Displaying publications 1 - 20 of 73 in total

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  1. 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
  2. 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
  3. Hb lepore/β0-thalassaemia with α+-thalassaemia interactions, a potential diagnostic pitfall
    Alauddin H, Mohamad Nasir S, Ahadon M, Raja Sabudin RZ, Ithnin A, Hussin NH, et al.
    Malays J Pathol, 2015 Dec;37(3):287-92.
    PMID: 26712677
    Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of both patients showed features of thalassaemia intermedia. Other laboratory investigations of the patients showed conflicting results. However, laboratory investigation results of the parents had led to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis, hence appropriate clinical management and genetic counseling.
    Matched MeSH terms: Siblings
  4. A case series of α-thalassemia intermedia due to compound heterozygosity for Hb Adana [HBA2: c179G>A (or HBA1); p.Gly60Asp] with other α-thalassemias in Malay families
    Alauddin H, Jaapar NA, Azma RZ, Ithnin A, Razak NF, Loh CK, et al.
    Hemoglobin, 2014;38(4):277-81.
    PMID: 24829075 DOI: 10.3109/03630269.2014.916720
    Hb Adana [HBA2: c179G>A (or HBA1); p.Gly60Asp] is a rare hemoglobin (Hb) variant due to a mutation at codon 59 of the α2- or α1-globin gene resulting in a glycine to aspartic acid substitution. Two siblings with a unique coinheritance of Hb Adana and Hb Constant Spring (Hb CS, α142, Term→Gln, TAA>CAA; HBA2: c.427 T>C) (α(codon 59)α/α(CS)α), were compared phenotypically with another two siblings carrying the Hb Adana mutation and a 3.7 kb deletion (α(codon 59)α/-α(3.7)). Although they all had α-thalassemia intermedia (α-TI), the former were clinically more severe than the latter. The first pair of siblings presented at a much younger age than the second pair and showed lower Hb levels and significant extramedullay hemopoiesis. Another case of a hydropic fetus as a result of Hb H/Hb Adana is also described. Their clinical phenotypes and hematological parameters are all presented for comparison.
    Matched MeSH terms: Siblings
  5. Fulltext Choroidal neovascularization secondary to Best's vitelliform macular dystrophy in two siblings of a Malay family
    Alisa-Victoria K, Jin-Poi T, Shatriah I, Zunaina E, Ngah NF
    Clin Ophthalmol, 2014;8:537-42.
    PMID: 24648718 DOI: 10.2147/OPTH.S55623
    Best's vitelliform macular dystrophy complicated with choroidal neovascularization is rare in children. We report three children from a Malay family of five siblings with Best's vitelliform macular dystrophy, in which two of them subsequently developed choroidal neovascularization. The possible pathogenesis of this rare condition is described and highlighted in this report.
    Matched MeSH terms: Siblings
  6. Fulltext Relational aggression in sibling context: scale revision and factor analysis in a taiwanese sample
    Anna Ong WH, Sanggari K, Wirawahida Kamarul Z
    Jurnal Psikologi Malaysia, 2017;31:67-78.
    The Forms and Functions of Aggression Questionnaire is widely used to measure the aggressive behavior in the peer context by many researchers. This scale was developed by Little, Jones, Henrich, and Hawley in 2003. It consists of four principle dimensions of aggressive behavior: overt and relational aggression, instrumental and reactive aggression. The objectives of this study were (i) to evaluate the revised and translated version of this scale by CFA, (ii) to know the reliability of the model, and (iii) to prove the validity of the model. The Chinese version appears to be a valid and reliable instrument for the assessment of relational aggression against sibling. The resulting model will help future researchers, especially related to aggression in the sibling context.
    Matched MeSH terms: Siblings
  7. Haematopoietic stem cell transplantation for inborn errors of immunity: 25-year experience from University of Malaya Medical Centre, Malaysia
    Ariffin H, Ab Rahman S, Jawin V, Foo JC, Amram NF, Mahmood NM, et al.
    J Paediatr Child Health, 2020 Mar;56(3):379-383.
    PMID: 31479560 DOI: 10.1111/jpc.14621
    AIM: Inborn errors of immunity (IEI) comprise a heterogeneous group of disorders of the immune system, most of which are curable by haematopoietic stem cell transplantation (HSCT). We present a 25-year audit of HSCT for IEI at a tertiary-level academic hospital in Malaysia.

    METHODS: Review of medical records of all cases of IEI who underwent HSCT between January 1993 and December 2018 at our centre. Diagnoses, complications, HSCT protocols and outcome data were studied.

    RESULTS: There were 20 patients (19 boys) with a median age at diagnosis of 11 months (range: 2 months to 12 years). Eleven of 19 (58%) had malnutrition at presentation. Donor sources were variable: 13 (65%) matched sibling donor (MSD), 4 (20%) human leukocyte antigen-haploidentical donor (HD) and 3 (15%) matched unrelated donor (MUD). Conditioning regimens were physician-dependent and adapted to each patient's clinical status. Grades III-IV acute graft-versus-host disease occurred in two of three cases who received MUD grafts, 50% in those who received HD, and 8% in the MSD group. Transplant-related mortality at day +100 was 5%. With a median follow-up of 7.5 years, 18 (90%) patients are alive and free of infections.

