The Experts below are selected from a list of 1266 Experts worldwide ranked by ideXlab platform
Marcio Carlos Machado - One of the best experts on this subject based on the ideXlab platform.
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the role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of acth dependent cushing s syndrome
Clinical Endocrinology, 2006Co-Authors: Marcio Carlos Machado, Sorahia Domenice, Maria Adelaide Albergaria Pereira, Maria Candida Barisson Villares Fragoso, Paulo Puglia, Berenice B. Mendonca, L R SalgadoAbstract:Summary Objective Bilateral inferior petrosal sinus sampling (BIPSS) with corticotrophin-releasing hormone (CRH) stimulation is currently the gold standard test for the differential diagnosis of ACTH-dependent Cushing's syndrome. Reports on the use of desmopressin in this approach are limited. The aim of this study was to evaluate the use of desmopressin during BIPSS in a cohort of patients with ACTH-dependent Cushing's syndrome. Design A retrospective case-record study. Patients Fifty-six patients with confirmed ACTH-dependent Cushing's syndrome underwent BIPSS with desmopressin stimulation when presenting negative pituitary tumour imaging. Measurements Central to peripheral (CEN:PER) ACTH gradient, lateralization of the ACTH source and surgical tumour confirmation were evaluated. Results A CEN:PER ACTH gradient was found in 40 patients under basal conditions (CEN:PER ≥ 2) and in 47 patients after desmopressin stimulation (CEN:PER ≥ 3). Ectopic ACTH-producing tumours (three lung carcinoid tumour, one Thymus carcinoid tumour and one Thymus Hyperplasia) were confirmed in five out of nine patients without the CEN:PER ACTH gradient, and four cases were false negative for Cushing's disease. Lateralization (IPS:IPS ≥ 1·4) was observed in 80·8% of patients under basal conditions (38/47) and in 97·8% after desmopressin (46/47), and it was surgically confirmed in 78·7%. There were no false-positive cases. Sensitivity and specificity were 92·1% and 100%, respectively. Conclusions Desmopressin improves the differential diagnosis of ACTH-dependent Cushing's syndrome by amplifying the CEN:PER and IPS:IPS ACTH gradients, and is therefore a useful ACTH secretagogue in BIPSS.
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Thymus Hyperplasia after resolution of hypercortisolism in acth dependent cushing s syndrome the importance of thymic vein catheterization
European Journal of Endocrinology, 2006Co-Authors: Malebranche Berardo Carneiro Da Cunha Neto, Marcio Carlos Machado, Flavia Mesquita, Nina R C Musolino, Andrea Cecilia Toscanini, Gilberto Ochman, Valter Angelo Sperling Cescato, Raul Marino, Manoel Jacobsen TeixeiraAbstract:Thymic Hyperplasia has been described after the resolution of hypercortisolism from several etiologies, causing great diagnostic dilemmas. We describe a case where the catheterization of the thymic vein was essential forthe differential diagnosis of a thymic enlargement in an adrenalectomized patient with ACTH-dependent Cushing's syndrome. The patient was a 48-year-old female with clinical and laboratorial data suggesting Cushing's disease. She underwent a transsphenoidal surgery with no tumor visualization and no remission of the syndrome. Histopathological studies disclosed a normal pituitary. She underwent a bilateral adrenalectomy and 8 months later a chest CT showed an increase of left thymic lobe, which was previously non-existent. After a negative 1 1 1 In-pentetreotide scintigraphy, the patient underwent simultaneous and bilateral catheterism of the petrosus sinuses and catheterization of the thymic and inominate veins and no ACTH gradient was shown among the sites of collection. She did not undergo thoracotomy and a follow-up was established. During the evolution, there was a spontaneous regression of the thymic lesion 38 months after the diagnosis. The ACTH gradient during the catheterization of thymic vein was essential for the differential diagnosis of the thymic enlargement tumor after hypercortisolism resolution in ACTH-dependent Cushing's syndrome, especially in this case, where the ACTH source was occult, thus avoiding an invasive surgical procedure for a benign entity with spontaneous resolution.
Manoel Jacobsen Teixeira - One of the best experts on this subject based on the ideXlab platform.
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Thymus Hyperplasia after resolution of hypercortisolism in acth dependent cushing s syndrome the importance of thymic vein catheterization
European Journal of Endocrinology, 2006Co-Authors: Malebranche Berardo Carneiro Da Cunha Neto, Marcio Carlos Machado, Flavia Mesquita, Nina R C Musolino, Andrea Cecilia Toscanini, Gilberto Ochman, Valter Angelo Sperling Cescato, Raul Marino, Manoel Jacobsen TeixeiraAbstract:Thymic Hyperplasia has been described after the resolution of hypercortisolism from several etiologies, causing great diagnostic dilemmas. We describe a case where the catheterization of the thymic vein was essential forthe differential diagnosis of a thymic enlargement in an adrenalectomized patient with ACTH-dependent Cushing's syndrome. The patient was a 48-year-old female with clinical and laboratorial data suggesting Cushing's disease. She underwent a transsphenoidal surgery with no tumor visualization and no remission of the syndrome. Histopathological studies disclosed a normal pituitary. She underwent a bilateral adrenalectomy and 8 months later a chest CT showed an increase of left thymic lobe, which was previously non-existent. After a negative 1 1 1 In-pentetreotide scintigraphy, the patient underwent simultaneous and bilateral catheterism of the petrosus sinuses and catheterization of the thymic and inominate veins and no ACTH gradient was shown among the sites of collection. She did not undergo thoracotomy and a follow-up was established. During the evolution, there was a spontaneous regression of the thymic lesion 38 months after the diagnosis. The ACTH gradient during the catheterization of thymic vein was essential for the differential diagnosis of the thymic enlargement tumor after hypercortisolism resolution in ACTH-dependent Cushing's syndrome, especially in this case, where the ACTH source was occult, thus avoiding an invasive surgical procedure for a benign entity with spontaneous resolution.
