[A case of von Recklinghausen's disease followed by intra-hepatic neurofibroma].
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Adult↗
Biomedical subjects
K Kiyosawa
Publications and source records attributed to K Kiyosawa.
Detection of chronic hepatitis C virus infection by four diagnostic systems: first-generation and second-generation enzyme-linked immunosorbent assay, second-generation recombinant immunoblot assay and nested polymerase chain reaction analysis.
Serum samples from 100 patients with non-A, non-B hepatitis-related chronic liver disease and 100 patients with hepatitis B-related chronic liver disease were tested by first-generation and second-generation enzyme-linked immunosorbent assays, a second-generation recombinant immunoblot assay and the nested polymerase chain reaction. In non-A, non-B hepatitis-related chronic liver disease, second-generation enzyme-linked immunosorbent assay (anti-c22 and/or c200) and second-generation recombinant immunoblot assay showed 98% positivity, whereas first-generation enzyme-linked immunosorbent assay (anti-c100-3) showed 89% positivity. The two second-generation recombinant immunoblot assay-negative samples were positive by nested polymerase chain reaction, but one second-generation recombinant immunoblot assay-positive sample was polymerase chain reaction negative. However, when this second-generation recombinant immunoblot assay-positive sample was tested by polymerase chain reaction using another set of primers, it was polymerase chain reaction positive. Therefore, 100% of the non-A, non-B hepatitis-related chronic liver disease serum samples were hepatitis C virus RNA positive by polymerase chain reaction. Nine hepatitis B-related chronic liver disease samples were first-generation enzyme-linked immunosorbent assay positive. Of the eight second-generation enzyme-linked immunosorbent assay-positive hepatitis B-related chronic liver disease samples, six were first-generation enzyme-linked immunosorbent assay positive and five were second-generation recombinant immunoblot assay positive and polymerase chain reaction positive. One indeterminate second-generation recombinant immunoblot assay sample was polymerase chain reaction negative. Therefore, second-generation recombinant immunoblot assay appears to be as useful as polymerase chain reaction for detecting a chronic hepatitis C virus infection, although some discrepancies were noted.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Detection of hepatitis C virus (HCV) RNA in paraffin embedded tissue sections of human liver of non-A, non-B hepatitis patients by in situ hybridization.
Localization of hepatitis C virus (HCV) RNA was investigated by non-radioactive in situ hybridization in human liver specimens of chronic non-A, non-B (NANB) hepatitis patients who were seropositive for antibodies to HCV (anti-HCV). For in situ hybridization, T-T dimerized synthetic oligodeoxynucleotide probes were used and DNAs hybridized in situ were detected immunohistochemically using specific antibodies against T-T dimer. The data demonstrates that HCV-RNA was localized in the cytoplasm of hepatocytes in human liver biopsies obtained from the patients with chronic NANB hepatitis seropositive for anti-HCV.
Adolescent↗
A possible association between basic amino acids of position 13 of DRB1 chains and autoimmune hepatitis.
Fifty-one patients with autoimmune hepatitis have been studied for HLA association by conventional serology and also by modified polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) genotyping. HLA-DR4 was significantly associated with autoimmune hepatitis (46 of 51 patients, 90.2%). DNA typing of the DRB1 gene for 43 DR4-positive patients by using the PCR-RFLP technique revealed that of 43 patients, 33 had DRB1*0405 (Dw15), five had DRB1*0406 (DwKT2), four had DRB1*0403 (Dw13a), two had DRB1*0401 (Dw4), two of 43 had DRB1*0407 (Dw13b) and one had DRB1*0408 (Dw14b). Thus, there was no significant difference in Dw frequencies between DR4-positive patients and DR4-positive healthy subjects. These findings suggest that the DR4-specific sequence (Val 11 and His 13 at amino acid positions 11 and 13, respectively), but not particular Dw-associated DR4 sequence, in the first domain of the DRB1 chain contributes to susceptibility to autoimmune hepatitis among Japanese. Interestingly, all five of the DR4-negative patients had the DR2 specificity (DRB1*1502 or 1601). Taken together, these results imply that the basic amino acids at position 13, which is present only on the DR2 and DR4 B1 molecules (Arg on DR2 and His on DR4), are most important for determining the predisposition to autoimmune hepatitis.
