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Posterior reversible encephalopathy syndrome (PRES) is an uncommon transient neuroradiological phenomenon that develops vasogenic cerebral edema and could be caused by some pharmacological agents, such as molecular-specific target agents. Lenvatinib belongs to the tyrosine kinase inhibitors and was approved in 2015 for progressive locally advanced or metastatic thyroid cancer refractory to radioactive iodine (I-131) treatment. Herein, we present the case of a 65-year-old woman who, while receiving treatment with lenvatinib for radioiodine-refractory metastatic papillary thyroid carcinoma, developed PRES without hypertension at the initial evaluation. Her clinical and radiological findings improved after withdrawing from the mentioned therapy, and later it was possible to re-incorporate lower doses of the medication, as described in the other three case reports found in the worldwide medical literature. The recognition of this entity is essential to timely suspend the drug and avoid greater comorbidity. This is the first paper reporting this kind of adverse event using lenvatinib in a Hispanic population.

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Resumen El complejo de Carney es una enfermedad caracterizada por lesiones en la piel, tumores endocrinos, cardiacos, gonadales y en otros órganos, que está asociada con mutaciones del gen PRKAR1A. Presentamos el caso clínico de una paciente con varias de las manifestaciones más características de este síndrome. Finalmente, se hace una revisión de la literatura.

Abstract Carney complex is a disease characterized by skin lesions, endocrine, cardiac, gonadal and other organ tumors, associated with mutations of the PRKAR1A gene. We present the clinical case of a patient with several of the most characteristic manifestations of this syndrome. Finally, there will be a review of the literature.

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RESUMEN Los glucocorticoides son medicamentos ampliamente usados para el manejo de muchas enfermedades crónicas; a pesar de esto, están asociados con múltiples efectos adversos que pueden afectar diferentes órganos. En la presente revisión se hará una descripción de los efectos derivados del uso de estos sobre el eje hipotálamo-hipófisis-adrenales y los diferentes sistemas, haciendo énfasis en el sistema cardiovascular y óseo.

SUMMARY Glucocorticoids are widely used medications for the management of many chronic diseases, but they are associated with multiple adverse effects that can affect different organs. In the present review, a description will be made of the effects derived from the use of these medications on the hypothalamic-pituitary-adrenal axis and other systems, with emphasis on the cardiovascular system and bone.

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RESUMEN El coronavirus 2 del síndrome respiratorio agudo grave es el tercer betacoronavirus desde el año 2003 capaz de ocasionar una infección del tracto respiratorio inferior, llevando, en casos críticos, al síndrome de dificultad respiratoria aguda y la muerte. La edad avanzada, la hipertensión arterial y la diabetes mellitus son, entre otros, tres factores determinantes en los peores desenlaces clínicos. Múltiples mecanismos pueden explicar la mayor susceptibilidad de las personas diabéticas a las infecciones respiratorias. La hiperglucemia crónica altera tanto a la inmunidad humoral como al celular. Esta enfermedad predispone a la sobreexpresión de la proteína de la membrana celular que sirve como receptora del virus y a una respuesta inflamatoria exacerbada, aumentando el riesgo de una descompensación y de la aparición de crisis hiperglicémicas. Ante la ausencia de un tratamiento efectivo o de una vacuna, todos los esfuerzos deben hacerse para procurar un buen control metabólico de los pacientes con diabetes mellitus con y sin COVID-19. Por lo anterior, se plantean en este artículo de reflexión, diferentes propuestas para el tratamiento de la diabetes mellitus en la unidad de cuidados intensivos, sin descartar la forma ambulatoria, en donde la telemedicina y otras tecnologías permitirán acortar la distancia y mantener las medidas de aislamiento preventivo.

SUMMARY Severe acute respiratory syndrome coronavirus 2 is the third beta-coronavirus since 2003 capable of causing lower respiratory tract infection, leading to severe cases of acute respiratory distress syndrome and death. Advanced age, high blood pressure and diabetes mellitus are three predictors of worse clinical outcomes. Multiple mechanisms could explain the greater susceptibility of diabetic people to respiratory infections. Chronic hyperglycemia alters both humoral and cellular immunity. This disease predisposes to virus receptor overexpression and an exaggerated inflammatory response, increasing the risk of decompensation and hyperglycemic crises. In the absence of an effective vaccine or treatment for the virus, this vicious circle should be stopped with an emphasis on controlling glucose. This paper presents different proposals for the treatment of diabetes mellitus both on an outpatient basis where telemedicine and other technologies will make it possible to continue adequate ambulatory care to maintain preventive isolation measures up to care in the intensive care unit.

