Displaying publications 1 - 20 of 352 in total

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  1. Ho HH, Sinaga DA, Arshad MKM, Kasim S, Lee JH, Khoo DZL, et al.
    PMID: 32021903 DOI: 10.1016/j.ijcha.2020.100469
    Background: Amphilimus-eluting stent (AES) is a novel polymer-free drug eluting stent that combines sirolimus with fatty acid as antiproliferative drug and has shown promising results in percutaneous coronary intervention.We evaluated the clinical safety and efficacy of AES in an all-comers South-East Asian registry.

    Methods: Between May 2014 to April 2017, 268 patients (88% male, mean age 60.1 ± 10.8 years) with 291 coronary lesions were treated with AES. The primary endpoint was major adverse cardiac events (MACE) ie a composite of cardiovascular mortality, myocardial infarction (MI) and target lesion revascularization (TLR) at 12-month follow-up.

    Results: The majority of patients presented with acute coronary syndrome (75%) and 75% had multi-vessel disease on angiography. Diabetes mellitus was present in 123 patients (46%). The most common target vessel for PCI was left anterior descending artery (43%) followed by right coronary artery (36%), left circumflex (10%) and left main (6%).The majority of lesions were type B-C (85%) by ACC/AHA lesion classification. An average of 1.25 ± 0.5 AES were used per patient, with mean AES diameter of 3.1 ± 0.4 mm and average total length of 34.8 ± 19.4 mm.At 12-month follow-up, 4% of patients developed MACE. MACE was mainly driven by cardiovascular mortality (1.5%), MI (2%) and TLR (1.5%). The rate of stent thrombosis was 1.5%.

    Conclusion: In a contemporary all-comers South-East Asian registry with high rate of diabetes mellitus, AES was found to be efficacious with a low incidence of MACE observed at 12-month follow-up.

    Matched MeSH terms: Registries
  2. Loo CH, Tan WC, Khor YH, Chan LC
    Med J Malaysia, 2018 04;73(2):73-77.
    PMID: 29703869 MyJurnal
    INTRODUCTION: Severe cutaneous adverse drug reactions (SCARs) are not uncommon and potentially lifethreatening. Our objective is to study the patient characteristics, the pattern of implicated drugs and treatment outcome among patients with SCARs.

    METHODS: A 10-year retrospective analysis of SCARs cases in Penang General Hospital was carried out from January 2006 to December 2015. Data collection is based on the Malaysian Adverse Drug Reactions Advisory Committee registry and dermatology clinic records.

    RESULTS: A total of 189 cases of SCARs were encountered (F:M ratio; 1.2:1.0; mean age of 45 year). The commonest manifestation was Stevens-Johnson Syndrome [SJS] (55.0%), followed by toxic epidermal necrolysis [TEN] (23.8%), drug rash with eosinophilia and systemic symptoms [DRESS] (12.7%), acute generalised exanthematous pustulosis [AGEP] (4.8%), SJS/TEN overlap syndrome (2.6%) and generalised bullous fixed drug eruptions [GBFDE] (1.1%). Mean time to onset for TEN/SJS/Overlap syndrome was 10.5±13 days; AGEP, three days; GBFDE, 2.5±0.7 days, and DRESS, 29.4±5.7 days. The most common drugs implicated were antibiotics (33.3%), followed by allopurinol (18.9%) and anticonvulsant (18.4%). Out of 154 cases of SJS/TEN/overlap syndrome, allopurinol was the commonest causative agents (20.1%). In DRESS, allopurinol accounts for 45.8% of the cases. The mortality rate in SJS, TEN and DRESS were 1.9%, 13.3% and 12.5% respectively. No mortality was observed in AGEP and GBFDE.

    CONCLUSION: The commonest manifestations of SCARs in our setting were SJS, TEN and DRESS. Allopurinol was the most common culprit. Thus, judicious allopurinol use is advocated and pre-emptive genetic screening for HLAB *5801 should be considered.

