- Audit of management of children and adolescents with congenital adrenal hyperplasia as per recent Endocrine Society guidelines. [Journal Article]Pediatr Endocrinol Diabetes Metab. 2023 Feb 03 [Online ahead of print]PE
- CONCLUSIONS: A shift to conservative surgical management of females, utility of neonatal screening for CAH, judicious use of growth promoting therapy is highlighted. Need for bone age testing, emergency hydrocortisone provision is warranted in our series.
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- Cortisol response in children with cancer and fever during chemotherapy: A prospective, observational study using random serum cortisol levels. [Journal Article]Cancer Med. 2023 Feb 03 [Online ahead of print]CM
- CONCLUSIONS: The incidence of an impaired HPA axis in pediatric cancer patients might be underestimated since 69% of the children in our study had a LCR during fever. Intake of dexamethasone, posaconazole and a time period of ≤7 days from the last glucocorticoid intake were additional risk factors for an LCR. However, we could not confirm that patients with a LCR fared worse than patients with a high cortisol response (HCR). Therefore, a different cortisol threshold may be necessary for defining an impaired HPA axis in febrile oncologic patients without concomitant symptoms of AI.
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- Clinical and functional analyses of the novel STAR c.558C>A in a patient with classic lipoid congenital adrenal hyperplasia. [Journal Article]Front Genet. 2023; 14:1096454.FG
- Objective: Congenital lipid adrenal hyperplasia (LCAH) is the most serious type of congenital adrenal hyperplasia and is caused by steroid-based acute regulatory (STAR) protein mutations. Herein, we report compound heterozygous mutations c.558C>A (p.S186 R) and c.772C>T (p.Q258*) in a newborn 46 XY patient diagnosed with classic LCAH and explore their clinical and functional characteristics. Meth…
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- Stress biomarkers in individuals with fibromyalgia syndrome: a systematic review with meta-analysis. [Journal Article]Pain. 2023 Jan 05 [Online ahead of print]PAIN
- Evidence suggests an involvement of hypothalamic-pituitary-adrenal (HPA) axis dysregulation in the development and maintenance of fibromyalgia syndrome (FMS). However, studies on the stress response via the HPA-axis in individuals with FMS show conflicting results. To better understand the relationship between FMS and HPA-axis dysregulation, we (1) systematically summarized the current level of e…
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- The Link Between Empty Sella Syndrome, Fibromyalgia, and Chronic Fatigue Syndrome: The Role of Increased Cerebrospinal Fluid Pressure. [Review]
- The etiopathogenesis of fibromyalgia (FM) and chronic fatigue syndrome (CFS) is not yet elucidated. Hypothalamo-pituitary-adrenal (HPA) axis dysfunction is reflected in the hormonal disturbances found in FM and CFS. Some study groups have introduced a novel hypothesis that moderate or intermittent intracranial hypertension may be involved in the etiopathogenesis of FM and CFS. In these conditions…
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- Exposure to dipentyl phthalate in utero disrupts the adrenal cortex function of adult male rats by inhibiting SIRT1/PGC-1α and inducing AMPK phosphorylation. [Journal Article]Environ Toxicol. 2023 Jan 30 [Online ahead of print]ET
- Di-n-pentyl phthalate (DPeP) is an endocrine-disrupting phthalate plasticizer. The objective of this study was to investigate the effect of DPeP on adrenocortical function in adult male rats following in utero exposure. DPeP (0, 10, 50, 100, and 500 mg/kg/day) was administered by gavage to pregnant Sprague-Dawley rats from gestational day 14 to 21. The morphology and function of the adrenal corte…
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- The clinical outcomes of imaging modalities for surgical management Cushing's disease - A systematic review and meta-analysis. [Meta-Analysis]
- CONCLUSIONS: A lack of controlled comparative studies makes it difficult to draw definitive conclusions. Within this limitation, the results suggest that despite improvements in radiological detection rates of Cushing's disease over the last four decades, there were no changes in clinical outcomes. Advances in imaging alone may be insufficient to improve surgical outcomes.
