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https://www.readbyqxmd.com/read/29678284/aberrant-g-protein-coupled-hormone-receptor-in-adrenal-diseases
#1
REVIEW
Matthieu St-Jean, Nada El Ghorayeb, Isabelle Bourdeau, André Lacroix
The regulation of cortisol or aldosterone production when ACTH of pituitary origin or the renin-angiotensin systems are suppressed in primary adrenal Cushing's syndrome or in primary aldosteronism is exerted by diverse genetic and molecular mechanisms. In addition to recently identified mutations in various genes implicated in the cyclic AMP or ion channel pathways, steroidogenesis is not really autonomous as it is frequently regulated by the aberrant adrenocortical expression of diverse hormone receptors, particularly G-protein coupled hormone receptors (GPCR) which can substitute for the normal function of ACTH or angiotensin-II...
April 2018: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29675278/adrenocorticotropic-hormone-secreting-pheochromocytoma-underlying-glucocorticoid-induced-pheochromocytoma-crisis
#2
Gil A Geva, David J Gross, Haggi Mazeh, Karine Atlan, Iddo Z Ben-Dov, Matan Fischer
Context: Pheochromocytomas are hormone secreting tumors of the medulla of the adrenal glands found in 0.1-0.5% of patients with hypertension. The vast majority of pheochromocytomas secrete catecholamines, but they have been occasionally shown to also secrete interleukins, calcitonin, testosterone, and in rare cases adrenocorticotropic hormone. Pheochromocytoma crisis is a life threatening event in which high levels of catecholamines cause a systemic reaction leading to organ failure. Case Description: A 70-year-old man was admitted with acute myocardial ischemia following glucocorticoid administration as part of an endocrine workup for an adrenal mass...
2018: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/29673697/adrenocortical-development-lessons-from-mouse-models
#3
Typhanie Dumontet, Isabelle Sahut-Barnola, Amandine Septier, Nathanaëlle Montanier, Ingrid Plotton, Florence Roucher-Boulez, Véronique Ducros, Anne-Marie Lefrançois-Martinez, Jean-Christophe Pointud, Mohamad Zubair, Ken-Ichirou Morohashi, David T Breault, Pierre Val, Antoine Martinez
The adrenocortical gland undergoes structural and functional remodelling in the fetal and postnatal periods. After birth, the fetal zone of the gland undergoes rapid involution in favor of the definitive cortex, which reaches maturity with the emergence of the zona reticularis(zR) at the adrenarche. The mechanisms underlying the adrenarche, the process leading to pre-puberty elevation of plasma androgens in higher primates, remain unknown, largely due to lack of any experimental model. By following up fetal and definitive cortex cell lines in mice, we showed that activation of protein kinase A (PKA) signaling mainly impacts the adult cortex by stimulating centripetal regeneration, with differentiation and then conversion of the zona fasciculata into a functional zR...
April 16, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29672894/unexpected-adverse-effect-of-metyrapone-a-case-report
#4
A Rouland, A Nguyen, C Fourmont, M Lapray, B Vergès, J M Petit, B Bouillet
Hypercortisolism, or Cushing's syndrome, is a disease which can be treated surgically or with medications such as mitotane, ketoconazole or metyrapone. However, the use of metyrapone is not common, and the adverse effects are not well known. A metyrapone overdose is usually associated with gastro-intestinal symptoms and signs of adrenal failure. To our knowledge, no other side effects have been reported in connection with an ingestion of high doses of metyrapone. This article is protected by copyright. All rights reserved...
