Displaying all 18 publications

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  1. 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.
  2. Khalid F, Chong LA
    Indian J Palliat Care, 2019 3 2;25(1):135-141.
    PMID: 30820116 DOI: 10.4103/IJPC.IJPC_111_18
    Objective: The objective of this study was to estimate palliative care needs and to describe the cohort of children with life-limiting illnesses (LLI) dying in hospitals.

    Design: This study was a retrospective cohort study. The national hospital admissions database was reviewed and children who had died who had life-limiting illnesses were identified.

    Setting: This study was conducted at Ministry of Health hospitals, Malaysia.

    Patients: Children aged 18 years and below who had died between January 1, 2012 and December 31, 2014.

    Main Outcome Measures: Life-limiting diagnoses based on Hain et al.'s directory of LLI or the ACT/RCPCH categories of life-limiting disease trajectories.

    Results: There were 8907 deaths and 3958 (44.4%) were that of children with LLI. The majority, 2531 (63.9%) of children with LLI were neonates, and the most common diagnosis was extreme prematurity <28 weeks with 676 children (26.7%). For the nonneonatal age group, the median age at admission was 42 months (1-216 months). A majority, 456 (32.0%) had diagnoses from the ICD-10 chapter "Neoplasms" followed by 360 (25.3%) who had a diagnoses from "Congenital malformations, deformations, and chromosomal abnormalities" and 139 (9.7%) with diagnoses from "Disease of the nervous system." While a majority of the terminal admissions were to the general ward, there were children from the nonneonatal age group, 202 (14.2%) who died in nonpediatric wards.

    Conclusion: Understanding the characteristics of children with LLI who die in hospitals could contribute toward a more efficient pediatric palliative care (PPC) service development. PPC service should include perinatal and neonatal palliative care. Palliative care education needs to extend to nonpediatric healthcare providers who also have to manage children with LLI.

  3. Chong LA, Khalid F
    Singapore Med J, 2016 Feb;57(2):77-80.
    PMID: 26893078 DOI: 10.11622/smedj.2016032
    There is increased awareness of paediatric palliative care in Malaysia, but no local published data on home care services. We aimed to describe the paediatric experience at Hospis Malaysia, a community-based palliative care provider in Malaysia.
  4. Sithasanan N, Chong LA, Ariffin H
    Med J Malaysia, 2007 Aug;62(3):247-8.
    PMID: 18246918 MyJurnal
    Phaeohyphomycosis consists of a group of mycotic infections characterized by the presence of dematiaceous (dark walled) septate hyphae. Splenic abscess and spontaneous rupture is an infrequent complication in children with haematological malignancies and can be life threatening. To the best of our knowledge this is the first report of a case of splenic rupture following the development of multiple abscesses secondary to infestation by this rare fungal species.
  5. Chong LA, Josephine P, Ariffin H
    Med J Malaysia, 2006 Jun;61(2):236-8.
    PMID: 16898320 MyJurnal
    We report a case of a child with severe congenital neutropenia (Kostmann's syndrome) who was treated with daily prophylactic subcutaneous granulocyte colony-stimulating factor (G-CSF) from the age of eight to sixteen years before being discontinued for poor haematological and clinical response. She did not have a HLA-matched sibling to enable bone marrow transplantation. She subsequently developed acute megakaryoblastic leukemia at the age of 17 years and succumbed during induction chemotherapy. The role of G-CSF in the pathogenesis of her malignant transformation to AML is complicated as this disorder has a propensity for myelodysplasia or AML as part of its natural history.
  6. Chong SY, Chong LA, Ariffin H
    Am J Emerg Med, 2010 Jun;28(5):603-6.
    PMID: 20579557 DOI: 10.1016/j.ajem.2009.02.006
    The aim of this study is to formulate an accurate estimate of the spinal needle depth for a successful lumbar puncture in pediatric patients.
  7. Chong LA, Chong PH, Chee J
    J Palliat Med, 2018 09;21(9):1242-1248.
    PMID: 29733235 DOI: 10.1089/jpm.2017.0626
    BACKGROUND: The provision of pediatric palliative care in Asia Pacific varies between countries and availability of essential medications for symptoms at the end of life in this region is unclear.

    OBJECTIVE: To determine medications available and used in the management of six symptoms at the end of life among pediatric palliative care practitioners in Asia Pacific. To identify alternative pharmacological strategies for these six symptoms if the oral route was no longer possible and injections are refused.

