We report the observation of a mother and her daughter who presented edema, hypoprotidemia and lymphopenia due to protein-losing enteropathy. Radiological, endoscopic and histological investigations revealed the diagnosis of primary intestinal lymphangiectasis or Waldmann's disease. Dietary treatment with middle chained triglycerides was effective. Familial cases are rarely described.
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CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease.
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Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.
Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown.
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Primary intestinal lymphangiectasia (Waldmann's disease).
Orphanet J Rare Dis
February 2008
Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown.
View Article and Find Full Text PDF[Familial Waldmann's disease].
Ann Med Interne (Paris)
October 2000
Fédération de Médecine Interne, Maladies Infectieuses et Pathologies Tropicales, Hôpital Saint-André, 1, rue Jean Burguet, 33075 Bordeaux Cedex.
We report the observation of a mother and her daughter who presented edema, hypoprotidemia and lymphopenia due to protein-losing enteropathy. Radiological, endoscopic and histological investigations revealed the diagnosis of primary intestinal lymphangiectasis or Waldmann's disease. Dietary treatment with middle chained triglycerides was effective.
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