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cushing s disease

Eric A Schmidt, Fabien Despas, Anne Pavy-Le Traon, Zofia Czosnyka, John D Pickard, Kamal Rahmouni, Atul Pathak, Jean M Senard
Intracranial pressure (ICP) is the pressure within the cranium . ICP rise compresses brain vessels and reduces cerebral blood delivery. Massive ICP rise leads to cerebral ischemia, but it is also known to produce hypertension, bradycardia and respiratory irregularities due to a sympatho-adrenal mechanism termed Cushing response. One still unresolved question is whether the Cushing response is a non-synaptic acute brainstem ischemic mechanism or part of a larger physiological reflex for arterial blood pressure control and homeostasis regulation...
2018: Frontiers in Physiology
Maryam K Al Badi, Ibrahim Al-Alwan, Mohammed Al-Dubayee, Ayed Al-Anzi, Maram S Al Turki, Noura Aloudah, Khaled O Alsaad
Oncocytic tumors are epithelial neoplasms that occur in various organs, including adrenal glands. Oncocytic adrenocortical adenomas and carcinomas are uncommon but well-known pathological entities in adults. However, generally oncocytic tumors, particularly in the adrenal glands, are very rare in the pediatric age-group. Most oncocytic adrenal tumors are not functional. We present a rare case of right-sided, functional oncocytic adrenocortical adenoma in a 5-year-old boy, who presented with clinical manifestations of precocious puberty and Cushing syndrome...
January 1, 2018: Pediatric and Developmental Pathology
A Albani, F Ferraù, A Ciresi, R Pivonello, C Scaroni, D Iacuaniello, M Zilio, V Guarnotta, A Alibrandi, E Messina, M Boscaro, C Giordano, A Colao, S Cannavo
PURPOSE: Patients with Cushing's disease (CD) experience metabolic alterations leading to increased cardiovascular mortality. Recently, the visceral adiposity index (VAI) has been proposed as a marker of visceral adipose tissue dysfunction (ATD) and of the related cardiometabolic risk. We aimed to evaluate the impact of 12-month pasireotide treatment on cardiometabolic risk in CD patients. METHODS: This is a multicentre, prospective, and observational study. Sixteen CD patients, referred to the Endocrine Units of the University Hospitals of Messina, Napoli, Padova, and Palermo (Italy), successfully treated with pasireotide for 12 month have been enrolled...
January 30, 2018: Endocrine
Eleni Daniel, Miguel Debono, Sharon Caunt, Constantine Girio-Fragkoulakis, Stephen J Walters, Scott A Akker, Ashley B Grossman, Peter J Trainer, John Newell-Price
PURPOSE: Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS: Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 μg s...
January 8, 2018: Pituitary
Stephanie B Tsai, Joanna Rhodes, Hongtao Liu, Tsiporah Shore, Michael Bishop, Melissa M Cushing, Usama Gergis, Lucy Godley, Justin Kline, Richard A Larson, Sebastian Mayer, Olatoyosi Odenike, Wendy Stock, Amittha Wickrema, Koen van Besien, Andrew S Artz
Haplo/cord transplantation combines an umbilical cord blood (UCB) graft with CD34-selected haploidentical cells and results in rapid hematopoietic recovery followed by durable UCB engraftment. We compared outcomes of transplants in older patients with acute myeloid leukemia (AML) or high-risk myelodysplastic syndromes (MDS) who received either HLA-matched unrelated donor cells (MUD) or haplo/cord grafts. Between 2007 and 2013, 109 adults ages 50 and older underwent similar reduced intensity conditioning (RIC) with fludarabine and melphalan and antibody-mediated T-cell depletion for AML (n=83) or high risk MDS (n=26) followed by either a MUD (n=68) or haplo/cord (n=41) graft...
December 27, 2017: Biology of Blood and Marrow Transplantation
Nadine El Asmar, Aman Rajpal, Warren R Selman, Baha M Arafah
Context and Objectives: Despite the development of hypocortisolemia after corticotroph surgical adenomectomy, 15% to 20% patients have recurrence of Cushing disease (CD). In this study, we investigated the effect of tumor size and the value of perioperative assessment of corticotropin (ACTH) and adrenal steroid levels in predicting recurrence. Design: Perioperatively, no glucocorticoids were administered until the serum cortisol was ≤3 μg/dL. Blood samples were obtained before and repeatedly after adenomectomy in 79 patients with CD...
