La maladie de Waldmann (lymphangiectasies intestinales primitives) est responsable d’une entéropathie exsudative par fuite de la lymphe dans la lumière. Very rare familial forms of Waldmann’s disease have been reported [1,4]. Go to: .. Vignes S. Lymphangiectasies intestinales primitives (maladie de Waldmann) . Publication – Article. Lymphangiectasies intestinales primitives (maladie de Waldmann). La Revue de Médecine Interne, 39(7), ,

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Primary intestinal lymphangiectasia (Waldmann’s disease)

The need for dietary control maladle to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. Waldmanm findings may show dilation of the intestinal loops, regular and diffuse thickening of the walls, plical hypertrophy and severe mesenteric edema and, in some cases, ascites [ 3031 ].

Enteral nutrition as a primary therapy for intestinal lymphangiectasia: Observations by magnifying endoscopy. Among 50 PIL patients reviewed, 3 had malignant lymphomas, that arose 3 to 25 years after the initial diagnosis [ 60 ].

The condition can also be a result of other illnesses such as constrictive pericarditis and pancreatitis. A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: They can be used after or in combination with a low-fat diet associated with MCT supplementation.


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Remission of malabsorption in congenital intestinal lymphangiectasia following chemotherapy for lymphoma. Albumin infusion is a symptomatic treatment proposed in patients with important serous effusion or uncomfortable lower limb edema. Prognosis and long-term outcome PIL is a chronic debilitating disorder requiring constraining long-term dietary control based on a low-fat regimen associated with supplementary MCT.

Only one case of a severe infection with group G streptococcal empyema was reported [ 67 ] and another had cryptococcal meningitis [ 68 ].

The mechanism of action of the somatostatin analog on the gastrointestinal tract remains unclear. J Med Assoc Thai.

These two conditions deteriorate the quality of life difficulty to put on shoes, unattractive aspect of leg. Indirect biological abnormalities are suggesting of PIL, such as hypoproteinemia, hypoalbuminemia, hypogammaglobulinemia with low IgG, IgA and IgM levels or lymphocytopenia. Primary intestinal lymphangiectasia PIL is a rare disorder characterized by ealdmann intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.

Maladie de Waldmann familiale – EM|consulte

Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple’s disease, Crohn’s disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Outline Masquer le plan. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with ensuing lymph loss.


Long-term PIL monitoring essentially concerns its predominant clinical manifestation edema. At present, lymphoscintigraphy is not a routine and useful methodology for PIL diagnosis.

Intestinal lymphangiectasia in systemic sclerosis. Orphanet J Rare Dis. Edema is the consequence of hypoprotidemia with decreased oncotic pressure. Histopathologic examination of a nodule confirmed the diagnosis of Kimura’s disease, which was associated with necrotic extracapillary glomerulonephritis.

Lymphoscintigraphy is an effective tool for identifying abnormal lymphatic tree in the upper or lower limb and also to confirm limb lymphedema, when the limb images are abnormal. Prognosis The prognosis varies. Clinical manifestations include edema related to hypoalbuminemia protein-losing enteropathyasthenia, diarrhea, lymphedema and failure to thrive in children. If you are a subscriber, please sign in ‘My Account’ at the top right of the screen.

Journal page Archives Contents list. Differential diagnosis The differential diagnosis includes enteropathy-associated T-cell lymphoma, Whipple disease, Crohn disease, sarcoidosis, tuberculosis with extrapulmonary involvementsystemic sclerosis see these termsand constrictive pericarditis. Lymphatic vessel disease I88—I89 ,