    Matched MeSH terms: Registries
  3. Wong K. H.
    MyJurnal
    Introduction: Breast cancer is the commonest cancer in Malaysia, predominantly among women under age of 60 and they often presented late. Borneo is home to multi-racial indigenous with heterogeneous background and breast cancer profile can be absolutely unique. This study aims to examine the demographic characteristics and stage at presentation in Sandakan women with newly diagnosed breast cancer. Methods: This is a cross-sectional, retrospec-tive study involving those women who were newly diagnosed with breast cancer from January 2016 to December 2018 in Duchess of Kent Hospital, Sandakan identified via hospital cancer registry and patient records. Only breast cancer with epithelial origin was included. Age, ethnicity and stages at presentation of breast cancer were analysed. Logistic regression was used to study their relationships. Results: 110 women were newly diagnosed as breast cancer. Mean age was 53.5 (SD 12.7), the youngest at 30 and the eldest at 97. Majority (64.6%) were local indigenous wom-en, constituted by 25.4% Sungai, 14.1% Kadazan-Dusun, 12.7% Bugis and the minorities. Chinese women are the main local non-indigenous (32.7%) followed by 2.7% Malay. 52.1% presented at late stages (stage III/IV). Subgroup analysis of T-staging revealed 41.7% had advanced symptoms (T3/T4). Indigenous group was more likely to present at younger ages (OR 12.0; 95%CI 1.5-93.8) and with advanced symptoms (OR 3.1; 95%CI 1.2-8.0). Conclusion: Awareness on breast cancer remains inadequate particularly among indigenous women. Difficult healthcare accessi-bility and incline towards traditional medicine could attribute to late presentation. Outreach awareness programmes are warranted in addition to mobile screening services.
    Matched MeSH terms: Registries
  4. Mahmud I, Kelley T, Stowell C, Haripriya A, Boman A, Kossler I, et al.
    JAMA Ophthalmol, 2015 Nov;133(11):1247-52.
    PMID: 26291752 DOI: 10.1001/jamaophthalmol.2015.2810
    Aligning outcome measures for cataract surgery, one of the most frequently performed procedures globally, may facilitate international comparisons that can drive improvements in the outcomes most meaningful to patients.
    Matched MeSH terms: Registries
  5. Jacob M, Sahu S, Singh YP, Mehta Y, Yang KY, Kuo SW, et al.
    Indian J Crit Care Med, 2020 Nov;24(11):1028-1036.
    PMID: 33384507 DOI: 10.5005/jp-journals-10071-23653
    Introduction: Fluid therapy in critically ill patients, especially timing and fluid choice, is controversial. Previous randomized trials produced conflicting results. This observational study evaluated the effect of colloid use on 90-day mortality and acute kidney injury (RIFLE F) within the Rational Fluid Therapy in Asia (RaFTA) registry in intensive care units.

    Materials and methods: RaFTA is a prospective, observational study in Asian intensive care unit (ICU) patients focusing on fluid therapy and related outcomes. Logistic regression was performed to identify risk factors for increased 90-day mortality and acute kidney injury (AKI).

    Results: Twenty-four study centers joined the RaFTA registry and collected 3,187 patient data sets from November 2011 to September 2012. A follow-up was done 90 days after ICU admission. For 90-day mortality, significant risk factors in the overall population were sepsis at admission (OR 2.185 [1.799; 2.654], p < 0.001), cumulative fluid balance (OR 1.032 [1.018; 1.047], p < 0.001), and the use of vasopressors (OR 3.409 [2.694; 4.312], p < 0.001). The use of colloids was associated with a reduced risk of 90-day mortality (OR 0.655 [0.478; 0.900], p = 0.009). The initial colloid dose was not associated with an increased risk for AKI (OR 1.094 [0.754; 1.588], p = 0.635).

    Conclusion: RaFTA adds the important finding that colloid use was not associated with increased 90-day mortality or AKI after adjustment for baseline patient condition.

    Clinical significance: Early resuscitation with colloids showed potential mortality benefit in the present analysis. Elucidating these findings may be an approach for future research.

    How to cite this article: Jacob M, Sahu S, Singh YP, Mehta Y, Yang K-Y, Kuo S-W, et al. A Prospective Observational Study of Rational Fluid Therapy in Asian Intensive Care Units: Another Puzzle Piece in Fluid Therapy. Indian J Crit Care Med 2020;24(11):1028-1036.

