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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Daily fecal excretion of I was twice the highest value obtained in controls. As per the Law relating to information storage and personal integrity, you have the right to oppose art 26 maladue that lawaccess art 34 of that law and rectify art 36 waldnann that law your personal data.

Resolution of longstanding protein-losing enteropathy in a patient with intestinal lymphangiectasia after treatment for malignant lymphoma. Intestinal lymphatics may be dilated in many villi or only a few. To our knowledge, this is the first case of Kimura’s disease naladie with extracapillary glomerulonephritis. The mechanism of hypoproteinemia in patients with regional enteritis and ulcerative colitis.

Protein-losing enteropathy and malabsorption in regional enteritis: The total exchangeable albumin pool, assessed with radio-labeled I-albumin, was low in all patients. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. 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.


Open in a separate window. Severe hypoalbuminaemia in a aaldmann lupus erythematosus-like patient.

Primary intestinal lymphangiectasia (Waldmann’s disease)

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. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia.

Contact Help Who are we? PIL outcome may be severe even life-threatening when malignant complications or serous effusion s occur. Bilateral breast edema in intestinal lymphangiectasia [letter] Breast J. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with ensuing lymph loss.

A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. Very rare familial cases of PIL have been reported. J Paediatr Child Health.

Histological examination of duodenum-jejunum and ileum biopsies confirms the presence of lacteal juice, dilated mucosal from moderate to severe and submucosal lymphatic vessels and also in the serosa with polyclonal normal plasma cells.

PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Maladie de Waldmann familiale.

Most cases involve young Asian males. Furthermore, somatostatin inhibits thoracic lymph flow in dogs [ 79 ].


J Med Assoc Thai. Disease name and synonyms Primary intestinal lymphangiectasia PIL. The interval from PIL diagnosis to lymphoma diagnosis was even 39 and 40 years in a study [ 61 ]. The documents contained in this web site are presented for information purposes only.

Primary intestinal lymphangiectasia PIL is a rare intestinal disease characterized by dilated intestinal lacteals which cause lymph leakage into the small bowel lumen.

Maladie de Waldmann familiale – EM|consulte

It is an uncommon disorder characterized by the replacement of hematopoietic cells and adipocytes by amorphous extracellular material composed malavie acid mucopolysaccharides. You can move this window by clicking on the headline. Other treatments In the literature, other treatments have been proposed to treat PIL.

PIL outcome may be severe even life-threatening when malignant complications lymphoma or serous effusion s pleural, pericardic occur. In addition, the therapy resulted in the disappearance endoscopically observed duodenal lesions [ 73 malaie.

Gastro-entéropathies exsudatives – EM|consulte

Outline Masquer le plan. Fatigue, abdominal pain, weight loss, inability to gain weight, failure to thrive in children, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present.

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