Résumé
A 36-year-old man underwent direct closure of an atrial septal defect through median sternotomy at the age of 14. He also underwent a mitral valve replacement with tricuspid annuloplasty using the same approach at the age of 18. The patient also presented with pretibial edema and congestive liver disease at the age of 27 and the pretibial edema progressed at the age of 35. Hypoalbuminemia (TP ; 3.6 g/dl, Alb ; 1.6 g/dl) was also observed. Further examinations were performed, which revealed that the right ventricular pressure curve presented a dip and plateau pattern by cardiac catheterization. Computed tomography of the chest additionally revealed thickened and calcified pericardium in the left ventricle. Abdominal scintigraphy showed tracer accumulation in the transverse colon hepatic flexure 4 h after intravenous administration of technetium-99m-labelled human serum albumin. The patient was diagnosed with a protein-losing gastroenteropathy caused by constrictive pericarditis. He underwent pericardiectomy via left anterior thoracotomy without cardiopulmonary bypass. No complications were present after the surgery, and he was discharged after 46 postoperative days. Following his discharge from the hospital, the pretibial edema disappeared, and serum albumin levels gradually increased and normalized within 3 months after the surgery (TP 7.1 g/dl, Alb 4.2 g/dl).
Résumé
Resumen Se presentan 2 casos de enfermedad de Ménétrier (EM) remitidos a nuestra institución por síndrome edematoso. Esta enfermedad de poca prevalencia es una gastropatía hipertrófica perdedora de proteínas que en la mayoría de los casos es de causa desconocida, aunque se ha asociado con procesos infecciosos. Se caracteriza por edema, hipoproteinemia, hipoalbuminemia y, en la infancia, es de carácter benigno y autolimitado.
Abstract We present two cases of Menétrier's Disease (MS) referred to our institution due to edema. The prevalence of this disease is low. It is a hypertrophic gastropathy which causes loss of proteins and which has unknown causes in the majority of cases although it has been associated with infectious processes. It is characterized by edema, hypoproteinemia, hypoalbuminemia. In childhood it is benign and self-limited.
Sujets)
Humains , Mâle , Nourrisson , Enfant d'âge préscolaire , Maladie , Hypoalbuminémie , Gastrite hypertrophique , Hypoprotéinémie , SyndromeRésumé
La enfermedad de Ménétrier infantil es una gastroenteropatía perdedora de proteínas caracterizada por hipertrofa de la mucosa gástrica, de causa desconocida, aunque la mayoría de los casos informados se han asociado a infecciones virales. Se manifesta clínicamente por edema y bioquímicamente por hipoproteinemia e hipoalbuminemia. Es poco común en los niños, en quienes tiene un carácter benigno y autolimitado, a diferencia de los adultos, en los que tiende a cronifcarse y, en ocasiones, a malignizarse. Se presenta un caso de enfermedad de Ménétrier infantil con síndrome ascítico edematoso posiblemente asociado a una infección por citomegalovirus.
Ménétrier's disease is a childhood protein-losing gastroenteropathy characterized by hypertrophy of the gastric mucosa, of unknown etiology, although most of reported cases have been associated with viral infections. Clinical manifestation is edema and biochemically there are hypoproteinemia and hypoalbuminemia. This disease is very rare in children and they have a benign and self-limiting course in contrast to adults where tend to be chronic and occasionally to become malignant. We present a child with Ménétrier disease with edema and ascites possibly associated with a cytomegalovirus infection.