Outcome of COVID-19 infections in patients with adrenal insufficiency and excess.

Background: Information on clinical outcomes of coronavirus disease 19 (COVID-19) infection in patients with adrenal disorders is scarce.

Methods: A collaboration between the European Society of Endocrinology (ESE) Rare Disease Committee and European Reference Network on Rare Endocrine Conditions via the European Registries for Rare Endocrine Conditions allowed the collection of data on 64 cases (57 adrenal insufficiency (AI), 7 Cushing's syndrome) that had been reported by 12 centres in 8 European countries between January 2020 and December 2021.

Results: Of all 64 patients, 23 were males and 41 females (13 of those children) with a median age of 37 and 51 years.

Parental Stress and Scalp Hair Cortisol in Excessively Crying Infants: A Case Control Study.

Background: Caring for an excessively crying infant (ECI) can be stressful for mothers and fathers and is associated with mental and bonding problems. Hair cortisol offers a unique measure for the biological reaction of the body to stress over time.

Methods: In this case-control study, scalp hair cortisol concentrations (HCC) were measured using liquid chromatography-tandem mass spectrometry (LC-MS) in 35 mothers and 23 fathers and their ECIs.

Mild perinatal adversities moderate the association between maternal harsh parenting and hair cortisol: Evidence for differential susceptibility.

It has been shown that following exposure to mild perinatal adversity, children have greater susceptibility to both the negative and positive aspects of their subsequent environment. In a large population-based cohort study (N = 1,776), we investigated whether mild perinatal adversity moderated the association between maternal harsh parenting and children's hair cortisol levels, a biomarker of chronic stress. Mild perinatal adversity was defined as late preterm birth (gestational age at birth of 34-37 weeks, 6 days) or small for gestational age (birth weight between the 2.

Hair cortisol concentrations exhibit a positive association with salivary cortisol profiles and are increased in obese prepubertal girls.

Cortisol, a key mediator of the stress response, has been associated with obesity and metabolic syndrome manifestations as early as in childhood. Scalp hair cortisol has been proposed as a reliable index of long-term circulating cortisol. We aimed to investigate whether obese prepubertal girls have higher scalp hair cortisol than normal-weight controls and whether hair cortisol levels are correlated with salivary cortisol concentrations in these groups.

High predictability of impaired glucose tolerance by combining cardiometabolic screening parameters in obese children.

Background: There is debate on which overweight and obese children should be screened for the presence of impaired glucose tolerance (IGT) by oral glucose tolerance testing (OGTT). The objective of the study was to identify risk factors predictive of the presence of IGT.

Methods: In a cohort of overweight children, who underwent OGTT, we determined the association of anthropometric and laboratory parameters with IGT and whether combining parameters improved the sensitivity of screening for IGT.

Mutations in TBL1X Are Associated With Central Hypothyroidism.

Context: Isolated congenital central hypothyroidism (CeH) can result from mutations in TRHR, TSHB, and IGSF1, but its etiology often remains unexplained. We identified a missense mutation in the transducin β-like protein 1, X-linked (TBL1X) gene in three relatives diagnosed with isolated CeH. TBL1X is part of the thyroid hormone receptor-corepressor complex.

Gonadal function in boys with newly diagnosed cancer before the start of treatment.

Study Question: Are Inhibin B and testosterone levels reduced in boys with newly diagnosed cancer prior to therapy?

Summary Answer: Pretreatment serum levels of Inhibin B and testosterone are significantly reduced in boys with newly diagnosed cancer, compared to reference values.

What Is Already Known: Disease-related gonadal impairment has been demonstrated in girls and young women diagnosed with cancer, prior to therapy.

Study Design, Size, Duration: We conducted a descriptive study in boys newly diagnosed with cancer between January 2006 and February 2014.

Nationwide Evaluation of Congenital Hypothyroidism Screening during Neonatal Extracorporeal Membrane Oxygenation.

Background: Thyroid hormone concentrations may deviate from normal values during critical illness. This condition is known as nonthyroidal illness syndrome (NTIS), and it can influence the results of screening for congenital hypothyroidism (CH) during neonatal extracorporeal membrane oxygenation (ECMO).

