[The diagnostic value of the Babkin reflex].
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Biomedical subjects
Publications and source records attributed to J Abe.
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Branched cyclomalto-oligosaccharides (cyclodextrins) were synthesised from cyclomalto-oligosaccharides and maltose or maltotriose through the reverse action of Pseudomonas isoamylase. The reaction rate was greater with maltotriose than with maltose, and with increasing size of the cyclomalto-oligosaccharide (cG6 less than cG7 less than cG8). Maltotriose is effective as both a side-chain donor and acceptor, and three isomers of 6-O-alpha-maltotriosylmaltotriose (branched G6) were formed through mutual condensation, but maltose was effective only as a side-chain donor. Each branched cyclomalto-oligosaccharide and G6 was purified by liquid chromatography, and their structures were determined by chemical, enzymic, and 13C-n.m.r. spectroscopic analyses.
Xeroderma pigmentosum is an unusual neurocutaneous disorder. Recent studies have classified patients with xeroderma pigmentosum into 10 groups by somatic cell hybridization methods. In this report we describe 32 patients with Group A xeroderma pigmentosum, including 1 patient with an atypical case, who were assessed for neurological complications. Of these patients, 17 had microcephaly, 13 short stature, and 21 mental retardation. In patients over 7 years of age, sensorineural deafness and spinocerebellar signs such as nystagmus, dysarthria, tremor, and ataxia were frequently observed; no patients below 7 years of age had such neurological complications. Electroencephalographic studies revealed abnormal slow and low voltage background activity. Two patients had focal abnormal discharges, one of whom developed versive seizures. Cranial computed tomographic scans revealed abnormalities, including ventricular dilatation, cerebral atrophy, cerebellar and brainstem atrophy, and cranial bone thickening. A patient with an atypical case of Group A xeroderma pigmentosum had less skin and neurological involvement, and higher levels of postultraviolet colony-forming ability and host cell reactivation than did a typical Group A case. It is possible that these less severe cytological findings are responsible for the less severe skin lesions and neurological complications noted clinically.
By means of the Doppler ultrasound method, the cerebral blood flow (CBF) was assessed in 21 children with epilepsy undergoing treatment with adrenocorticotrophic hormone (ACTH). The maximum reduction in the internal carotid velocity, as an index of CBF during therapy, was about 35 percent compared with the values before therapy. Furthermore, sequential computed tomography (CT) examinations of the same subjects were performed to evaluate the change in the area of the intracranial brain parenchyma during therapy. The maximum reduction in the parenchymal area during therapy was about 10 percent. This corresponds to a 20 percent reduction in CBF according to Poiseuille's law, however, the remaining reduction in CBF demonstrated by velocity measurement cannot be explained only by that mechanical vascular factor. From these findings, it is concluded that in order to elucidate the mechanism of the CBF reduction, physiological factors such as changes in metabolism during therapy should also be evaluated in addition to the mechanical and physical causes.
Seven proteinase inhibitors were isolated from winged bean seeds by ion-exchange chromatographies. These inhibitors had molecular weights of around 20,000, included four half-cystine residues, and were Kunitz-type inhibitors. Two (WTI-2 and 3) inhibited bovine trypsin strongly and four (WCI-1, 2, 3, and 4) inhibited bovine alpha-chymotrypsin, but in different ways. One mole of WCI-2 or -3 could inhibit 2 mol of alpha-chymotrypsin. The remaining inhibitor (WTCI-1) could bind both bovine trypsin and alpha-chymotrypsin at the molar ratio of 1:1, but not simultaneously. All four chymotrypsin inhibitors cross-reacted with rabbit anti-WCI-3 serum, while the other inhibitors did not.
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The effect of 1 alpha,25-dihydroxyvitamin D3 [1 alpha,25(OH)2D3], the active form of vitamin D3, on the relation between cell growth and differentiation was examined in a murine myelomonocytic leukemia cell line WEHI-3 which is known to produce high levels of interleukin-3. 1 alpha,25(OH)2D3 markedly inhibited proliferation of WEHI-3 cells in a time- and dose-dependent manner. Flow cytometric analysis of the cell cycle by a double staining method using fluorescein isothiocyanate-conjugated anti-bromodeoxyuridine and propidium iodide revealed that 1 alpha,25(OH2D3 increased the proportion and the number of cells accumulating in the G0-G1 phase and decreased those in the S phase. The phenotype of the surface antigens of the cells was of the T-cell lineage, but the cells became positive in macrophage-associated surface markers (Mac-1 and Ia) after treatment with 1 alpha,25(OH)2D3. The vitamin induced phagocytic activity, appearance of Fc receptors, nitroblue tetrazolium-reducing activity, and nonspecific esterase activity, indicating that the vitamin induces the cells to differentiate into macrophages. Furthermore, 1 alpha,25(OH)2D3 inhibited interleukin-3 production by the cells in a time- and dose-dependent manner. These results suggest that 1 alpha,25(OH)2D3 inhibits proliferation of WEHI-3 cells by blocking transition of the cells from the G0-G1 to the S phase, resulting in induction of the G0-G1-arrested cells to differentiate into macrophages. The relation between the suppression by 1 alpha,25(OH)2D3 of cell growth and interleukin-3 production remains to be elucidated in the future.
