Are children with protein-losing enteropathy after the Fontan operation at increased risk of cytomegalovirus enteropathy? A report of two cases.

INTRODUCTION: Aetiology of protein-losing enteropathy in single-ventricle type CHD is multi-factorial. REPORT: We describe two Fontan patients with protein-losing enteropathy who presented with cytomegalovirus-associated colitis. DISCUSSION: Fontan patients display risk factors for cytomegalovirus-induced gastroenteropathy that may affect lymph angiogenesis, disease development, and progression. CONCLUSION: Cytomegalovirus enteropathy may be common among Fontan patients who suffer from protein-losing enteropathy. Polymerase chain reaction is important for detection.

[Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child]. / Gastroenteropatía perdedora de proteínas y posible relación con una infección por citomegalovirus: Presentación de un caso de enfermedad de Ménétrier infantil.

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.

Gastroenteropatía perdedora de proteínas y posible relación con una infección por citomegalovirus: Presentación de un caso de enfermedad de Ménétrier infantil / Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child

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.(AU)

Ménétriers 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.(AU)

Gastroenteropatía perdedora de proteínas y posible relación con una infección por citomegalovirus: Presentación de un caso de enfermedad de Ménétrier infantil / Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child

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.

Gastroenteropatía perdedora de proteínas y posible relación con una infección por citomegalovirus: Presentación de un caso de enfermedad de Ménétrier infantil. / [Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child].

Ménétriers 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.

Pleconaril-resistant chronic parechovirus-associated enteropathy in agammaglobulinaemia.

A 14 year old common variable immunodeficiency patient developed severe protein-losing enteropathy. A chronic enteral infection with human parechovirus type 1 and norovirus was diagnosed. Treatment strategies aimed at virus eradication and providing supportive care were ineffective. The antipicornavirus agent pleconaril did not have any effect on viral replication. Symptoms improved on immunosuppressive therapy, suggesting infection-related immune dysregulation in an immunocompromised host.

Cytomegalovirus-associated protein-losing enteropathy resulting from lymphangiectasia in an immunocompetent child.

An immunocompetent 8-year-old boy with cytomegalovirus (CMV)-associated transient protein-losing enteropathy (PLE) is described. Colonoscopic examination revealed lymphoid hyperplasia of the terminal ileum. Histological examination of the biopsied specimens showed marked dilation of the lymphatic vessels. Primary CMV infection was demonstrated by serological test and polymerase chain reaction. The child had complete resolution of the disease without antiviral treatment. The present case suggests the etiologic role of CMV infection in PLE resulting from intestinal lymphangiectasia in childhood.

Cytomegalovirus-associated protein losing gastropathy in an immunocompetent adult: a case report.

Cytomegalovirus infection of the gastro-intestinal tract is frequent and may be serious in the immunocompromised patient. We report a case of cytomegalovirus infection in an immunocompetent young man who presented total food intolerance, pleural effusion and oedema as the result of severe protein losing hypertrophic gastropathy. Hypertrophic gastropathy with severe mucosal protein loss has been described in Menetrier's disease, a condition of unknown cause which involves foveolar hyperplasia of the gastric mucosa. Related hypoalbuminemia is responsible for a clinical picture of diffuse edema. In adults, the natural course of the disease is marked by a chronic course and carries a bad prognosis. In our case, the disease ran a protracted disabling course, likely shortened by ganciclovir therapy, followed by slow clinical, endoscopic and biochemical resolution after several' months course.

Eosinophilic gastroenteritis with cytomegalovirus infection in an immunocompetent child.

A 3-year-old boy developed transient protein-losing gastroenteropathy associated with cytomegalovirus (CMV) infection. Both IgG and IgM antibodies to CMV were positive in a serologic blood test. Upper gastrointestinal endoscopy showed multiple erosions throughout the body of the stomach, without enlarged gastric folds. Histological examination of the biopsy specimens indicated eosinophilic gastroenteritis and CMV infection. The patient had complete resolution without specific therapy for CMV in four weeks. An allergic reaction as well as CMV infection played important roles in the pathogenesis of this case.

[Cytomegalovirus-induced protein-losing gastropathy: a case report]. / Un raro caso di gastroenteropatia ipertrofica protido-disperdente da CMV.

Protein-losing gastropathy is an uncommon disease of uncertain etiology, known also as Menetrier's disease. In medical literature only 50 pediatric cases have been described. These childhood forms, in contrast to classic adult Menetrier's disease, have a typical benign and transient course, and require only supportive therapy. The role of Cytomegalovirus (CMV) in the pathogenesis has been demonstrated by gastric biopsy in one third of the cases. Also other infectious, allergic and immunological factors have been hypothesized. We describe a case of hypertrophic gastropathy with important protein-loss, admitted to our Pediatric Department for evaluation because of vomit, weight loss, abdominal pain and hypoalbuminemia. Gastric mucosal biopsy revealed a morphological evidence of CMV infection.

Cytomegalovirus-induced transient protein-losing hypertrophic gastropathy in an immunocompetent adult.

Transient protein-losing hypertrophic gastropathy with similarity to Ménétrier's disease is described. Acute infection with cytomegalovirus (CMV) could be shown to play a causative role. Immunodeficiency was ruled out. The 34-year-old patient had complete resolution of the disease without antiviral treatment. To our knowledge the present report is the first case of CMV-associated protein-losing hypertrophic gastropathy in an immunocompetent adult. To date, a similar disorder has only been described in children. CMV infection should be considered in patients with acute and symptomatic protein loss of gastrointestinal origin.