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Biomedical subjects

H Yabuuchi

Publications and source records attributed to H Yabuuchi.

At least 181 records · Page 10Linked to original sources
PubMed1982

Trial of split-product trivalent influenza vaccine in high-risk children.

Split-product trivalent influenza vaccine, containing H3N2, H1N1 and B, was given to 188 high-risk children and 25 healthy ones in the 4 year period from 1978 to 1981. A field trial involving 80 of these children was performed for evaluating the safety and immunogenicity of this vaccine. The antibody response to influenza vaccine in the patients was similar to that in normal children. The reaction to this vaccine of high-risk children was low, and no severe clinical reactions were observed after vaccination.

Antibody Formation↗
PubMed1982

[Maturation therapy for advanced neuroblastoma-therapeutic effects and problems].

Seven children with advanced neuroblastoma were treated with maturation therapy. This therapy consisted of 12 hours drip infusion of papaverine (40-45 mg/kg/day) for 2 days with or without high dose cyclophosphamide (2,000-3,600 mg/m2). During maturation therapy all patients received intravenous hyperalimentation. One patient who had received papaverine alone did not respond to the therapy. Among 6 patients treated with papaverine and high dose cyclophosphamide, 3 responded to the therapy, 2 did not and one died soon after the therapy. However, similar responses were obtained in 2 of 3 responders with high dose cyclophosphamide alone. After all, papaverine infusion was evaluated to be effective only in one patient. The clinically severe complications of papaverine infusion were somnolence in all patients and extrapyramidal symptoms in 6 patients. Because of these extremely high toxicity rates with little evidence of therapeutic effect, selection for this therapy should be carefully done and the treatment plan should be reconsidered.

Adrenal Gland Neoplasms↗
PubMed1982

Ultrastructural study of biopsy specimens of rectal mucosa. Its use in neuronal storage diseases.

Rectal mucosa biopsy specimens from patients with neuronal storage diseases were examined by electron microscopy. The diseases were Tay-Sachs disease, Sandhoff's disease, Niemann-Pick disease types B and C, late infantile metachromatic leukodystrophy, GM1 gangliosidosis type 1, beta-galactosidase-neuraminidase deficiency, I-cell disease, and mucopolysaccharidoses (Hunter's syndrome and Sanfilippo's syndrome type A). Unmyelinated nerve fibers, endothelial cells, fibroblasts, plasma cells, and histiocytes were seen in the specimens. Except for plasma cells, the results thus obtained for various cells were similar to those obtained from skin and conjunctival biopsy specimens, which have been already reported. There has been no report so far on ultrastructure of the plasma cell in these diseases. Storage materials, eg, dense bodies and membrane-bound vacuoles, were observed in the plasma cells in various storage diseases, with the exception of late infantile metachromatic leukodystrophy. Thus, electron microscopy of rectal mucosa is useful in making diagnoses and examining plasma cells in some neuronal storage diseases.

Adolescent↗
PubMed1982

[Adriamycin and Ara-C infusions in the treatment of refractory leukemia].

We have treated two pediatric patients with refractory ANLL and four with relapsed high-risk ALL with Adriamycin continuous Ara-C i.v. Four out of six patients were able to enter complete remission after first or second course of the treatment. All of them developed severe bone marrow suppression, and various degree of oral mucositis, liver dysfunction and gastrointestinal toxicity, and two patients died from side effects. Recently this combination chemotherapy is becoming a best regimen for the treatment of untreated ANLL. Although our cases are small in number, this combination therapy also seems to be effective for refractory ANLL and relapsed high-risk ALL. It is needless to say that we have to pay more attention when we use this regimen.

Acute Disease↗
PubMed1981

Urinary oligosaccharides of GM1-gangliosidosis. Different excretion patterns of oligosaccharides in the urine of type 1 and type 2 subgroups.

