16,294 results match your criteria Adrenal Insufficiency & Adrenal Crisis


Nonparathyroid Hypercalcemia.

Authors:
David Goltzman

Front Horm Res 2019 19;51:77-90. Epub 2018 Nov 19.

Primary hyperparathyroidism is among the most common causes of hypercalcemia. However, ingestion of medication, including hydrochlorathiazide, lithium, and foscarnet, excessive vitamin A ingestion, endocrinopathies such as hyperthyroidism, adrenal insufficiency, and acromegaly, abnormal nutrient intake such as parenteral nutrition in preterm infants and milk-alkali syndrome, and prolonged immobilization have all been associated with hypercalcemia. The most common cause of nonparathyroid hypercalcemia is neoplasia. Read More

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http://dx.doi.org/10.1159/000491040DOI Listing
November 2018

Pituitary dysfunction due to sports-related traumatic brain injury.

Pituitary 2019 Jan 14. Epub 2019 Jan 14.

Memorial Kayseri Hospital, Endocrinology Clinic, Kayseri, Turkey.

Purpose: After traumatic brain injury was accepted as an important etiologic factor of pituitary dysfunction (PD), awareness of risk of developing PD following sports-related traumatic brain injury (SR-TBI) has also increased. However there are not many studies investigating PD following SR-TBIs yet. We aimed to summarize the data reported so far and to discuss screening algorithms and treatment strategies. Read More

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http://dx.doi.org/10.1007/s11102-019-00937-zDOI Listing
January 2019

Recovery of the adrenal function after pituitary surgery in patients with Cushing Disease: persistent remission or recurrence?

Neuroendocrinology 2019 Jan 13. Epub 2019 Jan 13.


Background Cushing's disease (CD) represents the principal cause of endogenous hypercortisolism. The first-line therapy of CD is surgical removal of the ACTH secreting pituitary adenoma, which is generally followed by adrenal insufficiency (AI). Objective To analyse the recovery of the AI in patients with CD after pituitary surgery in relation with recurrence and persistent remission of CD. Read More

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http://dx.doi.org/10.1159/000496846DOI Listing
January 2019
1 Read

Catastrophic antiphospholipid syndrome causing ST-segment elevation myocardial infarction with non-obstructive coronary arteries.

BMJ Case Rep 2019 Jan 10;12(1). Epub 2019 Jan 10.

Cardiology, West Suffolk NHS Foundation Trust, Bury Saint Edmunds, UK.

A 51-year-old woman with known primary antiphospholipid syndrome presented with a 4-day history of chest and abdominal pain, inferior ST-segment elevation on a 12-lead ECG and a subtherapeutic international normalised ratio. In view of a significantly raised high-sensitivity troponin I assay, inferior wall hypokinesis on transthoracic echocardiography and despite unobstructed epicardial vessels on emergency coronary angiography, a diagnosis of myocardial infarction was made. Furthermore, the patient also developed both bilateral adrenal haemorrhages leading to acute adrenal insufficiency and microvascular thrombotic renal disease concurrently. Read More

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http://dx.doi.org/10.1136/bcr-2018-225495DOI Listing
January 2019

ATP-binding cassette transporter G1 deficiency is associated with mild glucocorticoid insufficiency in mice.

Biochim Biophys Acta Mol Cell Biol Lipids 2019 Jan 8. Epub 2019 Jan 8.

Division of BioTherapeutics, Leiden Academic Centre for Drug Research, Gorlaeus Laboratories, Einsteinweg 55, 2333CC Leiden, the Netherlands.

Objective: Since cholesterol is the sole precursor for glucocorticoid synthesis, it is hypothesized that genetic defects in proteins that impact the cellular cholesterol pool may underlie glucocorticoid insufficiency in humans. In the current study, we specifically focused on the cholesterol efflux mediator ATP-binding cassette transporter G1 (ABCG1) as gene candidate.

Methods: The adrenal transcriptional response to fasting stress was measured in wild-type mice to identify putative novel gene candidates. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S13881981183020
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http://dx.doi.org/10.1016/j.bbalip.2019.01.003DOI Listing
January 2019
1 Read

Acute Adrenal Insufficiency After an Antiemetic Dose of Dexamethasone: A Case Report.

A A Pract 2019 Jan 3. Epub 2019 Jan 3.

Miami Beach Anesthesiology Associates, Inc, Department of Anesthesiology, Mount Sinai Medical Center, Miami Beach, Florida.

