35 results on '"Paja-Fano M"'
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2. Deficiencia aislada de tirotropina adquirida. Un hallazgo excepcional de incierta etiología
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Paja Fano M, Elorza Olabegoya R, Lizarraga Zufiaurre A, and Egaña Zunzunegui N
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Endocrinology ,business.industry ,Endocrinology, Diabetes and Metabolism ,Thyrotropin deficiency ,Etiology ,Medicine ,Physiology ,business - Published
- 2010
3. Aldosteronismo primario senil asociado a carcinoma suprarrenal funcionante
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Rodríguez Iturriaga, J., primary, Gutiérrez Macías, A., additional, Paja Fano, M., additional, and Lizarraga Zufiaur, A., additional
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- 2014
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4. Ginecomastia e hipertiroidismo: estudio hormonal a propósito de un caso
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Paja Fano, M., primary, Oleaga Alday, A., additional, Astigarraga Aguirre, B., additional, and Elorza Olabegoya, R., additional
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- 2001
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5. Influence of smoking on cardiometabolic profile and surgical outcomes in patients with primary aldosteronism. A cohort study.
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Araujo-Castro M, Paja Fano M, González-Boillos M, Pascual-Corrales E, Parra Ramírez P, Rojas-Marcos PM, García-Cano A, Ruiz-Sanchez JG, Vicente A, Gómez-Hoyos E, Casterás A, Puig-Perez A, García Sanz I, Recasens M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo C, Manjón-Miguélez L, Rebollo Román Á, Robles Lázaro C, Recio JM, Morales-Ruiz M, Calatayud M, Jiménez López N, Meneses D, Sampedro Nuñez M, Ribas EM, Sanmartín Sánchez A, Diaz CG, Lamas C, Del Castillo Tous M, Serrano J, Michalopoulou T, Rodrigo ST, Chamorro RR, Aguila FJ, Moya Mateo EM, Gutiérrez-Medina S, and Hanzu FA
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Aim: To evaluate the influence of smoking on cardiometabolic profile and surgical outcomes in patients with primary aldosteronism (PA)., Methods: Multicenter retrospective study of patients with PA in 36 Spanish tertiary hospitals with available information on smoking habits (smokers and non-smokers [never smokers and ex-smokers])., Results: A total of 881 patients were included, of whom 180 (20.4%) were classified as smokers and 701 as non-smokers. At diagnosis, smokers and non-smokers did not differ in blood pressure or serum potassium levels between. However, smokers had a higher prevalence of left ventricular hypertrophy (LVH) than non-smokers (OR 2.0, 95% CI 1.23 to 3.25), and smokers were more likely to have severe LVH than non-smokers (12.5% vs. 6.6%, P=0.164). A larger mean tumor size of the adrenal nodule/s was observed in the smoking group (18.6±9.66 vs. 15.8±8.66 mm, P=0.002). In addition, the odds of mild autonomous cortisol secretion (MACS) was greater in smokers than in non-smokers (OR 2.1, 95% CI 1.14 to 4.06), but these differences disappeared when adjusted for the size of the adrenal nodule/s (adjusted OR 1.6, 95% CI 0.76 to 3.37). The rate of biochemical and hypertension cure was similar in both groups; however, hypertension cure tended to be more frequent in the non-smoker group (41.2% vs 29.9%, P=0.076)., Conclusions: Patients with PA who smoke have a higher prevalence of LVH and MACS and larger adrenal nodule/s than non-smokers. Smoking has no significant effect on the probability of hypertension response after adrenalectomy in patients with PA., (© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology.)
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- 2024
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6. Impact of primary aldosteronism on kidney function: results from the SPAIN-ALDO registry.
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Araujo-Castro M, Paja Fano M, González-Boillos M, Pascual-Corrales E, Martín Rojas-Marcos P, García-Cano A, Ruiz-Sanchez JG, Vicente A, Gómez-Hoyos E, Casterás A, Puig-Pérez A, García Sanz I, Recasens M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo CM, Manjón-Miguélez L, Rebollo Román A, Robles Lázaro C, María Recio J, Morales-Ruiz M, Calatayud M, Jiménez López N, Meneses D, Sampedro Nuñez M, Mena Ribas E, Sanmartín Sánchez A, Gonzalvo Diaz C, Lamas C, Castillo Tous MD, Serrano J, Michalopoulou T, Tenés Rodrigo S, Roa Chamorro R, Jaén Aguila F, Moya Mateo EM, Gutiérrez-Medina S, Hanzu FA, and Parra Ramírez P
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- Humans, Female, Male, Middle Aged, Spain epidemiology, Retrospective Studies, Aged, Kidney physiopathology, Renal Insufficiency, Chronic physiopathology, Renal Insufficiency, Chronic complications, Adult, Glomerular Filtration Rate, Cohort Studies, Hyperaldosteronism complications, Hyperaldosteronism physiopathology, Mineralocorticoid Receptor Antagonists therapeutic use, Adrenalectomy, Registries
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Aim: To evaluate the impact of aldosterone excess on renal function in individuals with primary aldosteronism and to compare its evolution after surgery or mineralocorticoid receptor antagonist (MRA) treatment., Methods: A multicentre, retrospective cohort study of primary aldosteronism patients in follow-up in 36 Spanish tertiary hospitals, who underwent specific treatment for primary aldosteronism (MRA or adrenalectomy)., Results: A total of 789 patients with primary aldosteronism were included, with a median age of 57.5 years and 41.8% being women. At primary aldosteronism diagnosis, the prevalence of chronic kidney disease (CKD) was 10.7% ( n = 84), with 75% of cases classified as state 3a ( n = 63). Primary aldosteronism patients with CKD had a longer duration of hypertension, a higher prevalence of type 2 diabetes, dyslipidaemia, cardiovascular events, hypokalaemia, and albuminuria. Unilateral adrenalectomy was performed in 41.8% of cases ( n = 330), and 459 patients were treated with MRA. After a median follow-up of 30.7 months (range 13.3-68.4), there was a significant decline in the estimated glomerular filtration rate (eGFR) in operated patients and those receiving MRA. During follow-up, 24.4% of patients with CKD at the time of primary aldosteronism diagnosis had normalized renal function, and 39% of those with albuminuria had albuminuria remission. There were no differences in renal function or albuminuria regression between the two therapy groups. However, development of albuminuria was less common in operated than in medically treated patients (0 vs. 6.0%, P = 0.009)., Conclusion: CKD affects around 10% of the patients with primary aldosteronism, with a higher risk in individuals with long-term hypertension, type 2 diabetes, dyslipidaemia, cardiovascular events, hypokalaemia, and albuminuria. At short-term, both MRA and surgical treatment lead to a reduction of renal function, but adrenalectomy led to higher renal protection., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)
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- 2024
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7. ERRATUM: Pegvisomant and pasireotide in PRL and GH co-secreting vs GH-secreting Pit-NETs.
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Araujo-Castro M, Biagetti B, Menéndez Torre E, Novoa-Testa I, Cordido F, Pascual-Corrales E, Rodríguez Berrocal V, Guerrero-Pérez F, Vicente A, Carlos Percovich Hualpa J, García-Centeno R, González-Fernández L, Dolores Ollero García M, Irigaray Echarri A, Dolores Moure Rodríguez M, Novo-Rodríguez C, Calatayud M, Villar-Taibo R, Bernabéu I, Alvarez-Escola C, Benítez Valderrama P, Tenorio-Jiménez C, Abellán Galiana P, Venegas E, González-Molero I, Iglesias P, Blanco-Carrera C, Vidal-Ostos De Lara F, de Miguel Novoa P, López Mezquita E, Alexandra Hanzu F, Aldecoa I, Aznar S, Lamas C, Aulinas A, Asla Q, Gracia Gimeno P, María Recio-Córdova J, Dolores Avilés-Pérez M, Asensio-Wandosell D, Sampedro-Núñez M, Cámara R, Paja Fano M, Ruz-Caracuel I, Fajardo C, Marazuela M, and Puig-Domingo M
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- Humans, Neuroendocrine Tumors drug therapy, Neuroendocrine Tumors metabolism, Pituitary Neoplasms drug therapy, Pituitary Neoplasms metabolism, Somatostatin analogs & derivatives, Somatostatin therapeutic use, Prolactin metabolism, Prolactin blood, Human Growth Hormone analogs & derivatives
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- 2024
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8. Pegvisomant and pasireotide in PRL and GH co-secreting vs GH-secreting Pit-NETs.
