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  1. Chua YA, Nurhaslina H, Gan SH
    Methods Find Exp Clin Pharmacol, 2008 Dec;30(10):739-43.
    PMID: 19271022 DOI: 10.1358/mf.2008.30.10.1316830
    Because durian (Durio zibethinus), which is known in Southeast Asia as "the king of fruits", is thought to have special body-warming properties, it should not be consumed with paracetamol due to a risk of toxic effects. The claim of warming properties, however, has not been scientifically proven. This study was conducted to investigate durian's hyperthermic effect and its toxicity when consumed together with paracetamol in rats. Five groups of rats (n=6) were fed with: 1) distilled water (4 ml/250 g), 2) homogenized durian (4 g/250 g), 3) paracetamol solution (2400 mg/kg), 4) durian (4 g/250 g) followed by paracetamol solution (2400 mg/kg), or 5) prazosin solution (15 mg/kg, pregavaged) followed 1 h later by durian (4 g/250 g) and paracetamol solution (2400 mg/kg). Rectal temperature, systolic blood pressure and serum alanine aminotransferase (ALT) levels were taken from each rat at baseline and after the various administrations at 1, 2 and 5 h. Our results showed that the body temperature of rats in the durian-treated group was not significantly elevated when compared to the control. However, there was a significant decrease in body temperature over time in animals from groups 4 and 5. We did not, however, observe a consistent pattern of blood pressure change. Serum chemical analysis for ALT also did not show any significant change in any of the groups. In conclusion, contrary to what some believe, even though durian was found to increase body temperature in some rats, this increment was not significant. Rats receiving the durian-paracetamol combination showed a significant drop in body temperature, which may explain the belief that the two mixtures are toxic. However, the exact mechanism of toxicity is still unknown.
    Matched MeSH terms: Asia, Southeastern
  2. Duong CN, Ra JS, Cho J, Kim SD, Choi HK, Park JH, et al.
    Chemosphere, 2010 Jan;78(3):286-93.
    PMID: 19931116 DOI: 10.1016/j.chemosphere.2009.10.048
    The effects of treatment processes on estrogenicity were evaluated by examining estradiol equivalent (EEQ) concentrations in influents and effluents of sewage treatment plants (STPs) located along Yeongsan and Seomjin rivers in Korea. The occurrence and distribution of estrogenic chemicals were also estimated for surface water in Korea and compared with seven other Asian countries including Laos, Cambodia, Vietnam, China, Indonesia, Thailand and Malaysia. Target compounds were nonylphenol (NP), octylphenol (OP), bisphenol A (BPA), estrone (E1), 17beta-estradiol (E2), 17alpha-ethynylestradiol (EE2) and genistein (Gen). Water samples were pretreated and analyzed by liquid-liquid extraction (LLE) and gas chromatography/mass spectrometry (GC/MS). The results showed that the treatment processes of Korean STPs were sufficient to reduce the estrogenic activity of municipal wastewater. The concentrations of phenolic xenoestrogens (i.e., NP, OP and BPA) in samples of Yeongsan and Seomjin rivers were smaller than those reported by previous studies in Korea. In most samples taken from the seven Asian countries, the presence of E2 and EE2 was a major contributor toward estrogenic activity. The EEQ concentrations in surface water samples of the seven Asian countries were at a higher level in comparison to that reported in European countries, America and Japan. However, further studies with more sampling frequencies and sampling areas should be carried out for better evaluation of the occurrence and distribution of estrogenic compounds in these Asian countries.
    Matched MeSH terms: Asia
  3. Nguyen QT, Naguib RN, Abd Ghani MK, Bali RK, Lee IM
    Int J Electron Healthc, 2008;4(2):184-207.
    PMID: 18676343
    This paper presents an overview of the healthcare systems in Southeast Asia, with a focus on the healthcare informatics development and deployment in seven countries, namely, Singapore, Cambodia, Malaysia, Thailand, Laos, the Philippines and Vietnam. Brief geographic and demographic information is provided for each country, followed by a historical review of the national strategies for healthcare informatics development. An analysis of the state-of-the-art healthcare infrastructure is also given, along with a critical appraisal of national healthcare provisions.
    Matched MeSH terms: Asia, Southeastern
  4. Wong JS, Tan F, Lee PY
    Asia Pac J Public Health, 2007;19(3):16-21.
    PMID: 18330400 DOI: 10.1177/101053950701900304
    Achieving treatment targets has been difficult in treating diabetic patients. This cross-sectional study describes the lipid profiles of patients with diabetes mellitus at a public primary health care centre in Sarawak, Malaysia. The targets for lipid control were based on the International Diabetes Federation recommendation (2002). 1031 patients (98% Type 2 Diabetes) were studied. Fasting lipid profiles were available in 990 (96%) patients. The mean total cholesterol was 5.3 +/- 1.0 mmol/L, Triglycerides 1.90 +/- 1.26 mmol/L, HDL-C 1.28 +/- 0.33 mmol/L and LDL-C 3.2 +/- 0.9 mmol/L. Overall, 22% of patients achieved the treatment target for LDL-C level < 2.6mmol/L. 67% of patients had HDL-C > 1.1 mmol/L and 42% of patients had a target TG level below 1.5 mmol/L. Of the 40% of patients who received lipid-lowering drug, 17% achieved LDL-C target, 50% had LDL-C 2.6-4.4 mmol/ L and 33% have LDL-C > 4.0 mmol/L. For the remaining 60% not receiving any lipid lowering therapy, 68% had LDL-C between 2.6-4.0 mmol/L and 7% had LDL-C level > 4 mmol/L. Dyslipidemia is still under-treated despite the availability of effective pharmacological agents and the greatly increased risk of cardiovascular diseases in diabetic patients.
    Matched MeSH terms: Asia
  5. Kim HS, Wu Y, Lin SJ, Deerochanawong C, Zambahari R, Zhao L, et al.
    Curr Med Res Opin, 2008 Jul;24(7):1951-63.
    PMID: 18547466 DOI: 10.1185/03007990802138731
    BACKGROUND: Data on achieving National Cholesterol Education Program Adult Treatment Panel III (ATP III) goals in Asia are limited.

