7 results on '"enfermedad de Cushing"'
Search Results
2. Corticotroph cell hyperplasia as a rare cause of ACTH-dependent Cushing syndrome.
- Author
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Ferri J, Martínez-Ibañez J, Terradez L, Savall E, Martínez-Hervás S, Oller MC, Lorente R, Ascaso JF, and Real JT
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- Humans, Adrenocorticotropic Hormone, Hyperplasia pathology, Corticotrophs metabolism, Corticotrophs pathology, Cushing Syndrome diagnosis, Cushing Syndrome etiology, Pituitary Neoplasms pathology, Adenoma diagnosis, Adenoma diagnostic imaging
- Abstract
Objective: Our aim was to characterise a cohort of patients with Cushing's disease (CD) who did not present pituitary adenoma in magnetic resonance imaging (MRI), needing a catheterisation of the inferior petrosal sinus (CIPS), and to study the pathological findings of the pituitary gland in these subjects after transsphenoidal surgery in order to establish the aetiology of CD. Furthermore, we evaluated possible differences in the features of the diagnosis between hyperplasia and adenoma., Subjects and Methods: We included 16 subjects. 17 CIPS were done. Hormonal parameters were measured using standard methods. A microscopic histochemical study following standard procedures and immunohistochemical analysis was performed. The diagnostic criteria for adenoma and hyperplasia were based on the WHO classification., Results: One patient was excluded for presenting an ACTH-producing bronchial neuroendocrine tumour. The 15 subjects with CD have a positive CIPS test indicating hypophyseal ACTH production. After transsphenoidal surgery, 12 patients showed a microadenoma and three (20%) a corticotroph cell hyperplasia. We found four recurrences after the transsphenoidal surgery (26%), with a mean time for recurrence of 105 months. We found that recurrence was more frequent in subjects with hyperplasia, and in those subjects with lower right/left ACTH ratio., Conclusion: Our study, which was focused on patients with CD with no pituitary adenoma detected by MRI and a positive CRH test after CIPS, has found that 20% showed corticotroph cell hyperplasia as the cause of CD. Right/left ACTH ratio after CIPS was useful to differentiate adenoma from hyperplasia. This finding may have important prognostic and treatment implications. More studies are necessary to confirm our result., (Copyright © 2022 SEEN and SED. Published by Elsevier España, S.L.U. All rights reserved.)
- Published
- 2022
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3. [Endoscopic endonasal surgery for sellar region pathology. An analysis of our first 200 patients. What we have learned].
- Author
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Reyes L, García S, Torales J, Halperín I, Alobid I, Hanzu F, Mora M, Valero R, and Enseñat J
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- Adolescent, Adult, Aged, Aged, 80 and over, Female, Humans, Male, Middle Aged, Nose, Postoperative Complications, Retrospective Studies, Treatment Outcome, Young Adult, Adenoma surgery, Endoscopy methods, Pituitary Neoplasms surgery
- Abstract
Introduction: Pituitary and sellar region tumours account for 10-15% of intracranial benign tumours, with pituitary adenoma being the most common one. In this article, a review is presented on 9 years of experience in surgical treatment using an endoscopic approach of sellar region lesions. The main features of our surgical technique will be explained, as well as the results in clinical and hormonal terms., Material and Methods: A retrospective analysis was conducted on 200 patients operated on due to sellar lesions by the same neurosurgeon (J.E.) using an endoscopic endonasal transsphenoidal approach between February 2006 and February 2015. The cases excluded were, those requiring extended approaches of the skull base, as well as craniopharyngiomas, inflammatory, metastatic, or malignant lesions., Results: Of the 200 patients treated (59.5% women, mean age of 51.7 years, range: 18-82 years old), there were: 7 Rathke cysts and 193 adenomas (26 micro-adenomas and 165 macro-adenomas). All of them sub-classified according to the degree of invasion of the cavernous sinus (Knosp 0, 1, and 2: 129 cases and Knosp 3 and 4: 71 cases). Total resection was achieved in 143 patients (71.5%), subtotal resection in 39 (19.5%), and partial resection in 18 (9%). In the group of higher occupancy of the cavernous sinus (Knosp 3 and 4) complete resection was achieved in 55.5% (40 of 71 patients). Hormonal remission was achieved in 34 patients with acromegaly (85%), 23 patients with prolactinomas (76%), and 30 patients with Cushing's disease (86%)., Conclusion: The results obtained in our series, due to the centralisation of pathology and experience, are comparable to those achieved in pituitary surgery reference centres. Early surgical exploration of cerebrospinal fluid leaks reduces the risk of post-surgical meningitis., (Copyright © 2016 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2016
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4. Assessment of the outcomes of the treatment of Cushing's disease in the hospitals of Castilla-La Mancha.
