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adrenal hyperplasia

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https://www.readbyqxmd.com/read/28102088/hyperandrogenism-in-a-postmenopausal-woman-a-rare-case-of-ectopic-adrenal-cortical-gland
#1
Antonella Guarino, Luisa Di Benedetto, Valentina Giovanale, Gian Luca Rampioni Vinciguerra, Antonella Stoppacciaro, Filippo Bellati, Donatella Caserta
Most frequent causes of androgenic manifestation are Cushing's syndrome, PCO, benign and malignant androgen-secreting non adrenal tumors and iatrogenic hirsutism. Hyperplasia or neoplasms of ectopic adrenocortical gland are rare. We report a case of a 63-year old female with hirsutism and alopecia. Laboratory data highlighted increased levels of androgens. Diagnostic imaging revealed normal morphology of adrenocortical gland and ovaries. In view of the clinical picture and suspected diagnosis of extra-adrenal cause, she underwent bilateral salpingo-oophorectomy...
January 19, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28100629/impact-of-food-alcohol-and-ph-on-modified-release-hydrocortisone-developed-to-treat-congenital-adrenal-hyperplasia
#2
Nayananjani Karunasena, Daniel Margetson, Greg Neal, Martin Whitaker, Richard J Ross
BACKGROUND: We developed a modified-release hydrocortisone, Chronocort®, to replace the cortisol rhythm in patients with congenital adrenal hyperplasia. Food, alcohol and pH affect drug absorption and it is important to assess their impact when replicating a physiological rhythm. SUBJECTS AND METHODS: In vitro dissolution to study impact of alcohol and pH on Chronocort®. A Phase 1, three-period, cross over study in 18 volunteers to assess the impact of food on Chronocort® and to compare bioavailability to immediate-release hydrocortisone...
January 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28095810/neonatal-screening-for-congenital-adrenal-hyperplasia-in-southern-brazil-a-population-based-study-with-108-409-infants
#3
Cristiane Kopacek, Simone Martins de Castro, Mayara Jorgens Prado, Claudia Maria Dornelles da Silva, Luciana Amorim Beltrão, Poli Mara Spritzer
BACKGROUND: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder associated with inborn errors of steroid metabolism. 21-hydroxylase enzyme deficiency occurs in 90 to 95% of all cases of CAH, with accumulation of 17 hydroxyprogesterone (17-OHP). Early diagnosis of CAH based on newborn screening is possible before the development of symptoms and allows proper treatment, correct sex assignment, and reduced mortality rates. This study describes the results obtained in the first year of a public CAH screening program in the state of Rio Grande do Sul, Brazil...
January 17, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28089605/commentary-to-urinary-continence-outcome-following-vaginoplasty-in-patients-with-congenital-adrenal-hyperplasia
#4
David A Diamond
No abstract text is available yet for this article.
December 11, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28079539/preoperative-ketoconazole-therapy-for-primary-bilateral-adrenocorticotropic-hormone-independent-macronodular-adrenal-hyperplasia-syndrome
#5
Rukma Parthvi, Sunil Mehra
No abstract text is available yet for this article.
January 5, 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/28073127/long-term-consequences-of-congenital-adrenal-hyperplasia-due-to-classic-21-hydroxylase-deficiency-in-adolescents-and-adults
#6
Ja Hye Kim, Jin-Ho Choi, Eungu Kang, Yoon-Myung Kim, Beom Hee Lee, Han-Wook Yoo
Background The management of congenital adrenal hyperplasia (CAH) from pediatric to adulthood is challenging to achieve optimal growth and puberty. This study characterizes the clinical outcomes of 21-hydroxylase deficiency. Methods 53 CAH patients were included (33 females, 15 and 18 patients with the salt-wasting [SW] and simple-virilizing [SV] forms; and 20 males, 16 and 4 patients with the SW and SV forms). We reviewed growth parameters, pubertal status, and long-term morbidities. Results In females, the age at pubertal onset and pubarche was 9...