    OBJECTIVE: To examine treatment patterns, goal attainment, and factors influencing treatment among patients in 6 Asian countries who were taking statins.

    METHODS: A retrospective cohort study was conducted in China, Korea, Malaysia, Singapore, Taiwan, and Thailand, where 437 physicians (41% cardiologists) recruited adults with hypercholesterolemia newly initiated on statin monotherapy.

    RESULTS: Of 2622 patients meeting inclusion and exclusion criteria, approximately 66% had coronary heart disease (CHD)/diabetes mellitus, 24% had no CHD but > or =2 risk factors, and 10% had no CHD and <2 risk factors. Most patients ( approximately 90%) received statins at medium or lower equipotency doses. Across all cardiovascular risk categories, 48% of patients attained ATP III targets for low-density lipoprotein cholesterol (LDL-C), including 38% of those with CHD/diabetes (goal: <100 mg/dL), 62% of those without CHD but with > or =2 risk factors (goal: <130 mg/dL), and 81% of those without CHD and <2 risk factors (goal: <160 mg/dL). Most patients who achieved goals did so within the first 3 months. Increasing age (odds ratio (OR)=1.015 per 1-year increment; 95% confidence interval (CI)=1.005-1.206; p=0.0038) and initial statin potency (OR=2.253; 95% CI=1.364-3.722; p=0.0015) were directly associated with goal attainment, whereas increased cardiovascular risk (OR=0.085; 95% CI=0.053-0.134; p<0.0001 for CHD/diabetes mellitus at baseline compared with <2 risk factors,) and baseline LDL-C (OR=0.990; 95% CI=0.987-0.993); p<0.0001 per 1-mg/dL increment) were inversely associated with LDL-C goal achievement. Limitations of this study include potential differences in treatment settings and cardiovascular risk factors between different countries and centers. In addition, the effects on cholesterol goal achievement of concomitant changes in lifestyle were not assessed.

