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ABSTRACT: Three Japanese patients with lecithin: cholesterol acyltransferase (LCAT) deficiency, the offspring of a consanguineous marriage, are described. In addition to the Characteristic clinical and laboratory findings of the disease, our patients had hitherto unreported manifestations, namely unconjugated hyperbilirubinemia, peripheral neuropathy and marked hypocholesterolemia. Although the mechanism of the unconjugated hyperbilirubinemia is not clear, the rde of impaired hepatic bilirubin uridine-diphosphate-glucuronyl transferase activity combined with another unknown factor(s) was postulated. Non-random assortment was observed between LCAT deficiency and haptoglobin types, as previously reported. The discovery of Japanese patients with LCAT deficiency indicates that the distribution of this hereditary metabolic disorder is not confined to the Western hemisphere.
Journal of Internal Medicine 04/2009; 204(1‐6):219 - 227. · 5.48 Impact Factor
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ABSTRACT: To find the best method to determine the stimulus intensity for median nerve somatosensory evoked potentials (SEP), we tried to activate muscle or cutaneous branch as preferentially as possible by using ordinary surface stimulation. We minutely moved the stimulating electrode at the wrist in normal subjects and changed the stimulus intensity stepwise at each site. We evaluated the correlation between the amplitudes of the SEP components and peripheral parameters such as the relative intensities to the motor threshold (rMT) or the sensory threshold (rST) and the amplitude of the sensory nerve action potential recorded over the index finger (SNAP2) or that of the compound muscle action potential (CMAP). The sensory parameters (rST and SNAP2) showed better correlation with SEP amplitude than the motor parameters (rMT and CMAP). In an extreme case, stimulation 40% over the motor threshold elicited no N9 response and only a small N20. Adjusting the stimulus intensity at slightly above the motor threshold, as recommended by most guidelines, in such a case would result in an erroneous result. We propose the stimulus intensity resulting in SNAP2 amplitude of 80% of its maximum as the optimal method because it consistently gave almost saturated SEP responses.
Journal of Clinical Neurophysiology 09/2007; 24(4):358-62. · 1.45 Impact Factor
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ABSTRACT: The prevalence of antimitochondrial antibody (AMA) in humans and its relationship to the development of primary biliary cirrhosis (PBC) are not well known. We have estimated the frequency of AMA in the general population, and studied its association with PBC.
We studies 1714 corporate workers (median age, 48 years; range, 30 to 59 years) who had an annual health check from 1998 to 1999 at Kawasaki Social Insurance Hospital in Japan. We used an indirect immunofluorescence method for screening serum AMA. We applied the prevalence of AMA-positive people in the study group to the general population in Japan. Then the inferred AMA-positive population was compared to the actual number of patients with PBC in statistics published by the Japanese Government.
AMA was detected in 11 of 1714 people (0.64%; 95% confidence interval, 0.26% to 1.02%). All these 11 sera reacted with 2-oxoacid-dehydrogenase complex by immunoblotting. Of these 11 individuals, none had subjective symptoms, all had normal serum bilirubin levels, and 6 had abnormal liver function test results. Using published statistics for the Japanese population, we inferred that there were approximately 336,472 AMA-positive people in Japan from age 30 to 59 years. The number of patients with symptomatic PBC recorded by the nationwide epidemiological survey of the Japanese Government was 2459. Thus, we inferred the rate of symptomatic PBC among AMA-positive persons to be about 0.73% (2459/336,472).
AMA is not a rare antibody in the general population, but few people develop recognizable PBC even if they have AMA.
Journal of Gastroenterology 02/2004; 39(3):255-9. · 4.16 Impact Factor
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Kazuhiko Miyake,
Toshiro Tango,
Yasuhiko Ota,
Keiji Mitamura,
Makoto Yoshiba, Makoto Kako,
Shigeki Hayashi,
Yusei Ikeda,
Norimasa Hayashida,
Shogo Iwabuchi,
Yuzuru Sato,
Toshiaki Tomi,
Naoya Funaki,
Naoaki Hashimoto,
Tomoyuki Umeda,
Jun Miyazaki,
Katsuaki Tanaka,
Yasuo Endo,
Hiroshi Suzuki
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ABSTRACT: A daily injection of glycyrrhizin (Stronger Neo-Minophagen C (SNMC) containing 40 mg glycyrrhizin in a 20 mL ampoule) lowers alanine aminotransferase (ALT) levels in patients with chronic viral hepatitis.
The therapeutic effects of intermittent administration of SNMC three times a week for 12 weeks were evaluated and compared between two doses (40 and 100 mL) in a randomized clinical trial.