January 10, 2017: Experimental and Clinical Endocrinology & Diabetes
https://www.readbyqxmd.com/read/28066990/management-of-adrenal-masses-in-patients-with-beckwith-wiedemann-syndrome
#7
Suzanne P MacFarland, Sogol Mostoufi-Moab, Kristin Zelley, Peter A Mattei, Lisa J States, Tricia R Bhatti, Kelly A Duffy, Garrett M Brodeur, Jennifer M Kalish
Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome, associated with both benign and malignant adrenal findings. Literature review and an institutional case series elucidate the wide spectrum of adrenal findings in BWS patients. The altered expression of the 11p15 region is likely related to adrenal gland hyperplasia and growth dysregulation. Given the absence of guidelines for managing adrenal findings in BWS, we propose a systematic approach to adrenal findings in BWS patients, to allow for maximum detection of potentially malignant pathology without posing additional risk to patients...
January 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28057157/-clinical-features-and-outcomes-of-congenital-adrenal-hyperplasia-with-adenomatoid-adrenal-gland
#8
Y L Gu, W J Gu, J T Dou, L Zang, J Du, G Q Yang, L J Yang, J M Ba, Z H Lü, Y M Mu, J M Lu
Objective: To analyze the clinical features and outcomes of congenital adrenal hyperplasia (CAH) with adenomatoid adrenal gland. Methods: Nineteen patients clinically confirmed as CAH with adenomatoid adrenal gland, from 2008 to 2015 in Deparment of Endocrinology of Chinese PLA General Hospital, were retrospectively analyzed, and the outcomes of the treatment were followed up. Results: Seventy-six patients were clinically confirmed as CAH, from 2008 to 2015 in PLA hospital. Nineteen of them have accompanied with adenomatoid adrenal gland...
December 27, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28032206/ovarian-adrenal-rest-tumors-undetected-by-imaging-studies-and-identified-at-surgery-in-three-females-with-congenital-adrenal-hyperplasia-unresponsive-to-increased-hormone-therapy-dosage
#9
Hua-Dong Chen, Li-E Huang, Zhi-Hai Zhong, Zhe Su, Hong Jiang, Jing Zeng, Jun-Cheng Liu
Patients with congenital adrenal hyperplasia have a predisposition for developing adrenal rest tumors. In contrast to testicular adrenal rest tumors, ovarian adrenal rest tumors are less common, and only a few cases have been reported in the literature. This report presents three Chinese female congenital adrenal hyperplasia patients (9 to 15 years of age) with small ectopic adrenal cortical nodules that were not detected by imaging but were diagnosed at surgery. All three patients developed virilization with elevation of 17- hydroxyprogesterone, androstenedione, and androgen levels despite receiving maximum adrenal hormone replacement therapy...
December 28, 2016: Endocrine Pathology
https://www.readbyqxmd.com/read/28026881/intrinsic-factors-adrenal-gland-morphology-and-disease-burden-in-captive-cheetahs-acinonyx-jubatus-in-south-africa
#10
Nina Gillis-Germitsch, Pamela-Rose Vybiral, Daryl Codron, Marcus Clauss, Antoinette Kotze, Emily P Mitchell
Adrenal gland weight (AW) and corticomedullary ratio (ACMR) are used as indicators of stress in animals. Captive cheetahs (Acinonyx jubatus) have higher ACMRs than free-ranging ones and stress has been linked to gastritis, amyloidosis, glomerulosclerosis, and myocardial fibrosis. We reviewed age, sex, body weight (BW), kidney weight (KW), and left AW and ACMR with necropsy findings in 51 South African captive cheetahs. Eleven common histopathologic lesions were counted for each animal as measure of its disease burden...