April 19, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29669941/activating-prkacb-somatic-mutation-in-cortisol-producing-adenomas
#5
Stéphanie Espiard, Matthias J Knape, Kerstin Bathon, Guillaume Assié, Marthe Rizk-Rabin, Simon Faillot, Windy Luscap-Rondof, Daniel Abid, Laurence Guignat, Davide Calebiro, Friedrich W Herberg, Constantine A Stratakis, Jérôme Bertherat
Mutations in the gene encoding the protein kinase A (PKA) catalytic subunit α have been found to be responsible for cortisol-producing adenomas (CPAs). In this study, we identified by whole-exome sequencing the somatic mutation p.S54L in the PRKACB gene, encoding the catalytic subunit β (Cβ) of PKA, in a CPA from a patient with severe Cushing syndrome. Bioluminescence resonance energy transfer and surface plasmon resonance assays revealed that the mutation hampers formation of type I holoenzymes and that these holoenzymes were highly sensitive to cAMP...
April 19, 2018: JCI Insight
https://www.readbyqxmd.com/read/29667493/non-adenomatous-pituitary-tumours-mimicking-functioning-pituitary-adenomas
#6
Zize Feng, Zhigang Mao, Zongming Wang, Bing Liao, Yonghong Zhu, Haijun Wang
OBJECTIVE: Pituicytomas and granular cell tumours (GCTs) of the neurohypophysis are considered non-adenomatous neoplasms in the sellar region. The association between hormone hypersecretion and the tumours is seldom discussed and unclear. Therefore, we attempt to investigate this association based on our experience and a review of the literature. METHODS: We report three patients who presented with Cushing's syndrome- or acromegaly-like symptoms at our institution...
April 18, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29661784/mechanisms-in-endocrinology-a-sense-of-time-of-the-glucocorticoid-circadian-clock-from-the-ontogeny-to-the-diagnosis-of-cushing-s-syndrome
#7
Ayrton Custodio Moreira, Sonir Roberto R Antonini, Margaret De Castro
The circadian rhythm of glucocorticoids has long been recognised within the last 75 years. Since the beginning, researchers have sought to identify basic mechanisms underlying the origin and emergence of the corticosteroid circadian rhythmicity among mammals. Accordingly, Young, Hall and Rosbash, laureates of the 2017 Nobel Prize in Physiology or Medicine, as well as Takahashi's group among others, have characterised the molecular cogwheels of the circadian system, describing interlocking transcription/translation feedback loops essential for normal circadian rhythms...
April 16, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29661471/do-the-diagnostic-criteria-for-subclinical-hypercortisolism-exist
#8
Antoine Tabarin
"Subclinical hypercortisolism" (SH) refers to a condition associated with a mild chronic increase in cortisol secretion. By definition, patients with SH do not exhibit specific symptoms of overt Cushing's syndrome (such as purple striae, easy bruising, proximal muscle weakness), SH has been preferred to "subclinical Cushing's syndrome", a semantic ambiguity since Cushing's syndrome is, by definition, a set of symptoms; and to the term "preclinical Cushing syndrome" because the progression toward overt clinical hypercortisolism is very rare...
April 13, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29655957/ectopic-cushing-syndrome-report-of-9-cases
#9
Marta Araujo Castro, Nuria Palacios García, Javier Aller Pardo, Cristina Izquierdo Alvarez, Laura Armengod Grao, Javier Estrada García
INTRODUCTION: Ectopic Cushing's syndrome (ECS) is a rare condition caused by ACTH secretion by extrapituitary tumors. Its low frequency makes it difficult to acquire experience in its management. The aim of this study was to describe patients with ECS seen at the endocrinology department of a tertiary hospital over 15 years. METHODS: This was a retrospective study of the clinical, biochemical and radiographic data, treatment, and course of patients with ECS seen from 2000 to 2015...
April 11, 2018: Endocrinología, Diabetes y Nutrición
https://www.readbyqxmd.com/read/29652235/harvey-cushing-s-management-of-neurogenic-thoracic-outlet-syndrome
#10
Courtney Pendleton, Allan J Belzberg, Robert J Spinner, Alfredo Quinones-Hinojosa
Harvey Cushing is widely regarded as one of the forefathers of neurosurgery, and is primarily associated with his work on intracranial pathology. However, he had a clinical and academic interest in peripheral nerve surgery. Through the courtesy of the Alan Mason Chesney Medical Archives, the surgical records of the Johns Hopkins Hospital from 1896 to 1912 were reviewed. The records of a single patient undergoing brachial plexus exploration and cervical rib resection were selected for detailed review. The operative report and accompanying illustrations demonstrate Cushing's interest in adding approaches to the pathology of the brachial plexus to his operative armamentarium...