Overall, the therapeutic response was better in the 53 patients allocated 100 mL than the 59 who were allocated to have 40 mL SNMC (P = 0.0243). At the completion of SNMC treatment, ALT levels decreased more extensively in the patients on 100 mL than those on 40 mL SNMC (-29 vs-50% in comparison with the baseline value, P = 0.0002). Minor side-effects occurred in both the patients on 100 mL (20%) and those on 40 mL (12%), but they did not require any therapies.
Intermittent SNMC would be efficient in suppressing ALT levels in patients with chronic viral hepatitis in a dose-dependent manner. Taken along with infrequent and very mild side-effects, long-term intermittent SNMC would benefit patients with chronic hepatitis by maintaining their quality of life with easier compliance.
Journal of Gastroenterology and Hepatology 12/2002; 17(11):1198-204. · 2.87 Impact Factor
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Makoto Yoshiba,
Kazuhiko Sekiyama,
Kazuaki Inoue,
Masaya Yamada, Makoto Kako,
Kozo Nagai,
Masao Takatori,
Shogo Iwabuchi,
Yasukiyo Sumino,
Katsuaki Tanaka,
Yukiya Hakozaki,
Kiyoshi Hasegawa,
Akitaka Shibuya
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ABSTRACT: We have attempted to predict the development of fulminant hepatic failure at the stage of severe acute hepatitis before the onset of coma. This prediction is valuable because it may be used to block the development of fulminant hepatic failure with appropriate medical treatment.
To establish a discrimination formula, we retrospectively compared 13 clinical and laboratory variables in 36 patients with acute viral hepatitis and prothrombin levels of 40% or less of the control value who later developed fulminant hepatic failure with these variables in 12 patients who recovered spontaneously. A prospective study of 58 patients who developed fulminant hepatic failure and 18 who spontaneously recovered confirmed the validity of this formula.
In the retrospective study,we established the following discrimination equation: Z = -0.89 + 1.74 x (causal viruses, 1 point for type A or type B in acute hepatitis B virus [HBV] infection, 2 points for others) + 0.056 x (total bilirubin, mg/dl)-0.014 x (cholinesterase, U/ml). A positive Z value indicates that fulminant hepatic failure will develop. In the prospective study, the specificity, sensitivity, predictive accuracy, and positive and negative predictive values were 0.833, 0.983, 0.947, 0.950, and 0.938, respectively.
The present study indicated that fulminant hepatic failure can be predicted, by a simple discrimination equation, at the stage of severe acute hepatitis.
Journal of Gastroenterology 02/2002; 37(11):916-21. · 4.16 Impact Factor
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ABSTRACT: The alterations of the alkaline phosphatase (ALP) activity in the rat liver following bile duct ligation were investigated
by electron microscopical techniques. Serum ALP activity reached the maximum at 24 hours after ligation and two isozymes of
ALP, high molecular and low molecular one, appeared in the serum. Bile canaliculi became dilated at 48 hours after ligation
and the microvilli were destructed and diminished in number. ALP activity was observed almost only on the bile canalicular
membrane of the liver cells in the control. On the other hand, in bile duct-ligated rat, the ALP activity on the canalicular
membrane was markedly increased initially, then it appeared on the lateral membrane, and finally on the sinusoidal membrane
also. It was not stainable on the canalicular membranes which lacked microvilli. The proposed pathway through which hepatic
ALP enters the blood stream in bile duct-ligated rats is as follows: ALP, being synthesized in the microsomes of hepatocytes,
is initially transferred to the bile canalicular membrane and diffused to lateral membrane through tight junction, reaches
to sinusoidal membrane then released into the blood stream.
Journal of Gastroenterology 04/1980; 15(6):600-605. · 4.16 Impact Factor
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ABSTRACT: The effect of bile duct ligation on enzyme activities in the subfractions of the rat liver plasma membrane was investigated. Two subfractions were isolated from the rat liver plasma membrane by homogenization and subsequent centrifugation in a discontinuous sucrose gradient. The light subfraction contained fragments of the bile canalicular surface and the heavy fraction contained fragments of the sinusoidal and lateral surfaces of the hepatocyte. Bile duct ligation decreased NaK ATPase and Mg-ATPase activity in the light subfraction, whereas it had no significant effect on these enzyme activities in the heavy fraction. Leucyl-β-naphthylamidase activity was reduced and alkaline phosphatase activity was increased in both subfractions. These facts suggested that ligation of the bile duct led to loss of the secretory polarity of the liver cell. The in vitro effects of some bile acids on the membranebound enzymes in the light subfraction were investigated, and a possible involvement of chenodeoxycholic acid in the alteration of enzyme activities in the bile canalicular membrane was suggested.
Experimental and Molecular Pathology.