Froda Vartdal - One of the best experts on this subject based on the ideXlab platform.
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myasthenia gravis patients with Thymus Hyperplasia and myasthenia gravis patients with thymoma display different hla associations
Tissue Antigens, 1991Co-Authors: Anne Spurkland, Nils Erik Gilhus, Kjersti S Ronningen, John A Aarli, Froda VartdalAbstract:Thirty myasthenia gravis (MG) patients (9 with thymoma, 12 with Thymus Hyperplasia and 9 with thymic atrophy) and 181 Norwegian healthy controls were serologically typed for HLA-A, -B and -DR antigens and genomically typed for HLA-DQA1 and HLA-DQB1 alleles by probing in vitro amplified DNA with sequence-specific oligonucleotides. In patients with Thymus Hyperplasia the frequency of the DQB1*0201 allele was increased compared to controls (RR = 3.5, p less than 0.05), whereas among the patients with thymoma this allele was not observed (RR = 0.06, p less than 0.01). The frequencies of HLA-B8, -DR3 and -DQA1*0501, which are in strong linkage disequilibrium with DQB1*0201, were increased in patients with Hyperplasia and reduced in patients with thymoma. The data suggest that different HLA genes predispose to two different forms of MG.
Christoph Loddenkemper - One of the best experts on this subject based on the ideXlab platform.
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autoimmunity intestinal lymphoid Hyperplasia and defects in mucosal b cell homeostasis in patients with pten hamartoma tumor syndrome
Gastroenterology, 2012Co-Authors: Mario Heindl, Norman Handel, Joanne Ngeow, Janina Kionke, Christian Wittekind, Manja Kamprad, Anne Rensingehl, Stephan Ehl, J Reifenberger, Christoph LoddenkemperAbstract:The Phosphatase And Tensin Homolog Deleted On Chromosome 10 (PTEN) regulates the phosphoinositol-3-kinase (PI3K)-AKT signaling pathway. In a series of 34 patients with PTEN mutations, we described gastrointestinal lymphoid Hyperplasia, extensive hyperplastic tonsils, Thymus Hyperplasia, autoimmune lymphocytic thyroiditis, autoimmune hemolytic anemia, and colitis. Functional analysis of the gastrointestinal mucosa-associated lymphoid tissue revealed increased signaling via the PI3K-AKT pathway, including phosphorylation of S6 and increased cell proliferation, but also reduced apoptosis of CD20(+)CD10(+) B cells. Reduced activity of PTEN therefore affects homeostasis of human germinal center B cells by increasing PI3K-AKT signaling via mammalian target of rapamycin as well as antiapoptotic signals.
L R Salgado - One of the best experts on this subject based on the ideXlab platform.
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the role of desmopressin in bilateral and simultaneous inferior petrosal sinus sampling for differential diagnosis of acth dependent cushing s syndrome
Clinical Endocrinology, 2006Co-Authors: Marcio Carlos Machado, Sorahia Domenice, Maria Adelaide Albergaria Pereira, Maria Candida Barisson Villares Fragoso, Paulo Puglia, Berenice B. Mendonca, L R SalgadoAbstract:Summary Objective Bilateral inferior petrosal sinus sampling (BIPSS) with corticotrophin-releasing hormone (CRH) stimulation is currently the gold standard test for the differential diagnosis of ACTH-dependent Cushing's syndrome. Reports on the use of desmopressin in this approach are limited. The aim of this study was to evaluate the use of desmopressin during BIPSS in a cohort of patients with ACTH-dependent Cushing's syndrome. Design A retrospective case-record study. Patients Fifty-six patients with confirmed ACTH-dependent Cushing's syndrome underwent BIPSS with desmopressin stimulation when presenting negative pituitary tumour imaging. Measurements Central to peripheral (CEN:PER) ACTH gradient, lateralization of the ACTH source and surgical tumour confirmation were evaluated. Results A CEN:PER ACTH gradient was found in 40 patients under basal conditions (CEN:PER ≥ 2) and in 47 patients after desmopressin stimulation (CEN:PER ≥ 3). Ectopic ACTH-producing tumours (three lung carcinoid tumour, one Thymus carcinoid tumour and one Thymus Hyperplasia) were confirmed in five out of nine patients without the CEN:PER ACTH gradient, and four cases were false negative for Cushing's disease. Lateralization (IPS:IPS ≥ 1·4) was observed in 80·8% of patients under basal conditions (38/47) and in 97·8% after desmopressin (46/47), and it was surgically confirmed in 78·7%. There were no false-positive cases. Sensitivity and specificity were 92·1% and 100%, respectively. Conclusions Desmopressin improves the differential diagnosis of ACTH-dependent Cushing's syndrome by amplifying the CEN:PER and IPS:IPS ACTH gradients, and is therefore a useful ACTH secretagogue in BIPSS.