    CONCLUSION: LDL-C goal attainment is low in Asians, particularly those with CHD/diabetes. More effective patient monitoring, treatments, including combining regimens and dose titration, and adherence to these treatments along with therapeutic lifestyle counseling may facilitate goal attainment.

    Matched MeSH terms: Asia
  6. Tanabe S, Kunisue T
    Environ Pollut, 2007 Mar;146(2):400-13.
    PMID: 16949712
    In this paper, we concisely reviewed the contamination of persistent organic pollutants (POPs) such as polychlorinated dibenzo-p-dioxins (PCDDs), dibenzofurans (PCDFs), biphenyls (PCBs), dichlorodiphenyltrichloroethane and its metabolites (DDTs), hexachlorocyclohexane isomers (HCHs), chlordane compounds (CHLs), hexachlorobenzene (HCB) in human breast milk collected from Asian countries such as Japan, China, Philippines, Vietnam, Cambodia, India, Malaysia, and Indonesia during 1999-2003. Dioxins, PCBs, CHLs in Japanese, and DDTs in Vietnamese, Chinese, Cambodian, Malaysian, and HCHs in Chinese, Indian, and HCB in Chinese breast milk were predominant. In India, levels of dioxins and related compounds (DRCs) in the mothers living around the open dumping site were notably higher than those from the reference site and other Asian developing countries, indicating that significant pollution sources of DRCs are present in the dumping site of India and the residents there have been exposed to relatively higher levels of these contaminants possibly via bovine milk.
    Matched MeSH terms: Asia
  7. Agusa T, Kunito T, Sudaryanto A, Monirith I, Kan-Atireklap S, Iwata H, et al.
    Environ Pollut, 2007 Feb;145(3):766-77.
    PMID: 16828209
    Concentrations of 20 trace elements were determined in muscle and liver of 34 species of marine fish collected from coastal areas of Cambodia, Indonesia, Malaysia and Thailand. Large regional difference was observed in the levels of trace elements in liver of one fish family (Carangidae): the highest mean concentration was observed in fish from the Malaysian coastal waters for V, Cr, Zn, Pb and Bi and those from the Java Sea side of Indonesia for Sn and Hg. To assess the health risk to the Southeast Asian populations from consumption of fish, intake rates of trace elements were estimated. Some marine fish showed Hg levels higher than the guideline values by U.S. Environmental Protection Agency and Joint FAO/WHO Expert Committee on Food Additives (JECFA). This suggests that consumption of these fish may be hazardous to the people.
    Matched MeSH terms: Asia, Southeastern
  8. Gibson RS, Cavalli-Sforza T
    Food Nutr Bull, 2012 Sep;33(3 Suppl):S214-20.
    PMID: 23193773
    Collection of nationwide food consumption data at the individual level is the preferred option for planning fortification programs. However, such data are seldom collected in low-income countries. In contrast, Food Balance Sheets (FBS), published annually for approximately 180 countries, may provide a source of national data for program planning.
    Matched MeSH terms: Asia, Southeastern
  9. Md Ralib AR, Ariff AR, Shuaib IL, Naing NN, George PJ, Abdullah JM
    PMID: 15691152
    A cross-sectional study was conducted to predict the outcome in patients with subdural empyema, using initial and post-treatment CT scan brain parameters. Data collection was done on those children who were diagnosed to have subdural empyema by CT scan of the brain with contrast, who underwent burrhole evacuation, from February 2000 until April 2002. Numerous factors, such as coma or loss of unconsciousness at diagnosis, age, types of antibiotic, microbiology, extension of empyema, associated cerebral infarction and ventriculitis, were analyzed. Poor prognosis was associated with loss of consciousness, and hypodensity by CT scan at presentation (p < 0.005). Patients with an extensive subdural empyema will have a good outcome if they are treated early and aggressively with antibiotics and burrhole evacuation.
    Matched MeSH terms: Asia, Southeastern
