Intestinal Lymphangiectasia Mechanism of Enteric Loss of Plasma-Protein and Fat

Mistilis, Steven P.; Skyring, A. P.; Stephen, D.D.

doi:10.1016/s0140-6736(65)91657-0

Cited by 98 publications

(43 citation statements)

References 13 publications

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“…Evidence for this mechanism of lymphocytopenia includes the following: (a) all of the proteinlosing gastroenteropathies associated with lymphocytopenia have demonstrable lymphatic abnormalities (10)(11)(12), while the nonlymnphocytopenic protein-losing gastroenteropathies do not (13)(14)(15); (b) in the lymphocytopenic protein-losing gastroenteropathies in which the protein loss is reversible, the lymphocytopenia is also reversible (11,(16)(17) ; (c) lymphatic fluid has been shown to leak directly into the bowel lumen in some patients with intestinal lymphangiectasia, either by aspiration of chyle from the duodenum (18,19) or by the appearance in the bowel lumen of contrast medium administered during lymphangiography (18,20) Thus it appears that patients with intestinal lymphangiectasia do lose lymphocytes as well as serum proteins into the gastrointestinal tract, most probably as part of the bulk loss of lymph fluid containing both cellular elements and serum proteins. The in vitro studies of lymphocyte function reported here were undertaken to assess whether the cellular immune defect observed in these patients is simply the result of the reduced number of peripheral blood lymphocytes.…”

Section: Resultsmentioning

confidence: 99%

Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man

Weiden¹,

Blaese²,

Strober³

et al. 1972

J. Clin. Invest.

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Section: Resultsmentioning

confidence: 99%

Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man

Weiden¹,

Blaese²,

Strober³

et al. 1972

J. Clin. Invest.

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“…(c) Finally, and most significantly, lymphatic fluid has been shown to leak directly into the bowel lumen in some patients with intestinal lymphangiectasia, i.e. chyle has been aspirated directly from the duodenum in some cases (32,33), and radiopaque lymphangiogram dye entered the bowel lumen after injection into the lymphatics of the foot in other cases (32,34). All these facts suggest that lymphocyte-rich lymph fluid is lost into the gastrointestinal tract, and this, in turn, results from the underlying intestinal lymphatic abnormality.…”

Section: Discussionmentioning

confidence: 99%

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Strober¹,

Wochner²,

Carbone³

et al. 1967

J. Clin. Invest.

188

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Abstract. 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/mm8 in contrast to 2500 + 600/ mm3 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.

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“…Excessive albumin secretion into the bowel may occur through a denuded mucosa or because of lymphatic abnormalities (58)(59)(60). How albumin normally enters the gastrointestinal tract is poorlyunderstood.…”

Section: Discussionmentioning

confidence: 99%

Use of 125I- and 51Cr-Labeled Albumin for the Measurement of Gastrointestinal and Total Albumin Catabolism*

Kerr¹,

Bois²,

Holt³

1967

J. Clin. Invest.

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Abstract. A method for the simultaneous measurement of gastrointestinal protein loss and total albumin turnover entailing the use of a combination of 125iodine-and 51chromium-labeled albumin is described. Albumin turnover was calculated by the measurement of albumin-125I plasma decay and cumulative urinary excretion, and the results obtained agreed closely with previous studies utilizing albumin-'31I. Gastrointestinal catabolism was calculated from the rate of fecal excretion of 51Cr and the specific activity of plasma albumin-51Cr, and these data were related to the calculated albumin turnover results. During the period of 6-14 days after administration, the ratio of specific activties of albumin-125I and -51Cr in plasma and in extravascular spaces or gastric and biliary secretions remained almost identical. Fecal excretion of 51Cr was also quite stable at this time. In six normal subjects gastrointestinal catabolism accounted for less than 10% of total albumin catabolism. Excessive gastrointestinal protein losses did not contribute to the low serum albumin in three patients with cirrhosis or in two adults with the nephrotic syndrome. Multiple mechanisms leading to hypoalbuminemia were demonstrated in other subjects with a variety of gastrointestinal disorders.

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Intestinal Lymphangiectasia Mechanism of Enteric Loss of Plasma-Protein and Fat

Cited by 98 publications

References 13 publications

Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man

Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Use of 125I- and 51Cr-Labeled Albumin for the Measurement of Gastrointestinal and Total Albumin Catabolism*

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