Epidemiological, clinical, and laboratory aspects in a case series of canine hyperadrenocorticism: 115 cases (2010-2014) / Aspectos epidemiológicos, clínicos e laboratoriais em uma série de cães com hiperadrenocorticismo: 115 casos (2010-2014)

Diseases' clinical-epidemiological characterization assists in directing the diagnosis. The objective of this study was to describe epidemiological, clinical and laboratorial aspects of a case series of canine hyperadrenocorticism (HAC). One-hundred fifteen records of dogs diagnosed by the low dose dexamethasone suppression test and/or ACTH stimulation test were evaluated. Of the cases, 81.3% were HAC ACTH-dependent and 18.7% HAC ACTH-independent. Females were more affected, representing 69.3% of the cases. The mean age was 10.3±2.5 years and 64.9% were gonadectomized. Most of the patients were small size dogs, weighting less than 10kg (73.9%). The most frequent breeds were: Poodle (27%), Dachshund (17.4%), and Yorkshire Terrier (10.4%). The most frequent clinical manifestations were polyphagia (86%), polydipsia (82.6%), polyuria (80%), abdominal enlargement (82.6%), thin skin (79.1%), muscular weakness (78.3%) and panting (74.8%). However, eventually unusual HAC-associated signs would be present in some dogs in a more important way compared with the classic disease´s clinical signs. The CBC showed neutrophilia (66%), eosinopenia (58.3%) and lymphopenia (42.6%) as main hematological abnormalities. The most common findings in serum biochemistry were increased alkaline phosphatase activity (81.74%), increased ALT activity (62.6%), hypercholesterolemia (66%) and hypertriglyceridemia (54.7%). Urinalysis revealed hyposthenuria in 14.9% and isostenuria in 13.5%; besides proteinuria in 50% of the cases. Abdominal ultrasound showed bilateral adrenal hyperplasia (92.2%) with adrenal asymmetry in 20.8% of the cases, in addition to hepatomegaly (80.9%), biliary sludge (67.8%) and hepatic hyperechogenicity (47.8%). It was concluded that small size gonadectomized female dogs, mainly Poodles and Dachshunds, presented higher frequency in the population studied, and that the main changes observed in clinical and complementary tests were polyphagia, polyuria, polydipsia, abdominal enlargement, adrenal hyperplasia, increased phosphatase alkaline and hyperlipidemia. These results corroborated to a better disease characterization at Brazil. This work concluded that the population studied resembles the profile describe in European and North American epidemiologic studies, and that the HAC dog´s clinical picture looks similar worldwide.(AU)

A caracterização clínica-epidemiológica de doenças auxilia no direcionamento do diagnóstico. O objetivo deste trabalho foi descrever aspectos epidemiológicos, clínicos e laboratoriais de uma série de casos de hiperadrenocorticismo (HAC) canino. Foram avaliados 115 prontuários de cães diagnosticados pelo teste de supressão por baixa dose de dexametasona e/ou teste de estimulação com ACTH. Os casos de HAC ACTH-dependentes representaram 81,3% da população, e 18,7% foram ACTH-independentes. As fêmeas foram mais acometidas, representando 69,3% dos casos. A média de idade foi 10,3 ± 2,5 anos e 64,9% eram gonadectomizados. A maioria dos cães foi de porte pequeno, de até 10 kg (73,9%). As raças mais frequentes foram Poodle (27%), Dachshund (17,4%) e Yorkshire (10,4%). As manifestações clínicas mais relatadas foram polifagia (86%), polidipsia (82,6%), poliúria (80%), abdome pendular (82,6%), atrofia cutânea (79,1%), fraqueza muscular (78,3%) e dispneia (74,8%). Entretanto, eventualmente sinais clínicos pouco associados ao HAC se manifestaram de forma mais importante que os sinais clássicos da doença. O hemograma revelou neutrofilia (66%), eosinopenia (58,3%) e linfopenia (42,6%) como principais alterações hematológicas. Na bioquímica sérica foi observado aumento de fosfatase alcalina (81,74% dos casos), aumento da atividade da ALT (62,6%), hipercolesterolemia (66%) e hipertrigliceridemia (54,7%). A urinálise revelou hipostenúria em 14,9% e isostenúria em 13,5%; além de proteinúria em 50% dos casos. A ecografia abdominal evidenciou hiperplasia bilateral de adrenal (92,2%) com assimetria de adrenais em 20,8% dos casos, além de hepatomegalia (80,9%), lama biliar (67,8%) e hiperecogenicidade hepática (47,8%). Concluiu-se que fêmeas castradas de pequeno porte, principalmente das raças Poodles e Dachshunds, apresentaram maior frequência na população estudada e que as principais alterações observadas clínicas e nos exames complementares foram polifagia, poliúria, polidipsia, aumento abdominal, hiperplasia da adrenal, aumento de fosfatase alcalina e hiperlipidemia. Estes resultados corroboram para melhor caracterização da doença no Brasil. Este estudo concluiu que a população estudada se assemelha ao perfil populacional de cães com HAC descrito em estudos Europeus e Norte Americanos de forma que o perfil dos casos ao redor do globo parece similar.(AU)

