keyword
MENU ▼
Read by QxMD icon Read
search

Primary adrenal insufficiency

keyword
https://www.readbyqxmd.com/read/28815660/absorption-and-tolerability-of-taste-masked-hydrocortisone-granules-in-neonates-infants-and-children-under-6-years-of-age-with-adrenal-insufficiency
#1
Uta Neumann, Martin J Whitaker, Susanna Wiegand, Heiko Krude, John Porter, Madhu Davies, Dena Digweed, Bernard Voet, Richard J Ross, Oliver Blankenstein
OBJECTIVES: There is no licensed, dose-appropriate formulation of hydrocortisone for children with adrenal insufficiency (AI) and patients rely on compounded adult medication. The aim of this study was to evaluate the absorption, palatability and safety of Infacort(®) , an immediate-release, granule formulation of hydrocortisone with taste masking. STUDY DESIGN: Single site with satellites attended by a "flying" doctor from investigator site. Open-label, single-dose study in three consecutive child cohorts (n=24) with AI; Cohort 1, children aged 2 to <6 years (n=12); Cohort 2, infants aged 28 days to <2 years (n=6); Cohort 3, neonates aged 1 to <28 days (n=6)...
August 16, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28794358/the-clinical-and-hormonal-characteristics-of-primary-adrenal-lymphomas-the-necessity-of-early-detection-of-adrenal-insufficiency
#2
Ko Harada, Kosuke Kimura, Masaya Iwamuro, Tomohiro Terasaka, Yoshihisa Hanayama, Eisei Kondo, Eiko Hayashi, Tadashi Yoshino, Fumio Otsuka
Objective To analyze the clinical and endocrine characteristics of patients with primary adrenal lymphoma. Patients We retrospectively reviewed the cases of five patients with primary adrenal lymphoma who were treated in our hospital between April 2004 and March 2015. We investigated the characteristics of the clinical and pathological findings, treatment, prognosis and complications of adrenal insufficiency. Results Adrenal insufficiency, which was confirmed by the laboratory data at the initial presentation, was observed in two cases...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28793974/corticotropin-releasing-factor-receptor-1-antagonism-is-ineffective-for-women-with-posttraumatic-stress-disorder
#3
Boadie W Dunlop, Elisabeth B Binder, Dan Iosifescu, Sanjay J Mathew, Thomas C Neylan, Julius C Pape, Tania Carrillo-Roa, Charles Green, Becky Kinkead, Dimitri Grigoriadis, Barbara O Rothbaum, Charles B Nemeroff, Helen S Mayberg
BACKGROUND: Medication and psychotherapy treatments for posttraumatic stress disorder (PTSD) provide insufficient benefit for many patients. Substantial preclinical and clinical data indicate abnormalities in the hypothalamic-pituitary-adrenal axis, including signaling by corticotropin-releasing factor, in the pathophysiology of PTSD. METHODS: We conducted a double-blind, placebo-controlled, randomized, fixed-dose clinical trial evaluating the efficacy of GSK561679, a corticotropin-releasing factor receptor 1 (CRF1 receptor) antagonist in adult women with PTSD...
July 4, 2017: Biological Psychiatry
https://www.readbyqxmd.com/read/28780517/mitotane-effects-on-the-hypothalamic-pituitary-adrenal-axis-in-patients-with-adrenocortical-carcinoma
#4
Giuseppe Reimondo, Soraya Puglisi, Barbara Zaggia, Vittoria Basile, Laura Saba, Paola Perotti, Silvia De Francia, Marco Volante, Maria Chiara Zatelli, Salvatore Cannavò, M Terzolo
OBJECTIVE: Mitotane, a drug used to treat adrenocortical cancer (ACC), inhibits multiple enzymatic steps of adrenocortical steroid biosynthesis, potentially causing adrenal insufficiency. Recent studies in vitro have also documented a direct inhibitory effect of mitotane at the pituitary level. The present study was aimed to assess the hypothalamic pituitary adrenal axis in ACC patients receiving mitotane. DESIGN AND METHODS: We prospectively enrolled 16 patients on adjuvant treatment with mitotane after radical surgical resection of ACC, who underwent standard hormone evaluation and h-CRH stimulation...
