Pheochromocytoma in neurofibromatosis type 1 during pregnancy.

Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma. Pheochromocytoma is an extremely rare condition during pregnancy, and the main clinical manifestation is hypertension. If not properly treated, pheochromocytoma has high maternal and fetal mortality rates. Early recognition and adequate clinical management before delivery have led to better outcomes in the last few decades. Despite the association of NF-1 and pheochromocytoma, there are few clinical reports of these two conditions in pregnant patients. We present a rare case of pheochromocytoma diagnosed during pregnancy in a patient with NF-1, and we describe the treatment and the obstetric and fetal outcomes. We also review other medical conditions related to NF-1 that complicated this patient's pregnancy.

Diagnóstico y tratamiento endocrinológico de las lesiones del área selar en la edad pediátrica / Endocrine diagnosis and treatment of sellar lesions in pediatric age

INTRODUCCIÓN: Las lesiones selares son heterogéneas en su naturaleza y abordaje. No todas requieren cirugía. OBJETIVOS: Describir las formas de presentación de las lesiones selares y la presencia de endocrinopatías a lo largo de la evolución, así como resaltar la importancia de la valoración endocrinológica e identificar aquellas lesiones subsidiarias de tratamiento hormonal. Pacientes y métodos Estudio retrospectivo de los niños menores de 14 años remitidos a nuestro centro por lesiones selares durante 12 años. Se recogieron las siguientes variables: sexo, edad, naturaleza de la lesión, presentación clínica, tamaño, tratamiento primario y presencia de endocrinopatías. RESULTADOS: Se incluyen 45 pacientes (25 mujeres) con edades comprendidas desde los 3 meses hasta 13,5 años (media 7,2 ± 4,1) y un tiempo de seguimiento de 6,2 ± 3,7 años. Se conoce la naturaleza de la lesión en 39 casos, de los cuales 4 se han tratado eficazmente por Endocrinología: 3 prolactinomas con cabergolina, y una hiperplasia hipofisaria con levotiroxina. El motivo de consulta fueron síntomas de neuropatía y oftalmopatía en 25/45 casos, y de endocrinopatía en 13/45. El periodo de síntomas endocrinológicos previos fue de 12,6 ± 18,2 meses, frente a 2,6 ± 4,9 meses de los neurooftalmológicos (p = 0,012). En el momento del diagnóstico, 24/45 pacientes presentaban alguna endocrinopatía, ascendiendo a 37/45 al final del seguimiento. CONCLUSIONES: Las lesiones del área selar requieren un abordaje multidisciplinario. El estudio endocrinológico es imprescindible para identificar las que son subsidiarias de tratamiento hormonal. Los síntomas o signos de endocrinopatía pueden aparecer antes que los de neuropatía u oftalmopatía, por lo que es fundamental reconocerlos para adelantar el diagnóstico. La evaluación hormonal debe repetirse periódicamente a lo largo de la evolución

INTRODUCTION: Sellar masses are a heterogeneous group of lesions, both in nature and management. Not all of them require surgery. OBJECTIVES: To describe the presenting symptoms of sellar masses and endocrine abnormalities occurring during follow-up. To emphasize the significance of endocrine assessment, and to identify lesions amenable to hormonal treatment. PATIENTS AND METHODS: A retrospective review of the records of children under 14 years of age referred to our center for sellar lesions during a period of 12 years. Data collected included sex, age, nature of lesion, clinical presentation, size, treatment, and endocrine abnormalities. Results Forty-five patients (25 females) aged 7.2 ± 4.1 years (range 0.25-13.5) were enrolled. Follow-up time was 6.2 ± 3.7 years. Lesion nature was known in 39 cases, 4 of which were successfully treated at the Endocrinology Department: 3 prolactinomas (with dopamine agonist) and one thyrotroph cell hyperplasia (with levothyroxine). The most common presenting symptoms were neurological and/or visual (25/45), followed by endocrine conditions (13/45). Duration of endocrine and neuro-ophthalmic symptoms was 12.6 ± 18.2 months and 2.6 ± 4.9 months (p = 0.012), respectively. Some endocrine conditions were found in 24/45 patients at the initial evaluation and in 37/45 patients at the end of follow-up. CONCLUSIONS: Management of sellar lesions requires a multidisciplinary effort. Endocrine tests are indispensable to identify lesions amenable to hormonal treatment. Endocrine disorders usually occurred before neurological and ophthalmological symptoms, and their identification may therefore allow for earlier diagnosis. Hormone assessment should be regularly performed during follow-up

