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  1. Mohan A, Podin Y, Tai N, Chieng CH, Rigas V, Machunter B, et al.
    PLoS Negl Trop Dis, 2017 Jun;11(6):e0005650.
    PMID: 28599008 DOI: 10.1371/journal.pntd.0005650
    BACKGROUND: Melioidosis is a serious, and potentially fatal community-acquired infection endemic to northern Australia and Southeast Asia, including Sarawak, Malaysia. The disease, caused by the usually intrinsically aminoglycoside-resistant Burkholderia pseudomallei, most commonly affects adults with predisposing risk factors. There are limited data on pediatric melioidosis in Sarawak.

    METHODS: A part prospective, part retrospective study of children aged <15 years with culture-confirmed melioidosis was conducted in the 3 major public hospitals in Central Sarawak between 2009 and 2014. We examined epidemiological, clinical and microbiological characteristics.

    FINDINGS: Forty-two patients were recruited during the 6-year study period. The overall annual incidence was estimated to be 4.1 per 100,000 children <15 years, with marked variation between districts. No children had pre-existing medical conditions. Twenty-three (55%) had disseminated disease, 10 (43%) of whom died. The commonest site of infection was the lungs, which occurred in 21 (50%) children. Other important sites of infection included lymph nodes, spleen, joints and lacrimal glands. Seven (17%) children had bacteremia with no overt focus of infection. Delays in diagnosis and in melioidosis-appropriate antibiotic treatment were observed in nearly 90% of children. Of the clinical isolates tested, 35/36 (97%) were susceptible to gentamicin. Of these, all 11 isolates that were genotyped were of a single multi-locus sequence type, ST881, and possessed the putative B. pseudomallei virulence determinants bimABp, fhaB3, and the YLF gene cluster.

    CONCLUSIONS: Central Sarawak has a very high incidence of pediatric melioidosis, caused predominantly by gentamicin-susceptible B. pseudomallei strains. Children frequently presented with disseminated disease and had an alarmingly high death rate, despite the absence of any apparent predisposing risk factor.

  2. Lim CC, Sitoh YY, Hui F, Lee KE, Ang BS, Lim E, et al.
    AJNR Am J Neuroradiol, 2000 Mar;21(3):455-61.
    PMID: 10730635
    BACKGROUND AND PURPOSE: An epidemic of suspected Japanese encephalitis occurred in Malaysia in 1998-1999 among pig farmers. In neighboring Singapore, an outbreak occurred among pig slaughterhouse workers. It was subsequently established that the causative agent in the outbreak was not the Japanese encephalitis virus but a previously unknown Hendra-like paramyxovirus named Nipah virus.

    METHODS: The brain MR images of eight patients with Nipah virus infection were reviewed. All patients tested negative for acute Japanese encephalitis virus. Seven patients had contrast-enhanced studies and six had diffusion-weighted examinations.

    RESULTS: All patients had multiple small bilateral foci of T2 prolongation within the subcortical and deep white matter. The periventricular region and corpus callosum were also involved. In addition to white matter disease, five patients had cortical lesions, three had brain stem involvement, and a single thalamic lesion was detected in one patient. All lesions were less than 1 cm in maximum diameter. In five patients, diffusion-weighted images showed increased signal. Four patients had leptomeningeal enhancement and four had enhancement of parenchymal lesions.

    CONCLUSION: The brain MR findings in patients infected with the newly discovered Nipah paramyxovirus are different from those of patients with Japanese encephalitis. In a zoonotic epidemic, this striking difference in the appearance and distribution of lesions is useful in differentiating these diseases. Diffusion-weighted imaging was advantageous in increasing lesion conspicuity.

  3. Sia TLL, Mohan A, Ooi MH, Chien SL, Tan LS, Goh C, et al.
    Open Forum Infect Dis, 2021 Oct;8(10):ofab460.
    PMID: 34646909 DOI: 10.1093/ofid/ofab460
    Background: Burkholderia pseudomallei, the causative agent of melioidosis, is intrinsically resistant to a broad range of antibiotics, including aminoglycosides. In Sarawak, Malaysia, a high proportion of melioidosis cases are caused by gentamicin-susceptible isolates. There are limited epidemiological and clinical data on these infections.

