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Adrenal adenomas

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https://www.readbyqxmd.com/read/28433999/circadian-plasma-cortisol-measurements-reflect-severity-of-hypercortisolemia-in-children-with-different-etiologies-of-endogenous-cushing-syndrome
#1
Amit Tirosh, Maya B Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Georgios Z Papadakis, Constantine A Stratakis
BACKGROUND: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet. METHODS: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6)...
April 21, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28423361/next-generation-sequencing-reveals-microrna-markers-of-adrenocortical-tumors-malignancy
#2
Łukasz Koperski, Marta Kotlarek, Michał Świerniak, Monika Kolanowska, Anna Kubiak, Barbara Górnicka, Krystian Jażdżewski, Anna Wójcicka
BACKGROUND: Adrenocortical carcinoma is a rare finding among common adrenocortical tumors, but it is highly aggressive and requires early detection and treatment. Still, the differential diagnosis between benign and malignant lesions is difficult even for experienced pathologists and there is a significant need for novel diagnostic methods. In this study we aimed to reveal a complete set of microRNAs expressed in the adrenal gland and to identify easily detectable, stable and objective biomarkers of adrenocortical malignancy...
April 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28422753/steroid-metabolome-analysis-reveals-prevalent-glucocorticoid-excess-in-primary-aldosteronism
#3
Wiebke Arlt, Katharina Lang, Alice J Sitch, Anna S Dietz, Yara Rhayem, Irina Bancos, Annette Feuchtinger, Vasileios Chortis, Lorna C Gilligan, Philippe Ludwig, Anna Riester, Evelyn Asbach, Beverly A Hughes, Donna M O'Neil, Martin Bidlingmaier, Jeremy W Tomlinson, Zaki K Hassan-Smith, D Aled Rees, Christian Adolf, Stefanie Hahner, Marcus Quinkler, Tanja Dekkers, Jaap Deinum, Michael Biehl, Brian G Keevil, Cedric H L Shackleton, Jonathan J Deeks, Axel K Walch, Felix Beuschlein, Martin Reincke
BACKGROUND: Adrenal aldosterone excess is the most common cause of secondary hypertension and is associated with increased cardiovascular morbidity. However, adverse metabolic risk in primary aldosteronism extends beyond hypertension, with increased rates of insulin resistance, type 2 diabetes, and osteoporosis, which cannot be easily explained by aldosterone excess. METHODS: We performed mass spectrometry-based analysis of a 24-hour urine steroid metabolome in 174 newly diagnosed patients with primary aldosteronism (103 unilateral adenomas, 71 bilateral adrenal hyperplasias) in comparison to 162 healthy controls, 56 patients with endocrine inactive adrenal adenoma, 104 patients with mild subclinical, and 47 with clinically overt adrenal cortisol excess...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28420444/case-report-acth-secreting-pituitary-carcinoma-metastatic-to-the-liver-in-a-patient-with-a-history-of-atypical-pituitary-adenoma-and-cushing-s-disease
#4
Amy S Joehlin-Price, Douglas A Hardesty, Christina A Arnold, Lawrence S Kirschner, Daniel M Prevedello, Norman L Lehman
BACKGROUND: Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature. CASE PRESENTATION: The patient is a 51-year-old woman with Cushing's disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA)...
April 18, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28420172/subtype-diagnosis-of-primary-aldosteronism-is-adrenal-vein-sampling-always-necessary
#5
REVIEW
Fabrizio Buffolo, Silvia Monticone, Tracy A Williams, Denis Rossato, Jacopo Burrello, Martina Tetti, Franco Veglio, Paolo Mulatero
Aldosterone producing adenoma and bilateral adrenal hyperplasia are the two most common subtypes of primary aldosteronism (PA) that require targeted and distinct therapeutic approaches: unilateral adrenalectomy or lifelong medical therapy with mineralocorticoid receptor antagonists. According to the 2016 Endocrine Society Guideline, adrenal venous sampling (AVS) is the gold standard test to distinguish between unilateral and bilateral aldosterone overproduction and therefore, to safely refer patients with PA to surgery...
