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At least 163 records · Page 9Linked to original sources
PubMed1978

Lymphangiectasia of the small intestine as an acute surgical problem.

Two cases of lymphangiectasia of the small intestine in elderly women are presented. The condition manifested itself in one case by a sudden perforation of the jejunum; in the other case by ileus of the terminal ileum. In both cases the disease process pursued a relentless course that ultimately led to the death of the patients. Elective surgery in patients with lymphangiectasia has been performed before. Acute surgery of lymphangiectasia of the small intestine with complaints of acute abdomen, obstruction or perforation as presenting signs of the disease has not been described previously.

Aged↗
PubMedDate not supplied

Characteristic endoscopic features of intestinal lymphangiectasia: correlation with histological findings.

Six patients with histologically proven intestinal lymphangiectasia were evaluated for the endoscopic features. White villi and/or spots, previously reported as endoscopic findings, were seen. Furthermore, white nodules and submucosal elevations with or without white mucosa were observed. All patients demonstrated the appearance of submucosal elevations. The four characteristic endoscopic features were correlated with histological findings. It is considered that these endoscopic features are of value for the diagnosis of intestinal lymphangiectasia in patients with protein-losing enteropathy.

Adolescent↗
PubMed1998

Intestinal lymphangiectasia, a disease characterized by selective loss of naive CD45RA+ lymphocytes into the gastrointestinal tract.

Intestinal lymphangiectasia (InL) is a disease characterized by hypoproteinemia and lymphocytopenia resulting from blocked intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract. This leads to immunologic abnormalities including hypogammaglobulinemia, skin anergy and impaired allograft rejection. In the present study, we evaluated whether the above immunologic abnormalities are secondary to a quantitative or qualitative disorder of T cells. In initial studies we demonstrated that adult InL patients' peripheral blood contain strikingly (and significantly) reduced numbers of CD4+/CD45RA+ T cells, whereas the numbers of CD4+/CD45RO+ T cells were only moderately (and not significantly) reduced. In addition, the CD4+/CD45RO+ T cell population contained an increased percentage of highly differentiated and previously sensitized cells, as demonstrated by decreased CD27 and CD31 expression and increased HLA-DR and CD69 expression. In subsequent functional studies, we showed that the InL CD4+/CD45RO+ T cells, when stimulated in vitro, proliferate fivefold less than control CD4+/CD45RO+ T cells and produce fourfold more IL-4 and threefold less IFN-gamma and IL-2. Thus, this cytokine production profile also reflects the highly differentiated nature of the residual cell population. Overall, these studies provide new information on the trafficking of naive/mature and Th1/Th2 T cell populations in this disease model.

Adult↗
PubMed1993

Acute jejunal ileus in intestinal lymphangiectasia.

A 26-year-old patient presented with epigastric pain of sudden onset and severe puffy swelling of both legs and forearms. An irregularly shaped nodular filling defect on selective jejunal films, severe hypoproteinemia, low IgG concentration, and lymphopenia were suggestive of primary intestinal lymphangiectasia with protein-losing enteropathy, and the patient was placed on a low-fat diet with medium-chain triglycerides. This initially improved his condition, but some weeks later he developed obstructive ileus of the small intestine. On laparotomy yellowish to whitish deposits were found to be present in some segments of the small intestine and a fist-sized mass 100 cm distal to the duodenojejunal flexure was resected without complications. Histologically, the submucosal lymphatics were dilated, and the jejunal wall showed extensive pseudocystic, intramural submucosal lymph edema with secondary bleeding and tight stenosis of the jejunal lumen. During the 14-month follow-up time after discharge the patient has been asymptomatic and working, on no treatment other than a low-fat diet with medium-chain triglycerides.

Acute Disease↗
PubMed1976

Comparative, immunological studies on lymphangiectasia of the small intestine revealed in protein losing gastroenteropathy and Behçet's disease.

Lymphangiectasia of the small intestine was demonstrated in 9 to 18 cases with protein losing gastroenteropathy and in 10 of 26 cases with Behçhet's disease. Protein losing gastroenteropathy was compared to Behçet's disease in view of immunological aspects. Immunoglobulin containing cells in the jejunal mucosa of protein losing gastroenteropathy were decreased, whereas Behçet's disease had normal or increased distribution. This suggested that suggested that immunoglobulin may be lost into the intestinal lumen or that production of those cells may be inhibited in protein losing gastroenteropathy. Decreased DNCB skin reaction and impaired blastoid transformation of peripheral lymphocytes in both of two diseases suggested that there should be immunological defect in those diseases. However, in Behçet's disease, investigation of the thymus disclosed hyperplasia and the presence of lymphoid follicle which does not appear in normal state. In conclusion, protein losing gastroenteropathy is in immunological deficiency state due to the congenital or acquired disorders of lymphatics which implies abnormal protein loss, decreased immunoglobulin containing cells in the jejunal mucosa and decreased sensitivity. On the contrary, in Behçet's disease hyperimmune state is present based on thymic hyperplasia.

Adult↗
PubMed1994

Intestinal lymphangiectasia: value of double-contrast radiographic study.