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Araujo-Castro M, Biagetti B, Menéndez Torre E, Novoa-Testa I, Cordido F, Pascual-Corrales E, Rodríguez Berrocal V, Guerrero-Pérez F, Vicente A, Percovich Hualpa JC, García-Centeno R, González-Fernández L, Ollero García MD, Irigaray Echarri A, Moure Rodríguez MD, Novo-Rodríguez C, Calatayud M, Villar-Taibo R, Bernabéu I, Alvarez-Escola C, Benítez Valderrama P, Tenorio-Jiménez C, Abellán Galiana P, Venegas E, González-Molero I, Iglesias P, Blanco-Carrera C, Vidal-Ostos De Lara F, de Miguel Novoa P, López Mezquita E, Hanzu FA, Aldecoa I, Aznar S, Lamas C, Aulinas A, Asla Q, Gracia Gimeno P, Recio-Córdova JM, Avilés-Pérez MD, Asensio-Wandosell D, Sampedro-Núñez M, Cámara R, Paja Fano M, Ruz-Caracuel I, Fajardo C, Marazuela M, and Puig-Domingo M
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- Humans, Male, Female, Middle Aged, Adult, Retrospective Studies, Pituitary Neoplasms drug therapy, Pituitary Neoplasms metabolism, Aged, Young Adult, Somatostatin analogs & derivatives, Somatostatin therapeutic use, Human Growth Hormone analogs & derivatives, Human Growth Hormone therapeutic use, Prolactin blood, Prolactin metabolism, Neuroendocrine Tumors drug therapy, Neuroendocrine Tumors metabolism, Acromegaly drug therapy, Acromegaly metabolism
- Abstract
The objective of the study was to evaluate the efficacy of second-line therapies in patients with acromegaly caused by a growth hormone (GH) and prolactin (PRL) co-secreting pituitary neuroendocrine tumor (GH&PRL-Pit-NET) compared to their efficacy in patients with acromegaly caused by a GH-secreting pituitary neuroendocrine tumor (GH-Pit-NET). This is a multicenter retrospective study of patients with acromegaly on treatment with pasireotide and/or pegvisomant. Patients were classified in two groups: GH&PRL-Pit-NETs when evidence of hyperprolactinemia and immunohistochemistry (IHC) for GH and PRL was positive or if PRL were >200 ng/dL regardless of the PRL-IHC and GH-Pit-NETs when the previously mentioned criteria were not met. A total of 28 cases with GH&PRL-Pit-NETs and 122 with GH-Pit-NETs met the inclusion criteria. GH&PRL-Pit-NETs presented at a younger age, caused hypopituitarism, and were invasive more frequently than GH-Pit-NETs. There were 124 patients treated with pegvisomant and 49 with pasireotide at any time. The efficacy of pegvisomant for IGF-1 normalization was of 81.5% and of pasireotide of 71.4%. No differences in IGF-1 control with pasireotide and with pegvisomant were observed between GH&PRL-Pit-NETs and GH-Pit-NETs. All GH&PRL-Pit-NET cases treated with pasireotide (n = 6) and 82.6% (n = 19/23) of the cases treated with pegvisomant normalized PRL levels. No differences in the rate of IGF-1 control between pegvisomant and pasireotide were detected in patients with GH&PRL-Pit-NETs (84.9% vs 66.7%, P = 0.178). We conclude that despite the more aggressive behavior of GH&PRL-Pit-NETs than GH-Pit-NETs, no differences in the rate of IGF-1 control with pegvisomant and pasireotide were observed between both groups, and both drugs have shown to be effective treatments to control IGF-1 and PRL hypersecretion in these tumors.
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- 2024
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9. Is adrenal venous sampling always necessary to differentiate between unilateral and bilateral primary aldosteronism? Lesson from the SPAIN-ALDO register.
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Parra Ramírez P, Martín Rojas-Marcos P, Paja Fano M, González Boillos M, Peris BP, Pascual-Corrales E, García Cano AM, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo CM, Manjón-Miguélez L, García Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales-Ruiz M, Calatayud M, Furio Collao SA, Meneses D, Sampedro Nuñez MA, Escudero Quesada V, Mena Ribas E, Sanmartín Sánchez A, Gonzalvo Diaz C, Lamas C, Guerrero-Vázquez R, Del Castillo Tous M, Serrano Gotarredona J, Michalopoulou Alevras T, Moya Mateo EM, Hanzu FA, and Araujo-Castro M
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- Humans, Female, Middle Aged, Male, Retrospective Studies, Spain epidemiology, Adult, Registries, Aged, Diagnosis, Differential, Adrenalectomy, Hyperaldosteronism blood, Hyperaldosteronism diagnosis, Hyperaldosteronism surgery, Adrenal Glands blood supply, Adrenal Glands diagnostic imaging, Aldosterone blood
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Purpose: To evaluate whether the clinical, biochemical and radiological features of patients with primary aldosteronism (PA) can predict both main subtypes of PA., Methods: A retrospective multicenter study of PA patients followed in 27 Spanish tertiary hospitals (SPAIN-ALDO Register). Only patients with confirmed unilateral or bilateral PA based on adrenal venous sampling (AVS) and/or postsurgical biochemical cure after adrenalectomy were included. Supervised regression techniques were used for model development., Results: 328 patients [270 unilateral PA (UPA), 58 bilateral PA (BPA)] were included. The area under the curve (AUC) for aldosterone/potassium ratio and aldosterone responses following saline infusion test were 0.602 [95%CI 0.520 to 0.684] and 0.574 [95% CI 0.446-0.701], respectively, to differentiate UPA from BPA. The AUC was 0.825 [95% 0.764-0.886] when the prediction model with seven parameters - comorbidities (dyslipidemia, cerebrovascular disease, sleep apnea syndrome [SAS]), systolic blood pressure (SBP), plasma aldosterone levels (PAC), hypokalemia and unilateral adrenal nodule >1 cm and normal contralateral adrenal gland on CT/MRI - was used. In patients without comorbidities, hypokalemia, SBP > 160 mmHg, PAC > 40 ng/dL, and unilateral adrenal lesions were associated with a likelihood of having a UPA of 98.5%. The chance of BPA was higher in individuals with comorbidities, SBP < 140 mmHg, normokalemia, low PAC levels, and no adrenal tumors on the CT/MRI (91.5%)., Conclusion: A combination of high PAC, SBP > 160 mmHg, low serum potassium, a unilateral adrenal nodule>1 cm and no comorbidities could predict a UPA with a 98.5% accuracy., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2024
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10. Differences in the clinical and hormonal presentation of patients with familial and sporadic primary aldosteronism.