Calidad de vida en pacientes operados de hipercortisolismo endógeno / Quality of life in patients operated for endogenous hypercortisolism

RESUMEN Introducción: El hipercortisolismo endógeno provoca cambios en diferentes áreas de la vida en el sentido percibido de bienestar, lo que afecta la calidad de vida; esta debe tenerse en cuenta en la atención integral al paciente. Objetivos: Describir la calidad de vida percibida en los pacientes con hipercortisolismo endógeno e identificar si existe relación entre calidad de vida y algunas características clínicas y hormonales. Métodos: Estudio transversal, 60 pacientes: grupo I (con hipercortisolismo endógeno operados con menos de cinco años) y grupo II (personas con sospecha clínica de hipercortisolismo endógeno), edad entre 20 y 60 años. Se aplicaron cuestionarios de calidad de vida relacionados con el hipercortisolismo endógeno: CushingQoL y Tuebingen CD-25 y planilla de recolección de datos generales, clínicos y hormonales. Se determinó mediana (variables cuantitativas) y frecuencia en las cualitativas. La asociación del puntaje de los cuestionarios y variables cuantitativas, mediante el coeficiente de correlación de Spearman. Se compararon los valores promedio del puntaje entre categorías de las variables cualitativas (clínicas y hormonales categorizadas), mediante la prueba U-Mann Whitney; se consideró diferencia estadística significativa con p ≤ 0,05. Resultados: La mediana del puntaje de los dominios psicoemocional, sexualidad, área social, área corporal, y la suma de todos los dominios fueron mayores en el grupo I. La sexualidad (4,5), el área corporal (6,0) y la suma de todos los dominios (36,5) del I vs. 1,0, 4,0 y 28,0 respectivamente (grupo II), del Tuebingen CD-25 mostraron diferencias estadísticamente significativas, demostrando peor calidad de vida en el grupo I. Con el CushingQoL la mediana del puntaje del grupo I fue menor que la del II (54,1 vs. 74,0), con diferencias estadísticamente significativas (p = 0,00), lo que evidencia peor calidad de vida para el grupo I. Conclusiones: El hipercortisolismo endógeno incide negativamente en la calidad de vida percibida en el grupo de pacientes operados, especialmente en las áreas social, corporal, cognitiva, sexual, y de la conducta alimentaria(AU)

ABSTRACT Introduction: Endogenous hypercortisolism causes changes in different areas of life and the perceived sense of well-being, which affects the quality of life. This should be taken into account in the patient comprehensive care. Objectives: To describe the quality of life perceived in patients with endogenous hypercortisolism and to identify if there is a relationship between quality of life and some clinical and hormonal characteristics. Methods: Cross-sectional study, 60 patients: group I (with endogenous hypercortisolism operated less than five years) and group II (people with clinical suspicion of endogenous hypercortisolism), age between 20 and 60 years. Quality of life questionnaires related to endogenous hypercortisolism were applied: CushingQoL and Tuebingen CD-25 and general, clinical and hormonal data collection form. Median (quantitative variables) and frequency in the qualitative ones were determined. The association of the score of questionnaires and quantitative variables were obtained by means of the Spearman correlation coefficient. The average score values were compared between categories of qualitative variables (clinical and hormonal categorized), using the U-Mann Whitney test; significant statistical difference was considered with p ≤ 0.05. Results: The median score of the psycho-emotional domains, sexuality, social area, body area, and the sum of all domains were higher in group I. Sexuality (4.5), body area (6.0) and the sum of all domains (36.5) of group I vs. 1.0, 4.0 and 28.0 respectively (group II), of the Tuebingen CD-25 showed statistically significant differences, which is worse quality of life in group I. Using the CushingQoL, the median score of group I showed to be lower than in group II (54.1 vs. 74.0) there are statistically significant differences (p = 0.00), which shows worse quality of life in group I. Conclusions: Endogenous hypercortisolism negatively affects the quality of life perceived in the group of operated patients, especially in the social, bodily, cognitive, sexual areas, and eating behaviour(AU)

