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  1. Familial Paroxysmal Rhabdomyolysis, non-exertional type, in a Malay family - a case report
    Hussain Imam Muhammad Ismail, Azizi Omar
    Malaysian Journal of Child Health, 1993;5(1):57-61.
    MyJurnal
    Familial Paroxysmal Rhabdomyolysis of the non-exertional variety is a rare but treatable disorder previously not described in Malaysia. We report 3 Malay siblings of a consanguinous marriage who developed myoglobulinuria during a febrile illness. The first 2 died without being investigated, but the last had creatinine kinase levels of 24,800 UIL suggesting acute rhabdomyolysis. Fasting appears to be a major precipitating factor, and glucose infusions combined with alkaline diuresis may be life-saving.
    Matched MeSH terms: Siblings
  2. Clinical characteristics and outcome of children with febrile convulsions
    Abdul Wahab Jantan, Zabidi Azhar Mohd Husin
    Malaysian Journal of Child Health, 1997;9(1):73-82.
    MyJurnal
    Objective: The clinical characteristics and out-come offebrile convulsions in children admitted to the University Hospital in Kubang Kerian were analysed in this retrospective study.

    Method: The medical records of 244 children aged between 6 months to 5 years who presented with their first convulsions between January 1989 to December 1990 were reviewed. Patients were followed till one year after their first febrile convulsions.

    Results: The mean age of presentation was 18.26 (s.d. 11.83) months. One hundred and thirty (54.5%) were males. Complex febrile convulsions were noted in 47.5% and simple febrile convulsions in 52.5%. Seventy-two children (29.5%) were less than one year old at the time offirst febrile convulsions. A family history offebrile convulsions was significantly higher in the complexfebrile convulsions group. Ten children (4.1%) presented with prolonged first febrile convulsions. Data on 117 children on follow-up were available for analysis. Recurrence of febrile convulsions occurred in fifty children (46.7%) with mean interval of 6.53 (s.d. 5.25) months. There was significant difference in children who presented with febrile convulsions at age of less than one year old and having family history offebrile convulsions with regard to recurrence. Three children developed epilepsy at a mean age of 31.56 months. Identifiable causes of febrile convulsions were upper respiratory infection, presumed viral infection (fever with rashes) and acute gastro-enteritis. Laboratory investiga-tions that were done were not helpful.