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Araujo-Castro M, Parra P, Martín Rojas-Marcos P, Paja Fano M, González Boillos M, Pascual-Corrales E, García Cano AM, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo CM, Manjón-Miguélez L, García Centeno R, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales-Ruiz M, Calatayud M, Furio Collao SA, Meneses D, Sampedro Nuñez M, Escudero Quesada V, Mena Ribas E, Sanmartín Sánchez A, Gonzalvo Diaz C, Lamas C, Del Castillo Tous M, Serrano Gotarredona J, Michalopoulou Alevras T, Moya Mateo EM, and Hanzu FA
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- Female, Humans, Aldosterone, Potassium, Hyperaldosteronism complications, Hyperaldosteronism genetics, Hyperaldosteronism epidemiology, Hypokalemia etiology
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Purpose: To compare the clinical and hormonal characteristics of patients with familial hyperaldosteronism (FH) and sporadic primary aldosteronism (PA)., Methods: A systematic review of the literature was performed for the identification of FH patients. The SPAIN-ALDO registry cohort of patients with no suspicion of FH was chosen as the comparator group (sporadic group)., Results: A total of 360 FH (246 FH type I, 73 type II, 29 type III, and 12 type IV) cases and 830 sporadic PA patients were included. Patients with FH-I were younger than sporadic cases, and women were more commonly affected (P = 0.003). In addition, the plasma aldosterone concentration (PAC) was lower, plasma renin activity (PRA) higher, and hypokalemia (P < 0.001) less frequent than in sporadic cases. Except for a younger age (P < 0.001) and higher diastolic blood pressure (P = 0.006), the clinical and hormonal profiles of FH-II and sporadic cases were similar. FH-III had a distinct phenotype, with higher PAC and higher frequency of hypokalemia (P < 0.001), and presented 45 years before sporadic cases. Nevertheless, the clinical and hormonal phenotypes of FH-IV and sporadic cases were similar, with the former being younger and having lower serum potassium levels., Conclusion: In addition to being younger and having a family history of PA, FH-I and III share other typical characteristics. In this regard, FH-I is characterized by a low prevalence of hypokalemia and FH-III by a severe aldosterone excess causing hypokalemia in more than 85% of patients. The clinical and hormonal phenotype of type II and IV is similar to the sporadic cases., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Araujo-Castro, Parra, Martín Rojas-Marcos, Paja Fano, González Boillos, Pascual-Corrales, García Cano, Ruiz-Sanchez, Vicente Delgado, Gómez Hoyos, Ferreira, García Sanz, Recasens Sala, Barahona San Millan, Picón César, Díaz Guardiola, Perdomo, Manjón-Miguélez, García Centeno, Rebollo Román, Gracia Gimeno, Robles Lázaro, Morales-Ruiz, Calatayud, Furio Collao, Meneses, Sampedro Nuñez, Escudero Quesada, Mena Ribas, Sanmartín Sánchez, Gonzalvo Diaz, Lamas, del Castillo Tous, Serrano Gotarredona, Michalopoulou Alevras, Moya Mateo and Hanzu.)
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- 2024
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11. Renin as a Biomarker to Guide Medical Treatment in Primary Aldosteronism Patients. Findings from the SPAIN-ALDO Registry.
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Parra Ramírez P, Martín Rojas-Marcos P, Paja Fano M, González-Boillos M, Pascual-Corrales E, García Cano AM, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo CM, Manjón-Miguélez L, Rebollo Román Á, Robles Lázaro C, Morales-Ruiz M, Calatayud M, Andree Furio Collao S, Meneses D, Sampedro-Nuñez MA, Mena Ribas E, Sanmartín Sánchez A, Gonzalvo Diaz C, Lamas C, Guerrero-Vázquez R, Del Castillo Tous M, Serrano Gotarredona J, Michalopoulou Alevras T, Tenés Rodrigo S, Roa Chamorro R, Jaen Aguila F, Moya Mateo EM, Hanzu FA, and Araujo-Castro M
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- Humans, Adrenalectomy, Aldosterone, Biomarkers, Mineralocorticoid Receptor Antagonists pharmacology, Mineralocorticoid Receptor Antagonists therapeutic use, Potassium metabolism, Registries, Renin metabolism, Retrospective Studies, Spain epidemiology, Cardiovascular Diseases drug therapy, Hyperaldosteronism diagnosis, Hyperaldosteronism drug therapy, Hyperaldosteronism epidemiology, Hypertension diagnosis, Hypertension drug therapy, Hypertension epidemiology
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Introduction: Primary aldosteronism (PA) is associated with several cardiometabolic comorbidities. Specific treatment by mineralocorticoid receptor antagonists (MRA) or adrenalectomy has been reported to reduce the cardiometabolic risk. However, the cardiovascular benefit could depend on plasma renin levels in patients on MRA., Aim: To compare the development of cardiovascular, renal and metabolic complications between medically treated patients with PA and those who underwent adrenalectomy, taking the renin status during MRA treatment into account., Methods: A multicenter retrospective study (SPAIN-ALDO Register) of patients with PA treated at 35 Spanish tertiary hospitals. Patients on MRA were divided into two groups based on renin suppression (n = 90) or non-suppression (n = 70). Both groups were also compared to unilateral PA patients (n = 275) who achieved biochemical cure with adrenalectomy., Results: Adrenalectomized patients were younger, had higher plasma aldosterone concentration, and lower potassium levels than MRA group. Patients on MRA had similar baseline characteristics when stratified into treatment groups with suppressed and unsuppressed renin. 97 (55.1%) of 176 patients without comorbidities at diagnosis, developed at least one comorbidity during follow-up (median 12 months vs. 12.5 months' follow-up after starting MRA and surgery, respectively). Surgery group had a lower risk of developing new cardiovascular events (HR 0.40 [95% CI 0.18-0.90]) than MRA group. Surgical treatment improved glycemic and blood pressure control, increased serum potassium levels, and required fewer antihypertensive drugs than medical treatment. However, there were no differences in the cardiometabolic profile or the incidence of new comorbidities between the groups with suppressed and unsuppressed renin levels (HR 0.95 [95% CI 0.52-1.73])., Conclusion: Cardiovascular, renal, and metabolic events were comparable in MRA patients with unsuppressed and suppressed renin. Effective surgical treatment of PA was associated with a decreased incidence of new cardiovascular events when compared to MRA therapy., (© 2024. Italian Society of Hypertension.)
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- 2024
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12. Effect of Obesity on Clinical Characteristics of Primary Aldosteronism Patients at Diagnosis and Postsurgical Response.
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Ruiz-Sánchez JG, Paja-Fano M, González Boillos M, Pla Peris B, Pascual-Corrales E, García Cano AM, Parra Ramírez P, Martín Rojas-Marcos P, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, García González JJ, Perdomo CM, Manjón Miguélez L, García Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales-Ruiz M, Hanzu FA, and Araujo-Castro M
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- Humans, Male, Middle Aged, Female, Antihypertensive Agents therapeutic use, Aldosterone, Renin, Retrospective Studies, Adrenalectomy, Obesity complications, Obesity epidemiology, Obesity drug therapy, Hyperaldosteronism complications, Hyperaldosteronism diagnosis, Hyperaldosteronism epidemiology, Hypertension drug therapy
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Context: Patients with obesity have an overactivated renin-angiotensin-aldosterone system (RAAS) that is associated with essential hypertension. However, the influence of obesity in primary aldosteronism (PA) is unknown., Objective: We analyzed the effect of obesity on the characteristics of PA, and the association between obesity and RAAS components., Methods: A retrospective study was conducted of the Spanish PA Registry (SPAIN-ALDO Registry), which included patients with PA seen at 20 tertiary centers between 2018 and 2022. Differences between patients with and without obesity were analyzed., Results: A total of 415 patients were included; 189 (45.5%) with obesity. Median age was 55 years (range, 47.3-65.2 years) and 240 (58.4%) were male. Compared to those without obesity, patients with obesity had higher rates of diabetes mellitus, chronic kidney disease, obstructive apnea syndrome, left ventricular hypertrophy, prior cardiovascular events, higher means of systolic blood pressure, and required more antihypertensive drugs. Patients with PA and obesity also had higher values of serum glucose, glycated hemoglobin A1c, creatinine, uric acid, and triglycerides, and lower levels of high-density lipoprotein cholesterol. Levels of blood aldosterone (PAC) and renin were similar between patients with and without obesity. Body mass index was not correlated with PAC nor renin. The rates of adrenal lesions on imaging studies, as well as the rates of unilateral disease assessed by adrenal vein sampling or I-6β-iodomethyl-19-norcholesterol scintigraphy, were similar between groups., Conclusion: Obesity in PA patients involves a worse cardiometabolic profile, and need for more antihypertensive drugs but similar PAC and renin levels, and rates of adrenal lesions and lateral disease than patients without obesity. However, obesity implicates a lower rate of hypertension cure after adrenalectomy., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.)