Carcinoma adrenal de rápida evolución / Rapidly evolving adrenal carcinoma

Introducción: el carcinoma suprarrenal primario es un tumor poco frecuente, altamente agresivo, de crecimiento rápido, con mayor incidencia entre los 40 y 60 años de edad. Los carcinomas funcionantes representan hasta un 79 por ciento de los tumores corticales, más frecuentes en el sexo femenino, y de estos el 50 por ciento se manifiestan clínicamente como un síndrome de Cushing. La extensión del tumor a estructuras vecinas es común y empeora el pronóstico. La supervivencia media es de 2 años desde el diagnóstico, en particular, cuando existen metástasis en hígado y pulmón. Objetivo: describir las características clínicas, los procederes diagnósticos y terapéuticos de una paciente con carcinoma adrenal de rápida evolución. Presentación del caso: paciente LRS, femenina, blanca, de 49 años de edad, con antecedentes de hipertensión arterial y diabetes mellitus tipo 2, que asiste a consulta por descontrol metabólico y de la hipertensión arterial. Al examen físico, se constatan signos sugestivos de hipercortisolismo, sintomatología que a los 2 meses se acentuó notablemente. Se realizaron estudios basales, dinámicos, imagenológicos y anatomopatológicos, que corroboraron el diagnóstico presuntivo. Se realiza adrenalectomía izquierda con adenectomía regional, y se confirma por anatomía patológica el diagnóstico de carcinoma suprarrenal izquierdo. A los pocos meses de la intervención la paciente fallece con metástasis óseas en columna vertebral. Conclusiones: el reconocimiento temprano de los síntomas y signos de hiperfunción adrenal es muy importante para el diagnóstico y tratamiento oportuno del carcinoma adrenal(AU)

Introduction: primary suprarrenal carcinoma is a highly aggressive rare carcinoma of rapid growth, with greater incidence in 40-60 years age group. The functioning carcinomas represent up to 79 percent of cortical tumors that are more frequent in females, and 50 percent of them clinically manifests as Cushing syndrome. The extension of the tumor to neighboring structures is common and worsens the prognosis. Mean survival rate is 2 years from the time of diagnosis, particularly when there are liver and lung metastases. Objective: to describe the clinical characteristics, the diagnostic and therapeutic procedures in a female patient with rapidly evolving adrenal carcinoma. Case presentation: a patient LRS, female, Caucasian, 49 years-old, with a history of blood hypertension and type 2 diabetes mellitus, who went to the hospital because of lack of metabolic control and blood hypertension. On physical examination, there were observed some signs suggestive of hypercortisolism, symptomatology that became notably acute two months later. Basal, dynamic, imaging and anatomopathological studies were made to corroborate the presumptive diagnosis. Left adrenalectomy with regional adenectomy was performed, and the diagnosis of left suprarenal carcinoma was confirmed through pathological anatomy. Few months later, the patient died from osseous metastasis in her spinal cord. Conclusions: early recognition of symptoms and signs of adrenal hyperfunction is very important for diagnosis and timely treatment of adrenal carcinoma(AU)

Síndrome de Cushing secundario a carcinoma de la glándula suprarrenal izquierda con metástasis hepáticas y trombosis tumoral masiva de la vena cava / Cushing´s syndrome due to a left adrenal cortical carcinoma with metastasis to the liver and a massive vena cava tumor thrombus

Se presenta el caso de una mujer de 47 años con cuadro clínico de siete meses de evolución caracterizado por aumento progresivo de peso, hipertensión arterial y diabetes mellitus de reciente aparición, hirsutismo facial y en tórax, alopecia frontal, alteraciones en la menstruación e hipopotasiemia. Se consideró el diagnóstico de síndrome de Cushing, por lo cual se iniciaron estudios e extensión para establecer su etiología. Durante su hospitalización presentó una evolución tórpida y falleció. En la autopsia clínica se encontró un carcinoma de la glándula suprarrenal izquierda, de 400 g, aproximadamente, con metástasis a hígado y trombosis masiva de la vena cava, lo que finalmente produjo su muerte.

A 47-year-old woman with a seven-month history of increasing weight, hypertension and recently diagnosed diabetes presented features of hirsutism, frontal baldness, amenorrhea and hypokalemia. These characteristics were considered diagnostic of Cushing´s syndrome, and studies were initialized to identify its etiology. During hospitalization, the patient presented a torpid evolution resulting in death. Clinical autopsy revealed a 400 g carcinoma in the left adrenal gland, liver metastasis and a massive vena cava tumor thrombus which was the final cause of death.

