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Primary adrenal insufficiency

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https://www.readbyqxmd.com/read/28624161/clinical-follow-up-of-the-first-sf-1-insufficient-female-patient
#1
Karine Gerster, Anna Biason-Lauber, Eugen J Schoenle
OBJECTIVE: Steroidogenic factor 1 (SF-1/NR5A1) plays a crucial role in regulating adrenal development, gonad determination and differentiation, and in the hypothalamic-pituitary control of reproduction and metabolism. In men (46, XY), it is known that mutations in SF-1/NR5A1 gene cause a wide phenotypic spectrum with variable degrees of undervirilization. In recent years, the role of SF-1 in the ovarian function was increasingly discussed and alterations in the gene were related to primary ovarian insufficiency...
June 14, 2017: Annales D'endocrinologie
https://www.readbyqxmd.com/read/28614010/diagnostic-accuracy-of-basal-cortisol-level-to-predict-adrenal-insufficiency-in-cosyntropin-testing-results-from-an-observational-cohort-study-with-804-patients
#2
Tristan Struja, Leonie Briner, Aline Meier, Alexander Kutz, Esther Mundwiler, Andreas Huber, Beat Mueller, Luca Bernasconi, Philipp Schuetz
CONTEXT: ACTH stimulation testing is the current standard for the assessment of primary and secondary adrenal insufficiency (AI). We aimed to investigate the value of basal cortisol level for prediction of AI. METHODS: We retrospectively analyzed 804 consecutive patients who had high-dose (250μg, HDT) or low-dose (1μg, LDT) ACTH testing as part of their diagnostic work-up. Site-specific cut-off levels for AI were <550 in HDT and <500nmol/L in LDT, respectively...
June 14, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28583942/management-of-endocrine-disease-risk-of-overtreatment-of-patients-with-adrenal-insufficiency-current-and-emerging-aspects
#3
Gherardo Mazziotti, Anna Maria Formenti, Stefano Frara, Elisa Roca, Pietro Mortini, Alfredo Berruti, Andrea Giustina
The effects of long-term replacement therapy of adrenal insufficiency (AI) are still a matter of controversy. In fact, the established glucocorticoid replacement regimens do not completely mirror the endogenous hormonal production and the monitoring of AI treatment may be a challenge for the lack of reliable biochemical markers. Consequently, several AI patients may be exposed to relative glucocorticoid excess that, even if mild, may potentially lead to development of chronic complications, such as diabetes mellitus, dyslipidemia, hypertension and fragility fractures with consequent impaired QoL and increased mortality risk...
June 5, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28572228/adrenal-insufficiency-recognition-and-management
#4
Agnieszka Pazderska, Simon Hs Pearce
Adrenal insufficiency is characterised by inadequate -glucocorticoid production owing to destruction of the adrenal cortex or lack of adrenocorticotropic hormone stimulation. In primary adrenal insufficiency, lack of mineralocorticoids is also a feature. Patients can present with an insidious onset of symptoms, or acutely in adrenal crisis, which requires prompt recognition and treatment. Chronic glucocorticoid therapy is the most common cause of adrenal insufficiency. The -diagnosis of adrenal insufficiency is made by -demonstrating low basal and/or stimulated serum cortisol and should be -followed by appropriate investigations to establish the -underlying aetiology...
June 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28571201/primary-hypothyroid-and-secondary-adrenal-insufficiency-searching-the-missing-link
#5
Subhodip Pramanik, Sayantani Chandra, Pradip Mukherjee, Subhankar Chowdhury
No abstract text is available yet for this article.
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28567296/thyrotoxicosis-leading-to-adrenal-crises-reveals-primary-bilateral-adrenal-lymphoma
#6
Catarina Roque, Ricardo Fonseca, Carlos Tavares Bello, Carlos Vasconcelos, António Galzerano, Sância Ramos
Primary adrenal lymphoma is a rare malignancy. It frequently presents bilaterally and with symptoms of adrenal insufficiency. Amiodarone may induce secondary organ dysfunction, and thyrotoxicosis develops in 15% of cases. The symptomatology of both conditions is nonspecific, especially in the elderly, and a high suspicion index is necessary for appropriate diagnosis. A 78-year-old female presented to the emergency department with confusion, nausea and vomiting. She had recently been to the emergency department with urinary tract infection, vomiting and acute hypochloremic hyponatremia...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28552871/a-case-of-stiff-person-syndrome-due-to-secondary-adrenal-insufficiency
#7
Yuri Mizuno, Hiroo Yamaguchi, Taira Uehara, Kenichiro Yamashita, Ryo Yamasaki, Jun-Ichi Kira
We report a case of flexion contractures in a patient's legs secondary to postpartum hypopituitarism. A 56-year-old woman presented with a 3-year history of worsening flexion contractures of the hips and knees. On admission, her hips and knees could not be extended, and she had muscle stiffness and tenderness to palpation of the lower extremities. We first suspected stiff-person syndrome or Isaacs' syndrome because of her muscle stiffness. However, multiple hormones did not respond to stimulation tests, and an MRI of the brain showed atrophy of the pituitary gland with an empty sella...