    Methods: We conducted a retrospective study of culture-confirmed melioidosis among adults admitted to Bintulu Hospital in Sarawak, Malaysia, from January 2011 until December 2016.

    Results: One hundred forty-eight adults with culture-confirmed melioidosis were identified. Of 129 (87%) tested, 84 (65%) had gentamicin-susceptible B pseudomallei. The average annual incidence of melioidosis was 12.3 per 100 000 population, with marked variation between districts ranging from 5.8 to 29.3 per 100 000 population. Rural districts had higher incidences of melioidosis and overwhelmingly larger proportions of gentamicin-susceptible B pseudomallei infection. Significantly more patients with gentamicin-susceptible infection had no identified risk factors, with diabetes less frequently present in this group. Ninety-eight percent had acute presentations. Pneumonia, reported in 71%, was the most common presentation. Splenic abscesses were found in 54% of those imaged. Bacteremia was present in 88%; septic shock occurred in 47%. Forty-five (35%) patients died. No differences in clinical, laboratory, or outcome characteristics were noted between gentamicin-susceptible and gentamicin-resistant infections.

    Conclusions: Gentamicin-susceptible B pseudomallei infections are common in Sarawak and dominate in the high-incidence rural interior regions. Clinical manifestations and outcomes are the same as for gentamicin-resistant B pseudomallei infections. Further studies are required to determine if all gentamicin-susceptible B pseudomallei infections in Sarawak are clonal and to ascertain their environmental drivers and niches.

  4. Sia TLL, Mohan A, Ooi MH, Chien SL, Tan LS, Goh C, et al.
    Open Forum Infect Dis, 2022 Feb;9(2):ofab653.
    PMID: 35097155 DOI: 10.1093/ofid/ofab653
    [This corrects the article DOI: 10.1093/ofid/ofab460.].
  5. Lee YL, Zaini AA, Idris AN, Abdullah RA, Wong JS, Hong JS, et al.
    J Paediatr Child Health, 2023 Jul;59(7):879-884.
    PMID: 37066819 DOI: 10.1111/jpc.16405
    AIMS: Knowledge on the spectrum of thyroid disorders amongst Turner syndrome (TS) patients in Southeast Asia is limited. This study aimed to evaluate the prevalence of thyroid autoimmunity, the spectrum of autoimmune thyroid disease and association with age and karyotype amongst Malaysian TS girls.

    METHODS: A cross-sectional study was conducted at 11 paediatric endocrine units in Malaysia. Blood samples for antithyroglobulin antibodies, antithyroid peroxidase antibodies and thyroid function test were obtained. In patients with pre-existing thyroid disease, information on clinical and biochemical thyroid status was obtained from medical records.

    RESULTS: Ninety-seven TS patients with a mean age of 13.4 ± 4.8 years were recruited. Thyroid autoimmunity was found in 43.8% of TS patients. Nineteen per cent of those with thyroid autoimmunity had autoimmune thyroid disease (Hashimoto thyroiditis in 7.3% and hyperthyroidism in 1% of total population). Patients with isochromosome X and patients with 45,X mosaicism or other X chromosomal abnormalities were more prone to have thyroid autoimmunity compared to those with 45,X karyotype (OR 5.09, 95% CI 1.54-16.88, P = 0.008 and OR 3.41, 95% CI 1.32-8.82, P = 0.01 respectively). The prevalence of thyroid autoimmunity increased with age (33.3% for age 0-9.9 years; 46.8% for age 10-19.9 years and 57.1% age for 20-29.9 years) with autoimmune thyroid disease detected in 14.3% during adulthood.

    CONCLUSION: Thyroid autoimmunity was significantly associated with the non 45,X karyotype group, particularly isochromosome X. Annual screening of thyroid function should be carried out upon diagnosis of TS until adulthood with more frequent monitoring recommended in the presence of thyroid autoimmunity.

