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https://www.readbyqxmd.com/read/29458220/incidence-of-autoimmune-and-related-disorders-after-resolution-of-endogenous-cushing-syndrome-in-children
#1
Christina Tatsi, Meg Keil, Charalampos Lyssikatos, Elena Belyavskaya, Constantine A Stratakis, Maya B Lodish
Glucocorticoids are widely used for immunosuppression in autoimmune diseases. After the resolution of hypercortisolemia, the immune system recovers allowing for autoimmune diseases to manifest. Here we investigated the presence of autoimmune and related diseases that developed after cure of endogenous Cushing syndrome (CS) in children. We identified 129 children who were diagnosed and successfully treated for endogenous CS at the National Institutes of Health from 1997 until 2017, and who were followed for at least 6 months after treatment...
February 19, 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29456353/the-utility-of-preoperative-acth-cortisol-ratio-for-the-diagnosis-and-prognosis-of-cushing-s-disease
#2
Alev Selek, Berrin Cetinarslan, Zeynep Canturk, Ilhan Tarkun, Ozlem Zeynep Akyay, Burak Cabuk, Savas Ceylan
Purpose: Cushing's syndrome (CS) is a rare disease having diagnostic difficulties. Many diagnostic tests have been defined but none of these are diagnostic alone. Determination of the cause is another problem which sometimes requires more sophisticated and invasive procedures. Therefore, we aimed to evaluate the utility of pretreatment plasma adrenocorticotropic hormone (ACTH)/cortisol ratios in patients with confirmed endogenous CS for the diagnosis and differential diagnosis of CS. Materials and Methods: This retrospective evaluation included 145 patients with the diagnosis of CS, 119 patients with Cushing's disease (CD), and 26 patients with ACTH-independent CS (AICS), in a university hospital...
January 2018: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29450857/hypokalemia-associated-with-pseudo-cushing-s-syndrome-and-magnesium-deficiency-induced-by-chronic-alcohol-abuse
#3
Masafumi Kurajoh, Keiko Ohsugi, Miki Kakutani-Hatayama, Takuhito Shoji, Hidenori Koyama
Hypokalemia and hypomagnesemia are frequently observed in patients with chronic alcoholism. However, the involvement of deranged cortisol regulation in patients with those conditions has not been reported. A 63-year-old Japanese male with chronic alcoholism was referred to the Department of Diabetes, Endocrinology and Metabolism for examination and treatment of hypokalemic periodic paralysis. Laboratory findings showed hypokalemia (2.3 mmol/l), as well as a high level of urinary excretion of potassium and hypomagnesemia (1...
February 15, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29447799/iatrogenic-cushing-s-syndrome-and-adrenal-insufficiency-in-infants-on-intranasal-dexamethasone-drops-for-nasal-obstruction-case-series-and-literature-review
#4
Rohan R Joshi, Alison Maresh
The use of intranasal steroid drops for nasal obstruction in infants is common practice and can prevent more invasive surgical procedures; however, it is not without complication. We describe 2 cases of iatrogenic Cushing's secondary to nasal steroids in infants with nasal obstruction, discuss the etiology of this unusual complication, and review previous literature reports. While reporting in the literature is sparse, these cases highlight the risk of development of adrenal insufficiency with usage of nasal steroid drops in infants as well as the need for close monitoring of administration and tapering of the drops...
February 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29442480/cushing-s-disease-major-difficulties-in-diagnosis-and-management-during-pregnancy
#5
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/29440375/preoperative-medical-treatment-in-cushing-s-syndrome-frequency-of-use-and-its-impact-on-postoperative-assessment-data-from-ercusyn
#6
Elena Valassi, Holger Franz, Thierry Brue, Richard A Feelders, Romana T Netea-Maier, Stylianos Tsagarakis, Susan M Webb, Maria Kostadinova Yaneva, Martin Reincke, Michael Droste, Irina Komerdus, Dominique Maiter, Darko Kastelan, Philippe Chanson, Marija Pfeifer, Christian J Strasburger, Miklos Toth, Olivier Chabre, Michal Krsek, Carmen Fajardo Montañana, Marek Bolanowski, Alicia Santos, Peter J Trainer, John A H Wass, Antoine Tabarin
BACKGROUND: Surgery is the definitive treatment of Cushing's syndrome (CS) but medications may also be used as a first-line therapy. Whether preoperative medical treatment (PMT) affects postoperative outcome remains controversial. OBJECTIVE: 1) Evaluate how frequently PMT is given to CS patients across Europe 2) examine differences in preoperative characteristics of patients who receive PMT and those who undergo primary surgery; 3) determine if PMT influences postoperative outcome in pituitary-dependent CS (PIT-CS)...
