application of anxiety reduction techniques. The data obtained were entered in the statistical package for the social sciences software, version 25. One-way ANOVA and paired t-test for matched groups were used to compare mean values of the 4 groups, in this study to determine their effectiveness. A p value of ≤0.05 was considered as statistically significant.

    Results: The mean age of patients in group 1 was 6.8 ± 2.1 years, group 2: 8.15 ± 2.27 years, group 3: 7.5 ± 2.3 years, and group 4: 7.27 ± 1.68 years. The intragroup comparisons of heart rate and facial image scores have shown a significant difference in before and after dental treatment procedures. Marked reduction in heart rate and facial image scale scores were found in patients belonging to group 1 (mobile applications) and group 2 (dental video songs). An increase in heart rate and facial image scale scores was seen in group 3 (tell-show-do) and the control group.

    Conclusion: The paediatric dental anxiety is a common finding in dental clinics. Behavior modification techniques like smartphone applications, "little lovely dentist," and "dental songs" can alleviate dental anxiety experienced by paediatric patients. The "tell-show-do" technique although most commonly used did not prove to be beneficial in the reduction of the anxiety levels.

    Matched MeSH terms: Child; Child, Preschool
  15. Abd Aziz A, Abdullah AF, Mahmud A
    Br J Hosp Med (Lond), 2007 Nov;68(11):616-7.
    PMID: 18087856 DOI: 10.12968/hmed.2007.68.11.27686
    Matched MeSH terms: Child, Preschool
  16. Abd Aziz N, Kadar M, Harun D, Mohd Rasdi HF
    Occup Ther Health Care, 2021 Apr;35(2):227-244.
    PMID: 33511894 DOI: 10.1080/07380577.2021.1876967
    This scoping review explores the implementation of video modeling (VM) by occupational therapists during the intervention process for children and adolescents with special needs. Four primary electronic databases were used to conduct the scoping review: PubMed, Scopus, CINAHL, and Proquest (Nursing and Allied Health). Research methodologies by Arksey and O'Malley were used as a framework to perform the review process. From the review, two themes emerged: (1) implementing VM by occupational therapists during the intervention process can successfully improve a client's desired skills; (2) VM can be applied through several types of technologies. The findings support occupational therapists' use of VM during interventions to positively enhance and promote desired skills among children and adolescents with special needs. Also, it can be incorporated into technological devices to suit a client's specific needs.
    Matched MeSH terms: Child; Disabled Children/rehabilitation*
  17. Abd Ghani A, Muslim NH, Zakaria MN
    Int J Pediatr Otorhinolaryngol, 2020 May;132:109907.
    PMID: 32018165 DOI: 10.1016/j.ijporl.2020.109907
    OBJECTIVE: Language disorders can affect reading skills contributing to poor academic achievements. As such, it is vital for speech language pathologists to identify the potential language-based reading problems among children. To achieve this, specific reading assessments should be developed and studied. Thus, the present study aimed to determine the effects of gender and academic achievement on oral reading fluency (ORF) and silent reading fluency (SRF) among Year 2 Malaysian children.

    METHODS: In this study that employed a comparative design, fifty-nine typically developing Malaysian children (24 boys, aged from 8 years, 0 months to 8 years, 11 months) were enrolled. They were chosen from three different classrooms (consisting of high achieving, medium achieving and low achieving students, respectively) in a primary school. Their ORF and SRF skills were assessed according to the established methods.

    RESULTS: As revealed by two-way ANOVA results, both ORF and SRF scores were found to be comparable between boys and girls (p > 0.05). In contrast, ORF and SRF results between the three classrooms were significantly different from each other (p 