Objectives: To determine the incidence of aberrant CH screening results in ECMO-treated neonates, to identify possible determinants, and to follow up patients with abnormal thyroid hormone concentrations.

Glucocorticoid receptor haplotype and metabolic syndrome: the Lifelines cohort study.

Objective: An excess of glucocorticoids (Cushing's syndrome) is associated with metabolic syndrome (MetS) features. Several single-nucleotide polymorphisms (SNPs) in the glucocorticoid receptor (GR) gene influence sensitivity to glucocorticoids and have been associated with aspects of MetS. However, results are inconsistent, perhaps due to the heterogeneity of the studied populations and limited samples.

Predicting the neurobehavioral side effects of dexamethasone in pediatric acute lymphoblastic leukemia.

Although dexamethasone is an effective treatment for acute lymphoblastic leukemia (ALL), it can induce a variety of serious neurobehavioral side effects. We hypothesized that these side effects are influenced by glucocorticoid sensitivity at the tissue level. We therefore prospectively studied whether we could predict the occurrence of these side effects using the very low-dose dexamethasone suppression test (DST) or by measuring trough levels of dexamethasone.

Acute Activation of Metabolic Syndrome Components in Pediatric Acute Lymphoblastic Leukemia Patients Treated with Dexamethasone.

Although dexamethasone is highly effective in the treatment of pediatric acute lymphoblastic leukemia (ALL), it can cause serious metabolic side effects. Because studies regarding the effects of dexamethasone are limited by their small scale, we prospectively studied the direct effects of treating pediatric ALL with dexamethasone administration with respect to activation of components of metabolic syndrome (MetS); in addition, we investigated whether these side effects were correlated with the level of dexamethasone. Fifty pediatric patients (3-16 years of age) with ALL were studied during a 5-day dexamethasone course during the maintenance phase of the Dutch Childhood Oncology Group ALL-10 and ALL-11 protocols.

Is poor neonatal adaptation after exposure to antidepressant medication related to fetal cortisol levels? An explorative study.

Background: As a marker for poor neonatal adaptation (PNA) is lacking, the diagnostic process is difficult and includes invasive additional testing.

Aims: In order to develop a marker, it is essential to gain insight into the etiology of PNA. We hypothesized that the fetal cortisol level may play a role in this etiology.

Hydrocortisone as an Intervention for Dexamethasone-Induced Adverse Effects in Pediatric Patients With Acute Lymphoblastic Leukemia: Results of a Double-Blind, Randomized Controlled Trial.

Purpose: Dexamethasone is a key component in the treatment of pediatric acute lymphoblastic leukemia (ALL), but can induce serious adverse effects. Recent studies have led to the hypothesis that neuropsychological adverse effects may be a result of cortisol depletion of the cerebral mineralocorticoid receptors. We examined whether including a physiologic dose of hydrocortisone in dexamethasone treatment can reduce neuropsychologic and metabolic adverse effects in children with ALL.

Scalp hair 17-hydroxyprogesterone and androstenedione as a long-term therapy monitoring tool in congenital adrenal hyperplasia.

Background: Glucocorticoid replacement therapy in congenital adrenal hyperplasia (CAH) is challenging, especially in children, because both over- and under-dosing may have profound and long-lasting adverse effects. Clinical follow-up parameters are largely nonspecific and slow to develop. Steroid concentrations in scalp hair may be a useful monitoring tool, as it provides information on both long-term steroid precursor and glucocorticoid exposure.

Splitting hair for cortisol? Associations of socio-economic status, ethnicity, hair color, gender and other child characteristics with hair cortisol and cortisone.

The aim of this study was to examine associations of SES and ethnicity with hair cortisol and cortisone and to identify potential child and family characteristics that can assist in choosing covariates and potential confounders for analyses involving hair cortisol and cortisone concentrations. Hair samples were collected in 2484 6-year-old children from the Generation R Study, a prospective cohort in Rotterdam, the Netherlands. Measurements for cortisol and cortisone were used as the outcome in regression analyses.

Determinants of hair cortisol and hair cortisone concentrations in adults.