  6. Podin Y, Kaestli M, McMahon N, Hennessy J, Ngian HU, Wong JS, et al.
    J Clin Microbiol, 2013 Sep;51(9):3076-8.
    PMID: 23784129 DOI: 10.1128/JCM.01290-13
    Misidentifications of Burkholderia pseudomallei as Burkholderia cepacia by Vitek 2 have occurred. Multidimensional scaling ordination of biochemical profiles of 217 Malaysian and Australian B. pseudomallei isolates found clustering of misidentified B. pseudomallei isolates from Malaysian Borneo. Specificity of B. pseudomallei identification in Vitek 2 and potentially other automated identification systems is regionally dependent.
  7. Mallika PS, Lee PY, Cheah WL, Wong JS, Syed Alwi SAR, Nor Hayati H, et al.
    Malays Fam Physician, 2011;6(2):60-65.
    MyJurnal
    Introduction: This study reports on the prevalence of diabetic retinopathy (DR) and risk factors among diabetic patients, who underwent fundus photography screening in a primary care setting of Borneo Islands, East Malaysia. We aimed to explore the preliminary data to help in the planning of more effective preventive strategies of DR at the primary health care setting.
    Materials and Methods: A cross-sectional study on 738 known diabetic patients aged 19-82 years was conducted in 2004. Eye examination consists of visual acuity testing followed by fundus photography for DR assessment. The fundus pictures were reviewed by a family physician and an ophthalmologist. Fundus photographs were graded as having no DR, NPDR, PDR and maculopathy. The data of other parameters was retrieved from patient’s record. Bi-variate and multivariate analysis was used to elucidate the factors associated with DR.
    Results: Any DR was detected in 23.7% (95% CI=21 to 27%) of the patients and 3.2% had proliferative DR. The risk factors associated with any DR was duration of DM (OR =2.5, CI=1.6 to 3.9 for duration of five to 10 years when compared to <5 years) and lower BMI (OR=1.8, CI=1.1 to 3.0). Moderate visual loss was associated with DR (OR=2.1, CI=1.2 to 3.7).
    Conclusions: This study confirms associations of DR with diabetic duration, body mass index and visual loss. Our data provide preliminary findings to help to improve the screening and preventive strategies of DR at the primary health care setting.
    Keywords: Diabetic retinopathy, epidemiology, screening, primary health care, Malaysia
    Study site: Klinik Kesihatan Jalan Masjid, Kuching, Sarawak, Malaysia
  8. Hang Cheung PW, Canavese F, Wei Chan CY, Him Wong JS, Shigematsu H, Kei Luk KD, et al.
    J Bone Joint Surg Am, 2022 Jan 06.
    PMID: 35006096 DOI: 10.2106/JBJS.21.00747
    BACKGROUND: For growing patients, it is ideal to have a growth plate visible in routine radiographs for skeletal maturity assessment without additional radiation. The proximal femoral epiphyseal ossification is in proximity to the spine; however, whether it can be used for assessing a patient's growth status remains unknown.

    METHODS: Two hundred and twenty sets of radiographs of the spine and the left hand and wrist of patients with idiopathic scoliosis were assessed for skeletal maturity and reliability testing. Risser staging, Sanders staging (SS), distal radius and ulna (DRU) classification, the proximal humeral ossification system (PHOS), and the novel proximal femur maturity index (PFMI) were used. The PFMI was newly developed on the basis of the radiographic appearances of the femoral head, greater trochanter, and triradiate cartilage. It consists of 7 grades (0 to 6) associated with increasing skeletal maturity. The PFMI was evaluated through its relationship with pubertal growth (i.e., the rate of changes of standing and sitting body height [BH] and arm span [AS]) and with established skeletal maturity indices. Longitudinal growth data and 780 corresponding spine radiographs were assessed to detect peak growth using receiver operating characteristic (ROC) curve analysis.