Procedimientos endoluminales/endoscópicos en el tratamiento de la obesidad / Intraluminal/endoscopic procedures in the treatment of obesity

En la actualidad contamos con escasas herramientas terapéuticas eficaces para combatir la creciente prevalencia de obesidad y comorbilidades asociadas. La cirugía bariátrica es el único tratamiento que ha demostrado su efectividad a largo plazo. Sin embargo, la alta complejidad técnica de la misma, junto a las características de los propios pacientes, implican un elevado riesgo quirúrgico e importante coste económico. Esto está llevando al desarrollo de nuevos procedimientos por vía endoscópica con un menor riesgo clínico y coste económico, manteniendo los beneficios en cuanto a morbimortalidad, incluso pudiendo servir de elemento «puente» previamente a la cirugía en los casos en que esta sea inevitable, pero permitiendo una pérdida de peso y control de comorbilidades que mejoren el riesgo prequirúrgico. El objeto de nuestra revisión es el análisis de las técnicas endoscópicas más relevantes en estos momentos

Few effective therapeutic tools are currently available to fight the increasing prevalence of obesity and its associated comorbidities. Bariatric surgery is the only treatment with proven long-term effectiveness, but is associated to a high surgical risk and significant economic costs because of its technical complexity and the characteristics of patients. This is leading to development of new endoscopic procedures with less clinical risks and economic costs, while maintaining the benefits in terms of morbidity and mortality, which could even serve as a bridging element before surgery in cases where this is unavoidable, allowing for preoperative weight loss and control of comorbidities in order to improve anesthetic risks and possible complications. The purpose of this review was to analyze the most relevant and promising endoscopic techniques currently available

Endocrine diagnosis and treatment of sellar lesions in pediatric age.

INTRODUCTION: Sellar masses are an heterogeneous group of lesions, both in nature and management. Not all of them require surgery. OBJECTIVES: To describe the presenting symptoms of sellar masses and endocrine abnormalities occurring during follow-up. To emphasize the significance of endocrine assessment, and to identify lesions amenable to hormonal treatment. PATIENTS AND METHODS: A retrospective review of the records of children under 14 years of age referred to our center for sellar lesions during 12 years. Data collected included sex, age, nature of lesion, clinical presentation, size, treatment, and endocrine abnormalities. RESULTS: Forty-five patients (25 females) aged 7.2 ± 4.1 years (range 0.25-13.5) were enrolled. Follow-up time was 6.2 ± 3.7 years. Lesion nature was known in 39 cases, 4 of which were successfully treated at the Endocrinology Department: 3 prolactinomas (with dopamine agonist) and one thyrotroph cell hyperplasia (with levothyroxine). The most common presenting symptoms were neurological and/or visual (25/45), followed by endocrine conditions (13/45). Duration of endocrine and neuro-ophthalmic symptoms was 12.6 ± 18.2 months and 2.6 ± 4.9 (P=.012), respectively. Some endocrine condition was found in 24/45 patients at the initial evaluation and in 37/45 patients at the end of follow-up. CONCLUSIONS: Management of sellar lesions requires a multidisciplinary effort. Endocrine tests are indispensable to identify lesions amenable to hormonal treatment. Endocrine disorders usually occurred before neurological and ophthalmological symptoms, and their identification may therefore allow for earlier diagnosis. Hormone assessment should be regularly performed during follow-up.

[Intraluminal/endoscopic procedures in the treatment of obesity]. / Procedimientos endoluminales/endoscópicos en el tratamiento de la obesidad.

Few effective therapeutic tools are currently available to fight the increasing prevalence of obesity and its associated comorbidities. Bariatric surgery is the only treatment with proven long-term effectiveness, but is associated to a high surgical risk and significant economic costs because of its technical complexity and the characteristics of patients. This is leading to development of new endoscopic procedures with less clinical risks and economic costs, while maintaining the benefits in terms of morbidity and mortality, which could even serve as a bridging element before surgery in cases where this is unavoidable, allowing for preoperative weight loss and control of comorbidities in order to improve anesthetic risks and possible complications. The purpose of this review was to analyze the most relevant and promising endoscopic techniques currently available.