Amino Acid Sequence↗
Protective effect of vitamin E supplementation on increased thermal stability of collagen in diabetic rats.
Products similar to non-enzymatic glycation end products are known to arise from the interactions between proteins and lipid peroxides in vitro. In this study, we assessed the effect of vitamin E, which possibly modifies lipid peroxide, on advanced glycation end products or similar products in vivo by measuring the fluorescence and thermal rupture time of tail tendon collagen in streptozotocin-induced diabetic rats. The diabetic rats and non-diabetic rats were fed a vitamin E supplemented diet, and a control diet starting 3 days after the streptozotocin injection. After the 4-week treatment, the serum lipid peroxide levels expressed as thiobarbituric acid reactants in the diabetic rats on control diet (15.9 +/- 2.6 mumol/l[SEM]) were significantly (p less than 0.05) higher than in the non-diabetic rats (7.9 +/- 1.3 mumol/l on control diet and 3.3 +/- 0.4 mumol/l on supplemented diet), but the levels in the diabetic rats on supplemented diet (7.9 +/- 2.3 mumol/l) were reduced to the normal levels. No significant differences were found in serum glucose and glycated haemoglobin levels within the diabetic rats on control and supplemented diets. Both the fluorescence and thermal rupture time of collagen were significantly (p less than 0.05) increased in the diabetic rats on both diets compared with those in the corresponding non-diabetic rats. Although there was no significant difference in the collagen-linked fluorescence within the diabetic rats on control and supplemented diets, the thermal rupture time was significantly (p less than 0.01) shortened with supplemented diet (10.8 +/- 0.7 min on supplemented diet vs 15.0 +/- 0.7 min on control diet).(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Clinical aspects and epidemiology of hepatitis B and C viruses in hepatocellular carcinoma in Japan.
The incidence of hepatocellular carcinoma (HCC) in Japan has increased over the past two decades. Of the 379 patients with HCC treated at Shinshu University Hospital over the past 20 years, 112 underwent treatment between 1971 and 1980 and 267 were treated between 1981 and 1990. The prevalence of hepatitis B virus-associated HCC and hepatitis C virus-associated HCC was 54% and 34%, respectively, during the first decade and 31% and 60%, respectively, during the second decade. Major factors contributing to the increased incidence of HCC include an increase in the incidence of type C chronic hepatitis and an increase in the incidence of cirrhosis of the liver, which in turn are the result of blood transfusions received about 30 years ago. Donated blood testing positive for hepatitis C virus antibody is currently rejected from the blood supply. However, the occurrence of post-transfusion hepatitis with the potential to develop into HCC has not been entirely eliminated. In addition, there is an as yet unelucidated route of horizontal transmission of hepatitis C virus.
Adult↗
Caroli's disease in three siblings.
Three sisters with cystic dilatation of the intrahepatic bile ducts (Caroli's disease) are reported. The index case, a 41-year-old woman with remittent high fever and right upper quadrant abdominal pain, was diagnosed as Caroli's disease with hepatic lithiasis and cholangitis based on findings of ultrasonography, computed tomography and endoscopic retrograde cholangiography. Her two older sisters were also examined and found to have the same disease without clinical symptoms. Their symptoms, locations of the dilated ducts and complications all varied. The hereditary mode of Caroli's disease in 13 families (32 cases) reported in the world literature including our study was examined. While Caroli's disease is thought to be an autosomal recessive disease, a conclusion on the hereditary mode of transmission could not be made in this study because of an insufficient investigation of family members, especially the parents.
Adult↗
Antibody to hepatitis C virus in patients with chronic schistosomiasis.