    RESULTS: The PFMI was found to be correlated with chronological age (τb = 0.522), growth rates based on standing BH (τb = -0.303), and AS (τb = -0.266) (p < 0.001 for all). The largest growth rate occurred at PFMI grade 3, with mean standing BH growth rates (and standard deviations) of 0.79 ± 0.44 cm/month for girls and 1.06 ± 0.67 cm/mo for boys. Growth rates of 0.12 ± 0.23 cm/mo (girls) and 0 ± 0 cm/mo (boys) occurred at PFMI grade 6, indicating growth cessation. Strong correlations were found between PFMI gradings and Risser staging (τb = 0.743 and 0.774 for girls and boys), Sanders staging (τb = 0.722 and 0.736, respectively), and radius (τb = 0.792 and 0.820) and ulnar gradings (τb = 0.777 and 0.821), and moderate correlations were found with PHOS stages (τb = 0.613 and 0.675) (p < 0.001 for all). PFMI gradings corresponded to as young as SS1, R4, U1, and PHOS stage 1. Fair to excellent interrater and intrarater reliabilities were observed. PFMI grade 3 was most prevalent and predictive for peak growth based on ROC results.

    CONCLUSIONS: The PFMI demonstrated clear pubertal growth phases with satisfactory reliability. Grade 3 indicates peak growth and grade 6 indicates growth cessation.

    CLINICAL RELEVANCE: The use of PFMI can benefit patients by avoiding additional radiation in skeletal maturity assessment and can impact current clinical protocol of patient visits. PFMI gradings had strong correlations with SS, DRU gradings, and Risser staging, and they cross-referenced to their established grades at peak growth and growth cessation. PFMI may aid in clinical decision making.

  9. Wong JS, Mohd Azri ZA, Subramaniam G, Ho SE, Palasubramaniam S, Navaratnam P
    Malays J Pathol, 2003 Dec;25(2):113-9.
    PMID: 16196367
    beta-Lactamases have been identified as the major cause of antimicrobial resistance to beta-lactam antibiotics in Escherichia coli. The activities of ampicillin-sulbactam and amoxicillin-clavulanate as well as a range of beta-lactam antibiotics were studied with 87 clinical E. coli isolates from patients of the University Malaya Medical Center using the disc diffusion technique. Susceptible, intermediate and resistant categories were established based on the diameter of zones of inhibition set by the National Committee for Clinical Laboratory Standards (NCCLS). The isolates were then classified into 6 phenotypes according to the criteria stated in the methodology: S (susceptible to all beta-lactams); TL (resistant to aminopenicillins; amoxicillin-clavulanate susceptible and susceptible or intermediate to ampicillin-sulbactam); TI (resistant to aminopenicillins and ampicillin-sulbactam; susceptible to amoxicilin-clavulanate); TH-IRT (resistant to aminopenicillins; intermediate or resistant to amoxicillin-clavulanate; resistant to ampicillin-sulbactam); ESBL (resistant to aminopenicillins and oxyimino cephalosporins; positive results with the double-disc diffusion test); and CP (resistant to aminopenicillins, beta-lactam-beta-lactamase inhibitor combinations, oxyimino cephalosporins and cephamycins). Results showed that the TL phenotype was the commonest (40.2% of the isolates) followed by S (31%), TH-IRT (16.1%), ESBL and CP (3.4% each) and TI (2.3%). One isolate showed both ESBL and CP phenotypes while two isolates were classified as inconclusive. Representatives from each phenotype were further analysed for the presence of beta-lactamases which revealed a predominance of TEM and SHV enzyme producers. PCR-SSCP analysis of the SHV gene from all the ESBL and CP isolates revealed the predominance of SHV 5-type enzyme which was concurrent with our previous studies.
  10. Islam MA, Wong KK, Sasongko TH, Gan SH, Wong JS
    Eur J Rheumatol, 2016 Sep;3(3):139-141.
    PMID: 27733946 DOI: 10.5152/eurjrheum.2015.0068
    Here we present a case report of three familial primary antiphospholipid syndrome (PAPS) patients from Malaysia. The three familial patients comprised two females and one male with a mean age of 26.3 years. The first diagnosis was made between 2005 and 2009, and all patients demonstrated deep vein thrombosis, high levels of IgM and IgG anticardiolipin antibodies, and received warfarin treatment international normalized ratio (INR) 2.0-3.0. The patients ceased to show clinical symptoms after treatment. Recently (August 2014), we investigated whether the levels of antiphospholipid antibodies remained elevated, and we found that seronegativity occurred in the patients. We suspect that prolonged anticoagulant treatment might be one of the causes of reduced levels of antiphospholipid antibodies in these familial PAPS patients.
  11. Orimaye SO, Wong JS, Golden KJ, Wong CP, Soyiri IN
    BMC Bioinformatics, 2017 Jan 14;18(1):34.
    PMID: 28088191 DOI: 10.1186/s12859-016-1456-0
    BACKGROUND: The manual diagnosis of neurodegenerative disorders such as Alzheimer's disease (AD) and related Dementias has been a challenge. Currently, these disorders are diagnosed using specific clinical diagnostic criteria and neuropsychological examinations. The use of several Machine Learning algorithms to build automated diagnostic models using low-level linguistic features resulting from verbal utterances could aid diagnosis of patients with probable AD from a large population. For this purpose, we developed different Machine Learning models on the DementiaBank language transcript clinical dataset, consisting of 99 patients with probable AD and 99 healthy controls.

