11 results on '"Rabin L"'
Search Results
2. Hemochromatosis (HFE) gene sequence analysis of formalin-fixed, paraffin-embedded liver biopsy specimens.
- Author
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Przygodzki RM, Goodman ZD, Rabin L, Centeno JA, Liu Y, Hubbs AE, and O'Leary TJ
- Subjects
- Age Factors, Codon genetics, Female, Genetic Testing methods, Humans, Iron metabolism, Liver chemistry, Male, Mutation genetics, Prospective Studies, Sensitivity and Specificity, Sex Factors, Formaldehyde metabolism, Hemochromatosis genetics, Hemochromatosis pathology, Liver pathology, Paraffin Embedding methods, Sequence Analysis, DNA methods, Tissue Fixation methods
- Abstract
Background: Hereditary hemochromatosis (HH) is a common disease predominantly characterized by mutations of the HFE gene., Methods and Results: We investigated the utility of HFE gene sequence analysis in the diagnosis of HH in 61 prospectively accrued formalin-fixed, paraffin-embedded liver biopsy specimens with clinical or histologic features suggestive of HH. Mutations in codons 63 or 282 of the HFE gene were identified by direct sequencing; in 21 of these samples, quantitative hepatic iron testing was also performed. Changes characteristic of HH were present in 16 (26%) of the cases, and 54% of the cases showed HFE gene mutations. The most common alteration was homozygous mutation of codon 282 (11 cases, 18%), followed by the combined 63 + 282 heterozygous mutation (3 cases, 5%). Two cases (3%) showed biallelic mutation of codon 63. The other 28 cases (46%) showed no sequence abnormalities. Weak iron staining did not exclude HH; intense staining did not reliably predict HH., Conclusion: When HH is clinically and/or histologically suspected, HFE gene sequencing of formalin-fixed, paraffin-embedded liver biopsy specimens is a rapid and cost-effective approach to genotypic diagnosis of HH.
- Published
- 2001
- Full Text
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3. Modulation of thioacetamide-induced hepatocellular necrosis by prostaglandins is associated with novel histologic changes.
- Author
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Bergasa NV, Borque MJ, Wahl LM, Rabin L, and Jones EA
- Subjects
- Alanine Transaminase blood, Animals, Dinoprost therapeutic use, Hepatic Encephalopathy chemically induced, Liver drug effects, Male, Necrosis, Rats, Rats, Sprague-Dawley, Thioacetamide, 16,16-Dimethylprostaglandin E2 therapeutic use, Dinoprost analogs & derivatives, Hepatic Encephalopathy drug therapy, Hepatic Encephalopathy pathology, Liver pathology
- Abstract
Cytoprotective effects of the prostaglandins 16,16-dimethyl PGE2 (dmPGE2) and PGF2 alpha tromethamine (PGF2 alpha) were evaluated in the rat model of acute hepatocellular necrosis induced by thioacetamide (TAA). dmPGE2 (100 micrograms/kg SC 8 hourly) did not induce a significant increase in survival when started after the onset of TAA-induced fulminant hepatic failure. However, priming with dmPGE2 (100 micrograms/kg SC 30 min before TAA) reduced TAA-induced elevations in serum ALT (684 +/- 68 (SEM) vs 274 +/- 135 IU/1, p less than 0.01). This phenomenon did not occur if dmPGE2 was administered after TAA or by the IP route. Modulation of TAA-induced centrizonal hepatocellular necrosis by dmPGE2 was associated with a striking increase in centrizonal ballooning of hepatocytes (p less than 0.01), and, as assessed by stereology, less hepatocellular necrosis and degenerative changes. PGF2 alpha, which in contrast to dmPGE2 does not act via cAMP, had no effect on TAA-induced changes in serum ALT or hepatic histology. These findings suggest that dmPGE2 decreases hepatocellular necrosis by activating surface membrane adenylate cyclase and consequently stimulating cAMP. Ballooning of hepatocytes could occur secondary to these membrane events and appears to be a marker of dmPGE2-induced cytoprotection in this model.
- Published
- 1992
- Full Text
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4. Cholesteryl ester storage disease: hepatopathology and effects of therapy with lovastatin.