Background: The analysis of hair cortisol concentrations (HairF) is a promising new tool for the assessment of long-term cortisol. With the development of multiple steroid analyses by means of liquid chromatography tandem-mass spectrometry (LC-MS/MS), the analysis of cortisone in hair (HairE) has also been facilitated. However, the influence of various types of determinants on HairF and HairE is still largely unknown.

LC-MS/MS-based method for long-term steroid profiling in human scalp hair.

Background: Liquid chromatography-tandem mass spectrometry (LC-MS/MS) is the method of choice for quantification of steroids. Human scalp hair provides the possibility to measure long-term retrospective steroid concentrations, which is especially useful for steroids with large time-dependent fluctuations in concentration, such as the glucocorticoid cortisol.

Aim: We set up and validated a LC-MS/MS-based method for long-term steroid profiling, quantifying cortisol, cortisone, testosterone, androstenedione, dehydroepiandrosterone sulphate (DHEAS) and 17-α-hydroxyprogesterone (17OHP).

The negative impact of being underweight and weight loss on survival of children with acute lymphoblastic leukemia.

Body mass index and change in body mass index during treatment may influence treatment outcome of pediatric patients with acute lymphoblastic leukemia. However, previous studies in pediatric acute lymphoblastic leukemia reported contradictory results. We prospectively collected data on body composition from a cohort of newly diagnosed Dutch pediatric patients with acute lymphoblastic leukemia (n=762, age 2-17 years).

[Neonatal screening for congenital hypothyroidism: more than 30 years of experience in the Netherlands].

Objective: To describe the Dutch neonatal screening programme for congenital hypothyroidism (CH).

Design: Descriptive study.

Method: Data on neonatal screening for CH in the period 1 January 1981 through 31 December 2011 were obtained from the Department for Vaccine Supply and Prevention Programmes of the Dutch National Institute for Public Health and the Environment (RIVM), laboratories and paediatricians to whom babies with abnormal screening results were referred.

Functional characterization of a new human melanocortin-4 receptor homozygous mutation (N72K) that is associated with early-onset obesity.

The melanocortin 4 receptor (MC4R) is expressed in the hypothalamus and is essential for regulation of appetite and energy expenditure. MC4R dysfunction in humans causes hyperphagia, impaired satiety and obesity. We have identified a novel c.

Glucocorticoid receptor polymorphisms and haplotypes and their expression in health and disease.

Cortisol is involved in many physiological processes, including immunosuppressive and anti-inflammatory actions, and therefore cortisol and its synthetic analogs are widely used to treat a large number of diseases. In glucocorticoid treatment, a large variability of clinical responses is observed. This variability may, in part, be ascribed to genetic variation in the glucocorticoid receptor (GR) gene.

Validation and reference ranges of hair cortisol measurement in healthy children.

Background: Cortisol is produced in a circadian rhythm controlled by the hypothalamus-pituitary-adrenal axis, making it cumbersome to measure long-term cortisol exposure. Hair has proven to be a reliable matrix for long-term cortisol measurement in adults and can be used as diagnostic tool for (cyclic) Cushing's syndrome. The diagnostic applicability in children has not been studied, nor have the effects of development and hair care been evaluated in children.

Five-year follow-up of effects of neonatal intensive care and morphine infusion during mechanical ventilation on diurnal cortisol rhythm.

Objective: To test the hypothesis that the diurnal cortisol secretion rhythm of children who as neonates had been hospitalized differs from that of children without a history of neonatal hospital admission and that this rhythm differs between these hospitalized children treated with either continuous morphine infusion or placebo.

Study Design: A follow-up cohort study was performed with 5-year-old children who as neonates participated in a randomized controlled trial of continuous morphine infusion (born 24-42 weeks' gestation), and a control group of healthy term born (≥ 37 weeks' gestation) children. Five saliva samples over a school day were assayed for cortisol concentrations.

Long-term cortisol levels measured in scalp hair of obese patients.

Objective: In obese subjects a relatively high cortisol output in urine has been observed compared to nonobese individuals. However, cortisol levels in blood, saliva, and urine in association with obesity have been inconsistent across studies, possibly due to the high variability of systemic cortisol levels. Cortisol levels measured in scalp hair provide a marker for long-term cortisol exposure, and have been associated with cardiovascular disease in an elderly population and to disease course in Cushing's disease.