    DESIGN AND SETTING: An online survey of all Asia Pacific Hospice Palliative Care Network (APHN) members was carried out to identify medications used for six symptoms (pain, dyspnea, excessive respiratory secretions, nausea/vomiting, restlessness, seizures) in dying children. Two scenarios were of interest: (1) hours to days before death and (2) when injectables were declined or refused.

    RESULTS: There were 54 responses from 18 countries. Majority (63.0%) of respondents were hospital based. About half of all respondents were from specialist palliative care services and 55.6% were from high-income countries. All respondents had access to essential analgesics. Several perceived that there were no available drugs locally to treat the five other commonly encountered symptoms. There was a wide variation in preferred drugs for treating each symptom that went beyond differences in drug availability or formulations.

    CONCLUSION: Future studies are needed to explore barriers to medication access and possible knowledge gaps among service providers in the region, so that advocacy and education endeavors by the APHN may be optimized.

  8. Chan LL, Lin HP, Chong LA, Hany A, Ariffin AW
    Med J Malaysia, 2009 Jun;64(2):124-9.
    PMID: 20058571
    Children who would benefit from a haematopoietic stem cell transplantation often lacked a compatible sibling donor. Unrelated cord blood transplantation was offered as an alternative donor source for patients with a variety of malignant and non-malignant diseases who had no further treatment options. Cord blood units were sourced from various international cord blood registries. The median nucleated and CD34+ cell doses were 8.7 x 10(7)/kg and 2.6 x 10(5)/kg respectively. In spite of adequate cell doses, a high rate of non-engraftment of 32% was observed. Acute graft-versus-host disease (GVHD) occurred in 14 out of the 15 patients who engrafted with 53% being grade III to IV GVHD. The five year disease free survival was 40.7% with infection and GVHD being the commonest causes of death. The five year disease free survival was 20.5% and 60.7% for malignant and non-malignant diseases respectively.
  9. Chong LA, Lee WS, Goh AYT
    Med J Malaysia, 2003 Mar;58(1):89-93.
    PMID: 14556330
    The profile of admissions staying less than 24 hours admitted to the paediatric wards of University Malaya Medical Center, Kuala Lumpur, over a period of six weeks was reviewed to ascertain the need of a short-stay ward. Ninety-three (22%) of the 428 admissions admitted during the study period were discharged within 24 hours, 56 (60%) were discharged within 12 hours. Major categories of admissions were: elective investigative procedures (43%), and emergency admissions (44%). Reasons for emergency admissions: infections 42%, minor trauma/cerebral concussion 25% and febrile/afebrile seizures 11%. Only 20% required percutaneous oximetry monitoring and 2% required observations more frequently than 2 hourly. There may be a case for a short stay ward in a big paediatric unit in Malaysia.
  10. Chong LA, Khoo EJ, Kamar AA, Tan HS
    Asian Bioeth Rev, 2020 Aug 13.
    PMID: 32837563 DOI: 10.1007/s41649-020-00142-2
    Malaysia had its first four patients with COVID-19 on 25 January 2020. In the same week, the World Health Organization declared it as a public health emergency of international concern. The pandemic has since challenged the ethics and practice of medicine. There is palpable tension from the conflict of interest between public health initiatives and individual's rights. Ensuring equitable care and distribution of health resources for patients with and without COVID-19 is a recurring ethical challenge for clinicians. Palliative care aims to mitigate suffering caused by a life-limiting illness, and this crisis has led to the awareness and urgency to ensure it reaches all who needs it. We share here the palliative care perspectives and ethical challenges during the COVID-19 pandemic in Malaysia.
  11. Ariffin H, Daud SS, Mohamed Z, Ibrahim K, Lee TF, Chong LA
    Singapore Med J, 2007 Apr;48(4):333-7.
    PMID: 17384881
