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 lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: J Comput Assist Tomogr. CT may be useful to identify localized intestinal lymphangiectasia [ 35 ]. Octreotide may be useful in PIL patients in combination with a low-fat diet.

Their efficacy is variable and insufficiently evaluated. In a recent paper, Hokari et al. Biopsies of the small intestine were examined under the microscope and found various levels of dilatation of the lymph vessels.

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Please review our privacy policy. This article has been cited by other articles in PMC. Differential diagnosis includes constrictive maadie, intestinal lymphoma, Whipple’s disease, Crohn’s disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis.

Remission of malabsorption in congenital intestinal lymphangiectasia following chemotherapy for lymphoma. Protein-losing enteropathy in constrictive pericarditis.

Primary intestinal lymphangiectasia (Waldmann’s disease)

Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. PIL may be suspected at birth or during pregnancy based on ultrasonography images, which can detect fetal ascites or lower limb lymphedema [ 6 ].

It is likely that the absence of fat in the diet prevents engorgement of the intestinal lymphatics with chyle, thereby preventing their rupture with its ensuing protein and T-cell loss.


Differential diagnosis The differential diagnosis is of special importance for subjects suspected of having PIL. Less than cases have been reported worldwide. Albumin infusion is a symptomatic treatment proposed in patients with important serous effusion or uncomfortable lower limb edema. Author information Article notes Copyright and License information Disclaimer. Disease name and synonyms Primary intestinal lymphangiectasia PIL.

Kimura’s disease is a rare and benign chronic inflammatory soft-tissue disorder of unknown origin. Contact Help Who are we?

Support Center Support Center. To our knowledge, this is the first case of Kimura’s disease associated with extracapillary glomerulonephritis. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. If you are a subscriber, please sign in ‘My Account’ fe the top right of the screen.

A year-old Mauritian man was hospitalized for nephrotic syndrome. Very rare familial cases of PIL have been reported. Corticosteroid-responsive intestinal lymphangiectasia secondary to an inflammatory process. The role of the gastrointestinal system in “idiopathic hypoproteinemia”. After a few weeks, this treatment makadie lead to reversal of clinical and biochemical signs albuminemia, immunoglobulin walcmann and lymphocyte counts [ 71 ].

Some secondary causes of intestinal lymphangiectasia have been identified as diseases responsible for anatomical or dynamic alterations of the lymphatic flow. The disease was first reported in by T. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia.

Orphanet: Maladie de Waldmann

They can be used after or in combination with a low-fat diet associated with MCT supplementation. Typically, dde is diffuse, nodular, small bowel wall-thickening and edema, which are a consequence of the dilated lymphatics within the villi, along with some degree of small bowel dilation, with, in few cases, a “halo sign” due to swelling and edema waldmannn 32 – 34 ].

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To date, PIL etiology is unknown. Sarcoidosis and protein losing enteropathy.

It has been shown that octreotide induces short-lasting splanchnic vasoconstriction in healthy volunteers and cirrhotic patients, and inhibits the absorption of triglycerides [ 78 ]. The density of wsldmann varies and their size ranges from mm to cm. Among 50 PIL patients reviewed, 3 dd malignant lymphomas, that arose 3 to 25 years after the initial diagnosis [ 60 ].

In addition, the therapy resulted in the disappearance endoscopically observed duodenal maadie [ 73 ]. Intestinal lymphangiectasia mechanism of enteric loss of plasma protein and fat. The primary causes can be classified into lymphatic leakage due to increased interstitial pressure and increased leakage of protein-rich fluids due to erosive or non-erosive gastrointestinal disorders. Received Jul 26; Accepted Feb Clinical description PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients.

Octreotide in the treatment of intestinal lymphangiectasia.

Maladie de Kimura associée à une glomérulonéphrite extra-capillaire nécrosante – EM|consulte

Axial abdominal CT images are obtained with oral and intravenous contrast medium enhancement. Languages Deutsch Polski Edit links. Intestinal lymphangiectasia in intraabdominal tuberculosis. Etiology and pathogenesis To date, PIL etiology is unknown. Aggressive bowel lymphoma in a patient with intestinal lymphangiectasia and widespread viral warts.

PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease or in patients who follow a mzladie diet. The mechanism of action of the somatostatin analog on the gastrointestinal tract remains unclear.