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Demographics and outcome of patients with congenital haemophilia in Sarawak, Malaysia

Tang ASO, Wong QY, Tan YY, Chieng CH, Ko CT, Ong GB, et al.

Med J Malaysia, 2021 Jan;76(1):51-55.
PMID: 33510109

INTRODUCTION: Sarawak has a population that is geographically and characteristically widely varied. This study aimed to determine the demographic profile of patients in Sarawak, Malaysia. Materials and Methods - A cross-sectional study was conducted in 2019 at four major haemophilia treatment centres in Kuching, Sibu, Bintulu and Miri Hospitals, Sarawak. Demographic and clinical data were collected with consents from patients.
RESULTS AND DISCUSSION: Ninety-six haemophilia patients were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%) haemophilia B(HB). Severe haemophilia patients were noted in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3% of the HA and 52.9% of the HB population had no identifiable family history of haemophilia. Two-thirds of the patients with severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79 (11.4%) of the HA population [3/79 (3.8%) high responders]. The median inhibitor titre was not significantly different between the different treatment groups - on demand versus prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value 0.297, Mann-Whitney test). None of the patients developed inhibitory alloantibodies to factor IX. Four HA patients (5.1%) underwent immune tolerance induction where one case had a successful outcome. Three severe HA patients received emicizumab prophylaxis and showed remarkable reduction in bleeding events with no thromboembolic events being reported. One female moderate HA patient received PEGylated recombinant anti-haemophilic factor. Eleven patients underwent radiosynovectomy. One mild HB patient succumbed to traumatic intracranial bleeding. Our data reported a prevalence (per 100,000 males) of 5.40 cases for all severities of HA, 2.46 cases for severe HA; 1.16 cases for all severities of HB, and 0.75 cases for severe HB. The overall incidence of HA and HB was 1 in 11,500 and 1 in 46,000, respectively.
CONCLUSION: This study outlines the Sarawakian haemophilia landscape and offers objective standards for forward planning. Shared responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.
Molecular Characterization of Avian Infectious Bronchitis Virus Isolated in Malaysia during 2014-2016

Leow BL, Syamsiah Aini S, Faizul Fikri MY, Muhammad Redzwan S, Khoo CK, Ong GH, et al.

Trop Biomed, 2018 Dec 01;35(4):1092-1106.
PMID: 33601856

Avian Infectious Bronchitis (IB) is a highly contagious disease which can cause huge economic losses to the poultry industry. Forty five IB viruses (IBV) were isolated from poultry in Malaysia during 2014-2016. Phylogenetic analysis of the spike glycoprotein 1 (S1) gene revealed that all isolates were clustered into five distinct groups. The predominant type of IBV isolated was QX strains (47%), second was 4/91 type (27%), followed by Malaysian strain MH5365/95 (13%), Massachusetts type (11%) and finally Taiwanese strains (2%). Four types of S1 protein cleavage recognition motifs were found among the isolates which includes HRRRR, RRSRR, RRFRR and RRVRR. To our knowledge, this is the first report describing the motif RRVRR and are unique to Malaysian strains. Six IBVs were grouped in Malaysian MH5365/95 strains. Among these, one isolate was different from others where it only shared 82% identity with MH5365/95 and to others. It formed its own branch in the Malaysian cluster suggesting it may be a variant unique to Malaysia. Alignment analysis of the S1 amino acid sequences indicated that point mutations, insertions and deletions contribute to the divergence of IB variants. This study indicated at least five groups of IBV are circulating in Malaysia with most of the isolates belonged to QX strains. As new IBV variants continue to emerge, further study need to be carried out to determine whether the current available vaccine is able to give protection against the circulating virus.
Paediatric middle aortic syndrome with endovascular treatment in East Malaysia

Hong Ong G, Benjamin Leong DK

Med J Malaysia, 2018 02;73(1):57-59.
PMID: 29531207 MyJurnal

Middle-aortic syndrome is a surgically curable cause of childhood hypertension. Open surgery is traditionally offered but with the advance of medical technology, endovascular approached is available in many country. Failure to control BP in open surgery is as low as 4.1% compares to 13% in endovascular approaches. However, mortality is 4% in open surgery almost 2 times higher than 2.3% in endovascular approach. This article presents a case of 10 years old child treated successfully without complication with endovascular balloon dilatation, as a first case of such disease in East Malaysia.
Fulltext Effects of immunonutrition on biomarkers in traumatic brain injury patients in Malaysia: a prospective randomized controlled trial

Rai VRH, Phang LF, Sia SF, Amir A, Veerakumaran JS, Kassim MKA, et al.

