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Intestinal Lymphangiectasia: Metabolic Studies

Intestinal Lymphangiectasia: Metabolic Studies Abstract INTESTINAL lymphangiectasia is associated with enteric loss of protein and malabsorption of fat.1-6 As a consequence, children with this disease fail to thrive,7,8 and display steatorrhea and hypoproteinemia with secondary edema of variable extent. Although the exact pathogenesis of this condition is as yet not completely understood, the response of a few patients with this syndrome to diet in which the fat is primarily medium-chain-triglycerides (MCT) has been remarkable.1,7-11 The purpose of this report is to present metabolic data obtained in a 15-year-old boy with isolated intestinal lymphangiectasia prior to and during MCT therapy. Report of a Case This white boy (SMH, 63-91-58), was the product of a normal full-term pregnancy. Delivery and neonatal period were uneventful. His growth, development, and general health were normal until age 4 years. At that time he underwent surgery for the repair of a right inguinal hernia. During the operation, milky References 1. Holt, P.R.: Dietary Treatment of Protein Loss in Intestinal Lymphangiectasia , Pediatrics 34:629-635 ( (Nov) ) 1964. 2. Herskovic, T., et al: Hypoproteinemia in Intestinal Lymphangiectasia , Pediatrics 40:345-353 ( (Sept) ) 1967. 3. Macromolecular Aspects of Protein Absorption and Excretion in the Mammalian Intestine: "The Specific Syndrome of Intestinal Lymphangiectasia": Report of the Fiftieth Ross Conference on Pediatric Research, Columbus, Ohio: Ross Laboratories, 1965, pp 94-105. 4. Mistilis, S.P.; Skyring, A.P.; and Stephen, D.D.: Intestinal Lymphangiectasia Mechanism of Enteric Loss of Plasma Protein and Fat , Lancet 1:77-80 ( (Jan 9) ) 1965.Crossref 5. Pomerantz, M., and Waldmann, T.A.: Systemic Lymphatic Abnormalities Associated With Gastrointestinal Protein Loss Secondary to Intestinal Lymphangiectasia , Gastroenterology 45:703-711 ( (Dec) ) 1963. 6. Waldmann, T.A.: Protein-Losing Enteropathy , Gastroenterology 50:422-443 ( (March) ) 1966. 7. Vescia, F.G., and Davis, J.H.: Treatment of Intestinal Lymphangiectasia , Gastroenterology 48: 287 ( (Feb) ) 1965. 8. Jeffries, G.H.; Chapman, A; and Sleisenger, M.H.: Low-Fat Diet in Intestinal Lymphangiectasia: Its Effect on Albumin Metabolism , New Eng J Med 270:761-766 ( (April 9) ) 1964.Crossref 9. Greenberger, N.J.; Ruppert, R.D.; and Tzagournis, M.: Use of Medium Chain Triglycerides in Malabsorption , Ann Intern Med 66:727-734 ( (April) ) 1967.Crossref 10. Yssing, M.; Jensen, H.; and Jarnum, S.: Dietary Treatment of Protein-Losing Enteropathy , Acta Paediat Scand 56:173-181 ( (March) ) 1967.Crossref 11. Holt, P.R.; Hashim, S.; and Van Itallie, T.B.: Treatment of Malabsorption Syndrome and Exudative Enteropathy With Synthetic Medium Chain Triglycerides , Amer J Gastroent 43:549-559 ( (June) ) 1965. 12. Strober, W., et al: Intestinal Lymphangiectasia: A Protein-Losing Enteropathy With Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection , J Clin Invest 46: 1643-1656, 1967.Crossref 13. Jarnum, S.: Fecal 131I-Output After Intravenous Injection of 131I-Labelled Human Serum Albumin In Normo- and Hypoproteinemic Subjects , J Clin Lab Invest 13:462-475 (No. (3) ) 1961.Crossref 14. Forbes, G.B., and Hursh, J.B.: Age and Sex Trends in Lean Body Mass Calculated From K40 Measurements: With a Note on the Theoretical Basis for the Procedure , Ann NY Acad Sci 110:255 ( (Sept 26) ) 1963.Crossref 15. Greulich, W.W., and Pyle, S.I.: Radiographic Development of the Hand and Wrist , ed 2, California: Stanford University Press, 1959. 16. Craven, C.E., et al: Congenital Chylous Ascites: Lymphangiographic Demonstration of Obstruction of the Cisterna Chyli and Chylous Reflex Into the Peritoneal Space and Small Intestine , J Pediat 70:340-344 ( (March) ) 1967.Crossref 17. Hargrove, M.D., Jr.; Mathews, W.R.; McIntyre, P.A.: Intestinal Lymphangiectasia With Response to Corticosteroids , Arch Intern Med 119:206-210 ( (Feb) ) 1967.Crossref 18. Mistilis, S.P., and Skyring, A.P.: Intestinal Lymphangiectasia , Amer J Med 40:634-641 ( (April) ) 1966.Crossref 19. Waldmann, T.A., et al: The Role of the Gastrointestinal System in "Idiopathic Hypoproteinemia," Gastroenterology 41:197-207 ( (Sept) ) 1961. 20. Forbes, G.B.: Chylothorax in Infancy: Observations on the Absorption of Vitamins A and D and on the Intravenous Replacement of Aspirated Chyle , J Pediat 25:191 ( (Sept) ) 1944.Crossref 21. Wiseman, G.: Absorption From the Intestine , New York: Academic Press, 1964. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1969 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1969.02100030180010
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Abstract