- Author
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Huguet I, Aguirre M, Vicente A, Alramadan M, Quiroga I, Silva J, and Lamas C
- Subjects
- ACTH-Secreting Pituitary Adenoma complications, Adult, Combined Modality Therapy, Comorbidity, Craniotomy, Diabetes Insipidus epidemiology, Diabetes Insipidus etiology, Endoscopy, Female, Humans, Hydrocortisone urine, Ketoconazole therapeutic use, Male, Middle Aged, Neoadjuvant Therapy, Pituitary ACTH Hypersecretion blood, Pituitary ACTH Hypersecretion etiology, Pituitary Neoplasms complications, Postoperative Complications epidemiology, Postoperative Complications etiology, Radiosurgery, Retrospective Studies, Spain epidemiology, Treatment Outcome, Young Adult, ACTH-Secreting Pituitary Adenoma surgery, Hypophysectomy methods, Pituitary ACTH Hypersecretion therapy, Pituitary Neoplasms surgery
- Abstract
Objective: Treatment of Cushing's disease poses interesting dilemmas in clinical practice. The aim of our study was to analyze the outcomes of the different treatments, the control and recurrence rates, and the complications derived from them., Material and Methods: Data were collected from the clinical records of 22 patients over 18 years of age (86.4% women). They had been diagnosed with Cushing's disease between 2000 and 2012, and were monitored at Complejo Hospitalario Universitario-Albacete, Hospital Virgen de la Salud-Toledo Hospital General Universitario de Ciudad Real, Hospital Virgen de la Luz-Cuenca, Hospital Nuestra Señora del Prado-Talavera de la Reina, and Complejo Hospitalario la Mancha Centro-Alcázar de San Juan., Results: Surgery was the treatment of choice in all patients. Biochemical cure was achieved in 72.2% of patients. Nine patients developed in the early postoperative period diabetes insipidus, which became in 2 patients only. Surprisingly, 3 patients with normal postoperative neurohypophyseal function later developed permanent diabetes insipidus. New hormone deficiencies occurred in 7 patients. Seventeen patients received ketoconazole before surgery (5 of them after surgery also), and 70% of them achieved normal urinary free cortisol levels. Three patients also received radiotherapy, and all of them were cured after a median follow-up of 85.5 months; they developed no tumors or other complications., Conclusions: Our study reports the outcomes of management of Cushing's disease in non-reference centers for this disease, possibly giving a realistic picture of standard clinical practice for the condition in Spain., (Copyright © 2014 SEEN. Published by Elsevier España, S.L.U. All rights reserved.)
- Published
- 2015
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5. Endoscopic endonasal surgery for pituitary tumors. Results in a series of 121 patients operated at the same center and by the same neurosurgeon.
- Author
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Torales J, Halperin I, Hanzu F, Mora M, Alobid I, De Notaris M, Ferrer E, and Enseñat J
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Female, Humans, Male, Middle Aged, Nose, Retrospective Studies, Treatment Outcome, Young Adult, Adenoma surgery, Natural Orifice Endoscopic Surgery, Neurosurgical Procedures methods, Pituitary Neoplasms surgery
- Abstract
Introduction: Pituitary adenomas account for approximately 15% of intracranial benign tumors. The neurosurgical results achieved since the endoscopic endonasal transsphenoidal (EET) approach was introduced in our center in 2005 are reported here., Patients and Methods: A retrospective analysis of 121 patients with sellar lesions (58% females, age 55.7 ± 16 years, range 18-82) who underwent EET surgery from February 2005 to January 2012 and were followed up for a mean time of 4.58 years (range 1.08-8.58)., Results: Six Rathke cleft cysts (3 intra-suprasellar, 1 intrasellar, 2 suprasellar); 114 pituitary adenomas (16 microadenomas, 98 macroadenomas), and 1 case of normal MRI were included. Baseline findings included hormonal changes in 59 patients (48,7%) and visual field changes in 38 patients (31%); in 7 patients (5.8%), clinical presentation was pituitary apoplexy. Complete resection was achieved in 77 patients (63.6%), subtotal resection in 29 (23.9%), and partial resection in 15 (12.3%). In patients with Grade 3 and 4 cavernous sinus invasion, resection was subtotal in 30% (12/39) and complete in 46% (18/39). Hormonal remission was achieved in 16 patients with Cushing disease (84%), 18 patients with prolactinoma (78.2%), and 18 patients with acromegaly (85,7%). There were 12 cases (9%) of cerebrospinal fluid leak, 4 cases of diabetes insipidus, and 3 cases with transient SIADH/hyponatremia. Seven patients developed panhypopituitarism. Postoperative mortality rate was 2.4%. One hundred and three patients (85.3%) were discharged from the hospital less than 48 hours after surgery., Conclusion: Our results are similar to those reported by renowned pituitary units. Results achieved using an endoscopic approach in pituitary neurosurgery are better than those of microneurosurgery for cavernous sinus invasion., (Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.)