    Matched MeSH terms: Registries
  6. Al-Herz W, Zainal M, Nanda A
    Front Immunol, 2021;12:751469.
    PMID: 34659256 DOI: 10.3389/fimmu.2021.751469
    Background and Objectives: Reports on skin manifestations in inborn errors of immunity (IEI) are based on retrospective analysis, small series, or isolated case reports. The present prospective study aimed to determine the spectrum of skin manifestations in children with IEI and their relevance to specific molecular defects.

    Materials and Methods: The data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of 2004-2020.

    Results: A total of 313 pediatric cases of IEI, 71% diagnosed at molecular level, were registered with a cumulative follow-up period of 29,734 months. Skin manifestations were seen in 40.3% of the patients, and they were among the presenting manifestations in 33%. Patients with skin manifestations were older at both onset and diagnosis ages of IEI symptoms, but this was statistically significant for the latter only. The diagnosis delay was significantly longer in patients with skin manifestations. There was a statistically significant association between having skin manifestations and IEI category, being more common in patients with complement deficiencies, combined immunodeficiencies, and diseases of immune dysregulation. There was no statistically significant association between having skin manifestations and both gender and survival. Skin infections were the most frequent manifestations followed by eczema and autoimmune associations. Among IEI with more than 10 cases, skin lesions were a consistent finding in dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome, ataxia-telangiectasia, and recombination activation gene (RAG)1 deficiency.

    Conclusions: Skin manifestations are common in IEI patients, and they had significant diagnosis delay and referral to specialists. Improvement of awareness about IEI is needed among pediatricians and dermatologists.

    Matched MeSH terms: Registries
  7. Chang G, Chan CW, Hartman M
    Asian Pac J Cancer Prev, 2011;12(6):1635-9.
    PMID: 22126512
    Breast cancer is the most common cancer in Singaporean women and the rate of increase in incidence is one of the highest in the world. In view of the significant contribution of delayed presentation to the disease burden in South East Asia, we reviewed the incidence of late presentation of breast cancer and the contributing factors in Singapore. Disease presentation was analysed using studies based on the Singapore Cancer Registry 2004-2008 and with data from women with breast cancer at the National University Hospital (NUH) in Singapore 1990-2007. Available literature from Singapore on factors contributing to delayed presentation was reviewed and presented here. The overall age-standardized 5-year relative survival for Singaporean women was 70% with only half diagnosed with localized cancer. Of all women diagnosed at NUH close to 20% presented at Stages III and IV. Given the magnitude of the problem of women presenting with more advanced stages of breast cancer, the National University of Singapore has joined a collaborative team with the University of Leeds (UK), the University of Malaya, and University of UAE to set up the UK-SEA-ME Psychosocial-Cultural Cancer Research Network to better understand late presentation.
    Matched MeSH terms: Registries
  8. Yap LB, Nguyen ST, Qadir F, Ma SK, Muhammad Z, Koh KW, et al.
    Acta Cardiol, 2016 Jun;71(3):323-30.
    PMID: 27594128 DOI: 10.2143/AC.71.3.3152093
    Matched MeSH terms: Registries/statistics & numerical data
  9. King TL, Tiong LL, Kaman Z, Zaw WM, Abdul Aziz Z, Chung LW
    J Stroke Cerebrovasc Dis, 2020 Sep;29(9):105012.
    PMID: 32807427 DOI: 10.1016/j.jstrokecerebrovasdis.2020.105012
    BACKGROUND: Located on the Borneo Island, Sarawak is the largest state of Malaysia and has a population distinctive from Peninsular Malaysia. The ischaemic stroke data in Sarawak had not been reported despite the growing number of patients annually. We aimed to investigate patient characteristics, management, and outcomes of ischaemic stroke in Sarawak and benchmark the results with national and international published data.

    METHODS: We included ischaemic stroke cases admitted to Sarawak General Hospital between June 2013 and August 2018 from Malaysia National Stroke Registry. We performed descriptive analyses on patient demographics, cardiovascular risk factors, prior medications, smoking status, arrival time, thrombolysis rate, Get With The Guidelines (GWTG)-Stroke measures, and outcomes at discharge. We also numerically compared the results from Sarawak with the published data from selected national and international cohorts.

