Early insulin-resistance, T2D and treatment options in childhood
Background T2D (Type 2 Diabetes) represents just the tip of the iceberg of the complex metabolic alterations associated with obesity and other clinical conditions associated to impaired adipose tissue storage. Summary Available data have suggested the presence of a continuous spectrum of metabolic alterations developed in the progression from IR to T2D, most of which are likely preventable through the early characterization of all the multiple risk factors involved. Therefore, the complete characterization of the natural history of the disease and the major modifiable factors represents a milestone in the daily care of yo...
Source: Hormone Research in Paediatrics - December 16, 2021 Category: Endocrinology Source Type: research

Hypogonadism in male infants and adolescents: new androgen formulations
Background Male hypogonadism may be associated with micropenis and cryptorchidism in newborn, absent or incomplete pubertal development when it occurs during childhood. During puberty, androgen replacement therapy plays a pivotal role in subjects with hypogonadism to induce sexual maturation, growth acceleration, anabolic effects on fat-free mass growth increasing muscle strength, directly and indirectly on the attainment of peak bone mass in young men. Moreover, in newborns with congenital hypogonadism, androgen therapy could be effective to increase genital size. Summary Testosterone replacement therapy (TRT) represen...
Source: Hormone Research in Paediatrics - December 16, 2021 Category: Endocrinology Source Type: research

Elevated Urinary VEGF-A, Transferrin, and Angiotensinogen Levels in Normoalbuminuric Children and Adolescents with Type 1 Diabetes: Can They Be Early Markers of Diabetic Kidney Disease?
Conclusion: Normoalbuminuric and normotensive children and adolescents with T1D have elevated urinary VEGF, AGT, and transferrin levels, which may indicate the development of DKD before albuminuria occurs. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - December 14, 2021 Category: Endocrinology Source Type: research

Elevated Urinary VEGF-A,Transferrin, and Angiotensinogen Levels in Normoalbuminuric Children and Adolescents with Type 1 Diabetes; Can They Be Early Markers of Diabetic Kidney Disease?
Objective: We hypothesized that diabetic kidney disease (DKD) begins early, before albuminuria occurs. We therefore aimed to assess potential early urinary biomarkers of (DKD) in normoalbuminuric and normotensive children and adolescents with Type 1 Diabetes (T1D) to evaluate the relationship between these markers and clinical and laboratory risk factors for DKD. Methods: This cross-sectional study included 75 children and adolescents with T1D (62% females, mean age 13.9 ± 3.2 years) with normoalbuminuria [an albumin/creatinine ratio (ACR) below 30 mg/g creatinine]. Fifty-five age- and sex-matched healthy children and ad...
Source: Hormone Research in Paediatrics - December 14, 2021 Category: Endocrinology Source Type: research

High glycemic variability is associated with worse Continuous Glucose Monitoring metrics in children and adolescents with type 1 diabetes
Objective: The primary aim of this study was to quantify the prevalence of children and adolescents with T1D who achieve the recommended target for CV identifying the determining factors to reach this target. The secondary aim was to examine the relationship between CV, the other metrics derived from CGM data and clinical parameters. Method: CGM data were collected from 805 children/adolescents with T1D. Several CGM metrics and patients' characteristics were evaluated. Participants were stratified by CV ≤36% and CV>36%. Binary logistic regression analysis was run to identify the determining factors of high CV. Results ...
Source: Hormone Research in Paediatrics - December 13, 2021 Category: Endocrinology Source Type: research

When Is a Positive Test for Pediatric Growth Hormone Deficiency a True-Positive Test?
Background: In most cases, the growth hormone stimulation test is a necessary component for the diagnosis of growth hormone deficiency (GHD) in children. Diagnostic testing can lead to unnecessary treatment of children with false-positive test results and omission of treatment in children with false-negative results. False-positive results are suggested by the absence of typical growth responses in treated children and false-negative results are suggested by continued growth failure in those left untreated.Summary: The probability that a positive test result indicates the presence of the condition (true positive) depends o...
Source: Hormone Research in Paediatrics - December 2, 2021 Category: Endocrinology Source Type: research

When is a positive test for pediatric growth hormone deficiency a true positive test?
Given the low prevalence rate of growth hormone deficiency (GHD) and the high false positive rates for growth hormone stimulation tests, the probability of GHD in a child with short stature and positive growth hormone stimulation tests is 0.028 (about 1 in 36). Without further information, most positive growth hormone stimulation tests will be false positives. Further study may yield the necessary improvements in the diagnostic accuracy for GHD in children. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - December 2, 2021 Category: Endocrinology Source Type: research

Circulating irisin levels in preadolescents and adolescents born preterm
Conclusion. Lower levels of irisin and a slightly unhealthy adiposity and cardiometabolic pattern were detected in preterm-born children in comparison to their full-term-born peers. Whether low irisin levels in preadolescents and adolescents born prematurely could be of prognostic value for the development of cardiometabolic sequelae later in life remains to be further studied. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - December 2, 2021 Category: Endocrinology Source Type: research

