Treatment of Girls and Boys with McCune-Albright Syndrome with Precocious Puberty - Update 2017

The most common endocrinopathy associated with McCune-Albright Syndrome (MAS) is peripheral precocious puberty (PP) which occurs far more often in girls than in boys. We will discuss the latest advancements in the treatment of precocious puberty in MAS that have been achieved during the past 10 years. However, due to the rarity of the condition and the heterogeneity of the disease, research in this field is limited particularly in regards to treatment in boys. In girls, a period of watchful waiting is recommended prior to initiating therapy due to extreme variability in the clinical course. This article will review in detail current pharmacologic treatment in girls, which typically consists of either inhibiting estrogen production or blocking estrogen action at the level of the end-organ. The two treatments with the most evidence at this time are Tamoxifen (which is an estrogen receptor modulator) and Letrozole (which is a 3rd generation aromatase inhibitor). This article will also review the current treatment strategies in boys which typically include using an androgen receptor blocker and an aromatase inhibitor. Due to the rarity of the condition, large multicenter collaborative studies are needed to further investigate efficacy and safety with the goal of establishing the gold standard for treatment of PP in children with MAS

Bicalutamide as an Androgen Blocker With Secondary Effect of Promoting Feminization in Male-to-Female Transgender Adolescents

PURPOSE: The purpose of the study was to describe the novel use of bicalutamide in transgender youth. METHODS: This is a retrospective review of patients with gender dysphoria followed in the pediatric endocrine clinic at Riley Hospital for Children. RESULTS: Of 104 patients with gender dysphoria, 23 male-to-female adolescents received bicalutamide 50 mg daily as a second-line puberty blocker after insurance company denial of a gonadotropin-releasing hormone analog. Six patients received estrogen concurrently. Of 13 patients treated exclusively with bicalutamide seen in follow-up, 84.6% had breast development within 6 months, the majority being ≥ Tanner stage III. CONCLUSIONS: Bicalutamide may be an alternative to gonadotropin-releasing hormone analogs in transgender male-to-female youth who are also ready to undergo physical transition

Concept for the Keck Next Generation Adaptive Optics system

The Next Generation Adaptive Optics (NGAO) system will represent a considerable advancement for high resolution astronomical imaging and spectroscopy at the W. M. Keck Observatory. The AO system will incorporate multiple laser guidestar tomography to increase the corrected field of view and remove the cone effect inherent to single laser guide star systems. The improvement will permit higher Strehl correction in the near-infrared and diffraction-limited correction down to R band. A high actuator count micro-electromechanical system (MEMS) deformable mirror will provide the on-axis wavefront correction to a number of instrument stations and additional MEMS devices will feed multiple channels of a deployable integral-field spectrograph. In this paper we present the status of the AO system design and describe its various operating modes

Trendy nr 4/2016

W nowym numerze TRENDÓW piszemy o kompetencjach kluczowych – informatycznych, matematycznych, technicznych, umiejętności uczenia się, innowacyjności i przedsiębiorczości. W kwietniu 2017 r. zastanowimy się nad tym, jak kształtować pozostałe – umiejętność porozumiewania się w języku ojczystymi obcym, kompetencje społeczne i obywatelskie, świadomość i ekspresję kulturalną. Kompetencje kluczowe to kompetencje, które wspierają rozwój osobisty, włączanie w życie społeczne, aktywne obywatelstwoi możliwość znalezienia zatrudnienia. Proces kształtowania i rozwijaniaw sobie kompetencji kluczowych trwa przez całe życie i nigdy się niekończy. Autorzy artykułów w tym numerze udowadniają, że kształtowanie kompetencji kluczowych nie jest trudne – to codzienność i stały element pracy w szkołach

Concept for the Keck Next Generation Adaptive Optics system

The Next Generation Adaptive Optics (NGAO) system will represent a considerable advancement for high resolution astronomical imaging and spectroscopy at the W. M. Keck Observatory. The AO system will incorporate multiple laser guidestar tomography to increase the corrected field of view and remove the cone effect inherent to single laser guide star systems. The improvement will permit higher Strehl correction in the near-infrared and diffraction-limited correction down to R band. A high actuator count micro-electromechanical system (MEMS) deformable mirror will provide the on-axis wavefront correction to a number of instrument stations and additional MEMS devices will feed multiple channels of a deployable integral-field spectrograph. In this paper we present the status of the AO system design and describe its various operating modes

