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Biomedical subjects

H Mabuchi

Publications and source records attributed to H Mabuchi.

At least 37 records · Page 2Linked to original sources
PubMed1990

A case of Class II malocclusion associated with a deeply impacted maxillary central incisor.

The patient was a 9-year-old girl with a skeletal Class II malocclusion characterized by maxillary protrusion, excessive overjet and deep overbite. The patient's maxillary left central incisor was deeply impacted. During the first stage, a lingual arch was employed after surgical exposure. During the secondary stage, all four first premolar teeth were extracted and then edgewise mechanotherapy was performed. The total treatment time was 6 years. The malocclusion was treated satisfactorily and resulted in correction of the esthetic and functional flaws after 1 year out of retention.

Cephalometry↗
PubMed1989

Molecular basis of lipid transfer protein deficiency in a family with increased high-density lipoproteins.

Plasma high density lipoproteins (HDL) are a negative risk factor for atherosclerosis. Increased HDL is sometimes clustered in families, but a genetic basis has never been clearly documented. The plasma cholesteryl ester transfer protein (CETP) catalyses the transfer of cholesteryl ester from HDL to other lipoproteins and therefore might influence HDL levels. Using monoclonal antibodies, we show that CETP is absent in two Japanese siblings who have markedly increased and enlarged HDL. Furthermore, they are homozygous for a point mutation in the 5'-splice donor site of intron 14 of the gene for CETP, a change that is incompatible with normal splicing of pre-messenger RNA. The results indicate that the family has an inherited deficiency of CETP due to a gene splicing defect, and illustrate the key role that CETP has in human HDL metabolism.

Adult↗
PubMed1989

A young type III hyperlipoproteinemic patient associated with apolipoprotein E deficiency.

A 13-year-old female patient had noticed tuberoeruptive xanthomas since 3 years of age. Her serum, VLDL, and IDL cholesterol levels were high (348, 158, and 60 mg/dL, respectively), while LDL and HDL cholesterol levels were 56 and 62 mg/dL, respectively. VLDL-cholesterol/serum triglyceride ratio was extremely high (0.86), suggesting type III hyperlipoproteinemia (HLP). Her apo E was undetectable by the single radial immunodiffusion studies and SDS-polyacrylamide gel electrophoresis. Her parents showed hypertriglyceridemia and her two siblings were normolipidemic, and their apo E levels were normal. Genomic DNA digested with BamHI or EcoRI did not show gross differences in the restriction fragment length between the apo-E-deficient patient and normal controls. Thus, apo E deficiency may be characterized by early appearance of clinical manifestations of type III HLP and higher VLDL-cholesterol/serum triglyceride ratio.

Adolescent↗
PubMed1989

The effect of renal transplantation on a major endogenous ligand retained in uremic serum.

The effects of renal transplantation on serum concentrations of 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid (CMPF) and indole-3-acetic acid (IAA), which are endogenous ligands retained in uremic serum, and on phenytoin binding to serum protein were investigated. IAA, a weakly bound ligand, was rapidly excreted by the transplanted kidney during the first one to three days after renal transplantation, but CMPF, a strongly bound ligand, was slowly excreted. The binding defect of phenytoin was partially corrected by transplantation during the period of study. The results suggested that the prolonged drug binding defect observed despite successful renal transplantation is caused by a slower decrease of strongly bound ligands such as CMPF retained in uremic serum; hypoalbuminemia, usually observed after transplantation, may also contribute to this phenomenon.

Adult↗
PubMed1989

Development of coronary heart disease in familial hypercholesterolemia.

