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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/29750931/relative-adrenal-insufficiency-is-associated-with-prolonged-postoperative-hemodynamic-instability
#1
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/29748231/management-of-endocrine-disease-differential-diagnosis-investigation-and-therapy-of-bilateral-adrenal-incidentalomas
#2
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
#3
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/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
#4
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
#5
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
#6
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/29694951/vanishing-17-hydroxyprogesterone-concentrations-in-21-hydroxylase-deficiency
#7
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
#8
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
#9
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
#10
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
#11
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/29661472/news-about-the-genetics-of-congenital-primary-adrenal-insufficiency
#12
Florence Roucher-Boulez, Delphine Mallet-Motak, Véronique Tardy-Guidollet, Rita Menassa, Claire Goursaud, Ingrid Plotton, Yves Morel
Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-threatening. Today, 80% of pediatric forms of PAI have a genetic origin but 5% have no clear genetic support. Recently discovered mutations in genes relating to oxidative stress have opened the way to research on genes unrelated to the adrenal gland. Identification of causal mutations in a gene responsible for PAI allows genetic counseling, guidance of follow-up and prevention of complications...
April 13, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29606279/therapeutic-patient-education-in-adrenal-insufficiency
#13
Laurence Guignat
It is essential to encourage patient autonomy in the management of their illness, and notably their participation in treatment education programs; specific programs target avoidance or early preventive treatment of acute adrenal insufficiency, which is a life-threatening complication. Therapeutic patient education is recommended by the two international consensus statements on the management of primary adrenal insufficiency and the French consensus on adrenal insufficiency. Although there is no common international reference framework to date, the objective of the French consensus was to provide a frame of reference to facilitate the development of therapeutic education for patients with adrenal insufficiency...
March 29, 2018: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29576868/a-rare-cause-of-primary-adrenal-insufficiency-due-to-a-homozygous-arg188cys-mutation-in-the-star-gene
#14
Lukas Burget, Laura Audí Parera, Monica Fernandez-Cancio, Rolf Gräni, Christoph Henzen, Christa E Flück
Steroidogenic acute regulatory protein ( STAR ) is a key protein for the intracellular transport of cholesterol to the mitochondrium in endocrine organs (e.g. adrenal gland, ovaries, testes) and essential for the synthesis of all steroid hormones. Several mutations have been described and the clinical phenotype varies strongly and may be grouped into classic lipoid congenital adrenal hyperplasia (LCAH), in which all steroidogenesis is disrupted, and non-classic LCAH, which resembles familial glucocorticoid deficiency (FGD), which affects predominantly adrenal functions...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29568655/posaconazole-induced-adrenal-insufficiency-in-a-case-of-chronic-myelomonocytic-leukemia
#15
Ann Miller, Lauren K Brooks, Silpa Poola-Kella, Rana Malek
Introduction: Posaconazole is an azole used in treatment and prophylaxis of a broad spectrum of fungal infections. Antifungals such as ketoconazole have been shown to cause primary adrenal insufficiency (AI) as a result of direct inhibition on the steroidogenesis pathway. There is only one reported case of primary AI induced by posaconazole in a patient with mucormycosis. We report a case of posaconazole-related primary AI. Case: A 63-year-old man with chronic myelomonocytic leukemia was admitted for fatigue and intermittent nausea and vomiting...
2018: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/29566378/adrenal-insufficiency-sex-reversal-and-angelman-syndrome-due-to-uniparental-disomy-unmasking-a-mutation-in-cyp11a1
#16
Ahlee Kim, Masanobu Fujimoto, Vivian Hwa, Philippe Backeljauw, Andrew Dauber
BACKGROUND/AIMS: Cholesterol side-chain cleavage enzyme (P450scc) deficiency is a rare genetic disorder causing primary adrenal insufficiency with or without a 46,XY disorder of sexual development (DSD). Herein, we report a case of the combination of primary adrenal insufficiency, a DSD (testes with female external genitalia in a setting of a 47,XXY karyotype), and Angelman syndrome. METHODS: Comprehensive genetic analyses were performed, including a single nucleotide polymorphism microarray and whole-exome sequencing...
2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29515921/acute-primary-adrenal-insufficiency-after-hip-replacement-in-a-patient-with-acute-intermittent-porphyria
#17
Adele Latina, Massimo Terzolo, Anna Pia, Giuseppe Reimondo, Elena Castellano, Micaela Pellegrino, Giorgio Borretta
Adrenal insufficiency is a potentially life-threatening condition when it occurs acutely, as in adrenal hemorrhage. Generally it is not reversible and requires chronic replacement therapy. Acute intermittent porphyria (AIP) is a rare genetic disease characterized by alterations in heme biosynthesis that result in accumulation of precursors in tissues. A crisis can be triggered by many conditions such as surgery and infections. Symptoms are similar to those of acute hypoadrenalism. Moreover, both conditions are characterized by hyponatremia...
2018: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/29506479/a-case-of-an-infant-suspected-as-image-syndrome-who-were-finally-diagnosed-with-mirage-syndrome-by-targeted-mendelian-exome-sequencing
#18
Yoon-Myung Kim, Go Hun Seo, Gu-Hwan Kim, Jung Min Ko, Jin-Ho Choi, Han-Wook Yoo
BACKGROUND: Adrenal hypoplasia is a rare congenital disorder, which can be classified into a non-syndromic form, without extra-adrenal features, and a syndromic form, with such features. Despite biochemical and molecular genetic evaluation, etiologic diagnosis cannot be performed in many patients with adrenal hypoplasia. CASE PRESENTATION: The patient in this case was a boy born at 31 weeks of gestation with a weight of 882 g (< 3rd percentile) to non-consanguineous parents...
March 5, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29446975/primary-adrenal-insufficiency-due-to-hereditary-apolipoprotein-ai-amyloidosis-endocrine-involvement-beyond-hypogonadism
#19
Adriana Pané, Sabina Ruiz, Aida Orois, Daniel Martínez, Mattia Squarcia, Lydia Sastre, Pablo Ruiz, Joan Caballería, Mireia Mora, Felicia A Hanzu, Irene Halperin
Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with liver, kidney, laryngeal, skin and myocardial involvement. Although primary hypogonadism (PH) is considered almost pathognomonic of this disease, until now, primary adrenal insufficiency (PAI) has not been described as a common clinical feature. Here, we report the first kindred with AApoAI amyloidosis in which PAI is well-documented...
February 15, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29426854/association-between-plasma-cortisol-and-death-or-vasopressor-refractory-hypotension-in-preterm-neonates-a-prospective-cohort-study
#20
Monisha Rameshbabu, Venkataseshan Sundaram, Naresh Sachdeva, Rama Walia, Shiv Sajan Saini, Sourabh Dutta
OBJECTIVES: Temporal relation between adrenal insufficiency and hypotension is poorly understood. We examined the association between basal and post-stimulation cortisol and death or vasopressor refractory hypotension in preterm neonates. STUDY DESIGN: Prospective cohort study in ≤30 weeks' and/or <1,250 g weight. Primary outcome-composite of death or vasopressor refractory hypotension by day 14 of life. Plasma cortisol levels were measured at 24-36 h (T1), 72-84 h (T2) and 10 days (T3), and post-stimulation cortisol at T1 and later at T2 and T3 if the adrenal response was inadequate earlier...
February 9, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
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