Displaying publications 41 - 60 of 103 in total

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  1. Xiao B, Deng X, Ng EY, Allen JC, Lim SY, Ahmad-Annuar A, et al.
    JAMA Neurol, 2018 01 01;75(1):127-128.
    PMID: 29131875 DOI: 10.1001/jamaneurol.2017.3363
    Matched MeSH terms: Age of Onset
  2. Manonmani V, Tan CT
    Singapore Med J, 1999 Jan;40(1):32-5.
    PMID: 10361483
    To determine the characteristics of newly diagnosed epilepsy in the multiracial population of Malaysia.
    Matched MeSH terms: Age of Onset
  3. Kareem BA, Das PK, Saad R
    Singapore Med J, 1994 Oct;35(5):527-8.
    PMID: 7701379
    Disappearing bone disease is a rare condition and usually affects young adults. Its aetiology is not known. A case of a 12-year-old female child, who had a fall and sustained a fracture mid shaft of femur and supracondylar region, is reported. On follow-up subsequent X-ray showed extensive osteolysis which was progressively affecting the other side of the pelvic girdle and femur. She was put on trial of calcitonin 50 IU by nasal spray for six months but there was no improvement. However, during the past three and half years the disease process had progressively extended to the other side of pelvic girdle and femur with fatal outcome. To the knowledge of the authors such progression in a short time has not been reported in any case so far.
    Matched MeSH terms: Age of Onset
  4. Manonmani V, Tan CT
    Singapore Med J, 1993 Oct;34(5):378-80.
    PMID: 8153678
    This is a report on 11 cases of Juvenile Myoclonic Epilepsy (JME) from the University Hospital, Kuala Lumpur, all of whom were diagnosed in the last one and a half years. This genetic syndrome is seen in all the three main racial groups: Chinese, Malays and Indians. It accounts for 2% of the epilepsy patients seen at the neurology clinic. Lack of awareness is the main hindrance to diagnosis.
    Study site: Neurology clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
    Matched MeSH terms: Age of Onset
  5. Alowayesh MS, Ahmed SF, Al-Hashel J, Alroughani R
    PLoS One, 2019;14(5):e0216646.
    PMID: 31086393 DOI: 10.1371/journal.pone.0216646
    BACKGROUND: Multiple Sclerosis (MS) is a chronic neurological disease with heavy economic and social burdens resulting in significant disability.

    OBJECTIVE: This study aims to (1) measure the cost of health resources utilization by MS patients and (2) to examine the difference in utilization and its attributed costs amongst patients who may have a different course of MS and expanded disability status scale (EDSS) scores.

    METHODS: A cross-sectional study using Kuwait National MS registry was conducted to estimate the costs of utilization of resources from 2011 to 2015.

    RESULTS: Between the period 2011-2015, 1344 MS patients were included in the registry. The average annual cost per MS patient has increased from $10,271 in 2011 to $17,296 in 2015. Utilization of disease-modifying therapies (DMTs) was the main driver of costs reaching 89.9% in 2015. Throughout the five-year period, the occurrence of relapses decreased from 21.8% to 12.2% (p <0.0001). During this same period, ambulatory relapse treatment increased by 5.8% while hospitalizations decreased by 2.6%. Patients with a moderate EDSS score (3.5-6) had the highest average cost (p<0.0001) compared to mild and severe EDSS scores.

    CONCLUSIONS: Multiple sclerosis has been a significant economic burden on the Kuwait healthcare system. DMTs are the main driver of cost.

    Matched MeSH terms: Age of Onset
  6. Moghaddam HM, Esfehani RJ, Panah NY, Esfehani AJ
    Ann Saudi Med, 2014 6 5;34(2):147-52.
    PMID: 24894784 DOI: 10.5144/0256-4947.2014.147
    BACKGROUND AND OBJECTIVES: The rate of consanguineous marriage is high in Middle Eastern countries such as Iran. The relationship between consanguineous marriage and congenital heart disease is discussed in some studies, but there is not much data for relationship between atrial septal defect (ASD) and consanguineous marriage. The aim of this study was to evaluate the relationship between consanguineous marriage and ASD echocardiographic characteristics.

    DESIGN AND SETTINGS: This was a cross-sectional study approved by Mashhad University of Medical Sciences ethics committee and took place in Mashhad, Iran, for a period of 3 years from August 2008 till September 2011.

    METHODS: In this cross-sectional study, 113 ASD patients participated and they were categorized into 3 groups on the basis of family relationship between their parents: first group-"no relationship," second group- "third degree relationship," and third group- "far relationship."