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OBJECTIVE: The aim was to describe the clinical features of patients with ectopic Cushing syndrome (ECS) from Colombia and compare these findings with other series to provide the best management for these patients. METHODS: Records of patients with ECS from 1986 to 2017 were retrospectively reviewed; patients with a diagnosis of adrenal or pituitary Cushing syndrome (CS) were excluded. RESULTS: Fourteen patients with ECS were analyzed in this study. The mean age was 54.4 (SD 17.1) years, and the female to male ratio was 1.33:1. Regarding the etiology of ECS, four patients had lung carcinoids (28.6%), three had small-cell lung carcinoma (21.4%), three had pancreatic neuroendocrine tumors (21.4%), one had medullary thyroid cancer (7.1%), one had non-metastatic pheochromocytoma (7.1%), one had metastatic thymoma (7.1%) and one patient had an occult source of ACTH (7.1%). The most common clinical features at presentation were moon-face, muscle weakness, diabetes mellitus and hypertension. Hyperpigmentation was present in 36% of patients, and 12 patients had hypokalemia with a mean value of 2.3 mEq/L (SD 0.71). The median basal cortisol, 24-hour urinary free cortisol (UFC) and ACTH were 30.5 ug/dL (IQR 21-59 ug/dL), 2,600 ug/24 h (IQR 253-6,487 ug/24 h) and 91 pg/mL (IQR 31.9-141.9), respectively. Thirteen patients (92.8%) had the site of the primary lesion identified. Six patients had undergone a surgical intervention to address the primary tumor. Resection was curative in 28.5% of patients. Death occurred in 57.1% of patients, and the median overall survival was 27 months. Intrathoracic tumors had the most aggressive behavior. CONCLUSION: ECS is a rare disease; however, it is associated with high morbidity and mortality. A rapid intervention supported by an interdisciplinary group is required to improve overall survival and quality of life.

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ABSTRACT Objective The aim was to describe the clinical features of patients with ectopic Cushing syndrome (ECS) from Colombia and compare these findings with other series to provide the best management for these patients. Materials and methods Records of patients with ECS from 1986 to 2017 were retrospectively reviewed; patients with a diagnosis of adrenal or pituitary Cushing syndrome (CS) were excluded. Results Fourteen patients with ECS were analyzed in this study. The mean age was 54.4 (SD 17.1) years, and the female to male ratio was 1.33:1. Regarding the etiology of ECS, four patients had lung carcinoids (28.6%), three had small-cell lung carcinoma (21.4%), three had pancreatic neuroendocrine tumors (21.4%), one had medullary thyroid cancer (7.1%), one had non-metastatic pheochromocytoma (7.1%), one had metastatic thymoma (7.1%) and one patient had an occult source of ACTH (7.1%). The most common clinical features at presentation were moon-face, muscle weakness, diabetes mellitus and hypertension. Hyperpigmentation was present in 36% of patients, and 12 patients had hypokalemia with a mean value of 2.3 mEq/L (SD 0.71). The median basal cortisol, 24-hour urinary free cortisol (UFC) and ACTH were 30.5 ug/dL (IQR 21-59 ug/dL), 2,600 ug/24 h (IQR 253-6,487 ug/24 h) and 91 pg/mL (IQR 31.9-141.9), respectively. Thirteen patients (92.8%) had the site of the primary lesion identified. Six patients had undergone a surgical intervention to address the primary tumor. Resection was curative in 28.5% of patients. Death occurred in 57.1% of patients, and the median overall survival was 27 months. Intrathoracic tumors had the most aggressive behavior. Conclusions ECS is a rare disease; however, it is associated with high morbidity and mortality. A rapid intervention supported by an interdisciplinary group is required to improve overall survival and quality of life