February 12, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29440131/diagnostic-accuracy-and-comparison-of-bipss-in-response-to-lysine-vasopressin-and-hcrh
#7
Kush Dev Singh Jarial, Anil Bhansali, Vivek Gupta, Paramjeet Singh, Kanchan K Mukherjee, Akhilesh Sharma, Rakesh Vasishta, Suja P Sukumar, Naresh Sachdeva, Rama Walia
CONTEXT: Bilateral inferior petrosal sinus sampling (BIPSS) using hCRH is currently considered the 'gold standard' test for the differential diagnosis of ACTH-dependent Cushing's syndrome (CS). Vasopressin is more potent than CRH to stimulate ACTH secretion as shown in animal studies, however; no comparative data of its use is available during BIPSS. OBJECTIVE: To study the diagnostic accuracy and comparison of hCRH and lysine vasopressin (LVP) stimulation during BIPSS...
February 12, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29426273/testosterone-and-cortisol-secreting-oncocytic-adrenocortical-adenoma-in-the-pediatric-age-group
#8
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29406000/neonatal-cushing-syndrome-a-rare-but-potentially-devastating-disease
#9
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/29390296/carney-complex-with-prkar1a-gene-mutation-a-case-report-and-literature-review
#10
Qiuli Liu, Dali Tong, Gaolei Liu, Yuting Yi, Dianzheng Zhang, Jun Zhang, Yao Zhang, Zaoming Huang, Yaoming Li, Rongrong Chen, Yanfang Guan, Xin Yi, Jun Jiang
RATIONALE: Carney complex (CNC) is a multiple neoplasia syndrome with autosomal dominant inheritance. CNC is characterized by the presence of myxomas, spotty skin pigmentation, and endocrine overactivity. No direct correlation has been established between disease-causing mutations and phenotype. PATIENT CONCERNS: A 16-year-old boy was admitted because of excessive weight gain over 3 years and purple striae for 1 year. Physical examination revealed Cushingoid features and spotty skin pigmentation on his face, lip, and sclera...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29387897/-endocrine-paraneoplastic-syndromes
#11
REVIEW
N Reisch, M Reincke
Endocrine paraneoplastic syndromes result from the production of bioactive substances from neoplastic cells, of endocrine or neuroendocrine origin. Typically these are located in the lungs, the gastrointestinal tract, pancreas, thyroid gland, adrenal medulla, skin, prostate or breast. In endocrine paraneoplastic syndromes the secretion of peptides, amines or other bioactive substances is always ectopic and not related to the anatomical source. The clinical presentation, however, is indistinguishable from a suspected eutopic endocrine tumor posing a diagnostic challenge...
January 31, 2018: Der Internist
https://www.readbyqxmd.com/read/29384929/case-report-of-a-bilateral-adrenal-myelolipoma-associated-with-cushing-disease
#12
Se Yoon Park, Mi Kyung Kwak, Hye Jeong Kim, Hyeong Kyu Park, Kyo-Il Suh, Myung Hi Yoo, So Young Jin, Sumi Yun, Dong Won Byun
RATIONALE: Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive. PATIENT CONCERNS: During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29379569/brain-atrophy-and-hypomyelination-associated-with-iatrogenic-cushing-syndrome-in-an-infant
#13
Sumeyra Dogan, Mehmet S Dogan, Filiz Tutunculer, Ozge Yapiciugurlar, Hakan Genchellac
Prolonged use of topical corticosteroids, particularly in infants, albeit rare, may lead to Cushing syndrome. Central nervous system abnormalities including brain atrophy and delayed myelination on cranial magnetic resonance imaging has been reported in patients with corticosteroid treatment. We herein report a 5-month-old female infant referred to Department of Pediatric Endocrinology, Edirne, Turkey with brain atrophy and myelination delay that might be due to iatrogenic Cushing syndrome caused by topical corticosteroid use...
