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Secondary Adrenal Insufficiency

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https://www.readbyqxmd.com/read/28542805/downregulation-of-cholesteryl-ester-transfer-protein-by-glucocorticoids-a-randomized-study-on-hdl
#1
Jorien Werumeus Buning, Lidya G Dimova, Frank G Perton, Uwe J F Tietge, André P van Beek, Robin P F Dullaart
BACKGROUND: High density lipoprotein (HDL) cholesterol is not decreased in hypercortisolism despite high triglycerides, which may be ascribed to effects on the cholesteryl ester transfer protein (CETP) pathway. We explored if CETP mRNA expression is modulated by glucocorticoid treatment in vitro. Effects of doubling the hydrocortisone (HCT) replacement dose on plasma CETP activity and HDL characteristics were tested in patients with secondary adrenal insufficiency. MATERIALS AND METHODS: Human THP-1 macrophages were incubated with corticosterone in vitro in the presence or absence of a liver X receptor (LXR) agonist, followed by determination of CETP mRNA levels by quantitative real-time PCR...
May 24, 2017: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28521880/pharmacokinetics-of-oral-hydrocortisone-results-and-implications-from-a-randomized-controlled-trial
#2
Jorien Werumeus Buning, Daan J Touw, Pauline Brummelman, Robin P F Dullaart, Gerrit van den Berg, Melanie M van der Klauw, Jasper Kamp, Bruce H R Wolffenbuttel, André P van Beek
CONTEXT AND OBJECTIVE: This study aimed at comparing pharmacokinetics of two different doses of hydrocortisone (HC) in patients with secondary adrenal insufficiency (SAI). DESIGN, SETTING AND PATIENTS: Forty-six patients with SAI participated in this randomized double-blind crossover study. INTERVENTION: Patients received two different doses of HC (0.2-0.3mg HC/kg body weight/day and 0.4-0.6mg HC/kg body weight/day). MAIN OUTCOME MEASURES: One- and two-compartment population models for plasma free cortisol, plasma total cortisol and salivary cortisol were parameterized...
June 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28500832/combined-pituitary-hormone-deficiency-in-a-girl-with-48-xxxx-and-rathke-s-cleft-cyst
#3
Surabhi Uppal, Youn Hee Jee, Marissa Lightbourne, Joan C Han, Constantine A Stratakis
BACKGROUND: Tetrasomy X is a rare chromosomal aneuploidy seen in girls, associated with facial dysmorphism, premature ovarian insufficiency and intellectual disability. A Rathke's cleft cyst (RCC) is a remnant of Rathke's pouch which may cause multiple pituitary hormone deficiencies by exerting pressure on the pituitary gland in the sella. METHODS/RESULTS: The patient was diagnosed with tetrasomy X by karyotyping during infancy. Brain MRI and multiple endocrine stimulation tests revealed RCC and combined pituitary hormone deficiency (growth hormone deficiency, secondary adrenal insufficiency and central hypothyroidism) likely due to RCC...
January 2017: Hormones: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28472507/a-case-report-of-hypoglycemia-and-hypogammaglobulinemia-david-syndrome-in-a-patient-with-a-novel-nfkb2-mutation
#4
Rayhan A Lal, Laura K Bachrach, Andrew R Hoffman, Jingga Inlora, Shannon Rego, Michael P Snyder, David B Lewis
Context: DAVID syndrome (Deficient Anterior pituitary with Variable Immune Deficiency) is a rare disorder in which children present with symptomatic ACTH deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, termed common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as either a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID...
May 3, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28443262/a-rare-association-of-central-hypothyroidism-and-adrenal-insufficiency-in-a-boy-with-williams-beuren-syndrome
#5
Devi Dayal, Dinesh Giri, Senthil Senniappan
Primary hypothyroidism related to morphological and volumetric abnormalities of the thyroid gland is one of the commonest of several endocrine dysfunctions in Williams-Beuren syndrome (WBS). We report a 10-month-old boy with WBS who presented with central hypothyroidism. During the neonatal period, he had prolonged jaundice, feeding difficulties and episodes of colic that continued during early infancy. Additionally, there was slowing of growth and mild developmental delay. He underwent surgical repair for supravalvular aortic stenosis at 6 months of age...
March 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28434547/stress-dose-corticosteroid-versus-placebo-in-neonatal-cardiac-operations-a-randomized-controlled-trial
#6
Pertti K Suominen, Juho Keski-Nisula, Tiina Ojala, Paula Rautiainen, Timo Jahnukainen, Johanna Hästbacka, Pertti J Neuvonen, Olli Pitkänen, Jussi Niemelä, Anu Kaskinen, Jukka Salminen, Risto Lapatto
BACKGROUND: Corticosteroids can improve the hemodynamic status of neonates with postoperative low cardiac output syndrome after cardiac operations. This study compared a prophylactically administered stress-dose corticosteroid (SDC) regimen against placebo on inflammation, adrenocortical function, and hemodynamic outcome. METHODS: Forty neonates undergoing elective open heart operations were randomized into two groups. The SDC group received perioperatively 2 mg/kg methylprednisolone, and 6 hours after the operation, a hydrocortisone infusion (0...
