METHODS: We prospectively identified patients presenting to the public or major private hospitals in Auckland (population = 1.61 million) between April 6, 2015 and April 5, 2016 with a seizure lasting 10 minutes or longer, with retrospective review to confirm completeness of data capture. Information was recorded in the EpiNet database.
RESULTS: A total of 477 episodes of SE occurred in 367 patients. Fifty-one percent of patients were aged <15 years. SE with prominent motor symptoms comprised 81% of episodes (387/477). Eighty-four episodes (18%) were nonconvulsive SE. Four hundred fifty episodes occurred in 345 patients who were resident in Auckland. The age-adjusted incidence of 10-minute SE episodes and patients was 29.25 (95% confidence interval [CI] = 27.34-31.27) and 22.22 (95% CI = 20.57-23.99)/100 000/year, respectively. SE lasted 30 minutes or longer in 250 (56%) episodes; age-adjusted incidence was 15.95 (95% CI = 14.56-17.45) SE episodes/100 000/year and 12.92 (95% CI = 11.67-14.27) patients/100 000/year. Age-adjusted incidence (10-minute SE) was 25.54 (95% CI = 23.06-28.24) patients/100 000/year for males and 19.07 (95% CI = 16.91-21.46) patients/100 000/year for females. The age-adjusted incidence of 10-minute SE was higher in Māori (29.31 [95% CI = 23.52-37.14]/100 000/year) and Pacific Islanders (26.55 [95% CI = 22.05-31.99]/100 000/year) than in patients of European (19.13 [95% CI = 17.09-21.37]/100 000/year) or Asian/other descent (17.76 [95% CI = 14.73-21.38]/100 000/year). Seventeen of 367 patients in the study died within 30 days of the episode of SE; 30-day mortality was 4.6%.
SIGNIFICANCE: In this population-based study, incidence and mortality of SE in Auckland lie in the lower range when compared to North America and Europe. For pragmatic reasons, we only included convulsive SE if episodes lasted 10 minutes or longer, although the 2015 ILAE SE classification was otherwise practical and easy to use.
DESIGN: We used genome sequencing data to assess the prevalence of mutations in syndromic HH genes in an international cohort of patients with HH of unknown genetic cause.
PATIENTS: We undertook genome sequencing in 82 infants with HH without a clinical diagnosis of a known syndrome at referral for genetic testing.
MEASUREMENTS: Within this cohort, we searched for the genetic aetiologies causing 20 different syndromes where HH had been reported as a feature.
RESULTS: We identified a pathogenic KMT2D variant in a patient with HH diagnosed at birth, confirming a genetic diagnosis of Kabuki syndrome. Clinical data received following the identification of the mutation highlighted additional features consistent with the genetic diagnosis. Pathogenic variants were not identified in the remainder of the cohort.
CONCLUSIONS: Pathogenic variants in the syndromic HH genes are rare; thus, routine testing of these genes by molecular genetics laboratories is unlikely to be justified in patients without syndromic phenotypes.
METHODS: A random cluster sampling cross-sectional survey was conducted in 2019. The study involved registered care providers for preschoolers under four years old without acute psychiatric illness. The Center for Epidemiologic Studies Depression Scale (Malay-CES-D) and Karasek's Job Content Questionnaires (Malay-JCQ) were used to assess depression symptoms and psychosocial job-related risks. Logistic regression (p preschools. All respondents were females, with a mean age of 32.1 years old, Malays (70.4%), married (55.0%), attained a diploma and above (50.6%) and had low income (80.1%). The prevalence of depressive symptoms and job strain was 28.7%. Final logistic regression revealed individual factors (married, stressful life events: assault and marital issues), job strain (AOR = 2.33, CI = 1.22, 4.44), and job insecurity (AOR = 1.29, CI = 1.07, 1.56) determine depressive symptoms. Good supervisor support was inversely associated with depression.
CONCLUSIONS: Job strain and insecurity contribute significantly to depressive symptoms among preschool care providers in Kuala Lumpur. The Social and Welfare Department can promote supportive supervision through skill training for supervisors, fostering a positive mental health environment for improving workers' mental health.
METHODS: The POCT was used to test 170 serum specimens collected through measles surveillance or vaccination programmes in Ethiopia, Malaysia and the Russian Federation: 69 were positive for measles immunoglobulin M (IgM) antibodies, 74 were positive for rubella IgM antibodies and 7 were positive for both. Also tested were 282 oral fluid specimens from the measles, mumps and rubella (MMR) surveillance programme of the United Kingdom of Great Britain and Northern Ireland. The Microimmune measles IgM capture enzyme immunoassay was the gold standard for comparison. A panel of 24 oral fluids was used to investigate if measles virus haemagglutinin (H) and nucleocapsid (N) genes could be amplified by polymerase chain reaction directly from used POCT strips.
FINDINGS: With serum POCT showed a sensitivity and specificity of 90.8% (69/76) and 93.6% (88/94), respectively; with oral fluids, sensitivity and specificity were 90.0% (63/70) and 96.2% (200/208), respectively. Both H and N genes were reliably detected in POCT strips and the N genes could be sequenced for genotyping. Measles virus genes could be recovered from POCT strips after storage for 5 weeks at 20-25 °C.
CONCLUSION: The POCT has the sensitivity and specificity required of a field-based test for measles diagnosis. However, its role in global measles control programmes requires further evaluation.
MATERIALS AND METHODS: A cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.
RESULTS: Children with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).
CONCLUSION: HRQoL in children with BA living with their native livers is comparable to healthy children.