    RESULTS: Among the 54 male and 59 female ASD patients, the most prevalent type of ASD was ASD secundum (85.0%) followed by sinus venosus (8.8%). A total of 56% patients were present in the first group and 15% and 29% in the second group and the third group, respectively." The relationship between consanguinity and type of ASD (P < .001) and gender (P < .001 each) was observed. The relationship between the age of onset of disease and consanguinity (P=.003) was also observed.

    CONCLUSION: Considering the fact that there is a high prevalence of ASD and consanguineous marriage in Iran and bearing in mind the results of the present study, we recommend educating couples about the outcomes of consanguineous marriage in pre-marriage counseling.

    Matched MeSH terms: Age of Onset
  7. Chalhoub NE, Wenderfer SE, Levy DM, Rouster-Stevens K, Aggarwal A, Savani SI, et al.
    Arthritis Rheumatol, 2022 Feb;74(2):263-273.
    PMID: 34279063 DOI: 10.1002/art.41930
    OBJECTIVE: To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology.

    METHODS: Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2). Physicians rated changes in renal and extrarenal childhood-onset SLE activity between 2 consecutive visits and proposed CS dosing (step 3). The SSR was developed using patient profile ratings (step 4), with refinements achieved in a physician focus group (step 5). A second type of patient profile describing the course of childhood-onset SLE for ≥4 months since kidney biopsy was rated to validate the SSR-recommended oral and intravenous (IV) CS dosages (step 6). Patient profile adjudication was based on majority ratings for both renal and extrarenal disease courses, and consensus level was set at 80%.

    RESULTS: Degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and time since kidney biopsy influenced CS dosing (steps 1 and 2). Considering these parameters in 5,056 patient profile ratings from 103 raters, and renal and extrarenal course definitions, CS dosing rules of the SSR were developed (steps 3-5). Validation of the SSR for up to 6 months post-kidney biopsy was achieved with 1,838 patient profile ratings from 60 raters who achieved consensus for oral and IV CS dosage in accordance with the SSR (step 6).

    CONCLUSION: The SSR represents an international consensus on CS dosing for use in patients with childhood-onset SLE and proliferative LN. The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials.

    Matched MeSH terms: Age of Onset
  8. Isah Tsamiya R, Mohd Nafi SN, Che Jalil NA, Mat Zin AA
    Malays J Pathol, 2024 Aug;46(2):233-246.
    PMID: 39207001
    INTRODUCTION: Young onset colorectal cancer (YOCRC) is a heterogenous CRC phenotype with an increasing trend globally. This study aims to determine FOXP3+ Treg cells, Mismatch Repair (MMR) proteins, and proto-oncogene B-Raf (BRAF) V600E status among YOCRC patients at Hospital Universiti Sains Malaysia.

    MATERIALS AND METHODS: This was a retrospective study of YOCRC (<50 years) over 8 years (January 2013 to December 2021). Immunohistochemistry staining of FOXP3, BRAFV600E, and MMR protein expression was performed using monoclonal antibodies. The staining intensity and percentage of positive cells were used to evaluate the staining using immunoreactive scoring. All data were analysed using descriptive and correlation statistics. A p-value of ≤ 0.05 was taken as statistically significant.

    RESULTS: A total of 65 YOCRC patients were diagnosed, out of which 53.8% had proficient MMR (pMMR) with a mean age of 41, while 46.2% had deficient MMR (dMMR) with a mean age of 35.5. The pMMR with the BRAFV600E+ group expressed higher FOXP3+Tregs (54.2%) than the dMMR with the BRAFV600E+ group (22.9%). Patients with lower FOXP3+Tregs were observed more in dMMR with BRAFV600E- (47%) than in pMMR with BRAFV600E- (5.9%). There was a statistically significant association between the density of expressed FOXP3+Tregs with MMR and BRAFV600E status (p=0.002).

    CONCLUSION: While most of the YOCRC had pMMR, others exhibited dMMR with loss of one or more MMR proteins. The presence of BRAFV600E demonstrated the YOCRC's sporadic nature. A high FOXP3+Treg expression was significantly associated with MMR and BRAFV600E status. Future research must be expanded to cover other hospitals to increase the sample size and include MLH1 hypermethylation testing.