    RESULTS: We analysed 1435 ischaemic stroke cases. The mean age was 60.1±13.2 years old; 64.9% were male; median baseline National Institute of Health Stroke Scale was seven points. Hypertension was the most prevalent risk factor of ischaemic stroke; 12.7% had recurrent stroke; 13.7% were active smokers. The intravenous thrombolysis rate was 18.8%. We achieved 80-90% in three GWTG-Stroke performance measures and 90-98% in four additional quality measures in our ischaemic stroke management. At discharge, 57% had modified Rankin Scale of 0-2; 6.7% died during hospitalisation. When compared with selected national and international data, patients in Sarawak were the youngest; Sarawak had more male and more first-ever stroke. Thrombolysis rate in Sarawak was higher compared with most studies in the comparison. Functional outcome at discharge in Sarawak was better than national cohort but still lagging behind when compared with the developed countries. In-hospital mortality rate in Sarawak was slightly lower than the national data but higher when compared with other countries.

    CONCLUSION: Our study described characteristics, management, and outcomes of ischaemic stroke in Sarawak. We achieved high compliance with most of GTWG-Stroke performance and quality indicators. Sarawak had better outcomes than the national results on ischaemic stroke. However, there is still room for improvement when compared with other countries. Actions are needed to reduce the cardiovascular burdens for stroke prevention, enhance healthcare resources for stroke care, and improve intravenous thrombolysis treatment in Sarawak.

