Multimorbidity and Polypharmacy Are Independently Associated With Mortality in Older People With Intellectual Disabilities: A 5-Year Follow-Up From the HA-ID Study.

We studied the association between multimorbidity, polypharmacy, and mortality in 1,050 older adults (50+) with intellectual disability (ID). Multimorbidity (presence of ≥ 4 chronic health conditions) and polypharmacy (presence ≥ 5 chronic medication prescriptions) were collected at baseline. Multimorbidity included a wide range of disorders, including hearing impairment, thyroid dysfunction, autism, and cancer.

Causes of Mortality in Older People With Intellectual Disability: Results From the HA-ID Study.

We aim to provide insight into the cause-specific mortality of older adults with intellectual disability (ID), with and without Down syndrome (DS), and compare this to the general population. Immediate and primary cause of death were collected through medical files of 1,050 older adults with ID, 5 years after the start of the Healthy Ageing and Intellectual Disabilities (HA-ID) study. During the follow-up period, 207 (19.

[Older adults with intellectual disabilities markedly decline in daily functioning over a 3‑year period: Results of the HA-ID study].

The responsibilities for the care of a significant portion of the population with an intellectual disability (ID) were recently transferred from the government to the municipalities. It is therefore important that policymakers and care professionals know how much support this population needs in their daily life. Therefore, this study focuses on the decline in daily functioning of older adults with ID (≥50 years, n = 703) over 3 years, and if daily functioning is a predictor for all-cause mortality.

Beneficial Effects of GH in Young Adults With Prader-Willi Syndrome: A 2-Year Crossover Trial.

Context: Patients with Prader-Willi syndrome (PWS) are severely at risk to develop morbid obesity, diabetes mellitus type 2, and cardiovascular disease, leading to high mortality. They have an increased fat mass (FM) and decreased lean body mass (LBM). During childhood, GH treatment counteracts the natural course of increasing obesity.

Physician-Reported Symptoms and Interventions in People with Intellectual Disabilities Approaching End of Life.

Background: Insights into symptoms and interventions at the end of life are needed for providing adequate palliative care, but are largely lacking for people with intellectual disabilities (IDs).

Objectives: We aimed at determining the prevalence rates of physician-reported symptoms from the Edmonton Symptom Assessment System (ESAS) at the moment that physicians recognized patient's death in the foreseeable future. In addition, we aimed at exploring provided interventions as reported by physicians in the period between physicians' recognition of death in the foreseeable future and patients' death.

Beneficial Effect of Growth Hormone Treatment on Health-Related Quality of Life in Children with Prader-Willi Syndrome: A Randomized Controlled Trial and Longitudinal Study.

Background/aims: Growth hormone (GH) treatment is beneficial for children with Prader-Willi syndrome (PWS), but data about health-related quality of life (HRQOL) and effects of GH treatment are scarce. We, therefore, investigated the effects of GH treatment on HRQOL in PWS children.

Methods: In a randomized controlled GH trial including 26 PWS children and during an 11-year longitudinal GH study in 76 children, we annually assessed HRQOL recorded by patients and parents, using a generic questionnaire (DUX25), containing 4 subdomains (Physical, Home, Social, and Emotional) and a PWS-specific questionnaire (DUXPW).

Beneficial Effects of Long-Term Growth Hormone Treatment on Adaptive Functioning in Infants With Prader-Willi Syndrome.

The aim of this study was to investigate the effect of growth hormone treatment on adaptive functioning in children with Prader-Willi syndrome. Vineland Adaptive Behavior Scale (VABS) was assessed during a randomized controlled trial (RCT) and after 7 years of growth hormone treatment. In the RCT, 75 children (42 infants and 33 prepubertal children) with Prader-Willi syndrome were included.

Behavior in children with Prader-Willi syndrome before and during growth hormone treatment: a randomized controlled trial and 8-year longitudinal study.

Information on behavior of children with Prader-Willi syndrome (PWS) and the effect of growth hormone (GH) treatment is scarce. Parents report less problem behavior during GH treatment. Forty-two pre-pubertal children, aged 3.

[Health care transition in young people with intellectual disabilities: from generalist to generalist].

The transition of medical care in young people with intellectual disabilities is not well organised in the Netherlands. This heterogeneous group, with a high rate of comorbidity, needs regular medical follow-up. During adolescence the paediatrician should preferably transfer medical care to a generalist, such as a physician for people with intellectual disabilities.