December 27, 2016: Zoo Biology
https://www.readbyqxmd.com/read/28018429/408-cases-of-genital-ambiguity-followed-by-single-multidisciplinary-team-during-23-years-etiologic-diagnosis-and-sex-of-rearing
#11
Georgette Beatriz De Paula, Beatriz Amstalden Barros, Stela Carpini, Bruna Jordan Tincani, Tais Nitsch Mazzola, Mara Sanches Guaragna, Cristiane Santos da Cruz Piveta, Laurione Candido de Oliveira, Juliana Gabriel Ribeiro Andrade, Guilherme Guaragna-Filho, Pedro Perez Barbieri, Nathalia Montibeler Ferreira, Marcio Lopes Miranda, Ezequiel Moreira Gonçalves, Andre Moreno Morcillo, Nilma Lucia Viguetti-Campos, Sofia Helena Valente Lemos-Marini, Roberto Benedito de Paiva Silva, Antonia Paula Marques-de-Faria, Maricilda Palandi De Mello, Andrea Trevas Maciel-Guerra, Gil Guerra-Junior
Objective. To evaluate diagnosis, age of referral, karyotype, and sex of rearing of cases with disorders of sex development (DSD) with ambiguous genitalia. Methods. Retrospective study during 23 years at outpatient clinic of a referral center. Results. There were 408 cases; 250 (61.3%) were 46,XY and 124 (30.4%) 46,XX and 34 (8.3%) had sex chromosomes abnormalities. 189 (46.3%) had 46,XY testicular DSD, 105 (25.7%) 46,XX ovarian DSD, 95 (23.3%) disorders of gonadal development (DGD), and 19 (4.7%) complex malformations...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27998513/sexual-orientation-and-medical-history-among-iranian-people-with-complete-androgen-insensitivity-syndrome-and-congenital-adrenal-hyperplasia
#12
Behzad S Khorashad, Ghasem M Roshan, Alistair G Reid, Zahra Aghili, Mehran Hiradfar, Mozhgan Afkhamizadeh, Ali Talaei, Azadeh Aarabi, Nosrat Ghaemi, Negin Taghehchian, Hedieh Saberi, Nazanin Farahi, Mohammad Reza Abbaszadegan
OBJECTIVE: To report sexual orientation, relationship status and medical history of Iranian people with Differences of Sex Development (DSD) who were raised female. METHODS: Our participants consisted of nineteen 46,XY individuals with Complete Androgen Insensitivity Syndrome (CAIS) and eighteen 46,XX individuals with Congenital Adrenal Hyperplasia (CAH) who were raised as females and older than 13years. As well as their relationship status and detailed medical history, an expert psychiatrist assessed their sexual orientation by a semi-structured psychiatric interview with them and, where applicable, their parents...
January 2017: Journal of Psychosomatic Research
https://www.readbyqxmd.com/read/27994303/accessory-spleen-in-the-splenic-hilum-a-cadaveric-study-with-clinical-significance
#13
Shabnam Mohammadi, Arya Hedjazi, Maryam Sajjadian, Naser Ghrobi, Maliheh Dadgar Moghadam, Maryam Mohammadi
AIM: An accessory spleen is an additional tissue of the spleen that may be found near the spleen. It is a congenital anomaly of the spleen that its incidence has been reported 10-30% of the population. Hence, the objective of this study was evaluation the incidence of accessory spleen in Iranian cadavers. METHOD: Sixty hundred and ninety three spleens (541 males, 152 females) were excised from cadavers in the dissection hall of Mashhad Forensic Medicine Organization cadavers...
October 2016: Medical Archives
https://www.readbyqxmd.com/read/27978607/neonatal-17-hydroxyprogesterone-levels-adjusted-according-to-age-at-sample-collection-and-birth-weight-improve-the-efficacy-of-congenital-adrenal-hyperplasia-newborn-screening
#14
Giselle Y Hayashi, Daniel F Carvalho, Mirela C de Miranda, Cláudia Faure, Carla Vallejos, Vinícius N Brito, Andresa De Santi, Guiomar Madureira, Berenice B Mendonca, Tânia A S S Bachega
The primary concern related to CAH newborn screening (NBS) is the high rate of false-positive results (FPR) associated with prematurity; false-negative results (FNR) can also occur due to precocious sample collection OBJECTIVE: To determine the neonatal 17-hydroxyprogesterone (N17OHP) normal range in newborns in Sao Paulo using different references according to age and birth-weight, and to establish the optimal NBS cut-off levels METHODS: N17OHP levels from 271,810 newborns (NBs) according to sample collection time (G1: 48-<72 hs and G2: ≥72 hs) and birth-weight (≤1,500g, 1,501-2,000g, 2,001-2,500 and >2,500g) were evaluated...
December 15, 2016: Clinical Endocrinology
https://www.readbyqxmd.com/read/27977405/monitoring-steroid-replacement-therapy-in-children-with-congenital-adrenal-hyperplasia
#15
Niels H Birkebaek, David M Hougaard, Arieh S Cohen
BACKGROUND: The objective of this study was to compare the analysis of 17-hydroxyprogesterone (17-OHP) by radio-immunoassay (RIA) in serum with analysis by liquid chromatography tandem mass spectrometry (LC-MS/MS) on dried blood spot samples (DBSS) for monitoring therapy in children with congenital adrenal hyperplasia (CAH), and to investigate differences in 17-OHP values during the day. METHODS: Fourteen children (8 females), median age 4.2 (0.3-16.0) years, were studied...