    Matched MeSH terms: Age of Onset
  9. Tohyama J, Nakashima M, Nabatame S, Gaik-Siew C, Miyata R, Rener-Primec Z, et al.
    J Hum Genet, 2015 Apr;60(4):167-73.
    PMID: 25631096 DOI: 10.1038/jhg.2015.5
    Recent progress in genetic analysis reveals that a significant proportion of cryptogenic epileptic encephalopathies are single-gene disorders. Mutations in numerous genes for early-onset epileptic encephalopathies have been rapidly identified, including in SPTAN1, which encodes α-II spectrin. The aim of this review is to delineate SPTAN1 encephalopathy as a distinct clinical syndrome. To date, a total of seven epileptic patients with four different in-frame SPTAN1 mutations have been identified. The major clinical features of SPTAN1 mutations include epileptic encephalopathy with hypsarrhythmia, no visual attention, acquired microcephaly, spastic quadriplegia and severe intellectual disability. Brainstem and cerebellar atrophy and cerebral hypomyelination, as observed by magnetic resonance imaging, are specific hallmarks of this condition. A milder variant is characterized by generalized epilepsy with pontocerebellar atrophy. Only in-frame SPTAN1 mutations in the last two spectrin repeats in the C-terminal region lead to dominant negative effects and these specific phenotypes. The last two spectrin repeats are required for α/β spectrin heterodimer associations and the mutations can alter heterodimer formation between the two spectrins. From these data we suggest that SPTAN1 encephalopathy is a distinct clinical syndrome owing to specific SPTAN1 mutations. It is important that this syndrome is recognized by pediatric neurologists to enable proper diagnostic work-up for patients.
    Matched MeSH terms: Age of Onset
  10. Chua KH, Lian LH, Khor WC, Lee WS, Hilmi I, Goh KL, et al.
    J Dig Dis, 2015 Apr;16(4):205-16.
    PMID: 25564941 DOI: 10.1111/1751-2980.12229
    The study aimed to investigate the association between the interferon regulatory factor 5 (IRF5) gene polymorphisms and the onset of Crohn's disease (CD) in a Malaysian cohort.
    Matched MeSH terms: Age of Onset
  11. Lim SY, Tan ZK, Ngam PI, Lor TL, Mohamed H, Schee JP, et al.
    Parkinsonism Relat Disord, 2011 Dec;17(10):761-4.
    PMID: 21839665 DOI: 10.1016/j.parkreldis.2011.07.009
    There are limited data on the prevalence of impulsive-compulsive behaviors and subsyndromal impulsive-compulsive behaviors in Asian patients with Parkinson's disease, who are treated with lower dosages of dopaminergic medications.
    Matched MeSH terms: Age of Onset
  12. Mustafa N, Kamarudin NA, Ismail AA, Khir AS, Ismail IS, Musa KI, et al.
    Diabetes Care, 2011 Jun;34(6):1362-4.
    PMID: 21498788 DOI: 10.2337/dc11-0005
    OBJECTIVE:
    To determine the prevalence of prediabetes and diabetes among rural and urban Malaysians.
    RESEARCH DESIGN AND METHODS:
    This cross-sectional survey was conducted among 3,879 Malaysian adults (1,335 men and 2,544 women). All subjects underwent the 75-g oral glucose tolerance test (OGTT).
    RESULTS:
    The overall prevalence of prediabetes was 22.1% (30.2% in men and 69.8% in women). Isolated impaired fasting glucose (IFG) and impaired glucose tolerance (IGT) were found in 3.4 and 16.1% of the study population, respectively, whereas 2.6% of the subjects had both IFG and IGT. Based on an OGTT, the prevalence of newly diagnosed type 2 diabetes was 12.6% (31.0% in men and 69.0% in women). The prediabetic subjects also had an increased prevalence of cardiovascular disease risk factors.
    CONCLUSIONS:
    The large proportion of undiagnosed cases of prediabetes and diabetes reflects the lack of public awareness of the disease.
    Matched MeSH terms: Age of Onset
  13. Razali R, Ahmad F, Rahman FN, Midin M, Sidi H
    Clin Neurol Neurosurg, 2011 Oct;113(8):639-43.