    Matched MeSH terms: Registries
  10. Fuziah MZ, Hong JY, Zanariah H, Harun F, Chan SP, Rokiah P, et al.
    Med J Malaysia, 2008 Sep;63 Suppl C:37-40.
    PMID: 19230245
    In Malaysia, Diabetes in Children and Adolescents Registry (DiCARE) was launched nationwide in August 2006 to determine and monitor the number, the time trend of diabetes mellitus (DM) patients, their socio-demographic profiles, outcome of intervention and facilitate research using this registry. This is an on going real time register of diabetic patients < or = 20 years old via the e-DiCARE, an online registration system. To date were 240 patients notified from various states in Malaysia. The mean age was 12.51 years (1.08-19.75) and 46.4% were boys. The mean age at diagnosis was 8.31 +/- 4.13 years old with an estimated duration of diabetes of 4.32 +/- 3.55 years. A total of 166/240 (69.2%) have T1DM, 42/240 (17.5%) have T2DM and 18/240 (7.5%) have other types of DM. Basis of diagnosis was known in 162 patients with T1DM and 41 patients with T2DM. In T1DM patients, 6.0% of the girls and 19.1% boys were overweight or obese. As for T2DM, 64.3% had their BMI reported: 66.7% girls and 91.6% boys were overweight or obese. Most patients (80.4%) practiced home blood glucose monitoring. Patients were seen by dietitian (66.7%), diabetes educator (50.0%), and optometrist or ophthalmologist (45.0%). Only 10.8% attended diabetic camps. In the annual census of 117 patients, the mean HbAlc level was 10.0% + 2.2 (range 5.2 to 17.0%). The early results of DiCARE served as a starting point to improve the standard of care of DM among the young in the country.
    Matched MeSH terms: Registries/statistics & numerical data*
  11. Thong MK, Ho JJ, Khatijah NN
    Ann. Hum. Biol., 2005 Mar-Apr;32(2):180-7.
    PMID: 16096215 DOI: 10.1080/03014460500075332
    Birth defects are one of the leading causes of paediatric disability and mortality in developed and developing countries. Data on birth defects from population-based studies originating from developing countries are lacking. One of the objectives of this study was to determine the epidemiology of major birth defects in births during the perinatal period in Kinta district, Perak, Malaysia over a 14-month period, using a population-based birth defect register. There were 253 babies with major birth defects in 17,720 births, giving an incidence of 14.3/1000 births, a birth prevalence of 1 in 70. There were 80 babies with multiple birth defects and 173 with isolated birth defects. The exact syndromic diagnosis of the babies with multiple birth defects could not be identified in 18 (22.5%) babies. The main organ systems involved in the isolated birth defects were cardiovascular (13.8%), cleft lip and palate (11.9%), clubfeet (9.1%), central nervous system (CNS) (including neural tube defects) (7.9%), musculoskeletal (5.5%) and gastrointestinal systems (4.7%), and hydrops fetalis (4.3%). The babies with major birth defects were associated with lower birth weights, premature deliveries, higher Caesarean section rates, prolonged hospitalization and increased specialist care. Among the cohort of babies with major birth defects, the mortality rate was 25.2% during the perinatal period. Mothers with affected babies were associated with advanced maternal age, birth defects themselves or their relatives but not in their other offspring, and significantly higher rates of previous abortions. The consanguinity rate of 2.4% was twice that of the control population. It is concluded that a birth defects register is needed to monitor these developments and future interventional trials are needed to reduce birth defects in Malaysia.
    Matched MeSH terms: Registries
  12. Tam, Jenn Zhueng, Sharifa Ezat Wan Putih, Noor Hassim
    MyJurnal
    The study objective is to examine the effects of the identified attributes (i.e. employee characteristics and ergonomics) that influences the chronic back pain disabilities acquired and benefits awarded via the recognition of workplace relatedness. As these factors are considered in work- related chronic back pain, we hypothesized that the ascertainment of work relatedness would depend on employees’ workplace conditions (poor ergonomic practices) and innate personal conditions (excellent pre-existing medical health). Two set of data was collected from a registry owned by a social security organization in 2012. Factor analysis and structural equation modeling (SEM) was used to analyze the data. As predicted, workplace conditions and employees’ pre- existing medical health status mediated occupational back pain to develop disability and monetary benefit relationships. In conclusion, the results support the importance of obtaining workplace evidence and employees’ pre- existing medical health status that produces stronger relationship in terms of the amount of benefits to be given compared to the lesser effects on disability. Both models with different samples showed consistent SEM results. The proposed model would be applicable in the heterogenous Asian setting and that occupational back pain consensus is the key element in providing effective social security protection.
    Matched MeSH terms: Registries
  13. Hooi LS
    Med J Malaysia, 1993 Jun;48(2):185-93.
    PMID: 8350794
    A registry of patients with end stage renal disease was started from 1st January 1990 at Hospital Sultanah Aminah, Johor Baru. There were 126 patients in 1990 and 129 in 1991. The peak age was 31 to 60 years old; males outnumbered females 1.5:1. Forty-three to fifty-six percent presented with small kidneys. Seventeen to twenty percent of patients had diabetes mellitus. In 1991, the racial distribution of patients was Malay: 50.4%, Chinese: 39.5%, Indian: 7.8% and others: 2.3%. The incidence of end stage renal disease in Johor Baru district was 79 per million per year in 1990 and 86 per million in 1991.