    Conclusions: Children with a family history of febrile convulsions were more likely to develop complex febrile convulsions. Routine investi-gations were rarely helpful. The recurrence rate is significantly influenced by the age of presentation and family history of febrile convulsions in siblings or either parent. The types offebrile convulsions did not significantly influence the recurrent rate.
    Matched MeSH terms: Siblings
  3. Severe mental retardation following asymptomatic hypoglycaemia in an infant with nesidioblastosis
    Rahmah, R., Wu, L.L., Roziana, A., Swaminathan, M., Kuhnle, U.
    Malaysian Journal of Child Health, 1997;9(1):93-96.
    MyJurnal
    Nesidioblastosis is a rare metabolic disease characterised by inappropriate insulin secretion often associated with life-threatening hypoglycaemia. While severe cases present in the newborn period, patients have been described later in infancy. Familial cases suggest an autosomal recessive trait, and recently mutations in the sulphonlurea receptor gene, possibly a regulator of insulin secretion, have been identified and associated with disease expression. We report a twin boy who developed normally until the age of six months when he was noted to regress. The boy is the older twin born to non-consanguinous parents. He presented to a hospital first at the age of 13 months with fever and generalised seizures. Low blood glucose was noted, but he recovered easily and was able to maintain euglycaemia during a 48-hour period of observation. Microcephaly and developmental delay were documented and anticonvulsant therapy was started. At 18 months, low blood glucose with high C-peptide was documented during reevaluation. Follow-ing a short trial of subcutaneous long-acting somatostatin analogue, the child was subjected to near-total pancreatectomy. The histology revealed findings consistent with nesidioblastosis. The child's condition improved but he remained significantly delayed This case emphasises the importance of recognising and treating hypoglycaemia early to avoid irreversible brain damage. It is interesting to note that the twin brother has always been well and is developmentally normal. Further studies to identify the inheritance pattern in the family would be of great interest.
    Matched MeSH terms: Siblings
  4. The search for a matched bone marrow donor in the Malaysian population
    Chan, L.L., Law, C.W., Hunn, P.S., Yew, C.B., Lim, P.P.L., Teo, L.T., et al.
    Malaysian Journal of Child Health, 1997;9(2):182-188.
    MyJurnal
    In Malaysia, an estimated 50 children per annum suffering from a variety of haematological and inherited disorders would benefit from bone marrow or stem cell trans-plantation. By mendelian inheritance 25% of these children would be able to find a sibling who is a matched histocompatible donor. For the remaining 75% to have a chance at survival, search from another source would have to be made. This could mean a mismatched non-sibling related donor or a matched unrelated donor. We studied the chance of a Malaysian patient finding a matched sibling donor and a matched unrelated donor. Human Leucocyte Antigen (HLA) data from patients and their siblings were analysed. The HLA data were matched against the largest Asian bone marrow donor registry in Taiwan. 95% of the 138,744 donors in this registry come from Taiwanese Hokkien ancestry.
    Matched MeSH terms: Siblings
  5. Risk factors for acute lower respiratory tract infections in hospitalised children in Kelantan
    Choo, C.M., Quah, B.S., Rostenberghe, H.V., Choo, K.E.
    Malaysian Journal of Paediatrics and Child Health, 1998;10(2):1-7.
    MyJurnal
    A case control study was conducted to identify the risk factors for acute lower respiratory tract infections (ALRI) in hospitalised children in Kelantan. One hun-dred and twenty children aged one month to five years hospitalised for ALRI were matched by age with 120 children as controls. Data on demography and expo-sure to putative risk factors were collected by interview-ing parents or caretakers. Anthropometric measure-ments were also carried out to assess the nutritional sta-tus of the children. For each risk factor studied, the odds ratios for exposure and disease were calculated by using univariate analysis followed by multiple logistic regression analysis to determine those factors which remained significant.
    The presence of sibling(s) who coughed at home (OR 12.1, 95% CI 5.2-28.1), crowding in bedroom (OR 4.4, 95% CI 2.1-9.0), weight-for-age < 3rd percentile (OR 9.0, 95% CI 3.1-25.8), lack of breast feeding (OR 9.4, 95% CI 2.3-38.4) and incomplete immunisation (OR 4.5, 95% CI 1.7-12.1) were significant indepen-dent risk factors for ALRI. Other factors like poverty, maternal education level, male sex, low birth weight, history of atopy, family history of asthma and indoor air pollution were not associated with an increased risk of ALRI.
    This study showed that poor nutritional status, inap-propriate child care practices and poor living conditions, particularly those related to crowding, predispose to ALRI in Kelantanese children necessitating hospital admission. A change in these factors may reduce the morbidity and mortality of childhood ALRI in Kelantan.
    Matched MeSH terms: Siblings
  6. Current issues in the management of thalassaemia
    Rahman Jamal
    Malaysian Journal of Child Health, 1998;10(1):1-8.
    MyJurnal
    The thalassaemias are the commonest single gene disorders amongst the inherited diseases. In Malaysia, there are an estimated 2200 transfusion dependent thalassaemia patients. With a carrier rate of 3-5%, 120-340 new cases of thalassaemia are expected to be born each year. The reference treatments for these patients are regular blood transfusions and iron chelation therapy. With optimal management, these patients are able to survive into the third or fourth decade of life and most importantly avoid the complications related to transfusions and iron overload. The use of desferal locally is still limited to only those who can afford i.e. about 30% of the cases. Treatment for some of the complications such as hepatitis C, diabetes mellitus, growth impairment and pubertal delay, are now available. Curative treatment approaches like bone marrow transplantation have now become standard treatment for eligible cases whilst cord blood transplantation may yet offer hope for those who are without compatible sibling donors. Research on globin gene therapy looks very promising but will probably take some time to deliver. Hb F switching is a very novel idea but so far the results are mainly anecdotal. Finally, the strive for optimal management of thalassaemia must come hand in hand with a prevention programme to achieve a reduction of new cases.
    Matched MeSH terms: Siblings
  7. Sleep problems among children with neurological disorders
    Khoo, T.B., Muhammad Ismail, H.I., Abdul Manaf, A.M.
    Malaysian Journal of Paediatrics and Child Health, 2000;12(1):22-28.
    MyJurnal
    A study was conducted to evaluate the extent of sleep problems among children aged between 6 to 15 years old who were followed up at Penang Hospital Paediatric Clinic for various neurological disorders and compared to those with other paediatric illnesses and their healthy siblings. A parental questionnaire was used to assess sleep problems in 48 children with neurological disorders and compared to 46 of their healthy siblings, 59 children with non-neurological paediatric illnesses and 67 of their healthy siblings. Sleep problems were clustered into five subscales: bedtime difficulties, parental involvement at time of sleep, sleep fragmentation, parasomnias and daytime drowsiness. Children with neurological disorders had significantly more sleep problems than did their siblings, those with non-neurological paediatric illnesses and their healthy siblings (p < 0.001). This was particularly so in areas of bedtime difficulties (p>0.001), the amount of parental involvement (p