To clarify the effect of hepatitis C virus (HCV) infection in patients with chronic schistosomiasis, 96 patients with schistosomiasis and 137 patients with chronic liver disease without schistosomal infection were analysed by domination of antibody to HCV (anti-HCV). In 45 of 96 schistosomiasis patients, the serum alanine aminotransferase (ALT) level was continuously elevated, and the positive rate of anti-HCV was 52.9%, which is almost the same prevalence rate as in patients with chronic liver disease (48.9%). In contrast, in the remaining 51 schistosomiasis patients, serum ALT level was continuously within the normal range and the positive rate of anti-HCV was 0%. Histological investigation showed that the positive rate of anti-HCV in HBsAg-negative schistosomiasis patients was 14% for hepatic fibrosis, 71% for chronic hepatitis, 80% for liver cirrhosis and 56% for hepatocellular carcinoma. In all anti-HCV-positive patients, serum ALT level was continuously elevated. The serum transaminase levels in anti-HCV-positive patients were higher than those in anti-HCV-negative patients. These data suggest that in patients with chronic schistosomiasis, HCV infection accelerates the derangement of liver function, and may be a major aetiological factor in the development of chronic hepatitis and liver cirrhosis, supporting a causative association between HCV infection and hepatocellular carcinoma.
Aged↗
New hepatocellular carcinoma cell line SUHC-1 established from a patient with hepatitis C virus RNA in serum.
A new human hepatocellular carcinoma cell (HCC) line, designated SUHC-1, was derived from a Japanese patient with hepatocellular carcinoma having antibody to hepatitis C virus (HCV) and HCV-RNA in his serum, and established in tissue culture. This cell line exhibited typical epithelial cell morphology in culture as observed by phase-contrast and electron microscopy. The SUHC-1 cells produced albumin and alpha 2-macroglobulin. Chromosomal analysis showed several rearrangements at short and long arms of chromosome 1, 17 and 20 (1p-, 1q-, i(1q), i(17q) and 20q+) with a modal number of 91. HCV-RNA was not detected in the supernatant of SUHC-1 cells by nested polymerase chain reaction assay or in the SUHC-1 cells by the in situ hybridization method. We concluded that complete HCV does not exist in the SUHC-1 cell line. The SUHC-1 cell line is the first line of HCC to have been derived from a patient with persistent HCV infection, and may provide a suitable model for studies of hepatocarcinogenesis related to HCV.
Aged↗
Insulin-dependent diabetes mellitus showing microangiopathic hemolytic anemia and chronic disseminated intravascular coagulation.
It has been reported that microangiopathic hemolytic anemia occasionally occurs in patients with severe diabetic microangiopathy. We report a case of insulin-dependent diabetes mellitus in whom microangiopathic hemolytic anemia and chronic disseminated intravascular coagulation (DIC) were thought to be complicated. The patient showed fragmentation hemolytic anemia and progressive diabetic microangiopathy, together with a mild elevation of serum fibrin(ogen) degradation products. Considering the state of chronic DIC, heparin therapy was started, but mild hemolysis persisted. It is possible that microangiopathic hemolytic anemia and chronic DIC cause a vicious cycle in patients with severe diabetic microangiopathy, leading to rapid progression of diabetic microangiopathy.
Adult↗
Persistence of viremia in patients with type-C chronic hepatitis during long-term follow-up.
To clarify the evolution of antibody to hepatitis C virus (anti-HCV) and of liver histologic findings during the natural course of type-C chronic hepatitis, 111 patients with biopsy-proven chronic hepatitis type C were consecutively enrolled in this study and were followed up biochemically, serologically, and histologically for more than 5 years. All were positive for the first- and second-generation antibody to HCV (anti-HCV-1 and anti-HCV-2). None received antiviral therapy during the follow-up period. At the end of follow-up, all remained positive for anti-HCV-2, but four patients turned negative for anti-HCV-1. HCV RNA, detected by the polymerase chain reaction method, was tested serially in 20 patients who persisted positive anti-HCV-1 and in 4 patients who lost anti-HCV-1. HCV RNA disappeared from only two patients, who lost anti-HCV-1 during the follow-up period. A normalization of the serum transaminase level was found in only two patients, who lost both anti-HCV-1 and HCV RNA. A repeat liver biopsy was performed in 62 patients with chronic hepatitis who were persistently positive for all HCV markers and in 4 patients who lost anti-HCV-1. Of the 62 patients who retained all HCV markers, 16 progressed to hepatocellular carcinoma, 6 to cirrhosis of the liver, and 1 had a normal liver, whereas the remaining 39 had chronic hepatitis. Two patients who lost both anti-HCV-1 and HCV RNA showed a normal liver. Of two patients who lost only anti-HCV-1 one progressed to hepatocellular carcinoma and one to chronic hepatitis.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Prevalence of antibody to hepatitis C virus in Japanese schoolchildren: comparison with adult blood donors.