  10. Macaulay V, Hill C, Achilli A, Rengo C, Clarke D, Meehan W, et al.
    Science, 2005 May 13;308(5724):1034-6.
    PMID: 15890885
    A recent dispersal of modern humans out of Africa is now widely accepted, but the routes taken across Eurasia are still disputed. We show that mitochondrial DNA variation in isolated "relict" populations in southeast Asia supports the view that there was only a single dispersal from Africa, most likely via a southern coastal route, through India and onward into southeast Asia and Australasia. There was an early offshoot, leading ultimately to the settlement of the Near East and Europe, but the main dispersal from India to Australia approximately 65,000 years ago was rapid, most likely taking only a few thousand years.
    Matched MeSH terms: Asia; Australasia
  11. Kawamura M, Wright FA, Declerck D, Freire MC, Hu DY, Honkala E, et al.
    Int Dent J, 2005 Aug;55(4):205-11.
    PMID: 16167608
    To identify similarities and differences in oral health attitudes, behaviour and values among freshman dental students.
    Matched MeSH terms: Asia
  12. Matsui M, Shimada T, Ota H, Tanaka-Ueno T
    Mol Phylogenet Evol, 2005 Dec;37(3):733-42.
    PMID: 15964212
    The genus Rana, notably diversified in Oriental regions from China to Southeast Asia, includes a group of cascade frogs assigned to subgenera Odorrana and Eburana. Among them, R. ishikawae and the R. narina complex represent the northernmost members occurring from Taiwan to the Ryukyu Archipelago of Japan. Relationships of these frogs with the continental members, as well as the history of their invasions to islands, have been unclear. The taxonomic status of Odorrana and related genera varies among authors and no phylogenetic reassessment has been done. Using partial sequences of mitochondrial 12S and 16S rRNA genes, we estimated phylogenetic relationships among 17 species of the section Hylarana including Odorrana and Eburana, and related species from the Ryukyus, Taiwan, China, Thailand, Malaysia, and Indonesia. We estimate that (1) Odorrana is monophyletic and encompasses species of Eburana and R. hosii, which is now placed in Chalcorana, (2) the ancestor of R. ishikawae separated from other Rana in the middle to late Miocene prior to its entry to the Ryukyu Archipelago, (3) the ancestor of the R. narina complex later diversified in continental Asia, and invaded the Ryukyu Archipelago through Taiwan, (4) the R. narina complex attained its current distribution within the Ryukyus through niche segregations, and (5) vicariance of R. hosii between Malay Peninsula and Borneo occurred much later than the divergence events in the R. narina complex. Current subgeneric classification of Rana, at least of Southeast Asian members, requires full reassessment in the light of phylogenetic relationships.
    Matched MeSH terms: Asia, Southeastern
  13. Rosli R, Tan MP, Gray WK, Subramanian P, Chin AV
    Int Psychogeriatr, 2016 Feb;28(2):189-210.
    PMID: 26450414 DOI: 10.1017/S1041610215001635
    The prevalence of dementia is increasing in Asia than in any other continent. However, the applicability of the existing cognitive assessment tools is limited by differences in educational and cultural factors in this setting. We conducted a systematic review of published studies on cognitive assessments tools in Asia. We aimed to rationalize the results of available studies which evaluated the validity of cognitive tools for the detection of cognitive impairment and to identify the issues surrounding the available cognitive impairment screening tools in Asia.
    Matched MeSH terms: Asia
  14. Tay E, Muda N, Yap J, Muller DW, Santoso T, Walters DL, et al.
    Catheter Cardiovasc Interv, 2016 Jun;87(7):E275-81.
    PMID: 26508564 DOI: 10.1002/ccd.26289
    OBJECTIVES: The objective of this study is to describe and compare the use of the MitraClip therapy in mitral regurgitation (MR) patients with degenerative MR (DMR) and functional MR (FMR).

