Feocromocitoma: nuevas perspectivas en diagnóstico y seguimiento / Pheochromocytoma: new insights in diagnosis and monitoring

La hipertensión arterial es una patología de menor frecuencia en pediatría que en la edad adulta, sin embargo existen diversas etiologías que la pueden originar y es fundamental realizar diagnóstico certero de las mismas. El feocromocitoma es un tumor endócrino, originado en el tejido cromafin y constituye una importante causa de hipertensión secundaria. Puede ser esporádico o formar parte de diversos síndromes familiares, como la enfermedad de von Hippel Lindau, la Neoplasia Endócrina Múltiple tipo 2, los síndromes de Feocromocitoma/ Paraganglioma Familiar tipo 1, 2, 3 y 4, y la Neurofibromatosis tipo 1. Además, en la actualidad se han descrito más de 15 genes susceptibles que han sido implicados en casos familiares. Siendo tumores altamente heredables, se recomienda realizar pruebas genéticas en todos los pacientes, incluso en aquellos con una historia familiar comprobable. El diagnóstico de la enfermedad hereditaria mejora la atención del paciente y permite el diagnóstico precoz en familiares

Arterial hypertension is a less frequent condition in paediatrics than in adulthood, but due to the high proportion of secondary causes, the search for the aetiology is crucial for an adequate diagnosis and treatment. Pheochromocytoma is an endocrine tumor originated in the chromaffin tissue. It is an important cause of secondary hypertension. It can be sporadic or part of several hereditary syndromes, such as von Hippel Lindau disease, Multiple Endocrine Neoplasia type 2, Pheochromocytoma/Familiar Paraganglioma syndromes type 1, 2, 3 and 4, and type 1 Neurofibromatosis. In addition, more than 15 susceptible genes have been described in familial pheochromocytoma. Being highly heritable tumors, genetic testing of tumor susceptibility is recommended in all patients, even in absence of a family history. The diagnosis of the hereditary disease improves the patient's attention and allows early diagnosis in relatives

Hipertensión de causa adrenal y embarazo / Hypertension of adrenal origin and pregnancy

La hipertensión arterial de causa adrenal asociada al embarazo es infrecuente. Su presencia genera una elevada morbilidad y mortalidad materna y fetal. Los cambios de la fisiología endocrina que acontecen en esta etapa, modifican el cuadro clínico y bioquímico de las enfermedades adrenales causantes de hipertensión. El objetivo de esta revisión es brindar información actualizada sobre el hipercortisolismo endógeno, el aldosteronismo primario y el feocromocitoma en la gestación -a los profesionales de la salud vinculados con la atención a embarazadas- que les permita diagnosticar y tratarlas temprana y adecuadamente mediante la aplicación del método clínico. Se revisa la literatura y se exponen los avances médicos en el tema(AU)

Blood hypertension of adrenal origin associated to pregnancy is unfrequent and generates high maternal and fetal morbidity and mortality. Changes in the endocrine physiology during this phase modify the clinical and biochemical picture of hypertension-causing adrenal diseases. The objective of this review was to provide health professionals in charge of pregnancy care with updated information on endogenous hypercortisolism, primary aldosteronism and pheochromocytoma in pregnancy. This will allow them to diagnose and treat this disorder early and adequately by means of the clinical method. Relevant literature was reviewed and the latest medical advances in this field were presented(AU)

The Overview of Endocrine Hypertension / 대한내과학회지

The prevalence of hypertension is approximately 30%. Hypertension is a major risk factor for stroke, ischemic heart disease and cardiac failure. In most, hypertension is essential or idiopathic, but a subgroup of approximately 15% has secondary hypertension. Endocrine hypertension is one of the common causes of secondary hypertension and is characterized by hormonal derangements. There are at least 14 endocrine disorders for which hypertension may be the initial clinical presentation. An accurate diagnosis of endocrine hypertension provides the clinician with a unique treatment opportunity. Therefore understanding about the appropriate diagnosis and management is very important.

Diagnostic value of adrenal CT scan for screening adrenal hypertension in out-patients with hypertension / 中华内分泌代谢杂志