August 5, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28764794/exploration-of-knowledge-and-understanding-in-patients-with-primary-adrenal-insufficiency-a-mixed-methods-study
#5
L M Shepherd, A A Tahrani, C Inman, W Arlt, D M Carrick-Sen
BACKGROUND: Primary adrenal insufficiency (PAI) is a rare and severe condition requiring lifelong steroid replacement. During acute illness or stressful events, it is important to appropriately adjust glucocorticoid dose; failure to do so may lead to an adrenal crisis. The aim of the study was to explore patients PAI knowledge and understanding of the condition, steroid replacement adjustment during acute illness or stress and provided education. METHODS: Ten adult patients with PAI were purposefully recruited from two hospitals in a tertiary NHS Trust in England, UK...
August 1, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28758924/unusual-onset-of-celiac-disease-and-addison-s-disease-in-a-12-year-old-boy
#6
Francesco Miconi, Emanuela Savarese, Giovanni Miconi, Gabriele Cabiati, Valentina Rapaccini, Nicola Principi, Susanna Esposito
BACKGROUND: Celiac disease (CD) is an autoimmune disorder deriving from an aberrant adaptive immune response against gluten-containing grains in genetically predisposed subjects. In a number of patients, CD is associated with one or more other autoimmune diseases. Primary Addison's disease (AD) and CD may co-exist, although this association is relatively uncommon in children. In addition, it is not precisely defined whether a gluten-free diet influences the course of AD. CASE PRESENTATION: A case of CD in a 12-year-old boy presenting as acute adrenal insufficiency is described here...
July 29, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28757874/a-rare-case-of-primary-bilateral-adrenal-lymphoma
#7
Veeraraghavan Meyyur Aravamudan, Phang Kee Fong, Yang Shiyao Sam, Pavel Singh, Siok-Bian Ng, Gollamudi Satya Pavan Kumar
Lymphoma may involve the adrenal glands, but primary lymphoma is rare. Only a few cases have been reported in medical literature. Primary adrenal lymphoma is extremely rare, accounting for <1% of non-Hodgkin lymphomas. We here present a case of a middle-aged female who presented with persistent fever for three weeks. She also reported significant weight loss of more than 10 kgs over the duration of three months. Computed tomography of the thorax and abdomen and pelvis demonstrated bilateral adrenal masses...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28750490/secondary-adrenal-insufficiency-from-the-physiopathology-to-the-possible-role-of-modified-release-hydrocortisone-treatment
#8
Rosa M Paragliola, Salvatore M Corsello
Central adrenal insufficiency represents a life-threatening disorder that results from a reduced cortisol production due to an impairment production of adrenocorticotropic hormone. In particular, secondary AI results from pituitary disease that impedes the release of ACTH, while tertiary adrenal insufficiency is caused from an impaired synthesis of corticotropin-releasing hormone. Central adrenal insufficiency has an estimated prevalence of 150-280 per million, resulting more common than primary AI. Prompt diagnosis and management of this condition is crucial, but the diagnostic investigation can often be challenging, in particular in cases of recent onset of secondary adrenal insufficiency...
July 27, 2017: Minerva Endocrinologica
https://www.readbyqxmd.com/read/28748012/-association-between-williams-syndrome-and-adrenal-insufficiency
#9
Meryem Rchachi, Maazou Mahamane Larwanou, Hanan El Ouahabi, Farida Ajdi
Williams syndrome is a developmental disorder including dysmorphia, cardiovascular malformations and a specific neuropsychological profile together with other associated disorders. We report the case of a 17-year old girl, born of a non-inbred marriage, with Williams syndrome discovered during an assessment of degree of failure to thrive. Its association with primary adrenal insufficiency makes it unique. Diagnosis is confirmed by cytogenetic and molecular analysis. Its management consists of the implementation of treatment for adrenal insufficiency associated with a clinico-biological monitoring...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28741070/late-onset-x-linked-adrenal-hypoplasia-dax-1-nr0b1-two-new-adult-onset-cases-from-a-single-center
#10
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/28689853/identification-of-patients-with-primary-ovarian-insufficiency-caused-by-autoimmunity
#11
Jing Gao, Xue Jiao, Yujie Dang, Jing Li, Guiyu Li, Ting Han, Yixun Liu, Yingying Qin, Zi-Jiang Chen
Autoimmune pathogenesis is responsible for a subset of primary ovarian insufficiency (POI) cases. The significance of autoantibodies for POI, however, remains unclear. A total of 250 women with idiopathic POI and 256 age-matched healthy women were enrolled. The presence in serum of adrenal cortex autoantibody (AAA), detected by indirect immunofluorescence and non-organ-specific antibodies, including antinuclear antibody, anti-cardiolipin antibody, and anti-double stranded DNA antibody, detected by enzyme-linked immunosorbent assay, was compared...
