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.
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.
METHODS: This was a cross-sectional study carried out from September 2016 to August 2017 at three tertiary hospitals in Northern Peninsular Malaysia.
RESULTS: A total of 62 patients were recruited, 83.9% of whom were male. The mean age was 29.2 with the median age of onset at 18 years old. The median duration of delay in diagnosis was 3 years. A quarter of them had positive family history. Nearly three-quarters were overweight and obese. About 12/62 (19.4%) had MetS, and it was comparable to healthy controls (15/62, 24.2%). HS patients had a significant higher risk of low-high-density lipoprotein (HDL) and obesity. Based on Hurley staging, 15/62 (24.2%) were in stage I, 38/62 (61.3%) and 9/62 (14.5%) in stages II and III, respectively. However, sonographic scoring showed 50% had severe stage of disease, and 56.9% of the patients had subclinical lesions. There was only a fair agreement between ultrasonography and Hurley staging of disease severity (k = 0.25; P = 0.004).
CONCLUSION: There was a male preponderance among HS patients in Northern Peninsular Malaysia with early age of onset and more severe disease. Only one-fifth had MetS, but they had significantly higher risks of obesity and low HDL. Ultrasonography examination was useful to detect subclinical lesions and providing a better understanding on disease severity.
METHODOLOGY: Retrospective review of all children who fulfilled the diagnostic criteria of CVS and who were seen at Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur and Paediatric Unit, Penang Hospital, Penang, Malaysia from 1987 to 1997.
RESULTS: Eight children with CVS were seen at the two units during the study period, five girls and three boys. All had cyclical, self-limited episodes of nausea and vomiting with symptom-free intervals. The mean age of onset was 5.9 years. The clinical features were similar to other series described in the literature. Only two of the eight children were described as 'perfectionist'. Two children identified stress as precipitating factors. Therapy to reduce the number of emeses during acute attacks and to prevent subsequent attacks had been ineffective.
CONCLUSION: There are similarities and differences in CVS from South-East Asian children as compared to those described in the literature.
METHODOLOGY: A prospective study was performed on children with diarrhoea of more than 14 days' duration who were managed at the Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur from 1 January 1996 to 31 December 1997.
RESULTS: Twenty-seven patients (14 boys and 13 girls) were studied. The median age of onset of diarrhoea was 6 months and the mean duration of symptoms before referral was 66.5 days. The underlying causes of diarrhoea were found to be: (i) prolonged diarrhoea due to well-defined entities (intestinal lymphangiectasia, two cases; congenital glucose-galactose malabsorption, one case; post-small bowel resection, one case; (ii) postenteritis diarrhoea (cow's milk protein intolerance, eight cases; secondary lactose intolerance, four cases; transient monosaccharide intolerance, one case; (iii) gastrointestinal infections (nontyphoid Salmonella gastroenteritis, three cases; trichuriasis, two cases; amoebiasis, one case; adenovirus, one case; (iv) cases in which a firm diagnosis could not be established (three cases). The mean duration of hospital admission was 63 days. Sixteen cases required a change in diet, while nine cases required total parenteral nutrition. One death occurred.
CONCLUSIONS: Chronic childhood diarrhoea in Malaysia had a variety of aetiologies. A specific diagnosis could be established in 90% of cases. Making a diagnosis was important because this led to appropriate therapy and a good outcome in 96% of cases.
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.
OBJECTIVE: To identify mutation-specific cancer risks for carriers of BRCA1/2.
DESIGN, SETTING, AND PARTICIPANTS: Observational study of women who were ascertained between 1937 and 2011 (median, 1999) and found to carry disease-associated BRCA1 or BRCA2 mutations. The international sample comprised 19,581 carriers of BRCA1 mutations and 11,900 carriers of BRCA2 mutations from 55 centers in 33 countries on 6 continents. We estimated hazard ratios for breast and ovarian cancer based on mutation type, function, and nucleotide position. We also estimated RHR, the ratio of breast vs ovarian cancer hazard ratios. A value of RHR greater than 1 indicated elevated breast cancer risk; a value of RHR less than 1 indicated elevated ovarian cancer risk.
EXPOSURES: Mutations of BRCA1 or BRCA2.
MAIN OUTCOMES AND MEASURES: Breast and ovarian cancer risks.
RESULTS: Among BRCA1 mutation carriers, 9052 women (46%) were diagnosed with breast cancer, 2317 (12%) with ovarian cancer, 1041 (5%) with breast and ovarian cancer, and 7171 (37%) without cancer. Among BRCA2 mutation carriers, 6180 women (52%) were diagnosed with breast cancer, 682 (6%) with ovarian cancer, 272 (2%) with breast and ovarian cancer, and 4766 (40%) without cancer. In BRCA1, we identified 3 breast cancer cluster regions (BCCRs) located at c.179 to c.505 (BCCR1; RHR = 1.46; 95% CI, 1.22-1.74; P = 2 × 10(-6)), c.4328 to c.4945 (BCCR2; RHR = 1.34; 95% CI, 1.01-1.78; P = .04), and c. 5261 to c.5563 (BCCR2', RHR = 1.38; 95% CI, 1.22-1.55; P = 6 × 10(-9)). We also identified an ovarian cancer cluster region (OCCR) from c.1380 to c.4062 (approximately exon 11) with RHR = 0.62 (95% CI, 0.56-0.70; P = 9 × 10(-17)). In BRCA2, we observed multiple BCCRs spanning c.1 to c.596 (BCCR1; RHR = 1.71; 95% CI, 1.06-2.78; P = .03), c.772 to c.1806 (BCCR1'; RHR = 1.63; 95% CI, 1.10-2.40; P = .01), and c.7394 to c.8904 (BCCR2; RHR = 2.31; 95% CI, 1.69-3.16; P = .00002). We also identified 3 OCCRs: the first (OCCR1) spanned c.3249 to c.5681 that was adjacent to c.5946delT (6174delT; RHR = 0.51; 95% CI, 0.44-0.60; P = 6 × 10(-17)). The second OCCR spanned c.6645 to c.7471 (OCCR2; RHR = 0.57; 95% CI, 0.41-0.80; P = .001). Mutations conferring nonsense-mediated decay were associated with differential breast or ovarian cancer risks and an earlier age of breast cancer diagnosis for both BRCA1 and BRCA2 mutation carriers.
CONCLUSIONS AND RELEVANCE: Breast and ovarian cancer risks varied by type and location of BRCA1/2 mutations. With appropriate validation, these data may have implications for risk assessment and cancer prevention decision making for carriers of BRCA1 and BRCA2 mutations.
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.