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https://www.readbyqxmd.com/read/28528327/three-quarters-adrenalectomy-for-infantile-onset-cushing-syndrome-due-to-bilateral-adrenal-hyperplasia-in-mccune-albright-syndrome
#1
Tomoyo Itonaga, Hironori Goto, Manabu Toujigamori, Yasuharu Ohno, Seigo Korematsu, Tatsuro Izumi, Satoshi Narumi, Tomonobu Hasegawa, Kenji Ihara
BACKGROUND: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS...
May 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28512133/quality-of-compounded-hydrocortisone-capsules-used-in-the-treatment-of-children
#2
Uta Neumann, Daniela Burau, Sarah Spielmann, Martin Whitaker, Richard J Ross, Charlotte Kloft, Oliver Blankenstein
Objectives: Due to the lack of paediatric licensed formulations, children are often treated with individualized pharmacy-compounded adult medication. An international web-based survey about the types of medication in children with adrenal insufficiency (AI) revealed that the majority of paediatric physicians are using pharmacy-compounded medication to treat children with AI. Observations of loss of therapy control in children with congenital adrenal hyperplasia with compounded hydrocortisone capsules and regained control after prescribing a new hydrocortisone batch led to this "real world" evaluation of pharmacy-compounded paediatric hydrocortisone capsules...
May 16, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28478756/-addisonian-crisis-induced-by-tacrolimus-and-trimethoprim-in-patient-with-adrenal-insufficiency
#3
Elena García Lobato, Claudia García Lobato, Susana Martín Clavo
No abstract text is available yet for this article.
May 1, 2017: Farmacia Hospitalaria
https://www.readbyqxmd.com/read/28474235/patients-treated-with-immunosuppressive-steroids-are-less-aware-of-sick-day-rules-than-those-on-endocrine-replacement-therapy-and-may-be-at-greater-risk-of-adrenal-crisis
#4
M R Salehmohamed, M Griffin, T Branigan, M Cuesta, C J Thompson
BACKGROUND: Patients taking corticosteroids for immune suppression are vulnerable to adrenal crisis during acute illness or if steroids are stopped abruptly. Although patients treated for adrenal failure in endocrine units are routinely provided with sick day guidelines, we wished to ascertain whether patients on immunosuppressive steroids are appropriately advised. AIM: This study sets out to compare patient awareness of steroid sick day rules in endocrine and non-endocrine patients...
May 4, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28458886/de-novo-disruption-of-promoter-and-exon-1-of-star-gene-reveals-essential-role-for-gonadal-development
#5
Anil Piya, Jasmeet Kaur, Alan M Rice, Himangshu S Bose
SUMMARY: Cholesterol transport into the mitochondria is required for synthesis of the first steroid, pregnenolone. Cholesterol is transported by the steroidogenic acute regulatory protein (STAR), which acts at the outer mitochondrial membrane prior to its import. Mutations in the STAR protein result in lipoid congenital adrenal hyperplasia (CAH). Although the STAR protein consists of seven exons, biochemical analysis in nonsteroidogenic COS-1 cells showed that the first two were not essential for pregnenolone synthesis...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28446514/autoimmune-polyendocrine-syndrome-type-1-in-an-indian-cohort-a-longitudinal-study
#6
Ghazala Zaidi, Vijayalakshmi Bhatia, Saroj Sahoo, Aditya Narayan Sarangi, Niharika Bharti, Li Zhang, Liping Yu, Daniel Eriksson, Sophie Bensing, Olle Kämpe, Nisha Bhavani, Surender K Yachha, Anil Bhansali, Alok Sachan, Vandana Jain, Nalini Shah, Rakesh Aggarwal, Amita Aggarwal, Muthuswany Srinivasan, Sarita Agarwal, Eesh Bhatia
OBJECTIVE: Autoimmune polyendocrine syndrome type 1 (APS 1) is a rare autosomal recessive disorder characterized by progressive organ-specific autoimmunity. There is scant information on APS1 in ethnic groups other than European Caucasians. We studied clinical aspects and autoimmune regulator (AIRE) gene mutations in a cohort of Indian APS1 patients. DESIGN: Twenty-three patients (19 families) from six referral centres in India, diagnosed between 1996-2016, were followed for [median (range)] 4 (0...