Abstract INTESTINAL lymphangiectasia is associated with enteric loss of protein and malabsorption of fat.1-6 As a consequence, children with this disease fail to thrive,7,8 and display steatorrhea and hypoproteinemia with secondary edema of variable extent. Although the exact pathogenesis of this condition is as yet not completely understood, the response of a few patients with this syndrome to diet in which the fat is primarily medium-chain-triglycerides (MCT) has been remarkable.1,7-11 The purpose of this report is to present metabolic data obtained in a 15-year-old boy with isolated intestinal lymphangiectasia prior to and during MCT therapy. Report of a Case This white boy (SMH, 63-91-58), was the product of a normal full-term pregnancy. Delivery and neonatal period were uneventful. His growth, development, and general health were normal until age 4 years. At that time he underwent surgery for the repair of a right inguinal hernia. During the operation, milky References 1. Holt, P.R.: Dietary Treatment of Protein Loss in Intestinal Lymphangiectasia , Pediatrics 34:629-635 ( (Nov) ) 1964. 2. Herskovic, T., et al: Hypoproteinemia in Intestinal Lymphangiectasia , Pediatrics 40:345-353 ( (Sept) ) 1967. 3. Macromolecular Aspects of Protein Absorption and Excretion in the Mammalian Intestine: "The Specific Syndrome of Intestinal Lymphangiectasia": Report of the Fiftieth Ross Conference on Pediatric Research, Columbus, Ohio: Ross Laboratories, 1965, pp 94-105. 4. Mistilis, S.P.; Skyring, A.P.; and Stephen, D.D.: Intestinal Lymphangiectasia Mechanism of Enteric Loss of Plasma Protein and Fat , Lancet 1:77-80 ( (Jan 9) ) 1965.Crossref 5. Pomerantz, M., and Waldmann, T.A.: Systemic Lymphatic Abnormalities Associated With Gastrointestinal Protein Loss Secondary to Intestinal Lymphangiectasia , Gastroenterology 45:703-711 ( (Dec) ) 1963. 6. Waldmann, T.A.: Protein-Losing Enteropathy , Gastroenterology 50:422-443 ( (March) ) 1966. 7. Vescia, F.G., and Davis, J.H.: Treatment of Intestinal Lymphangiectasia , Gastroenterology 48: 287 ( (Feb) ) 1965. 8. Jeffries, G.H.; Chapman, A; and Sleisenger, M.H.: Low-Fat Diet in Intestinal Lymphangiectasia: Its Effect on Albumin Metabolism , New Eng J Med 270:761-766 ( (April 9) ) 1964.Crossref 9. Greenberger, N.J.; Ruppert, R.D.; and Tzagournis, M.: Use of Medium Chain Triglycerides in Malabsorption , Ann Intern Med 66:727-734 ( (April) ) 1967.Crossref 10. Yssing, M.; Jensen, H.; and Jarnum, S.: Dietary Treatment of Protein-Losing Enteropathy , Acta Paediat Scand 56:173-181 ( (March) ) 1967.Crossref 11. Holt, P.R.; Hashim, S.; and Van Itallie, T.B.: Treatment of Malabsorption Syndrome and Exudative Enteropathy With Synthetic Medium Chain Triglycerides , Amer J Gastroent 43:549-559 ( (June) ) 1965. 12. Strober, W., et al: Intestinal Lymphangiectasia: A Protein-Losing Enteropathy With Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection , J Clin Invest 46: 1643-1656, 1967.Crossref 13. Jarnum, S.: Fecal 131I-Output After Intravenous Injection of 131I-Labelled Human Serum Albumin In Normo- and Hypoproteinemic Subjects , J Clin Lab Invest 13:462-475 (No. (3) ) 1961.Crossref 14. Forbes, G.B., and Hursh, J.B.: Age and Sex Trends in Lean Body Mass Calculated From K40 Measurements: With a Note on the Theoretical Basis for the Procedure , Ann NY Acad Sci 110:255 ( (Sept 26) ) 1963.Crossref 15. Greulich, W.W., and Pyle, S.I.: Radiographic Development of the Hand and Wrist , ed 2, California: Stanford University Press, 1959. 16. Craven, C.E., et al: Congenital Chylous Ascites: Lymphangiographic Demonstration of Obstruction of the Cisterna Chyli and Chylous Reflex Into the Peritoneal Space and Small Intestine , J Pediat 70:340-344 ( (March) ) 1967.Crossref 17. Hargrove, M.D., Jr.; Mathews, W.R.; McIntyre, P.A.: Intestinal Lymphangiectasia With Response to Corticosteroids , Arch Intern Med 119:206-210 ( (Feb) ) 1967.Crossref 18. Mistilis, S.P., and Skyring, A.P.: Intestinal Lymphangiectasia , Amer J Med 40:634-641 ( (April) ) 1966.Crossref 19. Waldmann, T.A., et al: The Role of the Gastrointestinal System in "Idiopathic Hypoproteinemia," Gastroenterology 41:197-207 ( (Sept) ) 1961. 20. Forbes, G.B.: Chylothorax in Infancy: Observations on the Absorption of Vitamins A and D and on the Intravenous Replacement of Aspirated Chyle , J Pediat 25:191 ( (Sept) ) 1944.Crossref 21. Wiseman, G.: Absorption From the Intestine , New York: Academic Press, 1964.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Feb 1, 1969

References