A total of 1,442 schoolchildren in the Matsumoto City area were investigated for the prevalence of hepatitis virus-related serum markers, including antibody to hepatitis C virus (anti-HCV) and an abnormal serum transaminase level. Despite the large number of children tested, none was positive for anti-HCV antibodies or had been diagnosed as having viral hepatitis. The prevalence of anti-HCV antibodies in children and adult blood donors in the same area increased significantly with age from 0% in the 6-15-year-old group to 1.14% in the 50-65-year-old group (P less than 0.001). Our results indicate that even if only the enzyme-linked immunosorbent assay results confirmed by the recombinant immunoblot assay are considered positive, the prevalence in children is significantly lower than that in blood donors (P less than 0.05). Six children were healthy carriers of hepatitis B virus (HBV); all had been born to carrier mothers. These results indicate that apparently healthy schoolchildren in Japan have a low exposure to HCV infection.
Adolescent↗
[The clinical usefulness of a new (the second generation) antibody to hepatitis C virus].
To clarify the clinical usefulness of a second generation antibody to hepatitis C virus (anti-HCV-2), we tested the anti-HCV-2 by enzyme immunosorbent assay (Imucheck HCV Ab) in comparison to the first generation antibody (anti-HCV-1). Serum samples were obtained from the patients with acute hepatitis, chronic liver diseases, alcoholic liver disease and autoimmune liver diseases. Furthermore, both antibodies were tested in serum samples from individuals in a hyperendemic area of non-A, non-B hepatitis. Anti-HCV-2 was detected earlier than anti-HCV-1 in acute type C hepatitis. The prevalence of anti-HCV-2 was higher than that of anti-HCV-1 in acute and chronic non-A, non-B liver diseases. However, prevalence of anti-HCV-2 was not increased in patients with non-viral liver disease such as pure alcoholic liver disease and autoimmune liver diseases. The HCV-infection rate was increased to about 40% by detection of anti-HCV-2 in individuals in hyperendemic area of non-A, non-B hepatitis. Thus, the assay of anti-HCV-2 is very useful to detect actual HCV infection. The accuracy and sensitivity of anti-HCV-2 were higher than those of anti-HCV-1.
Adult↗
Nucleolar antigens and autoantibodies in hepatocellular carcinoma and other malignancies.
Patients with hepatocellular carcinoma (HCC), gastrointestinal, lung, and ovarian cancers were shown to have autoantibodies to nuclear and nucleolar antigens as detected by immunofluorescence on cell substrates. The frequency of antinuclear antibodies (ANAs) was significantly higher (P less than 0.001) in patients with HCC (57/184 = 31%) than in patients with chronic hepatitis or liver cirrhosis (25/187 = 13%). Although a range of fluorescence patterns was observed, a higher percentage of nucleolar fluorescence was detected in HCC, and three of these nucleolar antigens were identified. They were NOR-90, nucleolus organizer region doublet polypeptides of 93 and 89 kDa involved in RNA polymerase I transcription; fibrillarin, a 34 kDa protein of the nucleolar U3 ribonucleoprotein particle which is engaged in preribosomal RNA processing; and nucleophosmin/protein B23, a 37 kDa polypeptide which is associated with ribosome maturation and cellular proliferation. All these antigens are nucleolar components that are engaged in some aspect of ribosome biosynthesis. Since autoantibodies to these nucleolar antigens have also been found in systemic autoimmune diseases, they do not represent autoimmune reactions unique to cancer but might reflect reaction pathways related to immune responses that are antigen-driven. The ANA response in HCC appears to be dynamic reactions to this antigen-drive since some patients with chronic liver disease showed seroconversion to ANA positivity, marked increase in titer and/or change in antibody specificity preceding or coincident with clinical detection of HCC. These changes in ANA showed a close temporal relationship with transformation from long-established chronic liver disease to HCC.