BMC Anesthesiol, 2017 06 15;17(1):81.
PMID: 28619005 DOI: 10.1186/s12871-017-0369-4

BACKGROUND: Head injury is one of the top three diagnosis leading to intensive care unit (ICU) admission in Malaysia. There has been growing interest in using immunonutrition as a mode of modulating the inflammatory response to injury or infection with the aim of improving clinical outcome. The aim of the present study was to evaluate the effect of an immunonutrition on biomarkers (IL-6, glutathione, CRP, total protein and albumin) in traumatic brain injury patients.
METHODS: Thirty six patients with head injury admitted to neurosurgical ICU in University Malaya Medical Centre were recruited for this study, over a 6-month period from July 2014 to January 2015. Patients were randomized to receive either an immunonutrition (Group A) or a standard (Group B) enteral feed. Levels of biomarkers were measured at day 1, 5 and 7 of enteral feeding.
RESULTS: Patients in Group A showed significant reduction of IL-6 at day 5 (p
Fulltext Stimulation of β-adrenergic receptors plays a protective role via increased expression of RAF-1 and PDX-1 in hyperglycemic rat pancreatic islet (RIN-m5F) cells

Safi SZ, Qvist R, Ong G, Karimian H, Imran M, Shah I

Arch Med Sci, 2017 Mar 01;13(2):470-480.
PMID: 28261303 DOI: 10.5114/aoms.2016.64131

INTRODUCTION: It is a widely held view that a progressive reduction of beta-cell mass occurs in the progression of diabetes. RAF-1 kinase and pancreas duodenal homeobox 1 (PDX-1) are major factors that promote survival of cells and maintain normal insulin functions. In this study we investigated the effect of a β-adrenergic receptor agonist and antagonist on RAF-1 and PDX-1, and their respective effects on apoptosis and insulin release in RIN-m5F cells.
MATERIAL AND METHODS: RIN-m5F cells were cultured in normal (5 mM) and high (25 mM) glucose to mimic diabetic conditions, followed by treatment with 5 µM, 10 µM and 20 µM of isoproterenol and isoproterenol + propranolol for 6, 12 and 24 h. Western blotting and reverse transcription analysis were performed to examine the expression of RAF-1 and PDX-1. Annexin-V-FITC and terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) assays were used to investigate apoptosis. ELISA was used to measure insulin levels. Reverse transcription polymerase chain reaction was conducted to investigate the expression of genes.
RESULTS: Stimulation of β-adrenergic receptors with isoproterenol significantly induced RAF-1 and PDX-1 genes in a concentration-dependent and time-independent manner. Changes were significant both at protein and mRNA levels. Up-regulation of RAF-1 and PDX-1 was accompanied by improved insulin levels and reduced apoptosis. Concentrations of 10 µM and 20 µM for 12 and 24 h were more effective in achieving significant differences in the experimental and control groups. Propranolol reversed the effect of isoproterenol mostly at maximum concentrations and time periods.
CONCLUSIONS: A positive effect of a β-adrenergic agonist on RAF-1 and PDX-1, reduction in β-cell apoptosis and improved insulin contents can help to understand the pathogenesis of diabetes and to develop novel approaches for the β-cell dysfunction in diabetes.
Clinical spectrum of children receiving palliative care in Malaysian Hospitals

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.
Frequent occurrence of gastric cancer in Asian kindreds with Li-Fraumeni syndrome

Ariffin H, Chan AS, Oh L, Abd-Ghafar S, Ong GB, Mohamed M, et al.

Clin Genet, 2015 Nov;88(5):450-5.
PMID: 25318593 DOI: 10.1111/cge.12525

Type of cancer and age of onset in individuals with inherited aberrations in the tumour suppressor gene TP53 are variable, possibly influenced by genetic modifiers and different environmental exposure. Since 2009, the modified Chompret criteria (MCC) have been used to identify individuals for TP53 mutation screening. Using the TP53 mutation database maintained by the International Agency for Research on Cancer (IARC), we investigated if the MCC, mainly developed for a Caucasian population, was also applicable in Asia. We identified several differences in Asian families compared with similar Caucasian cohorts, suggesting that identification and management of Li-Fraumeni syndrome in Asia do not completely mirror that of North America and Western Europe. Early gastric cancer (<40 years) may be considered a new addition to the MCC especially for Asian families.
Caffeic acid phenethyl ester (CAPE): scavenger of peroxynitrite in vitro and in sepsis models

Kassim M, Mansor M, Kamalden TA, Shariffuddin II, Hasan MS, Ong G, et al.