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- 2023
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13. Autonomous cortisol secretion in patients with primary aldosteronism: prevalence and implications on cardiometabolic profile and on surgical outcomes.
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Araujo-Castro M, Paja Fano M, Pla Peris B, González Boillos M, Pascual-Corrales E, García-Cano AM, Parra Ramírez P, Rojas-Marcos PM, Ruiz-Sanchez JG, Vicente A, Gómez-Hoyos E, Ferreira R, García Sanz I, Recasens M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Perdomo C, Manjón L, García-Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales M, Calatayud M, Collao SAF, Meneses D, Sampedro Nuñez MA, Escudero Quesada V, Ribas EM, Sanmartín Sánchez A, Diaz CG, Lamas C, Guerrero-Vázquez R, Del Castillo Tous M, Serrano J, Michalopoulou T, Moya Mateo EM, and Hanzu F
- Abstract
Purpose: The aim of this study was to evaluate the prevalence of autonomous cortisol secretion (ACS) in patients with primary aldosteronism (PA) and its implications on cardiometabolic and surgical outcomes., Methods: This is a retrospective multicenter study of PA patients who underwent 1 mg dexamethasone-suppression test (DST) during diagnostic workup in 21 Spanish tertiary hospitals. ACS was defined as a cortisol post-DST >1.8 µg/dL (confirmed ACS if >5 µg/dL and possible ACS if 1.8-5 µg/dL) in the absence of specific clinical features of hypercortisolism. The cardiometabolic profile was compared with a control group with ACS without PA (ACS group) matched for age and DST levels., Results: The prevalence of ACS in the global cohort of patients with PA (n = 176) was 29% (ACS-PA; n = 51). Ten patients had confirmed ACS and 41 possible ACS. The cardiometabolic profile of ACS-PA and PA-only patients was similar, except for older age and larger tumor size of the adrenal lesion in the ACS-PA group. When comparing the ACS-PA group (n = 51) and the ACS group (n = 78), the prevalence of hypertension (OR 7.7 (2.64-22.32)) and cardiovascular events (OR 5.0 (2.29-11.07)) was higher in ACS-PA patients than in ACS patients. The coexistence of ACS in patients with PA did not affect the surgical outcomes, the proportion of biochemical cure and clinical cure being similar between ACS-PA and PA-only groups., Conclusion: Co-secretion of cortisol and aldosterone affects almost one-third of patients with PA. Its occurrence is more frequent in patients with larger tumors and advanced age. However, the cardiometabolic and surgical outcomes of patients with ACS-PA and PA-only are similar.
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- 2023
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14. Diagnostic Accuracy of Adrenal Iodine-131 6-Beta-Iodomethyl-19-Norcholesterol Scintigraphy for the Subtyping of Primary Aldosteronism.
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Araujo-Castro M, Paja Fano M, González Boillos M, Pascual-Corrales E, García Cano AM, Parra Ramírez P, Martín Rojas-Marcos P, Vicente Delgado A, Casteràs A, Puig A, García Sanz I, Díaz Guardiola P, Robles Lázaro C, Núñez MAS, Guerrero-Vázquez R, Del Castillo Tous M, Michalopoulou Alevras T, Tenes Rodrigo S, and Hanzu FA
- Abstract
Purpose: To evaluate the diagnostic accuracy of the 131I-6β-iodomethyl-19-norcholesterol (NP-59) adrenal scintigraphy for the subtyping diagnosis of primary aldosteronism (PA), considering as gold standard for the diagnosis of unilateral PA (UPA), either the results of the adrenal venous sampling (AVS) or the outcome after adrenalectomy., Methods: A retrospective multicenter study was performed on PA patients from 14 Spanish tertiary hospitals who underwent NP-59 scintigraphy with an available subtyping diagnosis. Patients were classified as UPA if biochemical cure was achieved after adrenalectomy or/and if an AVS lateralization index > 4 with ACTH stimulation or >2 without ACTH stimulation was observed. Patients were classified as having bilateral PA (BPA) if the AVS lateralization index was ≤4 with ACTH or ≤2 without ACTH stimulation or if there was evidence of bilateral adrenal nodules >1 cm in each adrenal gland detected by CT/MRI., Results: A total of 86 patients with PA were included (70.9% (n = 61) with UPA and 29.1% (n = 25) with BPA). Based on the NP-59 scintigraphy results, 16 patients showed normal suppressed adrenal gland uptake, and in the other 70 cases, PA was considered unilateral in 49 patients (70%) and bilateral in 21 (30%). Based on 59-scintigraphy results, 10.4% of the patients with unilateral uptake had BPA, and 27.3% of the cases with bilateral uptake had UPA. The AUC of the ROC curve of the NP-59 scintigraphy for PA subtyping was 0.812 [0.707-0.916]. Based on the results of the CT/MRI and NP-59 scintigraphy, only 6.7% of the patients with unilateral uptake had BPA, and 24% of the cases with bilateral uptake had UPA. The AUC of the ROC curve of the model combining CT/MRI and 59-scintigraphy results for subtyping PA was 0.869 [0.782-0.957]., Conclusion: The results of NP-59 scintigraphy in association with the information provided by the CT/MRI may be useful for PA subtyping. However, their diagnostic accuracy is only moderate. Therefore, it should be considered a second-line diagnostic tool when AVS is not an option.
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- 2023
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15. The role of BRAF V600E mutation in post-surgical 131I therapy in papillary thyroid carcinoma: a study based on SPECT-CT uptake analysis.
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Domínguez-Ayala M, Mínguez-Gabiña P, Paja-Fano M, Bilbao-González A, Expósito-Rodríguez A, and Rodeño-Ortiz de Zarate E
- Subjects
- Humans, Thyroid Cancer, Papillary diagnostic imaging, Thyroid Cancer, Papillary genetics, Iodine Radioisotopes therapeutic use, Proto-Oncogene Proteins B-raf genetics, Prospective Studies, Mutation, Tomography, Emission-Computed, Single-Photon, Tomography, X-Ray Computed, Thyroid Neoplasms diagnostic imaging, Thyroid Neoplasms genetics, Thyroid Neoplasms radiotherapy, Carcinoma drug therapy, Carcinoma genetics, Carcinoma pathology, Carcinoma, Papillary diagnostic imaging, Carcinoma, Papillary genetics, Carcinoma, Papillary radiotherapy
- Abstract
Background: The BRAF V600E mutation (BRAF mut) in papillary thyroid cancer (PTC) has been associated with poor response to therapy with
131 I in patients with metastases but the results in postsurgical treatment are controversial. Our main objective is to investigate the impact of the mutation on the biokinetics of the administered131 I therapy after surgery., Methods: A prospective study was designed, from July 2015 to January 2018 which included patients with PTC receiving131 I therapy after surgical treatment. To study the biokinetics of the radioiodine in postoperative thyroid remnants, SPECT-CT images were acquired so as to obtain the following variables: percentage of remnant uptake at 2 and 7 days post-administration, effective half-life and time-integrated activity coefficient. All of them were compared depending on the mutational diagnosis and other clinical features and pathological variables., Results: Sixty-one patients, and in total 103 thyroid remnants, were included. About 59% of patients were BRAF mutated. The mutation was associated with classic variant (88.5% vs. 11.5%; P=0.0001), desmoplastic reaction (85.7% vs. 14.3%; P=0.002), smaller tumor size (1.5 vs. 2.1 cm; P=0.024), nodal disease (3.3 vs. 1; P=0.001) and advanced stages (76.9% vs. 23%; P=0.014). The BRAFmut group had a lower percentage of131 I uptake at 2 days (0.17% vs. 0.47%; P=0.001) and at 7 days (0.02% vs. 0.1%; P=0.013); and a lower time-integrated activity coefficient (0.05h vs. 0.17 h; P=0.002). In univariate analysis, in addition to the mutation, the histological variant was significant but only for time-integrated activity coefficient (P=0.04). In multivariate analysis, only mutation determined the 2-day uptake (P<0.001) and the time-integrated activity coefficient (P<0.001)., Conclusions: The BRAF V600E mutation is associated with lower131 I uptake in thyroid remnants. Furthermore, it is an independent factor that decreases the effect of post-surgical131 I therapy, and therefore, it could be used as a potential tool to optimize the treatment of PTC.- Published
- 2023
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16. Predictive model of hypertension resolution after adrenalectomy in primary aldosteronism: the SPAIN-ALDO score.
