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  • 1
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We present a case of chronic liver disease with selective and exclusive hepatocyte endoplasmic reticulum storage of δ1-antichymotrypsin in the form of granules, detected by specific immunohistochemistry at the light microscopy level and corresponding to material found in dilated endoplasmic reticulum of hepatocytes by electron microscopy. The patient had intermediate deficiency of δ1-antichymotrypsin. Thus, the hepatocyte accumulation of δ1-antichymotrypsin may indicate the presence of an export block resembling that of a closely-related protein, namely PiZ δ1-antitrypsin. It is proposed that hepatocyte storage of δ1-antichymotrypsin may be an expression of an inborn error of metabolism bearing the characteristics of endoplasmic reticulum storage diseases such as PiZ δ1-antitrypsin deficiency and hereditary hypofibrinogenaemia.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 21 (1992), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The histological and clinical findings in five cases of thymoma arising in paediatric patients have been studied. The age range was 11-15 years and no patient was affected by myasthenia gravis. All tumours were macroscopically encapsulated, but two of them displayed evidence of microscopic capsular invasion. Histologically, four cases were of the predominantly cortical type (organoid thymoma) with prominent areas of medullary differentiation and Hassall's bodies; one case was of the cortical type. All patients are alive and disease-free 3 months to 9 years after surgery. These findings suggest that thymoma in the paediatric age group may be characterized by fairly uniform clinicopathological features, with a low rate of association with myasthenia gravis and a favourable prognosis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 33 (1998), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims: The histochemical demonstration of hepatic copper is important in the diagnosis of Wilson's disease (WD). Conflicting results have been published with regard to the ability of different histochemical methods to demonstrate copper storage in the liver. Therefore, we evaluated the diagnostic value of three available histochemical methods in a large series of patients affected by WD.〈section xml:id="abs1-1"〉〈title type="main"〉Methods and resultsSeventy-nine consecutive liver needle biopsies, from 74 patients, 39 males and 35 females, aged 4–60 years (mean age 28.5 years) were stained with orcein, rhodanine and using Timm's method. On the basis of the histological picture, liver biopsies were subdivided into three groups: group A, steatosis; group B, interface hepatitis; group C, chronic hepatitis with bridging fibrosis and/or cirrhosis. In group A, 30.4% of the cases were positive using Timm's method, vs 13.2% using the rhodanine and 17.5% using the orcein method. In group B, Timm's method was positive in 40.1% while rhodanine and orcein showed positivity in 26.7%. In group C, the Timm's method stained 58.6%, rhodanine 36.6% and orcein 29.3% positively.〈section xml:id="abs1-2"〉〈title type="main"〉ConclusionsOur data show that: (1) Timm's silver stain is the most sensitive method for the demonstration of copper in all cases of WD; (2) rhodanine and orcein have minor value in the diagnosis of WD, especially in the early stages of the disease; (3) to increase the diagnostic value of histochemistry for copper multiple histochemical stains in serial sections are required; and (4) although hepatic copper concentration is highest in the early stages of WD, the histochemical demonstration fails in a large number of cases.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 35 (1999), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: To evaluate the value of polymerase chain reaction-in situ hybridization (PCR-ISH) for the detection of human papillomaviruses (HPV) in paraffin sections of cervical biopsies fixed either in 10% formalin or in Bouin's solution.〈section xml:id="abs1-2"〉〈title type="main"〉Methods and resultsWe analysed 40 biopsies from Italian women infected with the human immunodeficiency virus type 1 (HIV 1). In-situ hybridization techniques were performed with commercial biotinylated probes. The PCR-ISH was carried out by the ‘hot start modification’. Cervical intraepithelial neoplasia (CIN) was found in 23 of 40 patients (57.5%); eight cases showed condylomatous features. Human papillomavirus was detected in 42.5% by ISH and in 65% by PCR-ISH. Sixty-nine per cent of positive biopsies contained HPV 16, 18, 31 and 33. HPV 6 and 11 were found only in condylomata acuminata samples.〈section xml:id="abs1-3"〉〈title type="main"〉ConclusionsThe results point to a high incidence of HPV infection as well as of CIN in HIV-positive patients. Human papillomavirus type 16 appears to be most frequently associated with CIN. Polymerase chain reaction-ISH is more sensitive than ISH in the detection and typing of HPV DNA both in clinical and in ‘latent’ infections. The two techniques yielded the same results with either formalin- or Bouin's-fixed material.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1440