    INTRODUCTION: Percutaneous edge-to-edge repair of severe MR using the MitraClip device is approved for use in the USA for high risk DMR while European guidelines include its use in FMR patients as well.

    METHODS: The MitraClip in the Asia-Pacific Registry (MARS) is a multicenter retrospective registry, involving eight sites in five Asia-Pacific countries. Clinical and echocardiographic characteristics, procedural outcomes and 1-month outcomes [death and major adverse events (MAE)] were compared between FMR and DMR patients treated with the MitraClip.

    RESULTS: A total of 163 patients were included from 2011 to 2014. The acute procedural success rates for FMR (95.5%, n = 84) and DMR (92%, n = 69) were similar (P = 0.515). 45% of FMR had ≥2 clips inserted compared to 60% of those with DMR (P = 0.064).The 30-day mortality rate for FMR and DMR was similar at 4.5% and 6.7% respectively (P = 0.555). The 30-day MAE rate was 9.2% for FMR and 14.7% for DMR (P = 0.281). Both FMR and DMR patients had significant improvements in the severity of MR and NYHA class after 30 days. There was a significantly greater reduction in left ventricular end-diastolic diameter (P = 0.002) and end systolic diameter (P = 0.017) in DMR than in FMR.

    CONCLUSIONS: The MitraClip therapy is a safe and efficacious treatment option for both FMR and DMR. Although, there is a significantly greater reduction in LV volumes in DMR, patients in both groups report clinical benefit with improvement in functional class. © 2015 Wiley Periodicals, Inc.

