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"Adrenal Insufficiency"

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https://www.readbyqxmd.com/read/28919626/addison-s-disease-a-diagnostic-dilemma
#1
S Afroz, S Bain
Adrenal insufficiency is a rare disease, but is life threatening when overlooked. Addison's disease may be an acquired form of adrenal insufficiency due to the destruction or dysfunction of the adrenal cortex. It affects both glucocorticoid and mineralocorticoid function. Main presenting symptoms of Addison's disease such as fatigue, anorexia, vomiting and convulsion often mimics central nervous system (CNS) infections. We describe a case of Addison's disease who was initially misdiagnosed as a case of meningo-encephalitis subsequently renal tubular acidosis and finally Addison's disease...
July 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28919607/impact-of-adrenocortical-insufficiency-on-clinical-parameters-in-haemodynamically-stable-cirrhotic-patients-with-ascites
#2
H Ahmed, M R Karim, R K Paul, M Chowdhury, M S Alam, A Saha, F Rahman, M A Rouf
Cirrhosis has many complications regardless of the aetiology. Complications include splenomegaly, ascites, hepatic encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome and hepatocellular carcinoma and also linked to abnormalities in the endocrine system, including abnormal sex hormone metabolism, thyroid disease, osteoporosis, and, most recently identified, adrenal insufficiency. This prospective cohort study was done to evaluate the impact of adrenocortical insufficiency on clinical parameters in haemodynamically stable cirrhotic patients with ascites and had been performed at the inpatient of GHPD Department, BIRDEM, Dhaka, Bangladesh from April 2011 to March 2012...
July 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28916121/controversies-in-corticosteroid-use-for-sepsis
#3
Brit Long, Alex Koyfman
BACKGROUND: Severe sepsis and septic shock are potentially deadly conditions managed in the emergency department (ED). Management centers on source control, fluid resuscitation, broad-spectrum antimicrobials, and vasopressors as needed. The use of corticosteroids is controversial. OBJECTIVE: To evaluate the evidence behind corticosteroid therapy in patients with septic shock. DISCUSSION: Septic shock is associated with severe mortality and morbidity...
September 12, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28915118/delayed-diagnosis-of-proopiomelanocortin-pomc-deficiency-with-type-1-diabetes-in-a-9-year-old-girl-and-her-infant-sibling
#4
Elif Ozsu, Allison Bahm
BACKGROUND: Proopiomelanocortin (POMC) protein, encoded by the POMC gene, is the precursor of adrenocorticotropic hormone (ACTH) that is released from the anterior pituitary gland. Homozygous mutations in the POMC gene is associated with hyperphagia, severe and early-onset obesity, adrenal insufficiency, hypopigmentation of the skin and red hair. CASE PRESENTATION: A 9-year-old girl from a consanguineous family of Iraqi origin was diagnosed with type 1 diabetes...
September 15, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28913704/impact-of-etiology-age-and-gender-on-onset-and-severity-of-hyponatremia-in-patients-with-hypopituitarism-retrospective-analysis-in-a-specialised-endocrine-unit
#5
Dragana Miljic, Mirjana Doknic, Marko Stojanovic, Marina Nikolic-Djurovic, Milan Petakov, Vera Popovic, Sandra Pekic
BACKGROUND: Hyponatremia can unmask hypopituitarism and secondary adrenal insufficiency. This is important, since the need to screen for steroid deficiency, in patients with hyponatremia is often neglected. PATIENTS AND METHODS: In a retrospective study, twenty-five patients (13f/12m, age 58.9 ± 18.6 years) with hyponatremia (119.7 ± 10.5 mmol/L) were identified among 260 in-patients treated for hypopituitarism in our specialized endocrine unit, over the last decade...
September 14, 2017: Endocrine
https://www.readbyqxmd.com/read/28911151/expanding-the-phenotypic-and-genotypic-landscape-of-autoimmune-polyendocrine-syndrome-type-1
#6
Elizaveta M Orlova, Leila S Sozaeva, Maria A Kareva, Bergithe E Oftedal, Anette S B Wolff, Lars Breivik, Ekaterina Y Zakharova, Olga N Ivanova, Olle Kämpe, Ivan I Dedov, Per M Knappskog, Valentina A Peterkova, Eystein S Husebye
Context: Autoimmune polyendocrine syndrome type 1 (APS-1) is a rare monogenic autoimmune disease caused by mutations in the autoimmune regulator (AIRE) gene and characterized by chronic mucocutaneous candidiasis, hypoparathyroidism, and primary adrenal insufficiency. Comprehensive characterizations of large patient cohorts are rare. Objective: To perform an extensive clinical, immunological, and genetic characterization of a large nationwide Russian APS-1 cohort...