Glucagon Like Peptide 1 Receptor Agonists in the Treatment of Obesity
We presented the main outcomes of clinical trial programs called SCALE and STEP that supported a market authorization approval for liraglutide and semaglutide for the treatment of obesity in adult population. Then we summarised the studies on the efficacy of GLP-1 RA in paediatric obesity that have been accumulating from two larger studies with liraglutide and few other smaller studies with exenatide and liraglutide. The results indicate that GLP-1RA are safe, tolerable, and effective in reducing weight and also in improving cardiometabolic profile in children with obesity and poor response to lifestyle intervention alone....
Source: Hormone Research in Paediatrics - December 1, 2021 Category: Endocrinology Source Type: research

The invaluable role of consanguinity in providing insight into paediatric endocrine conditions: Lessons learned from congenital hyperinsulinism, monogenic diabetes, and short stature
Consanguineous families have often played a role in the discovery of novel genes, especially in paediatric endocrinology. At this time, it has been estimated that over 8.5% of all children worldwide have consanguineous parents. Consanguinity is linked to demographic, cultural and religious practises and is more common in some areas around the world than others. In children with endocrine conditions from consanguineous families, there is a greater probability that a single gene condition with autosomal recessive inheritance is causative. From 1966 and the first description of Laron syndrome, through the discovery of the f...
Source: Hormone Research in Paediatrics - November 30, 2021 Category: Endocrinology Source Type: research

Assessment of Serum Spexin Levels In Obese Adolescents with Metabolic Syndrome Antecedents: Preliminary Results
Objective: Spexin (SPX) is a novel peptide implicated in food intake and satiety. Spexin levels are reduced in obese patients. Aim: To compare serum SPX levels in obese adolescents versus healthy controls and to assess the associations of metabolic syndrome (metS) antecedents with serum SPX levels. Methods: Eighty consecutive obese adolescents aged 10-18 years and 80 healthy peers were enrolled. Anthropometric measurements, pubertal examinations and clinical blood pressure measurements were performed. Fasting blood samples were drawn for glucose, insulin, lipids, uric acid, alanine aminotransferase (ALT) and SPX. Metabol...
Source: Hormone Research in Paediatrics - November 26, 2021 Category: Endocrinology Source Type: research

Stress and Growth in children and adolescents
The infantile, childhood, and adolescent periods of growth and development represent times of increased vulnerability to stressors. The rate of growth in each period depends on the interplay of genetic, environmental, dietary, socioeconomic, developmental, behavioral, nutritional, metabolic, biochemical, and hormonal factors. A stressor may have an impact on growth directly through modulation of the growth hormone axis or indirectly through modulation of other factors. The adaptive response to stressors culminates in behavioral, physiological, and biochemical responses, which together support survival and conservation of ...
Source: Hormone Research in Paediatrics - November 23, 2021 Category: Endocrinology Source Type: research

Copeptin; utility in paediatric patients with hyponatraemia
We report 5 children with hyponatraemia in the context of different underlying pathologies. Copeptin concentrations were elevated in 4 cases (13.7, 14.4, 26.1, 233pmol/L; reference range 2.4 – 8.6pmol/L) suggesting that non-osmoregulated vasopressin release (syndrome of inappropriate antidiuretic diuretic hormone or SIADH) was the underlying mechanism for the low sodium levels. In one of the patients there was an underlying diagnosis of Schaaf-Yang syndrome (MAGEL 2 gene mutation) wit h a clinical picture suggestive of dysregulated vasopressin production with inappropriately high and then low copeptin release. In one hyp...
Source: Hormone Research in Paediatrics - November 23, 2021 Category: Endocrinology Source Type: research

Potentialities of gene therapy in pediatric endocrinology
Gene therapy has become an appealing therapeutic option in many pediatric fields including endocrinology. Unlike traditional drugs based on molecules that require repeated and frequently burdensome administrations, a single genetic therapeutic intervention may allow durable and curative clinical benefits. Although this highly innovative technology holds great promise for the treatment of monogenic diseases, its clinical application in the field of endocrinology has been so far challenging. In this review we will discuss various ex vivo and in vivo approaches and potential application of gene addition and gene editing appr...
Source: Hormone Research in Paediatrics - November 19, 2021 Category: Endocrinology Source Type: research

Quality of Life in Chilean Transgender Children and Adolescents
Conclusion: Ou r results revealed that TG children and adolescents have lower QOL than their CIS counterparts, especially regarding items related to mental health. Furthermore, their parents may underestimate their well-being, confirming the vulnerability of the TG population. This finding underlies the need to pe rform early assessments of QOL for early detection and intervention in aspects that could deteriorate their quality of life. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - November 17, 2021 Category: Endocrinology Source Type: research