Glycemic control by treatment modalities: national registry-based population data in children and adolescents with type 1 diabetes
AIMS To assess the differences in key parameters of type 1 diabetes (T1D) control associated with treatment and monitoring modalities including newly introduced hybrid closed-loop (HCL) algorithm in children and adolescents with T1D (CwD) using the data from the population-wide pediatric diabetes registry ČENDA. MATERIALS AND METHODS CwD younger than 19 years with T1D duration> 1 year were included and divided according to the treatment modality and type of CGM used: multiple daily injection (MDI), insulin pump without (CSII) and with HCL function, intermittently scanned continuous glucose monitoring (isCGM), real-time ...
Source: Hormone Research in Paediatrics - April 26, 2023 Category: Endocrinology Source Type: research

Key stages in the development and establishment of paediatric endocrinology - a template for future progress
Background Paediatric endocrinology became recognised in Western European countries in the 1960s and 1970s. It is now a thriving paediatric sub-specialty in many countries, but remains non-existent or in its infancy in others. We have had the privilege to work in Western centres of excellence and this review outlines the key stages in the development of modern centres, discussing the human and organisational issues that have under-pinned progress in the establishment of this paediatric sub-specialty. Summary Human determination, vision and ambition to create a modern centre and become a national flag-bearer in the field a...
Source: Hormone Research in Paediatrics - April 25, 2023 Category: Endocrinology Source Type: research

A critical review of upcoming new therapeutic options in paediatric endocrinology
Novel and innovative therapeutic options are now available or will soon become available for treating children and adolescents with endocrine disorders and diabetes. Some of these new medicines and procedures have already been proven effective and safe in adults at least in the short term but their use in children is still limited and some efficacy and safety related aspects raise caution especially in the long term. The purpose of this issue is to provide an overview of some of the medicines upcoming to the market, highlighting the advantages and the still existing grey zones. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 24, 2023 Category: Endocrinology Source Type: research

Hormone Replacement Therapy After Pubertal Induction in Adolescents and Young Adults with Turner Syndrome: A Survey Study.
Conclusion: Although most endocrinologists and gynecologists report confidence in prescribing HRT to AYA with TS after pubertal induction, there are clear differences in provider preferences based on specialty and higher volume of patients with TS in their practice. Additional studies on comparative effectiveness of the HRT regimens and evidence-based guidelines are necessary for AYA with TS. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 24, 2023 Category: Endocrinology Source Type: research

Effect of Growth Hormone Therapy on Pubertal Timing: Systematic Review and Meta-analysis
Conclusions Treatment with rhGH appears to promote earlier pubertal timing among children with ISS. Evidence was lacking in children with growth hormone deficiency due to the absence of studies with untreated controls. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 19, 2023 Category: Endocrinology Source Type: research

Assessment of nutritional status in the diagnostic evaluation of the child with growth failure
Current clinical guidelines provide information about the diagnostic work-up of children with growth failure. This mini-review focuses on the nutritional assessment, which has received relatively little attention in such guidelines. The past medical history, in particular a low birth size, early feeding problems and failure to thrive, can provide information that can increase the likelihood of nutritional deficits or several genetic causes. The current medical history should include a dietary history, and can thereby reveal a poorly-planned or severely restricted diet, which can be associated with nutritional deficiencies....
Source: Hormone Research in Paediatrics - April 13, 2023 Category: Endocrinology Source Type: research

Growth hormone dose modulation and final height in short children born small for gestational age: French real-life data
Conclusions GH therapy was fairly effective in most short children born SGA. No new safety concerns were identified. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 11, 2023 Category: Endocrinology Source Type: research

Improvements in glucose regulation in children and young people with cystic fibrosis related diabetes following initiation of Elexacaftor/Tezacaftor/Ivacaftor
Conclusion: ELX/TEZ/IVA has a positive impact on glycaemic control and insulin requirements in children with CFRD. Close monitoring is required when commencing treatment. Children with CFRD need counselling regarding possible reductions in insulin requirement and re-education regarding symptoms, signs and management of hypoglycaemia. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 11, 2023 Category: Endocrinology Source Type: research

The Genetic Landscape of Children Born Small for Gestational Age with Persistent Short Stature
Conclusions: The high diagnostic yield sheds a new light on the genetic landscape of SGA-SS, with a central role for the growth plate with substantial contributions from the GH-IGF-1 and thyroid axes and intracellular regulation and signalling. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 5, 2023 Category: Endocrinology Source Type: research

The genetic landscape of children born small for gestational age with persistent short stature (SGA-SS)
Introduction. Among children born small for gestational age, 10-15% fails to catch-up and remains short (SGA-SS). The underlying mechanisms are mostly unknown. We aim to decipher genetic aetiologies of SGA-SS within a large single-centre cohort. Methods. Out of 820 patients treated with growth hormone (GH), 256 were classified as SGA-SS (birth length and/or birth weight (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 5, 2023 Category: Endocrinology Source Type: research

Weekly Growth Hormone (Lonapegsomatropin) Causes Severe Transient Hyperglycemia in a Child with Obesity
Conclusion: To conclude, lonapegsomatropin-tcgd offers the convenience of a weekly, rather than daily GH treatment; however, this patient developed a rapid increase in insulin resistance and hyperglycemia requiring insulin. The discrepancy between the glucose of 500 mg/dL and A1c of 5.7%, along with the rapid resolution of hyperglycemia is further consistent with a medication side effect. Close glucose monitoring of patients initiated on weekly growth hormone is crucial, particularly in those with a history of pre-diabetes. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 4, 2023 Category: Endocrinology Source Type: research

Why should orchidopexy be performed in congenital hypogonadotropic hypogonadism, and when?
Conclusion: Gonadotropin deficiency may protect against testicular cancer and orc hidopexy in this context may be deferred. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - April 4, 2023 Category: Endocrinology Source Type: research

Erratum
Horm Res Paediatr (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 30, 2023 Category: Endocrinology Source Type: research

Molecular genetic analysis and growth hormone treatment in a three-generation Chinese family with Tricho-rhino-phalangeal syndrome I
Conclusions: The variation of c.880_882delAAG in TRPS1 was responsible for the pathogenesis of the Chinese family with TRPS I. The treatment of GH could be beneficial for the height outcome in TRPS I patients, and earlier initiation and longer duration of the therapy in prepubertal or early pubertal stage could be associated with better height outcomes. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 29, 2023 Category: Endocrinology Source Type: research

The effects of five years of growth hormone treatment on growth and body composition in patients with Temple syndrome
Conclusion: GH-treatment in patients with TS14 normalizes height SDS and improves body composition. There were no adverse effects or safety concerns during GH-treatment. (Source: Hormone Research in Paediatrics)
Source: Hormone Research in Paediatrics - March 28, 2023 Category: Endocrinology Source Type: research