September 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28898882/hospitalisation-in-children-with-adrenal-insufficiency-and-hypopituitarism-is-there-a-differential-burden-between-boys-and-girls-and-between-age-groups
#7
R Louise Rushworth, Georgina L Chrisp, Benjamin Dean, Henrik Falhammar, David J Torpy
BACKGROUND/AIMS: To determine the burden of hospitalisation in children with adrenal insufficiency (AI)/hypopituitarism in Australia. METHODS: A retrospective study of Australian hospitalisation data. All admissions between 2001 and 2014 for patients aged 0-19 years with a principal diagnosis of AI/hypopituitarism were included. Denominator populations were extracted from national statistics datasets. RESULTS: There were 3,779 admissions for treatment of AI/hypopituitarism in patients aged 0-19 years, corresponding to an average admission rate of 48...
September 12, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28895702/-x-linked-adrenoleukodystrophy-a-report-of-three-cases-the-importance-of-early-diagnosis
#8
Marta López Úbeda, Antonio de Arriba Muñoz, Marta Ferrer Lozano, José I Labarta Aizpún, María C García Jiménez
X-linked adrenoleukodystrophy is the most common peroxisomal disorder. This disease is caused by a defect in the ABCD1 gen. Saturated very long chain fatty acids are accumulated in serum, adrenal cortex and central nervous system white matter. The clinical spectrum is characterized by progressive neurological dysfunction and adrenal insufficiency with a devastating prognosis. We report a first case of X-linked adrenoleukodystrophy with fatal evolution which identified two asymptomatic family members and established a preventive treatment...
October 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28895486/iatrogenic-adrenal-insufficiency-secondary-to-combination-therapy-with-elvitegravir-cobicistat-tenofovir-disoproxil-fumarate-emtricitabine-and-interlaminar-triamcinolone-injection-in-an-aids-patient
#9
Chanie Wassner, Sutapa Maiti, Kurt Kodroff, Henry Cohen
We report the first identified case of suspected iatrogenic adrenal insufficiency after an interlaminar injection of triamcinolone acetonide while on concomitant Stribild (elvitegravir 150 mg/cobicistat 150 mg/tenofovir disoproxil fumarate 300 mg/emtricitabine 200 mg [EVG/c/TDF/FTC]). A 49-year-old female with HIV on EVG/c/TDF/FTC therapy presented to our endocrinology clinic to be evaluated for suspected Cushing syndrome. Prior to presentation, the patient had been given 2 interlaminar spinal injections of triamcinolone...
January 1, 2017: Journal of the International Association of Providers of AIDS Care
https://www.readbyqxmd.com/read/28893233/clinical-characteristics-of-adrenal-crisis-in-adult-population-with-and-without-predisposing-chronic-adrenal-insufficiency-a-retrospective-cohort-study
#10
Masahiro Iwasaku, Maki Shinzawa, Shiro Tanaka, Kimihiko Kimachi, Koji Kawakami
BACKGROUND: Adrenal crisis (AC) occurs in various clinical conditions but previous epidemiological studies in AC are limited to chronic adrenal insufficiency (AI) and sepsis. The aim of this study was to investigate characteristics of AC patients, including predisposing diseases and to describe candidate risk factors for AC such as comorbidities and glucocorticoid (GC) therapy. METHODS: We conducted a retrospective cohort study using a claims database on 7.4 million patients from 145 acute care hospitals between January 1, 2003 and April 30, 2014...
September 11, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28877961/adrenal-insufficiency-is-seen-in-more-than-one-third-of-patients-during-ongoing-low-dose-prednisolone-treatment-for-rheumatoid-arthritis
#11
Stina Willemoes Borresen, Marianne Klose, Bo Baslund, Åse Krogh Rasmussen, Linda Hilsted, Lennart Friis-Hansen, Henning Locht, Annette Hansen, Merete Lund Hetland, Magnus Christian Lydolph, Ulla Feldt-Rasmussen
OBJECTIVE: Patients receiving long-term glucocorticoid treatment are at risk of developing adrenal insufficiency during treatment. We investigated the prevalence of prednisolone-induced adrenal insufficiency in the particular clinical situation where patients receive ongoing low-dose (5 mg/day) prednisolone treatment, a dose by itself too low to cover glucocorticoid needs during stress. DESIGN AND METHODS: Cross-sectional study in 42 patients with rheumatoid arthritis (29 women, aged 36-86 years) treated with 5 mg prednisolone/day, who had received prednisolone for ≥6 months (median: 66, range: 6-444 months)...
