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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 13 (1998), S. 491-493 
    ISSN: 1437-9813
    Keywords: Key words Endothelin ; Biliary atresia ; Portal hypertension
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Biliary atresia (BA) is the end-result of a destructive inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tracts with the development of biliary cirrhosis and portal hypertension (PH). Endothelins (ET) are 21-amino-acid peptides of endothelial origin with potent vasoconstrictor activity that bind to various cells of the liver. Nothing is presently known about plasma ET levels in BA. The aim of this study was to determine the clinical significance of plasma ET levels in patients with BA after hepatic portoenterostomy (Kasai's procedure) and to correlate these with liver function tests (LFT) and PH. We measured plasma concentrations of ET in 19 patients with BA (5 boys and 14 girls; mean age 11.6 ± 5.5 years) after portoenterostomy and 10 age-matched controls. Patients were grouped according to outcome based on LFT: group A consisted of 9 patients with an ‘‘unfavorable outcome” and Group B 10 patients with a “favorable outcome”. The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA). No patient had ascites or hepatorenal syndrome. Plasma ET levels were significantly higher in patients with BA than in controls (3.42 ± 0.42 vs 1.75 ± 0.39 pg/ml, respectively; P 〈 0.01) and in patients in group A than in group B. (3.75 ± 0.25 vs 3.06 ± 0.23 pg/ml, respectively; P 〈 0.01). In group A, plasma ET levels were higher in patients with PH (n = 4) than in those without PH (n = 5) (3.99 ± 0.06 vs 3.64 ± 0.22 pg/ml, respectively; P 〈 0.05). We conclude that plasma ET levels are high in patients with BA, especially those with severe biliary cirrhosis, and that ET may partially contribute to development of PH in BA. The results of the present study also suggest that plasma ET concentrations may be a useful marker in the follow-up of patients with BA.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1437-9813
    Keywords: Key words Biliary atresia ; Bile lake ; α-glutathione-s-transferase (GST) ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Early identification of patients likely to deteriorate post-hepatic portoenterostomy for biliary atresia (BA) would be beneficial. α-Glutathione-s-transferase (α-GST) is a serologic marker of reactive hepatocellular damage because of its low molecular weight, uniform hepatic distribution, high cytosol concentration, and short half-life. We evaluated whether serum α-GST in post-surgical BA patients correlates with liver function (LF) and investigated its potential as a medium- to long-term marker of prognosis. Postoperative BA patients (n = 30; mean age: 11.8 ± 3.7 years) were divided into three groups based on average LF over the 3 months prior to this study. Group I (n = 8) were jaundice-free and had normal LF. Group II (n = 12) had moderate liver dysfunction, and group III (n = 10) had severe liver dysfunction. Serum α-GST was determined using a specific ELISA. Tissue α-GST was determined immunohistochemically, using liver needle-biopsy specimens. Bile lakes were found in 5 group II patients and 5 group III patients. Serum α-GST was significantly higher in group II (20.7 ± 8.4 ng/ml) than in groups I (4.7 ± 1.3 ng/ml) and III (8.0 ± 1.2 ng/ml) (P 〈 0.0001) and was highest in group II subjects with bile lakes. In control liver specimens α-GST distribution was weak but uniform throughout normal liver lobule hepatocytes. In group II there was strong staining in centrilobular hepatocytes, and in group III α-GST was only found in regenerative nodules. We conclude that α-GST may be a more sensitive indicator of hepatocellular damage in BA because its distribution is correlated to the proportion of functioning liver tissue present. This is the first report of this relationship, which has great implications for group II subjects because a sudden shift in concentration of α-GST may be a better predictor of impending hepatic dysfunction than conventional LF tests.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2307
    Keywords: Hepatic marginal zone B-cell lymphoma ; Mantle cell lymphoma ; MALT lymphoma ; Leu-1(CD5) ; Surface IgD
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a rare case of primary hepatic lymphoma, Stage II disease, in a 48-year-old male who had a solitary hepatic tumour measuring 4×4.5×3 cm. The tumour showed a nodular growth pattern and lymphoepithelial lesions with bile ducts. Some neoplastic nodules had a non-neoplastic atrophic germinal centre and/or a thin mantle cell layer. Morphologically, the neoplastic cells were centrocyte-like cells or intermediate lymphocytes. They expressed L26(CD20)+/LN-1(CDw75)±/LN-2(CD74)+/cyclin D1− and had a monotypic immunoglobulin of cytoplasmic IgM (к) on paraffin sections. The neoplastic cells or neoplastic nodules expressed surface IgM+/surface IgD±/Leu-1(CD5)+/DRC-1+/alkaline phosphatase+/B1(CD20)+/B4(CD19)− on fresh frozen sections. We therefore diagnosed this case as primary hepatic marginal zone B-cell lymphoma with mantle cell lymphoma phenotype. We confirm that it is difficult to differentiate extranodal marginal zone B-cell lymphoma (low grade B-cell lymphoma of mucosa-associated lymphoid tissue type; MALT lymphoma) and mantle cell lymphoma.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 17 (1987), S. 277-281 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three cases of chronic volvulus of the small bowel in midgut malrotation are presented, all of whom manifested similar angiographic findings: proximal occlusion of the superior mesenteric artery and vein and development of collateral vessels. These findings may indicate the pathophysiology of chronic volvulus in midgut malrotation; the volvulus is progressive and eventually results in the twisting of the mesenteric root itself, but because of its chronic nature collateral circulation develops, eliminating bowel necrosis. Computed tomography (CT), performed in two cases, revealed dilated, tortuous vessels in the mesentery in addition to the known CT finding of a whirl-like pattern of the volvulated small bowel loops. Sonography, performed in one case, showed an unique feature of whirling sonolucent layers probably representing the volvulated small bowel loops intermixed with dilated mesenteric collateral vessels. We would like to emphasize the usefulness of CT and sonography in the early diagnosis of those cases with vague and nonspecific clinical manifestations.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 18 (1988), S. 167-168 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An 8-year-old boy with traumatic duodenal hematoma is reported. Diagnosis is easy if one is familiar with its typical findings in modern imaging modalities.
    Type of Medium: Electronic Resource
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