    Matched MeSH terms: Child
  18. Abd Halim H, Abdul-Razak S, Md Yasin M, Isa MR
    Hum Vaccin Immunother, 2020 05 03;16(5):1040-1049.
    PMID: 31567057 DOI: 10.1080/21645515.2019.1674112
    Vaccine hesitant parents are linked with re-emergence of vaccine preventable diseases, but evidence is scarce locally. The Parent Attitudes about Childhood Vaccines (PACV) questionnaire was validated and used in the USA to identify vaccine hesitant parents. This study aimed to adapt and translate the 15-item PACV questionnaire from English into the Malay language, and to examine its validity and reliability. The sample population was parents of children aged 0-24 months, recruited at an urban government health clinic between November 2016-June 2017. During content validation, two items from the "Behavior" subdomain were identified as items with formative scale and excluded from exploratory factor analysis (EFA) but retained as part of demography. A total of 151 parents completed the questionnaire with response rate of 93.3%. Test-retest reliability was tested in 25 respondents four weeks later and the intra-class correlation was between 0.53 and 1.00. EFA of the 13 items showed possibility of two to four factor domains, but three domains were most conceptually equivalent. Two of the domains were similar to the original and one factor was identified de novo. One item was deleted due to poor factor loading of < 0.3. Therefore, the validated final PACV-Malay consisted of 12 items framed within three-factor domains. The PACV-Malay was reliable with total Cronbach alpha of 0.77. In conclusion, the PACV-Malay is a valid and reliable tool which can be used to identify vaccine hesitant parents in Malaysia. Confirmatory factor analysis and predictive validity are recommended for future studies.
    Matched MeSH terms: Child
  19. Abd Hamid IJ, Slatter MA, McKendrick F, Pearce MS, Gennery AR
    Blood, 2017 04 13;129(15):2198-2201.
    PMID: 28209722 DOI: 10.1182/blood-2016-11-748616
    Hematopoietic stem cell transplantation (HSCT) cures the T-lymphocyte, B-lymphocyte, and natural killer (NK)-cell differentiation defect in interleukin-2 γ-chain receptor (IL2RG)/JAK3 severe combined immunodeficiency (SCID). We evaluated long-term clinical features, longitudinal immunoreconstitution, donor chimerism, and quality of life (QoL) of IL2RG/JAK3 SCID patients >2 years post-HSCT at our center. Clinical data were collated and patients/families answered PedsQL Generic Core Scale v4.0 questionnaires. We performed longitudinal analyses of CD3+, CD4+ naive T-lymphocyte, CD19+, and NK-cell numbers from pretransplant until 15 years posttransplant. Thirty-one of 43 patients (72%) survived. Median age at last follow-up was 10 years (range, 2-25 years). Twenty-one (68%) had persistent medical issues, mainly ongoing immunoglobulin replacement (14; 45%), cutaneous viral warts (7; 24%), short stature (4; 14%), limb lymphoedema (3; 10%), and bronchiectasis (2; 7%). Lung function was available and normal for 6 patients. Longitudinal analysis demonstrated sustained CD3+, CD19+, and NK-cell output 15 years post-HSCT. CD4+ naive lymphocyte numbers were better in conditioned vs unconditioned recipients (P, .06). B-lymphocyte and myeloid chimerism were highly correlated (ρ, 0.98; P < .001). Low-toxicity myeloablative conditioning recipients have better B-lymphocyte/myeloid chimerism and are free from immunoglobulin replacement therapy. IL2RG/JAK3 SCID survivors free from immunoglobulin replacement have normal QoL.
    Matched MeSH terms: Child; Child, Preschool
  20. Abd Hamid IJ, Azman NA, Gennery AR, Mangantig E, Hashim IF, Zainudeen ZT
    Front Immunol, 2020;11:1923.
    PMID: 32983118 DOI: 10.3389/fimmu.2020.01923
    Introduction: Primary immunodeficiency diseases (PIDs) are under-reported in Malaysia. The actual disease frequency of PID in this country is unknown due to the absence of a national patient registry for PID. Objective: This systematic review aimed to determine the prevalence rates of PID cases diagnosed and published in Malaysia from 1st of January 1979 until 1st of March 2020. It also aimed to describe the various types of PIDs reported in Malaysia. Method: Following the development of a comprehensive search strategy, all published literature of PID cases from Malaysia was identified and collated. All cases that fulfilled the International Union of Immunological Societies (IUIS) classification diagnosis were included in the systematic review. Data were retrieved and collated into a proforma. Results: A total of 4,838 articles were identified and screened, with 34 publications and 119 patients fulfilling the criteria and being included in the systematic review. The prevalence rate was 0.37 per 100,000 population. In accordance with the IUIS, the distribution of diagnostic classifications was immunodeficiencies affecting cellular and humoral immunities (36 patients, 30.3%), combined immunodeficiencies with associated or syndromic features (21 patients, 17.6%), predominant antibody deficiencies (24 patients, 20.2%), diseases of immune dysregulation (13 patients, 10.9%), congenital defects in phagocyte number or function (20 patients, 16.8%), defects in intrinsic and innate immunity (4 patients, 3.4%), and autoinflammatory disorders (1 patient, 0.8%). Parental consanguinity was 2.5%. Thirteen different gene mutations were available in 21.8% of the cases. Conclusion: PIDs are underdiagnosed and under-reported in Malaysia. Developing PID healthcare and a national patient registry is much needed to enhance the outcome of PID patient care.
    Matched MeSH terms: Child; Child, Preschool
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