Screening for Cushing's syndrome in obese patients

Objectives: The aim of this study was to examine the frequency of Cushing's syndrome (CS) in obese patients devoid of specific clinical symptoms of Cushing's syndrome. Methods: A total of 150 obese patients (129 female, 21 male; mean age 44.41 ± 13.34 yr; mean BMI 35.76 ± 7.13) were included in the study. As a first screening step, we measured 24-h urinary free cortisol (UFC). An overnight 1-mg dexamethasone suppression test was also performed on all patients. Urinary free cortisol levels above 100 ìg/24 h were considered to be abnormal. Suppression of serum cortisol <1.8 ìg/dL after administration of 1 mg dexamethasone was the cut-off point for normal suppression. The suppression of the serum cortisol levels failed in all of the patients. Results: Measured laboratory values were as follows: ACTH, median level 28 pg/ml, interquartile range (IQR) 14-59 pg/ml; fasting glucose, 100 (91-113) mg/dL; insulin, 15.7 (7.57-24.45) mU/ml; fT4, 1.17 (1.05-1.4) ng/dL; TSH, 1.70 (0.91-2.90) mIU/L; total cholesterol, 209 (170.5-250) mg/dL; LDL-c, 136 (97.7-163) mg/dL; HDL-c, 44 (37.25-50.75) mg/dL; VLDL-c, 24 (17-36) mg/dL; triglycerides, 120.5 (86-165) mg/dL. The median UFC level of the patients was 30 ìg/24 h (IQR 16-103). High levels of UFC (>100 ìg/24 h) were recorded in 37 patients (24 percent). Cushing's syndrome was diagnosed in 14 of the 150 patients (9.33 percent). Etiologic reasons for Cushing's syndrome were pituitary microadenoma (9 patients), adrenocortical adenoma (3 patients), and adrenocortical carcinoma (1 patient). Conclusion: A significant proportion (9.33 percent) of patients with simple obesity were found to have Cushing's syndrome. These findings argue that obese patients should be routinely screened for Cushing's syndrome.

Experiencia en cirugía suprarrenal / Experience in adrenal surgery

Entre 1982 y 1995, nuestra unidad operó 42 pacientes con patología suprarrenal.Fueron 13 portadores de Enfermedad de Cushing, 11 con feocromocitomas, 6 con adenomas funcionantes, 5 neoplasias malignas, 5 tumores incidentales y 2 casos misceláneos. La vía de abordaje fue transperitoneal. Se efectuaron 14 adrenalectomías bilaterales y 27 unilaterales, incluidas 3 ampliadas por cáncer. La tasa de complicaciones postoperatorias fue de 20 por ciento, básicamente respiratorias. Un caso reoperado por hemoperitoneo, y 1 fallecido (2 por ciento). Otros 4 casos de cáncer suprarrenal fallecen dentro de 24 meses. Se mantienen en control actualmente, 33 de los 37 pacientes restantes, con sobrevida entre 2 y 13 años. La vía de abordaje abdominal es conveniente para el manejo de estas lesiones

Síndrome de Cushing / Cushing's syndrome

Se hace una revision del estado actual del sindrome de Cushing. Se pone enfasis en diferenciar las tres principales causas del sindrome. Es importante, ante la sospecha del mismo, determinar bioquimicamente su presencia. Con la introduccion de finas pruebas con inmunoensayo, al alcance de muchos laboratorios, es rápida la confirmacion del diagnostico. Conociendo los niveles plasmáticos de cortisol y de ACTH y el cortisol libre en la orina de 24 horas, se puede establecer si el sindrome es ACTH dependiente o independiente (adrenal). Gracias al perfeccionamiento de la imaginologia radiologica, la TAG o CT abdominal y la RM o MRI de la hipofisis, la localizacion de la causa se ha facilitado en el 95 por ciento de los casos. La hipofisectomia anterior trasesfenoidal y la adrenalectomia uni o bilateral, segun el cuadro clinicopatologico, permiten mejorar el pronostico de esta entidad.

