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Context There are no data on mortality of acromegaly diagnosed in older individuals. Objective This work aimed to compare clinical characteristics, growth hormone–related comorbidities, therapeutic approaches, and mortality rate of patients diagnosed before or after 2010 and to assess overall mortality rate compared with the general Spanish population. Methods A retrospective evaluation was conducted among Spanish tertiary care centers of 118 patients diagnosed with acromegaly at age 65 or older. Kaplan-Meier curves were constructed to trace survival, and Cox proportional hazard models were used to assess the risk factors associated with mortality. We also compared mortality with that of the Spanish population by using age- and sex-adjusted standardized mortality ratios (SMRs). Results No differences were found in first-line treatment or biochemical control, between both periods except for faster biochemical control after 2010. Twenty-nine (24.6%) patients died, without differences between groups, and had a median of follow-up 8.6 years (103, [72.3] months). Overall SMR was 1.02 (95% CI, 0.57-1.54), (0.60; 95% CI, 0.35-1.06) for men and (1.80; 95% CI, 1.07-2.94) for women. The most common cause of death was cardiovascular disease (CVD). Conclusion The mortality in patients with acromegaly diagnosed in older individuals was no different between both periods, and there was no overall SMR difference compared with the general Spanish population. However, the SMR was higher in women. As CVD is the leading cause of mortality, it seems advisable to initiate an intense CVD protective treatment as soon as acromegaly is diagnosed, particularly in women, in addition to tight acromegaly control to prevent excess mortality.

Introduction: It is estimated that 30-40% of patients with apparently sporadic pheochromocytomas (PHEOs) have an inherited predisposition syndrome. The aim of our study was to develop a predictive model of hereditary pheochromocytoma (PHEO) based on the clinical, hormonal, and radiological features present at the diagnosis of patients with PHEOs. Methods: A retrospective multicenter cohort study of patients with PHEOs with available genetic study from 18 tertiary hospitals. Clinical, biochemical, and radiological features were used to build a multivariate logistic regression model. The estimation of all possible equations was used to select the model with the best diagnostic accuracy (lower Akaike index (AIC)). Results: A total of 245 patients were included: 169 (69.0%) patients with sporadic PHEOs and 76 (31%) with hereditary PHEOs. The parsimonious predictive model with the highest diagnostic accuracy for the prediction of hereditary PHEO combined the variables age, non-cardiovascular disease, urinary norepinephrine levels and tumor size. The area under the ROC curve of this model was of 0.800 (0.705-0.887). Based on the predictive model, the probability of hereditary PHEO in patients older than 60 years with cardiovascular disease, high levels of urinary norepinephrine and unilateral PHEOs >60 mm was

To develop a predictive model of hypertension resolution after adrenalectomy in patients with primary aldosteronism (PA), based on their presurgical characteristics.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.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, BMI30 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.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.

The objective of our study was to determine the prevalence of glycemic disorders (diabetes mellitus and prediabetes) in patients with pheochromocytomas and sympathetic paragangliomas (PPGLs) and identify risk factors for their development and the likelihood of their resolution after surgery. A multicentric retrospective study of patients with PPGLs submitted to surgery between 2000 and 2021 in 17 Spanish hospitals was performed. Diabetes-specific data were collected at diagnosis, in the immediate- and long-term postsurgical follow-up. A total of 229 patients with PPGLs were included (218 with pheochromocytomas and 11 with sympathetic paragangliomas). Before surgery, glycemic disorders were diagnosed in 35.4% of the patients (n = 81): 54 with diabetes and 27 with prediabetes. The variables independently associated with a higher risk of glycemic disorders were sporadic PPGL (odds ratio (OR) = 3.26 (1.14–9.36)) and hypertension (OR = 3.14 (1.09–9.01)). A significant decrease in fasting plasma glucose and HbA1c levels was observed after surgery, in the short-term and long-term follow-up (P < 0.001). After a median follow-up of 48.5 months (range 3.3–168.9), after surgery, 52% of diabetic and 68% of prediabetic patients experienced a complete resolution. Lower body mass index (BMI) (P = 0.001), lower glucose levels (P = 0.047) and shorter duration of diabetes prior to surgery (P = 0.021) were associated with a higher probability of diabetes resolution. In conclusion, glycemic disorders in patients with PPGLs are present in more than a third of them at diagnosis. Sporadic PPGLs and hypertension are risk factors for their development. More than 50% of cases experience a complete resolution of the glycemic disorder after resection of the PPGLs.