June 21, 2017: Reproductive Biomedicine Online
https://www.readbyqxmd.com/read/28685093/bilateral-primary-adrenal-diffuse-large-b-cell-lymphoma-without-adrenal-insufficiency-a-case-report-and-review-of-the-literature
#12
Peijie Chen, Lu Jin, Yu Yang, Liangchao Ni, Shangqi Yang, Yongqing Lai
Primary adrenal lymphoma (PAL) is an infrequent malignant tumor, occurring in the bilateral adrenal glands as a mass in the majority of cases. The current study presents a case of bilateral primary adrenal diffuse large B cell lymphoma in a 52-year-old female patient, who presented with abdominal pain in the left lumbar region for ~2 weeks. Abdominal ultrasound examination and computed tomography scanning revealed a mass of 132×119×101 mm on the left adrenal gland and a mass of 53×27 mm on the right adrenal gland...
July 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28676275/testicular-adrenal-rest-tumor-tart-in-congenital-adrenal-hyperplasia
#13
Hatice Ozisik, Banu Sarer Yurekli, Ilgin Yildirim Simsir, Ilker Altun, Utku Soyaltin, Ezgi Guler, Huseyin Onay, Banu Sarsik, Fusun Saygili
Congenital adrenal hyperplasia is one of the most common autosomal recessive genetic disorders. Testicular adrenal tumors are significant complications of congenital adrenal hyperplasia. We would like to present two patients of testicular adrenal rest tumors. Patient 1 24 year-old male, he was diagnosed with congenital adrenal hyperplasia at the age of 8 due to precocious puberty. He received hydro-cortisone treatment until the age of 18. Testicular mass had been detected and right radical orchiectomy had been applied 6 months ago and reported as testicular adrenal rest tumor...
July 1, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28666219/lipid-induced-endoplasmic-reticulum-stress-in-x-linked-adrenoleukodystrophy
#14
Malu-Clair van de Beek, Rob Ofman, Inge Dijkstra, Frits Wijburg, Marc Engelen, Ronald Wanders, Stephan Kemp
X-linked adrenoleukodystrophy (ALD) is a progressive neurodegenerative disease that is caused by mutations in the ABCD1 gene and characterized by elevated levels of very long-chain fatty acids (VLCFA) in plasma and tissues, with the most pronounced increase in the central nervous system. Virtually all male patients develop adrenal insufficiency and myelopathy (adrenomyeloneuropathy), but a subset develops a fatal cerebral demyelinating disease (known as cerebral ALD). Female patients may also develop myelopathy, but adrenal insufficiency or leukodystrophy are very rare...
June 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28661915/endocrine-dysfunction-following-immune-checkpoint-inhibitor-therapy
#15
Bhavana Konda, Fadi Nabhan, Manisha H Shah
PURPOSE OF REVIEW: Immune checkpoint inhibitors (ICI) represent an important milestone in the modern era of antineoplastic therapy and have ushered optimism amongst oncologists and patients alike. These agents, however, are associated with significant potential toxicities, the importance of which cannot be overstated. The clinical presentation, diagnosis, and management strategies of immune-related endocrinopathies associated with ICI use are described in this case-based review. RECENT FINDINGS: An increasing number of ICI have shown promise in the management of various malignancies in the recent years...
June 28, 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/28637490/lipoid-congenital-adrenal-hyperplasia-by-steroidogenic-acute-regulatory-protein-star-gene-mutation-in-an-italian-infant-an-uncommon-cause-of-adrenal-insufficiency
#16
Carla Bizzarri, Elisa Pisaneschi, Mafalda Mucciolo, Stefania Pedicelli, Daniela Galeazzi, Antonio Novelli, Marco Cappa
BACKGROUND: Lipoid congenital adrenal hyperplasia (CAH) (OMIM n. 201710) is the most severe form of congenital adrenal hyperplasia. It is characterized by severe adrenal and gonadal steroidogenesis impairment due to a defect in the conversion of cholesterol to pregnenolone. Affected infants experience salt loss, but glucocorticoid and mineralocorticoid replacement therapy enables long-term survival. Classic lipoid congenital adrenal hyperplasia is relatively common in Japan and Korea but extremely rare in Caucasian populations...
June 20, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28624161/clinical-follow-up-of-the-first-sf-1-insufficient-female-patient
#17
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
#18
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
#19
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
#20
REVIEW
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
keyword
keyword
96363
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"