[Mannosidosis].
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Bone Marrow Transplantation↗
Biomedical subjects
S Yamaguchi
Publications and source records attributed to S Yamaguchi.
[A case of limited Wegener granulomatosis with hypereosinophilia].
A 51-year-old female was admitted to Nagano Matsushiro General Hospital because of fever, cough and dyspnea on exertion. Her laboratory data revealed leukocytosis with hypereosinophilia, a high erythrocyte sedimentation rate and c-reactive protein. Chest radiography revealed an infiltration shadow with a cavity in the right upper lobe. A lung abscess was diagnosed and antibiotics were administered. Laboratory results showed improvement, but chest radiography continued to show cavities. She was admitted to our hospital because of fever, left pleural effusion and progression of cavities on chest radiographs. She showed no abnormalities of the upper airway or kidney, and was negative for c-antineutrophil cytoplasmic antibody (c-ANCA). Because a positive c-ANCA was seen on day 8 of hospitalization, L-type limited Wegener granulomatosis (WG) was diagnosed according to Gross et al. Prednisolone (PSL) was administered, which improved the anemia, eosinophilia and the cavities. On day 7 of PSL administration, of the left pneumothorax occurred as a complication caused by perforation of the left chest cavity, but her clinical course was good after a cavernectomy was performed. Some studies have reported that limited WG shows a negative c-ANCA, and that antibiotic therapy improves inflammation. The L-type of limited WG revealed a low-grade positive ratio and titer of c-ANCA. Moreover, L-type limited WG responds well to therapy. We therefore selected PSL administration only against L-type limited WG. We have reported L-type limited WG with eosinophilia and the negative effects of c-ANCA at an early clinical stage.
Antibodies, Antineutrophil Cytoplasmic↗
[A Lambert-Faton myasthenic syndrome and subacute cerebellar degeneration with a favorable clinical course after resection of small-cell lung cancer].
A case of Lambert-Eaton myasthenic syndrome (LEMS) and subacute cerebellar degeneration (SCD) was associated with small-cell lung cancer (SCLC). The patient, a 52-year-old man, who had noticed impotence one year previously, began to have ataxic gait, scanning speech and thirst progressing for 3 months, followed by weakness of the lower limbs, bilateral blepharoptosis, and double vision. Electromyographic studies showed low amplitude of compound muscle action potential (CMAP) and waxing phenomenon in high frequency stimulation of the ulnar nerve. A chest x-ray showed a mass lesion in the left hilar region, and small cell lung cancer was diagnosed on the basis of biopsy specimens. Anti-voltage-gated calcium channel (VGCC) antibody was positive. Anti-Yo and anti-Hu antibodies were negative. The patient was treated by lobectomy and chemotherapy, which resulted in improvement in the LEMS and SCD. Anti-VGCC antibody, the CMAP amplitude, and waxing phenomenon were improved. Operable cases of SCLC are rare. But we propose that anti-neoplastic treatment including resection of the tumor is the first choice for the treatment of paraneoplastic syndrome associated with SCLC.
Autoantibodies↗
Microvascular vasodilatory responses to electric acupuncture in rat brain under acute hemorrhagic hypotension.
The present experiment attempted to evaluate the effect of electrical acupuncture on the cerebral microcirculation in anesthetized rats, using fluorescence videomicroscopy. Changes in the pial arteriolar diameter under acute hemorrhagic hypotension were examined quantitatively. The present results suggest that acupuncture may be effective in improving the cerebral microcirculation in hemorrhagic hypotension.
Anesthesia, General↗
Microcirculatory characterization of cerebral angiogenesis in mice using intravital videomicroscopy.