Resumen Introduccion Las herramientas para analizar la casuistica en consultas externas son escasas e insatisfactorias. El objetivo de este trabajo de la Sociedad Castellano Manchega de Endocrinologia, Nutricion y Diabetes (SCAMEND) fue el desarrollo de una herramienta que permita analizar la casuistica de las consultas externas de Endocrinologia y Nutricion teniendo en cuenta la complejidad de la patologia atendida. Material y metodos Se definio el Indice SCAMEND de Complejidad en Consultas Externas de Endocrinologia y Nutricion (ISCCE-EyN) mediante metodo Delphi con dos rondas entre especialistas en Endocrinologia y Nutricion, comparando la complejidad de cada patologia con la de una revision de hipotiroidismo primario. Resultados Las primeras visitas fueron consideradas mas complejas que las visitas sucesivas. La patologia tiroidea no neoplasica y el sobrepeso/obesidad sin complicaciones fueron consideradas las patologias menos complejas, mientras que las metabolopatias, los sindromes de neoplasias endocrinas multiples y el carcinoma suprarrenal fueron consideradas las mas complejas. El grado de consenso fue elevado en la mayoria de las patologias analizadas. Conclusiones Presentamos una herramienta que permite analizar la casuistica de las consultas externas de Endocrinologia y Nutricion teniendo en cuenta la complejidad inherente a la patologia del paciente atendido. Esta herramienta puede servir para realizar comparaciones entre centros, para asignar mejores recursos dentro de un determinado servicio o para la autoevaluacion.

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).Retrospective multicentric study of patients with PA on follow-up in twelve Spanish centers between 2018 and 2020.268 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).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.

Background: Craniopharyngioma (CP) is a rare tumor in the elderly whose clinical features and prognosis are not well known in this population. Aim: To evaluate the clinicopathological features and therapeutic outcomes of CP diagnosed in the elderly. Patients and Methods: This was a retrospective, multicenter, national study of CP patients diagnosed over the age of 65 years and surgically treated. Results: From a total of 384 adult CP patients, we selected 53 (13.8%) patients (27 women [50.9%], mean age 72.3 ± 5.1 years [range 65–83 years]) diagnosed after the age of 65 years. The most common clinical symptoms were visual field defects (71.2%) followed by headache (45.3%). The maximum tumor diameter was 2.9 ± 1.1 cm. In most patients, the tumor was suprasellar (96.2%) and mixed (solid-cystic) (58.5%). The surgical approach most commonly used was transcranial surgery (52.8%), and more than half of the patients (54.7%) underwent subtotal resection (STR). Adamantinomatous CP and papillary CP were present in 51 and 45.1%, respectively, with mixed forms in the remaining. Surgery was accompanied by an improvement in visual field defects and in headaches; however, pituitary hormonal hypofunction increased, mainly at the expense of an increase in the prevalence of diabetes insipidus (DI) (from 3.9 to 69.2%). Near-total resection (NTR) was associated with a higher prevalence of DI compared with subtotal resection (87.5 vs. 53.6%, p = 0.008). Patients were followed for 46.7 ± 40.8 months. The mortality rate was 39.6% with a median survival time of 88 (95% CI: 57–118) months. DI at last visit was associated with a lower survival. Conclusion: CP diagnosed in the elderly shows a similar distribution by sex and histologic forms than that diagnosed at younger ages. At presentation, visual field alterations and headaches are the main clinical symptoms which improve substantially with surgery. However, surgery, mainly NTR, is accompanied by worsening of pituitary function, especially DI, which seems to be a predictor of mortality in this population.