February 1, 2018: Journal of Clinical Endocrinology and Metabolism
Lillian R Sturmer, Dylan Dodd, Christina S Chao, Run-Zhang Shi
BACKGROUND: Late night salivary cortisol measurement is a clinically important and convenient screening test for Cushing's syndrome. Tandem mass spectrometry (LC-MS/MS) assays have superior sensitivity and specificity compared to immunoassays. Our goal was to improve a LC-MS/MS method to measure salivary cortisol in both adult and pediatric patients and to characterize its analytical performance by method validation and clinical performance by chart review. METHODS: We improved a LC-MS/MS method originally developed for urine cortisol to measure low level salivary cortisol...
January 2018: Steroids
Bharati Kochar, Edward L Barnes, Millie D Long, Kelly C Cushing, Joseph Galanko, Christopher F Martin, Laura E Raffals, Robert S Sandler
OBJECTIVES: Depression is prevalent in inflammatory bowel disease (IBD) patients. The impact of depression on IBD is not well-studied. It is unknown how providers should assess depression. METHODS: We used data from the Sinai-Helmsley Alliance for Research Excellence cohort, to assess methods of diagnosing depression and effects of baseline depression on disease activity at follow-up. A patient health questionnaire (PHQ-8) score ≥5 was consistent with mild depression...
January 2018: American Journal of Gastroenterology
Danae A Delivanis, Nicole M Iñiguez-Ariza, Muhammad H Zeb, Michael R Moynagh, Naoki Takahashi, Travis J McKenzie, Melinda A Thomas, Charalambos Gogos, William F Young, Irina Bancos, Venetsana Kyriazopoulou
CONTEXT: Abdominal visceral adiposity and central sarcopenia are markers of increased cardiovascular risk and mortality. OBJECTIVE: To assess whether central sarcopenia and adiposity can serve as a marker of disease severity in patients with adrenal adenomas and glucocorticoid secretory autonomy. DESIGN: Retrospective cohort study. PATIENTS: Twenty-five patients with overt Cushing's syndrome (CS), 48 patients with mild autonomous cortisol excess (MACE) and 32 patients with a nonfunctioning adrenal tumour (NFAT) were included...
February 2018: Clinical Endocrinology
Cristina Cocco, Carla Brancia, Giulia Corda, Gian-Luca Ferri
This review summarized different studies reporting the presence of autoantibodies reacting against cells of the pituitary (APAs) and/or hypothalamus (AHAs). Both APAs and AHAs have been revealed through immunofluorescence using different kinds of substrates. Autoantibodies against gonadotropic cells were mainly found in patients affected by cryptorchidism and hypogonadotropic hypogonadism while those against prolactin cells were found in different kinds of patients, the majority without pituitary abnormalities...
November 3, 2017: International Journal of Molecular Sciences
Jana Ježková, Josef Marek, Manuela Vaněčková
Pituitary adenomas are the most common tumours of the sellar region. A combination of neurosurgery, radiation and pharmacological approaches are applied for the treatment of pituitary adenomas. In certain cases, patient observation is another option. Neurosurgery is the first-choice treatment for acromegaly, Cushing´s disease and TSH secreting adenomas. Leksell gamma knife irradiation is used in the treatment of tumour residues. Until the effect of the irradiation is evident, pharmacological treatment must be administered...
2017: Casopís Lékar̆ů C̆eských
A S Azzi, N Driessens
Adrenal glands are specialized in biosynthesis of several hormones correlated to different clinical phenotypes in case of excess or lack of production. In addition to secretion disorders, tumors, secreting or not, can take place in adrenal glands. Incidentalomas are the most common adrenal diseases in clinical practice. The challenge of the management is to determine whether the lesion is benign or malignant and secreting or not in order to direct therapeutic management towards surgical option, pharmacotherapy or clinical follow-up...
2017: Revue Médicale de Bruxelles
Or Cohen-Inbar, Zhiyuan Xu, Cheng-Chia Lee, Chin-Chun Wu, Tomáš Chytka, Danilo Silva, Mayur Sharma, Hesham Radwan, Inga S Grills, Brandon Nguyen, Zaid Siddiqui, David Mathieu, Christian Iorio-Morin, Amparo Wolf, Christopher P Cifarelli, Daniel T Cifarelli, L Dade Lunsford, Douglas Kondziolka, Jason P Sheehan
Silent corticotroph staining pituitary adenoma (SCA) represents an uncommon subset of Non-Functioning adenomas (NFAs), hypothesized to be more locally aggressive. In this retrospective multicenter study, we investigate the safety and effectiveness of Stereotactic Radiosurgery (SRS) in patients with SCA compared with other non-SCA NFA's. Eight centers participating in the International Gamma-Knife Research Foundation (IGKRF) contributed to this study. Outcomes of 50 patients with confirmed SCAs and 307 patients with confirmed non-SCA NFA's treated with SRS were evaluated...