October 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28875708/fkbp5-methylation-as-a-possible-marker-for-cortisol-state-and-transient-cortisol-exposure-in-healthy-human-subjects
#12
Britta K Winkler, Hendrik Lehnert, Henrik Oster, Henriette Kirchner, Birgit Harbeck
Current glucocorticoid replacement regimens, in adrenal insufficiency, fail to mimic the physiological cortisol secretion, thereby fostering serious side effects. AIM: To experimentally evaluate the impact of CpG methylation within the FKBP5 gene as a possible short- and long-term marker for cortisol exposure in humans. MATERIALS & METHODS: An ACTH-stimulation test was carried out and methylation status of the FKBP5 gene in leukocytes was determined. RESULTS: A negative correlation between basal levels of methylation and serum cortisol was observed...
September 6, 2017: Epigenomics
https://www.readbyqxmd.com/read/28874394/looking-beyond-the-thyroid-advances-in-understanding-of-pheochromocytoma-and-hyperparathyroidism-phenotypes-in-men2-and-of-non-men2-familial-forms
#13
Carole Guerin, Pauline Romanet, David Taieb, Thierry Brue, André Lacroix, Frederic Sebag, Anne Barlier, Frederic Castinetti
Over the last years, the knowledge of MEN2 and non MEN2 familial forms of pheochromocytoma (PHEO) has increased. In MEN2, PHEO is the second most frequent disease: the penetrance and age at diagnosis depend on the mutation of RET. Given the prevalence of bilateral PHEO (50% by age 50), adrenal sparing surgery, aimed at sparing a part of the adrenal cortex to avoid adrenal insufficiency, should be systematically considered in patients with bilateral PHEO. Non MEN2 familial forms of PHEO now include more than 20 genes: however, only small phenotypic series have been reported, suggesting that phenotypic features of isolated hereditary PHEO must be better explored, and follow-up series are needed to better understand the outcome of patients carrying mutations of these genes...
September 5, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28871578/secondary-adrenal-insufficiency-following-nivolumab-therapy-in-a-patient-with-metastatic-renal-cell-carcinoma
#14
Toshiro Seki, Atsushi Yasuda, Masayuki Oki, Natsumi Kitajima, Atsushi Takagi, Nobuyuki Nakajima, Akira Miyajima, Masafumi Fukagawa
Currently, nivolumab (an anti-programmed cell death-1 receptor monoclonal antibody) is available for many types of advanced cancers in Japan. However, there have been few detailed case reports about endocrine-related adverse events of this therapy. Here, we report a patient with metastatic renal cell carcinoma who presented with secondary adrenal insufficiency following nivolumab therapy. Endocrinological assessment by rapid adrenocorticotropic hormone (ACTH) and corticotropin-releasing hormone (CRH) tests revealed that the patient's disorder was a secondary adrenal insufficiency due to pituitary dysfunction...
September 20, 2017: Tokai Journal of Experimental and Clinical Medicine
https://www.readbyqxmd.com/read/28863857/revised-consensus-statement-on-the-preventive-and-symptomatic-care-of-patients-with-leukodystrophies
#15
REVIEW
Laura A Adang, Omar Sherbini, Laura Ball, Miriam Bloom, Anil Darbari, Hernan Amartino, Donna DiVito, Florian Eichler, Maria Escolar, Sarah H Evans, Ali Fatemi, Jamie Fraser, Leslie Hollowell, Nicole Jaffe, Christopher Joseph, Mary Karpinski, Stephanie Keller, Ryan Maddock, Edna Mancilla, Bruce McClary, Jana Mertz, Kiley Morgart, Thomas Langan, Richard Leventer, Sumit Parikh, Amy Pizzino, Erin Prange, Deborah L Renaud, William Rizzo, Jay Shapiro, Dean Suhr, Teryn Suhr, Davide Tonduti, Jacque Waggoner, Amy Waldman, Nicole I Wolf, Ayelet Zerem, Joshua L Bonkowsky, Genevieve Bernard, Keith van Haren, Adeline Vanderver
Leukodystrophies are a broad class of genetic disorders that result in disruption or destruction of central myelination. Although the mechanisms underlying these disorders are heterogeneous, there are many common symptoms that affect patients irrespective of the genetic diagnosis. The comfort and quality of life of these children is a primary goal that can complement efforts directed at curative therapies. Contained within this report is a systems-based approach to management of complications that result from leukodystrophies...
