An unusual presentation of intestinal lymphangiectasia
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Keywords:
Lymphangiectasia
Presentation (obstetrics)
Chylous Ascites
Iliac fossa
Protein losing enteropathy
Intestinal lymphangiectasia is a rare cause of protein losing enteropathy.1 CMV infection is infrequently implicated in the pathogenesis of protein losing enteropathy and when this happens, the host is usually immunocompromised. 2-6 We present a unique case of intestinal lymphangiectasia in an immunocompetent adult, caused by CMV and EBV co-infection.
Protein losing enteropathy
Lymphangiectasia
Pathogenesis
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Protein losing enteropathy
Lymphangiectasia
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SUMMARY Using intravenous administration of radioactive-labeled chromic chloride, which binds to plasma proteins, protein-losing enteropathy was documented in 6 macaques ( Macaca mulatta and Macaca arctoides ) with progressive weight loss and hypoalbuminemia. Four clinically normal macaques lost 0.5%±0.1% of the label in feces over 4 days, compared with the diseased macaques, which lost from 1.0%–14.2% of the dose during the same period. Lesions in the small intestine (lymphangiectasia, amyloidosis of lamina propria, mast cell proliferation in the tunica muscularis and submucosa, and goblet cell hyperplasia) were associated with the protein-losing enteropathy.
Protein losing enteropathy
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Protein losing enteropathy
Lymphangiectasia
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Protein‐losing enteropathy is a primary or secondary manifestation of a group of conditions, and etiologies which are broadly divisible into those with mucosal injury on the basis of inflammatory and ulcerative conditions, mucosal injury without erosions or ulcerations, and lymphatic abnormalities. We describe the first case of protein‐losing enteropathy in a pediatric patient, with severe skeletal dysplasia consistent with thanatophoric dysplasia type I and DNA analysis that revealed a c.1949A>T (p.Lys650Met) in exon 15 of the FGFR3 gene. She presented with protein‐losing enteropathy in her 6th month. Post‐mortem examination revealed lymphangiectasia in the small intestine. To our knowledge, this is the first report of intestinal lymphangiectasia as a complication of skeletal dysplasia resulting in severe protein‐losing enteropathy. © 2016 Wiley Periodicals, Inc.
Protein losing enteropathy
Lymphangiectasia
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Protein losing enteropathy
Lymphangiectasia
Dilation (metric space)
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The authors report the case of a 56 yr old man suffering from oedema of the inferior limbs. This oedema was secondary to a protein-loosing enteropathy of lymphatic origin. Histological examination revealed intestinal lymphangiectasia. After reviewing all the cases of secondary intestinal lymphangiectasia, the diagnosis of primary lymphangiectasia was proposed. Middle chain triglycerides administration induced clinical and biological improvement.
Protein losing enteropathy
Lymphangiectasia
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Intestinal lymphangiectasia is a disease characterized by dilated intestinal lymphatics, protein-losing enteropathy, hypoalbuminemia, and edema. The immunologic status of 18 patients with intestinal lymphangiectasia was studied. Concentrations of IgG, IgA, and IgM were measured by immune precipitation and metabolism of these three immunoglobulins was studied using purified radioiodinated proteins. The serum concentration and total body pool of each immunoglobin were greatly reduced. The fraction of the intravascular protein pool catabolized per day was increased to 34% for IgG, 59% for IgA, and 66% for IgM; these are in contrast with control values of 7%, 28%, and 17%, respectively. Synthetic rates of the immunoglobulins were normal or slightly increased. Primary circulating antibody response was tested in five patients with Vi and tularemia antigens. Titers elicited in patients with the Vi antigen were significantly lower than those seen in a control group, whereas no difference was seen between patient and control responses to the tularemia antigen. Lymphocytopenia was noted in patients with intestinal lymphangiectasia. The mean circulating lymphocyte count was 710 +/- 340/mm(3) in contrast to 2500 +/- 600/mm(3) in controls. Cellular hypersensitivity was studied with skin tests and skin grafts. 91% of normal individuals reacted to at least one of the four skin test antigens: purified protein derivative, mumps, Trichophyton, and Candida albicans; in contrast, only 17% of patients with intestinal lymphangiectasia had a positive reaction. Each of three patients tested with dinitrochlorobenzene had a negative reaction. Finally, all four patients who received skin homografts have retained these grafts for at least 12 months. The immunological disorders in patients with intestinal lymphangiectasia appear to result from loss of immunoglobulins and lymphocytes into the gastrointestinal tract secondary to disorders of lymphatic channels. Lymphocyte depletion then leads to skin anergy and impaired homograft rejection.
Protein losing enteropathy
Hypoalbuminemia
Hypogammaglobulinemia
Lymphangiectasia
Lymphocytopenia
Hypoproteinemia
Chyle
Immunoglobulin A
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Primary intestinal lymphangiectasia is a rare disorder associated with protein-losing enteropathy. The main manifestations are those resulting from hypoalbuminemia. Diagnosis requires the typical endoscopic image of intestinal lymphangiectasia and increased 24-hour fecal alpha-1-antitrypsin clearance. Treatment is basically dietary.
Protein losing enteropathy
Hypoalbuminemia
Lymphangiectasia
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This is a report of 3 Chinese children with intestinal lymphangiectasia in Malaysia. Two children responded to a low fat diet and medium-chain triglyceride supplement. The third child has recurrent chylous ascites. None of the children has recurrent infections despite low CD4+ cells and low levels of IgG and IgA. Intestinal lymphangiectasia is a rare congenital disorder of the mesenteric lymphatic that leads to the obstruction of the lymphatics of the intestine and protein losing enteropathy. Restriction of dietary fat intake will usually result in remission. Recurrent chylous ascites is a problem and management can be difficult.
Chylous Ascites
Protein losing enteropathy
Lymphangiectasia
Chylomicron
Chyle
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