April 13, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29651813/contralateral-adrenal-width-predicts-the-duration-of-prolonged-post-surgical-steroid-replacement-for-subclinical-cushing-syndrome
#11
Masahiro Sugiura, Yusuke Imamura, Koji Kawamura, Satoshi Yamamoto, Tomokazu Sazuka, Kazuyoshi Nakamura, Shinichi Sakamoto, Hidekazu Nagano, Hisashi Koide, Tomoaki Tanaka, Takashi Imamoto, Akira Komiya, Tomohiko Ichikawa
OBJECTIVES: To identify pre-treatment factors affecting the duration of post-surgical steroid replacement in patients undergoing adrenalectomy for subclinical Cushing syndrome. METHODS: The present retrospective analysis included 64 patients who underwent unilateral laparoscopic adrenalectomy for subclinical Cushing syndrome. Adrenal tumor and contralateral adrenal sizes together with various clinical factors were studied in association with the duration of post-surgical steroid replacement...
April 12, 2018: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/29650225/an-update-on-cushing-syndrome-in-pediatrics
#12
Constantine A Stratakis
Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i.e., experienced, state-of-the-art) surgical treatment; and unavailability of well-tolerated and effective medications to control hypercortisolemia...
April 9, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29648902/diagnostic-evaluation-in-steroid-induced-myopathy-case-report-suggesting-clinical-utility-of-quantitative-muscle-ultrasonography
#13
Marco Alessandro Minetto, Cristina Caresio, Valentina D'Angelo, Fabio Lanfranco, Lucia Ghizzoni, Silvestro Roatta, Emanuela Arvat, Santosh Kesari
PURPOSE: We present a patient with adrenal Cushing's syndrome causing steroid myopathy. The purpose of the case report is to illustrate the clinical usefulness of quantitative muscle ultrasonography for the assessment of glucocorticoid-induced changes in muscle mass (MM) and structure. METHODS: Assessments of physical performance, muscle strength, MM (i.e., total body skeletal MM, appendicular skeletal MM, and thickness of lower limb muscles), and muscle structure (i...
April 12, 2018: Endocrine Research
https://www.readbyqxmd.com/read/29625700/quality-of-life-in-cushing-s-disease-a-long-term-issue
#14
Susan M Webb, Alicia Santos, Eugenia Resmini, Maria-Antonia Martínez-Momblán, Luciana Martel, Elena Valassi
The purpose of this review is to describe how quality of life (QoL) is impaired in patients with hypercortisolism due to Cushing's syndrome of any aetiology, including pituitary-dependent Cushing's disease. It is worse in active disease, but improvement after successful therapy is often incomplete, due to persistent physical and psychological co-morbidities, even years after endocrine "cure". Physical symptoms like extreme fatigability, central obesity with limb atrophy, hypertension, fractures, and different skin abnormalities severely impair the affected patients' everyday life...
April 3, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29621813/cushing-s-syndrome-a-historic-review-of-the-treatment-strategies-and-corresponding-outcomes-in-a-single-tertiary-center-over-the-past-half-century
#15
Anelia Nankova, Maria Yaneva, Atanaska Elenkova, Dimitar Tcharaktchiev, Marin Marinov, Asen Hadzhiyanev, Tanyo Sechanov, Georgi Gantchev, Georgi Todorov, Georgi Kirilov, Krasimir Kalinov, Maria Andreeva, Sabina Zacharieva
Cushing's syndrome (CS) is associated with serious comorbidities and an increased mortality rate that could be reduced only if strict biochemical control is achieved. The aim of this study was to show the 50-year experience of a single tertiary center in the management of CS patients - the different treatment modalities used over the years and the corresponding outcomes. It was a retrospective study of a large cohort of patients from the Bulgarian CS database: 613 patients (374 with ACTH-dependent and 239 with ACTH-independent CS)...
