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. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur

Human native kappa opioid receptor functions not predicted by recombinant receptors: Implications for drug design

International audienc

Radial and time resolved measurement of electron density in a copper vapour laser

International audienc

Accessibility measure for haemophilia patients: study of Anti-Haemophilic Factors availability in the Rhône-Alpes county (France)

International audienc

Le facteur VII active recombinant hors Autorisation de mise sur le marche a l'heure de la tarification a l'activite: enquete de pratique. [Off label use of recombinant activated factor VII: a practice survey]

OBJECTIVES: To evaluate the impact of local recommendations on off label use of recombinant activated factor VII. TYPE OF STUDY: Observational, retrospective. MATERIAL AND METHODS: Recommendations have been diffused by a local working group in 2004, concerning seven groups of indications (traumatology, cardiac, hepatic and various surgeries, obstetrics, neurology and haematology). Conformity to recommendations was studied according to 4 axes: respect of prescribing procedure, indication, posology, and pre ones required with the administration. RESULTS: There were thirty-one uses from 2004 to 2006. Thirty patients presented a massive haemorrhage (blood products before administration of recombinant activated factor VII expressed in median [min-max]: red blood cell unit=15 [0-37], fresh frozen plasma=11 [0-21]). Gravity score at the admission: SAPSII=55+/-22 (average+/-SD), 87% patients presented haemorrhagic shock. Prescribing procedure was in conformity with recommendations in 45%, indications were correct in 74%, posology in 55%, and 16% of prescriptions were completely conform. CONCLUSION: With the tariffing to the activity reform, the reimbursement of off label use of expensive molecules will depend on the respect of the evidence-based medicine. According to this study, the observance of local recommendations was insufficient. Rationalizing prescriptions for practice improvement seemed to be difficult and challenging

Studies on Halo Formation in a Long Magnetic Quadrupole FODO Channel First Experimental Results

A study is progress to prepare an experiment on the transport of an intense proton beam, with high optical qualities, through a periodic magnetic quadrupole FODO channel (29 periods). It is intended to provide a better understanding of how halo develops and can be controlled as a crucial issue for the design of high-intensity linear accelerators. Simulations were done showing that this experiment can be performed with a high-brightness proton, beam produced by a duoplasmatron source, with an energy of 500 keV and a current of tens of mA. Beam pulse length will be less than 1 ms and normalized emittance better than 1.p mm.mrad (90% of total beam).Thus, transverse tune can be sufficiently depressed in the transport channel to allow instabilities to grow and halo to develop or stay under control