Antibodies, Neoplasm↗
[Japanese clinical statistical data of patients with hepatitis C].
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Adolescent↗
[Cerebrospinal fluid levels of 28 kDa calcium-binding protein in patients with neurological diseases].
Vitamin D-dependent calcium-binding protein (Calbindin-D: CaBP) (MW 28 kDa) is present in high concentrations in the central nervous system (CNS), especially in the cerebellum. In the cerebellum, CaBP is localized in Purkinje cells. By using an enzyme immunoassay method, we measured the cerebrospinal fluid (CSF) levels of CaBP in 96 neurological patients and 24 control subjects, and evaluated the clinical usefulness of determining CaBP, especially in the diagnosis of cerebellar damage. CSF CaBP levels were strikingly elevated in patients with cerebellar lesions, including primary spinocerebellar degeneration (SCD), subacute cerebellar degeneration with lung cancer, Wernicke-Korsakoff syndrome, as well as in patients with cerebrovascular disease (CVD) without involvement of the cerebellum. In these 2 groups, neuron-specific enolase (NSE) levels were determined. The ratio of CaBP to NSE (CaBP/NSE) in patients with cerebellar lesions was higher than that in CVD, reflecting differences in lesions between these 2 groups. In the SCD group, multiple system atrophy (MSA) showed higher CaBP levels than the other types of SCD. Probably it reflects remarkable damage of the Purkinje cells in MSA. In MSA, the CaBP levels decreased with the duration of illness. In myelopathy, neuropathy, meningitis, multiple sclerosis, and other degenerative diseases, the increase of CaBP was not remarkable. Our results suggested that CSF CaBP is considered to be a marker of cerebellar damage.
Adolescent↗
[Central nervous system disorders in patients with myotonic dystrophy--in relation to respiratory dysfunction].
Although central nervous system (CNS) involvement, such as intellectual impairment simulating dementia, in myotonic dystrophy (MyD) has been well documented, the cause of this condition remains unclear. In has been reported that the progressive cases of MyD are often accompanied with respiratory disturbance and sleep apnea syndrome (SAS). We studied the relation between CNS involvement and respiratory disorders in 15 MyD patients. They consisted of 10 males and 5 females with ages ranging from 21 to 58 years (average 46 +/- 8.4 years old). Arterial blood gas (ABG) analysis, respiratory function test, and monitoring of arterial oxygen saturation (SaO2) during sleep were carried out. In some cases abnormal respiration during sleep was analyzed with polysomnography. For an assessment of CNS involvement the following examinations were performed; intelligence quotient (WAIS-IQ); electroencephalography (EEG); brain computed tomography (CT); and cerebrospinal fluid (CSF) levels of neuron-specific enolase (NSE), S-100b and creatine kinase BB isoenzyme (CK-BB) which were estimated by using enzyme immunoassay. ABG analysis demonstrated the presence of hypercapnia (PaCO2 > 45 torr) during wakefulness in MyD patients. During sleep 14 of the 15 patients showed frequent desaturation phenomenon (SaO2 < 90%), indicating the episodic hypoxemia. Polysomnographic study revealed the occurrence of SAS of both obstructive and central types in all the cases examined. IQ test disclosed intellectual impairment in 80% of the 15 patients, and EEG showed slowing of basic rhythm in the majority of the cases. On brain CT both enlarged ventricles and dilated sulci were commonly observed.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Al-type generalized amyloidosis showing a solitary duodenal tumor.
An amyloid tumor of the duodenum is a rare occurrence. A patient who presented with epigastric discomfort, in whom a barium meal study revealed a tumor of the duodenum is described. Upper gastrointestinal endoscopy showed a submucosal location of the tumor, and examination of a biopsy revealed amyloid deposition of the AL type. Diffuse tumor involvement of adjacent tissues of the duodenum and also deposits of amyloid fibrils in the liver and skin with no evidence of a chronic pre- existing disease in the patient led to the diagnosis of primary systemic amyloidosis. The distinguishing feature of this case was the formation of a solitary and relatively large tumor in the duodenum mimicking a submucosal tumor, which is in contrast to most reported cases of multiple and smaller amyloid tumors in other parts of the gastrointestinal tract.
Amyloidosis↗