Rhizomelia and Impaired Linear Growth in a Girl with Juvenile Paget Disease: The Natural History of the Condition
In this study, we report the follow-up of height, growth velocity, and skeletal maturation in a 16-year-old female patient with JPD. The patient was treated with cyclic doses of pamidronate starting at 2.5 years of age and with 2 doses of denosumab at the age of 8 years, when pamidronate was paused. In the following years, a sustainable decline in a heightz-score and a stunted pubertal growth spurt; despite appropriate maturation of the epiphyseal plates of the left hand, the proximal right humerus and both femora were observed. Whether this reflects the growth pattern in JPD or might be associated to the antiresorptive tr...
Source: Hormone Research in Paediatrics - July 14, 2021 Category: Endocrinology Source Type: research
The Impact of Stress on Health in Childhood and Adolescence in the Era of the COVID-19 Pandemic
Background: The ongoing pandemic of coronavirus disease 2019 (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is posing many challenges to global health. Efforts from the whole scientific community have shed light on the pathogenetic mechanisms and the clinical features of SARS-CoV-2 infection as well as on potential therapeutic strategies.Summary: The consequences of stress related to social isolation and anxiety generated by the pandemic on mental and physical health are collateral effects that are yet poorly investigated.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - July 14, 2021 Category: Endocrinology Source Type: research
Growth Hormone and Insulin-Like Growth Factor Dysregulation in Pediatric Chronic Kidney Disease
Poor growth is a common finding in children with chronic kidney disease (CKD) that has been associated with poor long-term outcomes. The etiology of poor growth in this population is multifactorial and includes dysregulation of the growth hormone (GH) and insulin-like growth factor (IGF) axis. In this review, we describe the data on GH resistance or insensitivity and inappropriate levels or reduced bioactivity of IGF proposed as contributing factors of growth impairment in children with CKD. Additionally, we describe the theorized negative effect of metabolic acidosis, another frequent finding in pediatric CKD, on the GH/I...
Source: Hormone Research in Paediatrics - July 13, 2021 Category: Endocrinology Source Type: research
Gonadal Hormone Substitution in People with PWS – an IPWSO Survey
(Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - July 13, 2021 Category: Endocrinology Source Type: research
The Impact of Hypo- and Hyperglycemia on Cognition and Brain Development in Young Children with Type 1 Diabetes
Human and experimental animal data suggest both hyperglycemia and hypoglycemia can lead to altered brain structure and neurocognitive function in type 1 diabetes (T1D). Young children with T1D are prone to extreme fluctuations in glucose levels. The overlap of these potential dysglycemic insults to the brain during the time of most active brain and cognitive development may cause cellular and structural injuries that appear to persist into adult life. Brain structure and cognition in persons with T1D are influenced by age of onset, exposure to glycemic extremes such as severe hypoglycemic episodes, history of diabetic keto...
Source: Hormone Research in Paediatrics - July 9, 2021 Category: Endocrinology Source Type: research
Serum Levels of Soluble Receptor for Advanced Glycation End Products Are Reduced in Euthyroid Children with Newly Diagnosed Hashimoto ’s Thyroiditis: A Pilot Study
Conclusions: sRAGE levels were decreased in euthyroid children/adolescents at the onset of HT, suggesting that autoimmunity per se seems to play an important role in such a reduction of sRAGE, irrespective of any functional alteration. Children and adolescents suffering from HT may exhibit increased susceptibility to oxidative damage, even when in euthyroid status.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - July 8, 2021 Category: Endocrinology Source Type: research
Safety and Effectiveness of Recombinant Human Growth Hormone in Children with Turner Syndrome: Data from the PATRO Children Study
Conclusion: These data suggest that biosimilar rhGH is well tolerated and effective in TS patients managed in real-life clinical practice. Optimization of rhGH dose may contribute to a higher AH.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - July 7, 2021 Category: Endocrinology Source Type: research
Transforming Education through a Global e-Learning Model for Pediatric Diabetes and Endocrinology
Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 30, 2021 Category: Endocrinology Source Type: research
Comparison of estradiol, testostosterone and CYP19 methylation levels between full term and preterm newborns
(Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 30, 2021 Category: Endocrinology Source Type: research
Determinants of Final Height in Patients Born Small for Gestational Age Treated with Recombinant Growth Hormone
Conclusion: In this large cohort of SGA patients who had reached their final height, we were able to confirm that growth hormone increases final height in short SGA children. In addition, we identified several factors associated with a better response to growth hormone treatment.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 28, 2021 Category: Endocrinology Source Type: research
Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System
Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk...
Source: Hormone Research in Paediatrics - June 24, 2021 Category: Endocrinology Source Type: research
Adult Height in 299 Patients with Turner Syndrome with or without Growth Hormone Therapy: Results and Literature Review
Conclusions: Our study strengthens the evidence for efficacy of GH therapy in patients with TS from different populations.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 16, 2021 Category: Endocrinology Source Type: research
Associations of Obesity with Linear Growth and Puberty
Background: The prevalence of obesity in childhood has increased dramatically in recent decades with increased risk of developing cardiometabolic and other comorbidities. Childhood adiposity may also influence processes of growth and puberty.Summary: Growth patterns of obesity during childhood have been shown to be associated with increased linear growth in early childhood, leading to accelerated epiphyseal growth plate (EGP) maturation. Several hormones secreted by the adipose tissue may affect linear growth in the context of obesity, both via the growth hormone IGF-1 axis and via a direct effect on the EGP. The observati...
Source: Hormone Research in Paediatrics - June 15, 2021 Category: Endocrinology Source Type: research
A Newborn Falsely Suspected of Congenital Hypothyroidism due to Mutated Thyroxine-Binding Globulin with Low Binding Affinity
Conclusions: The novelSERPINA7 variant in this patient led to a false suspicion of central hypothyroidism in the Dutch T4-based neonatal screening program. It is important to recognize patients with such TBG defects to prevent unnecessary additional testing and treatment.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 14, 2021 Category: Endocrinology Source Type: research
Prevention of Growth Failure in Turner Syndrome: Long-Term Results of Early Growth Hormone Treatment in the “Toddler Turner” Cohort
Conclusion: Although the ET group was taller throughout, height SDS at NAH was not significantly different between groups due to catch-down growth of ET girls during lapses in GH treatment after the Toddler study and similar long-term GH exposure overall. Early initiation of GH by age 6 years, followed by uninterrupted treatment during childhood, can prevent ongoing growth failure and enable attainment of height within the normal range during childhood, adolescence, and adulthood.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - June 10, 2021 Category: Endocrinology Source Type: research