August 20, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28859919/nationwide-analysis-of-adrenocortical-carcinoma-reveals-higher-perioperative-morbidity-in-functional-tumors
#16
Punam P Parikh, Gustavo A Rubio, Josefina C Farra, John I Lew
BACKGROUND: Current adrenalectomy outcomes for functional adrenocortical carcinoma (ACC) remain unclear. This study examines nationwide in-hospital post-adrenalectomy outcomes for ACC. METHODS: A retrospective analysis of the Nationwide Inpatient Sample database (2006-2011) to identify unilateral adrenalectomy patients for functional or nonfunctional ACC was performed. Patient demographics, comorbidities and postoperative outcomes were evaluated by t-test, Chi-square and multivariate regression...
August 25, 2017: American Journal of Surgery
https://www.readbyqxmd.com/read/28856224/an-analysis-of-the-relationship-between-serum-cortisol-and-serum-sodium-in-routine-clinical-patients
#17
Eleanor McLaughlan, Julian H Barth
OBJECTIVES: Adrenal insufficiency is an uncommon cause of hyponatraemia that should not be overlooked due to the severe consequences of an Addisonian crisis. Using the laboratory database of a large teaching hospital, we have explored the relationship between serum sodium and serum cortisol, and have estimated the frequency of hypoadrenalism in severely hyponatraemic patients. DESIGN AND METHODS: Data were gathered over a 23 month period from the Laboratory Information Management System at the Leeds Teaching Hospitals NHS Trust for instances where serum sodium and cortisol had been measured on a single sample...
August 2017: Pract Lab Med
https://www.readbyqxmd.com/read/28835258/acute-hypopituitarism-associated-with-periorbital-swelling-and-cardiac-dysfunction-in-a-patient-with-pituitary-tumor-apoplexy-a-case-report
#18
Nobumasa Ohara, Yuichiro Yoneoka, Yasuhiro Seki, Katsuhiko Akiyama, Masataka Arita, Kazumasa Ohashi, Kazuo Suzuki, Toshinori Takada
BACKGROUND: Pituitary tumor apoplexy is a rare clinical syndrome caused by acute hemorrhage or infarction in a preexisting pituitary adenoma. It typically manifests as an acute episode of headache, visual disturbance, mental status changes, cranial nerve palsy, and endocrine pituitary dysfunction. However, not all patients present with classical symptoms, so it is pertinent to appreciate the clinical spectrum of pituitary tumor apoplexy presentation. We report an unusual case of a patient with pituitary tumor apoplexy who presented with periorbital edema associated with hypopituitarism...
August 24, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28834559/improving-the-pre-hospital-safety-of-steroid-dependent-patients-in-northern-england-a-hospital-initiated-ambulance-service-registration-pathway
#19
Anna L Mitchell, Kerri Devine, Vikram Lal, Paul Galloway, Matthew House, Katherine White, Julie Watson, Steve Parry, Margaret Miller, Margaret Morris, R Andy James, Petros Perros, Simon Hs Pearce, Richard Quinton
Acute adrenal crisis is a life-threatening medical emergency requiring immediate recognition and treatment. Among individuals with primary adrenal insufficiency it has a frequency of 6-8 per 100 patient-years(1) . Unfortunately, delays in diagnosis and management of acute adrenal crisis are commonplace - even in patients with documented steroid-dependency - constituting an avoidable source of patient harm. This article is protected by copyright. All rights reserved.
August 23, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28828107/nontraumatic-adrenal-hemorrhage-the-adrenal-stress
#20
Marco Di Serafino, Rosa Severino, Valeria Coppola, Matilde Gioioso, Rosario Rocca, Francesco Lisanti, Enrico Scarano
Bilateral adrenal hemorrhage is a rare condition, which is burdened by potentially life-threatening consequences related to the development of acute adrenal insufficiency. Despite treatment with stress-dose glucocorticoids, a mortality rate of 15% has been reported, which varies according to the severity of underlying predisposing illness and could be much more higher if the adrenal insufficiency is not promptly recognized. An early diagnosis is crucial, though, because of nonspecific clinical and laboratory findings, adrenal hemorrhage is rarely suspected...
September 2017: Radiology case reports
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