2018: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/29357735/niche-point-of-care-endocrine-testing-reviews-of-intraoperative-parathyroid-hormone-and-cortisol-monitoring
#14
Li-Sheng Chen, Ravinder J Singh
Point-of-care (POC) testing, which provides quick test results in near-patient settings with easy-to-use devices, has grown continually in recent decades. Among near-patient and on-site tests, rapid intraoperative and intra-procedural assays are used to quickly deliver critical information and thereby improve patient outcomes. Rapid intraoperative parathyroid hormone (ioPTH) monitoring measures postoperative reduction of parathyroid hormone (PTH) to predict surgical outcome in patients with primary hyperparathyroidism, and therefore contributes to the change of parathyroidectomy to a minimally invasive procedure...
January 22, 2018: Critical Reviews in Clinical Laboratory Sciences
https://www.readbyqxmd.com/read/29348428/evaluation-of-quantitative-parameters-for-distinguishing-pheochromocytoma-from-other-adrenal-tumors
#15
Youichi Ohno, Masakatsu Sone, Daisuke Taura, Toshinari Yamasaki, Katsutoshi Kojima, Kyoko Honda-Kohmo, Yorihide Fukuda, Koji Matsuo, Toshihito Fujii, Akihiro Yasoda, Osamu Ogawa, Nobuya Inagaki
Adrenal tumors are increasingly found incidentally during imaging examinations. It is important to distinguish pheochromocytomas from other adrenal tumors because of the risk of hypertensive crisis. Although catecholamines and their metabolites are generally used to diagnose pheochromocytoma, false-positive test results are common. An effective screening method to distinguish pheochromocytoma from adrenal incidentalomas is needed. We analyzed 297 consecutive patients with adrenal incidentalomas. Our findings included 162 non-functioning tumors, 47 aldosterone-producing adenomas, 26 metastases, 22 cases of subclinical Cushing's syndrome, 21 pheochromocytomas, 12 cases of Cushing's syndrome, and 7 adrenocortical cancers...
January 18, 2018: Hypertension Research: Official Journal of the Japanese Society of Hypertension
https://www.readbyqxmd.com/read/29343284/extensive-armc5-genetic-variance-in-primary-bilateral-macronodular-adrenal-hyperplasia-that-started-with-exophthalmos-a-case-report
#16
Ping Jin, Muhammad Usman Janjua, Qin Zhang, Chang-Sheng Dong, Youbo Yang, Zhao-Hui Mo
BACKGROUND: Primary bilateral macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome characterized by the presence of bilateral secretory adrenal nodules. Recent studies have shown that primary bilateral macronodular adrenal hyperplasia is caused by combined germline and somatic mutations of the ARMC5 gene. Exophthalmos is an underappreciated sign of Cushing's syndrome. CASE PRESENTATION: A 52-year-old Chinese woman with progressively worsening bilateral proptosis presented to our hospital...
January 18, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29340776/ectopic-cushing-s-syndrome-secondary-to-olfactory-neuroblastoma
#17
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...
January 17, 2018: Acta Neurochirurgica
https://www.readbyqxmd.com/read/29327532/adrenal-oncocytoma-a-rare-functional-tumor-presenting-as-cushing-syndrome
#18
Hozefa Runderawala, Samrat Shah, Abizer Manked
Adrenal oncocytoma is very rare pathological variant of adrenal neoplasm. These are usually large and non-functional; however, rarely functional adrenal oncocytomas are also presented as Cushing's syndrome and pheochromocytoma. We report a case of adrenal oncocytoma in 38 year old female presented with symptoms of Cushing Syndrome.
December 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29315782/exogenous-cushing-syndrome-due-to-misuse-of-potent-topical-steroid
#19
Fatma Özgüç Çömlek, Sevim Örüm, Salih Aydın, Filiz Tütüncüler
We report an infant with exogenous Cushing syndrome after being treated for 2 months with a potent topical corticosteroid via the mother's application of topical clobetasol for diaper rash without a prescription. We emphasize that potent topical steroids should be used with great caution, especially when used under occlusion (e.g., diaper area) and that parents should be warned about potential side effects of these medications, particularly when used in infants.
January 9, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29313180/a-prospective-longitudinal-study-of-pasireotide-in-nelson-s-syndrome
#20
Eleni Daniel, Miguel Debono, Sharon Caunt, Constantine Girio-Fragkoulakis, Stephen J Walters, Scott A Akker, Ashley B Grossman, Peter J Trainer, John Newell-Price
PURPOSE: Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS: Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 μg s...
January 8, 2018: Pituitary
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