Dexamethasone is widely used in current practice for the prevention of postoperative nausea and vomiting. Although its use in the perioperative setting has been associated with several side effects (eg, hyperglycemia and suppression of adrenal cortisol production), their clinical significance remains questionable. We present the case of a healthy 27-year-old woman who developed acute adrenal insufficiency after receiving intraoperative dexamethasone for postoperative nausea and vomiting prophylaxis during a laparoscopic left oophorectomy. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000953DOI Listing
January 2019

Supplemental Glucocorticoids and Anesthesia for Non-Invasive Indications in Children with Central Adrenal Insufficiency: A Retrospective Study.

Paediatr Anaesth 2019 Jan 10. Epub 2019 Jan 10.

Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, USA.

Adrenal insufficiency is characterized by the deficiency of circulating adrenocortical hormones and can be primary or central. Primary adrenal insufficiency is due to disease of the adrenal cortex, central adrenal insufficiency to a deficit in hypothalamic/pituitary secretion of adrenocorticotropic hormone. Patients with adrenal insufficiency require basal steroid replacement and need supplemental glucocorticoids (GC) during periods of medical stress. Read More

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http://doi.wiley.com/10.1111/pan.13586
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http://dx.doi.org/10.1111/pan.13586DOI Listing
January 2019
1 Read

[Adrenocorticotropic hormone (ACTH) insensitivity syndrome: about a case].

Pan Afr Med J 2018 2;30:244. Epub 2018 Aug 2.

Centre Hospitalier National d'Enfants Albert Royer-Fann, Dakar, Sénégal.

Adrenocorticotropic hormone (ACTH) insensitivity syndrome is one of the rare causes of adrenal insufficiency in children. All described inherited ACTH insensitivity forms are of autosomal recessive origin. In our resource-poor Countries, many of these rare diseases are ignored or not diagnosed due to inadequate technical equipments. Read More

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http://dx.doi.org/10.11604/pamj.2018.30.244.15541DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307927PMC

Suprasellar chordoid glioma: a report of two cases.

Arch Endocrinol Metab 2018 ;62(6):648-654

Instituto de Investigaciones Neurológicas "Dr. Raúl Carrea", FLENI, Pathology, Buenos Aires, Argentina.

Chordoid glioma (CG) is considered a slow growing glial neoplasm. We report two new cases with endocrinological presentation, management and outcome. Case reports: 1) An 18 year-old female patient was admitted due to headaches, nausea and vomiting and visual abnormalities. Read More

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http://dx.doi.org/10.20945/2359-3997000000092DOI Listing
January 2018
1 Read

Organ-sparing procedures in GU cancer: part 1-organ-sparing procedures in renal and adrenal tumors: a systematic review.

Int Urol Nephrol 2019 Jan 8. Epub 2019 Jan 8.

Department of Urology, Baylor College of Medicine and Michael E. De Bakey VA Medical Center, Houston, TX, USA.

Purpose: Organ-sparing surgery (OSS) for the kidney and adrenals has emerged as the need for preservation of function is paramount in patients with poor functional reserve. As reports increasingly showed that oncological outcomes were equivalent to radical excision, elective OSS became a viable alternative in patients with otherwise normal reserve. In this review, we summarize the current knowledge of OSS for adrenal and renal tumors. Read More

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http://dx.doi.org/10.1007/s11255-018-02070-5DOI Listing
January 2019

Adrenal gland disorders, active component, U.S. Armed Forces, 2002-2017.

MSMR 2018 Dec;25(12):10-19

During 2002-2017, the most common incident adrenal gland disorder among male and female service members was adrenal insufficiency and the least common was adrenomedullary hyperfunction. Adrenal insufficiency was diagnosed among 267 females (crude overall incidence rate: 8.2 cases per 100,000 person-years [p-yrs]) and 729 males (3. Read More

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December 2018

Predicted Benign and Synonymous Variants in Cause Primary Adrenal Insufficiency Through Missplicing.

J Endocr Soc 2019 Jan 30;3(1):201-221. Epub 2018 Oct 30.

Centre for Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, United Kingdom.

Primary adrenal insufficiency (PAI) is a potentially life-threatening condition that can present with nonspecific features and can be difficult to diagnose. We undertook next generation sequencing in a cohort of children and young adults with PAI of unknown etiology from around the world and identified a heterozygous missense variant (rs6161, c.940G>A, p. Read More

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http://dx.doi.org/10.1210/js.2018-00130DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6316989PMC
January 2019

Identification and Functional Analysis of Six Mutations in Patients With X-Linked Adrenal Hypoplasia Congenita.

