ESPE Yearbook of Paediatric Endocrinology

J Clin Endocrinol Metab. 2020; 105(9): 3103–3107.https://pubmed.ncbi.nlm.nih.gov/32629476/In this narrative review, the authors discuss the potential role of novel regenerative therapies for the treatment of adrenal insufficiency, including gene therapy and cell replacement strategies. More specifically, the authors discuss the heterogeneity in adrenal function in patients with Addison...

To read the full abstract: Proc Natl Acad Sci USA. 2018; 115(51): 12997–13002.Humans are constantly challenged by multiple stressors, to which the body’s response and adaptation are essential. The adrenal gland plays a major role in the response to both physiological challenges and stress. Maintenance of the adrenal is partly accomplished by proliferation and differentiat...

To read the full abstract: Cell Rep. 2018; 22(5): 1236-1249Primary or secondary adrenal insufficiency (AI) results from adrenal failure or impairment of the hypothalamic-pituitary axis, respectively. The most frequent cause of primary AI is autosomal recessive congenital adrenal hyperplasia (CAH). Patients with AI need life-long treatment with exogenous steroids, which can be challenging, ...

To read the full abstract: PLoS One. 2018;13:e0191360Up to now most studies to prevent autoimmune T1DM have focused on directly suppressing the autoimmune response rather than to better understanding the intrinsic requirements for beta cell survival. In this trial intracellular heparan sulfate was investigated as an essential requirement for the survival of beta cells and a marker for beta...

Brief Summary: This study demonstrates that FADS2 is a major regulator of steroidogenesis in the adrenal gland due to its key role in shaping the lipidomic landscape of adrenocortical cells.Comment: The corticosteroids aldosterone and cortisol are produced in the zona glomerulosa and zona fasciculata of the adrenal cortex, respectively (1, 2). Excess production of aldosterone in primary hyperaldosteronism results in resistant hypertension and cardiovascu...

Brief Summary: This study explored the relationship between Somatostatin Receptor 2 (SSTR2) immunoreactivity and succinate dehydrogenase complex iron sulfur subunit B (SDHB) immunoreactivity, mutational status, and clinical behavior of paragangliomas (PPGLs), and evaluated SSTR-based therapies in metastatic PPGLs. The findings highlight SSTR2 expression as a novel biomarker for metastatic behavior in PCC, PGL and SDHB/ SDHx mutations. They also suggest that SSTR-based therapie...