Peer Review of Paediatric Endocrine Services in the UK: A Template for Quality and Service Improvement
Conclusions: The BSPED peer review of all UK centres providing paediatric endocrine services is shown to be feasible and provides a quality benchmark for replication by national services.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 31, 2021 Category: Endocrinology Source Type: research

A Complicated Case of COVID-19 and Hyperglycemic Hyperosmolar Syndrome in an Adolescent Male
We report the case of a critically ill adolescent male with obesity, type 2 diabetes, and COVID-19 who presented with hyperglycemic hyperosmolar syndrome (HHS). This case highlights a challenge for clinicians in distinguishing severe complications of COVID-19 from those seen in HHS. Youth with obesity and type 2 diabetes may represent a high-risk group for severe COVID-19 disease, an entity that to date has been well-recognized in adults but remains rare in children and adolescents.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 31, 2021 Category: Endocrinology Source Type: research

Post-Transplantation Diabetes Mellitus in Pediatric Patients
More than 80% of pediatric solid organ transplant (SOT) recipients now survive into young adulthood and many encounter transplant-related complications. Post-transplantation diabetes mellitus (PTDM), sometimes also referred to as post-transplant diabetes or new onset diabetes after transplant, occurs in 3 –20% of pediatric SOT recipients depending upon the organ transplanted, age at transplantation, immunosuppressive regimen, family history, and time elapsed since transplant. To diagnose PTDM, hyperglycemia must persist beyond the initial hospitalization for transplantation when a patient is on sta ble doses of immunosup...
Source: Hormone Research in Paediatrics - March 31, 2021 Category: Endocrinology Source Type: research

Genotypic Sex and Severity of the Disease Determine the Time of Clinical Presentation in Steroid 17 α-Hydroxylase/17,20-Lyase Deficiency
Conclusions: Early clinical presentation and diagnosis in 17OHD are associated with symptomatic hypertension in both 46,XX and 46,XY patients or inadequate virilization of external genitalia in 46,XY partial 17OHD. In the absence of these, the clinical presentation is at late pubertal ages at which time amenorrhea and elevated gonadotropins are the hints for diagnosis.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 29, 2021 Category: Endocrinology Source Type: research

Fertility of Women Treated during Childhood with Triptorelin (Depot Formulation) for Central Precocious Puberty: The PREFER Study
Conclusion: The results, based on a limited sample of patients, suggest that CPP treated with triptorelin does not negatively impact women ’s fertility in adulthood. These results need to be consolidated with a subsequent study performed when these women will have reached their mid-thirties.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 26, 2021 Category: Endocrinology Source Type: research

A Comparison of Patients with Central Precocious Puberty Who Have a Pubertal versus Prepubertal Ultrasensitive LH at Presentation
Conclusions: The LH-ICMA can be prepubertal even in girls with advanced development. To our knowledge, ours is the largest cohort of boys in whom the accuracy of a random LH-ICMA has been reported.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 25, 2021 Category: Endocrinology Source Type: research

Primary Dwarfism, Microcephaly, and Chorioretinopathy due to a PLK4 Mutation in Two Siblings
Conclusion: Biallelic variants inPLK4 lead to a syndrome where severe short stature, MCPH, and cognitive impairment are constant features. However, ocular, skeletal, and other neurological manifestations can vary upon the same genetic basis.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 23, 2021 Category: Endocrinology Source Type: research

Socioeconomic Status Is Related to Pubertal Development in a German Cohort
Conclusion: Puberty onset/duration and boys ’ age at mutation is affected by SES. The proposed cutoff levels for serum LH and FSH could provide a serological tool to determine gonadarche in boys.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 17, 2021 Category: Endocrinology Source Type: research

Late Recovery of Parathyroid Function after Total Thyroidectomy in Children and Adults: Is There a Difference?
Conclusion: Almost half of patients recovered from persistent hypoparathyroidism after 6 months; therefore, the term persistent instead of permanent hypoparathyroidism should be used. Recovery rates of parathyroid function in children and adults were similar. Regardless of age, predictive factors for recovery were PGRIS score = 4 and a serum calcium #x3e;2.25 mmol/L at 1 month.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 11, 2021 Category: Endocrinology Source Type: research

Early Adiposity Rebound Predicts Obesity and Adiposity in Youth with Congenital Adrenal Hyperplasia
Conclusions: AR in youth with classical CAH could serve as a useful clinical marker to identify those patients who are at higher risk for developing cardiometabolic risk factors during childhood and adolescence.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 10, 2021 Category: Endocrinology Source Type: research

Metabolic Effects of Growth Hormone Treatment in Short Prepubertal Children: A Double-Blinded Randomized Clinical Trial
Conclusion: Treatment with GH resulted in a dose-dependent decrease in insulin sensitivity, demonstrated by higher levels of fasting insulin and signs of insulin resistance in both HOMA indices and FSIVGTT examinations.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 8, 2021 Category: Endocrinology Source Type: research

Isolated 17, 20 Lyase Deficiency Secondary to a Novel CYB5A Variant: Comparison of Steroid Metabolomic Findings with Published Cases Provides Diagnostic Guidelines and Greater Insight into Its Biological Role
Conclusion: This is the fourth disease-causing variant inCYB5A in isolated 17, 20 lyase deficiency and the first associated with testicular microlithiasis. Establishing a biochemical diagnosis pre-pubertally should now be possible using urine steroid profiling, supported by synacthen and gonadotrophin stimulation testing. We recommend liquid chromatography-mass spectrometry/mass spectrometry rather than immunoassay for serum steroid analysis, early methaemoglobin measurement and surveillance should testicular microlithiasis be detected.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - February 24, 2021 Category: Endocrinology Source Type: research

Diagnosis of Growth Hormone Deficiency in Children: The Efficacy of Glucagon versus Clonidine Stimulation Test
Conclusions: Screening of GH status with CST first is more efficient than that with GST first in diagnosing GHD in short children with suspected GHD. It is suggested that performing CST first may reduce the need for a second provocative test and avoid patients ’ inconvenience of undergoing 2 serial tests.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - February 10, 2021 Category: Endocrinology Source Type: research

Birthweight Differences in Adolescent Monozygotic Twins Influence Androgens, Psychological Morbidity, and Health-Related Quality of Life
Conclusion: Our results show that the prenatal environment leading to bw differences exerts a long-lasting impact on diverging parental evaluation of mental health. Formerly smaller discordant twins showed significantly lower HrQoL regarding psychological well-being and moods and emotions. Higher androstenedione concentrations were linked to greater psychological well-being.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - February 10, 2021 Category: Endocrinology Source Type: research

Cortisol and Cortisone in Early Childhood in Very-Low-Birthweight Infants and Term-Born Infants
Conclusions: In VLBW infants, lower 11 β-HSD2 activity probably contributes to the long-term metabolic and cardiovascular risks. In VLBW infants, early insulin treatment could affect programming of the HPA axis, resulting in higher cortisol and cortisone levels during early childhood.Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - February 3, 2021 Category: Endocrinology Source Type: research