- Published
- 2014
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6. [Predictors of long-term remission after transsphenoidal surgery in Cushing's disease].
- Author
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Abellán Galiana P, Fajardo Montañana C, Riesgo Suárez PA, Gómez Vela J, Escrivá CM, and Lillo VR
- Subjects
- ACTH-Secreting Pituitary Adenoma complications, ACTH-Secreting Pituitary Adenoma metabolism, Adenoma complications, Adenoma metabolism, Adolescent, Adrenal Insufficiency drug therapy, Adrenal Insufficiency etiology, Adrenocorticotropic Hormone metabolism, Adult, Circadian Rhythm, Combined Modality Therapy, Deamino Arginine Vasopressin, Dexamethasone, Female, Follow-Up Studies, Glucocorticoids therapeutic use, Hormone Replacement Therapy, Humans, Hydrocortisone analysis, Hydrocortisone metabolism, Male, Metyrapone, Middle Aged, Neoplasm Recurrence, Local diagnosis, Pituitary ACTH Hypersecretion blood, Pituitary ACTH Hypersecretion drug therapy, Pituitary ACTH Hypersecretion etiology, Pituitary ACTH Hypersecretion urine, Pituitary Neoplasms complications, Pituitary Neoplasms metabolism, Postoperative Complications drug therapy, Postoperative Complications etiology, Remission Induction, Retrospective Studies, Saliva chemistry, Secretory Rate drug effects, Young Adult, ACTH-Secreting Pituitary Adenoma surgery, Adenoma surgery, Hypophysectomy methods, Pituitary ACTH Hypersecretion surgery, Pituitary Neoplasms surgery
- Abstract
There is no consensus on the remission criteria for Cushing's disease or on the definition of disease recurrence after transsphenoidal surgery, and comparison of the different published series is therefore difficult. A long-term recurrence rate of Cushing's disease ranging from 2%-25% has been reported. Predictors of long-term remission reported include: 1) adenoma-related factors (aggressiveness, size, preoperative identification in MRI), 2) surgery-related factors, mainly neurosurgeon experience, 3) clinical factors, of which dependence on and duration of glucocorticoid treatment are most important, and 4) biochemical factors. Among the latter, low postoperative cortisol levels, less than 2 mcg/dL predict for disease remission. However, even when undetectable plasma cortisol levels are present, long-term recurrence may still occur and lifetime follow-up is required. We report the preliminary results of the first 20 patients with Cushing's disease operated on at our hospital using nadir cortisol levels less than 2 mcg/dl as remission criterion., (Copyright © 2012 SEEN. Published by Elsevier Espana. All rights reserved.)