April 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28416583/physiological-and-pathological-roles-in-human-adrenal-of-the-glomeruli-defining-matrix-protein-npnt-nephronectin
#6
Ada Ee Der Teo, Sumedha Garg, Timothy Isaac Johnson, Wanfeng Zhao, Junhua Zhou, Celso Enrique Gomez-Sanchez, Mark Gurnell, Morris Jonathan Brown
Primary aldosteronism is a common cause of hypertension, which becomes refractory if undiagnosed, but potentially curable when caused by an aldosterone-producing adenoma (APA). The discovery of somatic mutations and differences in clinical presentations led to recognition of small but common zona glomerulosa (ZG)-like adenomas, distinct from classical large zona fasciculata-like adenomas. The inverse correlation between APA size and aldosterone synthase expression prompted us to undertake a systematic study of genotype-phenotype relationships...
April 17, 2017: Hypertension
https://www.readbyqxmd.com/read/28405951/posterior-retroperitonoscopic-adrenalectomy-a-back-door-access-with-an-unusually-rapid-learning-curve
#7
Sohail Bakkar, Gabriele Materazzi, Lorenzo Fregoli, Piermarco Papini, Paolo Miccoli
Posterior retroperitonoscopic adrenalectomy (PRA) has become a standard approach to the adrenal gland. The aim of this study was to report an initial experience with the procedure following a proper preparatory phase highlighting the rapidity, safety and effectiveness by which it could be introduced into a surgeon's practice. Between May 2015 and July 2016, 14 PRAs were performed in 14 patients (9 females and 5 males). The average age was 46 years, BMI: 25.5 kg/m(2), and ASA score: 2. Indications included: incidenatloma (n = 5), Conn's adenoma (n = 5), and Cushing's adenoma (n = 4)...
April 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28405879/histopathological-and-genetic-characterization-of-aldosterone-producing-adenomas-with-concurrent-subclinical-cortisol-hypersecretion-a-case-series
#8
Francesco Fallo, Isabella Castellano, Celso E Gomez-Sanchez, Yara Rhayem, Catia Pilon, Valentina Vicennati, Donatella Santini, Valeria Maffeis, Ambrogio Fassina, Paolo Mulatero, Felix Beuschlein, Martin Reincke
PURPOSE: Aldosterone-producing adenomas with concurrent subclinical cortisol hypersecretion are reported in an increasing number of patients. Five aldosterone-producing adenomas from patients with primary aldosteronism and subclinical hypercortisolism were examined. THE AIMS OF OUR STUDY WERE: (1) to analyze pathological features and immunohistochemical expression of CYP11B1 (11β-hydroxylase) and CYP11B2 (aldosterone synthase) in these tumors; (2) to investigate somatic mutations involved in adrenal steroid hypersecretion and/or tumor growth...
April 12, 2017: Endocrine
https://www.readbyqxmd.com/read/28402131/can-imaging-predict-subclinical-cortisol-secretion-in-patients-with-adrenal-adenomas-a-ct-predictive-score
#9
Cristina Mosconi, Valentina Vicennati, Dimitris Papadopoulos, Guido Di Dalmazi, Antonio M Morselli-Labate, Rita Golfieri, Renato Pasquali
OBJECTIVE: The aim of this study is to determine whether any correlation between CT findings and functional parameters exists to predict subclinical glucocorticoid secretion. MATERIALS AND METHODS: This is a retrospective database study of 55 patients with incidentally discovered adenomas, investigated through CT with an adrenal protocol, assessing diameters and attenuation values on the unenhanced and contrast-enhanced phases. Patients underwent blood cortisol and corticotropin evaluation and overnight dexamethasone suppression test (DST), in accordance with clinical recommendations...