Craniopharyngiomas (CPs) are histologically benign tumors that are associated with high levels of morbidity. Two clinicopathological variants - adamantinomatous (ACP) and papillary (PCP) - have been described. They differ in their molecular features, whereby activating mutations in BRAF (V600E) and CTNNB1 genes characterize PCP and ACP, respectively. Recently, both variants have been shown to express elevated PD-L1 protein expression, but ACP also exhibited tumor cell-intrinsic PD-1 expression. In this study we analyze these molecular alterations in 52 cases with a long follow-up and examine their associations with immunohistochemical and clinical characteristics. ACPs comprise 73.1% of cases, while 21.2% are PCPs. Aberrant nuclear immunoreactivity for β-catenin was observed in all ACPs. BRAF p.V600E mutations were observed in 90.9% of PCPs. Only one ACP case featured both alterations. Both types of CP exhibited strong nuclear staining for p63 with diffuse and basal distribution. ACP and PCP consistently expressed PD-L1, most in a substantial percentage of tumor cells, with a distinctive spatial distribution of expression in each subtype; only ACP demonstrated PD-1 expression. There was no evidence of differences in clinical prognosis between ACPs and PCPs. The identification of hallmark molecular signatures in the two CP variants is useful for sub-categorization in routine histopathology reporting. It is also pertinent to personalized therapy and for the development of improved non-invasive therapeutic strategies in this disease.

© 2019 The authors., Primary hyperparathyroidism is the most frequent manifestation of multiple endocrine neoplasia type 1 (MEN1) syndrome. Bone and renal complications are common. Surgery is the treatment of choice, but the best timing for surgery is controversial and predictors of persistence and recurrence are not well known. Our study describes the clinical characteristics and the surgical outcomes, after surgery and in the long term, of the patients with MEN1 and primary hyperparathyroidism included in the Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytomas and Paragangliomas (REGMEN). Eighty-nine patients (49 men and 40 women, 34.2 ± 13 years old) were included. Sixty-four out of the 89 underwent surgery: a total parathyroidectomy was done in 13 patients, a subtotal parathyroidectomy in 34 and a less than subtotal parathyroidectomy in 15. Remission rates were higher after a total or a subtotal parathyroidectomy than after a less than subtotal (3/4 and 20/22 vs 7/12, P, The Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytmas and Paragangliomas is supported by IPSEN Pharmaceutical. This work did not receive any other specific grant from any funding agency in the public, commercial or not-for-profit sector.

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 Senora del Prado-Talavera de la Reina, and Complejo Hospitalario la Mancha Centro-Alcazar 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 two patients only. Surprisingly, three patients with normal postoperative neurohypophyseal function later developed permanent diabetes insipidus. New hormone deficiencies occurred in seven patients. Seventeen patients received ketoconazole before surgery (five 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.

Resumen Objetivo El tratamiento de la enfermedad de Cushing plantea interesantes dilemas en la practica clinica. Nuestro objetivo es analizar los resultados y complicaciones de los distintos tratamientos de esta enfermedad. Material y metodos Se recopilaron datos mediante revision de las historias clinicas de 22 pacientes (86,4% mujeres), mayores de 18 anos, diagnosticados de enfermedad de Cushing entre los anos 2000 y 2012, con seguimiento en el Complejo Hospitalario Universitario de Albacete, Hospital Virgen de la Salud (Toledo), Hospital General de Ciudad Real, Hospital Virgen de la Luz (Cuenca), Hospital Nuestra Senora del Prado (Talavera de la Reina) y en el Complejo Hospitalario la Mancha Centro (Alcazar de San Juan). Resultados En todos los casos el tratamiento de eleccion fue el quirurgico. Se consiguio la curacion bioquimica en el 72,7%. Nueve pacientes desarrollaron diabetes insipida en el perioperatorio, de las que 2 resultaron permanentes. Tres pacientes desarrollaron diabetes insipida permanente, que no habia sido identificada en el perioperatorio. Aparecieron nuevos deficits hormonales adenohipofisarios posquirurgicos en 7 pacientes. Diecisiete pacientes recibieron tratamiento medico prequirurgico con ketoconazol (5 tambien postoperatorio), consiguiendose la normalizacion del cortisol libre urinario en el 70%. Tres pacientes se irradiaron por persistencia de hipersecrecion y, tras una mediana de seguimiento de 85,8 meses, se ha conseguido la curacion en todos; ninguno de ellos ha desarrollado tumores, alteraciones vasculares ni otra complicacion. Conclusiones Nuestro estudio presenta los resultados del manejo de la enfermedad de Cushing en centros que no son de referencia nacional para esta enfermedad, lo que posiblemente refleja la practica clinica habitual en esta enfermedad.