- Published
- 2013
- Full Text
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7. A novel human tumoroid 3D model of sustained ACTH-secreting cell cultures to study critically needed therapies for Cushing's disease
- Author
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Antonio C Fuentes-Fayos, Raúl M. Luque, [Fuentes-Fayos,AC, Luque,RM] Maimonides Biomedical Research Institute of Cordoba (IMIBIC), Cordoba, Spain. [Fuentes-Fayos,AC, Luque,RM] Department of Cell Biology, Physiology and Immunology, University of Cordoba, Cordoba, Spain. [Fuentes-Fayos,AC, Luque,RM] Reina Sofia University Hospital (HURS), Cordoba, Spain. CIBER Physiopathology of Obesity and Nutrition (CIBERobn), Cordoba, Spain., This work has been supported by the Spanish Ministry of Science, Innovation and Universities (Grant PID2019-105564RB-I00, and Predoctoral contract FPU16-05059), Junta de Andalucía (BIO-0139) and CIBERobn
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Medicine (General) ,Cell Culture Techniques ,3d model ,Bioinformatics ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Clinical Laboratory Techniques::Cytological Techniques::Cell Culture Techniques [Medical Subject Headings] ,Anatomy::Cells::Cells, Cultured::Cell Line [Medical Subject Headings] ,Tumor Cells, Cultured ,Organisms::Eukaryota::Animals [Medical Subject Headings] ,Medicine ,Corticotroph ,Cells, Cultured ,Diseases::Endocrine System Diseases::Pituitary Diseases::Hyperpituitarism::Pituitary ACTH Hypersecretion [Medical Subject Headings] ,Hormona adrenocorticotrópica ,Health Care::Health Care Facilities, Manpower, and Services::Health Facilities::Laboratories [Medical Subject Headings] ,High-Throughput Nucleotide Sequencing ,General Medicine ,Hipófisis ,ACTH-Secreting Pituitary Adenoma ,Chemicals and Drugs::Hormones, Hormone Substitutes, and Hormone Antagonists::Hormones::Peptide Hormones::Pituitary Hormones::Pituitary Hormones, Anterior::Pro-Opiomelanocortin [Medical Subject Headings] ,Cushing's disease ,Secreting cell ,Single-Cell Analysis ,Enfermedad de Cushing ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Clinical Laboratory Techniques::Cytological Techniques::Cell Culture Techniques::Primary Cell Culture [Medical Subject Headings] ,Phenomena and Processes::Reproductive and Urinary Physiological Phenomena::Reproductive Physiological Phenomena::Reproductive Physiological Processes::Reproduction [Medical Subject Headings] ,Corticotrofos ,Chemicals and Drugs::Hormones, Hormone Substitutes, and Hormone Antagonists::Hormones::Peptide Hormones::Pituitary Hormones::Pituitary Hormones, Anterior::Pro-Opiomelanocortin::Adrenocorticotropic Hormone [Medical Subject Headings] ,Anatomy::Cells::Endocrine Cells::Corticotrophs [Medical Subject Headings] ,MEDLINE ,Pituitary neoplasms ,General Biochemistry, Genetics and Molecular Biology ,Pituitary gland ,Text mining ,R5-920 ,Cell Line, Tumor ,Spheroids, Cellular ,Neoplasias hipofisarias ,Humans ,Pituitary ACTH Hypersecretion ,Cell Proliferation ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Rodentia::Muridae::Murinae::Mice [Medical Subject Headings] ,business.industry ,Gene Expression Profiling ,Computational Biology ,medicine.disease ,Hipersecreción de la hormona adrenocorticotrópica pituitaria (HACT) ,Commentary ,Diseases::Endocrine System Diseases::Pituitary Diseases::Pituitary Neoplasms [Medical Subject Headings] ,Adrenocorticotropic hormone ,business ,Phenomena and Processes::Physiological Phenomena::Physiological Processes::Homeostasis [Medical Subject Headings] ,Biomarkers - Abstract
Cushing disease (CD), although rare, is a life-threatening disorder caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma, which leads to excess adrenal-derived cortisol. Efficacious and safe medical therapies that control both hormonal hypersecretion and pituitary corticotroph tumor growth remain an unmet need in the management of CD. Translational research in pituitary tumors has been significantly hampered by limited quantities of surgically resected tissue for ex vivo studies, and unavailability of human pituitary tumor cell models.To characterize human corticotroph tumors at the cellular level, we employed single cell RNA-sequencing (scRNA-seq) to study 4 surgically resected tumors. We also used microarrays to compare individualized paired consecutive culture passages to understand transcriptional shifts as in vitro cultures lost ACTH secretion. Based on these findings, we then modified our in vitro culture methods to develop sustained ACTH-secreting human corticotroph tumoroid cultures.scRNA-seq identified 4 major cell populations, namely corticotroph tumor (73.6%), stromal (11.2%), progenitor (8.3%), and immune cells (6.8%). Microarray analysis revealed striking changes in extracellular matrix, cell adhesion and motility-related genes concordant with loss of ACTH secretion during conventional 2D culture. Based on these findings, we subsequently defined a series of crucial culture nutrients and scaffold modifications that provided a more favorable trophic and structural environment that could maintain ACTH secretion in in vitro human corticotroph tumor cultures for up to 4 months.Our human corticotroph tumoroid model is a significant advance in the field of pituitary tumors and will further enable translational research studies to identify critically needed therapies for CD.This work was partly funded by NCI P50-CA211015 and the Warley Trust Foundation.
- Published
- 2021
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