Shock, 2014 Aug;42(2):154-60.
PMID: 24667629 DOI: 10.1097/SHK.0000000000000179

Excessive free radical production by immune cells has been linked to cell death and tissue injury during sepsis. Peroxynitrite is a short-lived oxidant and a potent inducer of cell death that has been identified in several pathological conditions. Caffeic acid phenethyl ester (CAPE) is an active component of honeybee products and exhibits antioxidant, anti-inflammatory, and immunomodulatory activities. The present study examined the ability of CAPE to scavenge peroxynitrite in RAW 264.7 murine macrophages stimulated with lipopolysaccharide/interferon-γ that was used as an in vitro model. Conversion of 123-dihydrorhodamine to its oxidation product 123-rhodamine was used to measure peroxynitrite production. Two mouse models of sepsis (endotoxemia and cecal ligation and puncture) were used as in vivo models. The level of serum 3-nitrotyrosine was used as an in vivo marker of peroxynitrite. The results demonstrated that CAPE significantly improved the viability of lipopolysaccharide/interferon-γ-treated RAW 264.7 cells and significantly inhibited nitric oxide production, with effects similar to those observed with an inhibitor of inducible nitric oxide synthase (1400W). In addition, CAPE exclusively inhibited the synthesis of peroxynitrite from the artificial substrate SIN-1 and directly prevented the peroxynitrite-mediated conversion of dihydrorhodamine-123 to its fluorescent oxidation product rhodamine-123. In both sepsis models, CAPE inhibited cellular peroxynitrite synthesis, as evidenced by the absence of serum 3-nitrotyrosine, an in vivo marker of peroxynitrite. Thus, CAPE attenuates the inflammatory responses that lead to cell damage and, potentially, cell death through suppression of the production of cytotoxic molecules such as nitric oxide and peroxynitrite. These observations provide evidence of the therapeutic potential of CAPE treatment for a wide range of inflammatory disorders.
Fulltext Large volume osteosarcomas of the femur treated with total femoral replacement

Pan K, Chan W, Shanmugam P, Ong G, Kamaruddin F, Tan S

Malays Orthop J, 2014 Mar;8(1):32-6.
PMID: 25347294 MyJurnal DOI: 10.5704/MOJ.1403.015

Patients with extensive malignancies involving the femur often require total femoral replacement when their limbs can be salvaged. Reported series are small and involve heterogeneity of tumours. We present nine patients with osteosarcomas of the femur treated at our institution between 2003 and 2010 with a mean follow-up of 27 (6 to 56) months. Their ages ranged from 9 to 17 (mean 14 years). They had large volume tumours (mean 911 cm3) and presented late with a mean of 5.5 months from the onset of symptoms to definitive treatment. All patients underwent resection and total femur replacement. Six patients have died and two are alive with good function at the time of this report. One was lost to follow-up. These patients require a high level of treatment care and have a guarded prognosis.
Fulltext Myocardial injury after noncardiac surgery: a large, international, prospective cohort study establishing diagnostic criteria, characteristics, predictors, and 30-day outcomes

Botto F, Alonso-Coello P, Chan MT, Villar JC, Xavier D, Srinathan S, et al.

Anesthesiology, 2014 Mar;120(3):564-78.
PMID: 24534856 DOI: 10.1097/ALN.0000000000000113

