A GH secretagogue receptor agonist (LUM-201) elicits greater GH responses than standard GH secretagogues in subjects of a pediatric GH deficiency trial
IAbstract:
Introduction: LUM-201 (ibutamoren, formerly MK-0677) is an orally administered GH secretagogue receptor agonist under development for treatment of paediatric growth hormone deficiency (PGHD)
Methods: The GH Response to a single dose of LUM-201 and to other GH secretagogues used for diagnosis of PGHD were compared in 68 pediatric subjects participating in a trial for growth hormone deficiency
Results: LUM-201 elicited greater GH responses than observed in GHD diagnostic tests with arginine, glucagon, clonidine, L-dopa and insulin induced hypoglycemia (median and interquartile ranges 15.0 ng/mL (3.5, 49) versus...
Source: Hormone Research in Paediatrics - March 30, 2022 Category: Endocrinology Source Type: research
Congenital central hypothyroidism caused by a novel IGSF1 variant identified in a French family
This report of a rare case of neonatal CCH caused by IGSF1 deficiency highlights the importance of recognizing the neonatal signs of hypothyroidism to diagnose CCH as early as possible. Our results also show the importance of performing family genetic screening if a pathogenic variant is identified, to properly monitor carriers as CCH may develop over time. We suggest that these families should be followed up in the long-term to better understand the natural history of this syndrome and evaluate the need for hormone substitution. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 29, 2022 Category: Endocrinology Source Type: research
20 years of neonatal screening for congenital adrenal hyperplasia in north-eastern italy:
role of lc-ms/ms as a second tier test
Conclusions: Screening for CAH is useful in the neonatal diagnosis of classic form of 21-OHD, allowing a precocious treatment of affected children. The introduction of a LC-MS/MS second tier reduced the recall rate, avoiding unnecessary blood withdraws and medical evaluations and preventing stress t o families. Furthermore, it helped identify rarer forms of CAH. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 29, 2022 Category: Endocrinology Source Type: research
Erratum
Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 28, 2022 Category: Endocrinology Source Type: research
Improving Anxiety Screening in Patients with Turner Syndrome
Conclusion: Our study demonstrated a high prevalence of anxiety symptoms within a cohort of 92 females with TS. In alignment with current guidelines, these findings indicate the importance of routine neuropsychological assessments for timely recognition and subsequent management of anxiety, especi ally as milder presentations may otherwise go unnoticed. We have shown that screening tools, such as the PROMIS® Anxiety measure, can be easily utilized by non-mental health care providers (i.e. endocrinologists) who may see TS patients more frequently and be able to initiate impactful discussions surrounding mental health and f...
Source: Hormone Research in Paediatrics - March 21, 2022 Category: Endocrinology Source Type: research
Care for Transgender Young People
Clinicians of all disciplines, including pediatric endocrinologists, are likely to encounter transgender and gender-diverse (TGD) young people in their practice regardless of whether they specialize in gender-affirming medical care. Because of this, it is important to be aware of the ways in which medical professionals can affirm these individuals. Although gender-affirming therapy should always include affirmation including proper use of names and pronouns, the transition journey will look different for each patient. The gender-affirming care of TGD young people may include both medical and nonmedical interventions (e.g.,...
Source: Hormone Research in Paediatrics - March 10, 2022 Category: Endocrinology Source Type: research
The Care of Transgender Young People
Clinicians of all disciplines, including pediatric endocrinologists, are likely to encounter transgender and gender diverse (TGD) young people in their practice regardless of whether they specialize in gender-affirming medical care. Because of this, it is important to be aware of the ways in which medical professionals can affirm these individuals. Although gender-affirming therapy should always include affirmation including proper use of names and pronouns, the transition journey will look different for each patient. The gender-affirming care of TGD young people may include both medical and non-medical interventions (e.g....
Source: Hormone Research in Paediatrics - March 10, 2022 Category: Endocrinology Source Type: research
Basic Concepts and Hormonal Regulators of the Stress System
Background: Human organisms have to cope with a large number of external or internal stressful stimuli that threaten -or are perceived as threatening- their internal dynamic balance or homeostasis. To face these disturbing forces, or stressors, organisms have developed a complex neuroendocrine system, the stress system, which consists of the hypothalamic-pituitary-adrenal (HPA) axis and the locus caeruleus (LC)/norepinephrine (NE)-autonomic nervous system (ANS).
Summary: Upon exposure to stressors beyond a certain threshold, the activation of the stress system leads to a series of physiological and behavioral adaptations ...
Source: Hormone Research in Paediatrics - March 10, 2022 Category: Endocrinology Source Type: research
Switching to Weekly Lonapegsomatropin from Daily Somatropin in Children with Growth Hormone Deficiency: The fliGHt Trial
Conclusions. Lonapegsomatropin treatment outcomes were as expected across a range of ages and treatment experiences. Switching to lonapegsomatropin resulted in a similar AE profile to daily somatropin therapy. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 9, 2022 Category: Endocrinology Source Type: research
Metabolic and Bariatric Surgery in adolescents – for whom, when, and how?
Severe obesity in adolescence profoundly impacts health and social wellbeing. Lifestyle modifications are seldom successful in maintaining sufficient weight loss to mitigate the risk of complications. Metabolic and Bariatric surgery (MBS) is a standard treatment for adult patients and has emerged as an option for adolescent patients. Several high-quality studies of adolescent MBS show substantial and sustained improvements both in weight and cardiometabolic parameters, as well as a safety profile similar to that seen in adult patients. Patients report improvements in health- and weight-related quality of life. Concerns aro...
Source: Hormone Research in Paediatrics - March 9, 2022 Category: Endocrinology Source Type: research
Early Life Stress, Hormones, and Neurodevelopmental Disorders
Background: Early life stress (ELS) describes a broad spectrum of adverse and stressful prenatal events, namely, prenatal maternal stress (PMS), or early postnatal events, which can have detrimental long-term influences on the physiology, cognition, and behavior of an individual. There is abundant evidence indicating that ELS exerts its lasting effects on the physical and mental health of the individual, likely acting through a number of mediating mechanisms, including the disruption of developmental programming of the fetus. Neurodevelopmental disorders (NDDs), for example, attention deficit hyperactivity disorder (ADHD) ...
Source: Hormone Research in Paediatrics - March 8, 2022 Category: Endocrinology Source Type: research
Early Life Stress, Hormones and Neurodevelopmental Disorders
Background: Early Life Stress (ELS) describes a broad spectrum of adverse and stressful prenatal events, namely prenatal maternal stress (PMS), or early postnatal events, which can have detrimental long-term influences on the physiology, cognition, and behavior of an individual. There is abundant evidence indicating that ELS exerts its lasting effects on the physical and mental health of the individual, likely acting through a number of mediating mechanisms, including the disruption of developmental programming of the fetus. Neurodevelopmental disorders (NDDs), for example, attention deficit hyperactivity disorder (ADHD) a...
Source: Hormone Research in Paediatrics - March 8, 2022 Category: Endocrinology Source Type: research
Contents Vol. 94, 2021
Horm Res Paediatr 2021;94:I –VIII (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 4, 2022 Category: Endocrinology Source Type: research
Acknowledgement to the Reviewers
Horm Res Paediatr 2021;94:469 –470 (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 4, 2022 Category: Endocrinology Source Type: research
Novel treatment options in childhood bone diseases
(Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 2, 2022 Category: Endocrinology Source Type: research
