METHODS: A systematic search of databases was conducted according to the PRISMA guidelines. Articles reporting sex distribution and age of onset for AQP4 antibody-associated NMSOD were reviewed. An initially inclusive approach involving exploration with regression meta-analysis was followed by an analysis of just AQP4 antibody positive cases.
RESULTS: A total of 528 articles were screened to yield 89 articles covering 19,415 individuals from 88 population samples. The female:male sex ratio was significantly influenced by the proportion of AQP4 antibody positive cases in the samples studied (p onset cases, it was 5.48 (95% CI 4.10-7.33). The mean age of onset was significantly associated with the mean life expectancy of the population sampled (p onset for AQP4 antibody-positive cases in long-lived populations was 41.7 years versus 33.3 years in the remainder.
CONCLUSIONS: The female:male sex ratio and the mean age of onset of AQP4 antibody-associated NMOSD are significantly higher than MS. The sex ratio increases with the proportion of cases that are positive for AQP4 antibodies and the mean age of onset increases with population life expectancy.
METHODS: The database includes 4037 patients with a diagnosis of bipolar I disorder, previously collected at 36 collection sites in 23 countries. Generalized estimating equations (GEE) were used to adjust the data for country median age, and in some models, birth cohort. Model-based clustering (mixture analysis) was then performed on the age of onset data using the residuals. Clinical variables in subgroups were compared.
RESULTS: There was a strong birth cohort effect. Without adjusting for the birth cohort, three subgroups were found by clustering. After adjusting for the birth cohort or when considering only those born after 1959, two subgroups were found. With results of either two or three subgroups, the youngest subgroup was more likely to have a family history of mood disorders and a first episode with depressed polarity. However, without adjusting for birth cohort (three subgroups), family history and polarity of the first episode could not be distinguished between the middle and oldest subgroups.
CONCLUSION: These results using international data confirm prior findings using single country data, that there are subgroups of bipolar I disorder based on the age of onset, and that there is a birth cohort effect. Including the birth cohort adjustment altered the number and characteristics of subgroups detected when clustering by age of onset. Further investigation is needed to determine if combining both approaches will identify subgroups that are more useful for research.
SUMMARY: This study examined country-specific prevalence and incidence data of youth-onset T2D published between 2008 and 2019, and searched for national guidelines to expand the understanding of country-specific similarities and differences. Of the 1,190 articles and 17 congress abstracts identified, 58 were included in this review. Our search found the highest reported prevalence rates of youth-onset T2D in China (520 cases/100,000 people) and the USA (212 cases/100,000) and lowest in Denmark (0.6 cases/100,000) and Ireland (1.2 cases/100,000). However, the highest incidence rates were reported in Taiwan (63 cases/100,000) and the UK (33.2 cases/100,000), with the lowest in Fiji (0.43 cases/100,000) and Austria (0.6 cases/100,000). These differences in epidemiology data may be partly explained by variations in the diagnostic criteria used within studies, screening recommendations within national guidelines and race/ethnicity within countries. Key Messages: Our study suggests that published country-specific epidemiology data for youth-onset T2D are varied and scant, and often with reporting inconsistencies. Finding optimal diagnostic criteria and screening strategies for this disease should be of high interest to every country.
TRIAL REGISTRATION: Not applicable.
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.
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.
CASE REPORT: A 91 year old lady presented with an acute manic relapse for the past 2 weeks. She was previously on oral Sodium Valproate, and during this current admission was augmented with oral Quetiapine IR 100 mg bd. She remained unwell and was planned for right unilateral ECT with age-based dosing stimuli. After only 4 sessions, she showed complete resolution of her manic symptoms.
RESULT: In our case study, the patient showed rapid response to right unilateral ECT. Even though the Post Suppression Index (PSI) was not significant, there is some evidence that in elderly patients, burst suppression (not measured in this case) may be more accurate measure of ECT efficacy. The transient treatment emergent delirium was short lived and ECT was very tolerated in this patient.
CONCLUSION: Clinicians should not delay ECT in old-old patients who do not respond to pharmacologic treatment, as early switch to ECT results in rapid response with good safety profile.
OBJECTIVES: This study aimed to investigate the genetic architecture of EOPD in a multi-ethnic Malaysian cohort.
METHODS: 161 index patients with PD onset ≤50 years were recruited from multiple centers across Malaysia. A two-step approach to genetic testing was used, combining a next-generation sequencing-based PD gene panel and multiplex ligation-dependent probe amplification (MLPA).
RESULTS: Thirty-five patients (21.7%) carried pathogenic or likely pathogenic variants involving (in decreasing order of frequency): GBA1, PRKN, PINK1, DJ-1, LRRK2, and ATP13A2. Pathogenic/likely pathogenic variants in GBA1 were identified in thirteen patients (8.1%), and were also commonly found in PRKN and PINK1 (11/161 = 6.8% and 6/161 = 3.7%, respectively). The overall detection rate was even higher in those with familial history (48.5%) or age of diagnosis ≤40 years (34.8%). PRKN exon 7 deletion and the PINK1 p.Leu347Pro variant appear to be common among Malay patients. Many novel variants were found across the PD-related genes.
CONCLUSIONS: This study provides novel insights into the genetic architecture of EOPD in Southeast Asians, expands the genetic spectrum in PD-related genes, and highlights the importance of diversifying PD genetic research to include under-represented populations.