J Endocr Soc 2019 Jan 12;3(1):171-180. Epub 2018 Dec 12.

Center of Excellence for Medical Genetics, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Context: () mutations cause X-linked adrenal hypoplasia congenita (AHC) and hypogonadotropic hypogonadism (HH) in affected male patients. Affected individuals typically present with early-onset adrenal insufficiency and develop HH during puberty. Rare cases can present with late-onset adrenal insufficiency or other unusual phenotypes. Read More

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http://dx.doi.org/10.1210/js.2018-00270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6316980PMC
January 2019

Adrenal hypoplasia congenita in identical twins.

Saudi Med J 2019 Jan;40(1):87-92

Unit of Pediatric Endocrinology and Diabetes, Maternity and Children Hospital, Dammam, Kingdom of Saudi Arabia. E-mail.

We are presenting a monozygotic twin brothers presented at different ages with different presentations. Twin-A presented at age of 18 days with salt losing crisis. Investigations revealed high plasma renin with low-normal aldosterone. Read More

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http://dx.doi.org/10.15537/smj.2019.1.23337DOI Listing
January 2019

Initial free cortisol dynamics following blunt multiple trauma and traumatic brain injury: A clinical study.

J Int Med Res 2019 Jan 7:300060518819603. Epub 2019 Jan 7.

1 Department of Trauma Surgery, Ludwig Maximilians University Munich, Munich, Germany.

Objective: To determine free and total cortisol serum concentrations in the first 24 h after trauma and to evaluate the influence of traumatic brain injury (TBI) on their dynamics.

Methods: This prospective cohort study enrolled patients who had experienced multiple trauma and were admitted to a level 1 trauma centre. The patients were divided in two groups based on the presence of TBI according to clinical and radiological findings. Read More

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http://dx.doi.org/10.1177/0300060518819603DOI Listing
January 2019

Management of Adrenal Insufficiency Risk After Long-term Systemic Glucocorticoid Therapy in Duchenne Muscular Dystrophy: Clinical Practice Recommendations.

J Neuromuscul Dis 2019 Jan 2. Epub 2019 Jan 2.

Department of Pediatrics, Division of Endocrinology, University of Colorado School of Medicine, Aurora, Colorado, USA.

Long-term glucocorticoid therapy has improved outcomes in patients with Duchenne muscular dystrophy. However, the recommended glucocorticoid dosage suppresses the hypothalamic-pituitary-adrenal axis, leading to adrenal insufficiency that may develop during severe illness, trauma or surgery, and after discontinuation of glucocorticoid therapy. The purpose of this review is to highlight the risk of adrenal insufficiency in this patient population, and provide practical recommendations for management of adrenal insufficiency, glucocorticoid withdrawal, and adrenal function testing. Read More

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https://www.medra.org/servlet/aliasResolver?alias=iospress&a
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http://dx.doi.org/10.3233/JND-180346DOI Listing
January 2019
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Triple A syndrome: two siblings with a novel mutation in the AAAS gene.

Hormones (Athens) 2019 Jan 5. Epub 2019 Jan 5.

Eunice Kennedy Shriver National Institute of Child Health and Human Development, 10 Center Drive, Building 10, Room 1-3330, Bethesda, MD, 20892, USA.

Objective: Triple A syndrome is a rare autosomal recessive disorder caused by mutations in the AAAS gene on chromosome 12q13. Its main clinical features are alacrima, achalasia, and adrenal insufficiency, with most patients also having neurological symptoms and autonomic dysfunction. The neurologic manifestations are less well-understood, especially in children. Read More

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http://dx.doi.org/10.1007/s42000-018-0089-2DOI Listing
January 2019

Management of blunt adrenal gland injury in a community-based hospital.

Injury 2018 Dec 28. Epub 2018 Dec 28.

Department of Surgery, College of Medicine and Health Sciences, UAE University, Al-Ain, United Arab Emirates.

Introduction: Injury of the adrenal gland in blunt trauma is rare. The routine usage of the whole body computed tomography (CT) scan helps in early diagnosis. We aimed to study the incidence, mechanism of injury, management, and outcome of adrenal injury in blunt trauma patients treated in a community-based hospital. Read More

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http://dx.doi.org/10.1016/j.injury.2018.12.033DOI Listing
December 2018
1 Read

Successful treatment of severe arterial hypotension and anuria in a preterm infant with renal tubular dysgenesis- a case report.

