|
Current Opinion in Endocrinology, Diabetes and Obesity
Journal Prestige (SJR): 1.467  Citation Impact (citeScore): 3 Number of Followers: 48  Hybrid journal (It can contain Open Access articles)ISSN (Print) 1752-296X - ISSN (Online) 1752-2978 Published by LWW Wolters Kluwer  [297 journals]
|
- Editorial introductions
-
Free pre-print version: Loading...
Abstract:
No abstract available
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Pathophysiology of bilateral hyperaldosteronism
-
Free pre-print version: Loading...
Authors: Nanba; Kazutaka; Rainey, William E.
Abstract:
Purpose of review Renin-independent aldosterone production from one or both affected adrenal(s), a condition known as primary aldosteronism (PA), is a common cause of secondary hypertension. In this review, we aimed to summarize recent findings regarding pathophysiology of bilateral forms of PA, including sporadic bilateral hyperaldosteronism (BHA) and rare familial hyperaldosteronism.Recent findings The presence of subcapsular aldosterone synthase (CYP11B2)-expressing aldosterone-producing micronodules, also called aldosterone-producing cell clusters, appears to be a common histologic feature of adrenals with sporadic BHA. Aldosterone-producing micronodules frequently harbor aldosterone-driver somatic mutations. Other potential factors leading to sporadic BHA include rare disease-predisposing germline variants, circulating angiotensin II type 1 receptor autoantibodies, and paracrine activation of aldosterone production by adrenal mast cells. The application of whole exome sequencing has also identified new genes that cause inherited familial forms of PA.Summary Research over the past 10 years has significantly improved our understanding of the molecular pathogenesis of bilateral PA. Based on the improved understanding of BHA, future studies should have the ability to develop more personalized treatment options and advanced diagnostic tools for patients with PA.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Current approach of primary bilateral adrenal hyperplasia
-
Free pre-print version: Loading...
Authors: Delivanis; Danae A.; Vassiliadi, Dimitra A.; Tsagarakis, Stylianos
Abstract:
Purpose of review To discuss the most recent findings on the pathophysiology, the genetic and molecular causes of primary bilateral adrenal hyperplasia (PBAH). The diagnostic approach of patients with PBAH will also be presented in detail with an emphasis on the emerging diagnostic tools and finally, the treatment of PBAH will be discussed with an emphasis on the newest surgical and medical treatment approaches.Recent findings PBAH is a highly heterogeneous condition mostly detected incidentally on abdominal imaging. Based on the size of the nodules, PBAH is subdivided into primary bilateral macronodular adrenal hyperplasia (PBMAH) and micronodular adrenal hyperplasia. A substantial proportion of patients with PBMAH harbor a germline mutation of the armadillo repeat containing 5 tumor suppression gene and therefore genetic testing is strongly recommended. Measurements of plasma or urinary multisteroid profiles show promising results in that PBMAH has a distinctive plasma steroid fingerprint that can help in diagnosis and subtyping of PBMAH. Finally, although surgery is the mainstay of treatment of patients with PBAH, medical therapy is increasingly emerging as an alternative option.Summary PBAH is a poorly studied and therefore a challenging disease to diagnose and treat. Hopefully with these newest diagnostic and therapeutic tools, a more comprehensive approach will be adopted.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Optimal and novel imaging of the adrenal glands
-
Free pre-print version: Loading...
Authors: Navin; Patrick J.; Moynagh, Michael R.
Abstract:
Purpose of review Adrenal imaging forms an important role in the workup of adrenal masses. The purpose of this review is to briefly review the traditional role of imaging in adrenal diseases and highlight the most recent research and new applications aimed to improve diagnostic accuracy.Recent findings The current review will focus on new applications of computed tomography (CT), MRI and PET/CT imaging, addressing the implications of artificial intelligence and radiomics in progressing diagnostic accuracy.Summary The new applications of adrenal imaging are improving diagnostic accuracy and expanding the role of imaging, particularly with novel PET radiotracers and the use of artificial intelligence.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- The uncommon forms of congenital adrenal hyperplasia
-
Free pre-print version: Loading...
Authors: Auchus; Richard J.
Abstract:
Purpose of review Clinicians recognize 21-hydroxylase deficiency as the most common form of congenital adrenal hyperplasia (CAH), and many papers have been published on this condition. In contrast, much less awareness has been addressed to the other, rare forms of CAH.Recent findings The second most common form of CAH varies with country and ethnic background. In Brazil, 17-hydroxylase/17,20-lyase deficiency is the second most common, whereas 11-hydroxylase deficiency is most common in the Middle East. In Japan and Korea, both congenital lipoid adrenal hyperplasia and P450-oxidoreductase deficiency are more common than in the rest of the world. Finally, 3β-hydroxysteroid dehydrogenase/isomerase deficiency is rare worldwide, but pockets of affected populations, such as the Amish in Lancaster County, Pennsylvania are found. The treatment of each form varies by both the nature of steroids produced in excess above the enzymatic block and the deficiencies of steroids other than cortisol past these blocks.Summary This article summarizes the pathophysiology, diagnosis, and management of rare forms of CAH.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Emerging treatment for congenital adrenal hyperplasia
-
Free pre-print version: Loading...
Authors: White; Perrin C.