Resumen Objetivos Valorar el grado de control glucemico y del resto de factores de riesgo cardiovascular en una cohorte de pacientes diabeticos seguidos en consultas externas durante 4 anos. Conocer el patron de tratamiento utilizado y su evolucion. Material y metodos Estudio de 424 pacientes diabeticos tanto tipo 1 (DM1) como tipo 2 (DM2), seleccionados aleatoriamente en 2004 y seguidos hasta 2008. La cohorte final fue de 343 pacientes. Se recogieron datos epidemiologicos, factores de riesgo cardiovascular, complicaciones cronicas, datos de control glucemico, lipidico y tensional, asi como de tratamiento al inicio y a los 4 anos. Resultados Tras los 4 anos, el porcentaje de pacientes que conseguian una hemoglobina glicada inferior a 7% se mantuvo estable (DM1 18,5 en 2004 frente a 21,7% en 2008 y DM2 26,6 frente a 26,5%). En ambos tipos de diabetes se incremento de forma significativa el grado de consecucion de objetivos de control lipidico y tensional. Para conseguir estos resultados las pautas de tratamiento se complicaron de manera significativa. Conclusiones La estabilizacion del control glucemico objetivada tras 4 anos de seguimiento es un resultado positivo, considerando el largo tiempo de evolucion de la enfermedad, el deterioro progresivo de la funcion pancreatica y la complejidad de esta cohorte. Gracias a la optimizacion de los tratamientos utilizados, han mejorado de forma significativa el control tensional y lipidico del grupo estudiado.

Resumen Antecedentes y objetivo El cancer diferenciado de tiroides (CDT) es el tumor endocrino mas frecuente, con buen pronostico y supervivencias superiores al 85%. El objetivo de nuestro trabajo es conocer la supervivencia actual de nuestra serie de CDT y analizar los factores relacionados. Pacientes y metodos Realizamos un estudio retrospectivo de una cohorte de pacientes con CDT (n: 308), 93,5% eran papilares, el 78,2% eran mujeres con una edad media al diagnostico de 45,4 ± 15,8 anos y un tiempo de seguimiento de 8,9 ± 6,8 anos. Han sido tratados y seguidos de forma homogenea en nuestro centro hospitalario. Para cada paciente se ha recogido edad al diagnostico, sexo, histologia, estadio TNM, tratamientos empleados y fecha y causa de la muerte. La probabilidad de supervivencia fue calculada por el metodo de Kaplan Meier. Para analizar los factores relacionados con la supervivencia se realizo un analisis univariante (Log Rank test) y multivariante (Riesgos proporcionales de Cox). Resultados Se produjeron 26 casos de muerte, de ellos 15 pacientes (4,9%) murieron como consecuencia del CDT. La probabilidad de supervivencia acumulada del grupo total fue del 92,7%. En el analisis multivariante las variables asociadas de forma independiente con mortalidad por CDT fueron: metastasis a distancia, tipo histologico folicular, edad al diagnostico mas de 60 anos y afectacion extratiroidea. Discusion La probabilidad de supervivencia de nuestra serie es equiparable a la de la literatura. El conocimiento de los factores relacionados con peor supervivencia en el medio que trabajamos, es importante para establecer estrategias mas activas de tratamiento en pacientes de alto riesgo.

Resumen La Endocrinologia y Nutricion es la rama de la Medicina que se ocupa del estudio de la fisiologia y patologia del sistema endocrino, del metabolismo de las sustancias nutritivas, de la nutricion natural y artificial, de la promocion de la salud mediante la prevencion de las enfermedades relacionadas con la dieta y de las correspondientes tecnicas diagnosticas, terapeuticas y preventivas utilizadas en estas areas del conocimiento medico. El desarrollo de estas actividades requiere la definicion y la gestion de una serie de recursos complejos, tanto humanos como materiales, asi como una correcta planificacion del trabajo asistencial. Por lo tanto, resulta imprescindible conocer la Cartera de Servicios de un Servicio/Seccion o Departamento de Endocrinologia y Nutricion, ya que es una herramienta necesaria, valida y util para optimizar los recursos disponibles, aumentar la eficacia y la rentabilidad de la actuacion medica y mejorar la calidad de la asistencia.