April 20, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28422753/steroid-metabolome-analysis-reveals-prevalent-glucocorticoid-excess-in-primary-aldosteronism
#7
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/28421712/gastric-cancer-with-pituitary-metastasis-presenting-as-symptomatic-secondary-adrenal-insufficiency-case-report
#8
Xue Lin Dou, Na Zhou, Yu Lin Mai, Mei Guan, Zhao Sun, Xin Gao, Chun Mei Bai
Gastric cancer (GC) is one of the leading causes of cancer-related deaths worldwide, and Eastern Asia has the highest incidence.(1,2) The overall prognosis remains poor for advanced GC.(3).
April 19, 2017: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28351092/national-german-audit-of-diagnosis-treatment-and-teaching-in-secondary-adrenal-insufficiency
#9
Stephan Petersenn, Jürgen Honegger, Marcus Quinkler
No abstract text is available yet for this article.
March 28, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28329633/comorbidities-of-hospitalized-pemphigus-patients-in-the-united-states
#10
Derek Y Hsu Y Hsu
We sought to determine the comorbid health conditions and inpatient mortality associated with pemphigus in a U.S. inpatient cohort. The 2002-2012 Nationwide Inpatient Sample, which contains a representative 20% stratified sample of all inpatient hospitalizations in the US, was analyzed. Comorbidities were determined through ICD-9-CM codes. Survey weighted multivariate logistic regression models controlling for demographic factors were constructed to determine the association of pemphigus with various comorbidities...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28326739/a-case-of-pituitary-stalk-interruption-syndrome-with-intermittent-seizures-as-the-first-presentation
#11
Juan Li, Hongwei Jia, Anindita Chakraborty, Zhihong Gao
Pituitary stalk interruption syndrome (PSIS) is a congenital disease with isolated growth hormone deficiency (GHD) or multiple anterior pituitary hormone deficiencies (MPHD). The typical clinical manifestations of PSIS are growth retardation, hypoglycemia or delayed pubertal development. However, few reports showed cases of PSIS were diagnosed with acute epileptic seizures accompanied by hyponatremia. Here, we report an 18-year-old female presenting with episodes of intermittent seizures for 13 years. The electrolyte examination on many occasions has shown hyponatremia, even as low as 99...
December 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28130352/clinical-features-and-practice-patterns-of-treatment-for-adrenal-crisis-a-nationwide-cross-sectional-study-in-japan
#12
Yosuke Ono, Sachiko Ono, Hideo Yasunaga, Hiroki Matsui, Kiyohide Fushimi, Yuji Tanaka
CONTEXT: Adrenal crisis is an endocrine emergency that requires prompt diagnosis and treatment. However, the clinical features and practice patterns of treatment for adrenal crisis are not completely understood. OBJECTIVE: To investigate patient characteristics, comorbidities and treatments of adrenal crisis. METHODS: We conducted a cross-sectional study of patients who received intravenous glucocorticoids for adrenal crisis at admission from 1 July 2007 to 31 March 2014, using a national inpatient database in Japan...
March 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28119833/large-dose-glucocorticoid-induced-secondary-adrenal-insufficiency-in-spinal-cord-injury
#13
Soo Ho Park, Kang Hee Cho
OBJECTIVE: To investigate the incidence of adrenal insufficiency (AI) in patients with spinal cord injury (SCI) with symptoms similar to those of AI and to assess the relevance of AI and large-dose glucocorticoids in SCI. METHODS: The medical records of 228 patients who were admitted to the rehabilitation center after SCI from January 2014 to January 2016 were reviewed retrospectively. Twenty-nine of 228 patients had persistent symptoms suspicious for AI despite continuous care for more than 4 weeks...
December 2016: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/28073054/adrenal-failure-due-to-bilateral-adrenal-metastasis-of-rectal-cancer-a-case-report
#14
Yuki Imaoka, Fumito Kuranishi, Yoshiteru Ogawa, Hiroshi Okuda, Masahiro Nakahara
INTRODUCTION: It is rare for a patient to present with adrenal insufficiency secondary to bilateral adrenal metastases from a malignant colorectal tumor. CASE PRESENTATION: An 82-year-old Japanese man presented to our hospital with high fever and malaise. He was receiving oral chemotherapy for the treatment of rectal cancer with multiple metastases. Computed tomography showed new bilateral adrenal gland metastases. A rapid adrenocorticotropic hormone (ACTH) test showed adrenal insufficiency...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28063163/immediate-versus-modified-release-hydrocortisone-in-mitotane-treated-patients-with-adrenocortical-cancer
#15
Marianne Weigel, Stefanie Hahner, Mark Sherlock, Amar Agha, Lucy Ann Behan, Paul M Stewart, Wiebke Arlt, Daniela Beier, Kathrin Frey, Kathrin Zopf, Marcus Quinkler
OBJECTIVE: Mitotane induces hepatic CYP3A4 activity, resulting in accelerated cortisol inactivation, and also increases cortisol binding globulin (CBG). Therefore, higher hydrocortisone doses are required in patients with adrenocortical cancer (ACC) on mitotane treatment. Modified release hydrocortisone has not been used in mitotane-treated ACC patients yet. AIM: Case series to compare serum cortisol, calculated free serum cortisol and ACTH levels in ACC patients on mitotane treatment with immediate and modified release hydrocortisone...
