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"Adrenal Insufficiency"

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https://www.readbyqxmd.com/read/28446858/a-case-of-autoimmune-polyglandular-syndrome-ype-2-associated-with-atypical-form-of-scleromyxedema
#1
Oleksander Prylutskyi, Olga Prylutska, Anatoliy Degonskyi, Kseniia Tkachenko
BACKGROUND: Autoimmune polyglandular syndrome type 2 represents an uncommon endocrine disorder composed by Addison's disease with autoimmune thyroid disease (Schmidt's syndrome) and/or type 1 diabetes mellitus. Scleromyxedema is a rare progressive cutaneous mucinosis usually associated with systemic involvement and paraproteinemia. To the best of our knowledge, there is no case report of Schmidt's syndrome associated with scleromyxedema. CASE DETAILS: A 34-year-old woman was admitted to Donetsk Clinical Territorial Medical Association due to acute general weakness, reduced vision, dryness of integuments, memory decline, fatigue, weight loss, rash on the face trunk and extremities...
September 2016: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/28446514/autoimmune-polyendocrine-syndrome-type-1-in-an-indian-cohort-a-longitudinal-study
#2
Ghazala Zaidi, Vijayalakshmi Bhatia, Saroj Sahoo, Aditya Narayan Sarangi, Niharika Bharti, Li Zhang, Liping Yu, Daniel Eriksson, Sophie Bensing, Olle Kämpe, Nisha Bhavani, Surender K Yachha, Anil Bhansali, Alok Sachan, Vandana Jain, Nalini Shah, Rakesh Aggarwal, Amita Aggarwal, Muthuswany Srinivasan, Sarita Agarwal, Eesh Bhatia
OBJECTIVE: Autoimmune polyendocrine syndrome type 1 (APS 1) is a rare autosomal recessive disorder characterized by progressive organ-specific autoimmunity. There is scant information on APS1 in ethnic groups other than European Caucasians. We studied clinical aspects and autoimmune regulator (AIRE) gene mutations in a cohort of Indian APS1 patients. DESIGN: Twenty-three patients (19 families) from six referral centres in India, diagnosed between 1996-2016, were followed for [median (range)] 4 (0...
April 26, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28443262/a-rare-association-of-central-hypothyroidism-and-adrenal-insufficiency-in-a-boy-with-williams-beuren-syndrome
#3
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/28434648/nivolumab-in-patients-with-advanced-hepatocellular-carcinoma-checkmate-040-an-open-label-non-comparative-phase-1-2-dose-escalation-and-expansion-trial
#4
Anthony B El-Khoueiry, Bruno Sangro, Thomas Yau, Todd S Crocenzi, Masatoshi Kudo, Chiun Hsu, Tae-You Kim, Su-Pin Choo, Jörg Trojan, Theodore H Welling, Tim Meyer, Yoon-Koo Kang, Winnie Yeo, Akhil Chopra, Jeffrey Anderson, Christine Dela Cruz, Lixin Lang, Jaclyn Neely, Hao Tang, Homa B Dastani, Ignacio Melero
BACKGROUND: For patients with advanced hepatocellular carcinoma, sorafenib is the only approved drug worldwide, and outcomes remain poor. We aimed to assess the safety and efficacy of nivolumab, a programmed cell death protein-1 (PD-1) immune checkpoint inhibitor, in patients with advanced hepatocellular carcinoma with or without chronic viral hepatitis. METHODS: We did a phase 1/2, open-label, non-comparative, dose escalation and expansion trial (CheckMate 040) of nivolumab in adults (≥18 years) with histologically confirmed advanced hepatocellular carcinoma with or without hepatitis C or B (HCV or HBV) infection...
April 20, 2017: Lancet
https://www.readbyqxmd.com/read/28434547/stress-dose-corticosteroid-versus-placebo-in-neonatal-cardiac-operations-a-randomized-controlled-trial
#5
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/28423475/primary-lymphocytic-hypophysitis-clinical-characteristics-and-treatment-of-50-cases-in-a-single-centre-in-china-over-18-years
#6
Shuchang Wang, Linjie Wang, Yong Yao, Feng Feng, Hongbo Yang, Zhiyong Liang, Kan Deng, Hui You, Jian Sun, Bing Xing, Zimeng Jin, Renzhi Wang, Hui Pan, Huijuan Zhu
OBJECTIVE: Primary lymphocytic hypophysitis (LYH) is rare and it is often evaluated in a small case series. This study aimed to describe the diagnosis and treatment of primary LYH in a larger cohort. DESIGN: A retrospective study of the diagnosis and treatment of primary LYH was conducted at Peking Union Medical College Hospital from 1999 to 2016. PATIENTS: Fifty patients (28 histologically-diagnosed and 22 clinically-diagnosed) were eligible for inclusion...
