ABSTRACT
Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy . We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Intestine, Small/pathology , Lymphangiectasis, Intestinal/pathology , Lymphangiectasis, Intestinal/therapy , MaleABSTRACT
The syndrome of protracted diarrhea (PD) includes several diseases with diverse etiologies. This study was conducted to characterize the spectrum of causes, clinical manifestations, and the outcomes of PD. A retrospective analysis of the clinical and pathological findings was performed on 25 patients with diarrhea starting within the first 2 yr of life and a requirement of parenteral nutrition (PN). According to the intestinal histopathology, patients were classified into four groups: immune enteropathy (12 cases), lymphangiectasia (6 cases), epithelial dysplasia (5 cases), and unclassified (2 cases). All patients with epithelial dysplasia had earlier onset of diarrhea and longer duration of PN than those in the other groups. Three patients (12%) had an evidence of a familial condition. Five patients (three with microvillous inclusion disease and two with immune enteropathy) died. Sixteen patients recovered, and three (two with primary lymphangiectasia and one with microvillous inclusion disease) still had diarrhea. One patient underwent intestinal transplantation for tufting enteropathy. In conclusion, infants with PD should be referred to specialized centers where advanced diagnostic and therapeutic facilities are available, because histological analysis is critical for the diagnosis of PD, and PN or intestinal transplantation is the only therapeutic option in a subset of cases.
Subject(s)
Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Academic Medical Centers , Age of Onset , Autoimmune Diseases/pathology , Child, Hospitalized , Data Collection , Diarrhea/pathology , Enteritis/immunology , Intestinal Mucosa/pathology , Intestine, Small/immunology , Korea , Lymphangiectasis, Intestinal/pathology , Microvilli/pathology , Retrospective StudiesABSTRACT
Presentamos los hallazgos de autopsia de tres pacientes con numerosos pólipos intestinales que en el examen histológico mostraron las características propias de los quistes infangiectásicos.
Subject(s)
Humans , Male , Female , Middle Aged , Lymphangiectasis, Intestinal/pathology , Aged, 80 and overABSTRACT
Paciente de sexo femenino quien a los 6 meses presenta diarrea persistente, asociada a edemas y falla en el crecimiento. Los paraclínicos mostraron: hipoalbuminemia, linfopenia, hipocalcemia, sudan (++) en materia fecal y carotinemia baja. La biopsia intestinal reveló linfangiectasia intestinal primaria
Subject(s)
Humans , Female , Infant , Lymphangiectasis, Intestinal/congenital , Lymphangiectasis, Intestinal/diagnosis , Lymphangiectasis, Intestinal/epidemiology , Lymphangiectasis, Intestinal/etiology , Lymphangiectasis, Intestinal/pathology , Lymphangiectasis, Intestinal/physiopathology , Lymphangiectasis, Intestinal/rehabilitation , Lymphangiectasis, Intestinal/therapyABSTRACT
Informamos la observación de una niña de 18 meses con una enteropatía perdedora de proteínas con linfangiectasia intestinal, que presentó intenso edema generalizado, severa anemia, hipoalbuminemia e hipogamaglobulinemia, sin manifestaciones clínicas de malabsorción digestiva. La poca frecuencia de la entidad, la forma atípica de presentación y la excelente evolución lograda motivaron la presente comunicación
Subject(s)
Humans , Female , Infant , Protein-Losing Enteropathies/etiology , Lymphangiectasis, Intestinal/diagnosis , Anemia/complications , Protein-Losing Enteropathies/diagnosis , Protein-Losing Enteropathies/diet therapy , /drug therapy , /therapy , Lymphangiectasis, Intestinal/diet therapy , Lymphangiectasis, Intestinal/pathology , Triglycerides/therapeutic useABSTRACT
A gastroenteropatia perdedora de proteínas consequente à cirurgia cardíaca de Fontan-Kreutzer é complicaçäo pouco frequente e de difícil diagnóstico. Descreve-se o caso de criança com dez anos e oito meses de idade com cardiopatia congênita complexa operada com essa técnica, que desenvolveu edema por perda de proteíca quatro anos após a cirurgia. Após a exclusäo de patologias renais e hepáticas que justificassem a hipoproteinemia, procedeu-se o diagnóstico etiológico e topográfico da perda proteíca. O diagnóstico endoscópico e histológico nessa patologia é ressaltado