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RESUMEN Objetivo: describir la experiencia de los pacientes con insulinoma, diagnosticados y tratados entre los años 2002 y 2017 en tres hospitales de la ciudad de Medellín, Colombia. Métodos: estudio descriptivo y retrospectivo de pacientes con criterios bioquímicos para hipoglucemia hiperinsulínica y confirmación histopatológica de insulinoma. Resultados: se estudiaron 19 casos, 10 eran mujeres (52,6 %), la edad media al diagnóstico fue 43 años (D.E 15,5). Hubo cuatro casos de insulinoma multifocal (21,1 %), tres asociados con NEM-1 (15,8 %) y dos malignos (10,6 %). Todos presentaron hipoglucemia en ayunas y 63,2 % posprandial. En la prueba de ayuno, el nadir de glucemia sucedió antes de 48 horas en todos los casos, en promedio 9 horas (D.E 8,0). El diagnóstico bioquímico fue realizado con hipoglucemia e insulina elevada en todos los casos, aunque el péptido C fue reportado en nueve pacientes (47,3 %) y las sulfonilureas en dos (11,1 %). La localización preoperatoria se hizo por imágenes en 12 individuos (68,5 %) y las pruebas invasivas fueron necesarias en seis (31,5 %). Las pruebas diagnósticas fueron positivas en un 83 % para resonancia, 50 % para ecografía endoscópica y prueba de estímulo intraarterial con calcio y 100 % para ecografía intraoperatoria. La cirugía se realizó en 18 casos (94,7 %). La mortalidad (15,8 %) fue derivada de complicaciones en el posoperatorio temprano; la curación se logró en todos los casos. Conclusiones: el insulinoma en nuestro medio tiene características demográficas y clínicas similares a otras series. Existen limitaciones locales para el acceso a los estudios bioquímicos y en el rendimiento diagnóstico de las pruebas de localización.

SUMMARY Objective: The objective of the study was to describe the characteristics of patients with insulinoma in three hospitals in Medellín, Colombia, between 2002 and 2017. Methods: A retrospective analysis of patients with hyperinsulinemic hypoglycemia and histologic confirmation of insulinoma was conducted. Results: A total of 19 cases were identified. Ten women (52.6 %) and 9 males (47.4 %). The mean age at diagnosis was 43 years (S.D: 15.5). Four cases had multifocal insulinoma (21.1%), 3 cases were secondary to multiple endocrine neoplasia type 1 (15.8 %), and 2 of them were malignant (10.6 %). All patients presented fasting hypo-glycemia, and 63.2% presented post-prandial hypoglycemia. Glucose nadir in the fasting test occurred in the first 48 hours in all cases, with a mean time to hypoglycemia of 9 hours (S.D 8.0). The biochemical diagnosis was done with increased insulin in the presence of hypoglycemia. C-peptide was done in 9 patients (47.3 %), and sulfonylureas in 2 cases (11.1 %). Preoperative localization was done by imaging in 12 cases (68.5 %), and invasive tests were required in 6 cases (31.5 %). Localization tests were positive as follows: magnetic resonance imaging in 83%, endoscopic ultrasound in 50%, selective intra-arterial calcium injection in 50 %, and intraoperative ultrasound in 100%. Eighteen patients (94. 7%) underwent surgery. Mortality (15.8 %) was secondary to early post-operative complications. Conclusions: The characteristics of patients with insulinoma in Medellín are similar to other series. However, there are important local limitations for proper biochemical testing and imaging localization. This is the largest study in our country.

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El síndrome de Cushing es una enfermedad muy rara pero asociada a una morbimortalidad significativa. Se clasifica como dependiente de la hormona adrenocorticotrópica (ACTH: tumores hipofisiarios y ectópicos) o independiente de ACTH (lesiones de origen adrenal). En la mayoría de los casos, las lesiones responsables del síndrome corresponden a tumores hipofisiarios, seguida de lesiones adrenales y por último de tumores ectópicos (5-15% de todos los casos). En este artículo se hará una revisión de los aspectos epidemiológicos, clínicos, diagnósticos y terapéuticos más importantes de los tumores ectópicos causantes del síndrome de Cushing.

Cushing's syndrome is a very rare disease associated with significant morbidity and mortality. It is classified as adrenocorticotropic hormone (ACTH) dependent (Pituitary and ectopic tumors) or ACTH independent (lesions of adrenal origin). In most cases, pituitary tumors are responsible for the Cushing's syndrome, followed by adrenal lesions and finally by ectopic tumors (5-15% of all cases). This article describes the most important epidemiological, clinical, diagnostic and therapeutic aspects of ectopic tumors causing Cushing's syndrome.

