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Biomedical subjects

I Horiuchi

Publications and source records attributed to I Horiuchi.

64 records · Page 4Linked to original sources

Changes in high density lipoproteins in patients with hepatobiliary diseases. Levels and lipid composition of HDL2 and HDL3 and LCAT reaction.

Lipids of HDL (high density lipoproteins) and their subfractions (HDL2 and HDL3), and LCAT activity (lecithin: cholesterol acyltransferase) were determined in hepatobiliary diseases without severe hyperbilirubinemia (less than 10 mg/dl). The decrease in major lipid constituents (cholesterol and phospholipids) of HDL was mainly attributable to the decrease in those of HDL3, except in some liver diseases of acute or severe stage (acute hepatitis in an acute stage and hepatoma) which were accompanied with a simultaneous moderate decrease in those of HDL2 and in fatty liver which showed a preferential decrease in those of HDL2. The LCAT activity also decreased in several diseases. Some of the hepatobiliary diseases, on the contrary, showed an increase in HDL-triglycerides (mostly in HDL3 and in some diseases also in HDL2) which might participate to some extent in secondary hyperlipidemia in the liver parenchymal diseases, although they were the minor lipid constituents of HDL. From results that HDL3- but not HDL2-cholesterol levels significantly correlated with serum total protein, albumin and choline esterase, it was suggested that the decrease in large constituents of HDL, particularly of HDL3, is caused by hepatocellular dysfunction which causes inhibition of protein and lipid syntheses in the liver in most of the hepatobiliary diseases except for fatty liver which has a preferential decrease in HDL2 lipids.

Adult↗

A new case of familial lecithin: cholesterol acyltransferase (LCAT) deficiency--paradoxical findings regarding LCAT mass and activity in 23 members of a family.

LCAT activity and mass were assayed simultaneously in 23 members of a new family case, revealing two homozygotes with a markedly low HDL--cholesterol level and ester cholesterol ratio. The LCAT mass in these patients was only 0.8 and 0.9 micrograms/ml, respectively (normal range 4.4-8.1) and their LCAT activity was 4 and 6 nM/ml/h 37 degrees C (normal range 60-120). Apolipoprotein (Apo) A-I and II levels were significantly low; however, apolipoprotein E tended to be high. In two-dimensional electrophoresis, apo A-I isoform visualized the increase of immature apo A-I; that is, A-I2. One subject showed the clinical characteristics of classic LCAT deficiency; however, the other, who was a vegetarian, showed corneal opacities and red cell deformity, but not proteinuria. This suggests that a low fat diet which decreases the level of atherogenic large LDL, may lead to a more favourable prognosis with a reduced risk for renal insufficiency. There were two different types of LCAT abnormality in this family series. Among the 10 examined paternal kindred of the proband who was one of two homozygotes, seven had a low LCAT mass but normal LCAT activity with the exception of one kindred who had a low mass and low activity. In contrast, among his seven maternal kindred examined, two had a low LCAT activity but normal mass.

Adult↗

[A case of stiff-man syndrome with an antineuronal autoantibody against an 80 kDa protein].

A 23-year-old Japanese man had noted intermittent muscle stiffness in his lower limbs and trunk in addition to painful muscle cramps in his back over one year. The muscle stiffness continued and eventually spread to his upper limbs. Neurological examinations revealed saccadic ocular movement and stiffness in the masseter, neck and four limbs and truncal muscles, enhanced by both voluntary and passive movements. Although the mobility of his joints was severely limited, the muscle strength was preserved. Electromyograms showed the continuous firing of normal motor units at rest, which disappeared both during sleep and after the intravenous administration of 10 mg of diazepam. An oral glucose tolerance test showed a normal pattern. Both the adrenal and thyroid functions were normal. No antibody against glutamic acid decarboxylase was detected in either the patient's serum or cerebrospinal fluid (CSF). The serum antibody against the voltage-gated potassium channel was also negative. Western blot analysis revealed the presence of an antibody in his serum and CSF which reacted with an 80kDa protein in the rat cerebrum, cerebellum and human spinal cord, but not in the rat liver. This patient was thus diagnosed as having stiff-man syndrome. Treatment with oral diazepam (50 mg/day), a GABAA agonist, proved to be effective, whereas baclofen (75 mg/day), a GABAB agonist, was not. These results suggest the dysfunction of the GABA neurons in this case, similar to that as previously observed in other cases of stiff-man syndrome while, in addition, the function of the GABAB receptor was also markedly disturbed in this case.

Adult↗