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https://www.readbyqxmd.com/read/28530316/-radiosurgery-for-pituitary-adenomas
#1
REVIEW
Or Cohen-Inbar
Pituitary adenomas represent one of the most common types of intracranial tumors, accounting for 10-20% of intracranial tumors. While their macroscopic appearance and anatomical location are relatively homogeneous, pituitary tumors differ widely, generating a variety of neurological and endocrine clinical sequelae. Treatment options include hormone suppressive medical therapy, microscopic or endoscopic neurosurgical resection, radiosurgery, radiation therapy, or observation depending on the biochemical profile and the clinical status of the patient...
January 2017: Harefuah
https://www.readbyqxmd.com/read/28527171/lymphocytic-hypophysitis-mimicking-pituitary-macroadenoma
#2
Tarun Kumar Ralot, Jitesh Aggarwal, Raghavendra Haniadka, Kushal Gehlot, Nikhil Dongre, Swapnil Patil
Lymphocytic hypophysitis is an inflammatory /autoimmune disorder that primarily involves the pituitary gland and the pituitary stalk. The common clinical presentations include headache, nausea, vomiting, fatigue, features of hypopituitarism and diabetes insipidus as well as diplopia, orbital pain and bitemporal hemianopia. We report a case of lymphocytic hypophysitis which presented as hemichorea. Neuroimaging showed a mass in the sella tursica region which, on histopathological examination was suggestive of lymphocytic hypophysitis...
April 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28511971/learning-curve-for-the-endoscopic-endonasal-approach-for-suprasellar-craniopharyngiomas
#3
Hailin Ding, Ye Gu, Xiaobiao Zhang, Tao Xie, Tengfei Liu, Fan Hu, Yong Yu, Chongjing Sun
The endoscopic endonasal approach is considered an alternative minimally invasive approach for suprasellar craniopharyngiomas. However, the complicated surgical manipulations required by this approach have limited its application. We evaluate whether the approach features a learning curve. Thirty-three patients were retrospectively reviewed and grouped as early (17 patients) and late (16 patient) groups. The operation time, extent of removal, ophthalmology, endocrinology, reconstruction and modifications of standard technique were evaluated...
May 13, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28483366/lessons-from-monogenic-causes-of-growth-hormone-deficiency
#4
Thierry Brue, Alexandru Saveanu, Nicolas Jullien, Teddy Fauquier, Frédéric Castinetti, Alain Enjalbert, Anne Barlier, Rachel Reynaud
Through the multicentric international GENHYPOPIT network, 10 transcription factor genes involved in pituitary development have been screened in more than 1200 patients with constitutional hypopituitarism over the past two decades. The present report summarizes the main lessons learned from this phenotype-based genetic screening: (1) genetically determined hypopituitarism does not necessarily present during childhood; (2) constitutional hypopituitarism may be characterized by a pure endocrine phenotype or by various combinations of endocrine deficits and visceral malformations; (3) syndromic hypopituitarism may also be observed in patients with POU1F1 or PROP1 mutations; (4) in cases of idiopathic hypopituitarism, extensive genetic screening identifies gene alterations in a minority of patients; (5) functional studies are imperfect in determining the involvement of an allelic variant in a specific pituitary phenotype...
May 5, 2017: Annales D'endocrinologie
https://www.readbyqxmd.com/read/28476222/pituitary-hypoplasia
#5
REVIEW
Mariam Gangat, Sally Radovick
This article summarizes pituitary development and function as well as specific mutations of genes encoding the following transcription factors: HESX1, LHX3, LHX4, POU1F1, PROP1, and OTX2. Although several additional genetic defects related to hypopituitarism have been identified, this article focuses on these selected factors, as they have been well described in the literature in terms of clinical characterization of affected patients and molecular mechanisms of action, and therefore, are very relevant to clinical practice...
June 2017: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/28472507/a-case-report-of-hypoglycemia-and-hypogammaglobulinemia-david-syndrome-in-a-patient-with-a-novel-nfkb2-mutation
#6
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/28469927/clinical-course-of-pituitary-function-and-image-in-igg4-related-hypophysitis
#7
Takatoshi Anno, Fumiko Kawasaki, Maiko Takai, Ryo Shigemoto, Yuki Kan, Hideaki Kaneto, Tomoatsu Mune, Kohei Kaku, Niro Okimoto
A 76-year-old man had a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism. Based on various findings including the swelling of the pituitary gland, increase of serum IgG4 level and abundant IgG4-positive plasma cell infiltration in immunostaining of the pituitary gland, we diagnosed this subject as IgG4-related hypophysitis. In general, a high-dose glucocorticoid treatment is effective for IgG4-related disease. His clinical symptom, laboratory data and adrenal insufficiency were almost improved without any therapy...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28465267/outcome-of-endoscopic-transsphenoidal-surgery-for-acromegaly
#8
Jung Hee Kim, Kyu Yeon Hur, Jung Hyun Lee, Ji Hyun Lee, Young-Bem Se, Hey In Kim, Seung Hoon Lee, Do-Hyun Nam, Seong Yeon Kim, Kwang-Won Kim, Doo-Sik Kong, Yong Hwy Kim
OBJECTIVE: Endoscopic transsphenoidal surgery has recently been introduced in pituitary surgery. We investigated outcomes and complications of endoscopic surgery in two referral centers in Korea. METHODS: We enrolled 134 patients with acromegaly (microadenomas, n=15; macroadenomas, n=119) who underwent endoscopic transsphenoidal surgery at Seoul National University Hospital (n=74) and Samsung Medical center (n=60) between Jan 2009 and Mar 2016. Remission was defined as having a normal IGF-1 and a suppressed GH less than 1 ng/mL during an oral glucose tolerance test...
