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adrenal insufficiency

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https://www.readbyqxmd.com/read/29755708/growth-and-endocrine-function-in-tunisian-thalassemia-major-patients
#1
Naouel Guirat Dhouib, Monia Ben Khaled, Monia Ouederni, Habib Besbes, Ridha Kouki, Fethi Mellouli, Mohamed Bejaoui
β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 β-TM patients (16 males, 12 females) regularly transfused with packed red cell since early years of life...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29750931/relative-adrenal-insufficiency-is-associated-with-prolonged-postoperative-hemodynamic-instability
#2
Amine Mazine, Ismail Bouhout, Nadim Saydy, Louis-Mathieu Stevens, Maude Gagné-Loranger, Frédéric Vanden Eynden, Daniel Parent, Yoan Lamarche, Denis Bouchard
BACKGROUND: We assessed the hypothesis that a significant proportion of patients undergoing cardiac surgery suffer from postoperative relative adrenal insufficiency (RAI), and that this puts them at higher risk of persistent hemodynamic instability. METHODS: A prospective study included 135 patients who underwent cardiac surgery using cardiopulmonary bypass between July 2006 and December 2007. Adrenal function was assessed 36 hours after surgery using a 1 μg cosyntropin (ACTH) stimulation test...
May 8, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29749880/adrenal-insufficiency-due-to-ritonavir-triamcinolone-drug-drug-interaction-without-preceding-cushing-s-syndrome
#3
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/29748231/management-of-endocrine-disease-differential-diagnosis-investigation-and-therapy-of-bilateral-adrenal-incidentalomas
#4
Isabelle Bourdeau, Nada El Ghorayeb, Nadia Gagnon, Andre Lacroix
The investigation and management of unilateral adrenal incidentalomas have been extensively considered in the last decades. While bilateral adrenal incidentalomas represent about 15 percent of adrenal incidentalomas, they have been less frequently discussed. The differential diagnosis of bilateral incidentalomas includes metastasis, primary bilateral macronodular adrenal hyperplasia (BMAH) and bilateral cortical adenomas. Les frequent etiologies are bilateral pheochromocytomas, congenital adrenal hyperplasia, Cushing's disease or ectopic ACTH secretion with secondary bilateral adrenal hyperplasia, primary malignancies, myelolipomas, infections or hemorrhage...
May 10, 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29742971/insufficiency-of-the-zona-glomerulosa-of-the-adrenal-cortex-and-progressive-kidney-insufficiency-following-unilateral-adrenalectomy-case-report-and-discussion
#5
Joanna Kanarek-Kucner, Adrian Stefański, Rufus Barraclough, Tomasz Gorycki, Jacek Wolf, Krzysztof Narkiewicz, Michał Hoffmann
BACKGROUND: Primary aldosteronism (PA) is the most common cause of secondary hypertension and bilateral adrenal hyperplasia (BAH) and aldosterone-producing adenoma (APA) seem to be the most common causes of PA. Unilateral adrenalectomy (UA) is the preferred treatment for APA, although the benefits are still difficult to assess. CASE REPORT: We present a case report of a 69-year old man with a 30 year history of hypertension and probably long-standing PA due to APA, with typical organ complications...
May 9, 2018: Blood Pressure
https://www.readbyqxmd.com/read/29732365/corticosteroids-in-pediatric-heart-surgery-myth-or-reality
#6
REVIEW
Daniel P Fudulu, Ben Gibbison, Thomas Upton, Serban C Stoica, Massimo Caputo, Stafford Lightman, Gianni D Angelini
Background: Corticosteroids have been administered prophylactically for more than 60 years in pediatric heart surgery, however, their use remains a matter of debate. There are three main indications for corticosteroid use in pediatric heart surgery with the use of cardiopulmonary bypass (CPB): (1) to blunt the systemic inflammatory response (SIRS) induced by the extracorporeal circuit; (2) to provide perioperative supplementation for presumed relative adrenal insufficiency; (3) for the presumed neuroprotective effect during deep hypothermic circulatory arrest operations...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29731953/adrenal-insufficiency-in-cystic-fibrosis-a-rare-phenomenon
#7
Sébastien Préville-Ratelle, Adèle Coriati, Aurélie Ménard, Isabelle Bourdeau, François Tremblay, Yves Berthiaume
Background: The prevalence of adrenal insufficiency (AI) in cystic fibrosis (CF) is unknown. The frequent use of glucocorticoids (inhaled or systemic) may induce the long-term suppression of the hypothalamic-pituitary-adrenal axis. Methods: We reviewed the results of adrenocorticotropic hormone (ACTH) stimulation tests done over a 10-year period to evaluate adrenal function in 69 CF patients of the CHUM CF clinic. Clinical characteristics of AI patients were compared to adrenal-sufficient (AS) patients...
