Protein-losing gastroenteropathy: Concepts derived from lymphangiography

Lymphangiograms in 2 patients with protein-losing gastroenteropathy showed abnormalities of both abdominal and lower-extremity lymphatics.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/44359/1/10620_2005_Article_BF02237631.pd

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

Hepatitis B antigen and antibody in active chronic hepatitis and other liver diseases in Australia - A multicenter collaborative study

In a multicenter cooperative study, sera from 85 patients with active chronic hepatitis (ACH) were examined for the presence of hepatitis B (Australia) antigen (HBAg) by radioimmunoassay (RIA) and antibody to HBAg (anti-HBAg) by RIA and passive hemagglutination (PHA), the most sensitive currently available techniques. In addition, sera from 83 patients with other liver diseases, 98 other hospital patients, and 67 healthy controls were tested. HBAg was detected in 3 of the 85 patients (four percent) with ACH. In a further 3 patients (four percent) anti-HBAg was detected. Thus, 6 patients with ACH (seven percent) had evidence of present or prior infection with the hepatitis B virus (HBV). HBAg was also detected in 7 of the patients with other liver diseases, 2 of the other hospital patients, and none of the healthy controls. Anti-HBAg was detected in 17 of the non-ACH subjects. These results indicate that neither persistent nor prior self-limited infection with HBV is a major factor in the pathogenesis of ACH in Australia