    Matched MeSH terms: Registries*
  14. Lim YN, Lim TO, Lee DG, Wong HS, Ong LM, Shaariah W, et al.
    Med J Malaysia, 2008 Sep;63 Suppl C:5-8.
    PMID: 19230240
    The Malaysian National Renal Registry was set up in 1992 to collect data for patients on renal replacement therapy (RRT). We present here the report of the Malaysian dialysis registry. The objectives of this papar are: (1) To examine the overall provision of dialysis treatment in Malaysia and its trend from 1980 to 2006. (2) To assess the treatment rate according to the states in the country. (3) To describe the method, location and funding of dialysis. (4) To characterise the patients accepted for dialysis treatment. (5) To analyze the outcomes of the dialysis treatment. Data on patients receiving dialysis treatment were collected at initiation of dialysis, at the time of any significant outcome, as well as yearly. The number of dialysis patients increased from 59 in 1980 to almost 15,000 in 2006. The dialysis acceptance rate increased from 3 per million population in 1980 to 116 per million population in 2006, and the prevalence rate from 4 to 550 per million population over the same period. The economically advantaged states of Malaysia had much higher dialysis treatment rates compared to the less economically advanced states. Eighty to 90% of new dialysis patients were accepted into centre haemodialysis (HD), and the rest into the chronic ambulatory peritoneal dialysis (CAPD) programme. The government provided about half of the funding for dialysis treatment. Patients older than 55 years accounted for the largest proportion of new patients on dialysis since the 1990s. Diabetes mellitus has been the main cause of ESRD and accounted for more than 50% of new ESRD since 2002. Annual death rate averaged about 10% on HD and 15% on CAPD. The unadjusted 5-year patient survival on both HD and CAPD was about 80%. Fifty percent of dialysis patients reported very good median QoL index score. About 70% of dialysis patients were about to work full or part time. There has been a very rapid growth of dialysis provision in Malaysia particularly in the older age groups. ESRD caused by diabetes mellitus, despite being a preventable and treatable cause of ESRD--has increased and accounted for more than 50% of incident dialysis patients. Death and survival rates on dialysis are comparable to those from other countries.
    Matched MeSH terms: Registries/statistics & numerical data*
  15. Chee KY, Salina AA
    Med J Malaysia, 2014 Aug;69 Suppl A:46-54.
    PMID: 25417951 MyJurnal
    Research in schizophrenia has advanced tremendously. One hundred and seventy five articles related to Schizophrenia were found from a search through a database dedicated to indexing all original data relevant to medicine published in Malaysia between the years 2000-2013. This project aims to examine published research articles, in local and international journals in order to provide a glimpse of the research interest in Malaysia with regards to schizophrenia. Single case study, case series report, reviews and registry reports were not included in this review. Medication trial, unless it concerned a wider scope of psychopharmacology was also excluded from this review. A total of 105 articles were included in this review. Despite numerous genetics studies conducted and published, a definitive conclusion on the aetiology or mechanism underlying schizophrenia remains elusive. The National Mental Health - Schizophrenia Registry (NMHR) proved to be an important platform for many studies and publications. Studies stemmed from NMHR have provided significant insight into the baseline characteristic of patients with schizophrenia, pathway to care, and outcomes of the illness. International and regional collaborations have also encouraged important work involving stigma and discrimination in schizophrenia. Ministry of Health's hospitals (MOH) are the main research sites in the country with regards to schizophrenia research. Numbers of schizophrenia research are still low in relation to the number of universities and hospitals in the country. Some of the weaknesses include duplication of studies, over-emphasising clinical trials and ignoring basic clinical research, and the lack of publications in international and regional journals.
    Matched MeSH terms: Registries
  16. Ngim CF, Ibrahim H, Lai NM, Ng CS
    Prenat Diagn, 2015 Jan;35(1):51-9.
    PMID: 25156332 DOI: 10.1002/pd.4484
    We aimed to identify factors contributing to ineffective prevention of thalassaemia in Malaysia by studying events leading to the births of children with transfusion-dependent thalassaemia (TDT) including their parents' reproductive choices.
    Matched MeSH terms: Registries/statistics & numerical data
  17. Lee YY, Noridah N, Syed Hassan SA, Menon J
    PeerJ, 2014;2:e257.
    PMID: 24688841 DOI: 10.7717/peerj.257
