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Moss Holt posted an update 1 year, 5 months ago
s. As TBA enables screening for unknown autoantibodies, we suggest this method as a second step if commercial CBAs do not yield a result. Further studies are necessary to characterize such antibodies, evaluate pathogenicity, and answer the question whether positive CSF neuroreactivity should prompt an immunotherapeutic approach.
To describe the population of young people in Ireland diagnosed with narcolepsy with regards to vaccine exposure, symptomatology, investigation results and experience of medical treatment.
Retrospective review of medical records at the single tertiary referral centre for young people with narcolepsy in Ireland.
Sixty-seven patients were diagnosed with narcolepsy between July 2006 and July 2017. Sixty-one (91%) of these developed symptoms after receiving the Pandemrix vaccine. The population was largely homogeneous with low hypocretin (87.5%), HLA DQB1∗0602 positivity (95%) and unremarkable findings on MRI Brain (100%). 77.6% experienced cataplexy; we also measured high levels of obesity, school non-attendance and psychosocial complexity. Symptoms often continued despite treatment, with multiple medications prescribed in 76.1% of patients. Prescription of sodium oxybate was associated with a significant reduction in BMI standard deviation scores at 6 months, with improved IOTF obesity scores seen at 36 month follow-up.
This paper describes the experience of narcolepsy in children and young people in Ireland from 2006 – 2017at the national tertiary referral centre. Narcolepsy in children and young people in Ireland carries a significant burden of illness, with impact on participation in education as well as physical and mental health. Symptoms can be refractory to medical treatment. Referral to tertiary centres for prompt treatment and multidisciplinary input is essential.
This paper describes the experience of narcolepsy in children and young people in Ireland from 2006 – 2017 at the national tertiary referral centre. Narcolepsy in children and young people in Ireland carries a significant burden of illness, with impact on participation in education as well as physical and mental health. Symptoms can be refractory to medical treatment. Referral to tertiary centres for prompt treatment and multidisciplinary input is essential.Rett syndrome (RTT) is neurodevelopmental disorder affecting approximately 110000-15000 live female births, commonly associated with MECP2 gene mutations. Hand stereotypies and gait disturbance, as well as spasticity and dystonia, were noted in RTT since first descriptions. This review aimed to explore the prevalence of reported movement disorders in RTT.
Pubmed and Embase databases for papers describing features of movement disorders in RTT. Papers were selected if included description of case report, cohort or case-series of patients with RTT including descriptions of clinical features of their movement disorder. learn more Papers were divided into 3 epochs – i) Pre-1999,ii) 2000-2009, and iii) 2010 onwards.
32 studies (13 in the first, 10 in the second and 9 in the third epochs) reported on movement disorders in RTT. Hand stereotypies were almost universal, diminishing but not disappearing over time. Gait disturbance and ataxia/tremor were also very common (>50% cases). Hypertonia was also often reported, increhe relative contribution of dystonia and rigidity to hypertonia in RTT, as well as the impact of these impairments when present.
To analyse the data, according to recruiting place and sex, of the survey May Measure Month in 2018 (MMM18) in Spain, promoted by the International Society of Hypertension.
Subjects more than 18 years old were studied. MMM18 protocol was performed. Volunteers were recruited through the Spanish Society of Community Pharmacy (SEFAC) and the Spanish Society of Hypertension (SEH-LELHA). General linear models of blood pressure (BP) were carried out in subjects with and without treatment, and adjusted by age, sex, tobacco use, obesity and recruitment site.
7 511 individuals (mean age 51.7±19.6 years, 36.8% males) were screened. Systolic and diastolic BP was higher in males (129.0-16.7/119.6-18.2mmHg) (78.3-11.1/74.8-10.7mmHg) (p<0.001). There was a linear relationship between systolic BP, age and sex, with higher values in males (11.2mmHg in untreated and 4.5mmHg in treated) (p<0.001). Diastolic BP was inverted U-shaped, with highest level in males and between 50-55 years. The proportion of individuals igher in males. Our study suggests that gender differences described should be considered in the BP thresholds established.
To investigate whether a volume-outcome relationship exists for elective abdominal aortic aneurysm (AAA) surgery conducted within the National Health Service (NHS) in England.
This was an analysis of administrative data. Data were extracted from the Hospital Episodes Statistics database for England from April 2011 to March 2019 for all adult admissions for elective infrarenal AAA surgery. Data were extracted for the NHS trust and surgeon undertaking the procedure, the surgical technique used (open or endovascular), the financial year of admission, length of hospital and critical care stay during the procedure and subsequent emergency re-admissions (primary outcome) and deaths within 30 days. Multilevel modelling was used to adjust for hierarchy and confounding.
A dataset of 31829 procedures (8867 open, 22962 endovascular) was extracted. For open surgery, lower trust annual volume was associated with higher 30 day emergency re-admission rates and higher 30 day mortality. For open surgery, lower surgeon annual volume was associated with higher 30 day mortality and length of hospital stay greater than the median. For endovascular surgery, lower surgeon annual volume was associated with not having an overnight stay in critical care. None of the other volume-outcome relationships investigated was significant.
For elective infrarenal AAA surgery in the UK NHS, there was strong evidence of a volume-outcome relationship for open surgery. However, evidence for a volume-outcome relationship is dependent on the specific procedure undertaken and the outcome of interest.
For elective infrarenal AAA surgery in the UK NHS, there was strong evidence of a volume-outcome relationship for open surgery. However, evidence for a volume-outcome relationship is dependent on the specific procedure undertaken and the outcome of interest.