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Araujo-Castro M, Paja Fano M, González Boillos M, Pla Peris B, Pascual-Corrales E, García Cano AM, Parra Ramírez P, Martín Rojas-Marcos P, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, García González JJ, Perdomo CM, Manjón Miguélez L, García Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales-Ruiz M, and Hanzu FA
- Subjects
- Humans, Female, Adult, Middle Aged, Male, Adrenalectomy, Antihypertensive Agents therapeutic use, Spain, Treatment Outcome, Retrospective Studies, Hyperaldosteronism, Hypertension drug therapy, Diabetes Mellitus, Type 2 drug therapy
- Abstract
Purpose: To develop a predictive model of hypertension resolution after adrenalectomy in patients with primary aldosteronism (PA), based on their presurgical characteristics., Methods: A retrospective multicenter study of PA patients in follow-up in 20 Spanish tertiary hospitals between 2018 and 2021 was performed (SPAIN-ALDO Register). Clinical response postadrenalectomy was classified according to the primary aldosteronism surgical outcome (PASO) consensus criteria. The predictive model was developed using a multivariate logistic regression model with the estimation of all possible equations., Results: A total of 146 patients (54.8% females; mean age of 51.5 ± 10.9 years) with PA who underwent unilateral adrenalectomy were included. After a mean follow-up of 29.1 ± 30.43 months after surgery, hypertension cure was obtained in 37.7% ( n = 55) of the patients. The predictive model with the highest diagnostic accuracy to predict hypertension cure combined the variables female sex, use of two or fewer antihypertensive medications, hypertension grade 1, without type 2 diabetes and nonobesity. The area under the receiver operating characteristic curve of this model was 0.841 [0.769-0.914]. Based on this model, the group of patients with a higher probability of cure (80.4%) were those without type 2 diabetes, BMI <30 kg/m 2 , female sex, hypertension grade 1 and who use two or fewer antihypertensives. Our predictive model offered a slightly higher diagnostic accuracy than Wachtel's (area under the curve [AUC]: 0.809), Utsumi's (AUC: 0.804) and Zarnegar's (AUC: 0.796) models and was similar than the Burello's (AUC: 0.833) model., Conclusion: Female sex, use of two or fewer antihypertensive medications, hypertension grade 1, no type 2 diabetes and nonobesity may predict hypertension cure after adrenalectomy in patients with PA. Our score provides a potential tool to guide preoperative patient counseling., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2022
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17. Adrenal venous sampling in primary aldosteronism: Experience of a Spanish multicentric study (Results from the SPAIN-ALDO Register).
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Araujo-Castro M, Paja Fano M, González Boillos M, Pla Peris B, Pascual-Corrales E, García Cano AM, Parra Ramírez P, Rojas-Marcos PM, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Recasens Sala M, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, García González JJ, Perdomo CM, Manjón Miguélez L, García Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales-Ruiz M, and Hanzu FA
- Subjects
- Humans, Spain, Adrenal Glands diagnostic imaging, Adrenal Glands blood supply, Magnetic Resonance Imaging, Retrospective Studies, Aldosterone, Adrenalectomy, Hyperaldosteronism diagnosis, Hyperaldosteronism surgery
- Abstract
Objective: The aim of this study was to evaluate the rate of adrenal venous sampling (AVS) performance in patients with primary aldosteronism (PA), the main reasons for its non-performance, and the success and complications rate of this procedure in Spain. Moreover, the concordance between CT/MRI and AVS for PA subtyping was evaluated., Methods: A retrospective multicenter study of PA patient follow-up in 20 Spanish tertiary hospitals between 2018-2021 was performed (SPAIN-ALDO Register)., Results: Of the 440 patients with PA included in the study, 153 underwent AVS (34.8%). The main reasons for not performing AVS were: patient rejection to the procedure, low catheterization rate in the center and unilateral disease based on CT/MRI. The overall success rate was 44.4% (the left adrenal vein was properly canulated in 77.8% and the right adrenal vein in 48.4%). Only 3 patients experienced minor complications. In the 45 patients with unilateral disease according to AVS, CT/MRI indicated bilateral disease or normal adrenal glands in 17. In the 23 patients with bilateral disease, CT/MRI indicated unilateral disease in 14. However, no significant differences were observed in biochemical response (P = 0.051) and hypertension resolution (P = 0.150) between patients who underwent surgery based on CT/MRI results and those who underwent surgery based on AVS results., Conclusion: In our setting, AVS is still an underused technique in patients with PA. The low experience and success rate in AVS partially justify these results. More training for providers and patients needs to be done to include appropriate well performed AVS in the diagnosis algorithm of PA., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2022
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18. Non-endocrine hyperaldosteronism. When hormones are supporting players.
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Paja-Fano M, Gutiérrez-Macías A, and García-Barcina MJ
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- Humans, Hormones, Hyperaldosteronism complications
- Published
- 2022
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19. Sentinel lymph node biopsy in papillary thyroid cancer: Accuracy and application in clinical practice.
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Expósito Rodríguez A, Corta Gómez I, Domínguez Ayala M, García Carrillo M, González García AI, Gutiérrez Rodríguez MT, Oleaga Alday A, Paja Fano M, Rodríguez Iriarte ML, Ugalde Olano A, and Zabala Landa R
- Subjects
- Humans, Lymphatic Metastasis, Neoplasm Staging, Prospective Studies, Thyroid Cancer, Papillary surgery, Sentinel Lymph Node Biopsy methods, Thyroid Neoplasms pathology, Thyroid Neoplasms surgery
- Abstract
Introduction: The presence of lymph nodes metastasis in papillary thyroid cancer (PTC) modifies the type of surgical resection as well as the indication of the treatment with I
131 in the postoperative period. This therapeutic approach is based on the results of the diagnostic tests, like the cervical ultrasonography. Currently other methods of diagnostic are tested as selective sentinel lymph node biopsy (SLNB). It can complement to the ultrasound results. The aim was to validate the SLNB for use in the diagnosis of lymph node metastasis by papillary thyroid cancer., Methods: Observational prospective cohort study of 55 patients who underwent PTC without suspicion of lymph node involvement clinical or radiological, since February 2012 through February 2015, with a follow-up between 6 and 8 years. It was used99 Tc with intratumoral nanocoloid and a portable tube of the gamma camera for the detection of the sentinel node (SN)., Variables: age, gender, histological, analytical and preoperative and postoperative staging. The sensitivity, specificity and predictive values of technique was calculated. The validation was determined by calculating the detectability and the false negative results of the test., Results: 53 of the 55 patients (96,36%) there was the SN detection. The FN were 4 patients (7,5%). Of the rest, after applying the SLNB, 24 (48,9%) were kept as N0, 14 (28,5%) became N1a and 11 (22,4%) were classified as N1b. The differences observed in the study were significant (P < ,05). The sensitivity was 86,21%, the specificity of 100%, the PPV was 100% and the NPV of 85.71%. The diagnostic accuracy of 92,45%., Conclusion: The SLNB is a valid technique for use in patients suffering from papillary thyroid cancer with a high diagnostic accuracy., (Copyright © 2021 AEC. Published by Elsevier España, S.L.U. All rights reserved.)- Published
- 2022
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20. Differences in the presentation and evolution of primary aldosteronism in elderly (≥65 years) and young patients (<65 years).