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Resumen: el feocromocitoma es una enfermedad poco frecuente, correspondiente a un tumor derivadode las células cromafines, originado en la médula de la glándula suprarrenal. Cuando este tumor se origina en los paraganglios el término correcto es paraganglioma. Las manifestaciones clínicas son variables y secundarias a la masa tumoral o a la producción exagerada de catecolaminas. Los síntomas originados por el crecimiento de la masa tumoral incluyen dolor abdominal, malestar epigástrico, obstrucción de estructuras cercanas como la vía biliar y los uréteres, y, cuando está localizado en otros sitios como el mediastino, disnea. Los síntomas secundarios a la hiperproducción de catecolaminas son diaforesis, palpitaciones, cefalea, crisis hipertensivas, hipertensión sostenida, constipación, náuseas, vómito y, en la forma más exagerada, crisis catecolaminérgicas. El diagnóstico se realiza mediante la medición de metanefrinas libres en orina de 24 horas o en plasma, y como alternativa se puede realizar medición de catecolaminas en orina de 24 horas. La medición de ácido vanilmandélico es poco sensible, lo mismo que la medición de catecolaminas en plasma, la cual no es recomendada. El tratamiento de esta enfermedad es quirúrgico. En aquellos pacientes con feocromocitomamaligno o paraganglioma se puede realizar una estrategia de observación activa y en quienes tengan progresión de la enfermedad el uso de quimioterapia o, según el grado de compromiso, el uso de metayodobencilguanidina marcada con yodo-131 es la terapia de elección. El uso de terapia molecular diana utilizando inhibidores de tirosina quinasa es un área de investigación activa.

Abstract: Pheochromocytoma is a rare disease, corresponding to a tumor originated in the chromaphincells, and located in the adrenal medulla. When the tumor is located in the paraganglia the appropriate term is paraganglioma. Clinical manifestations are variable and secondary to the tumoral local extension or to the overproduction of catecholamines. Symptoms secondary to local growth of the tumor includes abdominal pain, epigastric discomfort, obstruction of nearby structures such as biliary tree and ureters, and when is located in other regions such as the mediastinum, dyspnea. Symptoms secondary to overproduction of catecholamines are diaphoresis, headache, hypertensive emergencies, sustained hypertension, constipation, nausea, vomiting and, in the most extreme form, catecholaminergic crisis. Diagnosis is based on the measurement of free plasmatic or urinary metanephrines.As an alternative can be used 24 hours urinary catecholamines. The detection of vanillylmandelic acid and plasma catecholamines has poor sensibility and is no longer recommended. The treatment of this entity is surgical resection. In those patients with malignant pheochromocytoma or paraganglioma an observation and watch strategy can be used. If disease progression is detected, the use of chemotherapy or iodine-131-labeled meta-iodobenzylguanidine, according to the severity of compromise, is the next step. The use of targeted molecular therapies using tyrosine kinase inhibitoris an area under active research.

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Se describe el caso de una paciente evaluada a los 29 años por un tumor tiroideo, clasificado inicialmente como un carcinoma insular de tiroides; presentaba gran compromiso tumoral en cuello, mediastino y pulmones. Tres años después de recibir tratamiento específico para su neoplasia tiroidea, desarrolló un síndrome de Cushing y lesiones hepáticas sugestivas de metástasis del tumor primario. Al revisar la patología se encontró, en lo que inicialmente se había clasificado como un carcinoma insular, un carcinoma medular de tiroides productor de hormona adrenocorticotrópica (ACTH). Con base en este caso, se presenta una revisión de la literatura médica sobre el tema.

Cushing syndrome secondary to a medullary thyroid carcinoma: report of a case and review of the literature We report the case of a 29-year old female who was evaluated because of a thyroid tumor. The initial pathological classification was an insular thyroid carcinoma. There was strong involvement in the neck, mediastinum and lungs. Three years after receiving specific therapy for her thyroid neoplasia, she developed a Cushing syndrome and liver lesions suggestive of metastases from the primary tumor. A review of the previous pathological material revealed a medullary thyroid carcinoma producing ACTH, instead of the insular carcinoma. Based on this case a review of the literature is presented.

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