ObjectiveTo evaluate the priority application of thin adrenal CT scanning in out-patients with hypertension for early diagnosis of adrenal hypertension.MethodsThe patients with hypertension were collected from January to August 2009,in whom the secondary hypertension ( including endocrine and renal hypertension) and malignant cancers were excluded.All patients accepted the adrenal CT scan firstly,and then the patients with the positive result of morphological abnormalities in the adrenal CT( including adenoma and hyperplasia)were evaluated for endocrine function of adrenal glands.Results A total 362 cases of hypertension were studied,in.cluding 162 males and 200 females.The average age was (60.9 ±9.3 ) year-old,the average duration of hypertension was ( 13.8 ±5.3 ) year.After adrenal thin layer scanner CT scan,35 patients ( 9.6％ ) with adrenal abnormalities were found.Endocrine functional assessments of adrenal glands were carried out among these patients,17 cases (4.7％) with functional lesions were diagnosed,including 14 cases(3.9％ )with primary hyperaldosteronism,2 cases(0.6％ )with Cushing's syndrome,and 1 case ( 0.3％ ) with pheochromocytoma.Conclusion This study revealed that the percentage of adrenal lesions in outpatients with hypertension was 9.6％.The percentage of adrenal hypertension was 4.7％.Thin layer CT scan seems to effectively screen the adrenal hypertension with adrenal lesions,and would be helpful to the diagnosis of endocrine hypertension.

Effect of hypercortisolism control on high blood pressure in Cushing's syndrome / Efecto del control del hipercortisolismo sobre la hipertensión arterial en el síndrome de Cushing

Many hypertensive patients affected by endogenous Cushing's syndrome (CS) persist with high blood pressure (HBP) despite good control of cortisol excess. We assessed the effect of preoperative ketoconazole administration and of definitive treatment of CS on arterial hypertension and analysed the factors involved in the persistence of hypertension. We assessed retrospectively 71 patients with CS and HBP (60 women, 11 men; 50 pituitary, 21 adrenal) successfully treated by surgery and/or radiotherapy; 19 of them received ketoconazole (KNZ) before surgery. After treatment, patients were divided into those with persistent high blood pressure (PHBP) and those with normal blood pressure (NBP). As possible predictive factors for PHBP we analysed age, duration and family history of HBP, pre-treatment 24 hour urinary free cortisol (24h-UFC) and body mass index (BMI). HBP normalized in 53 out of 71 patients (74.6%), regardless of the origin of Cushing's syndrome. PHBP patients were older (p=0.003), had longer duration (p=0.007) and higher systolic blood pressure before treatment (p=0.046) than NBP patients. Thirteen out of 19 patients (68.4%) treated with ketoconazole, normalized their hypertension and remained normotensive after successful surgery. Five patients became normotensive only after surgery. In conclusion: a) blood pressure levels normalized in most patients after remission of CS; b) ketoconazole was effective for the control of HBP, and seems to be a good indicator of post-surgical outcome, and c) higher age at presentation, longer duration of hypertension and higher systolic blood pressure figures before treatment negatively influence normalization of blood pressure after resolution of Cushing's syndrome.

Muchos pacientes con síndrome de Cushing (SC) permanecen hipertensos a pesar del control del exceso glucocorticoideo. Investigamos el efecto de la administración de ketoconazol (KNZ) y del tratamiento definitivo del SC sobre la hipertensión arterial (HTA), analizando su relación con diversos factores. Evaluamos 71 pacientes con SC e HTA (60 mujeres, 11 varones; 50 pituitarios, 21 adrenales) exitosamente tratados por cirugía y/o radioterapia; 19 de ellos recibieron KNZ antes de cirugía. Luego del tratamiento, fueron divididos en pacientes con HTA persistente (HTAP) y normal (HTAN). Como posibles factores predictivos de HTAP se analizaron edad, duración, historia familiar de HTA, cortisol libre urinario de 24 hs pre-tratamiento e índice de masa corporal. La HTA normalizó en 53/71 pacientes (74.6%) independientemente del origen del síndrome de Cushing. Los pacientes con HTAP fueron de mayor edad (p=0.003), con mayor duración previa (p=0.007) y valores mayores de presión arterial sistólica antes de tratamiento (p=0.046) que aquellos con HTAN. Trece de 19 pacientes (68.4 %) tratados con ketoconazol normalizaron su tensión arterial y se mantuvieron normotensos luego de cirugía exitosa. Cinco pacientes se tornaron normotensos solo después de cirugía. En conclusión: a) la HTA se normalizó en la mayoría de pacientes luego de remisión del SC, b) el ketoconazol fue efectivo para el control tensional y aparenta ser indicador de la evolución pos-quirúrgica, y c) mayor edad, duración más prolongada de la HTA y valores más altos de presión sistólica influencian negativamente la normalización de la presión arterial luego de resolución del síndrome de Cushing.

Studies on diagnosis and pathogenesis of pheochromocytoma / 中华内分泌代谢杂志

Pheochromocytoma is the catecholamine-secreting tumor arising from the adrenal medulla and extra-adrenal chromaffin cells and the important cause of endocrine hypertension, which leads to serious complications of the heart, brain and renal vascular events. The prevalence of phochromocytoma accounts for approximately 1.9% in the population with hypertension. The detection, diagnosis and treatment of pheochro-mocytoma at early stage are of uppermost importance. More efforts should be made to study the pathogenesis and management of pheochromocytoma.