Differences in COVID-19 Outcomes Among Patients With Type 1 Diabetes: First vs Later Surges

Background Outcomes of the novel coronavirus SARS-CoV-2 (COVID-19) have improved throughout the pandemic. However, whether outcomes of COVID-19 in the type 1 diabetes (T1D) population improved over time is unknown. Therefore, we aim to investigate differences in COVID-19 outcomes for patients with T1D in the US. Method We analyzed data collected via a registry of patients with T1D and COVID-19 from 56 sites between April 2020 and January 2021. First, we grouped cases into First Surge (04/09/2020 - 07/31/2020, n=188) and Late Surge (08/01/2020 - 01/31/2021, n=410). Then, we compared outcomes between both groups using descriptive statistics and logistic regression models. Results Adverse outcomes were more frequent during the first surge including Diabetic Ketoacidosis (32% versus 15%, p<0.001), severe hypoglycemia (4% versus 1%, p=0.04) and hospitalization (52% versus 22%, p<0.001). The First surge cases were older (28 +/- 18.8 years versus 18.8 +/- 11.1 years, p<0.001), had higher hemoglobin A1c (HbA1c) levels (Median (IQR): 9.3 (4.0) versus 8.4(2.8), <0.001) and use public insurance (n(%): 107 (57) versus 154 (38), p <0.001). There were five times increased odds of hospitalization for adults (OR 5.01 (2.11,12.63) in the first surge compared to the late surge. Conclusion COVID-19 cases among patients with T1D reported during the first surge had a higher proportion of adverse outcomes than those presented in a later surge

Evaluation of hypoglycemia resulting from hyperglycemic clamps

Hyperglycemic clamps are used as a gold standard measurement of beta cell function, allowing for measurement of a biphasic insulin response. Under this technique, the plasma glucose concentration is raised to a target concentration of 200 mg/dL by exogenous infusion of dextrose. The target glucose concentration is sustained by regular titration of the dextrose infusion rate. An adverse event commonly associated with hyperinsulinemic clamps or arginine infusions is post-clamp hypoglycemia (blood glu-cose \u3c60mg/dL). However, hyperglycemic clamp testing of nondiabetic individuals has not traditionally been associated with this outcome. In a recent study of beta cell function in nondiabetic individuals at genetic risk for type 1 diabetes and unrelated controls, we observed hypoglycemia in 11/67 clamps post discontinuation of dextrose infusion. We hypothesized that demographic or clamp related factors may be associated with hypoglycemia in the 60 minutes after discontinuation of dextrose infusion. Three non-diabetic populations were analyzed: islet Ab negative first-degree relatives of T1D pro-bands (n=19), Ab positive relatives of T1D probands (n=5), and healthy controls with no family history of T1D and undetectable Abs (n=15). Demographic factors (study group, age, BMI, sex, HbA1c, fasting glucose) and clamp factors (average glucose infusion rate during the clamp, maximum glucose infusion rate during the clamp, urine glucose, and urine volume) were compared among those who became hypoglycemic and those who did not. Intraindividual variability of post clamp hypoglycemia was also evaluated in those who had two clamp visits (n=27). Ten (25.64%) participants exhibit-ed hypoglycemia (average glucose of 50.97 mg/dL) in at least one clamp visit and 29 (74.36%) were normoglycemic (average glucose of 81.07 mg/dL) during the 60 minutes after discontinuation of dextrose. No differences were present in rates of hypoglycemia between participant groups. The mean HbA1c (5.009%) of participants who developed hypoglycemia post-clamp was lower than the mean HbA1c (5.195%) for participants who were normoglycemic after the clamp (p value=0.0403), however absolute differences were small and significant overlap existed between both groups. We did not detect significant differences between hypoglycemic and normoglycemic participants for any other demographic or clamp factors analyzed. With regards to patients with 2 available clamp visits, of the 8 subjects who developed hypoglycemia, only one experienced it during both visits. Hypoglycemia is not uncommon after hyperglycemic clamp testing. Hyperglycemic clamp testing should include universal post-clamp glucose monitoring, as no clear demographic factors could be used to identify risk of hypoglycemia