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Parra Ramírez P, Rojas-Marcos PM, Paja Fano M, González Boillos M, Pascual-Corrales E, García-Cano A, Ruiz-Sanchez JG, Vicente A, Gómez-Hoyos E, Ferreira R, García Sanz I, Recasens M, Pla Peris B, Barahona San Millan R, Picón César MJ, Díaz Guardiola P, Jesús García González J, Perdomo C, Manjón L, García-Centeno R, Percovich JC, Rebollo Román Á, Gracia Gimeno P, Robles Lázaro C, Morales M, Hanzu F, and Araujo-Castro M
- Abstract
Objective: To compare the presentation and evolution of primary aldosteronism (PA) in the elderly (≥65 years) and young patients (<65 years)., Methods: A retrospective multicenter study was performed in 20 Spanish hospitals of PA patients in follow-up between 2018 and 2021., Results: Three hundred fifty-two patients with PA <65 years and 88 patients ≥65 years were included. Older PA patients had a two-fold higher prevalence of type 2 diabetes, dyslipidemia, and cerebrovascular disease, but these differences disappeared after adjusting for hypertension duration. At diagnosis, diastolic blood pressure was lower than in young patients (83.3 ± 11.54 vs 91.6 ± 14.46 mmHg, P < 0.0001). No differences in the rate of overall correct cannulation (56.5% vs 42.3%, P = 0.206) or the diagnosis of unilaterality (76.9% vs 62.5%, P = 0.325) in the adrenal venous sampling (AVS) was observed between the elderly and young groups. However, there was a lower proportion of PA patients who underwent adrenalectomy in the elderly group than in the younger group (22.7% (n = 20) vs 37.5% (n = 132), P = 0.009). Nevertheless, no differences in the rate of postsurgical biochemical (100% (n = 14) vs 92.8% (n = 90), P = 0.299) and hypertension cure (38.6% (n = 51) vs 25.0% (n = 5), P = 0.239) were observed between both groups., Conclusion: Older patients with PA have a worse cardiometabolic profile than young patients with PA that it is related to a longer duration of hypertension. However, the results of the AVS, and adrenalectomy are similar in both groups. Therefore, the management of elderly patients with PA should be based not only on age, but rather on the overall medical, physical, social, and mental characteristics of the patients.
- Published
- 2022
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21. Evolution of the cardiometabolic profile of primary hyperaldosteronism patients treated with adrenalectomy and with mineralocorticoid receptor antagonists: results from the SPAIN-ALDO Registry.
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Araujo-Castro M, Paja Fano M, González Boillos M, Pla Peris B, Pascual-Corrales E, García Cano AM, Parra Ramírez P, Rojas-Marcos PM, Ruiz-Sanchez JG, Vicente Delgado A, Gómez Hoyos E, Ferreira R, García Sanz I, Díaz Guardiola P, García González JJ, Perdomo CM, Morales M, and Hanzu FA
- Subjects
- Adrenalectomy adverse effects, Female, Humans, Mineralocorticoid Receptor Antagonists therapeutic use, Potassium therapeutic use, Registries, Retrospective Studies, Spain epidemiology, Hyperaldosteronism drug therapy, Hyperaldosteronism epidemiology, Hyperaldosteronism surgery, Hypertension drug therapy, Hypertension epidemiology
- Abstract
Objective: To analyze the evolution of the cardiometabolic profile of patients with primary hyperaldosteronism (PA) after the treatment with surgery and with mineralocorticoid receptor antagonists (MRA)., Design: Retrospective multicentric study of patients with PA on follow-up in twelve Spanish centers between 2018 and 2020., Results: 268 patients with PA treated by surgery (n = 100) or with MRA (n = 168) were included. At baseline, patients treated with surgery were more commonly women (54.6% vs 41.7%, P = 0.042), had a higher prevalence of hypokalemia (72.2% vs 58%, P = 0.022) and lower prevalence of obesity (37.4% vs 51.3%, P = 0.034) than patients treated with MRA. Adrenalectomy resulted in complete biochemical cure in 94.0% and clinical response in 83.0% (complete response in 41.0% and partial response in 42.0%). After a median follow-up of 23.6 (IQR 9.7-53.8) months, the reduction in blood pressure (BP) after treatment was similar between the group of surgery and MRA, but patients surgically treated reduced the number of antihypertensive pills for BP control more than those medically treated (∆antihypertensives: -1.3 ± 1.3 vs 0.0 ± 1.4, P < 0.0001) and experienced a higher increased in serum potassium levels (∆serum potassium: 0.9 ± 0.7 vs 0.6 ± 0.8mEq/ml, P = 0.003). However, no differences in the risk of the onset of new renal and cardiometabolic comorbidities was observed between the group of surgery and MRA (HR = 0.9 [0.5-1.5], P = 0.659)., Conclusion: In patients with PA, MRA and surgery offer a similar short-term cardiovascular protection, but surgery improves biochemical control and reduces pill burden more commonly than MRA, and lead to hypertension cure or improvement in up to 83% of the patients., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
- Published
- 2022
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22. Immunotherapy-induced isolated ACTH deficiency in cancer therapy.
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Iglesias P, Peiró I, Biagetti B, Paja-Fano M, Cobo DA, García Gómez C, Mateu-Salat M, Genua I, Majem M, Riudavets M, Gavira J, Lamas C, Fernández Pombo A, Guerrero-Pérez F, Villabona C, Cabezas Agrícola JM, Webb SM, and Díez JJ
- Subjects
- Adrenocorticotropic Hormone deficiency, Aged, Endocrine System Diseases, Genetic Diseases, Inborn, Humans, Hypoglycemia, Immunotherapy adverse effects, Ipilimumab adverse effects, Male, Middle Aged, Retrospective Studies, Melanoma drug therapy, Nivolumab adverse effects
- Abstract
Central adrenal insufficiency (AI) due to isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) has been recently associated with immune checkpoint inhibitor (ICI) therapy. Our aim was to analyze the prevalence, clinical characteristics, and therapeutic outcomes in cancer patients with IAD induced by ICI therapy. A retrospective and multicenter study was performed. From a total of 4447 cancer patients treated with ICI antibodies, 37 (0.8%) (23 men (62.2%), mean age 64.7 ± 8.3 years (range 46-79 years)) were diagnosed with IAD. The tumor most frequently related to IAD was lung cancer (n = 20, 54.1%), followed by melanoma (n = 8, 21.6%). The most common ICI antibody inhibitors reported were nivolumab (n = 18, 48.6%), pembrolizumab (n = 16, 43.2%), and ipilimumab (n = 8, 21.6%). About half of the patients (n = 19, 51.4%) had other immune-related adverse events, mainly endocrine adverse effects (n = 10, 27.0%). IAD was diagnosed at a median time of 7.0 months (IQR, 5-12) after starting immunotherapy. The main reported symptom at presentation was fatigue (97.3%), followed by anorexia (81.8%) and general malaise (81.1%). Mean follow-up time since IAD diagnosis was 15.2 ± 12.5 months (range 0.3-55 months). At last visit, all patients continued with hormonal deficiency of ACTH. Median overall survival since IAD diagnosis was 6.0 months. In conclusion, IAD is a rare but a well-established complication associated with ICI therapy in cancer patients. It develops around 7 months after starting the treatment, mainly anti-PD1 antibodies. Recovery of the corticotropic axis function should not be expected.