    RESULTS: Our models learned several syntactic, lexical, and n-gram linguistic biomarkers to distinguish the probable AD group from the healthy group. In contrast to the healthy group, we found that the probable AD patients had significantly less usage of syntactic components and significantly higher usage of lexical components in their language. Also, we observed a significant difference in the use of n-grams as the healthy group were able to identify and make sense of more objects in their n-grams than the probable AD group. As such, our best diagnostic model significantly distinguished the probable AD group from the healthy elderly group with a better Area Under the Receiving Operating Characteristics Curve (AUC) using the Support Vector Machines (SVM).

    CONCLUSIONS: Experimental and statistical evaluations suggest that using ML algorithms for learning linguistic biomarkers from the verbal utterances of elderly individuals could help the clinical diagnosis of probable AD. We emphasise that the best ML model for predicting the disease group combines significant syntactic, lexical and top n-gram features. However, there is a need to train the diagnostic models on larger datasets, which could lead to a better AUC and clinical diagnosis of probable AD.

  12. Wong JS, Bau R, Ahmad F, Ismail R, Yip AFY
    Med J Malaysia, 2013 Apr;68(2):141-3.
    PMID: 23629560 MyJurnal
    Atrial fibrillation (AF) is usually asymptomatic and often associated with established cardiovascular risk factors such as hypertension. The prevalence atrial fibrillation in patients admitted to Malaysian hospitals has been determined, but asymptomatic atrial fibrillation (AAF) in hypertensive patients in the primary care setting is not established. This study reports the prevalence of AAF in hypertensive patients in Malaysia, in a primary care setting. The overall prevalence of AAF was 0.75% with no differences between the gender. The prevalence of AAF increases with age - in the age groups of 30-39, >40-49, >50-59, >60-69, 70-79 and >80 years old were 0%, 0.17%, 0.35%, 2.32%, 2.59%, and 0% respectively. Hypertensive patients with age of ≥ 61 year old were associated with a probability of 10.6 times higher for AAF. We suggest the age threshold to screen for AAF to be age of 60. It is estimated that there are 49,029 Malaysians with AAF in 2010. A large population is at risk of AAF-related complications. There is justification for an even greater emphasis on diagnostic, primary and secondary prevention strategies.
    Study site: Klinik Kesihatan Bintulu, Sarawak, Malaysia
  13. Teh CL, Wong JS, Ngeh NK, Loh WL
    Rheumatol Int, 2011 Sep;31(9):1153-7.
    PMID: 20349069 DOI: 10.1007/s00296-010-1435-0
    We performed a cross-sessional study of all systemic lupus erythematosus (SLE) pregnancies during a 4-year period (2006-2009) to describe the clinical features, maternal and foetal outcomes in our centre. There were 48 pregnancies in 44 women with SLE. Our patients have a mean age of 30.0 years (SD 6.36) and a mean disease duration of 40.67 months (SD 48.23). Our patients have complicated pregnancies: 32.7% have SLE flares, 17.3% have preeclampsia and 48.9% needed caesarean sections. There were 20.0% foetal losses and 17.8% preterm deliveries in our patients. SLE flares contributed to 60.0% of foetal losses in our patients. Lupus pregnancies in our centre generally have a good maternal and foetal outcome comparable to developed countries in Asia. The low incidence of APS, the high usage of hydroxychloroquine and the high SLE remission rate in our patients prior to conceptions contributed to the good outcome.
  14. Teh CL, Wong JS, Ngeh NK, Loh WL
    Lupus, 2009 Mar;18(3):278-82.
    PMID: 19213870 DOI: 10.1177/0961203308096661