2018: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/29730618/role-of-sampling-times-and-serum-cortisol-cut-off-concentrations-on-the-routine-assessment-of-adrenal-function-using-the-standard-cosyntropin-test-in-an-academic-hospital-from-spain-a-retrospective-chart-review
#8
Andrés E Ortiz-Flores, Elisa Santacruz, Lucía Jiménez-Mendiguchia, Ana García-Cano, Lia Nattero-Chávez, Héctor F Escobar-Morreale, Manuel Luque-Ramírez
OBJECTIVES: Aiming to validate the use of a single poststimulus sampling protocol for cosyntropin test short standard high-dose test (SST) in our institution, our primary objectives were (1) to determine the concordance between 30 and 60 min serum cortisol (SC) measurements during SST; and (2) to evaluate the diagnostic agreement between both sampling times when using classic or assay-specific and sex-specific SC cut-off values. The secondary objectives included (1) estimating the specificity and positive predictive value of 30 and 60 min sampling times while considering the suspected origin of adrenal insufficiency (AI); and (2) obtaining assay-specific cut-off values for SC after SST in a group of subjects with normal hypothalamic-pituitary-adrenal (HPA) axis...
May 5, 2018: BMJ Open
https://www.readbyqxmd.com/read/29720511/prednisolone-is-associated-with-a-worse-bone-mineral-density-in-primary-adrenal-insufficiency
#9
Kathrin Frey, Tina Kienitz, Julia Schulz, Manfred Ventz, Kathrin Zopf, Marcus Quinkler
CONTEXT: Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) therapy. Daily GC doses are often above the physiological cortisol production rate and can cause long-term morbidities such as osteoporosis. No prospective trial has investigated long-term effect of different GC-therapies on bone mineral density (BMD) in those patients. OBJECTIVES: To determine if patients on hydrocortisone (HC) or prednisolone show changes in BMD after follow-up of 5...
May 2, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29718281/comprehensive-endocrine-metabolic-evaluation-of-patients-with-alstr%C3%A3-m-syndrome-compared-to-bmi-matched-controls
#10
Joan C Han, Daniela P Reyes-Capo, Chia-Ying Liu, James C Reynolds, Evrim Turkbey, Ismail Baris Turkbey, Joy Bryant, Jan D Marshall, Jürgen K Naggert, William A Gahl, Jack A Yanovski, Meral Gunay-Aygun
Background: Alström syndrome (AS), a monogenic form of obesity, is caused by recessive mutations in the centrosome- and basal body-associated gene, ALMS1. AS is characterized by retinal dystrophy, sensory hearing loss, cardiomyopathy, childhood obesity, and metabolic derangements. Objective: We sought to characterize the endocrine and metabolic features of AS while accounting for obesity as a confounder by comparing patients with AS to BMI-matched controls. Methods: We evaluated 38 patients with AS (age 2-38y) who were matched with 76 controls (age 2-48y) by age, sex, race, and BMI...
April 27, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29716733/acute-adrenal-crisis-and-mortality-in-adrenal-insufficiency-still-a-concern-in-2018
#11
Stefanie Hahner
Despite established replacement therapy, mortality in patients suffering from chronic adrenal insufficiency is increasing. This may be partly explained by the fact that lack of adrenal stress hormones impairs the body's capacity to deal adequately with stress situations, resulting in life-threatening adrenal crises. Since many such situations are of rapid onset, concepts that allow for quick response to emergencies are particularly important. Optimal education for patients and relatives, improved awareness on the part of health professionals and the development of new easy-to-use drugs for acute therapy are of prime importance...
April 28, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29696788/delayed-peak-response-of-cortisol-to-insulin-tolerance-test-in-patients-with-prader-willi-syndrome
#12
Yuji Oto, Keiko Matsubara, Tadayuki Ayabe, Masahisa Shiraishi, Nobuyuki Murakami, Hiroshi Ihara, Tomoyo Matsubara, Toshiro Nagai
Deaths among children with Prader-Willi syndrome (PWS) are often related to only mild or moderate upper respiratory tract infections, and many causes of death remain unexplained. Several reports have hypothesized that patients with PWS may experience latent central adrenal insufficiency. However, whether PWS subjects suffer from alteration of the hypothalamus-pituitary-adrenal (HPA) axis remains unclear. This study aimed to explore the HPA axis on PWS. We evaluated the HPA axis in 36 PWS patients (24 males, 12 females; age range, 7 months to 12 years; median age 2...
April 25, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29695647/a-case-of-tsh-secreting-pituitary-adenoma-with-cyclic-fluctuations-in-serum-tsh-levels
#13
Hideyuki Okuma, Koshi Hashimoto, Takuya Ohashi, Masatomo Mihara, Isao Minami, Hajime Izumiyama, Shigekazu Sasaki, Naoko Inoshita, Hiroshi Nishioka, Shozo Yamada, Takanobu Yoshimoto
A 29-year-old man was referred to our department due to adrenal insufficiency with the inappropriate secretion of TSH (SITSH). Magnetic resonance imaging revealed a pituitary tumor. A weak TSH response in the TRH test, elevated sex hormone binding globulin (SHBG) levels, and the absence of a family medical history of SITSH or TRβ gene mutations supported the diagnosis of TSH-secreting pituitary adenoma (TSHoma). However, complete TSH suppression and a blunted cholesterol response in the T3 suppression test as well as normal glycoprotein α-subunit (α-GSU) levels were not compatible with TSHoma...
