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Pituitary insufficiency

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https://www.readbyqxmd.com/read/28741070/late-onset-x-linked-adrenal-hypoplasia-dax-1-nr0b1-two-new-adult-onset-cases-from-a-single-center
#1
Nikolaos Kyriakakis, Tolulope Shonibare, Julie Kyaw-Tun, Julie Lynch, Carlos F Lagos, John C Achermann, Robert D Murray
PURPOSE: DAX-1 (NR0B1) is an orphan nuclear receptor, which plays a critical role in development and regulation of the adrenal gland and hypothalamo-pituitary-gonadal axis. Mutations in NR0B1 lead to adrenal hypoplasia congenita (AHC), hypogonadotropic hypogonadism (HH) and azoospermia in men. Presentation is typically with adrenal insufficiency (AI) during infancy or childhood. To date only eight cases/kindreds are reported to have presented in adulthood. METHODS: We describe two new cases of men with DAX-1 mutations who presented in adulthood and who were diagnosed at a large University Hospital...
July 24, 2017: Pituitary
https://www.readbyqxmd.com/read/28739711/adrenal-insufficiency-related-to-anti-programmed-death-1-therapy
#2
Ryo Ariyasu, Atsushi Horiike, Takahiro Yoshizawa, Yosuke Dotsu, Junji Koyama, Masafumi Saiki, Tomoaki Sonoda, Shingo Nishikawa, Satoru Kitazono, Noriko Yanagitani, Makoto Nishio
BACKGROUND/AIM: Adrenal insufficiency is one of the adverse events (AEs) associated with anti-programmed death-1 (PD1) therapy. Delaying diagnoses can lead to serious conditions. It is necessary to elucidate detailed clinical features of these AEs. PATIENTS AND METHODS: Patients treated with anti-PD-1 monotherapy or in combination with anti-cytotoxic T cell lymphocyte-4 therapy at our hospital from January 2013 to December 2016 were identified. The patients' clinical characteristics and laboratory and radiologic findings were collected...
August 2017: Anticancer Research
https://www.readbyqxmd.com/read/28738798/efficacy-of-sellar-opening-in-the-pituitary-adenoma-resection-of-transsphenoidal-surgery-influences-the-degree-of-tumor-resection
#3
Shousen Wang, Yong Qin, Deyong Xiao, Liangfeng Wei
BACKGROUND: Endonasal transsphenoidal microsurgery is often adopted in the resection of pituitary adenoma, and has showed satisfactory treatment and minor injuries. It is important to accurately localize sellar floor and properly incise the bone and dura matter. METHODS: Fifty-one patients with pituitary adenoma undergoing endonasal transsphenoidal microsurgery were included in the present study. To identify the scope of sellar floor opening, CT scan of the paranasal sinus and MRI scan of the pituitary gland were performed for each subject...
July 24, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28730418/total-and-free-cortisol-levels-during-1-%C3%AE-g-25-%C3%AE-g-and-250-%C3%AE-g-cosyntropin-stimulation-tests-compared-to-insulin-tolerance-test-results-of-a-randomized-prospective-pilot-study
#4
Seenia Peechakara, James Bena, Nigel J Clarke, Michael J McPhaul, Richard E Reitz, Robert J Weil, Pablo Recinos, Laurence Kennedy, Amir H Hamrahian
PURPOSE: The appropriate cosyntropin dose during cosyntropin stimulation tests remains uncertain. We conducted a prospective, randomized pilot study to compare 1 μg IV low dose cosyntropin test, 25 μg IM medium dose cosyntropin test, and 250 μg IM standard dose cosyntropin test to evaluate secondary adrenal insufficiency. Insulin tolerance test was used as the gold standard. METHOD: The study included patients with hypothalamic/pituitary disease (n  = 10) with at least one pituitary axis deficiency other than ACTH deficiency and controls (n  = 12)...
