Displaying publications 21 - 27 of 27 in total

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  1. Lee WS, Boey CC, Goh AY, Chang KW, Iyngkaran N
    Singapore Med J, 1998 Sep;39(9):418-21.
    PMID: 9885723
    This is a report of 3 Chinese children with intestinal lymphangiectasia in Malaysia. Two children responded to a low fat diet and medium-chain triglyceride supplement. The third child has recurrent chylous ascites. None of the children has recurrent infections despite low CD4+ cells and low levels of IgG and IgA. Intestinal lymphangiectasia is a rare congenital disorder of the mesenteric lymphatic that leads to the obstruction of the lymphatics of the intestine and protein losing enteropathy. Restriction of dietary fat intake will usually result in remission. Recurrent chylous ascites is a problem and management can be difficult.
  2. Thong MK, Boey CC, Sheng JS, Ushikai M, Kobayashi K
    Singapore Med J, 2010 Jan;51(1):e12-4.
    PMID: 20200759
    We report two Malaysian siblings with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). The younger sibling, a six-month-old Chinese girl, presented with prolonged neonatal jaundice, and was investigated for biliary atresia. Urine metabolic screen showed the presence of urinary-reducing sugars, and she was treated with a lactose-free formula. NICCD was suspected based on the clinical history, examination and presence of urinary citrulline. Mutation study of the SLC25A13 gene showed the compound heterozygotes, 851del4 and IVS16ins3kb, which confirmed the diagnosis of NICCD in the patient and her three-year-old female sibling, who also had unexplained neonatal cholestasis. Long-term dietary advice, medical surveillance and genetic counselling were provided to the family. The diagnosis of NICCD should be considered in infants with unexplained prolonged jaundice. DNA-based genetic testing of the SLC25A13 gene may be performed to confirm the diagnosis retrospectively. An awareness of this condition may help in early diagnosis using appropriate metabolic and biochemical investigations, thus avoiding invasive investigations in infants with neonatal cholestasis caused by NICCD.
  3. Lee WS, Zainuddin H, Boey CC, Chai PF
    World J Gastroenterol, 2013 Dec 21;19(47):9077-83.
    PMID: 24379634 DOI: 10.3748/wjg.v19.i47.9077
    AIM: To determine the predictability of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and American Society for Gastrointestinal Endoscopy (ASGE) guideline with regard to appropriate endoscopic practice in children, positive endoscopic findings and contributive yield in clinical practice.

    METHODS: This was a descriptive, retrospective analysis, conducted at the Department of Paediatrics, University Malaya Medical Centre, Malaysia. All children who had esophagogastroduodenoscopy (EGD) and colonoscopy from January 2008 to June 2011 were included. An endoscopy was considered appropriate when its indication complied with the NASPGHAN and ASGE guideline. All endoscopic findings were classified as either positive (presence of any endoscopic or histologic abnormality) or negative (no or minor abnormality, normal histology); effecting a positive contributive (a change in therapeutic decisions or prognostic consequences) or non-contributive yield (no therapeutic or prognostic consequences).

    RESULTS: Overall, 76% of the 345 procedures (231 EGD alone, 26 colonoscopy alone, 44 combined EGD and colonoscopy) performed in 301 children (median age 7.0 years, range 3 months to 18 years) had a positive endoscopic finding. Based on the NASPGHAN and ASGE guideline, 99.7% of the procedures performed were considered as appropriate. The only inappropriate procedure (0.3%) was in a child who had EGD for assessment of the healing of gastric ulcer following therapy in the absence of any symptoms. The overall positive contributive yield for a change in diagnosis and/or management was 44%. The presence of a positive endoscopic finding was more likely to effect a change in the therapeutic plan than an alteration of the initial diagnosis. A total of 20 (5.8%) adverse events were noted, most were minor and none was fatal.

    CONCLUSION: The NASPGHAN and ASGE guideline is more likely to predict a positive endoscopic finding but is less sensitive to effect a change in the initial clinical diagnosis or the subsequent therapeutic plan.

  4. Lee WS, Lum SH, Lim CB, Chong SY, Khoh KM, Ng RT, et al.
    Hepatol Int, 2015 Apr;9(2):292-302.
    PMID: 25788179 DOI: 10.1007/s12072-014-9558-0
    BACKGROUND: Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.

