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adrenal insufficiency

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https://www.readbyqxmd.com/read/28802067/crh-stimulation-tests-for-the-infants-with-relative-adrenal-insufficiency
#1
Kougoro Iwanaga, Akane Yamamoto, Takashi Matsukura, Fusako Niwa, Masahiko Kawai
BACKGROUND: Very low birth weight (VLBW) infants are considered to be vulnerable to relative adrenal insufficiency (RAI); however, diagnosis is difficult in some clinical settings. Considering this background, it is necessary to establish a diagnosis of RAI in preterm infants. OBJECTIVE: In this study, we attempted to clarify the difference in response to CRH stimulation tests for preterm infants with or without RAI. METHODS: Between June 2009 and December 2015, we performed CRH stimulation tests for preterm infants born at a gestational age of <30 weeks at around two weeks of age...
August 12, 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/28774370/-recurrent-anorexia-and-pigmentation-of-skin-for-more-than-two-months-in-an-infant
#5
Zhang-Qian Zheng, Bing-Bing Wu, Miao-Ying Zhang, Wei Lu, Fei-Hong Luo
A 2-month-old boy presented with adrenal insufficiency, impaired liver function, hypertriglyceridemia, significantly elevated creatine kinase and electrolyte disturbance. Microarray comparative genomic hybridization (aCGH) analysis test showed a pathogenic 8.7 Mb deletion in the short arm of chromosome X (Xp21.3 - p21.1) and confirmed the diagnosis of complex glycerol kinase deficiency (cGKD). He was treated with hydrocortisone, coenzyme Q10 and L-carnitine and was subsequently followed up for 4 years. His serum cortisol levels returned to normal one week later after treatment, but the serum creatine kinase, triglyceride and aminotransferase levels were progressively increased along with mental retardation and decreased muscular strength...
August 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28768968/isolated-adrenocorticotropic-hormone-deficiency-following-chronic-subdural-hematoma-in-an-elderly-man-is-there-a-connection
#6
Satoshi Suzuki, Keiko Suzuki
The delayed diagnosis of adrenal insufficiency is relatively common because its symptoms are non-specific. One of the causes of adrenal insufficiency is isolated adrenocorticotropic hormone deficiency (IAD), which is sometimes caused by traumatic brain injury. Indeed, severe head trauma is considered to contribute to the incidence of this disease. However, the relationship between milder head trauma-such as chronic subdural hematoma - and the occurrence of hormonal deficiency is uncertain. We herein report the case of a 79-year-old man with IAD who presented with leg edema and pain in his extremities following a recent history of chronic subdural hematoma...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28765037/vitamin-d-effects-on-monocytes%C3%AC-ccl-2-il6-and-cd14-transcription-in-addison-s-disease-and-hla-susceptibility
#7
A U Kraus, M Penna-Martinez, G Meyer, K Badenhoop
Addisońs disease is a rare autoimmune disorder leading to adrenal insufficiency and life-long glucocorticoid dependency. Vitamin D receptor (VDR) polymorphisms and vitamin D deficiency predispose to Addisońs disease. Aim of the current study was, to investigate potential anti-inflammatory vitamin D effects on monocytes in Addisońs disease, focusing on inflammatory CCL-2 and IL6, as well on monocyte CD14 markers. Addisońs disease is genetically linked to distinct HLA susceptibility alleles. Therefore we analyzed, whether HLA genotypes differed for vitamin D effects on monocyte markers...
July 29, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28764794/exploration-of-knowledge-and-understanding-in-patients-with-primary-adrenal-insufficiency-a-mixed-methods-study
#8
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/28762136/predicting-cortisol-exposure-from-paediatric-hydrocortisone-formulation-using-a-semi-mechanistic-pharmacokinetic-model-established-in-healthy-adults
#9
Johanna Melin, Zinnia P Parra-Guillen, Niklas Hartung, Wilhelm Huisinga, Richard J Ross, Martin J Whitaker, Charlotte Kloft
BACKGROUND AND OBJECTIVE: Optimisation of hydrocortisone replacement therapy in children is challenging as there is currently no licensed formulation and dose in Europe for children under 6 years of age. In addition, hydrocortisone has non-linear pharmacokinetics caused by saturable plasma protein binding. A paediatric hydrocortisone formulation, Infacort(®) oral hydrocortisone granules with taste masking, has therefore been developed. The objective of this study was to establish a population pharmacokinetic model based on studies in healthy adult volunteers to predict hydrocortisone exposure in paediatric patients with adrenal insufficiency...