Intestinal lymphangiectasia is a disorder presenting as enteric protein loss through the dilated lymphatics without mucosal ulceration. To determine the double-contrast radiographic features and to assess the significance of them, five patients with intestinal lymphangiectasia were examined using single- and double-contrast small intestinal studies. The double-contrast examinations demonstrated clearly the main radiographic findings of smooth nodular protrusions, thickening of the mucosal folds, with no evidence of mucosal ulceration. Compared with the single-contrast study, smooth nodular protrusions were seen more often and in more widespread segments, particularly in the duodenum, on double-contrast study. Thickening of the mucosal folds was revealed similarly by both methods. Double-contrast study appears to be worthwhile to demonstrate the characteristic radiographic findings of this disease.

Adolescent↗
PubMed1986

Secretory diarrhea with protein-losing enteropathy, enterocolitis cystica superficialis, intestinal lymphangiectasia, and congenital hepatic fibrosis: a new syndrome.

Four infants had noninfectious intractable diarrhea, vomiting, anasarca, hepatomegaly, hypoglycemia, and malnutrition within the first 3 months of life. Their parents originated from the same Northeastern part of Quebec, and consanguinity was found in two kindreds. Diarrhea was secretory in three infants (mean stool volume 87 ml/kg/day, Na+ 108 mEq/L, Cl- 85 mEq/L). Hypoalbuminemia (mean 2.0 gm/dl), present in all infants, appeared to be secondary to a protein-losing enteropathy, which was documented in two infants. Histologic examination of the upper small intestine showed only mild to moderate villous atrophy. The remarkable findings were those of cystic dilation of the crypts and acute inflammation of crypts and lamina propria, all of which were most prominent in the colon and terminal ileum; the changes were progressive over time. Mild lymphangiectasia was found in all of the patients. Congenital hepatic fibrosis, present in all, was associated in one patient with a nonfunctional multicystic kidney. Prolonged total parenteral nutrition, intravenously administered albumin, antisecretory agents, and antibiotics were unsuccessful in controlling the disease. Although a total colectomy was followed by a temporary decrease in stool output and normalization of serum albumin concentration in one infant, the patients died between 4 and 21 months of age.

Anti-Bacterial Agents↗
PubMed2006

Acquired intestinal lymphangiectasia successfully treated with a low-fat and medium-chain triacylglycerol-enriched diet in a patient with liver transplantation.

Intestinal lymphangiectasia is defined as a dilatation of small bowel lymphatic capillaries and a loss of lymph into the bowel lumen. Clinically it is characterized by hypoproteinaemia and oedema. We present here a case of protein-losing enteropathy due to intestinal lymphangiectasia after liver transplantation in a 57-year-old man who was transplanted for hepatitis C virus. Four years after liver transplantation, the patient developed hypoalbuminaemia and ascites associated with recurrence of cirrhosis. The sudden fall in serum albumin led us to look for a cause of reduction other than or in addition to cirrhosis. Duodenal biopsies showed tall villi with dilated lymphatic vessels and widening of the villi caused by oedema, demonstrating intestinal lymphangiectasia. In this case a low-fat diet supplemented with medium-chain triacylglycerols achieved an early clinical improvement with increased serum albumin levels and ascites disappearance. Intestinal lymphangiectasia should be suspected in liver-transplanted patients developing hypoproteinaemia and hypoalbuminaemia after the recurrence of cirrhosis.

Diet, Fat-Restricted↗
PubMed1983

Macroglobulinaemia and intestinal lymphangiectasia: a rare association.

Two cases in which macroglobulinaemia was associated with intestinal lymphangiectasia are recorded. Immunoperoxidase stains demonstrated a high content of monoclonal IgM in the intestinal lymph. The seven previously recorded examples of this association are reviewed. It is concluded that the concurrence of these two conditions is not merely fortuitous, and that increased viscosity of the lymph consequent on its high IgM content may be important in the pathogenesis of the intestinal lymphangiectasia.

Aged↗
PubMed1984

Lipogranulomatous lymphangitis in canine intestinal lymphangiectasia.

Lipogranulomatous lymphangitis of the intestine occurred in four dogs with intestinal lymphangiectasia. All four presented with chronic diarrhea; three had ascites and two had hypoalbuminemia. Lipogranulomas appeared in lymphatics, often at the point of mesenteric attachment, of small intestine, ileum, or ileum and colon. Mesenteric lymphatics were obstructed and villous lacteals were distended. Mesenteric lymph nodes of one dog contained large lipid spaces and that same animal had a solitary subcapsular lipogranuloma of the liver. This disorder is one of several that result in protein-losing enteropathy in dogs.

Animals↗
PubMed1986

Sonography of intestinal lymphangiectasia.

Real-time ultrasonography was used to evaluate three pediatric patients with biopsy-proven intestinal lymphangiectasia. The sonographic findings were ascites, diffuse bowel wall thickening, mesenteric edema, dilated mesenteric lymphatics, and thickened walls of the gallbladder and urinary bladder. Recognition of the sonographic pattern of intestinal lymphangiectasia is useful since ultrasonography is often the first imaging examination performed in children with abdominal disorders.

Ascites↗