BACKGROUND: Myocardial injury after noncardiac surgery (MINS) was defined as prognostically relevant myocardial injury due to ischemia that occurs during or within 30 days after noncardiac surgery. The study's four objectives were to determine the diagnostic criteria, characteristics, predictors, and 30-day outcomes of MINS.
METHODS: In this international, prospective cohort study of 15,065 patients aged 45 yr or older who underwent in-patient noncardiac surgery, troponin T was measured during the first 3 postoperative days. Patients with a troponin T level of 0.04 ng/ml or greater (elevated "abnormal" laboratory threshold) were assessed for ischemic features (i.e., ischemic symptoms and electrocardiography findings). Patients adjudicated as having a nonischemic troponin elevation (e.g., sepsis) were excluded. To establish diagnostic criteria for MINS, the authors used Cox regression analyses in which the dependent variable was 30-day mortality (260 deaths) and independent variables included preoperative variables, perioperative complications, and potential MINS diagnostic criteria.
RESULTS: An elevated troponin after noncardiac surgery, irrespective of the presence of an ischemic feature, independently predicted 30-day mortality. Therefore, the authors' diagnostic criterion for MINS was a peak troponin T level of 0.03 ng/ml or greater judged due to myocardial ischemia. MINS was an independent predictor of 30-day mortality (adjusted hazard ratio, 3.87; 95% CI, 2.96-5.08) and had the highest population-attributable risk (34.0%, 95% CI, 26.6-41.5) of the perioperative complications. Twelve hundred patients (8.0%) suffered MINS, and 58.2% of these patients would not have fulfilled the universal definition of myocardial infarction. Only 15.8% of patients with MINS experienced an ischemic symptom.
CONCLUSION: Among adults undergoing noncardiac surgery, MINS is common and associated with substantial mortality.
An investigation of arsenic contamination in Peninsular Malaysia based on Centella asiatica and soil samples

Ong GH, Yap CK, Maziah M, Suhaimi H, Tan SG

Environ Monit Assess, 2013 Apr;185(4):3243-54.
PMID: 22821327 DOI: 10.1007/s10661-012-2787-6

The first objective of this study was to provide data of arsenic (As) levels in Peninsular Malaysia based on soil samples and accumulation of As in Centella asiatica collected from 12 sampling sites in Peninsular Malaysia. The second objective was to assess the accumulation of As in transplanted C. asiatica between control and semi-polluted or polluted sites. Four sites were selected which were UPM (clean site), Balakong (semi-polluted site), Seri Kembangan (semi-polluted site) and Juru (polluted site). The As concentrations of plant and soil samples were determined by Instrumental Neutron Activation Analysis. The As levels ranged from 9.38 to 57.05 μg/g dw in soils, 0.21 to 4.33 μg/g dw in leaves, 0.18 to 1.83 μg/g dw in stems and 1.32-20.76 μg/g dw in roots. All sampling sites had As levels exceeding the CCME guideline (12 μg/g dw) except for Kelantan, P. Pauh, and Senawang with P. Klang having the highest As in soil (57.05 μg/g dw). In C. asiatica, As accumulation was highest in roots followed by leaves and stems. When the As level in soils were higher, the uptake of As in plants would also be increased. After the transplantation of plants to semi-polluted and polluted sites for 3 weeks, all concentration factors were greater than 50 % of the initial As level. The elimination factor was around 39 % when the plants were transplanted back to the clean sites for 3 weeks. The findings of the present study indicated that the leaves, stems and roots of C. asiatica are ideal biomonitors of As contamination. The present data results the most comprehensive data obtained on As levels in Malaysia.
Gelam honey inhibits lipopolysaccharide-induced endotoxemia in rats through the induction of heme oxygenase-1 and the inhibition of cytokines, nitric oxide, and high-mobility group protein B1

Kassim M, Yusoff KM, Ong G, Sekaran S, Yusof MY, Mansor M

Fitoterapia, 2012 Sep;83(6):1054-9.
PMID: 22626749 DOI: 10.1016/j.fitote.2012.05.008

Malaysian Gelam honey has anti-inflammatory and antibacterial properties, a high antioxidant capacity, and free radical-scavenging activity. Lipopolysaccharide (LPS) stimulates immune cells to sequentially release early pro- and anti-inflammatory cytokines and induces the synthesis of several related enzymes. The aim of this study was to investigate the effect of the intravenous injection of honey in rats with LPS-induced endotoxemia. The results showed that after 4h of treatment, honey reduced cytokine (tumor necrosis factor-α, interleukins 1β, and 10) and NO levels and increased heme oxygenase-1 levels. After 24h, a decrease in cytokines and NO and an increase in HO-1 were seen in all groups, whereas a reduction in HMGB1 occurred only in the honey-treated groups. These results support the further examination of honey as a natural compound for the treatment of a wide range of inflammatory diseases.
Fulltext Determining the prevalence of intestinal parasites in three Orang Asli (Aborigines) communities in Perak, Malaysia

Sinniah B, Sabaridah I, Soe MM, Sabitha P, Awang IP, Ong GP, et al.