    PMID: 21684679 DOI: 10.1016/j.clineuro.2011.05.008
    Parkinson disease (PD) affects the lives of both the individuals and their family members. This study aims at investigating for clinical as well as socio-demographic factors associated with the perception of burden among the caregivers of individuals with PD in Malaysia.
    Matched MeSH terms: Age of Onset
  14. Teh CL, Kuan YC, Wong JS
    Rheumatol Int, 2009 Aug;29(10):1243-5.
    PMID: 19373465 DOI: 10.1007/s00296-009-0938-z
    We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.
    Matched MeSH terms: Age of Onset
  15. Hamid S, Yang YH, Peng KN, Ismail SM, Zain RB, Lim KP, et al.
    Oral Oncol, 2009 Jun;45(6):496-500.
    PMID: 18804411 DOI: 10.1016/j.oraloncology.2008.06.003
    The MDM2 SNP309 has been associated with increased expression of the protein which could suppress p53 function, and has been shown to modulate risk to cancer. We have previously shown that overexpression of MDM2 is a common event in oral cancers. In the present study, we determined the association between the MDM2 SNP309 polymorphism and oral cancer in 207 oral cancer patients and 116 normal subjects. We genotyped the MDM2 SNP309 by PCR-RFLP. Logistic regression was adapted to calculate odds ratios for MDM2 SNP309 polymorphism from univariate and multivariable adjusted models. Our results suggest that MDM2 SNP309 does not confer increased risk to oral cancer (OR=1.55, 95% CI=0.77-3.11). However, the GG/TG genotype was associated with later disease onset in women above 55 years of age. Collectively, our data suggests that MDM2 SNP309 may modulate the risk to oral cancer and is a modifier of the age at oral cancer onset in women above the age of 55 years.
    Matched MeSH terms: Age of Onset
  16. Thai AC, Mohan V, Khalid BA, Cockram CS, Pan CY, Zimmet P, et al.
    Diabetes Res Clin Pract, 2008 May;80(2):224-30.
    PMID: 18207602 DOI: 10.1016/j.diabres.2007.12.003
    In this paper, the islet autoimmunity status and relation to clinical characteristics, beta cell function and cardio-metabolic risk factors in young-onset Asian diabetic patients are evaluated at baseline. The study population consisted of 912 patients (from China, India, Malaysia and Singapore) with age 12-40 years and diabetes duration <12 months. Autoantibodies to glutamic acid decarboxylase (GADA) and tyrosine phosphatase (IA-2A), beta cell function and cardio-metabolic risk parameters were assessed. Among our young patient cohort, 105 (11.5%) patients were GADA and/or IA-2A positives (Ab +ve). Ab +ve patients were younger, leaner, had more severe hyperglycaemia and lower beta cell function. The frequency of metabolic syndrome was significantly lower in Ab +ve patients (27%) compared to Ab -ve patients (54%). However, a substantial proportion of patients in both groups of patients had atherogenic dyslipidaemia, hypertension and albuminuria (micro or macro). In our study cohort, only one in 10 Asian youth with new-onset diabetes had evidence of islet autoimmunity. At least 60% of Ab +ve and 50% of Ab -ve patients demonstrated classical features of type 1 and type 2 diabetes respectively. Regardless of autoimmunity status, the cardio-metabolic risk factors, in particular atherogenic dyslipidaemia, hypertension and albuminuria were common in our patients with young-onset diabetes.
    Matched MeSH terms: Age of Onset
  17. Chong VH, Telisinghe PU, Lim E, Abdullah MS, Idris F, Chong CF
    Asian Pac J Cancer Prev, 2015;16(16):7097-101.
    PMID: 26514496
    BACKGROUND: Nasopharyngeal carcinoma (NPC) is linked to Epstein Barr virus infection and is particularly common in the Far East, particularly among some Chinese groups. Certain ethnicities have been reported to have low incidence of NPC. This study looked at NPC in Brunei Darussalam over a three decade period.