April 26, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28444358/primary-adrenal-and-chest-wall-tuberculosis-presenting-as-an-adrenal-crisis
#7
Seol A Jang, Ji Hyun Park, Kyung Ae Lee
No abstract text is available yet for this article.
April 24, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28421928/my-cortisol
#8
(no author information available yet)
My Cortisol is an app that helps to train parents and carers in how to give life-saving injections to children at risk of adrenal crisis.
April 19, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28416079/management-of-childhood-congenital-adrenal-hyperplasia-an-integrative-review-of-the-literature
#9
Louise Fleming, Marcia Van Riper, Kathleen Knafl
Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care, and associated health issues for children with CAH and the family response to the condition. Four literature indexes were searched, with 39 articles included. Four themes emerged: (a) Health- and Development-Related Issues, (a) Effects of Excess Androgens, (c) Life Experience of CAH, and (d) Managing and Averting Adrenal Crisis...
April 13, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28413667/paraganglioma-of-the-bladder-in-a-kidney-transplant-recipient-a-case-report
#10
Hélène Lazareth, Daniel Cohen, Viorel Vasiliu, Claire Tinel, Frank Martinez, Jean-Pierre Grünfeld, Marie-France Mamzer, Christophe Legendre, Rebecca Sberro-Soussan
Renal transplantation has been associated with a significantly increased risk of developing cancer, including bladder neoplasia, with urothelial carcinoma being the most frequent type of bladder cancer. Bladder paraganglioma, also referred to as extra-adrenal pheochromocytoma, is a rare but severe condition that may cause a severe hypertensive crisis during handling and mobilization of the tumor. We herein present the case of a 67-year-old kidney transplant recipient with a bladder polyp consistent with paraganglioma of the bladder...
April 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28392195/clinical-characteristics-of-taiwanese-children-with-congenital-adrenal-hyperplasia-due-to-21-hydroxylase-deficiency-detected-by-neonatal-screening
#11
Shih-Yao Liu, Cheng-Ting Lee, Yi-Ching Tung, Yin-Hsiu Chien, Wuh-Liang Hwu, Wen-Yu Tsai
BACKGROUND/PURPOSE: Neonatal screening for congenital adrenal hyperplasia (CAH) has been conducted in Taiwan since 2000. This study aimed to determine the clinical characteristics of Taiwanese children with CAH due to 21-hydroxylase deficiency (21-OHD) detected by neonatal screening. METHODS: From 2000 to 2015, 26 neonates (14 boys and 12 girls) with classic 21-OHD detected by neonatal screening and confirmed at National Taiwan University Hospital were enrolled...
April 6, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28391254/primary-pigmented-nodular-adrenocortical-disease-literature-review-and-case-report-of-a-6-year-old-boy
#12
Dragan Katanić, Dejan Kafka, Mirjana Živojinov, Jovan Vlaški, Zorana Budakov, Marija Knežević Pogančev, Ivana Vorgučin, Tomislav Ćuk
Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood...
April 10, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28390450/a-complex-interplay-cognitive-behavioural-therapy-for-severe-health-anxiety-in-addison-s-disease-to-reduce-emergency-department-admissions
#13
Jo Daniels, Elizabeth Sheils
BACKGROUND: Addison's disease (AD) is a rare chronic illness caused by adrenocortical insufficiency. Due to the pivotal role of the regulating hormone cortisol in AD, there is a common symptom overlap between the presentation of anxiety and adrenal crisis. Previous literature has identified the prevalence of anxiety in endocrinological disorders, however there is a paucity of research examining the complex interplay between AD and anxiety. AIMS: This paper describes a single case study of a patient with severe health anxiety and co-morbid AD...