Trop Biomed, 2012 Jun;29(2):200-6.
PMID: 22735840 MyJurnal

This study was conducted to determine the prevalence of intestinal parasites among children and adult Orang Aslis (Aborigines) from different locations in Perak. Faecal samples were collected and analyzed using the direct smear and formal ether sedimentation technique. Some of the faecal samples were stained using the Modified Acid fast stain for Cryptosporidium. Nail clippings of the respondents and the soil around their habitat were also analyzed. Of the 77 stool samples examined, 39 (50.6%) were positive for at least one intestinal parasite. The most common parasite detected was Trichuris trichiura (39.0%) followed by Ascaris lumbricoides (26.9%), Entamoeba coli (5.2%), Giardia lamblia (5.2%), Blastocystis hominis (3.9%), hookworm (3.9%), Entamoeba histolytica (1.3%), Iodamoeba butschlii (1.3%) and Cryptosporidium sp. (1.3%) respectively. Some respondents had single parasites (24.7%), some with two parasites (18.2%). Some with three parasites (6.5%) and one had four parasites species (1.3%). The parasites were slightly more common in females (54.7%) than males ((41.7%). The parasites were more common in the 13-20 year age group (90.9%) followed by 1-12 years (69.6%), 21-40 year age group (34.8%) and least in the 41-60 year age group (27.8%). Nail examinations of the respondents did not show any evidence of parasites. One had a mite, three had pollen grains and one had yeast cells isolated from the finger nails. Soil samples taken around their houses showed only one sample with a nematode ova and one with oocyst which was of a non human origin.
Fulltext Non-bacterial chronic recurrent osteomyelitis of the clavicle

Pan K, Chan W, Ong G, Zulqarnaen M, Norlida D

Malays Orthop J, 2012 Mar;6(1):57-60.
PMID: 25279046 MyJurnal DOI: 10.5704/MOJ.1203.005

This report details the case of a 12-year-old girl with a painful, progressive swelling of the medial portion of the clavicle with no history of trauma or other constitutional symptoms. All laboratory investigations were normal except for an elevated erythrocyte sedimentation rate (ESR). Initial plain radiographs showed a destructive lesion with magnetic resonance imaging showing features of malignancy. Biopsies revealed osteomyelitis, but with negative bacterial cultures and no evidence of malignancy. Treatment with antibiotics did not result in a favourable response. Over time, the swelling increased in size with episodic exacerbations of pain. Follow-up radiographs showed sclerosis and hyperostosis. After five years, this was recognized as non-bacterial chronic recurrent osteomyelitis of the clavicle.
Fulltext Gelam honey scavenges peroxynitrite during the immune response

Kassim M, Mansor M, Suhaimi A, Ong G, Yusoff KM

Int J Mol Sci, 2012;13(9):12113-29.
PMID: 23109904 DOI: 10.3390/ijms130912113

Monocytes and macrophages are part of the first-line defense against bacterial, fungal, and viral infections during host immune responses; they express high levels of proinflammatory cytokines and cytotoxic molecules, including nitric oxide, reactive oxygen species, and their reaction product peroxynitrite. Peroxynitrite is a short-lived oxidant and a potent inducer of cell death. Honey, in addition to its well-known sweetening properties, is a natural antioxidant that has been used since ancient times in traditional medicine. We examined the ability of Gelam honey, derived from the Gelam tree (Melaleuca spp.), to scavenge peroxynitrite during immune responses mounted in the murine macrophage cell line RAW 264.7 when stimulated with lipopolysaccharide/interferon-γ (LPS/IFN-γ) and in LPS-treated rats. Gelam honey significantly improved the viability of LPS/IFN-γ-treated RAW 264.7 cells and inhibited nitric oxide production-similar to the effects observed with an inhibitor of inducible nitric oxide synthase (1400W). Furthermore, honey, but not 1400W, inhibited peroxynitrite production from the synthetic substrate 3-morpholinosydnonimine (SIN-1) and prevented the peroxynitrite-mediated conversion of dihydrorhodamine 123 to its fluorescent oxidation product rhodamine 123. Honey inhibited peroxynitrite synthesis in LPS-treated rats. Thus, honey may attenuate inflammatory responses that lead to cell damage and death, suggesting its therapeutic uses for several inflammatory disorders.
Fulltext Chikungunya virus-associated death in Malaysia

Sam IC, Kamarulzaman A, Ong GS, Veriah RS, Ponnampalavanar S, Chan YF, et al.