January 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27974295/cardiovascular-health-growth-and-gonadal-function-in-children-and-adolescents-with-congenital-adrenal-hyperplasia
#16
REVIEW
Christiaan F Mooij, Emma A Webb, Hedi L Claahsen van der Grinten, Nils Krone
After the introduction of replacement therapy with glucocorticoids and mineralocorticoids in the 1950s, congenital adrenal hyperplasia (CAH) is no longer a life-limiting condition. However, due to the successful introduction of medical steroid hormone replacement, CAH has become a chronic condition, with associated comorbidities and long-term health implications. The aim of treatment is the replacement of mineralocorticoids and glucocorticoids and the normalisation of elevated androgen concentrations. Long-term consequences of the condition and current treatment regimens include unfavourable changes in the cardiovascular risk profile, impaired growth, testicular adrenal rest tumours (TART) in male and subfertility in both male and female patients with CAH...
December 14, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27967304/histopathological-classification-of-cross-sectional-image-negative-hyperaldosteronism
#17
Yuto Yamazaki, Yasuhiro Nakamura, Kei Omata, Kazue Ise, Yuta Tezuka, Yoshikiyo Ono, Ryo Morimoto, Yukinaga Nozawa, Celso E Gomez-Sanchez, Scott A Tomlins, William E Rainey, Sadayoshi Ito, Fumitoshi Satoh, Hironobu Sasano
CONTEXT: Approximately half of primary aldosteronism (PA) have clinically evident disease according to clinical (hypertension) and/or laboratory (aldosterone and renin levels) findings but do not have nodules detectable in routine cross-sectional imaging. However, the detailed histopathologic, steroidogenic and pathobiological features of cross-sectional image negative PA have not been well characterized. OBJECTIVE: Examine histopathology, steroidogenic enzyme expression and somatic mutation status of aldosterone-driver genes in adrenals from cross-sectional image negative hyperaldosteronism...
December 14, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27967205/gestational-hyperandrogenism-in-developmental-programming
#18
C Hakim, V Padmanabhan, A K Vyas
Androgen excess (hyperandrogenism) is a common endocrine disorder affecting women of reproductive age. Potential causes of androgen excess in women include: polycystic ovary syndrome, congenital adrenal hyperplasia (CAH), adrenal tumors, and racial disparity amongst many others. During pregnancy, luteoma, placental aromatase deficiency, and fetal CAH are additional causes of gestational hyperandrogenism. This paper reviews the various phenotypes of hyperandrogenism during pregnancy, its origin, pathophysiology, and the impact of hyperandrogenism on fetal developmental trajectory and offspring consequences...
December 14, 2016: Endocrinology
https://www.readbyqxmd.com/read/27966633/structure-based-activity-prediction-of-cyp21a2-stability-variants-a-survey-of-available-gene-variations
#19
Carlos D Bruque, Marisol Delea, Cecilia S Fernández, Juan V Orza, Melisa Taboas, Noemí Buzzalino, Lucía D Espeche, Andrea Solari, Verónica Luccerini, Liliana Alba, Alejandro D Nadra, Liliana Dain
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency accounts for 90-95% of CAH cases. In this work we performed an extensive survey of mutations and SNPs modifying the coding sequence of the CYP21A2 gene. Using bioinformatic tools and two plausible CYP21A2 structures as templates, we initially classified all known mutants (n = 343) according to their putative functional impacts, which were either reported in the literature or inferred from structural models. We then performed a detailed analysis on the subset of mutations believed to exclusively impact protein stability...
December 14, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27965122/assembling-a-functional-clitoris-and-vulva-from-a-pseudo-penis-a-surgical-technique-for-an-adult-woman-with-congenital-adrenal-hyperplasia
#20
Wiebren A A Tjalma
BACKGROUND: Congenital adrenal hyperplasia (CAH) is associated with a genital deformation that may cause a negative body image. The genital ambiguity is generally "corrected" surgically during early infancy. The advantage is a psychological benefit. The disadvantages are multiple surgical procedures and the loss of orgasm. CASE: A 22-year-old woman with CAH consulted for genital reconstructive surgery. She had a pseudo-penis of 4 cm and could achieve an orgasm by masturbating...
December 10, 2016: Journal of Pediatric and Adolescent Gynecology
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