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- Relative adrenal insufficiency is a risk factor and endotype of sepsis - A proof-of-concept study to support a precision medicine approach to guide glucocorticoid therapy for sepsis. [Journal Article]
- 25-60% of septic patients experience relative adrenal insufficiency (RAI) and glucocorticoid (GC) is frequently used in septic patients. However, the efficacy of GC therapy and whether GC therapy should be based on the status of RAI are highly controversial. Our poor understanding about the pathogenesis of RAI and a lack of RAI animal model present significant barriers to address these critical i…
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- Screening for non-classic congenital adrenal hyperplasia in women: New insights using different immunoassays. [Journal Article]
- CONCLUSIONS: Optimal RIA-based basal 17OHP cutoff was comparable with that recommended in guidelines. The results suggest adopting a higher 17OHP cutoff when using ELISA. LH : FSH ratio improves the negative predictive value of basal 17OHP.
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- Intestinal tuberculosis in a patient with Cushing's syndrome. [Case Reports]
- A 39-year-old woman presented with cushingoid features was worked up and diagnosed to have ACTH-independent Cushing's syndrome. Computed tomography of the whole abdomen revealed a left adrenal mass. She was scheduled for elective laparoscopic left adrenalectomy, however, a few days prior to the surgery, the patient had hematochezia. Colonoscopy revealed multiple ulcers on the terminal ileum, to w…
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- Intraoperative Predictor of Remission in Cushing Disease. [Journal Article]Oper Neurosurg (Hagerstown). 2022 Dec 19 [Online ahead of print]ON
- CONCLUSIONS: Intraoperative ACTH may predict outcome after surgery in Cushing disease; furthermore, investigation is warranted.
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- Patterns of stress response to foreign eggs by a rejecter host of an obligate avian brood parasite. [Journal Article]Ecol Evol. 2023 Jan; 13(1):e9691.EE
- One of the most effective defenses of avian hosts against obligate brood parasites is the ejection of parasitic eggs from the nests. Despite the clear fitness benefits of this behavior, individuals within so-called "egg-rejecter" host species still show substantial variation in their propensity to eliminate foreign eggs from the nest. We argue that this variation can be further understood by stud…
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- Hypothalamic-pituitary-adrenal axis and renin-angiotensin-aldosterone system in adulthood PTSD and childhood maltreatment history. [Journal Article]Front Psychiatry. 2022; 13:967779.FP
- Accumulated evidence shows that psychological trauma and posttraumatic stress disorder (PTSD) are associated with dysfunction in the hypothalamic-pituitary-adrenal (HPA) axis. Besides the HPA axis hormones, recent evidence suggests that the renin-angiotensin-aldosterone (RAA) system and genetic factors may be involved in trauma/PTSD as well as in HPA axis regulation. This study attempted to bette…
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- CUSHING'S SYNDROME DUE TO PRIMARY BILATERAL MACRONODULAR ADRENAL HYPERPLASIA AND METACHRONOUS PHEOCHROMOCYTOMA. [Case Reports]Acta Endocrinol (Buchar). 2022 Jul-Sep; 18(3):361-367.AE
- CONCLUSIONS: Pheochromocytoma with synchronous ACTH-independent CS originating from the same adrenal gland is very rare. To the best of our knowledge,our case is the first one describing the coexistence of overt ACTH-independent CS due to PBMAH and metachronous PCC.The importance of detailed re-evaluation of patients with recurrent ACTH-independent CS is highlighted here.