- Author
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Di Bisceglie AM, Ishak KG, Rabin L, and Hoeg JM
- Subjects
- Biopsy, Child, Child, Preschool, Cholesterol Ester Storage Disease drug therapy, Cholesterol Ester Storage Disease enzymology, Female, Humans, Liver ultrastructure, Microscopy, Electron, Cholesterol Ester Storage Disease pathology, Liver pathology, Lovastatin therapeutic use
- Abstract
We describe three patients with cholesteryl ester storage disease. Diagnosis was confirmed by demonstrating a deficiency in lysosomal acid cholesteryl hydrolase activity in cultured skin fibroblasts from each of these patients. All had hepatomegaly, elevated serum aminotransferase activities and hyperlipoproteinemia. Histological examination of liver biopsy specimens before treatment revealed accumulation of fat within hepatocytes, bile duct epithelium and endothelial and Kupffer cells. Cholesterol crystals were recognized by their birefringence in frozen sections. A striking feature was the presence of markedly hypertrophied Kupffer cells and portal macrophages with foamy, tan-colored cytoplasm that stained readily with the periodic acid-Schiff reagent and aldehyde fuchsin. Periportal fibrosis was noted in all cases; incomplete cirrhosis was present in one case. Distinctive and hitherto undescribed lysosomal accumulations of triglyceride and cholesterol crystals were noted. The patients were treated with lovastatin, a cholesterol-lowering agent, for at least 12 mo. No significant changes were seen in serum lipoprotein concentrations or liver histopathology after therapy. Thus lovastatin did not have an obviously beneficial effect on abnormal lipid metabolism in these patients.
- Published
- 1990
- Full Text
- View/download PDF
5. Morphometric study of hepatic ultrastructure in alcoholic hepatitis. Veterans Administration Cooperative Study Group on Alcoholic Hepatitis.
- Author
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Chen TS, Murphy DP, Marquet G, Chedid A, Mendenhall CL, and Rabin L
- Subjects
- Biopsy, Humans, Liver pathology, Liver Diseases pathology, Male, Liver ultrastructure, Liver Cirrhosis, Alcoholic pathology
- Abstract
We undertook a morphometric analysis of hepatocellular organelles in an attempt to correlate their changes with the clinical stages of patients with alcoholic hepatitis. Although hepatic ultrastructural alterations did not correlate with disease severity, we found significant differences between patient and control groups in the measured parameters of non-organelle cytoplasm, mitochondria, SER, RER, glycogen, and lipid.
- Published
- 1987
6. The liver in long-term survivors of marrow transplant--chronic graft-versus-host disease.
- Author
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Berman MD, Rabin L, O'Donnell J, Gratwohl AA, Graw RG Jr, Deisseroth AB, and Jones EA
- Subjects
- Adolescent, Adult, Alanine Transaminase blood, Anemia, Aplastic therapy, Aspartate Aminotransferases blood, Bilirubin blood, Biopsy, Cholestasis, Intrahepatic etiology, Graft vs Host Disease pathology, Hepatitis B Surface Antigens analysis, Humans, Immunoglobulin G analysis, Immunoglobulin M analysis, Leukemia, Myeloid, Acute therapy, Liver pathology, Male, Time Factors, Transplantation, Homologous, Bone Marrow Transplantation, Graft vs Host Disease physiopathology, Liver physiopathology
- Abstract
We have studied five long-term survivors of allogeneic bone marrow transplantation. All exhibited prolonged serum biochemical evidence of hepatic dysfunction during 2- to 5-year periods of follow-up. Two patients developed chronic cholestasis without pruritus. The serum of a third patient became chronically positive for HBsAg. A fourth patient developed an acute hepatic syndrome and high titers of antibody to cytomegalovirus. Nuclear, mitochondrial, and smooth muscle antibodies were not detected. Seven liver biopsies, obtained from three of the patients, all revealed a hepatocellular necroinflammatory lesion suggestive of chronic active hepatitis, a paucity of interlobular bile ducts, and intrahepatic cholestasis. Possible etiologies for these hepatic changes include reactivation of chronic non-A, non-B hepatitis and chronic graft-versus-host disease per se. Our study emphasizes the diagnostic problems posed by hepatic dysfunction occurring in an immunosuppressed multiply-transfused patient after bone marrow transplantation.
- Published
- 1980
- Full Text
- View/download PDF
7. Does primary biliary cirrhosis in men differ from primary biliary cirrhosis in women?
- Author
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Rubel LR, Rabin L, Seeff LB, Licht H, and Cuccherini BA
- Subjects
- Adult, Aged, Autoantibodies analysis, Bile Ducts pathology, Bilirubin blood, Cholestasis etiology, Female, Humans, Liver Cirrhosis, Biliary complications, Liver Cirrhosis, Biliary pathology, Male, Middle Aged, Mitochondria, Liver immunology, Liver pathology, Liver Cirrhosis, Biliary physiopathology, Sex Characteristics
- Abstract
Primary biliary cirrhosis is infrequently diagnosed in men, so that the clinical, biochemical and histopathological spectrum of this disease in men has not been evaluated. Therefore, we studied 30 men who had a histological diagnosis of primary biliary cirrhosis and had positive tests for antimitochondrial antibodies. Five patients had no hepatobiliary symptoms, and two of these patients had neither biochemical nor histological evidence of cholestasis. These 30 male patients' findings were compared with the findings in 30 age-matched women who also had primary biliary cirrhosis and antimitochondrial antibodies. Six of these patients were asymptomatic. Clinical findings and symptomatic status, in addition to biochemical and histopathological features, were generally similar in both male and female patients. The possible significance of higher serum alkaline phosphatase activities and lower frequency of occurrence of piecemeal necrosis in men with primary biliary cirrhosis, as compared with women, requires further study.