July 20, 2017: Endocrine
https://www.readbyqxmd.com/read/28721596/central-adrenal-insufficiency-following-traumatic-brain-injury-a-missed-diagnosis-in-the-critically-injured
#5
Eileen Fan, Peter W Skippen, Michael A Sargent, David D Cochrane, Jean-Pierre Chanoine
BACKGROUND: High-dose steroid administration is no longer recommended in the treatment of acute traumatic brain injury (TBI) as it failed to prove beneficial in improving patients' outcome. However, a masked benefit of steroid administration in TBI management was that it provided corticosteroid replacement therapy in patients with TBI-related central adrenal insufficiency. CASE PRESENTATION: We report the case of a 12-year-old boy who suffered a severe TBI from a motor vehicle accident that resulted in complete deficiency of anterior pituitary function...
July 18, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28721216/switch-to-restoration-therapy-in-a-testosterone-treated-central-hypogonadism-with-erythrocytosis
#6
B Cangiano, C Cacciatore, L Persani, M Bonomi
We describe a case of severe erythrocytosis caused by testosterone replacement therapy in a 66-year-old man affected with hypogonadotropic hypogonadism (HH) determining osteoporosis, resolved by switching to restoration therapy with clomiphene citrate. The patient complained fatigue, loss of libido and defective erections and a spontaneous vertebral fracture despite bisphosphonate therapy and vitamin D supplementation. The examinations proved isolated HH and he was therefore treated with testosterone gel with regression of specific manifestations but elevated hemoglobin and hematocrit values...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28715822/long-term-cortisol-concentration-in-scalp-hair-of-asthmatic-children-using-inhaled-corticosteroids-a-case-control-study
#7
Maaike P Smit, Ed H G van Leer, Gerard Noppe, Yolanda B de Rijke, Dieneke Kramer van Driel, Erica L T van den Akker
INTRODUCTION: Inhaled corticosteroids (ICS) can interfere with the hypothalamic-pituitary-adrenal (HPA) axis and may lead to adrenal insufficiency, resulting in a decrease of cortisol production. Cortisol levels measured in scalp hair provide a marker for long-term cortisol exposure. Data regarding hair cortisol concentration (HCC) in asthmatic children with ICS therapy are scarce. The aim of this study is to compare HCC in asthmatic children under ICS treatment with a healthy control group...
July 14, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28693284/%C3%AE-human-chorionic-gonadotropin-secreting-intracranial-germ-cell-tumor-associated-with-high-testosterone-in-an-adult-man-a-case-report
#8
Wen-Ping Yang, Hung-Yu Chien, Yi-Chun Lin
A 38-year-old male patient presented with general weakness, polydipsia and a body weight loss of 10 kg in two years. Hypopituitarism with central hypothyroidism and central adrenal insufficiency were noted at Taipei City Hospital (Taipei, Taiwan). However, hypogonadotropic hypergonadism was also observed. The patient was diagnosed with an intracranial β-human chorionic gonadotropin (β-hCG) secreting germ-cell tumor, and brain magnetic resonance imaging revealed that the tumor involved the pineal gland, stalk, posterior pituitary gland, right basal ganglion, hypothalamus, corpus callosum and posterior hippocampus...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28687446/preservation-of-hormonal-function-by-identifying-pituitary-gland-at-endoscopic-surgery
#9
Stefan Linsler, Renate Hero-Gross, Bettina Friesenhahn-Ochs, Salman Sharif, Frank Lammert, Joachim Oertel
OBJECTIVE: The endonasal endoscopic approach has been established for perisellar tumor surgery with a higher resection rate and reduced complications. We analyzed the potential to identify the pituitary gland under endoscopic view, at surgery and see its relation to postoperative hormonal insufficiency in endonasal endoscopic procedures. METHODS: Between January 2011 and January 2014, 70 cases of pituitary adenomas with preoperative intact pituitary function underwent endoscopic endonasal transsphenoidal procedures for intrasellar pathologies...
July 4, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28678331/impact-of-postoperative-pituitary-tumor-adrenal-insufficiency-and-hormone-replacement-therapy-hrt-on-tumor-recurrence
#10
G-X Wei, S-L Wang, L Zhang, Y-Q Ma, H-S Xing
OBJECTIVE: The present study was aimed to investigate the impact of postoperative pituitary tumor adrenal insufficiency and hormone replacement therapy (HRT) on tumor recurrence. PATIENTS AND METHODS: The prospective study included 143 pituitary tumor patients as study subjects. Within 6 months after the operation, the study was planned to explore differences (if any) between the tumor recurrence rate of patients treated with hormone replacement therapy and the patients without hormone replacement therapy...