Growth hormone combined with child-specific motor training improves motor development in infants with Prader-Willi syndrome: a randomized controlled trial.

Although severe motor problems in infants with Prader-Willi syndrome (PWS) are striking, motor development has never been studied longitudinally and the results of growth hormone (GH) treatment on motor development are contradictory. The authors studied whether GH treatment can enhance the effect of physical training on motor development in infants with PWS. Twenty-two infants were followed for two years during a randomized controlled trial.

Beneficial effects of growth hormone treatment on cognition in children with Prader-Willi syndrome: a randomized controlled trial and longitudinal study.

Background: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.

Methods: Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment.

Efficacy and safety of long-term continuous growth hormone treatment in children with Prader-Willi syndrome.

Background: Children with Prader-Willi syndrome (PWS) have abnormal body composition and impaired growth. Short-term GH treatment has beneficial effects.

Objectives: The aim of the study was to investigate effects of long-term continuous GH treatment on body composition, growth, bone maturation, and safety parameters.

Bone mineral density and effects of growth hormone treatment in prepubertal children with Prader-Willi syndrome: a randomized controlled trial.

Background: Bone mineral density (BMD) is unknown in children with Prader-Willi syndrome (PWS), but is decreased in adults with PWS. In patients with GH deficiency, BMD increases during GH treatment.

Objectives: The aim of the study was to evaluate BMD in children with PWS and to study the effects of GH treatment.

Randomized controlled trial to investigate the effects of growth hormone treatment on scoliosis in children with Prader-Willi syndrome.

Context: The prevalence of scoliosis in children with Prader-Willi syndrome (PWS) is 30-80%, depending on age. Although reports about effects of GH treatment on scoliosis in children with PWS are limited, scoliosis is generally considered a contraindication for GH treatment.

Objective: The aim was to study the effects of GH treatment on the onset of scoliosis and curve progression in children with PWS.

Cognition and behavior in pre-pubertal children with Prader-Willi syndrome and associations with sleep-related breathing disorders.

Prader-Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, and sleep-related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral and cognitive abnormalities and SRBD have been found.

Randomized controlled GH trial: effects on anthropometry, body composition and body proportions in a large group of children with Prader-Willi syndrome.

Background: Prader-Willi syndrome (PWS) children have impaired growth, and abnormal body composition. Previous 1-year controlled studies showed improvement of height and body composition during GH-treatment.

Objective: To evaluate growth, body composition and body proportions during GH-treatment in a large group of PWS children.

High prevalence of central adrenal insufficiency in patients with Prader-Willi syndrome.

Context: The annual death rate of Prader-Willi syndrome (PWS) patients is very high (3%). Many of these deaths are sudden and unexplained.

Objective: Because most deaths occur during moderate infections and PWS patients suffer from various hypothalamic insufficiencies, we investigated whether PWS patients suffer from central adrenal insufficiency (CAI) during stressful conditions.

Breathing disorders in Prader-Willi syndrome: the role of obesity, growth hormone treatment and upper respiratory tract infections.

Prader-Willi syndrome is a neurogenetic disorder characterized by a number of signs and symptoms, including muscular hypotonia in infancy, hypogonadism, obesity and short stature. Neurobehavioral abnormalities and cognitive impairment are common. In addition, breathing abnormalities have been described, including sleep-related breathing disorders, abnormal chemoreceptor sensitivity and pulmonary function abnormalities.

Psychomotor development in infants with Prader-Willi syndrome and associations with sleep-related breathing disorders.

Prader-Willi syndrome (PWS) is a neurogenetic disorder with hypotonia, psychomotor delay, obesity, short stature, and sleep-related breathing disorders. The aim of this study was to evaluate the association between psychomotor development and sleep-related breathing disorders in PWS infants. Bayley Scales of Infant Development were performed in 22 PWS infants, with a median (interquartile range, IQR) age of 1.

Thyroid function and outcome in children who survived meningococcal septic shock.

Objective: To investigate the time course of thyroid function, factors that affect it, and its relationship to outcome in children surviving meningococcal septic shock.

Design And Setting: Observational cohort study in a university-affiliated pediatric intensive care unit (PICU).

Patients And Participants: We divided the 44 children admitted to the PICU who survived meningococcal septic shock into those with short-stay (<7 days, n=33) or long-stay (>or=7 days, n=11).