May 26, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28546232/genetic-defects-in-pediatric-onset-adrenal-insufficiency-in-japan
#8
Naoko Amano, Satoshi Narumi, Mie Hayashi, Masaki Takagi, Kazuhide Imai, Toshirou Nakamura, Rumi Hachiya, Goro Sasaki, Keiko Homma, Tomohiro Ishii, Tomonobu Hasegawa
CONTEXT: Most patients with pediatric-onset primary adrenal insufficiency (PAI), such as 21-hydroxylase deficiency, can be diagnosed by measuring the urine or serum levels of steroid metabolites. However, the etiology is often difficult to determine in a subset of patients lacking characteristic biochemical findings. OBJECTIVE: To assess the frequency of genetic defects in Japanese children with biochemically uncharacterized PAI, and characterize the phenotypes of mutation-carrying patients...
May 25, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28536830/transcatheter-aortic-valve-implantation-in-patients-on-corticosteroid-therapy
#9
Ryosuke Higuchi, Tetsuya Tobaru, Kenichi Hagiya, Mike Saji, Keitaro Mahara, Itaru Takamisawa, Jun Shimizu, Shuichiro Takanashi, Morimasa Takayama
Transcatheter aortic valve implantation (TAVI) is recommended for patients who are inoperable or at high risk for surgical aortic valve replacement (SAVR). Corticosteroid therapy is considered to be a risk factor for SAVR, but there is a paucity of information about TAVI in patients taking corticosteroids. The aim of this study is to elucidate the outcome of TAVI in patients on chronic corticosteroid therapy, compared with SAVR. We retrospectively analyzed patients on corticosteroid therapy who underwent TAVI (n = 21) or SAVR (n = 30) for severe aortic stenosis in Sakakibara Heart Institute...
May 23, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28502948/disseminated-cryptococcosis-with-adrenal-insufficiency-and-meningitis-in-an-immunocompetent-individual
#10
Misa Ito, Takashi Hinata, Kaku Tamura, Ayano Koga, Toshimitsu Ito, Hiroko Fujii, Fumihiko Hirata, Hidenari Sakuta
We present a case of cryptococcosis with adrenal insufficiency and meningitis in a healthy host without any risk factors. Antifungal therapy did not reduce the cryptococcal antigen titers of the cerebrospinal fluid and serum or the bilateral adrenal gland enlargement. It was suggested that the adrenal glands were the focus of persistent fungemia. Removal of both adrenal glands brought about a response to antifungal therapy. We conclude that if antifungal therapy is ineffective, bilateral adrenalectomy is an effective measure for treatment of such patients...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28494535/tak-264-mln0264-in-previously-treated-asian-patients-with-advanced-gastrointestinal-carcinoma-expressing-guanylyl-cyclase-c-results-from-an-open-label-non-randomized-phase-1-study
#11
Yung-Jue Bang, Toshimi Takano, Chia-Chi Lin, Adedigbo Fasanmade, Huyuan Yang, Hadi Danaee, Takayuki Asato, Thea Kalebic, Hui Wang, Toshihiko Doi
Purpose: This phase 1 dose-escalation portion of the study evaluated the safety, pharmacokinetics (PK), and antitumor activity of TAK-264 in Asian patients with advanced gastrointestinal (GI) carcinoma or metastatic or recurrent gastric or gastroesophageal junction adenocarcinoma expressing guanylyl cyclase C (GCC). Materials and Methods: Adult patients with advanced GI malignancies expressing GCC (H-score ≥ 10) received TAK-264 on day 1 of 3-week cycles as 30-minute intravenous infusions for up to 1 year or until disease progression or unacceptable toxicity...
May 10, 2017: Cancer Research and Treatment: Official Journal of Korean Cancer Association
https://www.readbyqxmd.com/read/28456143/a-first-case-of-adrenomyeloneuropathy-with-mutation-y174s-of-the-adrenoleukodystrophy-gene
#12
Yukio Horikawa, Mayumi Enya, Nobuaki Yoshikura, Junichi Kitagawa, Shigeo Takashima, Nobuyuki Shimozawa, Jun Takeda
The patient first noticed spasticity and weakness in his legs. He was diagnosed with chronic myelogenous leukemia (CML); the symptoms were attributed to neuropathy associated with CML. By treatment with dasatinib, he achieved complete hematological remission, but his difficulty in walking was not improved. His neurological symptom worsened together with an increase in body temperature and then disappeared together with a normalized body temperature, which may be attributed to the Uhthoff's phenomenon often observed in multiple sclerosis...
February 15, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28450305/mechanisms-in-endocrinology-update-on-pathogenesis-of-primary-adrenal-insufficiency-beyond-steroid-enzyme-deficiency-and-autoimmune-adrenal-destruction
#13
Christa Flueck
Primary adrenal insufficiency (PAI) is potentially life threatening, but rare. In children genetic defects prevail, while adults suffer mostly from acquired forms. The spectrum of genetic defects has increased in recent years with the use of next generation sequencing methods and reaches now far beyond genetic defects in known enzymes of steroidogenesis. Cofactor disorders such as P450 oxidoreductase (POR) deficiency manifesting as a complex form of congenital adrenal hyperplasia with a broad clinical phenotype have come to the forth...
April 27, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28446514/autoimmune-polyendocrine-syndrome-type-1-in-an-indian-cohort-a-longitudinal-study
#14
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
#15
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
#16
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
#17
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%C3%A2-years
#18
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
#19
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/28398936/endogenous-glucocorticoid-response-to-single-dose-dexamethasone-for-croup-in-children-a-pharmacodynamic-study
#20
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
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