    The follow-up of chimerism status after allogeneic haematopoietic stem cell transplantation (HSCT) is essential to predict successful engraftment to assess the development of graft-versus-host disease, graft rejection and disease relapse. Analysis of short tandem repeats (STR) via polymerase chain reaction is frequently used for chimerism determination. However, most commercially-available kits have been designed for forensic purposes and may not be optimal for chimerism analysis. The present study aims to identify suitable STR markers for patient-donor pairs of predominantly Malay and Chinese ethnicity using two commercially-available forensic kits.
  12. Daud SS, Ibrahim K, Choong SS, Vengidasan L, Chong LA, Ariffin H
    Anal Biochem, 2010 Feb 15;397(2):181-5.
    PMID: 19822126 DOI: 10.1016/j.ab.2009.10.008
    Following hematopoietic stem cell transplantation (HSCT), it is important to determine whether engraftment is successful and to track the dynamic changes of the graft. Tandem repeats such as minisatellites and microsatellites are currently the most established markers for chimerism application. We have developed a reliable method to quantitatively evaluate engraftment status in post-allogeneic HSCT patients using variable number of tandem repeat (VNTR) markers and "lab-on-a-chip" microfluidic electrophoresis technology. Following identification of an informative marker by conventional polymerase chain reaction (PCR), donor chimerism percentage was calculated based on a standard curve generated from artificially mixed patient-donor DNA-specific alleles in serial dilutions. All PCR products were mixed with commercial gel dye and loaded into Agilent DNA 1000 microfluidic LabChips for DNA sizing and quantitation. In 44 patients, separation of pretransplant and donor DNA fragments was resolved clearly and accomplished rapidly within 30min. Chimerism analysis using this platform is able to detect an amount as low as 6.3% donor DNA with acceptable coefficient of variation values. We also demonstrated concordant chimerism analysis findings using both microchip tandem repeats and real-time PCR quantitation of insertion-deletion polymorphisms. This microchip platform obviates the need for fluorescently labeled primers or any post-PCR sample manipulation. Quantitative monitoring of post-HSCT chimerism status using microfluidic electrophoresis is a useful tool for both large- and small-scale post-HSCT chimerism centers.
  13. Ariffin H, Garcia JC, Daud SS, Ibrahim K, Aizah N, Ong GB, et al.
    Pediatr Blood Cancer, 2009 Jul;53(1):108-11.
    PMID: 19260099 DOI: 10.1002/pbc.21983
    Children with Down syndrome and acute megakaryoblastic leukemia (DS-AMKL) have been shown to have increased sensitivity to cytarabine based chemotherapy. The excellent prognosis in patients with DS-AMKL may be due to mutations in the GATA1 gene leading to reduced expression of the enzyme cytidine deaminase. This leads to a decreased ability to convert cytarabine into its inactive metabolite, resulting in high intracellular concentration of this cytotoxic agent. We report two cases of DS-AMKL with GATA1 mutations who had poor outcome. These patients had high expression levels of cytidine deaminase mRNA transcripts. We speculate that other factors can affect overall outcome in patients with DS-AMKL irrespective of the presence of GATA1 mutations.
  14. Lee WS, Chong LA, Begum S, Abdullah WA, Koh MT, Lim EJ
    J Pediatr Hematol Oncol, 2001 May;23(4):244-6.
    PMID: 11846304
    We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor V deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.
  15. Nazree NE, Mohamed Z, Reynolds GP, Mohd Zain S, Masiran R, Sidi H, et al.
    Asia Pac Psychiatry, 2016 Dec;8(4):260-268.
    PMID: 27787964 DOI: 10.1111/appy.12210
    INTRODUCTION: The occurrence of female sexual dysfunction (FSD) in patients with major depressive disorder (MDD) receiving selective serotonin reuptake inhibitors (SSRIs) treatment gives negative impacts on patients' quality of life and causes treatment discontinuation. We aimed to investigate whether genetic polymorphism of identified candidate gene is associated with FSD in our study population.