    Study site: Penang Hospital Paediatric Clinic
    Matched MeSH terms: Siblings
  8. Consent for lumbar puncture -factors that influence the decision
    Malik, A.S.
    Malaysian Journal of Paediatrics and Child Health, 2000;12(1):35-41.
    MyJurnal
    Lumbar puncture (LP) is an important procedure both for diagnostic and therapeutic purposes. In Kelantan, Malaysia, on many occasions consent for this procedure is not granted by patients or guardians. The aim of this study was to find out the factors that influence the decision to grant or refuse the consent for LP. This was a cross-sectional study in which 86 parents (who agreed or refused to give consent for LP on their child) were interviewed by using a standard questionnaire. A scoring system was used to assess their knowledge about the purpose and technique of the procedure. Consent for LP was granted in 23 and refused in 27 children. The refusal rate was significantly higher when family members other than parents made the decision. The factors which did not play a statistically significant role in decision-making included: age of the child and parents; gender of the patient; number of children in the family; patient's birth order among the siblings; place of residence; monthly income of the family; parents' level of education; and number of days after admission when LP was requested. The factors that positively influenced the decision to give consent included knowledge about the purpose of LP and underlying disease. In order to receive positive consent for LP it is concluded that the parents/guardians and other family members who may influence the decision-making should be explained the role of LP in (1) making diagnosis and (2) choosing right antibiotics for treatment. They should also be informed about the side-effects of antibiotics, which may be used unnecessarily in unconfirmed cases of central nervous system infections.
    Matched MeSH terms: Siblings
  9. A case of T-cell acute lymphoblastic leukemia after treatment of acute promyelocytic leukemia
    Bee PC, Gan GG, Sangkar JV, Teh A, Goh KY
    Int J Hematol, 2004 May;79(4):358-60.
    PMID: 15218965
    We diagnosed T-cell acute lymphoblastic leukemia (T-ALL) with multiple cytogenetic abnormalities in a 17-year-old girl a year after she had received a diagnosis of acute promyelocytic leukemia (APML). After the diagnosis of APML in June 2001, the patient was treated with idarubicin and all-trans-retinoic acid. In September 1999, her younger sister also received a diagnosis of APML and to date has remained well. T-ALL after remission of APML is very rare, and only 1 such case has been reported. Possible causes include therapy-related reasons, genetic susceptibility to leukemia, and environmental exposure.
    Matched MeSH terms: Siblings
  10. Malaysian siblings with friedreich ataxia and chorea: a novel deletion in the frataxin gene
    Spacey SD, Szczygielski BI, Young SP, Hukin J, Selby K, Snutch TP
    Can J Neurol Sci, 2004 Aug;31(3):383-6.
    PMID: 15376485
    BACKGROUND: Friedrich ataxia (FRDA1) is most often the result of a homozygous GAA repeat expansion in the first intron of the frataxin gene (FRDA gene). This condition is seen in individuals of European, North African, Middle Eastern and Indian descent and has not been reported in Southeast Asian populations. Approximately 4% of FRDA1 patients are compound heterozygotes. These patients have a GAA expansion on one allele and a point mutation on the other and have been reported to have an atypical phenotype.