The present study investigated microcirculatory characteristics of the cerebral neovasculature induced in mice, using basic fibroblast growth factor (bFGF) and platelet derived growth factor (PDGF). The nylon-mesh sandwich (collagen gel/growth factor in bovine serum albumin embedded in between two nylon-mesh pieces) was used to induce angiogenesis. After different days of incubation, the observations of neocapillaries were done on the upper surface of the nylon-mesh, using fluorescence video-microscopy. The neocapillary diameter, red cell velocity, and the neocapillary density were evaluated based on the video-image. The neocapillaries were visible on the upper surface of the mesh on the 6th day after the incubation, and red cells started to flow from the day 7. The neocapillary red cell velocity increased with days after incubation, but its level was less than that of the pre-existing capillaries. The neocapillary diameter decreased as the neocapillaries got matured. The neocapillary density was dependent on the doses of bFGF and PDGF. The neocapillary diameter did not alter with the higher concentration as well as with different growth factors. Both bFGF and PDGF showed an increase in red cell velocity at high concentration.
Animals↗
Microcirculatory responses to repeated embolism-reperfusion in cerebral microvessels of cat: a fluorescence videomicroscopic study.
Intra-carotid injections of degradable starch microspheres (DSMs) can induce DSM embolism-reperfusion in the level of cerebral arterioles. Vascular responses of cerebral arterioles to repeated DSM embolism (ischemia)-reperfusion were examined when DSMs were injected twice through a carotid artery with a time interval of 30 min. Arteriolar diameter was measured from images of arterioles filled with rhodamine-B isothiocyanate dextran and red cell velocity was measured with a dual window technique using FITC-labeled red cells as a flow tracer. DSM embolization induced ischemia (flow reduction including stasis) for approximately 10 min in the level of microvessels (arterioles). Cerebral arterioles began to dilate immediately after embolism induced by the DSM injection and vasodilation was sustained until reperfusion. After reperfusion the arteriole began to constrict and the arteriolar diameter returned to the initial diameter level at approximately 20 min after the DSM injection. The arteriolar diameters for the second DSM embolism showed a similar response to those for the first embolism in 7 out of 8 cats. It can be concluded that the vascular response of cerebral arterioles to the second embolism-reperfusion could not be affected by the first embolism-reperfusion.
Animals↗
Photic induction of mPer1 and mPer2 in cry-deficient mice lacking a biological clock.
Mice lacking mCry1 and mCry2 are behaviorally arrhythmic. As shown here, cyclic expression of the clock genes mPer1 and mPer2 (mammalian Period genes 1 and 2) in the suprachiasmatic nucleus and peripheral tissues is abolished and mPer1 and mPer2 mRNA levels are constitutively high. These findings indicate that the biological clock is eliminated in the absence of both mCRY1 and mCRY2 (mammalian cryptochromes 1 and 2) and support the idea that mammalian CRY proteins act in the negative limb of the circadian feedback loop. The mCry double-mutant mice retain the ability to have mPer1 and mPer2 expression induced by a brief light stimulus known to phase-shift the biological clock in wild-type animals. Thus, mCRY1 and mCRY2 are dispensable for light-induced phase shifting of the biological clock.
Animals↗
Saffron extract prevents acetaldehyde-induced inhibition of long-term potentiation in the rat dentate gyrus in vivo.
We have previously found that alcohol extract of Crocus sativus L. (CSE), commonly known as saffron, prevents ethanol-induced inhibition of hippocampal long-term potentiation (LTP). In the present study, CSE (250 mg/kg, p.o.) was also effective in preventing acetaldehyde-induced inhibition of LTP in the dentate gyrus of anesthetized rats. These results suggest that CSE can prevent aversive effects induced by ethanol and its metabolite acetaldehyde.
Acetaldehyde↗
Fibrinogen Niigata with impaired fibrin assembly: an inherited dysfibrinogen with a Bbeta Asn-160 to Ser substitution associated with extra glycosylation at Bbeta Asn-158.