April 12, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28400483/somatic-and-inherited-mutations-in-primary-aldosteronism
#10
Fabio Luiz Fernandes-Rosa, Sheerazed Boulkroun, Maria-Christina Zennaro
Primary aldosteronism (PA), the most common form of secondary hypertension, is caused in the majority of cases by unilateral aldosterone producing adenoma (APA) or bilateral adrenal hyperplasia. In the last years, recurrent somatic mutations in KCNJ5, CACNA1D, ATP1A1, and ATP2B3 were proved to be associated with APA development, explaining more than 50% of sporadic APA. The identification of these mutations have allowed the establishment of a model for APA development involving modifications in the intracellular ionic equilibrium and in the regulation of cell membrane potential, leading to autonomous aldosterone overproduction...
April 11, 2017: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/28391254/primary-pigmented-nodular-adrenocortical-disease-literature-review-and-case-report-of-a-6-year-old-boy
#11
Dragan Katanić, Dejan Kafka, Mirjana Živojinov, Jovan Vlaški, Zorana Budakov, Marija Knežević Pogančev, Ivana Vorgučin, Tomislav Ćuk
Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood...
April 10, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28390812/characterization-of-pancreatic-islet-cell-tumors-and-renal-tumors-induced-by-a-combined-treatment-of-streptozotocin-and-nicotinamide-in-male-sd-rats
#12
Yuki Kato, Koichi Masuno, Kae Fujisawa, Noriko Tsuchiya, Mikinori Torii, Atsuko Hishikawa, Takeshi Izawa, Mitsuru Kuwamura, Jyoji Yamate
We herein investigated the histopathological features, including proliferative activity and immunoexpression, of pancreatic islet cell tumors (ICTs) in male SD rats induced by streptozotocin (STZ) and nicotinamide (NA), and discussed their relevance to biological behaviors and prognoses. A total of 70 and 43% of rats developed ICTs 37-45 weeks after the treatment with STZ (50 or 75mg/kg, i.v.) and NA (350mg/kg, twice, p.o.), respectively. Among the islet tumors observed in the STZ/NA-treated groups, 75% were adenomas, while 25% were carcinomas...
April 5, 2017: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/28385310/prevalence-and-clinical-manifestations-of-primary-aldosteronism-encountered-in-primary%C3%A2-care%C3%A2-practice
#13
Silvia Monticone, Jacopo Burrello, Davide Tizzani, Chiara Bertello, Andrea Viola, Fabrizio Buffolo, Luisa Gabetti, Giulio Mengozzi, Tracy A Williams, Franco Rabbia, Franco Veglio, Paolo Mulatero
BACKGROUND: Despite being widely recognized as the most common form of secondary hypertension, among the general hypertensive population the true prevalence of primary aldosteronism (PA) and its main subtypes, aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia (BAH), remains a matter of debate. OBJECTIVES: This study sought to determine the prevalence and clinical phenotype of PA in a large cohort of unselected patients with hypertension, consecutively referred to our hypertension unit, by 19 general practitioners from Torino, Italy...
April 11, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28378050/adrenal-vein-sampling-for-primary-aldosteronism-a-2-week-protocol-for-withdrawal-of-renin-stimulating-antihypertensives
#14
Kevin C Ching, Debbie L Cohen, Douglas L Fraker, Scott O Trerotola
OBJECTIVE: For evaluation of primary aldosteronism, international guidelines recommend a 4-6 week withdrawal of spironolactone, eplerenone, and amiloride prior to adrenal vein sampling (AVS). It is not always feasible to withdraw these drugs in patients with severe hypertension and hypokalemia. We present our experience evaluating the efficacy and clinical outcomes of a 2-week protocol for withdrawal of renin-stimulating antihypertensives prior to AVS. DESIGN: A single-center retrospective review of all patients who underwent AVS for primary aldosteronism between January 2014 and December 2015...