    OBJECTIVE: To describe a novel dinucleotide deletion in the FRDA gene in two Malaysian siblings with FRDA1.

    SETTING: Tertiary referral university hospital setting.

    PATIENTS AND METHODS: A previously healthy 10-year-old Malaysian boy, presented with fever, lethargy, headaches, dysarthria, dysphagia, vertigo and ataxia which developed over a one week period. His neurological exam revealed evidence of dysarthria and ataxia, mild generalized weakness and choreoform movements of the tongue and hands. His reflexes were absent and Babinski sign was present bilaterally. A nine-year-old sister was found to have mild ataxia but was otherwise neurologically intact.

    RESULTS: Molecular genetic studies demonstrated that both siblings were compound heterozygotes with a GAA expansion on one allele and a novel dinucleotide deletion on the other allele.

    CONCLUSIONS: We describe a novel dinucleotide deletion in the first exon of the FRDA gene in two siblings with FRDA1. Additionally this is the first report of FRDA1 occurring in a family of southeast Asian descent, it demonstrates intrafamilial phenotypic variability, and confirms that atypical phenotypes are associated with compound heterozygosity.

    Matched MeSH terms: Siblings
  11. Fulltext Past Fluoride Exposure From Ingested Toothpaste Extrapolated From Younger Siblings And Fluorosis Status
    Tan, B.S., Razak, I.A., Foo, L.C.
    Ann Dent, 2004;11(1):-.
    MyJurnal
    The aim of the study was to assess the association between past fluoride exposures from ingested toothpaste and current fluorosis manifestations in 10- 11 year old index subjects. Fluorosis was assessed with the Dean’s Index in 1343 10-11 year old index subjects. Two hundred index subjects who had younger 4-5 year old siblings were sub sampled and fluoride exposures from ingested toothpaste in their younger siblings (proxy subjects) were determined. The values for the fluoride ingested per brushing of the proxy subjects were utilised together with past frequency of toothbrushing of index subjects to extrapolate on the past exposure of index subjects. The mean extrapolated past fluoride exposure from ingestion of toothpaste was highly variable; 671.7 ug ± 739.3 ug (sem= 56.9). It was higher in the subjects with fluorosis (697.3 ug) than in those without fluorosis (646.89 ug) but differences were not of statistical significance. This approach of extrapolation has not been reported elsewhere and need to be validated. The implications of the present methodology to estimate past fluoride exposure is discussed.
    Matched MeSH terms: Siblings
  12. Further delineation of Al-Gazali syndrome (multiple skeletal abnormalities with anterior segment anomalies of the eye and early lethality) in a Malaysian family
    Chan LG, Ting HS
    Clin. Dysmorphol., 2005 Jan;14(1):1-5.
    PMID: 15602085
    We present two siblings from a consanguineous Malaysian family with multiple skeletal abnormalities, anterior segment anomalies of the eye and early lethality. These features are consistent with a syndrome first described by Al-Gazali and we provide further delineation of the syndrome.
    Matched MeSH terms: Siblings
  13. Fulltext Patterns of health ailments and other health problems among children at a relief camp, Aceh, Indonesia; a two months post tsunami study
    Adlina, S., Soe, Soe Aye, Narimah, A.H.H., Nuraliza, A.S.
    Malaysian Journal of Public Health Medicine, 2005;5(1):31-37.
    MyJurnal
    On December 26, 2004, an earthquake triggered a devastating tsunami that caused death and destruction in twelve countries including India, Indonesia, Malaysia, Maldives, Seychelles, Somalia, Sri Lanka and Thailand. One of the authors was a volunteer with FELDA WAJA AMAN MALAYSIA medical relief team that served the Aceh victims from 16th February to 24th February 2005 (8 weeks post tsunami). A study to determine the pattern of health ailments was conducted among children aged 18 years and below based at Seuneubok Camp, 30 km from Banda Aceh. All respondents were from Pulau Aceh and the total number of children seen and examined was 60. About 18% had lost their fathers, 10 % had lost their mothers and 27% had lost one or more of their siblings. 77% suffered some form of health ailments. The common health ailments were diarrhea (61%), respiratory complaints (59%) and fever (20%). About 38 % of preschoolers had loss of appetite and 28% had sleep disturbances. About 35% of the elementary school children suffered from sleep disturbances, 29% of the young adolescents suffered from headaches and 24% had sleep disturbances. Nearly a quarter (24%) of all the children felt fearful and anxious about the disaster. Nevertheless, 56% of the respondents wanted to return back to Pulau Aceh, although 14 % did not want to go back. Interestingly, 73% of the children voiced their gratitude to God for having been saved from death.