Matern Health Neonatol Perinatol 2018 20;4:27. Epub 2018 Dec 20.

University Children's Hospital Würzburg, Josef-Schneider-Straße 2, 97080 Würzburg, Germany.

Background: Oligohydramnios sequence can be caused by renal tubular dysgenesis (RTD), a rare condition resulting in pulmonary and renal morbidity. Besides typical features of Potter-sequence, the infants present with severe arterial hypotension and anuria as main symptoms. Establishing an adequate arterial blood pressure and sufficient renal perfusion is crucial for the survival of these infants. Read More

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http://dx.doi.org/10.1186/s40748-018-0095-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302415PMC
December 2018

Graves' Thyrotoxicosis Leading to Adrenal Decompensation and Hyperandrogenemia in a Pediatric Patient with Salt-Wasting Congenital Adrenal Hyperplasia.

Case Rep Endocrinol 2018 22;2018:2359205. Epub 2018 Nov 22.

Pediatric Endocrinology, Rhode Island Hospital/The Warren Alpert Medical School of Brown University, 111 Plain Street, 3rd Floor, Providence, RI 02903, USA.

Introduction: Thyroid hormone is known to accelerate glucocorticoid turnover. In a thyrotoxic state, individuals with adrenal insufficiency are unable to increase endogenous cortisol production to compensate for increased turnover, placing them at risk for symptoms of glucocorticoid deficiency and adrenal crisis. In patients with salt-wasting congenital adrenal hyperplasia (SW-CAH), hyperandrogenemia is a measurable reflection of relative glucocorticoid insufficiency. Read More

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http://dx.doi.org/10.1155/2018/2359205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282132PMC
November 2018
1 Read

Unilateral Adrenalectomy Could Be a Valid Option for Primary Nodular Adrenal Disease: Evidence From Twins.

J Endocr Soc 2019 Jan 24;3(1):129-134. Epub 2018 Oct 24.

Division of Endocrinology, Hôpital Erasme, Brussels, Belgium.

Primary pigmented nodular adrenal disease (PPNAD) accounts for <1% of ACTH-independent Cushing syndrome. We describe the case of twin female patients with PPNAD who both had sustainable disease control after unilateral adrenalectomy, which corroborates current evidence in favor of unilateral adrenalectomy for a subset of patients with PPNAD. Patient A presented with a 10-kg weight gain over the past year and facial plethora. Read More

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http://dx.doi.org/10.1210/js.2018-00261DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302904PMC
January 2019

Mycobacterium brisbanense lung infection facilitated by steroid induced adrenal insufficiency.

APMIS 2018 Dec 25. Epub 2018 Dec 25.

Department of Infectious Diseases, Rigshospitalet, Copenhagen, Denmark.

Mycobacterium brisbanense is rapid-growing nontuberculous mycobacteria. It was first described in 2004 as a human pathogen and only one case report has previously been published. We report a case of M. Read More

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http://dx.doi.org/10.1111/apm.12911DOI Listing
December 2018

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency.

Eur J Endocrinol 2018 Dec 1. Epub 2018 Dec 1.

H Falhammar, Depatment of Endocrinology, Metabolism and Diabetes, Karolinska Universitetssjukhuset, Stockholm, 171 76, Sweden.

Non-classic congenital adrenal hyperplasia (NCAH) is a relatively common disorder regardless of ethnicity, but most cases are never diagnosed, especially in males. A baseline 17-hydroxyprogesterone measurement may be used for screening, but 17-hydroxyprogesterone measurement after ACTH-stimulation is the gold standard. We advocate a CYP21A2 mutation analysis to verify the diagnosis, for genetic counselling and for better prognostic and treatment guidance. Read More

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http://dx.doi.org/10.1530/EJE-18-0712DOI Listing
December 2018

An association with hypopituitarism and 9q subtelomere deletion syndrome.

Clin Case Rep 2018 Dec 25;6(12):2371-2375. Epub 2018 Oct 25.

Department of Pediatrics Keio University School of Medicine Tokyo Japan.