We attempted to use CDDP for patients with advanced cancers of the gastrointestinal system by intra-arterial infusion, giving consideration to the side effects of CDDP. Of 19 cases treated with CDDP, 17 cases were evaluable. These 17 cases comprised 10 cases of gastric cancer, 1 of pancreatic cancer and 6 of colon cancer. Therapeutic effects were as follows. According to the criteria for judgement of solid cancers, there were 10 evaluable cases which comprised 1 case of PR, 2 of MR, 4 of NC and 3 of PD. According to the criteria for judgement of malignant ascites, there were 6 evaluable cases which comprised 4 effective and 2 non-effective cases. As to side effects, nausea and vomiting were observed in 10 cases, numbness in 1, fever in 1 and aggravation of diabetes in 2. From the above results, intra-arterial infusion of CDDP is considered to be an effective method for the treatment of advanced cancers of the gastrointestinal system, especially of malignant ascites.
Cerebral blood flow was assessed by ultrasound in 12 children with intractable epilepsy who were treated with ACTH. The average maximal blood velocity (A/L) and end-diastolic blood velocity (d) of the internal carotid artery were measured, before, during and after ACTH therapy in each subject. The right and left mean A/L and d values were significantly decreased during ACTH therapy, and these values returned to the previous levels after the treatment. Cerebral function in children treated with ACTH may be affected by a decrease in cerebral blood flow.
The H-reflex was studied in 53 children with cerebral palsy, and the results were compared with those obtained for 56 normal control subjects. Pairs of identical stimuli were delivered and the time course of recovery of the amplitude of the H-reflex was determined. Recovery of the H-reflex was increased in the normal control group aged 0 to 12 mos compared to that in the normal control group aged 1 to 9 yrs, especially at interstimulus intervals from 100 to 800 msec. In children with spasticity, marked recoveries of the H-reflex were observed in both age groups, 0 to 12 mos and 1 to 9 yrs, compared to those in normal controls. In the cases of athetosis, recovery of the H-reflex was also marked. In children with ataxia, it was not pronounced and similar to that in normal controls. From these findings, an H-reflex study was considered to be useful for evaluation of central nervous system function in childhood.
Neutral oligosaccharides were isolated from urine of an adult patient with glycogen storage disease type II, a deficiency of lysosomal acid alpha-glucosidase, by chromatography on columns of activated charcoal, Dowex 50 X 2 and Dowex 1 X 2. Total neutral oligosaccharides in the urine of the patient were increased about 5-fold as compared with those in normal controls. The most accumulated oligosaccharide was separated by Bio-Gel P-2 column chromatography, and finally purified by paper chromatography. Based on various studies, including carbohydrate analysis, chemical ionization mass spectrometry, fast atom bombardment mass spectrometry, degradation by glucoamylase and isopullulanase, and methylation analysis, the structure of this oligosaccharide was deduced to be Glc alpha 1----6Glc alpha 1----4Glc alpha 1----4Glc. This oligosaccharide appears to be accumulated in urine of the patient with acid alpha-glucosidase deficiency as an end product of the hydrolysis of glycogen.
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Scanning electron microscopy of Sarcoptes scabiei var. hominis was carried out on a single mite. The ultrastructural morphology of Sarcoptes is illustrated.
Prostaglandin E1 (PGE1) was administered to 27 infants in whom pulmonary or systemic blood flow was entirely or significantly dependent upon the patency of the ductus arteriosus. In 12 patients with pulmonary atresia or severe pulmonary stenosis, PGE1 infusion was followed by an improvement in hypoxemia and acidemia (group I). In 2 patients with left ventricular outflow-tract obstruction, PGE1 infusion was followed by an improvement in arterial blood pressure, peripheral perfusion and urine output (group II). In 5 patients with d-transposition of the great arteries and intact ventricular septum who had persistent severe hypoxemia after creation of an interatrial communication, PGE1 infusion improved the arterial oxygenation with dilatation of the ductus arteriosus (group III). Seven patients (3 of group I, 2 of group II and 2 of group III) failed to respond to PGE1. There were no fatal side effects. It is concluded that PGE1 therapy is highly effective in stabilizing pre-operative conditions of infants with ductus-dependent congenital heart disease.
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Seven hybridoma clones producing monoclonal antibodies (HBJ8, HBJ27, HBJ67, HBJ71, HBJ98, HBJ104 and HBJ127) were selected from hybridomas prepared by fusion between P3 X Ag8.653 mouse myeloma cells and spleen cells of a BALB/c mouse immunized with T24 human urinary bladder cancer cells, and the binding specificity of, and the molecular characters of the antigens defined by, these monoclonal antibodies were examined. The cell-surface antigens detected with these monoclonal antibodies from T24 bladder cancer cells were as follows: 1) HBJ27-defined gp85 antigen common in all human cells or tissues tested, 2) HBJ98- or HBJ127-defined gp125 antigen distributed in all epithelial and non-epithelial human tumor cell lines tested and in basal layers of the skin or esophagus, proximal tubules of the kidney, and crypts of the gastric and intestinal mucosa, 3) HBJ8-defined gp(40/90) and HBJ67-defined gp83 antigens distributed in a characteristic portion of epithelial tumor cell lines, 4) HBJ71-defined antigen of protein nature and HBJ104-defined antigen of unknown character, both being detected from immunizing T24 cells and a few epithelial tumor cell lines. All these monoclonal antibodies could bind with certain portions of fresh bladder cancer tissues from patients.