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Cushing syndrome management

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https://www.readbyqxmd.com/read/29846662/diverticular-perforation-a-fatal-complication-to-forestall-in-cushing-s-syndrome
#1
Zahraa Abdul Sater, Smita Jha, Raven McGlotten, Iris Hartley, Mustapha El Lakis, Katherine Araque Triana, Lynnette K Nieman
Context: Patients taking exogenous glucocorticoids are at risk of gastrointestinal (GI) complications including peptic ulcer disease with perforation, and gastric bleeding. However, little is known about the GI co-morbidity in patients with endogenous hypercortisolemia. Case descriptions: We describe six patients with endogenous Cushing's Syndrome (CS) who developed sudden perforation of colonic diverticula requiring urgent exploratory laparotomy. Most of these patients shared the following features of Cushing's syndrome: significant skin thinning, severe hypercortisolemia (24-hour urine free cortisol ≥10X the upper limit of normal), ectopic secretion of ACTH, and severe hypokalemia...
May 28, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29791181/-cyclic-cushings-syndrome-a-case-study-and-overview
#2
Jana Kollerová, Ján Malina, Andrej Šteňo, Pavol Povinec, Juraj Payer
Cushings syndrome and especially Cushing´s disease represent diagnostically and therapeutically complicated medical situations. In some patients, cyclic changes in cortisol production additionally hamper the diagnosis in terms of source identification and management of hormone overproduction. It may not be clear, whether the patient is cured or not even years after the treatment. It is a rare disorder variant, but we assume that it is underdiagnosed and it´s incidence is actually higher. The article deals with a complicated course of diagnosis and treatment in a patient with cyclic Cushings syndrome...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29760304/metyrapone-responsive-ectopic-acth-secreting-pheochromocytoma-with-a-vicious-cycle-via-a-glucocorticoid-driven-positive-feedback-mechanism
#3
Minako Inoue, Ken Okamura, Chie Kitaoka, Fumio Kinoshita, Ryo Namitome, Udai Nakamura, Masaki Shiota, Kenichi Goto, Toshio Ohtsubo, Kiyoshi Matsumura, Yoshinao Oda, Masatoshi Eto, Takanari Kitazono
In ectopic ACTH-secreting pheochromocytoma, combined ACTH-driven hypercortisolemia and hyper-catecholaminemia are serious conditions, which can be fatal if not diagnosed and managed appropriately, especially when glucocorticoid-driven positive feedback is suggested with a high ACTH/cortisol ratio. A 46-year-old man presented with headache, rapid weight loss, hyperhidrosis, severe hypertension and hyperglycemia without typical Cushingoid appearance. Endocrinological examinations demonstrated elevated plasma and urine catecholamines, serum cortisol and plasma ACTH...
May 15, 2018: Endocrine Journal
https://www.readbyqxmd.com/read/29754643/pregnancy-in-patients-with-cushing-s-syndrome
#4
REVIEW
Marcio Carlos Machado, Maria Candida Barisson Vilares Fragoso, Marcello Delano Bronstein
Progress in diagnosis and treatment of endocrine diseases has made pregnancy possible for women with endocrinopathies, including Cushing's syndrome (CS). The risk of maternal-fetal complications in patients who are not biochemically controlled, however, is substantial. Therefore, the surgical and/or medical control of hypercortisolism is mandatory prior to conceiving. A diagnosis of de novo CS during gestation is difficult due to changes in the hypothalamic-pituitary-adrenal axis during pregnancy, which may lead to some clinical features suggestive of CS along with abnormal laboratory tests...
June 2018: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/29754639/adrenal-surgery-for-cushing-s-syndrome-an-update
#5
REVIEW
Guido Di Dalmazi, Martin Reincke
Recent advances in the molecular pathogenesis and the natural history of Cushing's syndrome have improved the understanding of the management of this disease. The long-term efficacy of several cortisol-lowering medical treatments is currently under evaluation. However, adrenalectomy is a safe option for the treatment of patients affected by Cushing's syndrome. Unilateral adrenalectomy is the gold standard for treatment of adrenocortical adenomas associated with hypercortisolism. Bilateral adrenalectomy has been widely used in the past as definitive treatment of bilateral macronodular hyperplasia and persistent or recurrent Cushing's disease...