    Matched MeSH terms: Asia
  15. Baek JY, Kang CI, Kim SH, Ko KS, Chung DR, Peck KR, et al.
    Diagn Microbiol Infect Dis, 2016 Jun;85(2):218-20.
    PMID: 27083121 DOI: 10.1016/j.diagmicrobio.2016.02.022
    Tedizolid phosphate is a second-generation oxazolidinone prodrug that is potential activity against a wide range of Gram-positive pathogens, including methicillin-resistant Staphylococcus aureus, penicillin-resistant streptococci, and vancomycin-resistant enterococci. The in vitro activity of tedizolid and other comparator agents against multidrug-resistant (MDR) pneumococci from various Asian countries were evaluated. Of the S. pneumoniae clinical pneumonia isolates collected during 2008 and 2009 from 8 Asian countries (Korea, Taiwan, Thailand, Hong Kong, Vietnam, Malaysia, Philippines, and Sri Lanka), 104 isolates of MDR pneumococci were included in this study. Antimicrobial susceptibility testing for 18 antimicrobial agents was performed by broth microdilution method. Tedizolid was highly active against pneumococci. All isolates tested were inhibited at a tedizolid minimum inhibitory concentration (MIC) value of ≤0.25μg/ml (ranged from ≤0.03μg/ml to 0.25μg/ml). The MIC50 and MIC90 of tedizolid against MDR pneumococci were both 0.12μg/ml, while MIC50 and MIC90 of linezolid were 0.5μg/ml and 1μg/ml, respectively. In addition, tedizolid maintained the activity against S. pneumoniae regardless of the extensively drug-resistant (XDR) phenotype of the isolates. The activity of tedizolid was excellent against all types of MDR pneumococci, exhibiting and maintaining at least 4-fold-greater potency compared to linezolid, regardless of resistance phenotypes to other commonly utilized agents. Tedizolid has the potential to be an agent to treat infections caused by MDR pneumococci in the Asia.
    Matched MeSH terms: Asia
  16. Kumar T, Abd Majid MA, Onichandran S, Jaturas N, Andiappan H, Salibay CC, et al.
    Infect Dis Poverty, 2016 Jan 13;5:3.
    PMID: 26763230 DOI: 10.1186/s40249-016-0095-z
    Access to clean and safe drinking water that is free from pathogenic protozoan parasites, especially Cryptosporidium parvum and Giardia lamblia that cause gastrointestinal illness in humans, is still an issue in Southeast Asia (SEA). This study is the first attempt to detect the aforementioned protozoan parasites in water samples from countries in SEA, using real-time polymerase chain reaction (qPCR) assays.
    Matched MeSH terms: Asia, Southeastern
  17. Han CT, Flaherty G
    J Travel Med, 2015 Sep-Oct;22(5):312-7.
    PMID: 26095866 DOI: 10.1111/jtm.12221
    BACKGROUND: Patients with complex medical comorbidities travel for protracted periods to remote destinations, often with limited access to medical care. Few descriptions are available of their preexisting health burden. This study aimed to characterize preexisting medical conditions and medications of travelers seeking pre-travel health advice at a specialized travel medicine clinic.
    METHODS: Records of travelers attending the Galway Tropical Medical Bureau clinic between 2008 and 2014 were examined and information relating to past medical history was entered into a database. Data were recorded only where the traveler had a documented medical history and/or was taking medications.
    RESULTS: Of the 4,817 records available, 56% had a documented medical history and 24% listed medications. The majority of travelers with preexisting conditions were female. The mean age of the cohort was 31.68 years. The mean period remaining before the planned trip was 40 days. Southeast Asia was the most popular single destination, and 17% of travelers with medical conditions were traveling alone. The most frequently reported conditions were allergies (20%), insect bite sensitivity (15%), asthma (11%), psychiatric conditions (4%), and hypertension (3%). Of the 30 diabetic travelers, 14 required insulin; 4.5% of travelers were taking immunosuppressant drugs, including corticosteroids. Half of the female travelers were taking the oral contraceptive pill while 11 travelers were pregnant at the time of their pre-travel consultation.
    CONCLUSIONS: This study provides an insight into the medical profile of travelers attending a travel health clinic. The diverse range of diseases reported highlights the importance of educating physicians and nurses about the specific travel health risks associated with particular conditions. Knowledge of the effects of travel on underlying medical conditions will inform the pre-travel health consultation.
    Matched MeSH terms: Asia
  18. Guadamuz TE, Cheung DH, Wei C, Koe S, Lim SH
    PLoS One, 2015;10(5):e0126658.
    PMID: 25973907 DOI: 10.1371/journal.pone.0126658
    BACKGROUND: Poor HIV testing uptake by MSM may be attributable to unique challenges that are localized in Southeast Asia.

    OBJECTIVE: To characterize MSM who never tested for HIV, to identify correlates of never testing, and to elucidate the perceived barriers to HIV testing.

    METHODS: The present study used data from the Asian Internet MSM Sex Survey (AIMSS) and restricted the analysis to 4,310 MSM from the ten member countries of the Association of South East Asian Nations (ASEAN).

    RESULTS: Among MSM participants from ASEAN in our sample, 1290 (29.9%) reported having never been tested for HIV, 471 (10.9%) tested for HIV more than 2 years ago, and 2186 (50.7%) reported their last test date was between 6 months and two years ago, with only 363 (8.4%) of these men having been tested in the past 6 months. In multivariable logistic regression, younger MSM (age 15-22 years old [AOR: 4.60, 95% CI: 3.04-6.96]), MSM with lower education (secondary school or lower [AOR: 1.37, 95% CI: 1.03-1.83]), MSM who identify as bisexual or heterosexual (compared to gay-identified) (AOR: 1.94, 95% CI: 1.60-2.35), and MSM who had never used a condom with male partners (AOR: 1.61, 95% CI: 1.32-1.97) had higher odds of never been HIV tested. Main reason for not being tested was a low risk perception of HIV exposure (n = 390, 30.2%).