Endocrinology and Clinical Nutrition are branches of medicine that deal with the study of the physiology of body glands and hormones and their disorders,the intermediate metabolism of nutrients, enteral and parenteral nutrition, the promotion of health through the prevention of diet-related diseases, and the appropriate use of the diagnostic, therapeutic, and preventive tools related to these disciplines. The development of Endocrinology and Clinical Nutrition support services requires accurate definition and management of a number of complex resources, both human and material, as well as adequate planning of the care provided. It is therefore essential to know the services portfolio of an ideal Department of Endocrinology and Clinical Nutrition because this is a useful, valid and necessary tool for optimizing available resources, increasing efficiency, and improving the quality of care.

Introduccion Actualmente, el hiperparatiroidismo primario (HPP) se considera una enfermedad escasamente sintomatica y poco progresiva. El HPP normocalcemico (HPPN) es una entidad reconocida e identificada esencialmente por complicaciones asociadas al HPP (osteoporosis y urolitiasis). Pacientes y metodo Estudio de 56 pacientes con HPP no operados o con seguimiento minimo de 1 ano previo a la paratiroidectomia. Se subdividio a los pacientes en HPP hipercalcemico (HPPH) (27 pacientes) y HPPN (29 pacientes). Se pretendio analizar las comorbilidades hipertension arterial [HTA] y nefrolitiasis) en el grupo total y en cada subgrupo, valorar la evolucion anual de calcemia, fosfatemia, paratirina PTH) y fosfatasa alcalina sericas, calciuria, filtrado glomerular y densidad mineral osea lumbar durante un periodo de 1 a 5 anos y hallar posibles diferencias entre HPPH y HPPN para estas variables. Resultados El 55,4% de los pacientes presentaban HTA y se registraron episodios de nefrolitiasis en el 37,5% de los casos. No se apreciaron cambios a lo largo del tiempo en los parametros bioquimicos y densitometricos ni en el grupo total ni por subgrupos. Excepto en los valores de calcio, fosforo y PTH sericos no se hallaron diferencias entre HPPH y HPPN. Conclusiones El HPP parece una enfermedad poco progresiva si bien no se incluyo en este estudio a los pacientes remitidos precozmente a cirugia con seguimiento previo escaso que podrian corresponder a casos mas agresivos. El HPPN es una entidad asociada a complicaciones similares a las del HPPH por lo que pensamos deberia someterse a los mismos criterios de derivacion quirurgica.

Objetivo Valorar el grado de control glucemico, lipidico y de presion arterial (PA), asi como el porcentaje de antiagregacion plaquetaria, en pacientes diabeticos asistidos en una consulta de atencion especializada. Pacientes y metodo Estudio transversal de una cohorte de 424 pacientes vistos en 2 meses de 2004. Se recogieron datos sobre glucohemoglobina (HbA 1c ), perfil lipidico, PA, antecedentes de microangiopatia y macroangiopatia y tratamientos utilizados. En 296 pacientes se analizaron retrospectivamente los resultados del ano 2000. Resultados En 2004, la HbA 1c media fue de 7,8 ± 1,2% (en el 25% de los pacientes era 1c , 8,5% ± 1,3%; cLDL, 128,2 ± 28,5 mg/dl; PA sistolica y diastolica media, 140,2 ± 17,9 y 77,9 ± 12,1 mmHg). El 38% de los pacientes (el 84% en prevencion secundaria y el 26% en prevencion primaria) utilizaban antiagregacion plaquetaria, frente a un 17% en el ano 2000. Conclusiones Aunque se ha producido una importante mejora en el control de los factores de riesgo vascular, gracias al abordaje integral de los pacientes, todavia el grado de control de nuestros diabeticos es insuficiente. Hemos evidenciado una mejora en el uso de antiagregacion plaquetaria, aunque su utilizacion es poco frecuente, sobre todo en prevencion primaria.