    METHODS: Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.

    RESULTS: At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].

    CONCLUSIONS: Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.
  5. Myint K, Jacobs K, Myint AM, Lam SK, Lim YA, Boey CC, et al.
    Front Immunol, 2021;12:702301.
    PMID: 34539633 DOI: 10.3389/fimmu.2021.702301
    Recurrent abdominal pain (RAP) is a common medically unexplained symptom among children worldwide. However, the biological mechanisms behind the development of functional and behavioral symptoms and changes in blood markers have not been well explored. This study aimed to assess changes in the concentrations of inflammatory markers, including cytokines and tryptophan catabolites, in the serum of children with RAP compared to those with subclinical infections. Children with RAP but without organic diseases were included, and those with asymptomatic intestinal parasitic infections were used as a subclinical infection cohort. Blood samples were collected and used to measure the cytokine profile using Multiplex Immunoassay and tryptophan catabolites using high performance liquid chromatography. Children with RAP showed significantly higher concentrations of serum tumor necrotic factor-α, p<0.05, but lower concentrations of IL-10, p<0.001, IL-6, p<0.001 and brain-derived neurotrophic factors (BDNF) p<0.01. In addition, a significant increase in the metabolite of the kynurenine pathway, 3-hydroxyanthranilic acid (3-HAA) p<0.01, a significant decrease in the concentrations of anthranilic acid (AA) p<0.001, together with an increased ratio of serum 3-HAA to AA (3-HAA/AA) p<0.001, was found in this cohort. These findings indicate the significant activation of the immune system and presence of inflammation in children with RAP than those with subclinical parasitic infections. Moreover, children with RAP tested with the Strengths and Difficulties Questionnaire (SDQ), displayed high psychological problems though these SDQ scores were not statistically associated with measured cytokines and kynurenine metabolites. We however could hypothesize that the pro-inflammatory state together with concomitant low concentrations of BDNF in those children with RAP could play a role in psychological stress and experiencing medically unexplained symptoms.
  6. Ooi CJ, Makharia GK, Hilmi I, Gibson PR, Fock KM, Ahuja V, et al.
    J Gastroenterol Hepatol, 2016 Jan;31(1):45-55.
    PMID: 25819140 DOI: 10.1111/jgh.12956
    Inflammatory bowel disease (IBD) was previously thought to be rare in Asia, but emerging data indicate rising incidence and prevalence of IBD in the region. The Asia Pacific Working Group on Inflammatory Bowel Disease was established in Cebu, Philippines, at the Asia Pacific Digestive Week conference in 2006 under the auspices of the Asian Pacific Association of Gastroenterology with the goal of developing best management practices, coordinating research, and raising awareness of IBD in the region. The consensus group previously published recommendations for the diagnosis and management of ulcerative colitis with specific relevance to the Asia-Pacific region. The present consensus statements were developed following a similar process to address the epidemiology, diagnosis, and management of Crohn's disease. The goals of these statements are to pool the pertinent literature specifically highlighting relevant data and conditions in the Asia-Pacific region relating to the economy, health systems, background infectious diseases, differential diagnoses, and treatment availability. It does not intend to be all comprehensive and future revisions are likely to be required in this ever-changing field.
  7. Ooi CJ, Makharia GK, Hilmi I, Gibson PR, Fock KM, Ahuja V, et al.
    J Gastroenterol Hepatol, 2016 Jan;31(1):56-68.
    PMID: 25819311 DOI: 10.1111/jgh.12958
    The Asia Pacific Working Group on Inflammatory Bowel Disease was established in Cebu, Philippines, at the Asia Pacific Digestive Week conference in 2006 under the auspices of the Asian Pacific Association of Gastroenterology (APAGE) with the goal of developing best management practices, coordinating research and raising awareness of IBD in the region. The consensus group previously published recommendations for the diagnosis and management of ulcerative colitis (UC) with specific relevance to the Asia-Pacific region. The present consensus statements were developed following a similar process to address the epidemiology, diagnosis and management of Crohn's disease (CD). The goals of these statements are to pool the pertinent literature specifically highlighting relevant data and conditions in the Asia-Pacific region relating to the economy, health systems, background infectious diseases, differential diagnoses and treatment availability. It does not intend to be all-comprehensive and future revisions are likely to be required in this ever-changing field.
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