    We performed a retrospective study of all systemic lupus erythematosus (SLE) pregnancies during a two-year period (2006-2007) to describe the clinical features, maternal and foetal outcomes in our centre. There were 17 pregnancies in 16 women with SLE. Our patients have a mean age of 28.31 years (SD 5.24) and a mean disease duration of 38.62 months (SD 38.03). Our patients have complicated pregnancies: 35.3% have SLE flares, 21.1% have preeclampsia and 47.4% needed caesarean sections. There were 15.8% foetal losses and 12.5% preterm deliveries in our patients. All the foetal losses occurred in patients with severe SLE flares during pregnancies. Lupus pregnancies in our centre generally have a good maternal and foetal outcome comparable with developed countries. The low incidence of antiphospholipid syndrome, the high usage of hydroxychloroquine and the high SLE remission rate in our patients before conceptions are the possible factors contributing to the good outcome.
  15. Wong JS, Ong TA, Chua HH, Tan C
    Asian J Surg, 2007 Jan;30(1):80-1.
    PMID: 17337378
    Djenkol beans or jering (Pithecellobium jeringa) is a traditional delicacy consumed by the local population in Malaysia. Jering poisoning or djenkolism is characterized by spasmodic pain, urinary obstruction and acute renal failure. The underlying pathology is an obstructive nephropathy, which is usually responsive to aggressive hydration and diuretic therapy. We present a case of djenkolism following ingestion of jering. The patient required urgent bilateral ureteric stenting following the failure of conservative therapy. Healthcare providers need to recognize djenkolism as a cause of acute renal failure and the public educated on this potential health hazard.
  16. Wong EY, Chew PT, Chee CK, Wong JS
    Am J Ophthalmol, 1997 Dec;124(6):797-804.
    PMID: 9402826
    PURPOSE: To evaluate the effectiveness and safety of diode laser contact transscleral cyclophotocoagulation in Asian patients with refractory glaucoma by lower energy settings with an innovative probe featuring a glass ball tip that focused the laser beam onto the ciliary body.

    METHODS: This prospective clinical study included consecutive Asian patients with dark irides and confirmed for glaucoma. Only one eye of each patient was treated. Diode laser contact transscleral cyclophotocoagulation treatment was performed with the center of the probe placed 1.5 mm behind the limbus. About 30 pulses of 810-mm laser radiation (power, 1.8 to 2.0 W; duration, 0.3 to 0.5 second) were applied around the eye. Patients were examined at fixed postoperative intervals. Intraocular pressure levels and postoperative complications were recorded. The relation between patient and disease characteristics, total laser energy delivered, and intraocular pressure effects were analyzed.

    RESULTS: Thirty-three patients were studied, with a mean follow-up period of 9.4 months. An average 56% of patients showed a 30% or greater drop in intraocular pressure. About 38% of patients achieved sustained intraocular pressure lowering to below 22 mm Hg at 18 months. Complications were few and included transient hypotony and iritis.

    CONCLUSIONS: In Asian patients with refractory glaucoma or painful glaucomatous eyes with poor visual acuity (defined for this study as worse than 20/200), low-energy-setting diode laser contact transscleral cyclophotocoagulation by means of the glass ball probe is relatively effective and safe.

  17. Sia T, Podin Y, Chuah TB, Wong JS
    Eur Heart J Case Rep, 2018 Jun;2(2):yty055.
    PMID: 31020134 DOI: 10.1093/ehjcr/yty055
    Introduction: As a causal organism in infective endocarditis, Burkholderia pseudomallei is rare. Burkholderia pseudomallei is intrinsically resistant to aminoglycosides but a gentamicin-susceptible strain was discovered in Sarawak, Malaysian Borneo in 2010. We report the first occurrence of infective endocarditis due to the gentamicin-susceptible strain of B. pseudomallei.

