Publications by authors named "Roderick de Lind van Wijngaarden"
Context: The pathophysiology of hypogonadism in boys with Prader-Willi Syndrome (PWS) remains uncertain. Several reports described hypogonadotropic hypogonadism, some reported primary gonadal failure, and others a combination of both.
Objectives: The aim of the study was to evaluate gonadal function over time in boys with PWS and the effect of GH treatment.
Context: Premature pubarche (PP) is reported in children with Prader-Willi Syndrome (PWS). Pubarche is preceded by adrenarche - an increase in serum levels of adrenal androgens, most specifically dehydroepiandrosterone sulphate (DHEAS).
Objectives: To assess DHEAS levels, the age at and progression of pubarche and the prevalence of PP in children with PWS.
Study Design: Case report.
Objective: To describe a sudden spinal cord injury in a girl with Prader-Willi syndrome (PWS) 1 week after posterior T2-L3 fusion.
Summary Of Background Data: PWS is a genetic disorder characterized by several features including growth deficiency, hypotonia, obesity, and spinal deformities.
Cardiovascular and metabolic risk profile and acylation-stimulating protein levels in children with Prader-Willi syndrome and effects of growth hormone treatment.
J Clin Endocrinol Metab
April 2010
Context: Reports on the cardiovascular and metabolic risk profile in children with Prader-Willi syndrome (PWS) and the effects of GH treatment are scarce. Acylation-stimulating protein (ASP) stimulates glucose uptake and triglyceride storage in adipose tissue.
Objectives: The aim was to study the metabolic and cardiovascular risk profile and ASP levels and to investigate the effects of GH treatment.
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.
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.
Background: The annual death rate of patients with Prader-Willi syndrome (PWS) is high (3%). Many deaths of children are sudden and unexplained. Sleep apneas have been suggested to play a role in sudden deaths.
View Article and Find Full Text PDFContext: 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.
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.
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.