Oligosaccharide patterns obtained by gel filtration of the urine of GM1-gangliosidosis Type 1 patients are quite different from those of GM1-gangliosidosis Type 2. By studies of oligosaccharides in the four major peaks obtained from the Type 1 subgroup using sequential exoglycosidase digestion, methylation analysis, and periodate oxidation, the structures of 15 oligosaccharides: Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 3Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6Man beta 1 leads to 4GlcNAc, Man alpha 1 leads to 6(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 3)Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 3)Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6[Gal beta 1 leads to 4GlcNAc beta 1 leads to 4(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2)Man alpha 1 leads to 3]Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 6(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2)Man alpha 1 leads to 6(Gal beta 1 leads to 4Glc NAc beta 1 leads to 2Man alpha 1 leads to 3)Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 6(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2)Man alpha 1 leads to 6[Gal beta 1 leads to 4GlcNAc beta 1 leads to 4(Gal beta 1 leads to 4GlcNAc beta 1 leads to 2)Man alpha 1 leads to 3]Man beta 1 leads to 4GlcNAc, Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 6, and 3(Gal beta 1 leads to 4GlcNAc beta 1 leads to 3Gal beta 1 leads to 4GlcNAc beta 1 leads to 2Man alpha 1 leads to 3 and 6)Man beta 1 leads to 4GlcNAc, (formula see text) were elucidated. The amounts of total oligosaccharides excreted in the urine of the Type 2 subgroup were approximately one-tenth of those of Type 1. Moreover, the last eight oligosaccharides shown above, which have a Gal beta 1 leads to 4GlcNAc beta 1 leads to 3Gal beta 1 leads to 4GlcNAc beta 1 leads to outer chain, were completely missing in the urine of Type 2.

Carbohydrate Conformation↗
PubMed1981

Intrapleural therapy of malignant pleurisy in patients with neuroblastoma.

Three children with advanced neuroblastoma developed pleural effusion in the course of their disease. This unusual complication was successfully treated with intrapleural administration of a nonspecific "immunostimulatory" agent; cell wall skeleton of Nocardia rubra (N-cws). The side effects of this procedure were mild and well tolerated even in infants in spite of their terminal stage. Intrapleural therapy in this study seemed to be useful for the treatment of pediatric malignant pleurisy.

Administration, Topical↗
PubMed1981

A new approach to detection of heterozygotes for adenosine deaminase deficiency: a hypothetical method.

In a second and third families with ADA deficiency found in Japan, we tried a new approach to evaluate heterozygote detection. This is based on the hypothesis that ADA activity of red blood cell is the quantitative sum of the activities of ADA proteins expressed by two allelic genes at the ADA autosomal locus, and that these activities are not changed by the gene transmission from parents to children. We have detected red blood cell-ADA activities expressed by the one normal allelic gene in heterozygotes (including parents and paternal or maternal grandfather or grandmother) and from these values have determined combinations for the pair of ADA activities expressed by the two allelic genes of other family members. These combinations were consistently made in all relatives examined in the two families, and we conclude that several members of each family who were judged to have nil activity in the combinations were heterozygotes for ADA deficiency.

Adenosine Deaminase↗
PubMed1981

Secondary hyperparathyroidism with 1,25-dihydroxyvitamin D deficiency and pseudohypoparathyroidism in childhood: relationship between plasma 1,25-dihydroxyvitamin D and parathyroid hormone levels and urinary cyclic AMP response to exogenous PTH.

In order to clarify the complex interrelationship between serum calcium, 1,25-dihydroxyvitamin D (1,25(OH)2D), and parathyroid hormone (PTH), and the urinary excretion of cyclic AMP (cAMP) in response to exogenous PTH in pseudohypoparathyroidism (PHP) and related diseases, we investigated 3 patients with parathyroid disorders before and after treatment with 1 alpha-hydroxyvitamin D3 (1 alpha-OH-D3). Low plasma 1,25(OH)2D before treatment increased after giving 1 alpha-OH-D3 (0.1 micrograms/kg/day), whereas high plasma PTH measured by the c-terminal assay (C-PTH) decreased in all 3. No response in urinary cAMP was found before or after treatment in 2 patients with PHP type I, despite the fall of plasma C-PTH. However, in one patient with extremely high plasma C-PTH but normal N-PTH (measured by a homologous radioimmunoassay using 1-34 human PTH), urinary cAMP response to exogenous PTH was increased after treatment with 1 alpha-OH-D3. We suggest that he had pseudopseudohypoparathyroidism (PPHP) with Albright's hereditary osteodystrophy and a partial deficiency of renal 1 alpha-hydroxylase. In this patient secondary hyperparathyroidism is thought to be due to 1,25(OH)2D deficiency, and the decreased responsiveness to exogenous PTH before treatment due to excess PTH occupying renal receptors.