April 29, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28458931/dental-abnormalities-in-pituitary-dwarfism-a-case-report-and-review-of-the-literature
#9
Franco Ferrante, Sergio Blasi, Rolando Crippa, Francesca Angiero
Hypopituitarism is a disorder caused by a reduced level of trophic hormones that may be consequent on different destructive processes. The clinical manifestations depend on the type of hormone involved. A deficiency of growth hormone (GH) in children causes the lack of growth known as pituitary dwarfism. The case is reported of a patient with pituitary dwarfism, multiple dental anomalies, functional prosthetic problems, and a revision of the literature. She was subjected to prosthetic rehabilitation without surgical intervention, using zirconium substructures, thus eliminating the potential complications that may require trauma surgery...
2017: Case Reports in Dentistry
https://www.readbyqxmd.com/read/28458894/prop1-gene-mutations-in-a-36-year-old-female-presenting-with-psychosis
#10
Durgesh Prasad Chaudhary, Tshristi Rijal, Kunal Kishor Jha, Harpreet Saluja
SUMMARY: Combined pituitary hormonal deficiency (CPHD) is a rare disease that results from mutations in genes coding for transcription factors that regulate the differentiation of pituitary cells. PROP1 gene mutations are one of the etiological diagnoses of congenital panhypopituitarism, however symptoms vary depending on phenotypic expression. We present a case of psychosis in a 36-year-old female with congenital panhypopituitarism who presented with paranoia, flat affect and ideas of reference without a delirious mental state, which resolved with hormone replacement and antipsychotics...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28458461/endocrine-status-of-patients-with-septo-optic-dysplasia-fourteen-japanese-cases
#11
Mikiko Koizumi, Shinobu Ida, Yasuko Shoji, Yuri Etani, Yoshikazu Hatsukawa, Nobuhiko Okamoto
A clinical diagnosis of septo-optic dysplasia (SOD) is made when two or more of the classical triad of optic nerve hypoplasia, pituitary hormone abnormalities or midline brain defects. To date, a clinical study of SOD, regarding its endocrinological features in particular, has not been undertaken in Japan. We retrospectively evaluated 14 SOD patients at our institution. Hormonal dysfunction was present in 78% of cases: ten cases presented combined hypopituitarism and one case presented precocious puberty. GHD and hypothyroidism were the most common endocrinopathies...
2017: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/28453858/digenic-inheritance-of-prokr2-and-wdr11-mutations-in-pituitary-stalk-interruption-syndrome
#12
Shana E McCormack, Dong Li, Yeon Joo Kim, Ji Young Lee, Soo-Hyun Kim, Robert Rapaport, Michael A Levine
Context: Pituitary stalk interruption syndrome (PSIS, ORPHA95496) is a congenital defect of the pituitary gland characterized by the triad of a very thin/interrupted pituitary stalk, an ectopic (or absent) posterior pituitary gland, and hypoplasia or aplasia of the anterior pituitary gland. Complex genetic patterns of inheritance of this disorder are increasingly recognized. Objective: The objective of this study was to identify a genetic etiology of PSIS in an affected child...
April 27, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28450934/microscopic-surgery-for-pituitary-adenomas-to-preserve-the-pituitary-gland-and-stalk
#13
Hao-Yu Li, Cheng-Yuan Feng, Chi Zhang, Jun Su, Jian Yuan, Yuanyang Xie, Yiwei Liao, Xianrui Yuan, Qing Liu
Surgery is the primary treatment of choice for all symptomatic pituitary adenomas except prolactinomas. Common postoperative complications include endocrinopathies, vision impairment and cerebrospinal fluid leak. The present study assessed 153 continuous microscopic surgeries for pituitary adenomas performed by an author of the present study between 2010 to 2014. Patients underwent either transphenoidal or transcranial surgery depending on their individual tumor characteristics. Five typical cases are presented in the present study and intraoperative identification and preservation of the gland and stalk were discussed...