June 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28661901/long-term-and-late-treatment-consequences-endocrine-and-metabolic-effects
#11
Thang S Han, Helena K Gleeson
PURPOSE OF REVIEW: Cancer therapies often result in the 'late effect of cancer treatment' whereby secondary health complications emerge years after radiotherapy and chemotherapy. This review focuses on endocrine and metabolic consequences in adult cancer survivors as late treatment effects. RECENT FINDINGS: Endocrine and metabolic disorders are among the most common late effects. Endocrine disorders include hypopituitarism, which leads to growth hormone deficiency, hypogonadism, adrenal insufficiency and hypothyroidism and related clinical manifestations...
June 28, 2017: Current Opinion in Supportive and Palliative Care
https://www.readbyqxmd.com/read/28650200/the-cortisol-c-reactive-protein-ratio-and-negative-affect-reactivity-in-depressed-adults
#12
Edward C Suarez, John S Sundy
OBJECTIVE: We evaluated the effect of the cortisol (CORT) to high sensitivity C-reactive protein (hsCRP) ratio on stress-induced negative affect (NA) reactivity and whether the association was moderated by depressive symptom severity and gender. The CORT/CRP ratio was used to evaluate the integrity of the negative feedback loop between the hypothalamic-pituitary-adrenal axis and inflammatory response system. METHOD: Basal CORT and hsCRP levels were measured in fasting blood samples from 198 medication-free and nonsmoking healthy men and women...
June 26, 2017: Health Psychology: Official Journal of the Division of Health Psychology, American Psychological Association
https://www.readbyqxmd.com/read/28649232/ion-channels-of-pituitary-gonadotrophs-and-their-roles-in-signaling-and-secretion
#13
REVIEW
Stanko S Stojilkovic, Ivana Bjelobaba, Hana Zemkova
Gonadotrophs are basophilic cells of the anterior pituitary gland specialized to secrete gonadotropins in response to elevation in intracellular calcium concentration. These cells fire action potentials (APs) spontaneously, coupled with voltage-gated calcium influx of insufficient amplitude to trigger gonadotropin release. The spontaneous excitability of gonadotrophs reflects the expression of voltage-gated sodium, calcium, potassium, non-selective cation-conducting, and chloride channels at their plasma membrane (PM)...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28648512/the-multiple-genetic-causes-of-central-hypothyroidism
#14
REVIEW
Luca Persani, Marco Bonomi
An insufficient stimulation by thyrotropin (TSH) of an otherwise normal thyroid gland represents the cause of Central Hypothyrodism (CeH). CeH is about 1000-folds rarer than Primary Hypothyroidism and often represents a real challenge for the clinicians, mainly because they cannot rely on adequately sensitive parameters for diagnosis or management, as it occurs with circulating TSH in PH. Therefore, CeH diagnosis can be frequently missed or delayed in patients with a previously unknown pituitary involvement...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28645353/actualit%C3%A3-s-autour-de-la-prise-en-charge-des-diab%C3%A3-tes-insipides-centraux-management-of-central-diabetes-insipidus-in-2016
#15
H Lasolle, F Borson-Chazot
Diabetes insipidus is a syndrome that associates both hypotonic polyuria and polydipsia, due to insufficient or ineffective arginine vasopressin (AVP) synthesis, or to AVP resistance. The diagnosis between central/renal origin, or an abnormal thirst regulation (primary polydipsia) is required to organize an adapted management. Because water deprivation tests are not reliable, it's often based on medical history, response to treatment and MRI. Copeptin is an AVP precursor which could be very helpful for the diagnosis...