July 31, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28759390/cholestasis-and-hepatic-iron-deposition-in-an-infant-with-complex-glycerol-kinase-deficiency
#10
Diana Montoya-Williams, Meredith Mowitz
We present a 6-week-old male infant with persistent hyperbilirubinemia, hypertriglyceridemia, elevated creatine kinase levels, and transaminitis since the second week of life. When he developed hyperkalemia, clinical suspicion was raised for adrenal insufficiency despite hemodynamic stability. A full endocrine workup revealed nearly absent adrenocorticotropic hormone. Coupled with his persistent hypertriglyceridemia (peak of 811 mg/dL) and elevated creatine kinase levels (>20 000 U/L), his corticotropin level lead to a clinical diagnosis of complex glycerol kinase deficiency (GKD), also known as Xp21 deletion syndrome...
July 2017: Pediatrics
https://www.readbyqxmd.com/read/28758924/unusual-onset-of-celiac-disease-and-addison-s-disease-in-a-12-year-old-boy
#11
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
#12
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/28752153/iatrogenic-cushing-s-syndrome-caused-by-intranasal-steroid-use
#13
Fatma Dursun, Heves Kirmizibekmez
Cushing's syndrome (CS) is common after oral steroid use and has also been reported following topical or inhaled use, but it is extremely uncommon after intranasal administration. This is the case of a 6-year-old child who developed Cushing's syndrome after intranasal application of dexamethasone sodium phosphate for a period of 6 months. Pediatricians and other clinical practitioners should be aware that high-dose and long-term nasal steroid administration may cause iatrogenic Cushing's syndrome characterized by complications of glucocorticoid excess as well as serious and even life-threatening complications of adrenal insufficiency...
2017: Northern Clinics of Istanbul
https://www.readbyqxmd.com/read/28750490/secondary-adrenal-insufficiency-from-the-physiopathology-to-the-possible-role-of-modified-release-hydrocortisone-treatment
#14
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/28748191/the-yin-and-yang-between-plasma-glucose-levels-and-cortisol-replacement-therapy-in-schmidt-s-syndrome
#15
Christopher A Newton, Eleni Sheehan, Kathleen Wyne, Kenneth Cusi, Julio Leey, Hans K Ghayee
Objective: To illustrate how steroid replacement in adrenal insufficiency can influence the development of hypoglycemia in a patient with type 1 diabetes mellitus (T1D). Methods: We describe the case of a 36-year-old female patient with T1D and Addison's disease (Schmidt's syndrome) on multiple daily insulin injections who presented with recurrent hypoglycemia despite being on physiological replacement doses of hydrocortisone. Results: With the assistance of continuous glucose monitoring technology, a pattern of nocturnal hypoglycemia was clearly identified...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28748012/-association-between-williams-syndrome-and-adrenal-insufficiency
#16
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
#17
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
#18
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/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
#19
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/28724838/csk-regulates-blood-pressure-by-controlling-the-synthetic-pathways-of-aldosterone
#20
Sung-Moon Kim, Ji-One Kang, Ji Eun Lim, Sue-Yun Hwang, Bermseok Oh
BACKGROUND: Blood pressure is regulated by a network of diverse physiological pathways. The C-terminal Src kinase (CSK) locus (15q24) is associated with blood pressure in various ethnic groups. It was recently reported thatCskinsufficiency increases blood pressure through Src. The mechanisms of hypertension inCsk(+/-)mice are examined further in this study.Methods and Results:To identify a causal component responsible for hypertension inCsk(+/-), the heart rate was measured by electrocardiogram and plasma volume by Evans blue dilution...
July 20, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
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