March 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28448761/sleeping-cupid-by-caravaggio-what-diagnosis
#14
Paolo Pozzilli, Marco Cappa
The Sleeping Cupid, by Italian painter Caravaggio is a masterpiece painted in 1608. Signs of an endocrine disease are hidden among the shadows of the painting. We decided to conduct a survey among endocrinologists practicing in Italy and asked them if they could identify a specific disease condition in Caravaggio's Sleeping Cupid. A total of 86 members from two Italian endocrine societies completed the survey, 67 from the Society of Endocrinology and 19 from the Society of Paediatric Endocrinology and Diabetology...
April 27, 2017: Endocrine Practice
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/28438051/similarity-of-female-central-hypogonadotropic-hypogonadism-and-postmenopause
#16
I Ilovayskaya, V Zektser, L Lazebnik
OBJECTIVES: Central (hypogonadotropic) hypogonadism in women could be a cause of persistent amenorrhea and hypoestrogenemia as observed in postmenopause. This study aimed to compare the clinical, hormonal and biochemical features in women with non-physiological (central hypogonadism) and physiological (postmenopause) hypoestrogenemia. METHODS: A total of 161 young women, median age 24.9 years (interquartile range (IQR) 21.2; 30.5) with central hypogonadism (with isolated hypogonadotropic hypogonadism, n = 76, and with hypopituitarism, n = 85), 53 healthy young women, median age 23...
April 24, 2017: Climacteric: the Journal of the International Menopause Society
https://www.readbyqxmd.com/read/28425105/the-incidence-of-anterior-pituitary-hormone-deficiencies-in-patients-with-microprolactinoma-and-idiopathic-hyperprolactinaemia
#17
L A Behan, P Moyles, M Cuesta, B Rogers, R K Crowley, J Ryan, P Brennan, D Smith, W Tormey, M Sherlock, C J Thompson
INTRODUCTION: Patients with microprolactinoma and idiopathic hyperprolactinaemia are not generally considered to be at risk of hypopituitarism and are therefore not routinely screened for this abnormality. In our clinical practice we have observed a number of patients with non-macroadenomatous hyperprolactinaemia to have anterior pituitary hormone deficits. AIMS: We aimed to establish the frequency and clinical significance of anterior pituitary hormone deficiencies, comparing patients with radiologically proven microprolactinomas and patients with idiopathic hyperprolactinaemia...
April 20, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28423663/a-novel-total-pituitary-hormone-index-as-an-indicator-of-postoperative-pituitary-function-in-patients-undergoing-resection-of-pituitary-adenomas
#18
Shousen Wang, Biao Li, Chenyu Ding, Deyong Xiao, Liangfeng Wei
The purpose of this study was to investigate the differences between pre- and postoperative pituitary hormone levels in patients undergoing surgical resection of pituitary adenoma and to identify factors associated with preoperative hypopituitarism.Data from 81 patients with histologically confirmed functioning and non-functioning pituitary adenomas (NFPA) who underwent transsphenoidal resection from January 2011 to December 2013 were retrospectively analyzed. Logistic regression was applied to analyze factors associated with preoperative hypopituitarism...
March 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28415806/lithium-protects-hippocampal-progenitors-cognitive-performance-and-hypothalamus-pituitary-function-after-irradiation-to-the-juvenile-rat-brain
#19
Kai Zhou, Cuicui Xie, Malin Wickström, Amalia M Dolga, Yaodong Zhang, Tao Li, Yiran Xu, Carsten Culmsee, Per Kogner, Changlian Zhu, Klas Blomgren
Cranial radiotherapy in children typically causes delayed and progressive cognitive dysfunction and there is no effective preventive strategy for radiation-induced cognitive impairments. Here we show that lithium treatment reduced irradiation-induced progenitor cell death in the subgranular zone of the hippocampus, and subsequently ameliorated irradiation-reduced neurogenesis and astrogenesis in the juvenile rat brain. Irradiation-induced memory impairment, motor hyperactivity and anxiety-like behaviour were normalized by lithium treatment...
March 16, 2017: Oncotarget
https://www.readbyqxmd.com/read/28382019/transcriptome-analysis-showed-a-differential-signature-between-invasive-and-non-invasive-corticotrophinomas
#20
Leonardo Jose Tadeu de Araújo, Antonio Marcondes Lerario, Margaret de Castro, Clarissa Silva Martins, Marcello Delano Bronstein, Marcio Carlos Machado, Ericka Barbosa Trarbach, Maria Candida Barisson Villares Fragoso
ACTH-dependent hypercortisolism caused by a pituitary adenoma [Cushing's disease (CD)] is the most common cause of endogenous Cushing's syndrome. CD is often associated with several morbidities, including hypertension, diabetes, osteoporosis/bone fractures, secondary infections, and increased cardiovascular mortality. While the majority (≈80%) of the corticotrophinomas visible on pituitary magnetic resonance imaging are microadenomas (MICs, <10 mm of diameter), some tumors are macroadenomas (MACs, ≥10 mm) with increased growth potential and invasiveness, exceptionally exhibiting malignant demeanor...
2017: Frontiers in Endocrinology
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