We studied the development of coronary artery disease in 10 homozygous and 692 heterozygous patients with familial hypercholesterolemia. Seventy-five (22%) male heterozygotes and 35 (10%) female heterozygotes were affected by myocardial infarction, which was first noted in men in the 3rd decade of life and in women in the 4th decade of life. Thirty-eight (70%) out of the deceased 54 heterozygous patients died of coronary heart disease. The mean age at death was significantly less in male heterozygotes (54 years) than in female heterozygotes (69 years). Five homozygous and 105 male and 56 female heterozygous patients received coronary angiographic evaluation. The regression equations between age (X) and coronary stenosis index (Y) obtained by assigning score (0 to 5) to each of 15 coronary artery segments were Y = 1.57X - 20.43 (r = 0.956, p less than 0.05) in the homozygotes, Y = 0.52X - 9.11 (r = 0.438, p less than 0.001) in the male heterozygotes, and Y = 0.47X - 12.54 (r = 0.343, p less than 0.01) in the female heterozygotes. From these data, we can assume that coronary artery stenosis detectable by angiography will occur after 17 and 25 years of age in male and female heterozygotes, respectively, and the treatment of heterozygotes with lipid-lowering drugs can be delayed until late adolescence.

Adult↗
PubMed1989

[The development of health risk appraisal for Japanese as a new health educational tool].

Health risk appraisal (HRA) is a new health educational tool widely-used in the United States, which informs clients about how their health habits and lifestyles affect their probability of dying from potentially preventable causes and helps to motivate them to reduce their personal health risks. It personalizes mortality statistics and epidemiologic data by combining these data with a person's risk factors. We have got started the development of HRA for Japanese with reference to a new version of HRA named "Healthier People" revised by the Carter Center of Emory University and the Centers for Disease Control in the United States.

Health Education↗
PubMed1988

Effects of CS-514 (eptastatin), an inhibitor of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, on serum lipid and apolipoprotein levels in heterozygous familial hypercholesterolemic patients treated by low density lipoprotein (LDL)-apheresis.

Nine heterozygous patients with familial hypercholesterolemia (FH) were treated by low density lipoprotein (LDL)-apheresis using dextran sulfate cellulose columns. After more than 3 procedures of LDL-apheresis without drug therapy, combination therapy with LDL-apheresis and CS-514 (eptastatin), an inhibitor of 3-hydroxy-3-methylglutaryl coenzyme (HMG-CoA) reductase, at a dose of 10 mg twice daily was started. Pre- and post-apheresis serum cholesterol levels were decreased significantly by CS-514, from 289 +/- 24 mg/dl (mean +/- SEM) to 247 +/- 25 mg/dl and from 118 +/- 7 mg/dl to 106 +/- 9 mg/dl, respectively. Pre- and post-apheresis apolipoprotein B levels decreased significantly on CS-514 from 160 +/- 9 mg/dl to 138 +/- 8 mg/dl and from 58 +/- 6 mg/dl to 45 +/- 6 mg/dl, respectively. No adverse effects were observed during the combination therapy. Thus, the addition of an inhibitor of HMG-CoA reductase to LDL-apheresis is a useful method for further reducing serum cholesterol and apolipoprotein B levels in FH heterozygotes.

Adult↗
PubMed1988

Removal of apolipoprotein E-enriched high density lipoprotein by LDL-apheresis in familial hypercholesterolaemia: a possible activation of the reverse cholesterol transport system.

The presence of apo E-containing HDL in familial hypercholesterolaemia was investigated and its removal by LDL-apheresis using a dextran sulphate cellulose column was demonstrated by measurement of the apo E/apo A-I molar ratio of serum and by nondenaturing polyacrylamide gel electrophoresis followed by immunoblotting. The molar ratios of apo E/apo A-I in the density greater than 1.063 kg/l fraction of serum obtained from two homozygous patients with familial hypercholesterolaemia were higher (0.021 and 0.030) than that from normal subjects (mean +/- SE 0.011 +/- 0.002) (P less than 0.05). Polyacrylamide gel electrophoresis and immunoblotting showed an increase in apo E-containing HDL similar to HDL2, in the plasma obtained from the homozygous patient with familial hypercholesterolaemia. The increased amounts of apo E-enriched HDL were removed from plasma by adsorption with a dextran-sulphate cellulose column. These results suggested that LDL-apheresis using the dextran-sulphate cellulose column, may cause an increase in the turnover rate of the apo E-containing HDL and thus facilitate cholesterol removal from the peripheral tissues.

Adolescent↗
PubMed1988

Displacement by anionic drugs of endogenous ligands bound to albumin in uremic serum.