Síndrome de Cushing: una revisión a propósito de una casuística nacional / Cushing's syndrome: a review apropos of a national series

Cushing's syndrome occurs rarely; in analyzing 50 cases studied at our institution we discuss the following aspects: syndromatic diagnosis, etiologic differentiation into the 3 categories of the syndrome, and therapeutic strategies for each variety. We postulate that non-endocrinologists should be responseible for the syndromatic diagnosis, easily done by using 2 simple tools: the measurement of basal free urinary cortisol and the performance of and overnight suppression of the adrenal axis with 1 mg of dexamethasone (Nugent's test). In contrast, the etiologic diagnosis and the therapeutic interventions should be strictly restricted to highly specialized institutions having well seasoned endocrinologists, a reliable endocrine laboratory, easy access to computed tomographies of the brain and abdomen as well as to nuclear resonance imaging of the brain. The usefulness of our in-house devised vasopressin challenge following overnight dexamethasone suppression for the etiologic diagnosis is highlighted. Neurosurgical expertise in the transphenoidal approach to the pituitary gland as well surgeons well experience in adrenal surgery sre a must to offer a reasonable chance of success to patients with the syndrome. Forty one (82 percent) of the series were female patients, 78 percent were pituitary-depent and 22 percent pituitary-independent Cushings. Six out of 8 (75 percent) of the adrenal tumors were carcinomas. Only 3 patients (6 percent) qualified as ectopic ACTH syndormes. The easiest variety to diagnose and treat was the adrenal adenoma (2 cases); adrenal carcinomas were always incurable. The ectopic ACTH syndrome was amenable to successful medical treatment with ketoconazole or surgical resolution with complete resection od the offending tumor (1 of 3 cases) or bilateral adrenalectomy (2 of 3 cases) Pituitary-dependent Cushing are quite tricky to diagnose and difficult to treat. Transphenoidal resection of the offending microadenoma was successful in only 43.5 percent (10/23) of cases and we experienced 3 recurrences of the syndrome even after 8 years of successful removal of the pituitary adenoma. The remainder had to be cured by bilateral adrenalectomy. Seven out 39 patients with Cushing's disease (18 percent) ultimately died for a variety of reasons; six out of 6 patients (100 percent) with adrenal carcinoma died of dissemination; 2 out of 2 adrenal adenomas cured and 1 out of the 3 ectopic ACTH syndromes died of dissemination of a malignant thymic carcinoma. We conclude that Cushing's syndrome is a serious, underdiagnosed disorder, which should be suspected and diagnosed by the non specialized physician and then referred to a specialized center for expert etiologic diagnosis and surgical therapy

Tumores de hipófisis: presentación clínica y evaluación del tratamiento por cirugía transfenoidal / Pituitary tumors: clinical presentation and assesment of transphenoidal surgical treatment

The clinical manifestations and the surgical treatment results of 280 patients (179 female), undergoing a total of 319 operations at the Asenjo Institute of Neurosurgery were retrospectively analyzed. The surgical approach for the first operation was transphenoidal in 89.3 per cent of patients and transcraneal in the rest. Tumors were non secretory in 169 (60.4 per cent)patients, prolactinomas in 75 (27.8 per cent) and produced acromegalia in 29 (10.4 per cent) and Cushing syndrome in 7 (2.5 per cent) patients. There was extraselar extension in 42 per cent of women and 71 per cent of men. The surgical treatment of prolactinomas corrected endocrine alterations in 25 of 29 and visual alterations in 18 of 27 patients assessed. Surgical treatment of acromegalia improved endocrine alterations in 11 of 13 and visual alterations in 4 of 10 patients assessed. Treatment of non secretory tumors corrected visual alterations in 38 of 64 patients assessed. The principal complication of transphenoidal surgery was transient diabetes insipidus in 6.8 per cent of patients. Overall mortality was 2.3 per cent for transphenoidal surgery and 5.6 per cent for transcraneal surgery. It is concluded that transphenoidal surgery is effective for the management extraselar complications of pituitary tumors and endocrine management of selected cases

Tratamiento quirúrgico de los síndromes de hiperfuncion corticosuprarrenal / Surgical treatment of adrenal cortical hyperfunction syndros

Se presentan 20 pacientes adultos operados por hiperfunción corticosuprarrenalÑ 9 mostraron síndrome de Cushing (5 adenomas y 4 carcinomas), 6 hiperplasias por enfermedad de Cushing, 2 virilización, 2 hiperaldosteronismo y 1 feminización, confirmados por análisis de laboratorio. La TAC fue el método de localización preoperatorio de tumores adrenales más útil. Se encontró diferencia significativa en edad, duración de los síntomas y tamaño del tumor entre los pacientes con hipercortisolismo debido a adenoma y aquellos con carcinoma. La resección quirúrgica ofreció la curación a los pacientes con patología benigna, y un aceptable control de la enfermedad en carcinomas.