April 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29605372/emberger-syndrome-a-rare-association-with-hearing-loss
#16
Faisal Zawawi, Meirav Sokolov, Thomas Mawby, Karen A Gordon, Blake C Papsin, Sharon L Cushing
Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES...
May 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29594118/the-many-faces-of-primary-aldosteronism-and-cushing-syndrome-a-reflection-of-adrenocortical-tumor-heterogeneity
#17
REVIEW
Ozgur Mete, Kai Duan
Adrenal cortical tumors constitute a heterogeneous group of neoplasms with distinct clinical, morphological, and molecular features. Recent discoveries of specific genotype-phenotype correlations in adrenal cortical adenomas have transformed our understanding of their respective endocrine syndromes. Indeed, a proportion of patients with primary aldosteronism are now known to harbor adrenal cortical adenomas with heterogeneous molecular alterations ( KCNJ5, ATP1A1, ATP2B3 , and CACNA1D ) involving the calcium/calmodulin kinase signaling pathway...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29589383/bone-health-in-adrenal-disorders
#18
REVIEW
Beom Jun Kim, Seung Hun Lee, Jung Min Koh
Secondary osteoporosis resulting from specific clinical disorders may be potentially reversible, and thus continuous efforts to find and adequately treat the secondary causes of skeletal fragility are critical to ameliorate fracture risk and to avoid unnecessary treatment with anti-osteoporotic drugs. Among the hyperfunctional adrenal masses, Cushing's syndrome, pheochromocytoma, and primary aldosteronism are receiving particularly great attention due to their high morbidity and mortality mainly by increasing cardiovascular risk...
March 2018: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29587720/spontaneous-adrenocorticotropic-hormone-acth-normalisation-due-to-tumour-regression-induced-by-metyrapone-in-a-patient-with-ectopic-acth-syndrome-case-report-and-literature-review
#19
Hideyuki Iwayama, Sho Hirase, Yuka Nomura, Tatsuo Ito, Hiroyuki Morita, Kazuo Otake, Akihisa Okumura, Junko Takagi
BACKGROUND: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is caused by tumours releasing ACTH. Ectopic ACTH-producing tumour regression is rarely induced using steroidogenesis inhibitors. We presented a case of EAS in which ACTH production by a lung tumour was reduced by metyrapone (MTP) and also reviewed previous cases of ectopic ACTH production suppressed via steroidogenesis inhibition. CASE PRESENTATION: A 71-year-old female with general fatigue, central obesity and impaired glucose tolerance was diagnosed with Cushing's syndrome due to elevated ACTH (192...
March 27, 2018: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/29587644/a-novel-germline-armc5-mutation-in-a-patient-with-bilateral-macronodular-adrenal-hyperplasia-a-case-report
#20
Qiuli Liu, Dali Tong, Jing Xu, Xingxia Yang, Yuting Yi, Dianzheng Zhang, Luofu Wang, Jun Zhang, Yao Zhang, Yaoming Li, Lianpeng Chang, Rongrong Chen, Yanfang Guan, Xin Yi, Jun Jiang
BACKGROUND: Bilateral macronodular adrenal hyperplasia (BMAH) is a rare cause of Cushing's syndrome (CS). BMAH is predominantly believed to be caused by two mutations, a germline and somatic one, respectively, as described in the two-hit hypothesis. In many familial cases of BMAH, mutations in armadillo repeat containing 5 (ARMC5), a putative tumor suppressor gene, are thought to induce the disorder. The objective of this study was to report a case in which the patient presented with BMAH induced by a novel heterozygous germline ARMC5 mutation (c...
March 27, 2018: BMC Medical Genetics
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