Adolescent↗
PubMed1981

Properties of sulfatases in cultured skin fibroblasts of multiple sulfatase deficient patients.

Various sulfatase activities were assayed in cultured skin fibroblasts from patients with multiple sulfatase deficiency (MSD). MSD cell lines displayed deficiencies of arylsulfatase A and iduronate sulfatase, but activities of arylsulfatase B, N-acetylgalactosamine 6-sulfate sulfatase and N-acetylglucosamine 6-sulfate sulfatase were within normal ranges, but not consistently. Arylsulfatase A, minor anionic arylsulfatase and N-acetylgalactosamine 6-sulfate sulfatase in MSD cell lines had similar Km, pH optima, inhibitory or activator sensitivity to that of normal skin fibroblasts. Arylsulfatase B in MSD cell lines also had properties similar to that of normal skin fibroblasts, except an abnormal heat stability. From our results, we conclude that properties of arylsulfatase A, minor anionic arylsulfatase and N-acetylgalactosamine 6-sulfate sulfatase in MSD fibroblasts were intact. On the other hand, arylsulfatase B in MSD might be a functionally abnormal enzyme.

Cell Line↗
PubMed1981

Plasma active vitamin D concentration in low birthweight infants with rickets and its response to vitamin D treatment.

Impairment of 25-hydroxylation may be a cause of rickets in infants of low birthweight, weighing between 2000 and 2500 g. In addition there may be impairment of 1 alpha-hydroxylation in infants weighing less than 2000 g. Our data show that a supplementary dose of vitamin D2 of at least 500 IU daily is a reasonable regimen for infants who weighed between 2000 and 2500 g at birth. However for infants who weighed less than 2000 g with a gestation of under 38 weeks, administration of 1 alpha-OHD3 may be more effective in preventing rickets.

25-Hydroxyvitamin D 2↗
PubMed1981

Effect of dibutyryl adenosine 3',5'-monophosphate administration on plasma concentrations of 1,25-dihydroxyvitamin D in pseudohypoparathyroidism type I.

The effect of dibutyryl cAMP on plasma concentrations of 1,25-dihydroxyvitamin D [1,25-(OH)2D] was studied in two patients with pseudohypoparathyroidism (PHP) type I, five normal adults, and three normal children as controls. In normal adults, plasma 1,25-(OH)2D tended to increase 3 h after the infusion of 2.5 mg/kg dbcAMP and was significantly increased after 6 h. In normal children, plasma 1,25-(OH)2D increased 3 h after the infusion, then gradually decreased; in the patients with PHP type I, it increased greatly from 6.8 and 11.2 pg/ml to 264.6 and 128.2 pg/ml 3 h after the infusion. These results suggest that the renal mechanism for the response to parathyroid hormone is intact distal to the renal adenylate cyclase in patients with PHP type I.

24,25-Dihydroxyvitamin D 3↗
PubMed1981

Electronmicroscopy of conjunctival biopsy in mannosidosis.

Electronmicroscopic examination was performed on conjunctival biopsies from two adolescent siblings with mannosidosis. Fibroblasts and endothelial cells contained membrane-bound vacuoles and vesicles that contained homogeneous osmiophilic globules. These vesicles seem to be pathognomonic of mannosidosis. Plasma cells also contained membrane-bound vacuoles, suggesting inhibition of the immunoglobulin production.

Adult↗