    CONCLUSION: Outcome of HSCT for IEI in our centre is comparable with international reports. HSCT results using HD and MUD grafts are also good despite challenges from acute graft-versus-host disease, providing a feasible alternative for patients without matched donors.

    Matched MeSH terms: Siblings
  8. Fulltext Co-inheritance of compound heterozygous Hb Constant Spring and a single -alpha(3.7) gene deletion with heterozygous deltabeta thalassaemia: a diagnostic challenge
    Azma RZ, Othman A, Azman N, Alauddin H, Ithnin A, Yusof N, et al.
    Malays J Pathol, 2012 Jun;34(1):57-62.
    PMID: 22870600
    Haemoglobin Constant Spring (Hb CS) mutation and single gene deletions are common underlying genetic abnormalities for alpha thalassaemias. Co-inheritance of deletional and non-deletional alpha (alpha) thalassaemias may result in various thalassaemia syndromes. Concomitant co-inheritance with beta (beta) and delta (delta) gene abnormalities would result in improved clinical phenotype. We report here a 33-year-old male patient who was admitted with dengue haemorrhagic fever, with a background history of Grave's disease, incidentally noted to have mild hypochromic microcytic red cell indices. Physical examination revealed no thalassaemic features or hepatosplenomegaly. His full blood picture showed hypochromic microcytic red cells with normal haemoglobin (Hb) level. Quantitation of Hb using high performance liquid chromatography (HPLC) and capillary electrophoresis (CE) revealed raised Hb F, normal Hb A2 and Hb A levels. There was also small peak of Hb CS noted in CE. H inclusions was negative. Kleihauer test was positive with heterocellular distribution of Hb F among the red cells. DNA analysis for alpha globin gene mutations showed a single -alpha(-3.7) deletion and Hb CS mutation. These findings were suggestive of compound heterozygosity of Hb CS and a single -alpha(-3.7) deletion with a concomitant heterozygous deltabeta thalassaemia. Co-inheritance of Hb CS and a single -alpha(-3.7) deletion is expected to result at the very least in a clinical phenotype similar to that of two alpha genes deletion. However we demonstrate here a phenotypic modification of alpha thalassemia presumptively as a result of co-inheritance with deltabeta chain abnormality as suggested by the high Hb F level.
    Matched MeSH terms: Siblings
  9. Fulltext Factor IX mutations in haemophilia B patients in Malaysia: a preliminary study
    Balraj P, Ahmad M, Khoo AS, Ayob Y
    Malays J Pathol, 2012 Jun;34(1):67-9.
    PMID: 22870602 MyJurnal
    Haemophilia B is caused by coagulation defects in the factor IX gene located in Xq27.1 on the X chromosome. Identification of mutations contributing to defective factor IX may be advantageous for precise carrier and prenatal diagnosis. We studied 16 patients from 11 families, consisting of 8 patients of the Malay ethnic group, of which 6 were siblings. Factor IX mutations have not been previously reported in the Malay ethnic group. The functional region of the factor IX gene was sequenced and mutations were identified in either the exon or intronic regions in 15 of the patients. One novel mutation, 6660_6664delTTCTT was identified in siblings with moderate form of haemophilia B. Mutations identified in our patients when linked with disease severity were similar to findings in other populations. In summary, this preliminary data will be used to build a Malaysian mutation database which would facilitate genetic counseling.
    Matched MeSH terms: Siblings
  10. Mutational characterization of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in Malaysia
    Balraj P, Lim PG, Sidek H, Wu LL, Khoo AS
    J. Endocrinol. Invest., 2013 Jun;36(6):366-74.
    PMID: 23027774 DOI: 10.3275/8648
    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is a common autosomal recessive disorder. Our objective was to identify the 21-hydroxylase active gene, CYP21A2 mutations in Malaysian 21-OHD patients using different techniques.
    Matched MeSH terms: Siblings
  11. 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
  12. Fulltext Identification of two novel mutations, PSEN1 E280K and PRNP G127S, in a Malaysian family
    Ch'ng GS, An SS, Bae SO, Bagyinszky E, Kim S
    Neuropsychiatr Dis Treat, 2015;11:2315-22.
    PMID: 26396515 DOI: 10.2147/NDT.S86334
    Alzheimer's disease (AD) is the most common form of dementia, which can be categorized into two main forms: early onset AD and late onset AD. The genetic background of early onset AD is well understood, and three genes, the APP, PSEN1, and PSEN2 have been identified as causative genes. In the current study, we tested three siblings from Malaysia who were diagnosed with early onset dementia, as well as their available family members. The family history was positive as their deceased father was similarly affected. Patients were tested for mutations in APP, PSEN1, PSEN2, and PRNP. A novel variant, E280K, was discovered in exon 8 of PSEN1 in the three siblings. In silico analyses with SIFT, SNAP, and PolyPhen2 prediction tools and three-dimensional modeling were performed, and the results suggested that the mutation is probably a pathogenic variant. Two additional pathogenic mutations were previously been described for codon 280, E280A, and E280G, which could support the importance of the E280 residue in the PS1 protein contributing to the pathogenic nature of E280K. Additional ten family members were screened for the E280K mutation, and all of them were negative. Six of them presented with a variety of neuropsychiatric symptoms, including learning disabilities, epilepsy, and schizophrenia, while four family members were asymptomatic. A novel PRNP G127S mutation was found in a step-niece of the three siblings harboring the PSEN1 E280K mutation. In silico predictions for PRNP G127S mutation suggested that this might be possibly a damaging variant. Additional studies to characterize PRNP G127S would be necessary to further understand the effects of this mutation.
