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Biomedical subjects

K Tada

Publications and source records attributed to K Tada.

At least 343 records · Page 19Linked to original sources
PubMed1987

Nystagmus and subnormal electroretinographic response in nonketotic hyperglycinemia.

We examined ophthalmologically two siblings with nonketotic hyperglycinemia who had negligible activities of glycine cleavage system in the liver. Both had jerky nystagmus. The elder brother had a normal electroretinographic response, while the younger brother's response was subnormal. There may be a close association of subnormal electroretinographic response and hyperglycinemia, because glycine can act as an inhibitory neurotransmitter in the retina.

Amino Acid Metabolism, Inborn Errors↗
PubMed1987

A new treatment of painful amputation neuroma: a preliminary report.

Two cases of painful amputation neuroma treated by a new method are described. A vascularized island transfer from a less important finger was used, but the accompanying nerve was divided and its distal end sutured to the proximal end of the nerve in the recipient finger, after excision of the neuroma. Four years and two years later, no clinical evidence of neuroma was present at either amputation stump or donor site.

Amputation Stumps↗
PubMed1987

Effect of 1 alpha-hydroxyvitamin D3 on osteoporosis induced by immobilization combined with ovariectomy in rats.

The effect of low-dose 1 alpha-hydroxyvitamin D3 [1 alpha(OH)D3] on experimental osteoporosis in rats induced by a combination of hemicordotomy and ovariectomy was evaluated. 1 alpha(OH)D3 (0.1 microgram/kg 1 alpha(OH)D3 three times a week) increased the femur score and photo-density. The administration of 1 alpha(OH)D3 also significantly increased the dry weight and ash weight of the bone. Histomorphometry revealed a significant decrease of bone resorption by the administration of 1 alpha(OH)D3. Significant decreases in 45Ca accretion rate and 14C-proline uptake were also observed. Bone formation in the 1 alpha(OH)D3 treated group was thought to decrease following the decrease of bone resorption.

Animals↗
PubMed1987

Nonketotic hyperglycinemia: analyses of glycine cleavage system in typical and atypical cases.

The molecular nature of the glycine cleavage system was investigated in eight patients with typical (neonatal) and two patients with atypical (late onset) nonketotic hyperglycinemia (NKH). The overall activity of the glycine cleavage system was found to be decreased in all of the liver and brain tissue studied, but it was undetectable or extremely low in typical NKH, whereas there was some residual activity in atypical NKH. Six patients with typical NKH had a specific defect in the P protein, and one a defect in the T protein; the activity of the T protein was defective in one patient with atypical NKH.

Amino Acid Oxidoreductases↗
PubMed1987

Ulnar deficiency.

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Abnormalities, Multiple↗
PubMed1987

Occurrence of both (25R)- and (25S)-3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acids in urine from an infant with Zellweger's syndrome.

Urine from a patient with Zellweger's syndrome was examined for bile acids after fractionation into three groups according to mode of conjugation. 3 alpha,7 alpha,12 alpha-Trihydroxy-5 beta-cholestanoic acid was the predominant bile acid of the unconjugated and glycine-conjugated bile acid fractions. Smaller amounts of cholic acid and 1 beta-, 6 alpha-, 24-, and 26-hydroxylated derivatives of 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid were found in both fractions in similar proportions. The bile acid spectrum of the taurine-conjugated bile acid fraction was different from those of the other two fractions in the occurrence of two new compounds as the major constituents. These compounds were tentatively identified as two epimers at C-23 of 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestano-26,23-lactone, which were probably artifacts formed from the corresponding tetrahydroxycholestanoic acids during the procedures for extraction after hydrolysis. High-performance liquid chromatographic analysis revealed that 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid excreted into the urine as the unconjugated form consisted of a mixture of (25R)- and (25S)-isomers in the ratio of about 7:3.

Bile Acids and Salts↗
PubMed1987

A resuscitation puzzle: acute acquired methemoglobinemia.

We treated a 32-day-old baby suffering acute acquired methemoglobinemia induced by topical application of aniline cocaine. Although acute acquired methemoglobinemia with severe cyanosis and distress is potentially fatal, this rare syndrome is easily curable if it is correctly diagnosed.

