Gastropatía hiperplásica glandular gigante (Enfermedad de Ménétrier)
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Rev. bol. ped.
Abstract
La enfermedad de Ménétrier se caracteriza por un crecimiento glandular con engrosamiento de pliegues en las paredes del estómago, constituyendo parte de un grupo de gastropatías hiperplásicas perdedoras de proteínas de etiología aún no precisada. Es una dolencia que ocurre usualmente en personas con edades entre 30 a 60 años, siendo muy rara en la edad pediátrica. Clinicamente se presenta con diarrea recurrente, vómitos, dolor abdominal y pérdida de peso. No existe un tratamiento específico, requiriendo solo un manejo sintomático de soporte. Los autores informan el caso de un niño de 7 años, con esta enfermedad, la cual se presentó con síntomas poco habituales (edemas, ascitis y derrame pleural); en los estudios de gabinete se confirmó por ultrasonido, ascitis, derrame pleural y una hiperecogenecidad del estómago por la cual se procedió a realizar un tránsito gastrointestinal, constatando irregularidades en el llenado gástrico. El estudio endoscópico demostró un fundus gástrico de forma irregular, engrosado, con secreción espesa, viscosa y transparente en todo el estómago. El tratamiento instaurado consistió en la administración de albúmina, con la cual se pudo obtener la desaparición de su sintomatología (edemas, ascitis y derrame pleural); posteriormente se continuó con la administración sintomática de protectores gástricos y dieta hiperproteica. Fue dado de alta tres semanas despúes sin molestias significativas.
Ménétrier's disease is characterized for a giant fold of tissue to grow in the stomach wall. This disease belongs to a group of hyperplastic gastrophaties that interfere with the absorption of protein; its cause is unknown. This is a disorder that appears between the age of 30 to 60 years old. It´s not common in adult people being unusual in pediatric patients. The symptoms include diarrhea, vomiting, tenderness in the middle part of the abdomen and loss of weight; there is not an especific treatment, only symptomatic and support to reduce its symptoms. We report about a child 7-years old who appeared with unusual symptoms like edemas, ascitis, and pleural effusion, confirmed by imaging studies, besides an increased diameter of the stomach wall. We made a gastrointestinal X-ray study, seeing irregularities in the filling and the walls of the stomach. Through a gastric endoscopy we observed a thick stomach wall, with lot of secretions. The symptomatic treatment was carried out with albumin, gastric protectors and a high-protein diet. The patient left the hospital 3 weeks later without outstanding symptoms.
Ménétrier's disease is characterized for a giant fold of tissue to grow in the stomach wall. This disease belongs to a group of hyperplastic gastrophaties that interfere with the absorption of protein; its cause is unknown. This is a disorder that appears between the age of 30 to 60 years old. It´s not common in adult people being unusual in pediatric patients. The symptoms include diarrhea, vomiting, tenderness in the middle part of the abdomen and loss of weight; there is not an especific treatment, only symptomatic and support to reduce its symptoms. We report about a child 7-years old who appeared with unusual symptoms like edemas, ascitis, and pleural effusion, confirmed by imaging studies, besides an increased diameter of the stomach wall. We made a gastrointestinal X-ray study, seeing irregularities in the filling and the walls of the stomach. Through a gastric endoscopy we observed a thick stomach wall, with lot of secretions. The symptomatic treatment was carried out with albumin, gastric protectors and a high-protein diet. The patient left the hospital 3 weeks later without outstanding symptoms.
Description
Vol. 42, No. 3