Optimizing the Timing of Highest Hydrocortisone Dose in Children and Adolescents With 21-Hydroxylase Deficiency.

Context: Hydrocortisone treatment of young patients with 21-hydroxylase deficiency (21OHD) is given thrice daily, but there is debate about the optimal timing of the highest hydrocortisone dose, either mimicking the physiological diurnal rhythm (morning), or optimally suppressing androgen activity (evening).

Objective: We aimed to compare 2 standard hydrocortisone timing strategies, either highest dosage in the morning or evening, with respect to hormonal status throughout the day, nocturnal blood pressure (BP), and sleep and activity scores.

Methods: This 6-week crossover study included 39 patients (aged 4-19 years) with 21OHD.

[Myocardial infarction in a 16-year old following inhalation of butane gas].

Background: Butane gas is inhaled by young people with the aim of getting 'high'. This can cause coronary spasm with myocardial infarction and ventricular fibrillation as a result.

Case Description: We report on a 16-year-old male who collapsed at home after sniffing butane.

Preterm birth does not affect bone mineral density in young adults.

Objective: Previous studies showed conflicting data on the effect of prematurity on bone mineral density (BMD) in infants and children. Only a few studies investigated the long-term effects of prematurity on BMD in early adulthood. The objective of our study was to assess the long-term effects of preterm birth on BMD of the total body (BMD(TB)), lumbar spine (BMD(LS)) and bone mineral apparent density of the LS (BMAD(LS)).

Should short children born small for gestational age with a distance to target height <1 standard deviation score be excluded from growth hormone treatment?

Context: The criteria for starting growth hormone (GH), an approved treatment for short children born small for gestational age (SGA), differ between Europe and the USA. One European requirement for starting GH, a distance to target height (DTH) of > or =1 standard deviation score (SDS), is controversial.

Objective: To investigate the influence of DTH on growth during GH treatment in short SGA children and to ascertain whether it is correct to exclude children with a DTH <1 SDS from GH.

Serum insulin-like growth factor-binding protein-2 levels and metabolic and cardiovascular risk factors in young adults and children born small for gestational age.

Background: IGF binding protein (IGFBP)-2 might protect against cardiovascular disease. Small for gestational age (SGA) birth could be associated with a higher risk for type 2 diabetes mellitus and cardiovascular disease in later life. No data are available on the relationship between serum IGFBP-2 levels and cardiovascular risk factors in young adults and children born SGA.

The PPAR-gamma Pro12Ala polymorphism associates with weight gain during GH-treatment in short children born small for gestational age.

Context: Short children born small for gestational age (SGA) have a lean phenotype with lower insulin sensitivity and higher blood pressure. GH treatment results in weight gain, and a decrease in blood pressure and insulin sensitivity. However, not all children respond in the same way.

Type 2 diabetes gene TCF7L2 polymorphism is not associated with fetal and postnatal growth in two birth cohort studies.

Background: An inverse association between birth weight and the risk of developing type 2 diabetes (T2D) in adulthood has been reported. This association may be explained by common genetic variants related to insulin secretion and resistance, since insulin is the most important growth factor in fetal life. The objective of this study was to examine whether T2D gene polymorphism TCF7L2 rs7903146 is associated with growth patterns from fetal life until infancy.

Interactions between TCF7L2 genotype and growth hormone-induced changes in glucose homeostasis in small for gestational age children.

Context: The Transcription factor 7-like 2 (TCF7L2) rs7903146 gene polymorphism has been associated with risk of developing type 2 diabetes mellitus (DM), possibly by decreasing insulin secretion. Small for gestational age (SGA) birth has been associated with type 2 DM in later life. Growth hormone (GH) treatment reduces insulin sensitivity and increases insulin secretion.

The effect of growth hormone treatment on metabolic and cardiovascular risk factors is similar in preterm and term short, small for gestational age children.

Context: We previously reported that short, small for gestational age (SGA) children who were born preterm have a lower body fat percentage and a higher blood pressure, insulin secretion and disposition index than short SGA children born at term. Whether preterm birth also influences these parameters during GH treatment is unknown.

Objective: To compare blood pressure, insulin sensitivity, beta-cell function and body composition during 4 years of GH treatment, between preterm and term short SGA children.

Does preterm birth influence the response to growth hormone treatment in short, small for gestational age children?

Objective: To investigate whether prematurity has an independent influence on the response to GH treatment in short, small for gestational age (SGA) children.

Design: A longitudinal 3-year GH study.

Patients: A total of 392 prepubertal non-GH-deficient, short SGA children, comprising 138 preterm (< 36 weeks) and 254 term (>or= 36 weeks) children.

Cardiovascular risk factors in parents of short children born small for gestational age.

Small for gestational age (SGA) children have a higher prevalence of cardiovascular risk factors at a young age. It is not known whether this increased risk is caused by their size at birth, a familial predisposition for cardiovascular disease or smallness at birth or a combination of these factors. The cardiovascular risk profile of parents of SGA children is unknown.

Plasma matrix metalloproteinase-9 levels and blood pressure in short children born small for gestational age and effects of growth hormone treatment.

Context: Short small-for-gestational-age (SGA) children have an increased systolic blood pressure (BP) that decreases during long-term GH treatment. The underlying mechanism is still unknown. Matrix metalloproteinases (MMPs) are zinc-dependent endoproteinases that are involved in the remodelling of the extracellular matrix (ECM) and are thought to play a role in atherosclerosis.

Independent effects of prematurity on metabolic and cardiovascular risk factors in short small-for-gestational-age children.

Context: Both small-for-gestational-age (SGA) and preterm birth have been associated with an increased incidence of adult cardiovascular disease and diabetes mellitus type 2. However, it is unclear whether preterm birth has an additional effect on cardiovascular risk factors in short children born SGA.

Objective: Our objective was to investigate whether prematurity has an independent influence on several cardiovascular risk factors within a population of short SGA children.

Infliximab in a child with therapy-resistant systemic vasculitis.

Treatment of systemic vasculitides is usually based on the use of corticosteroids and other immunosuppressive drugs. We describe a 10-year-old girl with systemic vasculitis resistant to immunosuppressive treatment who had a rapid and impressive response to treatment with infliximab.