Hypoproteinemia in intestinal lymphangiectasia. Contribution of albumin "trapping" in the lymphedematous extremity.
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In the Noonan syndrome various anomalies can be demonstrated radiologically. Of particular interest are cardiovascular lesions, such as atypical pulmonary stenosis, hypertrophic cardiomyopathy and the lymphangiectasias. Skeletal anomalies can be found, including retardation of bone maturation and sternal abnormalities. Other anomalies concern the urinary tract (pyeloureteral stenosis) and the digestive system. Radiological diagnostic procedures allow to disclose various elements of this polymalformative syndrome. They should be performed whenever suggestive clinical signs are present, e.g. hypertelorism, gonadal dysgenesis, down-slanting palpebral fissures and shield-like chest.
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Tumors and tumor-like lesions of the duodenum and the Vater's ampulla are discussed in a clinical-pathological conference. Tumors and precancerous conditions are rarely found in the duodenum and the ampulla, nevertheless these areas should be investigated carefully during endoscopy of the upper gastrointestinal tract. About 12-15% of lesions described as tumors or tumor-like lesions are located in the duodenum. Histological findings of biopsy material from this region are the main topic of the conference. Incidence and differential diagnosis of the typical endoscopical and histological findings of the different tumors and tumor-like lesions are described.
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Intestinal lymphangiectasia of obscure etiology is frequent among Norwegian Lundehunds ("puffin-dog"). A study of three Lundehunds with typical symptoms, including wasting, diarrhea and ascites, revealed segmental distention of lymphatics resembling primary lymphangiectasia in man. Atrophy, fusion and balloon-like swelling of villi with occasional rupture of lacteals were seen. Subepithelial fluid accumulation caused bulging of the epithelium. Microvilli showed partial or complete atrophy. The protein loss in the Lundehund enteropathy may be due to rupture of lacteals, increased paracellular permeability, caused by enhanced hydrostatic tissue pressure, and decreased absorption through a malformed brush border.
A case of Noonan's syndrome with pulmonary lymphangiectasia detected with lymphoscintigraphy is reported.
Veins and lymph ducts are closely linked anatomically, embryologically and physiologically. It is therefore not surprising that such a close relationship should reemerge in the pathology of each. Post-phlebitic disorders provide a classic example of this. Microlymphography, using fluorescence, and isotopic phlebolymphography produce positive, unequivocable arguments for the contribution of lymph to the genesis of post-phlebitic disorders; these methods are better than direct lymphology which is technically difficult and not altogether without its dangers. These findings should, of course, have bearing on therapy.
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Three patients with intra-abdominal lymphangiectatic cysts, with details of their clinical radiologic, and pathologic findings, provide examples of the diverse and confusing clinical manifestations of this uncommon lesion. Their diversity is largely the result of their variable size and location within the mesentery, omentum, or mesocolon. Radiologic examination provides the greatest aid in preoperative diagnosis, and is useful in differentiation from ascites and other abdominal lesions. The characteristic histologic appearance of lymphangiectatic cysts provides their ultimate classification.
Primary intestinal lymphangiectasia is a rare cause of protein-losing enteropathy. We report here our first case, a young girl presenting at the age of ten months at the Department of Paediatrics, Singapore General Hospital with generalised oedema and diagnosed to have intestinal lymphangiectasia proven by intestinal biopsy. The literature on some aspects of the subjects is also reviewed.
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