Hypopituitarism could have been overlooked so far in the patients with 9q subtelomere deletion syndrome (9qSTDS); thus, further investigations or reevaluation of clinical information, especially hormonal evaluations, are warranted to determine whether hypopituitarism is a rare or relatively common presentation in patients with 9qSTDS. Read More

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http://dx.doi.org/10.1002/ccr3.1591DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293262PMC
December 2018

An Update on Addison's Disease.

Exp Clin Endocrinol Diabetes 2018 Dec 18. Epub 2018 Dec 18.

Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany.

Addison's disease - the traditional term for primary adrenal insufficiency (PAI) - is defined as the clinical manifestation of chronic glucocorticoid- and/or mineralocorticoid deficiency due to failure of the adrenal cortex which may result in an adrenal crisis with potentially life-threatening consequences. Even though efficient and safe pharmaceutical preparations for the substitution of endogenous gluco- and mineralocorticoids are established in therapy, the mortality in patients with PAI is still increased and the health-related quality of life (HRQoL) is often reduced.PAI is a rare disease but recent data report an increasing prevalence. Read More

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http://dx.doi.org/10.1055/a-0804-2715DOI Listing
December 2018
7 Reads
1.760 Impact Factor

"Migratory" Pattern of Corticosteroid-Related Multifocal Bone Infarction on Bone Scintigraphy.

Clin Nucl Med 2019 Feb;44(2):155-156

Department of Diagnostic Radiology, Mayo Clinic, Scottsdale, AZ.

We present serial bone scintigraphy examinations in a 38-year-old woman who presented with posterior left rib pain in the setting of adrenal insufficiency secondary to pituitary damage as a result of radiation treatment for orbital sarcoma. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002403DOI Listing
February 2019

Glucocorticoid management of adrenal insufficiency in the UK: assessment using real-world data.

Endocr Connect 2018 Dec 1. Epub 2018 Dec 1.

R Petermann, Shire Pharmaceuticals Limited, Vienna, Austria.

Background And Objectives: Glucocorticoids are used to manage adrenal insufficiency (AI). We describe treatments used in the UK and real-world clinical outcomes for each treatment.

Methods: We used 2010-2016 primary care data from The Health Improvement Network (THIN). Read More

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http://dx.doi.org/10.1530/EC-18-0418DOI Listing
December 2018

Umbralisib in combination with ibrutinib in patients with relapsed or refractory chronic lymphocytic leukaemia or mantle cell lymphoma: a multicentre phase 1-1b study.

Lancet Haematol 2019 Jan 14;6(1):e38-e47. Epub 2018 Dec 14.

Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.

Background: Patients with relapsed or refractory high-risk chronic lymphocytic leukaemia or mantle cell lymphoma often do not derive durable benefit from ibrutinib monotherapy. We hypothesised that dual B-cell receptor pathway blockade would be tolerable and efficacious. We investigated a next-generation phosphoinositide-3-kinase-δ inhibitor (PI3K-δi), umbralisib, plus a Bruton tyrosine kinase inhibitor (BTKi), ibrutinib, in relapsed or refractory chronic lymphocytic leukaemia and mantle cell lymphoma. Read More

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http://dx.doi.org/10.1016/S2352-3026(18)30196-0DOI Listing
January 2019

Short-Term Steroid Regimen for Adult Steroid-Sensitive Minimal Change Disease.

Am J Nephrol 2018 Dec 17;49(1):54-63. Epub 2018 Dec 17.

Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya,

Background: In pediatric patients with steroid-sensitive nephrotic syndrome, recent trials have revealed that a 2-month, short-term steroid regimen is not inferior to an extended steroid course. However, the optimal duration of initial steroid therapy for adult steroid-sensitive minimal change disease (MCD) remains unclear.

Objectives: The aim of present study was to evaluate the effectiveness of a 2-month, short-term steroid regimen in the treatment of adult steroid-sensitive MCD patients. Read More

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http://dx.doi.org/10.1159/000495352DOI Listing
December 2018
5 Reads

Primary hypophysitis and other autoimmune disorders of the sellar and suprasellar regions.

Rev Endocr Metab Disord 2018 Dec 13. Epub 2018 Dec 13.

Medicover GmbH, Berlin/Hannover, Germany.