June 2018: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/29754632/genetics-of-cushing-s-syndrome
#6
REVIEW
Laura C Hernández-Ramírez, Constantine A Stratakis
The knowledge on the molecular and genetic causes of Cushing's syndrome (CS) has greatly increased in the recent years. Somatic mutations leading to overactive 3',5'-cyclic adenosine monophosphate/protein kinase A and wingless-type MMTV integration site family/beta-catenin pathways are the main molecular mechanisms underlying adrenocortical tumorigenesis. Corticotropinomas are characterized by resistance to glucocorticoid negative feedback, impaired cell cycle control and overexpression of pathways sustaining ACTH secretion...
June 2018: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/29749880/adrenal-insufficiency-due-to-ritonavir-triamcinolone-drug-drug-interaction-without-preceding-cushing-s-syndrome
#7
S Noe, H Jaeger, S Heldwein
We present the case of a 58-year-old HIV-infected patient with adrenal insufficiency after local injection of triamcinolone, most likely due to drug-drug interaction with his ritonavir-boosted antiretroviral therapy (ART). This is the first case of adrenal insufficiency occurring without prior symptoms of Cushing's syndrome in a patient on a booster-containing ART. We want to draw attention to this seemingly rare, but potentially life-threatening medical condition that can occur even in short-term use of glucocorticoids in low doses in patients on booster-containing ART and summarize some considerations for management...
January 1, 2018: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/29685132/acth-independent-cushing-s-syndrome-with-bilateral-cortisol-secreting-adrenal-adenomas-a-case-report-and-review-of-literatures
#8
Jia Wei, Sheyu Li, Qilin Liu, Yuchun Zhu, Nianwei Wu, Ying Tang, Qianrui Li, Kaiyun Ren, Qianying Zhang, Yerong Yu, Zhenmei An, Jing Chen, Jianwei Li
BACKGROUND: Adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome (CS) with bilateral cortisol-secreting adenomas has been rarely reported in the literatures. Precise recognition and management of this disorder constitute a challenge to clinicians due to the difficulty of exact location of the functional lesions. CASE PRESENTATION: We herein report a new case of a Chinese female patient with a complaint of exertional dyspnea for over 10 years. ACTH-independent CS was diagnosed based on undetectable ACTH and unsuppressed cortisol levels by dexamethasone...
April 23, 2018: BMC Endocrine Disorders
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...
May 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/29621813/cushing-s-syndrome-a-historic-review-of-the-treatment-strategies-and-corresponding-outcomes-in-a-single-tertiary-center-over-the-past-half-century
#11
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
#12
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/29554355/bilateral-adrenalectomy-in-congenital-adrenal-hyperplasia-a-systematic-review-and-meta-analysis
#13
Diana MacKay, Anna Nordenström, Henrik Falhammar
Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal (HPA) axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing's syndrome being a frequent complication of adequate suppression. Bilateral adrenalectomy, with subsequent replacement of glucocorticoids and mineralocorticoids at physiological doses, has been proposed as an alternative therapeutic strategy. Objective: To review the outcomes following bilateral adrenalectomy for CAH...
March 15, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29535866/case-series-of-paraneoplastic-cushing-syndrome-in-small-cell-lung-cancer
#14
Carine Ghassan Richa, Khadija Jamal Saad, Georges Habib Halabi, Elie Mekhael Gharios, Fadi Louis Nasr, Marie Tanios Merheb
The objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29523633/management-of-endocrine-disease-management-of-cushing-s-syndrome-during-pregnancy-solved-and-unsolved-questions
#15
REVIEW
Thierry Brue, Vincent Amodru, Frederic Castinetti
With fewer than 200 reported cases, Cushing's syndrome (CS) in pregnancy remains a diagnostic and therapeutic challenge. In normal pregnancies, misleading signs may be observed such as striae or hypokalemia, while plasma cortisol and urinary free cortisol may rise up to 2- to 3-fold. While the dexamethasone suppression test is difficult to use, reference values for salivary cortisol appear valid. Apart from gestational hypertension, differential diagnosis includes pheochromocytoma and primary aldosteronism...