April 9, 2017: Behavioural and Cognitive Psychotherapy
https://www.readbyqxmd.com/read/28355719/-a-clinical-analysis-of-123-cases-of-primary-empty-sella
#14
J Li, H W Jia, C L Wang, R Zhang, M Y Qu, W Li, M H Yuan, J Cui, Q He, H Y Wei, T H Zhu, Z S Ma, W Liu, Z L Dong, Z G Gao
Objective: This study was conducted to analyze the clinical characteristics and pituitary function of patients with primary empty sella (PES). Methods: The clinical data from 123 hospitalized adult patients with PES from January 2010 to May 2016 were retrospectively studied. Results: (1) The average age of the 123 (male 43, female 80) PES patients was (59.2±13.6) years (ranging 24-92 years), among whom 61% patients were in the age group between 50-69 years. (2) The symptoms of the patients included fatigue (56...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28320817/symptomatic-adrenal-suppression-among-children-in-canada
#15
Ellen B Goldbloom, Arati Mokashi, Elizabeth A Cummings, Sharon Abish, Susanne M Benseler, Hien Q Huynh, Wade Watson, Alexandra Ahmet
BACKGROUND: Adrenal suppression (AS) is an under-recognised side effect of glucocorticoid (GC) use. AS may go undetected until a physiological stress precipitates an adrenal crisis. The incidence of AS has not been established. We sought to estimate the minimum national incidence and presenting features of paediatric symptomatic AS. METHODS: Through the established methodology of the Canadian Paediatric Surveillance Program, over 2500 paediatricians were surveyed monthly for 2 years (April 2010-March 2012) to report new cases of symptomatic AS...
April 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28256113/clinical-characteristics-of-patients-with-adrenal-insufficiency-in-a-general-hospital
#16
Ye Yeon Lee, Nan Hee Cho, Jong Won Lee, Nam Kyung Kim, Hye Soon Kim, Mi Kyung Kim
BACKGROUND: Adrenal insufficiency (AI) is a life-threatening disorder caused by the deficiency of adrenal steroid hormones. This retrospective cross-sectional study investigated the characteristics of patients with AI in Korea. METHODS: All consecutive patients with suspected AI who received care at a tertiary referral center in Korea in 2014 and underwent adrenocorticotropic hormone stimulation or insulin-tolerance testing were identified through a review of medical charts...
March 2017: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28245538/-improvement-in-hyperglysemia-following-unilateral-adrenalectomy-for-acth-independent-macronodular-adrenal-hyperplasia-aimah-a-case-report
#17
Junichi Ikeda, Kouei Muguruma, Takaaki Inoue, Teruhisa Nishida, Shigenari Kawakita, Takashi Murota, Haruyuki Ohsugi, Nae Takizawa, Hidefumi Kinoshita, Tadashi Matsuda, Amika Noda, Keita Utsunomiya
Adrenal corticotropin (ACTH) -independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is the treatment of choice, but lifetime steroid replacement is essential. Here we report a case of AIMAH whose hyperglycemia was improved following unilateral adrenalectomy. A 42-year-old woman with serious intellectual disability and intractable epilepsy presented with polydipsia. Casual blood glucose and hemoglobin A1c (HbA1c) were 322 mg/dl and 8.5%, respectively...
January 2017: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/28239466/nivolumab-induced-myxedema-crisis
#18
Uqba Khan, Humaira Rizvi, Dahlia Sano, Jane Chiu, Tarik Hadid
BACKGROUND: Nivolumab is an anti-programmed cell death (anti-PD-1) monoclonal antibody that is approved by Food and Drug Administration for treatment of metastatic non-small cell lung cancer, metastatic melanoma, relapsed Hodgkin lymphoma and advanced renal cell cancer. We report a rare case of myxedema crisis induced by nivolumab in a patient with metastatic squamous cell carcinoma of lung. CASE PRESENTATION: Fifty three-year old woman with metastatic squamous cell carcinoma currently on treatment with nivolumab presented with diffuse facial and tongue swelling, slurred speech, depressed mentation, fatigue and weakness...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28217067/septo-optic-dysplasia-de-morsier-s-syndrome
#19
Pedro Reis, Joana Mourão
Septo-optic dysplasia (SOD)/de Morsier's syndrome is characterized by optic nerve hypoplasia, pituitary endocrine dysfunction, and midline brain abnormalities. Hypopituitarism, hypothyroidism, hypogonadism, and adrenal insufficiency can lead to severe hypoglycemia, adrenal crisis, seizures, and sudden death. Anesthetic management of SOD was associated with high perioperative mortality. A 9-year-old male child proposed for dental treatments/extractions. Medical history of SOD with hypopituitarism, hypothyroidism, and delayed psychomotor development was observed...
January 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28197344/from-bad-to-worse-paraganglioma-diagnosis-during-induction-of-labor-for-coexisting-preeclampsia
#20
Sasima Dusitkasem, Blair H Herndon, Dalton Paluzzi, Joseph Kuhn, Robert H Small, John C Coffman
Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features...
2017: Case Reports in Anesthesiology
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