October 2016: Annales D'endocrinologie
https://www.readbyqxmd.com/read/28634705/how-does-pregnancy-affect-the-patients-with-pituitary-adenomas-a-study-on-113-pregnancies-from-turkey
#16
Z Karaca, S Yarman, I Ozbas, P Kadioglu, M Akturk, F Kilicli, H S Dokmetas, R Colak, H Atmaca, Z Canturk, Y Altuntas, N Ozbey, N Hatipoglu, F Tanriverdi, K Unluhizarci, F Kelestimur
OBJECTIVE: Data regarding pregnancies in relation to pituitary tumors are limited. The effects of pregnancy on pituitary adenomas and the effects of adenoma itself (hormonal activity, mass effects and pituitary insufficiency) and/or treatment on the ongoing gestation and developing fetus were evaluated. METHODS: The study was a retrospective study. A questionnaire involving questions regarding medical history before index gestation, history of related pregnancy, result of index gestation and postpartum follow-up of the patients was filled by the investigator in one of the eight Referral Endocrinology Centers from Turkey...
June 20, 2017: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/28625504/mutations-in-armc9-which-encodes-a-basal-body-protein-cause-joubert-syndrome-in-humans-and-ciliopathy-phenotypes-in-zebrafish
#17
Julie C Van De Weghe, Tamara D S Rusterholz, Brooke Latour, Megan E Grout, Kimberly A Aldinger, Ranad Shaheen, Jennifer C Dempsey, Sateesh Maddirevula, Yong-Han H Cheng, Ian G Phelps, Matthias Gesemann, Himanshu Goel, Ohad S Birk, Talal Alanzi, Rifaat Rawashdeh, Arif O Khan, Michael J Bamshad, Deborah A Nickerson, Stephan C F Neuhauss, William B Dobyns, Fowzan S Alkuraya, Ronald Roepman, Ruxandra Bachmann-Gagescu, Dan Doherty
Joubert syndrome (JS) is a recessive neurodevelopmental disorder characterized by hypotonia, ataxia, abnormal eye movements, and variable cognitive impairment. It is defined by a distinctive brain malformation known as the "molar tooth sign" on axial MRI. Subsets of affected individuals have malformations such as coloboma, polydactyly, and encephalocele, as well as progressive retinal dystrophy, fibrocystic kidney disease, and liver fibrosis. More than 35 genes have been associated with JS, but in a subset of families the genetic cause remains unknown...
July 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28624161/clinical-follow-up-of-the-first-sf-1-insufficient-female-patient
#18
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/28615049/a-case-of-acute-sheehan-s-syndrome-and-literature-review-a-rare-but-life-threatening-complication-of-postpartum-hemorrhage
#19
Shinya Matsuzaki, Masayuki Endo, Yutaka Ueda, Kazuya Mimura, Aiko Kakigano, Tomomi Egawa-Takata, Keiichi Kumasawa, Kiyoshi Yoshino, Tadashi Kimura
BACKGROUND: Sheehan's syndrome occurs because of severe postpartum hemorrhage causing ischemic pituitary necrosis. Sheehan's syndrome is a well-known condition that is generally diagnosed several years postpartum. However, acute Sheehan's syndrome is rare, and clinicians have little exposure to it. It can be life-threatening. There have been no reviews of acute Sheehan's syndrome and no reports of successful pregnancies after acute Sheehan's syndrome. We present such a case, and to understand this rare condition, we have reviewed and discussed the literature pertaining to it...
June 14, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/28604944/the-influence-of-graves-orbitopathy-treatment-with-intravenous-glucocorticoids-on-adrenal-function
#20
Urszula Ambroziak, Gabriela Bluszcz, Tomasz Bednarczuk, Piotr Miśkiewicz
INTRODUCTION: Graves' orbitopathy (GO) is the commonest extrathyroidal feature of Graves' disease. For active, moderate to severe GO intravenous glucocorticoid pulse therapy (ivGCS) is the first-line treatment, which might be followed by oral glucocorticoid therapy. Glucocorticoid treatment, especially in the time of withdrawal, carries a risk of adrenal insufficiency. The aim of this study was to assess the pituitary-adrenal axis function in patients with GO before and at the cessation of ivGCS, and after further oral glucocorticoid therapy...
June 12, 2017: Endokrynologia Polska
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