    Matched MeSH terms: Siblings
  13. 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
  14. 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
  15. Fulltext Childhood brain injury: a review
    Chee, Piau Wong, Ee, Lin Tay
    Neurology Asia, 2015;20(2):105-115.
    MyJurnal
    Childhood brain injury is an important and complicated public health issue worldwide. Extensive work has been done in this field. This review highlights issues that are frequently misinterpreted or overlooked in the management of childhood brain injury. The incidence of traumatic brain injury is higher than non-traumatic brain injury. However it is frequently over-reported due to various confounding factors. In ascertaining the severity of injury, assessment of brainstem functions is important and should be included in routine clinical assessment. Most rehabilitative efforts are usually aimed at improving the physical outcome. However, non-physical sequelae are also common and may be more disabling with significant impact on the learning and functioning of the child. These areas, which include depression, cognitive functioning and health-related quality of life of children, should not be overlooked in the management of childhood brain injury. In addition to caregiver’s stress, family dynamic and siblings’ well-being also play a crucial role in the recovery process of the child. By highlighting the frequently missed issues in the management of childhood brain injury, it is hoped that clinicians and professionals could pay more attention to these issues and provide a comprehensive medical care for the patients and their families.
    Matched MeSH terms: Siblings
  16. Familial neuromyelitis optica spectrum disorder in a pair of sisters
    Cheo SW, Ong SAM, Low QJ, Tan YA, Chia YK
    QJM, 2020 Oct 01;113(10):743-746.
    PMID: 32240316 DOI: 10.1093/qjmed/hcaa107
    Matched MeSH terms: Siblings
  17. The Heritability of Palatal Rugae Morphology Among Siblings*, †
    Chong JA, Syed Mohamed AMF, Marizan Nor M, Pau A
    J Forensic Sci, 2020 Nov;65(6):2000-2007.
    PMID: 32692413 DOI: 10.1111/1556-4029.14507
    Although there is clinical applicability of the palatal rugae as an identification tool in forensic odontology, controversy exists whether the palatal rugae patterns are stable or variable. The greater the genetic component, the higher the probability that palatal rugae patterns are stable. The aim of this study was to compare the palatal rugae morphology between full siblings and the proportion of variability due to genetic component. This cross-sectional study was conducted on digital models of 162 siblings aged 15-30 years old. The palatal rugae patterns were assessed with Thomas and Kotze (1983) classification using Geomagic Studio software (3D Systems, Rock Hill, SC). The palatal rugae morphology between siblings showed significantly similar characteristics for total number of left rugae (p = 0.001), left primary rugae (p = 0.017), secondary rugae for right (p = 0.024) and left sides (p = 0.001), right straight rugae (p = 0.010), and right convergent rugae (p = 0.005) accounting for at least 6.25%-12.8% of the variability due to heredity. Despite the similarities found, the palatal rugae patterns showed significant differences between siblings of at least 46.9% (p = 0.001). Zero heritability was found in 9 of the 14 rugae patterns. Meanwhile, total number of rugae, primary, backward, and convergent rugae showed moderate heritability (h2  > 0.3) and total number of secondary rugae showed high heritability (h2  > 0.6). In conclusion, despite the individuality characteristics, an appreciable hereditary component is observed with significant similarities found between sibling pairs and the palatal rugae patterns were both environmentally and genetically influenced.
    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. 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
  20. Predictors of the yield of mobilized peripheral blood CD34+ cells in HLA-matched sibling donor
    Fadilah SA, Mohd-Razif MI, Seery ZA, Nor-Rafeah T, Wan-Fariza WJ, Habsah A, et al.
    Transfus Apher Sci, 2013 Dec;49(3):583-9.
    PMID: 24012241 DOI: 10.1016/j.transci.2013.07.032
    We examined the donor factors that may affect the yield of peripheral blood stem cell (PBSC) mobilized from healthy donors. Pre-apheresis PB-CD34(+) cell count was the only factor that correlated with PBSC yield. Leukocyte count (LC) and monocyte count (MC) correlated with PB-CD34(+) cell. Male gender and PB-CD34(+) cell count of at least 87.1/μL and 69.8/μL on day-4 and -5 of G-CSF were associated with the ability to harvest at least 5×10(6)/kg CD34(+) cells after one apheresis. We concluded that gender and PB-CD34(+) cell count are important predictors of PBSC yield. LC and MC may serve as surrogate markers for estimating the PB-CD34(+) cell count.
    Matched MeSH terms: Siblings
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