    Matched MeSH terms: Siblings
  14. Grossly delayed graft function in a living related kidney transplant recipient: a case report
    Thye YL, Lim SK, Wong CM, Keng TC, Chong YB, Tan SY
    Transplant Proc, 2008 Sep;40(7):2435-6.
    PMID: 18790259 DOI: 10.1016/j.transproceed.2008.07.051
    Delayed graft function is a potentially challenging problem especially in cadaveric kidney transplant recipients. It adversely impacts long-term graft survival. It is rarely seen in living kidney transplants. Recovery of graft function usually occurs within a month. The chances of recovery of graft function diminish with further prolongation of delayed function. In fact, recovery of graft function after 3 months has rarely been described, we report herein recovery of graft function after 132 days of nonfunction in a living related kidney transplant.
    Matched MeSH terms: Siblings
  15. Fulltext Biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency
    Ngu LH, Zabedah MY, Shanti B, Teh SH
    Malays J Pathol, 2008 Dec;30(2):109-14.
    PMID: 19291920 MyJurnal
    We report the biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency. Organic aciduria typical of this rare inborn error metabolism was found when the elder sibling presented with an episode of severe ketoacidosis at 20 months of age, which consisted of excessive excretion of ketones, tiglylglycine, 2-methyl-3-hydroxybutyrate, and 2-methylacetoacetate. Blood acylcarnitiness profile showed elevation of C5OH-carnitine, which represents 2-methyl-3-hydroxybutyrylcarnitine. A similar biochemical profile was identified in the younger sibling during screening although he had only mild clinical symptoms. Both patients reported a favourable outcome on follow-up.
    Matched MeSH terms: Siblings
  16. Social anxiety problem among medical students in Universiti Malaya Medical Center (UMMC) – A cross-sectional study
    Salina, M., Ng, C.G., Gill, J.S., Chin, J.M., Chin, C.J., Yap, W.F.
    Malaysian Journal of Psychiatry, 2008;17(2):0-0.
    MyJurnal
    Objective: To study the prevalence of social anxiety problem and potential risk factors that may be associated with social anxiety among medical students. Methods: Social Phobia Inventory (SPIN) and a questionnaire assessing gender, religion, number of siblings, type of school and partner status were given to 167 final year medical students. Results: There were 101 respondents of which 56% of the medical students scored > 19 in the Social Phobia Inventory (SPIN) suggesting that they were having social anxiety problem. None of the variables analysed were significantly associated with social anxiety. Conclusion: More than half of the medical students have significant social anxiety symptoms. No specific variables were found to be significantly associated with those at risk of developing social anxiety disorder.
    Matched MeSH terms: Siblings
  17. Bed sharing among mother-infant pairs in Klang district, Peninsular Malaysia and its relationship to breast-feeding
    Tan KL
    J Dev Behav Pediatr, 2009 Oct;30(5):420-5.
    PMID: 19827222 DOI: 10.1097/DBP.0b013e3181ba083a
    OBJECTIVE: The aim of the study was to determine the prevalence of mother-infant bed sharing in Klang district, Peninsular Malaysia and to identify factors associated with bed sharing.

    METHOD: This was a cross-sectional study involving 682 mother-infant pairs with infants up to 6 months attending government clinics in Klang district, Peninsular Malaysia. Data were collected by face-to-face interview using a pretested structured questionnaire for a 4-month period in 2006. Data regarding maternal, paternal, obstetric, infant, occupancy, breast-feeding characteristics, and bed-sharing practice were collected. Data on bed sharing were based on practice in the past 1-month period. Bed sharing was defined as an infant sharing a bed with mother, and infant must be within arms reach from the mother, whereas a bed was defined as either a sleeping mattress placed on a bed frame or placed on the floor. The prevalence of bed sharing was estimated. Relationship and magnitude of association between independent factors and bed sharing were examined using odds ratio and 95% confidence interval. Logistic regression analysis was used to control for confounding factors.