June 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29442480/cushing-s-disease-major-difficulties-in-diagnosis-and-management-during-pregnancy
#16
Francisca Caimari, Rosa Corcoy, Susan M Webb
Pregnancy in women with a diagnosis of Cushing' syndrome (CS) is an extremely rare event and its diagnosis and treatment are a real medical challenge. During pregnancy, the hypothalamus-pituitary-adrenal axis undergoes major changes leading to a significant increase in plasma cortisol levels throughout gestation. The difficulties in diagnosis are related to the resemblance of symptoms of CS and those of pregnancy, and to the complex interpretation of the screening tests. Moreover, the diagnostic work up in the postnatal period may be difficult in the first weeks postpartum...
February 13, 2018: Minerva Endocrinologica
https://www.readbyqxmd.com/read/29406000/neonatal-cushing-syndrome-a-rare-but-potentially-devastating-disease
#17
REVIEW
Christina Tatsi, Constantine A Stratakis
Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome...
March 2018: Clinics in Perinatology
https://www.readbyqxmd.com/read/29340776/ectopic-cushing-s-syndrome-secondary-to-olfactory-neuroblastoma
#18
Kenny Yu, Federico Roncaroli, Tara Kearney, David Ewins, Deepa Beeharry, Thomas Naylor, David Ray, Rajiv Bhalla, Kanna Gnanalingham
We present the case of a patient with Cushing's syndrome secondary to ectopic ACTH secretion. A MR of the head showed a left-sided nasal mass extending down from the cribriform plate. The patient underwent endoscopic resection with nearly complete removal of the mass. Histological examination showed an ACTH-secreting olfactory neuroblastoma (ONB). The patient's cortisol levels returned to normal range after surgery and have remained normal for over a year. ONB is a rare cause for ectopic ACTH secretion. This case highlights the diagnostic and management difficulties in patients with ectopic ACTH secretion, and provides a brief review of ONB...
May 2018: Acta Neurochirurgica
https://www.readbyqxmd.com/read/29202350/case-report-ectopic-cushing-s-syndrome-in-a-young-male-with-hidden-lung-carcinoid-tumor
#19
Ghanem Aljassem, Hazem Aljasem
INTRODUCTION: Ectopic Cushing syndrome is a form of Cushing's in which a tumor outside the pituitary gland produces adrenocorticotropic hormone (ACTH). Small cell carcinoma and carcinoid of the lung comprises half of its cases. The main purpose of this study is to present a case of ectopic Cushing syndrome caused by a hidden lung carcinoid and how to manage it. PRESENTATION OF CASE: Here we present a case of a 26year old young male complains of increased weight and appetite, proximal muscle weakness, easy bruising and appearing of purple striae on his abdomen, with a final diagnosis of ACTH secreting lung carcinoid...
2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29158903/severe-cushing-s-syndrome-and-bilateral-pulmonary-nodules-beyond-ectopic-acth
#20
Carlos Tavares Bello, Emma van der Poest Clement, Richard Feelders
Cushing's syndrome is a rare disease that results from prolonged exposure to supraphysiological levels of glucocorticoids. Severe and rapidly progressive cases are often, but not exclusively, attributable to ectopic ACTH secretion. Extreme hypercortisolism usually has florid metabolic consequences and is associated with an increased infectious and thrombotic risk. The authors report on a case of a 51-year-old male that presented with severe Cushing's syndrome secondary to an ACTH-secreting pituitary macroadenoma, whose diagnostic workup was affected by concurrent subclinical multifocal pulmonary infectious nodules...
2017: Endocrinology, Diabetes & Metabolism Case Reports
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