April 26, 2018: Endocrine Journal
https://www.readbyqxmd.com/read/29694951/vanishing-17-hydroxyprogesterone-concentrations-in-21-hydroxylase-deficiency
#14
Thomas Reinehr, Juliane Rothermel, Andreas Wegener-Panzer, Michaela F Hartmann, Stefan A Wudy, Paul-Martin Holterhus
We present a boy with a genetically proven congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. While massively elevated 17-hydroxyprogesterone (17-OHP) concentrations after birth led to the diagnosis, 17-OHP concentrations became immeasurable starting with the second year of life even though the dose of hydrocortisone was continuously decreased to ∼7 mg/m2/day. Furthermore, 17-OHP levels were immeasurable during the ACTH test and after withdrawing hydrocortisone medication. In contrast, ACTH levels increased after cessation of hydrocortisone treatment suggesting complete primary adrenal cortex failure...
April 25, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29690985/-delayed-diagnosis-of-adrenal-crisis
#15
Theis Chang Gielstrup, Esben Søndergaard, Jesper Fjølner
Adrenal crisis is a life-threatening emergency with excess mortality of patients with adrenal insufficiency. This case report is about delayed diagnosis of a patient with unknown primary adrenal insufficiency, reduced consciousness and shock.
April 9, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29685115/acquired-modification-of-sphingosine-1-phosphate-lyase-activity-is-not-related-to-adrenal-insufficiency
#16
Gulin Sunter, Ece Oge Enver, Azad Akbarzade, Serap Turan, Pinar Vatansever, Dilek Ince Gunal, Goncagul Haklar, Abdullah Bereket, Kadriye Agan, Tulay Guran
BACKGROUND: Congenital sphingosine-1-phosphate (S1P) lyase deficiency due to biallelic mutations in SGPL1 gene has recently been described in association with primary adrenal insufficiency and steroid-resistant nephrotic syndrome. S1P lyase, on the other hand, is therapeutically inhibited by fingolimod which is an oral drug for relapsing multiple sclerosis (MS). Effects of this treatment on adrenal function has not yet been evaluated. We aimed to test adrenal function of MS patients receiving long-term fingolimod treatment...
April 23, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29682773/mortality-data-from-the-european-adrenal-insufficiency-registry-patient-characterization-and-associations
#17
Marcus Quinkler, Bertil Ekman, Pinggao Zhang, Andrea M Isidori, Robert D Murray
OBJECTIVE: Mortality from primary and secondary adrenal insufficiency (AI; PAI and SAI, respectively) is 2-3-fold higher than in the general population. Mortality relates to cardiovascular disease, acute adrenal crisis (AC), cancer and infections; however, there has been little further characterization of patients who have died. DESIGN/METHODS: We analysed real-world data from 2034 patients (801 PAI, 1233 SAI) in the European Adrenal Insufficiency Registry (EU-AIR; NCT01661387)...
April 22, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29675257/treatment-of-addison-s-disease-during-pregnancy
#18
Diana Oliveira, Adriana Lages, Sandra Paiva, Francisco Carrilho
Addison's disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison's disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29673814/assessment-of-hair-cortisol-as-a-potential-biomarker-for-possible-adrenal-suppression-due-to-inhaled-corticosteroid-use-in-children-with-asthma-a-retrospective-observational-study
#19
Laura Smy, Kaitlyn Shaw, Ursula Amstutz, Michelle Staub, Shahnaz Chaudhry, Anne Smith, Bruce Carleton, Gideon Koren
BACKGROUND: Inhaled corticosteroids (ICS) are the recommended long-term control therapy for asthma in children. However, concern exists regarding potential adrenal suppression with chronic ICS use. Our pilot study reported that hair cortisol in children was 50% lower during ICS therapy than prior to therapy, suggestive of adrenal suppression. OBJECTIVE: To evaluate hair cortisol concentration (HCC) as a potential biomarker for possible adrenal suppression from ICS use in children with asthma...
April 16, 2018: Clinical Biochemistry
https://www.readbyqxmd.com/read/29670764/corticosteroid-weaning-in-stable-heart-transplant-patients-guidance-by-serum-cortisol-level
#20
David A Baran, Cheryl Rosenfeld, Mark J Zucker
Background: Despite earlier studies describing the feasibility of steroid weaning in heart transplant patients, the majority of patients are maintained on steroid therapy for life. We examined a strategy based on a single morning serum cortisol measurement. Methods: We assigned stable posttransplant patients, who were maintained on tacrolimus, mycophenolate mofetil, and corticosteroids, into one of two groups based on a screening morning cortisol level. Patients with a cortisol < 8 micrograms/deciliter were assigned to a "maintenance" group and the others were assigned to the weaning group and steroids were tapered off over 4-6 weeks...
2018: Journal of Transplantation
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