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showing 10 items of 9912 documents
Efficacy and safety of lomitapide in familial chylomicronaemia syndrome
2022
Familial chylomicronaemia syndrome (FCS) is a rare autosomal recessive disorder, resulting in elevated triglycerides (TGs), abdominal pain and pancreatitis. Treatment options are limited. Lomitapide, a microsomal triglyceride transfer protein inhibitor, is approved for the treatment of homozygous familial hypercholesterolaemia. Whether its therapeutic use may be extended to FCS remains unknown. The aim of this study was to evaluate the efficacy and safety of lomitapide in adult patients with FCS.The open-label, single-arm 'LOCHNES' study of lomitapide in FCS enrolled patients18 years with genetically confirmed FCS, elevated fasting TG ≥ 750 mg/dL and history of pancreatitis. Patients were a…
Blood sulfur-amino acid concentration reflects an impairment of liver transsulfuration pathway in patients with acute abdominal inflammatory processes
2001
Whole-blood free amino acids were measured in a control group made up of eight healthy women fasted for 12 h and also in eight patients with acute pancreatitis, five patients with acute cholecystitis and seven patients with acute appendicitis. Blood was withdrawn immediately on admission to hospital and again 3 d later following a controlled peripheral parenteral nutrition diet; this is with the exception of the appendicitis group. L-CYSTATHIONINE AND l-methionine concentrations were significantly higher in pancreatitis and appendicitis patients when compared with controls. In the pancreatitis and cholecystitis patients, l-serine concentration was also significantly higher when compared wit…
Health-related quality of life in parents of adolescents one year into the COVID-19 pandemic: a two-year longitudinal study.
2022
Abstract Aim For many adults, their role as a parent is a vital part of their life that may influence their health-related quality of life (HRQOL) and vary with the age of their child. The aim of the present study was to describe and compare sociodemographic and psychological factors, pain and HRQOL in parents of adolescents assessed at baseline and 2 years later,—during the COVID-19 pandemic. Methods A longitudinal study of 309 parents from the general Norwegian population was conducted. The parents were chosen based on their adolescent’s school belonging and responded to a web-based questionnaire. We used data collected at baseline (T1), when the adolescents were aged 14–15 years (2018/20…
Psycho-emotional adjustment in parents of adolescents: A cross-sectional and longitudinal analysis of the impact of the COVID pandemic
2021
Abstract Purpose: adolescence is a time of change and it generally entails a greater family vulnerability thus; the aim of this study was to identify the risk factors for poor emotional adjustment to COVID among parents of adolescents. Design and methods: 94 parents of adolescents (11–18 years old, M = 13.90, SD = 1.85) participated at different times during the state of alert in Spain. 91.5% were mothers. Their ages ranged from 35 to 63 years (M = 46.54; SD = 5.09). The variables assessed were anxiety, depression and stress (DASS), moods (MOOD), somatization (SCL) and resilience (CD-RISC). Descriptive analyses, t-tests, ANOVAs, correlations, and hierarchical regressions were performed. All…
CONFAMI Study: Effects of home confinement during the COVID19 epidemic on the lives of children and their families
2021
The aim of this study is to understand the changes within families during confinement motivated by the COVID-19 pandemic and to explore the psycho-emotional experiences of children and their parents in this new situation. Confinement necessarily induced significant changes in daily family routines, particularly for work, education, leisure and social activities. In the more vulnerable pediatric population, several authors have warned of the need to consider the impact of lockdown measures during COVID-19 on the psychological impact and well-being.This is an anonymous online survey with methodology combining quantitative and qualitative analyses. The questions targeted several themes such as…
Maternally inherited diabetes and deafness (MIDD): unusual occult exocrine pancreatic manifestation in an affected German family
2000
The mitochondrial (mt) 3243 DNA mutation is an underlying cause of maternally inherited diabetes and deafness (MIDD) syndrome and the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). We report an affected German MIDD pedigree with maternal lineage over three generations. The index patient, her mother, her maternal aunt and her maternal grandmother all suffered from diabetes and premature hearing loss and were positive on testing for the mt 3243 DNA mutation. The 27-year-old index patient had a history of grand mal seizures. As sequela of abdominal ultrasound and confirmed by magnetic resonance cholangio-pancreaticography, she was diagnose…
Cervical Cancer Screening in Women With Severe Mental Disorders An Approach to the Spanish Context
2018
Background The incidence of invasive cervical cancer and its mortality have been reduced through primary and secondary prevention. Screening rates tend to be lower in vulnerable groups, such as people with severe mental disorders, who have a later detection of cancer and a higher mortality. The access of these women to cervical cancer screening is uncertain in our context. Objective The aim of this study was to determine the cervical cancer screening rates in women with severe mental disorders. Methods This was a descriptive cross-sectional study. Women 25 to 65 years old who were admitted during 2016 to the psychiatric unit of a public hospital in Spain were included in the study, and it w…
Consistencies and discrepancies in self- and observer-rated anxiety scales. A comparison between the self- and observer-rated Marks-Sheehan scales.
1990
The Marks-Sheehan anxiety scales are the only scales where self-ratings and observer ratings are perfectly matched by the number, the content and the scaling of the items. Therefore these scales are an excellent tool to investigate the compatibility and to study different structures in self- and observer ratings. This was done by using the data material on the Marks-Sheehan scales of the Cross National Collaborative Panic Study. In this study 1168 outpatients who met the DSM-III criteria for panic disorder were randomly allocated either to alprazolam, imipramine or placebo treatment. Our results show that the Marks-Sheehan scales are highly comparable to other established rating scales. Bot…
Self- and observer assessment in anxiolytic drug trials: A comparison of their validity
1990
Self-rating scales are considered to be less useful for comparing different treatments in anxiety patients than observer-rating scales. However, the empirical evidence for this assumption is not adequate. A self-rating inventory of 35 items related to anxiety was perfectly parallel with an observer-rating inventory. Both instruments were used in the Cross National Collaborative Panic Study to compare the efficacy of imipramine, alprazolam and placebo in an 8-week drug trial in a sample of 1168 outpatients. The variance of the self-rating assessments was about two times higher. Both scales were equally sensitive to change; however, the measurement of change by means of the self-rating scale …
Validation of the Spanish Version of the Beginning Breastfeeding Survey-Cumulative: A Follow-up Study
2019
Background: The Beginning Breastfeeding Survey-Cumulative (BBSC) is an instrument that assesses the overall maternal perception of breastfeeding effectiveness during the early postpartum period. Research aims: The aims of this study were to adapt and validate a Spanish version of the BBSC and provide new evidence regarding its validity. Methods: A standard forward and back-translation process was used to obtain the Spanish version of the questionnaire (BBSC-E). A sample of 793 breastfeeding mothers was used. Data were obtained from clinical records and questionnaires self-administered at discharge and at 1–4 months postpartum. The factorial structure of the BBSC-E was examined with both an …