Female ; Humans ; Infant ; Lymphangiectasis, Intestinal ; diagnosis ; pathology ; therapy ; Male

Adult ; Aged ; Female ; Humans ; Intestine, Small ; surgery ; Lymphangiectasis, Intestinal ; pathology ; surgery ; Male ; Middle Aged

Adult ; Humans ; Intestine, Small ; pathology ; Liver Cirrhosis ; complications ; Lymphangiectasis, Intestinal ; etiology ; Male

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.
Academic Medical Centers ; Age of Onset ; Autoimmune Diseases/pathology ; Child, Hospitalized ; Child, Preschool ; Data Collection ; Diarrhea/*pathology ; Enteritis/immunology/*pathology ; Female ; Human ; Infant ; Infant, Newborn ; Intestinal Mucosa/pathology ; Intestine, Small/immunology/pathology ; Korea ; Lymphangiectasis, Intestinal/pathology ; Male ; Microvilli/pathology ; Retrospective Studies

Distended lacteals, described as expanded white villi in duodenum, are strongly indicative of primary intestinal lymphangiectasia. In the present study, we evaluated the significance of white spots present in the duodenal mucosa of dogs with lymphocytic plasmacytic enteritis (LPE). Fifty dogs with LPE were included in this study, and white spots were detected in the duodenal mucosa in 22 dogs during endoscopy. Hypoproteinemia was more frequent in dogs with white spots than in dogs without spots (p = 0.02). Serum protein and albumin concentration were significantly lower in LPE dogs with white spots (p = 0.038) compared to LPE dogs without white spots (p = 0.039). There was a significant correlation between white spots density and lymphatic dilatation histological scores (p = 0.023; rho = 0.481). These results suggest that the presence of white spots in the duodenal mucosa of dogs is not a finding exclusive for intestinal lymphangiectasia. Low serum protein and albumin concentrations together with lymphatic dilatation seem to be related to the presence of white spots in the duodenal mucosa of LPE dogs.
Animals ; Biopsy/veterinary ; Blood Proteins/metabolism ; Dog Diseases/blood/*pathology ; Dogs ; Duodenum/*pathology ; Endoscopy/veterinary ; Female ; Histocytochemistry/veterinary ; Intestinal Mucosa/*pathology ; Lymphangiectasis, Intestinal/blood/pathology/*veterinary ; Male ; Retrospective Studies ; Statistics, Nonparametric

A 47-yr-old man with hepatitis B virus associated liver cirrhosis was admitted to our hospital with diarrhea and generalized edema and diagnosed as protein-losing enteropathy due to intestinal lymphangiectasia by intestinal biopsy and 99mTc albumin scan. During hospitalization, he received subcutaneous octreotide therapy. After 2 weeks of octreotide therapy, follow-up albumin scan showed no albumin leakage, and the serum albumin level was sustained. We speculate that liver cirrhosis can be a cause of intestinal lymphangiectasia and administration of octreotide should be considered for patients with intestinal lymphangiectasia whose clinical and biochemical abnormalities do not respond to a low-fat diet.
Adolescent ; Adult ; Duodenum/pathology ; Female ; Hepatitis B/complications ; Hepatitis B Virus/metabolism ; Human ; Intestinal Diseases/*drug therapy/virology ; Jejunum/pathology ; Liver Cirrhosis/*drug therapy/virology ; Lymphangiectasis, Intestinal/*drug therapy/virology ; Male ; Middle Aged ; Octreotide/*pharmacology ; Protein-Losing Enteropathies/*drug therapy

Acetylcholinesterase ; analysis ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Australia ; Biopsy ; Calbindin 2 ; analysis ; Child ; Diagnosis, Differential ; Endoscopy, Gastrointestinal ; Eosinophilic Esophagitis ; pathology ; Hirschsprung Disease ; metabolism ; pathology ; Humans ; Intestinal Diseases ; pathology ; Lymphangiectasis, Intestinal ; immunology ; pathology ; Multiple Endocrine Neoplasia Type 2b ; pathology ; Nervous System Diseases ; pathology ; Quality Control