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- Age-related and individual features of the HPA axis stress responsiveness under constant light in nonhuman primates. [Journal Article]
- The hypothalamic-pituitary-adrenal (HPA) axis is a key adaptive neuroendocrine system, dysfunction of which plays an important role in the increasing incidence of stress-dependent age-related pathology. Among the environmental factors effecting increase age-related diseases, great importance is given to disturbances of the light-dark schedule, particularly with increased illumination at night. Wh…
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- Complete and sustained remission of hypercortisolism with pasireotide treatment of an adrenocorticotropic hormone (ACTH)-secreting thoracic neuroendocrine tumour: an n-of-1 trial. [Journal Article]Endocr J. 2023 Jan 25 [Online ahead of print]EJ
- N-of-1 trials can serve as useful tools in managing rare disease. We describe a patient presenting with a typical clinical picture of Cushing's Syndrome (CS). Further testing was diagnostic of ectopic Adrenocorticotropic Hormone (ACTH) secretion, but its origin remained occult. The patient was offered treatment with daily pasireotide at very low doses (300 mg bid), which resulted in clinical and …
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- Immune checkpoint inhibitor-mediated hypophysitis: no place like home. [Journal Article]Clin Med (Lond). 2023 Jan; 23(1):81-84.CM
- Ambulatory emergency care forms a fundamental part of the strategy of trying to ensure safe and sustainable acute care services. Immune checkpoint inhibitor(ICI)-mediated hypophysitis is an important life-threatening complication of therapy. Patients presenting with clinical features and findings consistent with ICI-mediated hypophysitis were considered in the current study. In the absence of sev…
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- ACTH-independent production of 11-oxygenated androgens and glucocorticoids in an adrenocortical adenoma. [Case Reports]Eur J Endocrinol. 2022 Dec 01; 187(6):K39-K45.EJ
- Due to its rarity, biochemical and histologic characteristics of androgen and glucocorticoid co-secreting adrenocortical adenomas are largely unknown. Herein, we report a case of adrenocortical adenoma that caused marked hyperandrogenemia and mild autonomous cortisol secretion. In this study, we investigated serum steroid profiles using liquid chromatography-tandem mass spectrometry (LC-MS/MS) an…
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- Glucocorticoid receptor expression in patients with cardiac arrest in the early period after the return of spontaneous circulation: a prospective observational single-centre study. [Journal Article]
- CONCLUSIONS: This study revealed that GR expression and cell counts rapidly decreased, whereas plasma total cortisol levels increased in the early period after ROSC among patients who experienced CA. Our findings provide important information about GR level and function, and immunosuppressive status in these patients. Assessing GR expression in patients who experienced CA may help screening for those who are more sensitive to GC therapy.
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- Gene fusions are frequent in ACTH-secreting neuroendocrine neoplasms of the pancreas, but not in their non-pancreatic counterparts. [Journal Article]Virchows Arch. 2023 Jan 24 [Online ahead of print]VA
- Ectopic Cushing syndrome is a rare clinical disorder resulting from excessive adrenocorticotrophic hormone (ACTH) produced by non-pituitary neoplasms, mainly neuroendocrine neoplasms (NENs) of the lung, pancreas, and gastrointestinal tract, and other less common sites. The genetic background of ACTH-producing NENs has not been well studied. Inspired by an index case of ACTH-producing pancreatic N…
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- [Steroid profiling characteristics in pediatrc adrenal diseases]. [Journal Article]Probl Endokrinol (Mosk). 2022 Nov 03; 68(6):110-120.PE
- CONCLUSIONS: Results of our study steroid profiling can be used as additional differential diagnosis method in patients with adrenocortical adenomas with or without hormonal hyperproduction (ACTH-independent Cushing syndrome and incidentaloma). Further studies are needed to identify steroid markers for subtyping pediatric adrenal diseases.
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- [Survival predictors in patients with ectopic acth syndrome]. [Journal Article]Probl Endokrinol (Mosk). 2022 Aug 15; 68(6):30-42.PE
- CONCLUSIONS: The prognosis of patients with EAS is influenced by the age of diagnosis, NET localization, distant metastases and level of LNSC. The most common cause of ectopic ACTH syndrome was bronchopulmonary NET which was associated with the best survival rate.
