A Summary of the Endocrine Society Clinical Practice Guidelines on Congenital Adrenal Hyperplasia due to Steroid 21-Hydroxylase Deficiency

Steroid 21-hydroxylase deficiency accounts for about 95% of cases of congenital adrenal hyperplasia (CAH). Newborns are currently being screened for the classical forms of this disease throughout the United States and in 12 other countries. As such, it seems important to develop the best practice guidelines for treating not only infants and children, but affected adults as well. This report gives a brief overview of the most recent expert opinion and clinical practice guidelines for CAH as formulated by The Endocrine Society Task Force

Institutional convergence and a lingering two-speed Euro area: a response

This contribution responds to the Comment entitled 'Institutional quality convergence in the Euro area countries: a note and further evidence' on our earlier published article in the Journal of Contemporary European Studies, Volume 28, Issue 3. By using the same data on Government Effectiveness, Regulatory Quality, Rule of Law and Control of Corruption analysed in the Comment, we confirm the coexistence of certain processes of institutional convergence and, at the same time, a two-speed Euro area in institutional terms, with an increasing gap between core and periphery countries. These results help to complement and enrich their conclusions

Institutional convergence and a lingering two-speed Euro area: a response

This contribution responds to the Comment entitled 'Institutional quality convergence in the Euro area countries: a note and further evidence' on our earlier published article in the Journal of Contemporary European Studies, Volume 28, Issue 3. By using the same data on Government Effectiveness, Regulatory Quality, Rule of Law and Control of Corruption analysed in the Comment, we confirm the coexistence of certain processes of institutional convergence and, at the same time, a two-speed Euro area in institutional terms, with an increasing gap between core and periphery countries. These results help to complement and enrich their conclusions

Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline

Objective: We developed clinical practice guidelines for congenital adrenal hyperplasia (CAH)

Discovery of small molecule vanin inhibitors: new tools to study metabolism and disease

Contains fulltext : 111323.pdf (publisher's version ) (Closed access)Vanins are enzymes with pantetheinase activity and are presumed to play a role in the recycling of pantothenic acid (vitamin B5) from pantetheine. Pantothenic acid is an essential nutrient required to synthesize coenzyme A, a cofactor involved in many biological processes such as fatty acid synthesis and oxidation of pyruvate to fuel the citric acid cycle. Hydrolysis of pantetheine also liberates cysteamine, a known antioxidant. Vanin-1 is highly expressed in liver and is under transcriptional control of PPAR-alpha and nutritional status, suggesting a role in energy metabolism. The lack of potent and specific inhibitors of vanins has hampered detailed investigation of their function. We hereby report the design, synthesis, and characterization of a novel pantetheine analogue, RR6, that acts as a selective, reversible, and competitive vanin inhibitor at nanomolar concentration. Oral administration of RR6 in rats completely inhibited plasma vanin activity and caused alterations of plasma lipid concentrations upon fasting, thereby illustrating its potential use in chemical biology research