    RESULTS: The prevalence of bed sharing among mothers with infants aged between 1 and 6 months was 73.5% (95% confidence interval: 70.0-76.7). In multivariate analysis, urban/rural differences, mothers' ethnicity, occupation, family income, husbands' support on bed sharing, number of children younger than 12 years staying in the house, and breast-feeding were associated with bed sharing.

    CONCLUSIONS: These factors need to be considered in analyzing the overall risks and benefits of bed sharing, paying attention to breastfeeding practices.
    Matched MeSH terms: Siblings
  18. Fulltext A rare X-linked inherited mucocutaneous syndrome in two siblings
    Chong LA, Ariffin H
    Med J Malaysia, 2009 Dec;64(4):327-9.
    PMID: 20954562 MyJurnal
    We report on an 11 year-old boy with dyskeratosis congenita who presented with dystrophic nails, dysphagia, hyperpigmentation and oral leukoplakia. He had a brother who died 14 years earlier with similar presenting symptoms and aplastic anaemia. Genetic studies of our patient demonstrated the presence of a DKC1 mutation and confirmed our diagnosis. Further genetic screening revealed that his mother and one of his four sisters are heterozygous for the same mutation.
    Matched MeSH terms: Siblings
  19. Fulltext Challenge and support for breastfeeding in highly motivated Malaysian mothers
    Nazatul, S.B., Ruby, H.
    JUMMEC, 2009;12(2):70-73.
    MyJurnal
    The exclusive breastfeeding rate in Malaysia is very low. However in recent years the awareness of breastfeeding among mothers has increased. A preliminary qualitative research was carried out on these motivated mothers. The objective of this study was to understand the challenges encountered by breastfeeding mothers and to explore the support and motivation received by them. Information from the motivated mothers was obtained from focus group discussion. Some obstacles faced by the mothers were lack of knowledge on breastfeeding and lack of support from health professionals, parents and siblings. Facilities to express breast milk while at work were not readily available. The main motivation to breastfeed came from the mother herself and support from the husband. A holistic approach must be used to help mothers to continue breastfeeding. This includes breastfeeding promotion and education, setting up more Baby Friendly Hospitals, availability of breastfeeding support groups and provision of enough breastfeeding facilities at work and public places.
    Matched MeSH terms: Siblings
  20. Fulltext Stress and coping strategies among medical students in National University of Malaysia, Malaysia University of Sabah and Universiti Kuala Lumpur Royal College of Medicine Perak
    Johari, A.B., Noor Hassim
    Malaysian Journal of Community Health, 2009;15(2):106-115.
    MyJurnal
    Introduction : Stress is part of our life. It can happen anywhere including in medical school. Medical school is perceived as being stressful because their difficulties in education, longest period of study and dealing with the patients. Stress can be perceive as negative or positive. Coping strategies are the method that we can use to prevent stress when it comes to us. The aim of this study was to determine the prevalence of stress and coping strategies among of medical students in National University of Malaysia, Malaysia University of Sabah and Universiti Kuala Lumpur Royal College of Medicine Perak.
    Methods : This study involved 450 medical students through stratified sampling in which 150 medical students from each of the three universities. This study was conducted through self administered questionnaires. The questionnaires included were socio demographic factor, Personal Stress Inventory (using Stress Symptoms Scale with 52 items), BRIEF COPE (Coping Orientation for Problems Experienced with 28 items). The determination of cut off point for stress symptoms score was using Receiver Operating Characteristic (ROC) curve.
    Results : Response rate was 90.8%. The prevalence of stress among medical students were 44.1%. The contributory factors to the stress were financial problems, stress of up coming examination period, relationship problems with parents, peers, siblings and lecturers. Coping mechanisms which had significant association with stress includes self distraction, venting of emotion, denial, behavioral disengagement, humor and self blaming. Multiple linear regression analysis revealed a significant association (p
    Matched MeSH terms: Siblings
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