- Published
- 2021
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23. Clinical and genetic characteristics in patients under 30 years with sporadic pituitary adenomas.
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Martínez de LaPiscina I, Portillo Najera N, Rica I, Gaztambide S, Webb SM, Santos A, Moure MD, Paja Fano M, Hernandez MI, Chueca-Guindelain MJ, Hernández-Ramírez LC, Soto A, Valdés N, and Castaño L
- Subjects
- Adolescent, Adult, Age of Onset, Child, Child, Preschool, Chile epidemiology, Cohort Studies, Female, Gene Frequency, Genetic Association Studies, Genetic Predisposition to Disease, Genetic Testing, Germ-Line Mutation, Humans, Infant, Infant, Newborn, Loss of Heterozygosity, Male, Spain epidemiology, Young Adult, Adenoma diagnosis, Adenoma epidemiology, Adenoma genetics, Pituitary Neoplasms diagnosis, Pituitary Neoplasms epidemiology, Pituitary Neoplasms genetics
- Abstract
Objective: Pituitary adenomas (PA) are rare in young patients, and additional studies are needed to fully understand their pathogenesis in this population. We describe the clinical and genetic characteristics of apparently sporadic PA in a cohort of young patients., Design: Clinical and molecular analysis of 235 patients (age ≤ 30 years) with PA. Clinicians from several Spanish and Chilean hospitals provided data., Methods: Genetic screening was performed via next-generation sequencing and comparative genomic hybridization array. Clinical variables were compared among paediatric, adolescent (<19 years) and young adults' (≥19-30 years) cohorts and types of adenomas. Phenotype-genotype associations were examined., Results: Among the total cohort, mean age was 17.3 years. Local mass effect symptoms were present in 22.0%, and prolactinomas were the most frequent (44.7%). Disease-causing germline variants were identified in 22 individuals (9.3%), more exactly in 13.1 and 4.7% of the populations aged between 0-19 and 19-30 years, respectively; genetically positive patients were younger at diagnosis and had larger tumour size. Healthy family carriers were also identified., Conclusions: Variants in genes associated with syndromic forms of PAs were detected in a large cohort of apparently sporadic pituitary tumours. We have identified novel variants in well-known genes and set the possibility of incomplete disease penetrance in carriers of MEN1 alterations or a limited clinical expression of the syndrome. Despite the low penetrance observed, screening of AIP and MEN1 variants in young patients and relatives is of clinical value.
- Published
- 2021
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24. Sentinel lymph node biopsy in papillary thyroid cancer: Accuracy and application in clinical practice.
- Author
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Expósito Rodríguez A, Corta Gómez I, Domínguez Ayala M, García Carrillo M, González García AI, Gutiérrez Rodríguez MT, Oleaga Alday A, Paja Fano M, Rodríguez Iriarte ML, Ugalde Olano A, and Zabala Landa R
- Abstract
Introduction: The presence of lymph nodes metastasis in papillary thyroid cancer modifies the type of surgical resection as well as the indication of the treatment with
131 I in the postoperative period. This therapeutic approach is based on the results of the diagnostic tests, like the cervical ultrasonography. Currently other methods of diagnostic are tested as selective sentinel lymph node biopsy (SLNB). It can complement to the ultrasound results. The aim was to validate the SLNB for use in the diagnosis of lymph node metastasis by papillary thyroid cancer., Methods: Observational prospective cohort study of 55 patients who underwent papillary thyroid cancer without suspicion of lymph node involvement clinical or radiological, since February 2012 through February 2015, with a follow-up between 6 and 8 years. It was used99 Tc with intratumoral nanocoloid and a portable tube of the gamma camera for the detection of the sentinel node., Variables: age, gender, histological, analytical and preoperative and postoperative staging. The sensitivity, specificity and predictive values of technique was calculated. The validation was determined by calculating the detectability and the false negative results of the test., Results: In 53 of the 55 patients (96.36%) there was the sentinel node detection. The false negative were 4 patients (7.5%). Of the rest, after applying the SLNB, 24 (48.9%) were kept as N0, 14 (28.5%) became N1a and 11 (22.4%) were classified as N1b. The differences observed in the study were significant (P<.05). The sensitivity was 86.21%, the specificity of 100%, the PPV was 100% and the NPV of 85.71%. The diagnostic accuracy was 92.45%., Conclusions: The SLNB is a valid technique for use in patients suffering from papillary thyroid cancer with a high diagnostic accuracy., (Copyright © 2021 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.)- Published
- 2021
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25. A remarkable cause of endocrine hypertension.
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Paja-Fano M, Martínez-Martínez AL, Monzón-Mendiolea A, and Espiga-Alzola J
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- 2020
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26. [Is there life after teduglutide?]
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Martínez Martínez AL, Calles Romero LA, Etxeberría Martín E, Monasterio Jiménez O, Zabalegui Eguinoa A, Paja Fano M, Monzón Mendiolea A, Cadenas González A, Cabriada Nuno JL, and Iglesias Hernández NC
- Subjects
- Crohn Disease therapy, Fluid Therapy, Humans, Jejunostomy, Liraglutide therapeutic use, Male, Middle Aged, Parenteral Nutrition, Product Recalls and Withdrawals, Gastrointestinal Agents adverse effects, Gastrointestinal Agents therapeutic use, Intestinal Diseases drug therapy, Peptides adverse effects, Peptides therapeutic use, Short Bowel Syndrome drug therapy
- Abstract
Introduction: Intestinal failure (IF) is defined as a reduction in intestinal function below the minimum necessary for the absorption of nutrients, requiring intravenous supplementation to maintain health and/or growth. The most common cause is short bowel syndrome (SBS). Approximately 50% of patients with SBS have IF and require parenteral support. Teduglutide is a human glucagon-like peptide-2 analogue (GLP-2) approved for the treatment of patients with SBS. Clinical trial results have proven its efficacy by reducing volume and days of administration of parenteral nutrition and fluid therapy. Few publications evaluate the effects on long-term bowel function in respondent patients after teduglutide suspension. A patient with type I SBS (terminal jejunostomy) due to multiple surgeries for Crohn's disease, who was treated with liraglutide for one year and sequential teduglutide for 21 months, is described. With the former, a reduction in the need for contribution and debit by jejunostomy was observed. The GLP-2 analogue achieved a greater reduction in the hydric disbalance that allowed the suspension of the nocturnal suerotherapy, with weight gain and maintenance of nutritional parameters; situation maintained two years after its suspension.
- Published
- 2020
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27. Diagnostic and treatment delay in primary hyperparathyroidism. A pending issue.