Trop Biomed, 2010 Aug;27(2):343-7.
PMID: 20962735

Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which causes fever, rash, and arthralgia. In the past, life-threatening complications were very rarely reported. However, during the recent worldwide outbreaks, there have been several reports of unusually severe complications and deaths. Malaysia is experiencing a nationwide outbreak of CHIKV, with over 10 000 patients affected since April 2008. We report the first case of culture-confirmed CHIKV-associated death in Malaysia, in a patient with fever, rash, acute exacerbation of pre-existing heart failure, rhabdomyolysis, and multiple organ failure. CHIKV infections may cause atypical, severe or fatal presentations.
Fulltext Mild autosomal recessive osteopetrosis: successful treatment with bone marrow transplant

Othman IS, Ibrahim H, Hii KC, Ong GB, Menon BS

Med J Malaysia, 2009 Dec;64(4):325-6.
PMID: 20954561 MyJurnal

We describe a 5 1/2 year old boy who was diagnosed with mild autosomal recessive osteopetrosis based on the presence of bony sclerosis, extramedullary haematopoeisis, leukoerythroblastosis and visual impairment who had an allogeneic bone marrow transplant from a matched sibling donor. Conditioning regime was busulphan 16 mg/kg and cyclophosphamide 200 mg/kg. Apart from transient hypercalcaemia, there were no major post transplant complications. Four years post transplant, the extramedullary haematopoeisis has resolved completely with normal blood counts. Apart from a fracture after a trivial fall two months after transplant, he has not suffered any fracture related limb deformities.
Fulltext Early presentation of right adrenal mass, hepatoblastoma and hepatic cavernous haemangioma in Beckwith-Wiedemann Syndrome

Teh SH, Ong GB

Med J Malaysia, 2007 Oct;62(4):345-6.
PMID: 18551945 MyJurnal

Beckwith-Wiedemann Syndrome (BWS) is associated with early development of embryonal tumours usually in the first four years of life. We describe a patient who presented with a right adrenal cyst in the first month of life and hepatoblastoma in the third month of life. A cavernous haemangioma was subsequently found in the resected tumour.
Impact of bispectral index monitoring on propofol administration in patients undergoing cardiopulmonary bypass

Chiu CL, Ong G, Majid AA

Anaesth Intensive Care, 2007 Jun;35(3):342-7.
PMID: 17591126

Propofol anaesthesia using target control infusion during cardiac surgery has become more popular recently. However, without depth of anaesthesia monitoring, the standard target concentration used may be higher than necessary to maintain adequate hypnosis during hypothermic cardiopulmonary bypass. The purpose of this study was to evaluate the effect of bispectral index monitoring on propofol administration during hypothermic cardiopulmonary bypass. After ethics committee approval and written informed consent, 20 New York Heart Association class I-III patients scheduled for elective cardiac surgery requiring hypothermic cardiopulmonary bypass were studied in this prospective randomised controlled trial. In group C, routine anaesthesia was practised, where patients received propofol at target concentration between 1.5 to 2.5 microg/ml during cardiopulmonary bypass. In group B, the target concentration was titrated to a bispectral index value of 40 to 50. Mean arterial pressure and bispectral index were recorded at various time intervals. The use of propofol, phenylephrine, sodium nitroprusside and adrenaline were recorded. The median propofol administration in group B was significantly less than that in group C (2.9 mg/kg/h compared to 6.0 mg/kg/h). The bispectral index value during bypass was significantly lower in group C than in group B, reflecting a deeper state of anaesthesia. There was no difference in the use of inotropes, vasoconstrictors or vasodilators. Bispectral index monitoring enables a 50% reduction in propofol administration at this standard dose during hypothermic cardiopulmonary bypass.
Fulltext Chromosome 13q deletion with Cornelia de Lange syndrome phenotype

Ngo CT, Alhady M, Tan AK, Norlasiah IS, Ong GB, Chua CN

Med J Malaysia, 2007 Mar;62(1):74-5.
PMID: 17682579

A 3-year-old girl with facial dysmorphic features suggestive of Cornelia de Lange syndrome was seen in the ophthalmology unit for a right leukocoria. The leukocoria was found to be caused by a large retinoblastoma and the right eye was enucleated. Chromosomal analysis revealed partial chromosome 13q deletion involving band 14 which is associated with a high risk of retinoblastoma. This case shows that patient with chromosome 13q deletion syndrome cannot be diagnosed based on dysmorphic features only. Chromosomal analysis is warranted in all infants with facial dysmorphism suggestive of Cornelia de Lange syndrome so that those with chromosome 13q deletion can be referred early for early detection of retinoblastoma.

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