October 2017: Journal of Neuro-oncology
Prachi Bansal, Anurag Lila, Manjunath Goroshi, Swati Jadhav, Nilesh Lomte, Kunal Thakkar, Atul Goel, Abhidha Shah, Shilpa Sankhe, Naina Goel, Neelam Jaguste, Tushar Bandgar, Nalini Shah
PURPOSE: Transsphenoidal surgery (TSS) is the primary treatment modality for Cushing's disease (CD). However, the predictors of post-operative remission and recurrence remain debatable. Thus, we studied the post-operative remission and long-term recurrence rates, as well as their respective predictive factors. METHODS: A retrospective analysis of case records of 230 CD patients who underwent primary microscopic TSS at our tertiary care referral centre between 1987 and 2015 was undertaken...
November 2017: Endocrine Connections
S Chen, Z X Pan, L Lu, A L Tong, H Pan, H J Zhu, Y Yao, M Feng, R Z Wang, Z L Lu
Objective: To elucidate the clinical characteristics and localization diagnosis of patients with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) in adolescence. Methods: The clinical data, laboratory examination and localization diagnosis from 35 patients aged less than 18 years old with adolescent CS who were treated at Peking Union Medical College Hospital between January 1990 and March 2012 were analyzed. Results: There were 29 cases of Cushing's disease (CD) and 6 cases of ectopic ACTH syndrome (EAS)...
September 12, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Matthew J Shepard, Mohamed A Elzoghby, Daffer Ghanim, M Beatriz S Lopes, John A Jane
BACKGROUND: Most sellar gangliocytomas are discovered with a concurrent pituitary adenoma, also known as a mixed gangliocytoma-adenoma (MGA). MGAs are rare, with fewer than 100 cases reported in the literature to date and only 1 previously documented surgical series. Because MGAs are radiologically indistinguishable from pituitary adenomas, they are often diagnosed after surgery. Combined with the paucity of clinical outcome data for these tumors, this makes their diagnosis and management challenging...
December 2017: World Neurosurgery
H Fan, H Z Li, W F Xu, Z G Ji, Y S Zhang
OBJECTIVE: To investigation the diagnosis and treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome. METHODS: The clinical characters of 57 cases of ecotopic ACTH syndrome from Jan. 1996 to Dec. 2016 were collected and analyzed. The 57 cases included 32 males and 25 females. The age ranged from 11 to 68 years (average 32 years). ACTH levels significantly increased from 16.5 to 365.6 pmol/L, with average 77.6 pmol/L (normal range <10.1 pmol/L). The pituitary MRI did not found lesions...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Partha Pratim Chakraborty, Shinjan Patra, Sugata Narayan Biswas, Rana Bhattacharjee
Primary adrenal myelolipomas, relatively rare benign tumours of the adrenal cortex are typically unilateral, hormonally inactive and asymptomatic, hence often diagnosed as 'adrenal incidentaloma'. Bilateral adrenal myelolipomas, in particular, may be associated with underlying endocrinopathies associated with elevated circulating adrenocorticotropic hormone (ACTH) concentration. Subclinical cortisol hypersecretion, irrespective of its ACTH dependency, does not manifest typical clinical phenotype of hypercortisolemia, and thus termed subclinical Cushing's syndrome...
August 16, 2017: BMJ Case Reports
B S Yurekli, B Karaca, A Kisim, E Bozkurt, H Atmaca, S Cetinkalp, G Ozgen, C Yilmaz, S Uzunoglu, R Uslu, F Saygili
PURPOSE: Gossypol, a naturally occurring compound in cottonseeds, has anticancer effects against several tumor cell lines. It has been extensively studied in clinical trials and is well tolerated with a favorable safety profile. AT-101, a derivative of R (-)-gossypol, binds to Bcl-2 family proteins and induces apoptosis in vitro. Although transsphenoidal surgical excision of the pituitary corticotroph adenoma is the gold standard of care, it is not successful all the time. Medical therapy for Cushing's disease still remains a challenge for the clinicians...
July 20, 2017: Journal of Endocrinological Investigation
N V Marques, L Kasuki, M C Coelho, C H A Lima, L E Wildemberg, M R Gadelha
INTRODUCTION: Pituitary adenomas (PA) occur mainly as sporadic disease, but familial syndromes are found in approximately 5% of cases. Identification of these syndromes is important in order to diagnose individuals at risk at an earlier stage. AIMS: To evaluate the frequency of familial PA in a reference outpatient clinic devoted to PA treatment and to identify family members suspected to have pituitary disease. METHODS: Patients with PA were interviewed with respect to the presence of family members with diagnosis of PA or with signs or symptoms suggestive of them...
December 2017: Journal of Endocrinological Investigation
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