    Case presentation: A 29-year-old man presented with pneumonia and melioidosis septicaemia. His condition was complicated with infective endocarditis and septic emboli to the brain. Despite difficulties in reaching a diagnosis, the patient was successfully treated using intravenous gentamicin and ceftazidime and was discharged well.

    Discussion: The role of gentamicin in the treatment of the gentamicin-susceptible strain of B. pseudomallei remains unclear.

  18. Fuah KW, Lim CTS, Pang DCL, Wong JS
    Saudi J Kidney Dis Transpl, 2018 2 20;29(1):207-209.
    PMID: 29456232 DOI: 10.4103/1319-2442.225177
    Tranexamic acid (TXA) is an antifibrinolytic agent commonly used to achieve hemostasis. However, there have been a few case reports suggesting that high-dose intravenous TXA has epileptogenic property. In patients with renal impairment, even administering the usual recommended dose of TXA can induce seizure episodes. We present here a patient on hemodialysis who developed seizures after receiving two doses of TXA over 5 h period.
  19. Ng BW, Wong JS, Toh TH
    BMJ Case Rep, 2021 Dec 22;14(12).
    PMID: 34937752 DOI: 10.1136/bcr-2021-245154
    Pheochromocytomas are rare in children. The diagnosis is usually established from a raised urinary or plasma catecholamine or their metabolites. We present a girl aged 11 years who manifested with a hypertensive crisis secondary to an adrenal tumour but with unexpectedly normal urinary metanephrine and catecholamine results. She improved spontaneously following the crisis and underwent surgery later. The histopathological study confirmed a pheochromocytoma with large central necrosis. Her genetic screening reported a pathogenic von Hippel-Lindau gene mutation. Surveillance scan postsurgery detected no other tumours. Following the catecholamine crisis, an acute infarct occurred, resulting in extensive tumour necrosis and subsequent rapid remission of symptoms and paradoxically normal biochemical markers. Although not unheard of in adults, we believe this is the first reported case of an extensive spontaneous necrosis resulting in a biochemically normal pheochromocytoma in a child.
  20. Teh CL, Wong JS, Soo HH
    Rheumatol Int, 2012 Jan;32(1):265-8.
    PMID: 21243497 DOI: 10.1007/s00296-010-1745-2
    We performed a cross-sectional study of the demography, clinical and laboratory features of patients with polymyositis and dermatomyositis followed up in our centre from 2006 to 2009. There were 12 cases, with the majority of them (58.3%) being woman. They have a mean age of 57.8 years and mean disease duration of 11.83 (SD 9.92) months. Our patients comprised of multi-ethnic groups with predominantly Chinese (83.3%), Sarawak natives (8.3%) and Malays (8.3%). They have a mean lag time to diagnosis of 3.67 (SD 4.27) months. Nine (75%) patients had dermatomyositis and 3(25%) had polymyositis. The common clinical manifestations found in our patients were proximal myopathy (100%), neck weakness (33.3%), dysphagia (33.3%) and interstitial lung disease (33.3%). For the nine patients with dermatomyositis, the most common dermatological manifestations were shawl sign (88.9%) and V sign (88.9%). Muscle enzymes were raised in 91.7% of patients. Electromyographies were carried out in four patients, and only one of our patients had muscle biopsy. Only 41.7% of our patients have positive ANA. The majority received prednisolone (100%) and hydroxychloroquine (58.3%). Malignancy occurred in five (three nasopharyngeal carcinomas, one sigmoid colon cancer and one lung cancer) out of the nine dermatomyositis patients but none in the polymyositis group. The mortality rate in our group was 4(33.3%) over the 4-year period. This study demonstrated the rarity of PM/DM in our centre with considerable lag time to diagnosis in our patients. Despite lack of muscle biopsy in our centre, our centre achieved appropriate diagnosis and management of PM/DM.
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