The pituitary gland and the hypothalamus can be affected by autoimmune-mediated structural and functional disruption. These autoimmune-mediated diseases occur more commonly in females and are often found during pregnancy or in the post-partum period. Autoimmune diseases can either affect parts of the pituitary or hypothalamus, or can involve both sellar and suprasellar structures. Read More

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http://link.springer.com/10.1007/s11154-018-9480-1
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http://dx.doi.org/10.1007/s11154-018-9480-1DOI Listing
December 2018
4 Reads

Late adult-onset adrenomyeloneuropathy evolving with atypical severe frontal lobe syndrome: Importance of neuroimaging.

Radiol Case Rep 2019 Mar 5;14(3):309-314. Epub 2018 Dec 5.

Department of Medical, Surgical, Neurological, Metabolic Sciences, and Aging, 2nd Division of Neurology, Center for Rare Diseases and InterUniversity Center for Research in Neurosciences, University of Campania "Luigi Vanvitelli", Via Sergio Pansini, 80131 Naples, Italy.

X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease affecting the nervous system and the adrenal glands. It is caused by a mutation of the gene, resulting in the impaired degradation of very long-chain fatty acids and their subsequent accumulation in several organs and tissues. X-ALD is notable for its high phenotypical variability, that includes isolated adrenocortical insufficiency, slowly progressive myelopathy with paraparesis, ataxia, and peripheral neuropathy to severe childhood cerebral forms. Read More

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http://dx.doi.org/10.1016/j.radcr.2018.11.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282458PMC

Case report of dysregulation of primary bile acid synthesis in a family with X-linked adrenoleukodystrophy.

Medicine (Baltimore) 2018 Dec;97(49):e13353

Department of Clinical Biochemistry, Jagiellonian University Medical College, Krakow, Poland.

Rationale: X-linked adrenoleukodystrophy (X-ALD) is a rare disorder caused by mutations in the ABCD1 gene, coding for peroxisomal membrane transporter adrenoleukodystrophy (ALD) protein. The disease is characterized by accumulation of very long chain fatty acids (VLCFAs) in tissues. Adult adrenomyeloneuropathy (AMN) and the cerebral inflammatory form of ALD are the main phenotypes presenting various symptoms. Read More

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http://dx.doi.org/10.1097/MD.0000000000013353DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310492PMC
December 2018

Steroidogenic differentiation and PKA signaling are programmed by histone methyltransferase EZH2 in the adrenal cortex.

Proc Natl Acad Sci U S A 2018 Dec 12;115(52):E12265-E12274. Epub 2018 Dec 12.

Génétique Reproduction & Développement, CNRS UMR 6293, Inserm U1103, Université Clermont Auvergne, 63001 Clermont-Ferrand, France;

Adrenal cortex steroids are essential for body homeostasis, and adrenal insufficiency is a life-threatening condition. Adrenal endocrine activity is maintained through recruitment of subcapsular progenitor cells that follow a unidirectional differentiation path from zona glomerulosa to zona fasciculata (zF). Here, we show that this unidirectionality is ensured by the histone methyltransferase EZH2. Read More

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http://dx.doi.org/10.1073/pnas.1809185115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310834PMC
December 2018

Significant Neutrophilic Emperipolesis in Squamous Cell Carcinoma.

Case Rep Oncol Med 2018 13;2018:1301562. Epub 2018 Nov 13.

Department of Ear, Nose, and Throat Disease, Bakırköy Sadi Konuk Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.

A 53-year-old man was admitted for tooth mobility. A mass was identified at the tooth base by CT. Histopathology of the excisional biopsy revealed a moderately differentiated squamous cell carcinoma. Read More

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http://dx.doi.org/10.1155/2018/1301562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260552PMC
November 2018
1 Read

Against the Odds.

Authors:
Emily M Bucholz

JAMA 2018 Dec;320(22):2313-2314

Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.

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http://dx.doi.org/10.1001/jama.2018.17821DOI Listing
December 2018

[Syndrome of inappropriate secretion of antidiuretic hormone in multiple myeloma patients treated with bortezomib, lenalidomide, and dexamethasone combination therapy].

Rinsho Ketsueki 2018 ;59(11):2423-2427

Division of Hematology, Department of Medicine, Showa University School of Medicine.

Hyponatremia occurs while receiving bortezomib-containing combination therapy in multiple myeloma (MM) ; however, the mechanism of hyponatremia remains unclear. A 65-year-old female with MM was treated with bortezomib, lenalidomide, and dexamethasone. Fourteen days after chemotherapy initiation, she developed hyponatremia (serum sodium, 127 mEq/l, compared with 136 mEq/l before chemotherapy) with plasma hypo-osmolality and urine hyper-osmolality. Read More

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http://dx.doi.org/10.11406/rinketsu.59.2423DOI Listing
January 2018
2 Reads

Long-acting intramuscular ACTH stimulation test for the diagnosis of secondary adrenal insufficiency in children.