    METHODS: This is a cross-sectional study. A total of 95 female patients with MDD who met the criteria of the study were recruited and were specifically assessed on the sexual function by trained psychiatrists. Patients' DNA was genotyped for BDNF Val66Met polymorphism using real-time polymerase chain reaction.

    RESULTS: The prevalence of FSD in this study is 31.6%. In the FSD group, patients with problematic marriage were significantly more frequent compared with patients who did not have problematic marriage (P = 0.009). Significant association was detected in the lubrication domain with BDNF Val66Met polymorphism (P = 0.030) using additive genetic model, with even stronger association when using the recessive model (P = 0.013).

    DISCUSSION: This study suggested that there was no significant association between BDNF Val66Met with FSD. However, this polymorphism is significantly associated with lubrication disorder in patients treated with SSRIs.

  16. Chong LA, Khalid F, Khoo TB, Teh SH, Kuan GL, Aina Mariana AM, et al.
    Med J Malaysia, 2017 02;72(1):32-36.
    PMID: 28255137 MyJurnal
    INTRODUCTION: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services.

    METHODS: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out.

    RESULTS: There were 315 patients analysed, 90 (28.6%) and 46 (14.6%) were neonates and adolescents respectively. The main ICD-10 diagnostic categories for all patients were identified to be 'Congenital malformations, deformations and chromosomal abnormalities' 117 (37.1%), 'Diseases of nervous system' 76 (24.1%) and 'Neoplasms' 60 (19.0%). At referral 156 (50%) patients had holistic needs assessments. Patients with 'Diseases of nervous system' were assessed to have significantly more physical needs than the other two diagnostic categories. Majority of patients who knew of their diagnosis and prognosis were those with malignancy. Over a fifth of referrals were at their terminal admission. Of 144 who died, 111 (77.1%) had advanced care plans. There was bereavement follow-up in 98 (68.1%) patients.

    CONCLUSION: Patients referred for palliative care have varied diagnoses and needs. To ensure all paediatricians are competent to deliver quality care to all children, further education and training initiatives is imperative.

  17. Yeoh AE, Ariffin H, Chai EL, Kwok CS, Chan YH, Ponnudurai K, et al.
    J Clin Oncol, 2012 Jul 1;30(19):2384-92.
    PMID: 22614971 DOI: 10.1200/JCO.2011.40.5936
    PURPOSE: To improve treatment outcome for childhood acute lymphoblastic leukemia (ALL), we designed the Malaysia-Singapore ALL 2003 study with treatment stratification based on presenting clinical and genetic features and minimal residual disease (MRD) levels measured by polymerase chain reaction targeting a single antigen-receptor gene rearrangement.
    PATIENTS AND METHODS: Five hundred fifty-six patients received risk-adapted therapy with a modified Berlin-Frankfurt-Münster-ALL treatment. High-risk ALL was defined by MRD ≥ 1 × 10(-3) at week 12 and/or poor prednisolone response, BCR-ABL1, MLL gene rearrangements, hypodiploid less than 45 chromosomes, or induction failure; standard-risk ALL was defined by MRD ≤ 1 × 10(-4) at weeks 5 and 12 and no extramedullary involvement or high-risk features. Intermediate-risk ALL included all remaining patients.
    RESULTS: Patients who lacked high-risk presenting features (85.7%) received remission induction therapy with dexamethasone, vincristine, and asparaginase, without anthracyclines. Six-year event-free survival (EFS) was 80.6% ± 3.5%; overall survival was 88.4% ± 3.1%. Standard-risk patients (n = 172; 31%) received significantly deintensified subsequent therapy without compromising EFS (93.2% ± 4.1%). High-risk patients (n = 101; 18%) had the worst EFS (51.8% ± 10%); EFS was 83.6% ± 4.9% in intermediate-risk patients (n = 283; 51%).
    CONCLUSION: Our results demonstrate significant progress over previous trials in the region. Three-drug remission-induction therapy combined with MRD-based risk stratification to identify poor responders is an effective strategy for childhood ALL.
  18. Hng SY, Thinakaran AS, Ooi CJ, Eg KP, Thong MK, Tae SK, et al.
    Singapore Med J, 2023 Sep 19.
    PMID: 37870036 DOI: 10.4103/singaporemedj.SMJ-2022-093
    INTRODUCTION: : Asian children with cystic fibrosis (CF) managed in Malaysia have significant morbidity with limited access to life-sustaining treatments. We determined the morbidity and treatment cost of CF in a resource-limited country.

    METHODS: This cross-sectional study included all children diagnosed with CF in our centre. Data on clinical presentation, genetic mutation, serial spirometry results and complications were collected. Out-of-pocket (OOP) and healthcare costs over 1 year were retrieved for patients who were alive. Cohen's d and odds ratio (OR) were used to determine the effect size.

    RESULTS: Twenty-four patients were diagnosed with CF. Five patients died at a median (range) age of 18 (0.3-22) years. F508deletion (c. 1521_1523delCTT) was found in 20% of the alleles, while 89% of the variants were detected in nine patients. Body mass index (BMI) Z score was >-1.96 in 70.6% of patients. Two thirds (68%) were colonised with Pseudomonas aeruginosa, and this was associated with lower weight (P = 0.009) and BMI (P = 0.02) Z scores. Only 18% had FEV1 Z scores >-1.96. Early symptom onset (d = 0.74), delayed diagnosis (d = 2.07), a low FEF25-75 Z score (d = 0.82) and a high sweat conductance (d = 1.19) were associated with death. Inpatient cost was mainly from diagnostic tests, while medications contributed to half of the outpatient cost.

    Healthcare utilisation cost was catastrophic, amounting to 20% of the total income.

    CONCLUSION: Asian children with CF suffer significant complications such as low weight, low lung function and shortened lifespan. P. aeruginosa colonisation was frequent and associated with poor growth. Healthcare cost to parents was catastrophic.

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