    MATERIALS AND METHODS: The cancer registry from 1986 to 2014 maintained by the State Laboratory was retrospectively reviewed. The age standardized rates (ASR) and the age specific incidence rates (ASIR) were calculated. Non NPC tumors were excluded from the study.

    RESULTS: Altogether, there were a total of 450 NPC cases diagnosed accounting for 4.4% of all total cancer cases over the study period, declining from 10.3% in 1986-1990 to 2.3% in 2011-2014. The most common tumor type was the undifferentiated carcinoma (96.4%). The case characteristics were mean age 50.4 ± 14.4 years old, male 69%, and predominately Malays 74.4%, followed by Chinese 16.7%. The mean age of diagnosis increased over the study period from 45.6 ± 17.1 years (1986-1989) to 54.1 ± 12.5 years (ANOVA, p<0.01 for trend). There were no differences in the mean age of diagnosis between the ethnic groups or genders. The ASR showed a declining trend from 11.1 per 100,000 in 1986-1990 to 5.95 per 100,000 in 2011-2014, similar trends been observedfor both genders. Among the age groups, declining trends were seen in all the other age groups apart from the >70 years group. The overall ASRs for the Malays and Chinese were 7.92/100,000 and 8.83/100,000 respectively, both showing declining trends.

    CONCLUSIONS: The incidence of NPC in Brunei Darussalam is comparable to rates reported from Singapore and Malaysia, but higher than rates reported from the other Southeast Asian nations. Unlike higher rates reported for Chinese compared to the Malays in other countries, the rates between the Malays and Chinese in our study was comparable. Importantly, the ASR is declining overall and for both genders and ethnic groups.

    Matched MeSH terms: Age of Onset
  18. Syed Nong Chek SR, Robinson S, Mohd Affandi A, Baharum N
    Int J Dermatol, 2016 Oct;55(10):1092-5.
    PMID: 27061170 DOI: 10.1111/ijd.13307
    BACKGROUND: Psoriasis involving the face is visible and can cause considerable emotional distress to patients. Its presence may also confer a poorer prognosis for the patient. This study sought to evaluate the characteristics of facial psoriasis in Malaysia.

    METHODS: A cross-sectional study conducted using data from the Malaysian Psoriasis Registry from 2007 to 2011. Specific risk factors, i.e., age, age of onset, gender, duration of disease, obesity group, body surface area, Dermatology Life Quality Index (DLQI), family history of psoriasis, nail involvement, psoriatic arthritis, phototherapy, systemic therapy, clinic visit, days of work/school, and hospital admission due to psoriasis in the last 6 months were analyzed.

    RESULTS: A total of 48.4% of patients had facial psoriasis. Variables significantly associated with facial psoriasis are younger age, younger age of onset of psoriasis of ≤ 40 years, male, severity of psoriasis involving >10% of the body surface area, higher DLQI of >10, nail involvement, and history of hospitalization due to psoriasis.

    CONCLUSION: This study found that facial psoriasis is not as rare as previously thought. Ambient ultraviolet light, sebum, and contact with chemicals from facial products may reduce the severity of facial psoriasis, but these factors do not reduce the prevalence of facial psoriasis. The association with younger age, younger age of onset, higher percentage of body surface area involvement, higher DLQI of > 10, nail involvement, and hospitalization due to psoriasis support the notion that facial psoriasis is a marker of severe disease.

    Matched MeSH terms: Age of Onset
  19. Rajagopal R, Ariffin H, Krishnan S, Abdullah WA, Lin HP
    J Pediatr Hematol Oncol, 2015 Jul;37(5):391-5.
    PMID: 25929614 DOI: 10.1097/MPH.0000000000000344
    Review of the management of 6 young girls with vaginal yolk sac tumor over 25 years showed that the α-fetoprotein levels normalized in 5/6 within 4 cycles of primary cisplatin, bleomycin, etoposide (PEB)/carboplatin, etoposide, bleomycin (JEB)/cisplatin, vinblastine, bleomycin (PVB) chemotherapy. Radioimaging revealed residual tissue but viable tumor was found in only 1 of 2 biopsied. Resection/biopsy is necessary to avoid giving additional primary chemotherapy or to identify patients who need different treatment. If markers do not decay appropriately, PEB/JEB/PVB chemotherapy should not be continued. Taxol-containing salvage chemotherapy regimens, adjuvant modern radiotherapeutic treatment, and fertility-saving curative surgery should then be considered. Despite having mostly advanced disease, 5/6 patients were cured, 2 with chemotherapy alone.
    Matched MeSH terms: Age of Onset
  20. Wang F, Wang CL, Tan CT, Manivasagar M
    Lupus, 1997;6(3):248-53.
    PMID: 9104731 DOI: 10.1177/096120339700600306
    The aims of this study were to examine the clinical and laboratory features of Malaysian patients with systemic lupus erythematosus (SLE) and to identify any difference in disease expression between the different genders and among the three major ethnic groups of Malaysia. Retrospective analysis of all patients with SLE admitted to and followed-up at University Hospital Kuala Lumpur from 1974-90 was undertaken. Ethnic Chinese had the highest prevalence of SLE compared to other ethnic groups. There was a high incidence of renal disease, 74% of patient had significant proteinuria and half of these had associated nephrotic syndrome. Indian patients had significantly less incidence of skin manifestation compared to other racial groups. No difference in disease expression was detected between the ethnic Chinese and Indians and between the male and female patients. The overall 5 y and 10 y survival rates were 82% and 70% respectively. Indian patients had the poorest survival rates. Survival rates are similar among the Chinese and Malay patients. Our findings are in broad agreement with those previously reported.
    Matched MeSH terms: Age of Onset
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