J Pediatr Endocrinol Metab 2018 Dec 7. Epub 2018 Dec 7.

Division of Pediatric Endocrinology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Background The diagnosis of adrenal insufficiency (AI) is based on the basal and stimulated levels of serum cortisol in response to the short Synacthen test (SST). In patients with secondary AI (SAI) due to hypothalamic-pituitary-adrenal (HPA) axis defects, the SST has been validated against the insulin tolerance test (ITT), which is the gold standard. However, injection Synacthen is not easily available in some countries, and endocrinologists often use Acton-Prolongatum (intramuscular [IM] long-acting adrenocorticotropic hormone [ACTH]) in place of Synacthen. Read More

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http://www.degruyter.com/view/j/jpem.ahead-of-print/jpem-201
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http://dx.doi.org/10.1515/jpem-2018-0330DOI Listing
December 2018
2 Reads

Associated auto-immune disease in type 1 diabetes patients: a systematic review and meta-analysis.

Eur J Endocrinol 2019 Feb;180(2):135-144

Department of Internal Medicine, Section Endocrinology & Metabolism.

Introduction The association between type 1 diabetes (T1D) and other auto-immune diseases is well known. However, a quantitative overview of all associated auto-immune diseases and their prevalence in T1D is lacking. Methods We searched PubMed, Web of Science, EMBASE and Cochrane library in September 2018 to identify relevant articles about the prevalence of the following associated auto-immune diseases in T1D cohorts: auto-immune thyroid disease, celiac disease, gastric autoimmunity including pernicious anemia, vitiligo and adrenal gland insufficiency. Read More

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https://eje.bioscientifica.com/view/journals/eje/aop/eje-18-
Publisher Site
http://dx.doi.org/10.1530/EJE-18-0515DOI Listing
February 2019
4 Reads

Clinical decision making and application of an active rehabilitation program for a person with the neuromuscular symptoms of Allgrove syndrome: a case report.

Authors:
Joseph T Adams

Physiother Theory Pract 2018 Dec 3:1-8. Epub 2018 Dec 3.

a Rusk Rehabilitation, Clinical Instructor, Department of Rehabilitation Medicine NYU School of Medicine , NYU Langone Health , New York , NY , USA.

Background: Allgrove syndrome is a multisystem disorder first described in 1978 and is classically associated with esophageal achalasia, alacrima, and adrenal insufficiency. Allgrove syndrome is caused by homozygous and/or compound heterozygous mutations on Chromosome 12q13, designated as "AAA" (Achalasia, Addisonianism Alacrima). AAA encodes the protein ALADIN (Alacrima, Achalasia, aDrenal Insufficiency Neurologic disorder), a member of the nuclear porin family forming the nuclear pore complex. Read More

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http://dx.doi.org/10.1080/09593985.2018.1548049DOI Listing
December 2018
2 Reads

SGPL1 Deficiency: A Rare Cause of Primary Adrenal Insufficiency.

J Clin Endocrinol Metab 2018 Dec 3. Epub 2018 Dec 3.

Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics & Pediatric Endocrinology Inter-Institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.

Context: Multiple autosomal recessive genes have been etiologically linked to Primary Adrenal Insufficiency (PAI). Recently, SGPL1 gene mutations were recognized as causes of steroid-resistant nephrotic syndrome type 14 (NPHS14), a sphingolipidosis with multisystemic manifestations including PAI. Interestingly, this is the only monogenetic form of nephrotic syndrome (NS) and the sole sphingolipidosis causing adrenal disease. Read More

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https://academic.oup.com/jcem/advance-article/doi/10.1210/jc
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http://dx.doi.org/10.1210/jc.2018-02238DOI Listing
December 2018
18 Reads

Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies.

Int J Pediatr 2018 1;2018:1739831. Epub 2018 Nov 1.

Division of Endocrinology, Department of Pediatrics, Nationwide Children's Hospital/The Ohio State University College of Medicine, Columbus, Ohio, USA.

Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex. Destruction or dysfunction of the adrenal cortex is the cause of primary adrenal insufficiency, while secondary adrenal insufficiency is a result of pituitary or hypothalamic disease. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality. Read More

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http://dx.doi.org/10.1155/2018/1739831DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236909PMC
November 2018
3 Reads

Isolation and characterization of adrenocortical progenitors involved in the adaptation to stress.

Proc Natl Acad Sci U S A 2018 Dec 4;115(51):12997-13002. Epub 2018 Dec 4.

Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany;

The adrenal gland is a master regulator of the human body during response to stress. This organ shows constant replacement of senescent cells by newly differentiated cells. A high degree of plasticity is critical to sustain homeostasis under different physiological demands. Read More

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http://dx.doi.org/10.1073/pnas.1814072115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6304967PMC
December 2018
3 Reads

Pharmacokinetics of Glucocorticoid Replacement Before and After Bariatric Surgery in Patients With Adrenal Insufficiency.

J Endocr Soc 2018 Dec 20;2(12):1338-1344. Epub 2018 Sep 20.

Department of Internal Medicine, Rijnstate Hospital, TA Arnhem, Netherlands.

Adequate glucocorticoid replacement in patients with primary or secondary adrenal insufficiency is essential to maintain general well-being. Little is known about the effects of bariatric surgery on glucocorticoid absorption. This study evaluates glucocorticoid absorption before and after bariatric surgery, with assessment of plasma cortisol profiles in five patients receiving glucocorticoid replacement therapy for primary (n = 1) or secondary (n = 4) adrenal insufficiency. Read More

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http://dx.doi.org/10.1210/js.2018-00239DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262195PMC
December 2018
2 Reads

Chronic Mucocutaneous Candidiasis in Autoimmune Polyendocrine Syndrome Type 1.

Front Immunol 2018 19;9:2570. Epub 2018 Nov 19.

Department Parasitology-Mycology, CHU, Lille, France.

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is an autosomal recessive disease caused by mutations in the autoimmune regulator (AIRE) gene, characterized by the clinical triad of chronic mucocutaneous candidiasis (CMC), hypoparathyroidism, and adrenal insufficiency. CMC can be complicated by systemic candidiasis or oral squamous cell carcinoma (SCC), and may lead to death. The role of chronic infection in the etiopathogenesis of oral SCC is unclear. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02570
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http://dx.doi.org/10.3389/fimmu.2018.02570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6254185PMC
November 2018
8 Reads

Response to Letter to the Editor: "Characterization of the CYP11A1 Non-synonymous Variant p.E314K in Children Presenting with Adrenal Insufficiency".

J Clin Endocrinol Metab 2018 Nov 30. Epub 2018 Nov 30.

National Institutes of Health Clinical Center.

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http://dx.doi.org/10.1210/jc.2018-02512DOI Listing
November 2018
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Letter to the Editor: "Characterization of the CYP11A1 Non-synonymous Variant p.E314K in Children Presenting with Adrenal Insufficiency".

J Clin Endocrinol Metab 2018 Nov 30. Epub 2018 Nov 30.

Laboratoire de Biochimie et Biologie Moléculaire Grand Est, UM Pathologies Endocriniennes Rénales Musculaires et Mucoviscidose, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France.

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http://dx.doi.org/10.1210/jc.2018-02415DOI Listing
November 2018
9 Reads

Abnormal Glycerol Metabolism in a Child with Global Developmental Delay, Adrenal Insufficiency, and Intellectual Disability.

Clin Chem 2018 Dec;64(12):1785-1787

Department of Laboratory Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA;

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http://dx.doi.org/10.1373/clinchem.2018.293696DOI Listing
December 2018
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Malnutrition prevalence in adrenal insufficiency among hospitalized elderly patients: limitations of the body mass index in the assessment of malnutrition.

Asia Pac J Clin Nutr 2018 ;27(6):1175-1181

Department of Internal Medicine, College of Medicine, Chungbuk National University, Cheongju, Korea.

Background And Objectives: This study aimed to evaluate malnutrition prevalence and usefulness of the body mass index (BMI) in the assessment of malnutrition in hospitalized elderly patients with adrenal insufficiency (AI).

Methods And Study Design: 318 hospitalized AI patients were diagnosed by a rapid ACTH stimulation test with a history of steroid treatment and compared with 374 control patients. Nutrition was assessed using the Malnutrition Universal Screening Tool (MUST). Read More

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http://dx.doi.org/10.6133/apjcn.201811_27(6).0002DOI Listing
January 2018
1 Read