April 19, 2017: Clinical Endocrinology
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/28413626/non-infectious-chemotherapy-associated-acute-toxicities-during-childhood-acute-lymphoblastic-leukemia-therapy
#9
REVIEW
Kjeld Schmiegelow, Klaus Müller, Signe Sloth Mogensen, Pernille Rudebeck Mogensen, Benjamin Ole Wolthers, Ulrik Kristoffer Stoltze, Ruta Tuckuviene, Thomas Frandsen
During chemotherapy for childhood acute lymphoblastic leukemia, all organs can be affected by severe acute side effects, the most common being opportunistic infections, mucositis, central or peripheral neuropathy (or both), bone toxicities (including osteonecrosis), thromboembolism, sinusoidal obstruction syndrome, endocrinopathies (especially steroid-induced adrenal insufficiency and hyperglycemia), high-dose methotrexate-induced nephrotoxicity, asparaginase-associated hypersensitivity, pancreatitis, and hyperlipidemia...
2017: F1000Research
https://www.readbyqxmd.com/read/28398936/endogenous-glucocorticoid-response-to-single-dose-dexamethasone-for-croup-in-children-a-pharmacodynamic-study
#10
Natasha Gill, Natalie Sirizzotti, David Johnson, Gary Joubert, Andrew S Kucey, Alvin Tieu, Brad L Urquhart, Melanie Columbus, Rodrick Lim, Michael Rieder, Shruti Mehrotra, Emily D Hartjes, Naveen Poonai
OBJECTIVES: Dexamethasone is associated with adrenal insufficiency in adults and children with chronic disease. This association has not been studied after single-dose oral dexamethasone, the standard of care for children with croup. We hypothesized that single-dose oral dexamethasone in children with croup is associated with a transient decrease in endogenous glucocorticoids. METHODS: We conducted a prospective, 2-arm, pharmacodynamic study of single-dose oral dexamethasone 0...
April 11, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28395280/triple-a-syndrome-preliminary-response-to-the-antioxidant-n-acetylcysteine-treatment-in-a-child
#11
Maria Candida Barisson Villares Fragoso, Edoarda Vasco de Albuquerque Albuquerque, Ana Luiza de Almeida Cardoso, Paula Waki Lopes da Rosa, Rodrigo Bomeny de Paulo, Maria Heloisa Massola Schimizu, Antonio Carlos Seguro, Marisa Passarelli, Katrin Koehler, Angela Huebner, Madson Q Almeida, Ana Claudia Latronico, Ivo Jorge Prado Arnhold, Berenice Bilharinho Mendonca
INTRODUCTION: Triple A syndrome (AAAS) is a rare autosomal recessive disorder characterized by alacrima, achalasia, ACTH-resistant adrenal insufficiency, autonomic dysfunction, and progressive neurodegeneration. Increased oxidative stress, demonstrated in patients' fibroblasts in vitro, may be a central disease mechanism. N-acetylcysteine protects renal function in patients with kidney injuries associated with increased oxidative stress and improves viability of AAAS-knockdown adrenal cells in vitro...
April 10, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28394668/use-of-cortisol-and-adrenal-weight-at-pediatric-postmortem
#12
Rebecca Morrison, Jansher Khan, Peter Galloway, Jane McNeilly, Syed Faisal Ahmed, Dawn Penman
We studied the relationship between adrenal weight and postmortem cortisol level in cases of infant death, and examined use of these measurements in adrenal insufficiency. We analyzed procurator-fiscal postmortem reports in the West of Scotland over a three year period. Combined adrenal weight was expressed as percentage of total body weight (%TBW). Of 106 cases, median (5(th), 95(th)) %TBW was 0.056 (0.025, 0.23) and median plasma cortisol was 8.4 ug/dl (1.0, 47.1). There was no correlation between %TBW and plasma cortisol (r = 0...