    Aim. Helicobacter pylori (H. pylori) infection is exceptionally rare in population from the north-eastern region of Peninsular Malaysia. This provides us an opportunity to contemplate the future without H. pylori in acute non-variceal upper gastrointestinal (GI) bleeding. Methods. All cases in the GI registry with GI bleeding between 2003 and 2006 were reviewed. Cases with confirmed non-variceal aetiology were analysed. Rockall score > 5 was considered high risk for bleeding and primary outcomes studied were in-hospital mortality, recurrent bleeding and need for surgery. Results. The incidence of non-variceal upper GI bleeding was 2.2/100,000 person-years. Peptic ulcer bleeding was the most common aetiology (1.8/100,000 person-years). In-hospital mortality (3.6%), recurrent bleeding (9.6%) and need for surgery (4.0%) were uncommon in this population with a largely low risk score (85.2% with score ≤5). Elderly were at greater risk for bleeding (mean 68.5 years, P = 0.01) especially in the presence of duodenal ulcers (P = 0.04) despite gastric ulcers being more common. NSAIDs, aspirin and co-morbidities were the main risk factors. Conclusions. The absence of H. pylori infection may not reduce the risk of peptic ulcer bleeding in the presence of risk factors especially offending drugs in the elderly.
    Matched MeSH terms: Registries
  18. Ganesananthan S, Rajvinder S, Anil R, Kiew KK, Ng KL, Rosaida MS, et al.
    Med J Malaysia, 2004;59 Suppl C:48.
    Background: Achalasia cardia is an uncommon disease that is often detected late and is associated with significant morbidity. It is a primary esophageal motility disorder diagnosed based on a good history, barium swallow, upper endoscopy and a standard esophageal manometry.
    Materials and Methods: We reviewed complete available records of treatment naïve patients with achalasia cardia from 1st January 2000 till April 2004.
    Results: A total of 40 patients, with average presenting age at 44±16 (range 19-73) years with 14 males: 26 females with 20 Malays: 15 Chinese: 5 Indians, were suitable for further analysis. The classical symptom of dysphagia to liquids and solids were noted in all cases (100%). These patients learnt that water and sometimes-aerated drinks aid in flushing food down. Symptoms of regurgitation (36 patients-90%), heartburn (15 patients-37.5%), weight loss (10 patients–25%), nocturnal cough (16 patient-40%), retrosternal chest discomfort (2 patient-5%) and hemetemesis (2 patient-5%) was noted. One patient had aspiration pneumonia and another had concomitant active pulmonary tuberculosis and 8 had concomitant constipation (20%). In this series the duration of illness before diagnosis was 5±6 (range
    0.3- 30) years and their presenting weight was 53±13 (range 33-82) kg. Barium swallow diagnosed achalasia in 27 patients (67.5%) and a dysmotility disorder in 7 cases (17.5%). There were 10 patients with mega-esophagus and two had epiphrenic diverticulum. There was no pseudoachalasia. Standard esophageal manometry, performed in 36 cases, demonstrated aperistalsis with one vigorous achalasia. The manometric assembly failed to pass through the sphincter in 14 cases and hence LOS assessment was not possible. Four cases demonstrated normal LOS pressure but demonstrated incomplete relaxation (normotensive achalasia). Pneumatic dilatation was performed in 38 newly cases without any complications with excellent symptomatic relief and a 3-12 month post procedural weight gain of 7±5 (range: 0-19) kg. Six patients required a second dilatation and another required two further dilatation. The durability of the total 45 pneumatic dilatations during this short study period was excellent at 24±12 (range 2-48) months.
    Conclusion: A primary esophageal motility disorder must be excluded in any patients who present with dysphagia, with or without regurgitation and a "normal" upper endoscopy. Achalasia is not uncommon, often delayed in diagnosis and has a varied presentation. Although there is no cure for achalasia, but early detection and treatment certainly relieves symptoms and prevents complications. Pneumatic dilatation in our center has excellent durability without any complications.
    Matched MeSH terms: Registries
  19. Ganesananthan S, Kew ST, Ngau YY, Ong J, Matvinder S, Liew SH, et al.
    Med J Malaysia, 2001;56 Suppl A:47.
    Matched MeSH terms: Registries
  20. Thong MK
    Am J Med Genet C Semin Med Genet, 2019 06;181(2):254-261.
    PMID: 30801969 DOI: 10.1002/ajmg.c.31690
    The United Nations General Assembly adopted the 2030 Agenda for Sustainable Development in November 2015 which included a set of 17 measurable "sustainable development goals" (SDGs). The SDGs included targets to end preventable deaths of newborns and children under 5 years of age by 2030, universal health care coverage, reduction of premature mortality from noncommunicable diseases (NCDs) by 33% as well as support the development and research for medicines for both communicable and NCDs. Although some successes were achieved in combating communicable diseases and improved childhood mortality rates, health systems in Asia are generally characterized by lack of accurate epidemiological information on congenital disorders, lack of human and financial resources, and inadequate focus on public health strategies to ensure targeted interventions, low level knowledge on congenital disorders amongst the community and healthcare providers and the ethical dilemma of managing rare congenital disorders in an environment of low national health expenditures. These bottlenecks must be addressed systematically and interventions such as the use of innovative epidemiological tools to overcome lack of data, increased efforts to standardize rare disease nomenclature and classification and renewed interest in birth defects registries by countries in the region must be considered. Targeted curative and public health approaches currently used in thalassaemia and neural tube defects may be used for other congenital disorders in Asian countries. The implementation of congenital disorders-related research, prevention, care, and treatment delivery services must be integrated into existing health systems in order to be effective to achieve the targets of SDG2030.
    Matched MeSH terms: Registries
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