April 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28033660/wide-spectrum-of-nr5a1-related-phenotypes-in-46-xy-and-46-xx-individuals
#16
REVIEW
Sorahia Domenice, Aline Zamboni Machado, Frederico Moraes Ferreira, Bruno Ferraz-de-Souza, Antonio Marcondes Lerario, Lin Lin, Mirian Yumie Nishi, Nathalia Lisboa Gomes, Thatiana Evelin da Silva, Rosana Barbosa Silva, Rafaela Vieira Correa, Luciana Ribeiro Montenegro, Amanda Narciso, Elaine Maria Frade Costa, John C Achermann, Berenice Bilharinho Mendonca
Steroidogenic factor 1 (NR5A1, SF-1, Ad4BP) is a transcriptional regulator of genes involved in adrenal and gonadal development and function. Mutations in NR5A1 have been among the most frequently identified genetic causes of gonadal development disorders and are associated with a wide phenotypic spectrum. In 46,XY individuals, NR5A1-related phenotypes may range from disorders of sex development (DSD) to oligo/azoospermia, and in 46,XX individuals, from 46,XX ovotesticular and testicular DSD to primary ovarian insufficiency (POI)...
December 2016: Birth Defects Research. Part C, Embryo Today: Reviews
https://www.readbyqxmd.com/read/28018694/anterior-hypopituitarism-and-treatment-response-in-hunter-syndrome-a-comparison-of-two-patients
#17
Munier A Nour, Paola Luca, David Stephure, Xing-Chang Wei, Aneal Khan
Hypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Endocrine testing revealed growth hormone (GH) deficiency, secondary adrenal insufficiency, and tertiary hypothyroidism. An improvement in growth velocity with hormone replacement (GH, thyroxine, and corticosteroid) was seen; however, final adult height remained compromised...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28013434/the-effect-of-local-injection-of-methylprednisolone-acetate-on-the-hypothalamic-pituitary-adrenal-axis-among-patients-with-greater-trochanteric-pain-syndrome
#18
George Habib, Shada Elias, Muhanned Abu-Elhaija, Fahed Sakas, Fadi Khazin, Suheil Artul, Adel Jabbour, Haneen Jabaly-Habib
Greater trochanteric pain syndrome (GTPS) is a common clinical entity for which the most effective treatment is local corticosteroid injection (LCI). There are no studies on the effect of LCI among patients with GTPS on the hypothalamic-pituitary-adrenal axis. The present study recruited nonselected patients diagnosed with GTPS. After consenting, participants received low dose (1 μg) of adrenocorticotropin hormone (ACTH) stimulation test at 09:00. Immediately following the test, participants received a LCI of 80 mg of methylprednisolone acetate at the greater trochanteric region...
December 24, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27995051/bilateral-adrenal-histoplasmosis-in-an-immunocompetent-man-from-texas
#19
Colin J Rog, Daniel G Rosen, Francis H Gannon
Disseminated histoplasmosis affecting the adrenal gland(s) of immunocompetent adults is a very rare infection. Here, we present a case of bilateral adrenal histoplasmosis in an immunocompetent, 62-year-old gentleman from Texas along with a brief review of the published literature. Given the risk of patient decompensation secondary to adrenal insufficiency and the wide availability of effective treatments, adrenal histoplasmosis must be considered even in immunocompetent adults who acquire adrenal masses.
December 2016: Medical Mycology Case Reports
https://www.readbyqxmd.com/read/27900224/perioperative-severe-hypotension-in-a-patient-with-multiple-endocrine-neoplasia-type-iib-and-bilateral-adrenalectomies-time-to-review-the-evidence-for-stress-dose-steroids
#20
Jens Tan, Acsa Zavala, Katherine B Hagan, Antoinette Van Meter, Uduak Ursula Williams, Wei Zhang, Pascal Owusu-Agyemang
Multiple endocrine neoplasia type IIb (MEN IIb) is an endocrine disorder which can manifest with tumors such as pheochromocytomas and neuromas. We present the case of a patient with MEN IIb, after bilateral adrenalectomies, on maintenance steroid replacement, who underwent a neuroma resection and developed severe hypotension. There is persistent controversy regarding the general administration of perioperative "stress dose" steroids for patients with adrenal insufficiency. While the most recent literature suggests that stress dose steroids are unnecessary for secondary adrenal insufficiency, the rarer form of primary adrenal insufficiency always requires supplemental steroids, specifically hydrocortisone, when undergoing surgical procedures...
2016: Case Reports in Anesthesiology
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