April 4, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28377801/current-best-practice-in-the-management-of-patients-after-pituitary-surgery
#15
REVIEW
Alessandro Prete, Salvatore Maria Corsello, Roberto Salvatori
Sellar and parasellar masses are a common finding, and most of them are treated surgically via transsphenoidal approach. This type of surgery has revolutionized the approach to several hypothalamic-pituitary diseases and is usually effective, and well-tolerated by the patient. However, given the complex anatomy and high density of glandular, neurological and vascular structures in a confined space, transsphenoidal surgery harbors a substantial risk of complications. Hypopituitarism is one of the most frequent sequelae, with central adrenal insufficiency being the deficit that requires a timely diagnosis and treatment...
March 2017: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28368480/genetic-and-histopathologic-inter-tumor-heterogeneity-in-primary-aldosteronism
#16
Kei Omata, Yuto Yamazaki, Yasuhiro Nakamura, Sharath K Anand, Justine A Barletta, Hironobu Sasano, William E Rainey, Scott A Tomlins, Anand Vaidya
Context: Whether primary aldosteronism (PA) is the consequence of a monoclonal or multiclonal process is unclear. Case Description: A 48-year-old man with severe bilateral PA refractory to medical therapy underwent unilateral adrenalectomy of the dominant adrenal. Although computed tomography (CT) showed three left-sided cortical nodules, post-surgical histopathology and genetic analysis revealed five different adrenocortical adenomas. Two zona fasciculata (ZF)-like aldosterone producing adenomas (APAs) each harbored distinct known somatic KCNJ5 mutations (L168R and T158A)...
March 24, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28365908/spinal-adrenal-cortical-adenoma-associated-with-beckwith-wiedemann-syndrome-case-report-and-review-of-the-literature
#17
Javier Giner, Isabel Esteban, Fernando Carceller, Javier Saceda, R M Regojo
PURPOSE: Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. To date, only eight spinal cases have been reported. This is the third reported case in pediatric population, the first one associated with genetic syndrome and the first benign to recur...
April 1, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28359061/intrafamilial-phenotypic-variability-and-consequences-of-non-compliance-with-treatment-in-congenital-adrenal-hyperplasia-and-congenital-hypothyroidism-within-a-single-family%C3%A2
#18
Nicola Improda, Caroline Ponmani, Nadia Schoenmakers, Senthil Senniappan, Abigail Atterbury, Angela Barnicoat, Krishna Chatterjee, Mehul T Dattani
BACKGROUND: Coexistence of congenital adrenal hyperplasia (CAH) and congenital hypothyroidism (CH) due to TG mutation in the same non-consanguineous family is rare. CASE SERIES: We report 4 siblings born to unrelated parents, the father being an asymptomatic carrier of homozygous p.V281L and heterozygous p.I172N CYP21A2 mutations. Sibling 1 had salt-wasting CAH (CYP21A2 genotype Intron 2 splice/p.I172N and p.V281L). She also had CH (TG genotype p.R296/ p.T1416Rfs*30) and learning difficulties...
March 30, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28355719/-a-clinical-analysis-of-123-cases-of-primary-empty-sella
#19
J Li, H W Jia, C L Wang, R Zhang, M Y Qu, W Li, M H Yuan, J Cui, Q He, H Y Wei, T H Zhu, Z S Ma, W Liu, Z L Dong, Z G Gao
Objective: This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES). Methods: The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied. Results: (1) The average age of the 123 (male 43, female 80) PES patients was (59.2±13.6) years (ranging 24-92 years), among whom 61% patients were in the age group between 50-69 years. (2) The symptoms of the patients included fatigue (56...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28348073/from-benign-adrenal-incidentaloma-to-adrenocortical-carcinoma-an-exceptional-random-event
#20
Ines Belmihoub, Stephane Silvera, Mathilde Sibony, Bertrand Dousset, Paul Legmann, Xavier Bertagna, Jerome Bertherat, Guillaume Assie
New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Clinically he had a normal blood pressure and no sign of hormonal hypersecretion. The hormonal work-up showed no hormone excess: urinary free cortisol level was normal, the diurnal cortisol rhythm was respected and urinary catecholamine metabolites levels were normal...
March 27, 2017: European Journal of Endocrinology
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