ISSN 1662-4009 (online)

ey0019.2-2 | Neonatal hypoglycaemia | ESPEYB19

2.2. Oral dextrose gel for the treatment of hypoglycaemia in newborn infants

T Edwards , G Liu , M Battin , DL Harris , JE Hegarty , PJ Weston , JE Harding

Cochrane Database Syst Rev. 2022 Mar 18;3(3):CD011027. doi: 10.1002/14651858.CD011027.pub3. PMID: 35302645.Brief Summary: This systematic review assessed the evidence for oral dextrose gel to treat hypoglycemia in newborns. Oral dextrose gel compared with placebo gel probably improved the correction of hypoglycaemic events and may result in a slight reduction in the risk of ma...

ey0019.2-12 | Neonatal diabetes mellitus | ESPEYB19

2.12. Developmental defects and impaired network excitability in a cerebral organoid model of KCNJ11 p.V59M-related neonatal diabetes

G Dalgin , AK Tryba , AP Cohen , SY Park , LH Philipson , SAW Greeley , AJ 3rd Garcia

Sci Rep. 2021 Nov 3;11(1):21590. doi: 10.1038/s41598-021-00939-7. PMID: 34732776.Brief Summary: This study describes the molecular and electrophysiological analysis of the cerebral network generated from cerebral organoids from human induced pluripotent stem cells (hiPSCs) on a patient with neonatal diabetes mellitus due to a KCNJ11 mutation. Some patients w...

ey0019.5-11 | Translational highlights | ESPEYB19

5.11. A reference range for plasma levels of inorganic pyrophosphate in children using the ATP sulfurylase method

E Bernhard , Y Nitschke , G Khursigara , Y Sabbagh , Y Wang , F Rutsch

J Clin Endocrinol Metab. 2022 Jan 1;107(1):109-118.Abstract: https://pubmed-ncbi-nlm-nih-gov/34498693/In Brief: This study established a standard range of Inorganic Pyrophosphate (PPi) between 2.36 and 4.44 µM (5th-95th percentiles) in the blood plasma of children and adolescents aged 0 to 18 years, using the ATP sulfurylase assay. There was no sex difference and the range is similar t...

ey0019.6-14 | Gender Incongruence: Wider impacts of gender affirming hormone (GAH) treatments | ESPEYB19

6.14. Sex hormones drive changes in lipoprotein metabolism

GA Robinson , J Peng , H Peckham , A Radziszewska , G Butler , I Pineda-Torra , EC Jury , C Ciurtin

iScience. 2021 Oct 11;24(11):103257. PMID: 34761181, doi: 10.1016/j.isci.2021.103257.Brief Summary: The authors explored the relationship between sex hormones and lipids in pre-pubertal children, young post-pubertal cisgender men and women, and transgender adolescents on gender affirming hormone (GAH) tre...

ey0019.8-6 | Important for Clinical Practice | ESPEYB19

8.6. Characteristics of growth in children with classic Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency during adrenarche and beyond

T Troger , G Sommer , M Lang-Muritano , D Konrad , B Kuhlmann , U Zumsteg , CE Fluck

J Clin Endocrinol Metab. 2022; 107(2): e487-e499. PMID: 34599587https://pubmed.ncbi.nlm.nih.gov/34599587/Brief Summary: This study describes the growth of adequately treated children with classical Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency during adrenarche and beyond. Patients with and without significant bone age advancement, and thus differing height prediction...

ey0019.9-6 | Fertility issues and reproductive outcomes in childhood cancer survivors | ESPEYB19

9.6. Serum anti-mullerian hormone levels and risk of premature ovarian insufficiency in female childhood cancer survivors: systematic review and network meta-analysis

Torella M. , Riemma G. , De Franciscis P. , L Verde M. , Colacurci N.

gaetano.riemma@unicampania.it Cancers 2021, 13, 6331. PMID: 34944951.Brief Summary: This systematic review and meta-analysis examined the value of anti-Müllerian hormone (AMH) to predict premature ovarian failure (POI) in childhood cancer survivors (CCSs), compared to the general population.The potential toxic effect of alkylating agents and body irradiation on female...

ey0019.10-1 | Reviews | ESPEYB19

10.1. A century past the discovery of insulin: global progress and challenges for type 1 diabetes among children and adolescents in low-income and middle-income countries

ZA Bhutta , RA Salam , A Gomber , L Lewis-Watts , T Narang , JC Mbanya , G Alleyne

Lancet. 2021 Nov 13;398(10313):1837-1850. https://pubmed.ncbi.nlm.nih.gov/34774146/Brief Summary: This review summarizes key aspects of type 1 diabetes (T1D), such as epidemiology, pathogenesis, risk factors and management. While reviewing and discussing these topics, the central theme of the review is the burden of T1D and the challenges encountered in its daily management in low- and midd...

ey0019.13-7 | Diabetes | ESPEYB19

13.7. Mortality amongst children and adolescents with type 1 diabetes in sub-Saharan Africa: the case study of the changing diabetes in children program in Cameroon

JC Katte , G Lemdjo , MY Dehayem , AG Jones , TJ McDonald , E Sobngwi , JC Mbanya

jcmbanya@yahoo.co.uk Pediatr Diabetes 2022; 23: 33–37. doi: 10.1111/pedi.13294Brief Summary: Despite provision of free insulin by the Changing Diabetes in Children (CDiC) program, mortality remains high in children and adolescents with type 1 diabetes (T1D) in Cameroon, in particular in rural settings and in families with no formal education.<p cl...

ey0017.5-3 | Advances in Clinical Practice | ESPEYB17

5.3. Skeletal changes following hematopoietic stem cell transplantation in osteopetrosis

G Shapiro , J Fishleder , P Stepensky , N Simanovsky , V Goldman , R Lamdan

To read the full abstract: J Bone Miner Res. 2020 Apr 24. doi: https://pubmed.ncbi.nlm.nih.gov/32329913/In brief: Hematopoietic stem cell transplantation remains the only curative treatment in children with severe osteopetrosis. According to this study, increased serum calcium and phosphate serve as good markers of successful engraftment, which leads to significant but incomplete n...

ey0017.6-5 | Differences/Disorders of Sex Development: Basic Research | ESPEYB17

6.5. Undifferentiated spermatogonia regulate Cyp26b1 expression through NOTCH signaling and drive germ cell differentiation

PA Parekh , TX Garcia , R Waheeb , V Jain , P Gandhi , ML Meistrich , G Shetty , MC Hofmann

To read the full abstract: FASEB J. 2019, Jul; 33: 8423–35. doi: https://www.ncbi.nlm.nih.gov/pubmed/30991836Retinoic acid (RA) is essential for the regulation of many developmental events including germ cell differentiation. In the developing testis, tight spatiotemporal control of RA levels is maintained by the enzyme CYP26B1, which inactivates RA. CYP26B1 expres...