Brugia pahangi: susceptibility and macroscopic pathology of golden hamster.
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Lymphangiectasia of the large intestine associated with protein-losing enteropathy is reported. A 33-yr-old man suffered from diarrhea, sometimes mixed with blood. Colonoscopic study revealed reddish and edematous mucosa with multiple flat elevated lesions and giant folds in the localized segment of the rectosigmoid. An intestinal clearance test of alpha 1-antitrypsin revealed the association of protein-losing enteropathy. Operation was performed successfully, resulting in a marked improvement of symptoms and laboratory data, especially serum total protein, albumin, IgG, and Leu-2a-positive cells (suppressor/cytotoxic T cells). Giant folds consisted of the submucosal edema and hyperplasia of the epithelial glands with cystic dilatation of glands, and flat elevated lesions consisted of mucosal and submucosal edema associated with intestinal lymphangiectasia, adipose tissue, and blood capillaries. The population of the Leu-2a-positive cells in the lamina propria and intraepithelial layer was decreased and that of the Leu-3a-positive cells (helper/inducer T cells) in the lamina propria was increased.
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A 35-yr-old woman with intestinal lymphangiectasia was treated with trans-4-aminomethyl cyclohexane carboxylic acid. After 6 wk of antiplasmin therapy, her serum total protein increased to normal levels and 131I-polyvinyl pyrrolidone excretion was also normalized. With a daily administration of trans-4-aminomethyl cyclohexane carboxylic acid, the patient has manifested no symptoms for 8 yr up to the present. Throughout the clinical course, the values for euglobulin lysis time showed a close relationship to changes in serum total protein. It was then suggested that increased plasma fibrinolysis may enhance lymphatic permeability to plasma proteins. During this treatment, a decreased percentage of T lymphocytes became normalized together with serum immunoglobulin values. In addition, the therapy has resulted in the disappearance of duodenal lesions observed endoscopically.
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A unique case of enamel hypoplasia is presented. This appears to be the first case to be published in connection with the rare protein-losing enteropathy, intestinal lymphangiectasia. It seems likely that if the treatment of systemic disease is delayed, disruption of normal tooth formation is inevitable.
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Renal peripelvic multicystic lymphangiectasia is an unusual benign peripelvic fluid collection. The sonographic appearance, etiology, and differential diagnosis of peripelvic fluid collections are reviewed.
This article describes the immunologic and pulmonary abnormalities and the chemical composition of pleural effusion fluid in a patient with intestinal lymphangiectasia as they are effected by therapeutic measures during a 7-year period. Lymphedema was first noticed in the patient at 3 years of age, and pleural effusions developed 7 years later. Thoracentesis demonstrated that the right pleural fluid was yellow, clear, and had the composition of lymph. The left pleural fluid was milky and had a higher triglyceride and lymphocyte content than the right pleural fluid. Complete removal of pleural fluid transiently increased total lung capacity to a maximum of 52% predicted. Strict dietary management with a low-fat and high-protein diet resulted in a transient partial reversal of circulating lymphopenia and low T cell concentration. This was accompanied by a decrease in lymphocyte and T cell concentration in the pleural fluid. Unstimulated mononuclear cells from pleural fluid synthesized increased amounts of DNA, and added mitogens or antigens further increased DNA synthesis. Dietary therapy had a minimal effect on this DNA synthesis. Despite circulating hypogammaglobulinemia, normal antibody activity was detected. The proportion of B cells in pleural fluid was greater than that in the circulation, and dietary therapy did not alter this difference. Pulmonary physiology improved during the initial 9-month period of diet therapy, but then the rate of fluid accumulation increased, causing respiratory compromise. Stability was achieved by a right-sided pleurodesis, followed 18 months later by a left pleurodesis with the addition of a shunt to provide internal lymph drainage.
Peripheral blood lymphocyte subsets were enumerated in five children with abnormal lymphatic circulation (three with lymphangiectasia, one with chylothorax, and one child with chyloperitoneum). All patients were lymphopenic. The percentage and absolute number of blood T-lymphocytes (CD3) were low in two children and normal in the other children. The percentage and absolute number of helper/inducer lymphocytes (CD4) were markedly reduced in all patients. The percentage of suppressor/cytotoxic lymphocytes (CD8) was normal or elevated in all children, and the absolute number of CD8 cells was normal in three patients. The CD4/CD8 ratio was reversed in all patients. In the two patients tested, the proliferative response of peripheral blood mononuclear cells to mitogens was reduced. T-lymphocyte subsets were measured in the pleural or peritoneal fluid of three patients, and the CD4/CD8 ratio was normal or increased. In each child, the CD4/CD8 ratio in the lymphatic fluids was markedly higher than the CD4/CD8 ratio of the blood (4.0/0.45, 1.75/0.95, and 1.3/0.85). The reversed CD4/CD8 ratio in the blood in cases of chronic loss of chyle may be due to either selective transport of CD4 lymphocytes into the lymphatic fluids or a shorter half-life of CD8 compared to CD4 lymphocytes. This finding may in part explain the abnormal cellular immunity previously observed in patients with lymphangiectasia.
A case of a child with a rare giant congenital cavernous lymphangioma giving rise to elephantiasis of the left lower extremity is presented. Plain radiographs revealed underlying bone lesions and magnetic resonance (MR) imaging accurately defined extension of the process into the pelvis and retroperitoneum.
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OBJECTIVE: To evaluate whether surgical management of vulval lymphoedema and/or lymphangiectasia conveys any longstanding patient benefit. PATIENT AND METHODS: A qualitative analysis of signs and symptoms that occurred before and after surgical treatment for vulval lymphoedema and/or lymphangiectasia was performed. This was done by analysis of patient notes and telephone conducted interview. RESULTS: From a hospital database search, four patients were found to have had surgical treatment--three for lymphangioma and one for lymphangiectasia. Overall there was a clear improvement in the signs and symptoms associated with these conditions. In particular, all patients reported an improvement (i.e. a reduction or elimination) in the amount of oedema following surgery. CONCLUSION: Carbon dioxide laser therapy and superficial radiotherapy have been previously described for the management of vulval lymphangioma and lymphangiectasia with limited success, whereas our data suggests surgery offers a more permanent solution. In particular, labial reduction seems to be more successful than methods such as lymphovenous anastomoses and lymphangioplasties. A single operation may provide benefit for up to ten years. This approach has the potential to allow patients to be rehabilitated to normal life and activity.