    CONCLUSION: Current HIV prevention response must not leave MSM "in the dark," but instead meet them where they are by utilizing the Internet creatively through social media and smart phones. As ASEAN Economic Community (AEC) is quickly becoming a reality, so must there be an equally fast and united response to slowing down the HIV epidemics among MSM in ASEAN.

    Matched MeSH terms: Asia, Southeastern
  19. Jiamsakul A, Chaiwarith R, Durier N, Sirivichayakul S, Kiertiburanakul S, Van Den Eede P, et al.
    J Med Virol, 2016 Feb;88(2):234-43.
    PMID: 26147742 DOI: 10.1002/jmv.24320
    HIV drug resistance assessments and interpretations can be obtained from genotyping (GT), virtual phenotyping (VP) and laboratory-based phenotyping (PT). We compared resistance calls obtained from GT and VP with those from PT (GT-PT and VP-PT) among CRF01_AE and subtype B HIV-1 infected patients. GT predictions were obtained from the Stanford HIV database. VP and PT were obtained from Janssen Diagnostics BVBA's vircoType(TM) HIV-1 and Antivirogram®, respectively. With PT assumed as the "gold standard," the area under the curve (AUC) and the Bland-Altman plot were used to assess the level of agreement in resistance interpretations. A total of 80 CRF01_AE samples from Asia and 100 subtype B from Janssen Diagnostics BVBA's database were analysed. CRF01_AE showed discordances ranging from 3 to 27 samples for GT-PT and 1 to 20 samples for VP-PT. The GT-PT and VP-PT AUCs were 0.76-0.97 and 0.81-0.99, respectively. Subtype B showed 3-61 discordances for GT-PT and 2-75 discordances for VP-PT. The AUCs ranged from 0.55 to 0.95 for GT-PT and 0.55 to 0.97 for VP-PT. Didanosine had the highest proportion of discordances and/or AUC in all comparisons. The patient with the largest didanosine FC difference in each subtype harboured Q151M mutation. Overall, GT and VP predictions for CRF01_AE performed significantly better than subtype B for three NRTIs. Although discrepancies exist, GT and VP resistance interpretations in HIV-1 CRF01_AE strains were highly robust in comparison with the gold-standard PT.
    Matched MeSH terms: Asia
  20. Choy YS, Bhattacharya K, Balasubramaniam S, Fietz M, Fu A, Inwood A, et al.
    Mol Genet Metab, 2015 May;115(1):41-7.
    PMID: 25892708 DOI: 10.1016/j.ymgme.2015.03.005
    Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is caused by deficient activity of the enzyme, N-acetylgalactosamine-4-sulfatase, resulting in impaired degradation of the glycosaminoglycan dermatan sulfate. Patients experience a range of manifestations including joint contractures, short stature, dysostosis multiplex, coarse facial features, decreased pulmonary function, cardiac abnormalities, corneal clouding and shortened life span. Recently, clinicians from institutions in the Asia-Pacific region met to discuss the occurrence and implications of delayed diagnosis and misdiagnosis of MPS VI in the patients they have managed. Eighteen patients (44% female) were diagnosed. The most common sign presented by the patients was bone deformities in 11 patients (65%). Delays to diagnosis occurred due to the lack of or distance to diagnostic facilities for four patients (31%), alternative diagnoses for two patients (15%), and misleading symptoms experienced by two patients (15%). Several patients experienced manifestations that were subtler than would be expected and were subsequently overlooked. Several cases highlighted the unique challenges associated with diagnosing MPS VI from the perspective of different specialties and provide insights into how these patients initially present, which may help to elucidate strategies to improve the diagnosis of MPS VI.
    Matched MeSH terms: Asia
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