- Published
- 1984
- Full Text
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8. Talc in liver tissue of intravenous drug abusers with chronic hepatitis. A comparative study.
- Author
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Allaire GS, Goodman ZD, Ishak KG, and Rabin L
- Subjects
- Adult, Blood Transfusion, Chronic Disease, Crystallization, Female, Hepatitis etiology, Humans, Kupffer Cells analysis, Macrophages analysis, Male, Microscopy, Electron, Scanning, Microscopy, Polarization, Middle Aged, Spectrophotometry, Hepatitis metabolism, Liver analysis, Substance Abuse, Intravenous complications, Talc analysis
- Abstract
To determine the frequency of talc microcrystals in liver tissue of intravenous (IV) drug abusers and the significance of this finding, the authors reviewed, with light and polarizing microscopy, sections of liver tissue from 70 patients with chronic hepatitis and a history of active (45) or past (25) IV drug abuse. Birefringent crystalline particles consistent with talc were found in 44 cases (63%), 31 associated with active and 13 with past drug abuse. The microcrystals were situated predominantly in hypertrophied portal macrophages; there were no well-formed granulomas. Scanning electron microscopic and energy-dispersive spectrophotometry performed on eight of the positive cases showed the characteristic "flake-pastry" appearance and chemical composition (silicon and magnesium) of talc. For comparison, the authors similarly examined 70 cases of posttransfusion chronic hepatitis, all of which had negative findings for talc, and 70 cases of chronic hepatitis with no documented risk factors for viral hepatitis, of which two had positive findings for talc, even though IV drug abuse was denied by the two patients. The authors conclude that talc is frequently present in the liver of IV drug abusers and whenever encountered it strongly suggests IV drug abuse. Only two patients (1.4%) with a negative history also had talc.
- Published
- 1989
- Full Text
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9. Quinidine hepatotoxicity. A report of a case and review of the literature.
- Author
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Koch MJ, Seeff LB, Crumley CE, Rabin L, and Burns WA
- Subjects
- Alanine Transaminase blood, Aspartate Aminotransferases blood, Chemical and Drug Induced Liver Injury pathology, Humans, Male, Middle Aged, Chemical and Drug Induced Liver Injury etiology, Liver drug effects, Quinidine adverse effects
- Abstract
A case is described of presumed quinidine hepatotoxicity, characterized by the development of fever, abnormal serum transaminase values, which improved after cessation of the drug but recurred after a challenge dose, and centrizonal hepatocellular necrosis detected on liver biopsy. Morphological changes on electron microscopy, consistent with a drug reaction, are also described. Pertinent features of previous case reports are analyzed, and the histological findings by light microscopy of the present and past cases are discussed. It is suggested that the development of unexplained fever within 1 month of quinidine administration should lead to consideration of possible hepatotoxicity.
- Published
- 1976
10. Liver surface characteristics as observed during laparoscopy correlated with biopsy findings.
- Author
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Heit HA, Johnson LF, and Rabin L
- Subjects
- Biopsy, Double-Blind Method, Humans, Liver Diseases diagnosis, Prospective Studies, Laparoscopy, Liver pathology, Liver Diseases pathology
- Published
- 1978
- Full Text
- View/download PDF
11. Ductal Plate Malformation in a Nonhuman Primate.
- Author
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WALLACE, S., BLANCHARD, T., RABIN, L., DICK, E., ALLAN, J., and HUBBARD, G.
- Subjects
CERCOPITHECUS aethiops ,LIVER ,CELLS ,COLLAGEN ,BILE duct diseases ,PRIMATES ,MICROSCOPY ,EXTRACELLULAR matrix proteins ,VETERINARY pathology ,DISEASES - Abstract
The article describes the malformation of the ductal plate in a six-year-old, male African Green monkey. According to the authors, light microscopic examination of the liver showed several spindle cells dispersed within interconnecting, broad bands of collagen, up to 200μm in width, which often spanned and connected portal areas. They add that ductal plate malformation is a basic feature of bile duct disorders, which can be divided into diseases characterized by biliary dilation or ectasia or those characterized by involution or destructive cholangitis.
- Published
- 2009
- Full Text
- View/download PDF
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