Anesthetics, Local↗
PubMed1987

Nonketotic hyperglycinemia: clinical and metabolic aspects.

The molecular nature of the glycine cleavage system was investigated in 16 patients with nonketotic hyperglycinemia (NKH). The overall activity of the glycine cleavage system was found to be decreased in all of the liver and brain tissue studied. It was undetectable or extremely low in the neonatal type of NKH, whereas there was some residual activity in the infantile type of NKH. Thus the clinical phenotypes do seem to relate to the degree of the defect in the glycine cleavage system. In the neonatal type, a specific defect in P protein was found in 9 cases and a specific defect in T protein in 2 cases. In the infantile type, a partial defect in T protein was found in 2 cases. Differential diagnosis between NKH and ketotic hyperglycinemia is described. A feasibility of prenatal diagnosis of NKH by chorionic villus biopsy is provided.

Brain Chemistry↗
PubMed1987

Glycogen storage disease type 1b: genetic disorder involving the transport system of intracellular membrane.

Glycogen storage diseases (GSD) type 1b is the first example of a genetic disorder involving the transport system of an intracellular membrane. It was revealed that the primary defect in GSD type 1b was a deficiency in the microsomal glucose-6-phosphate (G6P) translocase, based on the findings that the glucose-6-phosphatase activity was highly latent in the fresh liver homogenates. Further evidence of this defect in GSD type 1b has been provided by a membrane filter method which measures the uptake of 14C-G6P by microsomes. The clinical symptoms and enzymatic studies in our patients suggest that there is genetic heterogeneity in GSD 1b and the clinical severity depends on the level of residual activities of G6P translocase.

Adult↗
PubMed1987

Treatment of phenylketonuria with a formula consisting of low-phenylalanine peptide. A collaborative study.

A method of preparation of a more palatable therapeutic formula for phenylketonuria (PKU), consisting of low-phenylalanine peptide (LPP), was reported. There were no adverse effects and, in fact, there was a reduced frequency of diarrhea in patients who received LPP formula for more than 6 months. The LPP formula can be used not only as a more palatable therapeutic milk for PKU, but also as an ingredient to make more palatable foods of low-phenylalanine content.

Adolescent↗
PubMed1987

Partial and complete adenine phosphoribosyltransferase deficiency associated with 2,8-dihydroxyadenine urolithiasis: kinetic and immunochemical properties of APRT.

We have studied adenine phosphoribosyltransferase (APRT) in the hemolysates from the families of 2,8-dihydroxyadenine urolithiasis associated with partial deficiency of APRT (the Japanese type) and complete deficiency of APRT (the null type). The APRT in the control subjects was found to be heat-stable at the physiological concentration of phosphoribosylpyrophosphate (PRPP), which was close to the value of its Km for PRPP. The APRT in the Japanese type showed 10 times higher Km values for PRPP and needed a comparably increased level of PRPP for stability in vitro. No change in red cell PRPP was found in the Japanese type of APRT deficiency. The content of APRT enzyme protein was decreased in the hemolysates of the Japanese type, probably due to its lability at the level of PRPP present in the cells. The heterozygote of the null type also had labile enzyme molecules at the physiological PRPP concentration.

Adenine↗
PubMed1987

Regional cerebral metabolic rate for glucose and cerebrospinal fluid monoamine metabolites in subacute sclerosing panencephalitis.

Regional cerebral metabolic rate for glucose (rCMRglu) and cerebrospinal fluid monoamine metabolites were measured in two cases of subacute sclerosing panencephalitis (SSPE) with different clinical courses. A marked decrease in rCMRglu was found in the cortical gray matter of a patient with rapidly developing SSPE (3.6-4.2 mg/100 g brain tissue/min). However, the rCMRglu was preserved in the caudate and lenticular nuclei of the patient (7.7 mg/100 g/min). The rCMRglu in a patient with slowly developing SSPE revealed patterns and values similar to those of the control. Cerebrospinal fluid monoamine metabolites; homovanilic acid and 5-hydroxyindoleacetic acid, were decreased in both rapidly and slowly developing SSPE. These data indicated that rCMRglu correlated better with the neurological and psychological status and that dopaminergic and serotonergic abnormalities have been implicated in pathophysiology of SSPE.

Adolescent↗