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Paja-Fano M, Martínez-Martínez AL, and Monzón-Mendiolea A
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- Female, Humans, Hypercalcemia complications, Hyperparathyroidism, Primary etiology, Male, Middle Aged, Retrospective Studies, Delayed Diagnosis, Hyperparathyroidism, Primary diagnosis, Hyperparathyroidism, Primary therapy, Time-to-Treatment
- Abstract
Introduction: Primary hyperparathyroidism (PHPT) remains underdiagnosed among patients with hypercalcemia, potentially causing increased morbidity., Objective: To identify in surgically operated patients the presence of overlooked hypercalcemia and patients with criteria for surgery (CFS) for PHPT at least one year prior to referral to Endocrinology, and to determine whether this diagnostic delay leads to increased morbidity., Methods: An observational study was carried out in 116 consecutive patients. We evaluated electronic medical records registered at least 12 months prior to referral and divided them in four groups: hypercalcemia with CFS (group 1), hypercalcemia without CFS (group 2), normocalcemia (group 3), and cases without previous biochemical evaluation (group 4)., Results: A total of 84 patients (72.4%) had a previous measurement of serum calcium at a time interval of ≥ 12 months. Sixty-six (56.9%) had hypercalcemia and 43 of them (37%) had ≥ 1 CFS, with an average delay of 57 months in receiving proper evaluation. Almost half of the calcemia measurements in group 1 had been made in the emergency room. Patients from group 1 were younger, and had a greater frequency of nephrolithiasis and renal impairment than patients in group 4. The serum calcium values at referral were similar in both groups and higher than the values found in patients from the other two groups., Discussion: In patients with PHPT and CFS, referral to an endocrinologist is made with an average delay of almost 5 years. The identified causes of this delay, which conditions more kidney disease, are unrecognized hypercalcemia and/or unawareness of the surgical criteria, while calcium elevations promote referral. Interventions are needed to avoid this delay in the diagnosis and resolution of PHPT., (Copyright © 2020 SEEN y SED. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2020
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28. Immunohistochemical detection of the BRAF V600E mutation in papillary thyroid carcinoma. Evaluation against real-time polymerase chain reaction.
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Paja Fano M, Ugalde Olano A, Fuertes Thomas E, and Oleaga Alday A
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- Adolescent, Adult, Aged, Aged, 80 and over, Antibodies, Monoclonal immunology, Biopsy, Large-Core Needle, Child, Female, Humans, Immunohistochemistry economics, Male, Middle Aged, Predictive Value of Tests, Prospective Studies, Proto-Oncogene Proteins B-raf immunology, Reproducibility of Results, Sensitivity and Specificity, Sequence Analysis, DNA, Young Adult, Biomarkers, Tumor genetics, Immunohistochemistry methods, Mutation, Missense, Neoplasm Proteins genetics, Point Mutation, Proto-Oncogene Proteins B-raf genetics, Real-Time Polymerase Chain Reaction economics, Thyroid Cancer, Papillary genetics
- Abstract
Introduction: The BRAF V600E mutation is the most common genetic change in papillary thyroid carcinoma and is associated with a poorer clinical course. Usual methods for its study (DNA sequencing or molecular test based on PCR) are expensive and time-consuming. Recently, immunohistochemistry (IHC) for BRAF mutation has been introduced., Objective: To compare the results of IHC and real time PCR (RT-PCR) in the detection of BRAF V600E mutation in papillary thyroid carcinoma. Analysis of clinical and pathological differences depending on RT-PCR results is included., Methods: A prospective study was performed in 82 consecutive samples, 54 of them taken through a core needle biopsy. IHC was performed on tissue fixed for 24hours with 10% neutral formalin using the anti-BRAF V600E (VE-1) mouse monoclonal primary antibody and was rated as positive or negative. DNA was extracted from formalin-fixed, paraffin-embedded tissues by manual microdissection, and BRAF mutation was detected by RT-PCR using the Cobas® 4800 BRAF V600 mutation test (Roche)., Results: Both techniques were concordant in 81 cases, and BRAF was positive in 49. Discordance appeared in a follicular variant showing positive IHC and negative RT-PCR, attributed to histological heterogeneity. Cost of materials for IHC was less than half of the cost for RT-PCR., Conclusions: IHC appears to be a reliable, economical and easily available alternative to molecular biology techniques for routine detection of the BRAF V600E mutation in papillary thyroid carcinoma patients, provided optimal fixation conditions are used. It may be a useful technique in hospitals with no access to molecular biology techniques., (Copyright © 2017 SEEN. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2017
- Full Text
- View/download PDF
29. Paraganglioma and cyanotic congenital heart disease: The role of tisular hipoxia.
- Author
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Oleaga-Alday A, Goñi-Goicoechea F, Calles-Romero L, Pérez de Ciriza-Cordeu M, and Paja-Fano M
- Subjects
- Adult, Biomarkers, Tumor analysis, Cyanosis complications, Disease Susceptibility, Eisenmenger Complex metabolism, Female, Gene Expression Regulation, Heart Defects, Congenital metabolism, Humans, Hypertension etiology, Hypoxia genetics, Multimodal Imaging, Normetanephrine urine, Paraganglioma diagnostic imaging, Paraganglioma metabolism, Paraganglioma surgery, Phenotype, Retroperitoneal Neoplasms diagnostic imaging, Retroperitoneal Neoplasms metabolism, Retroperitoneal Neoplasms surgery, Signal Transduction, Eisenmenger Complex complications, Heart Defects, Congenital complications, Hypoxia complications, Paraganglioma etiology, Retroperitoneal Neoplasms etiology
- Published
- 2015
- Full Text
- View/download PDF
30. Senile primary aldosteronism associated with adrenal carcinoma.
- Author
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Rodríguez Iturriaga J, Gutiérrez Macías A, Paja Fano M, and Lizarraga Zufiaur A
- Published
- 2014
- Full Text
- View/download PDF
31. [Isolated adult thyrotropin deficiency. An unusual finding of unknown etiology].
- Author
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Paja Fano M, Egaña Zunzunegui N, Lizarraga Zufiaurre A, and Elorza Olabegoya R
- Subjects
- Adenoma complications, Adenoma diagnosis, Adult, Age of Onset, Asthenia etiology, Congenital Hypothyroidism, Humans, Hyperlipoproteinemia Type II etiology, Hypothyroidism diagnosis, Hypothyroidism epidemiology, Incidental Findings, Magnetic Resonance Imaging, Male, Middle Aged, Pituitary Apoplexy complications, Pituitary Neoplasms complications, Thyroxine deficiency, Thyroxine therapeutic use, Hypothyroidism etiology, Pituitary Neoplasms diagnosis, Thyrotropin deficiency
- Published
- 2010
- Full Text
- View/download PDF
32. [Megacolon and pheochromocytoma. An exceptional association].
- Author
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Moreno Rengel C, Paja Fano M, Martín Martín E, and Elorza Olabegoya JR
- Subjects
- Aged, Female, Humans, Adrenal Gland Neoplasms complications, Megacolon complications, Pheochromocytoma complications
- Published
- 2002
33. [Gynecomastia and hyperthyroidism: hormonal study of one case].
- Author
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Paja Fano M, Oleaga Alday A, Astigarraga Aguirre B, and Elorza Olabegoya R
- Subjects
- Adult, Gynecomastia blood, Hormones blood, Humans, Hyperthyroidism blood, Male, Gynecomastia etiology, Hyperthyroidism complications
- Published
- 2001
34. [Type 1 multiple endocrine neoplasia associated to thymic carcinoid. Clinical and genetic study of one case].
- Author
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Paja Fano M, Goñi Goicoechea F, Yoldi Arrieta A, and Ramón Elorza Olabegoya J
- Subjects
- Adult, Humans, Male, Carcinoid Tumor diagnosis, Multiple Endocrine Neoplasia Type 1 diagnosis, Thymus Neoplasms diagnosis
- Published
- 2001
- Full Text
- View/download PDF
35. [Hyperkalemic myopathy and Addison's disease].
- Author
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Paja Fano M, Astigarraga Aguirre B, Yoldi Arrieta A, and Elorza Olabegoya JR
- Subjects
- Addison Disease diagnosis, Aged, Aged, 80 and over, Humans, Male, Muscular Diseases diagnosis, Syndrome, Addison Disease complications, Hyperkalemia complications, Muscular Diseases complications
- Published
- 1998
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