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- Multilimbed membrane guanylate cyclase signaling system, evolutionary ladder. [Review]
- One monumental discovery in the field of cell biology is the establishment of the membrane guanylate cyclase signal transduction system. Decoding its fundamental, molecular, biochemical, and genetic features revolutionized the processes of developing therapies for diseases of endocrinology, cardio-vasculature, and sensory neurons; lastly, it has started to leave its imprints with the atmospheric …
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- High fat diet exacerbates stress responsivity in juvenile traumatic brain injury. [Journal Article]J Neurotrauma. 2023 Jan 21 [Online ahead of print]JN
- Traumatic brain injury (TBI) is one of the leading causes of death for children in the United States. Juveniles are more likely to sustain TBIs than most other age groups, and TBI has been shown to result in increased anxiety and stress behaviors. In addition, the hypothalamic-pituitary-adrenal (HPA) axis has previously been shown to become dysregulated after a TBI. Furthermore, many children con…
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- An Approach to Traumatic Brain Injury-Related Hypopituitarism: Overcoming the Pediatric Challenges. [Case Reports]
- Traumatic brain injury (TBI)-related hypopituitarism is a rare polymorphic complication of brain injury, with very little data, particularly concerning children and teenagers. This is a comprehensive review of the literature regarding this pathology, starting from a new pediatric case. The research was conducted on PubMed and included publications from the last 22 years. We identified nine origin…
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- Advances in Molecular Pathophysiology and Targeted Therapy for Cushing's Disease. [Review]
- Cushing's disease is caused by autonomous secretion of adrenocorticotropic hormone (ACTH) from corticotroph pituitary neuroendocrine tumors. As a result, excess cortisol production leads to the overt manifestation of the clinical features of Cushing's syndrome. Severe complications have been reported in patients with Cushing's disease, including hypertension, menstrual disorders, hyperglycemia, o…
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- Glucocorticoid-sensitive period of corticotroph development-Implications for mechanisms of early life stress. [Journal Article]J Neuroendocrinol. 2022 Dec 27 [Online ahead of print]JN
- Corticotrophs are intermediaries in the hypothalamic-pituitary-adrenal (HPA) axis, which plays a crucial role in stress response in vertebrates. The HPA axis displays an intricate mode of negative feedback regulation, whereby the peripheral effector, cortisol inhibits the secretion of its upstream regulator, adrenocorticotropic hormone (ACTH) from proopiomelanocortin (POMC)-expressing cells in th…
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- Remission, complications, and overall survival in transsphenoidal pituitary surgery-a Swedish single-center experience of 578 patients. [Journal Article]Acta Neurochir (Wien). 2023 Jan 20 [Online ahead of print]AN
- CONCLUSIONS: Our results regarding remission rates and complications are in comparison with previous studies. Surgery of pituitary lesion can be considered a safe and efficient surgery. We noted lower rates of CSF leakage in the later part of the study period and believe that this, in part, was an effect by the introduction of a multidisciplinary surgical skull base team and increased surgical experience.
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- Reduced hydrogen sulfide production contributes to adrenal insufficiency induced by hypoxia via modulation of NLRP3 inflammasome activation. [Journal Article]
- Objective: Adrenocortical responsiveness is critical for maintaining glucocorticoids production and homeostasis during stress. We sought to investigate adrenocortical responsiveness during hypoxia in mice and the mechanisms responsible for the regulation of adrenal responsiveness.Methods: (1) Adult male WT mice were randomly divided into four groups: normoxia, hypoxia (24h), hypoxia (72h), hypoxi…
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- Gene Mutations in Cushing's Syndrome. [Review]
- Prolonged exposure to glucocorticoids can result in the development of Cushing's syndrome. Excess serum cortisol can occur due to several factors including exogenous steroids, pituitary and adrenal adenoma, and ectopic ACTH secretion.
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