April 10, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28377801/current-best-practice-in-the-management-of-patients-after-pituitary-surgery
#13
REVIEW
Alessandro Prete, Salvatore Maria Corsello, Roberto Salvatori
Sellar and parasellar masses are a common finding, and most of them are treated surgically via transsphenoidal approach. This type of surgery has revolutionized the approach to several hypothalamic-pituitary diseases and is usually effective, and well-tolerated by the patient. However, given the complex anatomy and high density of glandular, neurological and vascular structures in a confined space, transsphenoidal surgery harbors a substantial risk of complications. Hypopituitarism is one of the most frequent sequelae, with central adrenal insufficiency being the deficit that requires a timely diagnosis and treatment...
March 2017: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28362713/relative-adrenal-insufficiency-in-cardiogenic-shock-is-there-a-need-for-action
#14
Josef Briegel, Patrick Möhnle, M Vogeser, Christoph Thiemermann, Peter Radermacher
No abstract text is available yet for this article.
March 30, 2017: Shock
https://www.readbyqxmd.com/read/28361161/steroids-in-traditional-chinese-medicine-what-is-the-evidence
#15
Foon Yin Fung, Yeh Ching Linn
Local healthcare providers often question the possible steroidal activity of traditional Chinese medicine (TCM) herbs or herbal products and implicate them as a cause for adrenal insufficiency or Cushing's syndrome in patients with a history of TCM intake. We conducted a comprehensive database search for evidence of potential glucocorticoid, mineralocorticoid, androgenic or oestrogenic activity of herbs or herbal products. Overall, there are not many herbs whose steroidal activity is well established; among these, most cases were based on preclinical studies...
March 2017: Singapore Medical Journal
https://www.readbyqxmd.com/read/28351092/national-german-audit-of-diagnosis-treatment-and-teaching-in-secondary-adrenal-insufficiency
#16
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/28339821/russell-s-viper-envenomation-acute-hypopituitarism-or-acute-primary-adrenal-insufficiency
#17
A Krishnamurthy, V Y Vishnu
No abstract text is available yet for this article.
February 28, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28339808/russell-s-viper-envenomation-acute-hypopituitarism-or-acute-primary-adrenal-insufficiency
#18
Srinivas Rajagopala, Molly Mary Thabah
No abstract text is available yet for this article.
February 28, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28339047/hypothalamo%C3%A2-hypophysial-system-in-rats-with-autotransplantation-of-the-adrenal-cortex
#19
Nae Takizawa, Susumu Tanaka, Souichi Oe, Taro Koike, Tadashi Matsuda, Hisao Yamada
Patients with bilateral pheochromocytoma often require an adrenalectomy. Autotransplantation of the adrenal cortex is an alternative therapy that could potentially be performed instead of receiving glucocorticoid replacement following adrenalectomy. Adrenal cortex autotransplantation aims to avoid the side effects of long‑term steroid treatment and adrenal insufficiency. Although the function of the hypothalamo‑hypophysial system is critical for patients who have undergone adrenal cortex autotransplantation, the details of that system, with the exception of adrenocorticotropic hormone in the subjects with adrenal autotransplantation, have been overlooked for a long time...
May 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28334791/isolated-acth-deficiency-probably-induced-by-autoimmune-related-mechanism-evoked-with-nivolumab
#20
Keiko Kitajima, Kenji Ashida, Naoko Wada, Ryoko Suetsugu, Yukina Takeichi, Shohei Sakamoto, Hiroshi Uchi, Takamitsu Matsushima, Motoaki Shiratsuchi, Keizo Ohnaka, Masutaka Furue, Masatoshi Nomura
Nivolumab, an anti-programmed death-1 antibody, is a breakthrough treatment for several malignancies. Its specific adverse effects caused by autoimmunity are termed immune-related adverse events, which involve several endocrine dysfunctions. Herein, we report two cases of isolated adrenocorticotropic hormone (ACTH) deficiency induced by nivolumab for the treatment of metastatic malignant melanoma. Case 1 was a 39-year-old man and Case 2 was a 50-year-old woman, both of whom presented with progressive melanoma...
February 18, 2017: Japanese Journal of Clinical Oncology
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