    Keywords: Hepatitis C virus ; Genotypes ; Chronic active hepatitis ; Interferon
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The behavior of hepatitis C virus (HCV) infection with regards to type and number of HCV genotypes (tested with genotype-specific nested polymerase chain reaction) was evaluated in 60 patients with anti-HCV-positive chronic active hepatitis without cirrhosis [17 untreated and 43 subjects undergoing single or repeat courses of interferon (IFN) therapy] during a mean follow-up period of 76±18 months. In untreated patients (2 genotype I, 6 genotype II, 9 mixed infections) 4 out of 9 mixed infections selected for genotype II at the end of follow-up. Of the 43 treated patients 10 were long-term responders with histological remission, 6 were shortterm responders, and 22 did not respond. Fifteen of the latter patients received another course of IFN therapy, and only 3 patients responded. Eight of the 10 responders had infection with a single genotype (4 gt I, 3 gt II, 3 gt III). After IFN therapy, all but 2 patients cleared the HCV infection. The responders to the second IFN course (1 gt I, 1 gt II, 1 gt III) remained viremic. Of the shortterm responders, 2/6 patients had genotype II and 4 had a mixed infection (3 gt II±I and 1 gt II±III); gt III became prevalent in the latter in all but one patient. Of the nonresponders 18/24 had more than one genoytpe, 5 were genotype II at baseline and one had genotype I. At the end of the follow-up period 15/18 with mixed infection had selected for gt II (P〈0.01 vs. untreated patient). Thirteen of 18 nonresponders who selected genotype II during follow-up developed cirrhosis, compared with none among the four untreated which also selected for genotype II (P〈0.01) and with none of the patients maintaining their baseline genotype (P〈0.01). In conclusion, patients with single HCV genotype, other than gt II, respond better to IFN, which seems to easily suppress HCV genotypes other than II. Genotype II is scarcely inhibited and becomes predominant during follow-up. In the patients selecting for genotype II, cirrhosis develops more rapidly than in untreated patients, where the selection for genotype II occurs at much slower rate.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Neurogenic arthrogryposis ; Parenchymal giant cell transformation ; Pigmentary liver disease ; Ductopenia ; Renal tubulopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on five patients from three families with neurogenic arthrogryposis, cholestasis and tubular renal dysfunction. Despite a similar clinical picture the liver histology showed a broad pathological spectrum, ranging from pigment storage to parenchymal giant cell transformation and ductopenia. The findings are compared with those of other cases from the literature in search of a correct nosology of the syndrome characterized by arthrogryposis, renal and liver disease. Conclusion We propose to consider the picture of arthrogryposis, renal tubular dysfunction and cholestasis as a single syndrome.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 36 (1991), S. 1143-1146 
    ISSN: 1573-2568
    Keywords: hepatocellular carcinoma ; chronic hepatitis ; HBV DNA ; viral DNA integration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a series of 325 HBV chronically infected children observed over an 18-year period, three developed HCC. These three children were born in southern Italy, a region characterized by a high endemic HBV infection rate; each had been infected perinatally, developed an acute hepatitis, and became a chronic carrier. Two of the three with cirrhosis were HBsAg positive at the time their HCC was detected. The remaining case had seroconverted to HBsAb but HBV-DNA integration could be demonstrated in the absence of cirrhosis; moreover HBV antigens were not expressed in the tissue of this case. The interval between HBV infection and HCC appearance in these three cases ranged from six to 11 years. A similarity between these three Italian cases and the majority of HCC, arising in chronically infected children in the Far East is noted.
    Type of Medium: Electronic Resource
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