Impaired binding of anionic drugs to serum albumin in patients with uremia is thought to be due to the accumulation of endogenous substances that bind to albumin. In this study the displacement by the anionic drugs diazepam, warfarin, and salicylic acid, which are known to be representative drugs for the binding sites on the albumin molecule, of several endogenous ligands that bind to albumin in uremic serum was examined. The free fractions of the ligands bound to albumin were separated by ultrafiltration in the presence and the absence of test drugs and assayed by high-performance liquid chromatography. Diazepam displaced indoxyl sulfate (IS), hippuric acid (HA), and indole-3-acetic acid (IAA), and warfarin displaced IS, HA, ISAA, and 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid from serum albumin. However, salicylic acid did not displace the substance examined. The methods reported here are useful for determining the binding sites of the endogenous ligands on albumin and to clarify the drug-ligand interaction on albumin molecule in uremic serum.

Binding Sites↗
PubMed1988

A major inhibitor of phenytoin binding to serum protein in uremia.

A major endogenous ligand substance, 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid (CMPF), accumulated in the sera of uremic patients, inhibited phenytoin binding to pooled serum obtained from healthy subjects and to human serum albumin in a concentration usually observed in the sera of patients with uremia. This suggests that CMPF is a major drug-binding inhibitor present in uremic serum and may be one of the so-called 'uremic toxins'.

Adult↗
PubMed1988

A major endogenous ligand substance involved in renal failure.

The clinical significance of a major endogenous ligand substance, 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid (CMPF), present in the sera of patients with renal failure, was examined. In patients with chronic renal failure, the serum CMPF concentration was correlated with the serum creatinine concentration, but not in patients with acute renal failure. The accumulation of CMPF in the sera of patients with chronic renal failure may reflect the 'chronicity' of renal failure. Plasma exchange was more effective than direct hemoperfusion for the elimination of CMPF retained in uremic serum.

Acute Kidney Injury↗
PubMed1988

Inhibition of hepatic glutathione S-transferases by a major endogenous ligand substance present in uremic serum.

A major endogenous ligand substance present in uremic serum, 3-carboxy-4-methyl-5-propyl-2-furanpropanoic acid, inhibited the in vitro activity of bovine and rat liver glutathione S-transferases. Our results suggested that endogenous ligand substances retained in uremic serum have some toxic activities other than the inhibition of drug binding to serum protein observed in uremia.

Animals↗
PubMed1988

Decreased serum cholesteryl-ester transfer activity in a patient with familial hyperalphalipoproteinemia.

Lipoprotein patterns and cholesteryl-ester transfer activity (CETA) were examined in a patient with familial hyperalphalipoproteinemia (FHALP). The proband was a 41-year-old Japanese male. He was found to have hypercholesterolemia, with a serum total cholesterol level of 382 mg/dl and a HDL-cholesterol level of 177 mg/dl. HDL showed a high cholesterol/Apo AI ratio. His father, all of his siblings and one of his children showed high HDL-cholesterol levels (91, 100, 70, 108, 75 and 98 mg/dl, respectively). These data suggest that all members of his family were heterozygotes. He had neither cutaneous or tendinous xanthomas nor any clinical signs of atherosclerosis. The proband appears to have only one-tenth of the normal level of CETA. However, the level of lipid-transfer protein I (LTP-I) activity was near normal. Thus, this patient is most likely to have an exaggerated level of LTP-I inhibitor(s). Effects of probucol on serum lipoprotein and apolipoprotein levels were studied in our patient. Treatment with 250 mg of probucol twice daily reduced total serum cholesterol, low density lipoprotein (LDL) and HDL-cholesterol levels by 33.32 and 33%, respectively. Apo AI, B and E levels decreased by 22, 16 and 35% respectively. HDL-cholesterol/Apo AI ratio decreased from 0.9 to 0.76. CETA showed no significant changes. However, cholesterol ester mass transfer increased from 10.8 to 14.9% after treatment with probucol. These results suggest that probucol appears to be a useful drug for FHALP.

Adolescent↗