Abstract:
Purpose of review Although the basic treatment of congenital adrenal hyperplasia (CAH) is well established, there are active clinical research projects to more closely mimic the normal diurnal rhythm of cortisol secretion and to reduce total glucocorticoid doses to minimize adverse metabolic effects.Recent findings We review clinical studies on CAH treatment published in the last 18 months or currently underway according to ClinicalTrials.gov listings. These can be grouped into several broad themes: alternative dosing forms of hydrocortisone with altered pharmacokinetics or easier dose titration; corticotropin-releasing hormone receptor antagonists that reduce corticotropin (ACTH) secretion and thereby reduce adrenal androgen secretion; androgen biosynthesis inhibitors; a first clinical trial of a gene therapy vector.Summary Alternative dosing forms of hydrocortisone are, or will shortly be, marketed, but cost may be a barrier to utilization, at least in the US market. Trials of corticotropin releasing hormone receptor antagonists and androgen biosynthesis inhibitors are currently underway. The author believes that trials of gene therapy for CAH are premature.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Adrenal insufficiency and pregnancy
-
Free pre-print version: Loading...
Authors: Alexandraki; Krystallenia I.; Kaltsas, Gregory A.
Abstract:
Introduction Adrenal insufficiency is a disorder characterized by the failure of adrenocortical function because of distorted function of hypothalamic–pituitary– adrenal (HPA) axis. Pregnancy is a state of a physiological glucocorticoid excess as the HPA axis is functioning at a higher level.Purpose of review The aim of the present review was to shed light on current evidence of adrenal insufficiency management during pregnancy, along with maternal and neonatal outcomes.Recent findings A recent multicenter study under the auspices of the European Network for the Study of Adrenal Tumours (ENSAT) presented real-life data of pregnant women with adrenal insufficiency documenting an increased use of hydrocortisone (or mineralocorticoids when needed according to the level of disorder) replacement treatment, increased rates of caesarean section, preterm delivery and adrenal crises along with peripartum and postpartum complications but no maternal or neonatal fatality. These data were in agreement with those obtained from previously published studies.Conclusion The limited published evidence is in line with the present guidelines as real-life data did not document any increased fatality among pregnant women or newborns. Prospective data with prolonged follow-up are needed to shed more light on appropriate dose adjustments to avoid the risks of under-replacement or over-replacement of glucocorticoid and/or mineralocorticoid drugs and their sequelae.Summary A recent multicenter study by ENSAT presented real-life data of pregnant women with adrenal insufficiency documenting an increased use of hydrocortisone as replacement treatment during pregnancy, along with an increased rate of caesarean section and preterm delivery, adrenal crises, peripartum and postpartum complications but no maternal or neonatal fatality. These data are in agreement with those of a previously published study and also confirm the statements made by the recent guidelines. Prospective data are needed aiming to develop precise therapeutic protocols during each trimester of pregnancy according to the different causes of adrenal insufficiency.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- The role for long-term use of dehydroepiandrosterone in adrenal
insufficiency-
Free pre-print version: Loading...
Authors: Bennett; Gillian; Cussen, Leanne; O’Reilly, Michael W.
Abstract:
Purpose of review Dehydroepiandrosterone (DHEA) is an androgen produced by the zona reticularis of the adrenal gland. Patients with adrenal insufficiency will have a deficiency of DHEA. Unlike glucocorticoid and mineralocorticoid replacement, DHEA supplementation is not considered essential for life and is therefore not routinely replaced in adrenal failure. DHEA deficiency is associated with morbidity, including adverse impacts on metabolic function, quality of life and sexuality in multiple studies. The role for replacement, however, remains unclear.Recent findings The benefits of DHEA supplementation have been definitively demonstrated in a number of historical studies of patients with primary and secondary adrenal insufficiency. Beneficial impacts on quality of life, body composition, bone health and metabolic markers have been demonstrated. However, published data are inconsistent; controversies persist around the exact role of DHEA replacement and around which patient cohorts are most likely to benefit. There is also a paucity of recent randomized controlled trials in the medical literature to inform on optimal dose and duration of DHEA replacement in adrenal failure.Summary Here, we review the evidence for DHEA supplementation in patients with adrenal insufficiency. We highlight knowledge gaps in the medical literature and areas that should be prioritized for future research endeavours.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
- Supportive management of patients with pheochromocytoma/paraganglioma
undergoing noninvasive treatment-
Free pre-print version: Loading...
Authors: Talvacchio; Sara; Nazari, Matthew A.; Pacak, Karel
Abstract:
Purpose of review Many publications review perioperative management of pheochromocytomas/paragangliomas (PPGLs); however, a large population, including 10–20% of metastatic PPGL patients, have inoperable disease. This has necessitated the development of noninvasive treatments (e.g., radio/chemotherapy), which, in affording disease-modification, have led to an ever-growing population of surviving patients with inoperable PPGL. These patients experience debilitating symptoms arising from discomforts related to the masses themselves (e.g., pain from osseous metastasis) and symptoms from tumoral catecholamine production and release. Unfortunately, management of these conditions is not yet well-defined. Adding further insult-to-injury, these noninvasive treatments can trigger catecholamine release, worsening catecholamine-induced symptoms. Herein, we detail these ailments and their management, especially while patients receive these noninvasive treatments.Recent findings Improved diagnostic evaluations have allowed for earlier detection of PPGL, prolonging survival in patients with inoperable PPGLs. Accordingly, noninvasive treatment strategies have rapidly evolved alongside state-of- the-art theranostics and genetic testing, which inform ongoing management and therapeutic response.Summary While treatments afford improved survival, there must be a corresponding attention to quality-of-life. This is ensured by employing supportive management, which mitigates debilitating symptoms. This is best accomplished with a multidisciplinary approach and familiarity with genetic and biochemical determinants which guide patient education and management.
PubDate: Wed, 01 Jun 2022 00:00:00 GMT-
-