A novel BbetaAsn-160 (TAA) to Ser (TGA) substitution has been identified in fibrinogen Niigata derived from a 64-year-old asymptomatic woman, who is heterozygotic for this abnormality. The mutation creates an Asn-X-Ser-type glycosylation sequence, and a partially sialylated biantennary oligosaccharide was linked to the BbetaAsn-158 residue. The functional abnormality was attributed to delayed lateral association of normally formed double-stranded protofibrils based on normal cross-linking of fibrin gamma-chains and tissue-type plasminogen activator-catalyzed plasmin generation by polymerizing fibrin monomers. Enzymatic removal of all the N-linked oligosaccharides from fibrinogen Niigata accelerated fibrin monomer polymerization that reached the level of untreated normal fibrin monomers, but the thrombin time was prolonged from 18.2 seconds to 113 seconds (normal: 11.2 seconds to 8.9 seconds). By scanning electron micrographic analysis, Niigata fibrin fibers were found to be more curvilinear than normal fibrin fibers. After deglycosylation, Niigata fibers became straight being similar to untreated normal fibrin fibers, whereas normal deglycosylated fibrin appeared to be less-branched than untreated normal or deglycosylated Niigata fibrin. Although normal and Niigata fibrins were similar to each other in permeation and compaction studies, deglycosylated normal and Niigata fibrins had much higher permeability and compaction values, indicating that deglycosylation had brought about the formation of more porous networks. The enzymatic deglycosylation necessitates an Asn to Asp change at position Bbeta-158 that is responsible for reducing the fiber thickness because of either local repulsive forces or steric hindrance in the coiled-coil region.
Amino Acid Substitution↗
Three-Phase Behavior in a Water/C(12)EO(8)/Propanol/Cyclohexane/Heptane System.
We studied the three-phase behavior and dissolution behavior of propanol (C(3)OH) in a water/C(12)EO(8)/C(3)OH/cyclohexane (c-C(6))/heptane (C(7)) system at 35 and 45 degrees C. Without C(12)EO(8), a three-phase region (IIIa) consisting of C(3)OH, aqueous (W), and oleic (O) phases exists between R(oil) (c-C(6)/c-C(6) + C(7)) = 0.2 and 0.3 (w/w) above 35 wt% C(3)OH. The C(3)OH phase originates from the W phase and becomes identical to the O phase with increasing R(oil). In the presence of C(12)EO(8), the three-phase region expands below 20 wt% C(3)OH. The surfactant phase behaves in two ways according to the role of C(3)OH. When R(oil) < 0.2, the D(p) phase forms a closed-loop miscibility gap with a W phase. The added C(3)OH acts as a lipophilic cosurfactant and a water-soluble cosolvent. When R(oil) > 0.3, a microemulsion (D) phase changes from water-rich to oil-rich in a chiral three-phase body. Most C(3)OH added acts as a lipophilic cosurfactant at R(oil) = 1. The two types of three-phase behavior are transformed into each other via region IIIa. C(3)OH cooperatively acts with C(12)EO(8) and a higher-order phase is formed. Copyright 1999 Academic Press.
Journal Article↗
Relationship between the clinical manifestations, computed tomographic findings and the outcome in 80 patients with primary pontine hemorrhage.
The relationship between the clinical manifestations, computed tomographic (CT) findings and the outcome in 80 patients with primary pontine hemorrhage (PPH) was analyzed to clarify factors predicting the outcome. Patients were 58 males and 22 females (60. 5+/-12.9 years old) with PPH. Correlations between the clinical manifestations, CT findings and the outcome was assessed with multivariate regression analysis. The initial level of consciousness and the transaxial size of the hematoma on CT were strongly related to the outcome. In patients with small hematoma (with the transaxial size </=20 mm), pupillary abnormalities of onset and acute hydrocephalus were related to the outcome of PPH.
Aged↗
Application of delayed extraction matrix-assisted laser desorption ionization time-of-flight mass spectrometry for analysis of sphingolipids in tissues from sphingolipidosis patients.
Sphingolipidosis is due to defects in enzymes involved in hydrolysis of sphingolipids. We analyzed sphingolipids in tissues from patients with sphingolipidosis, including Farber disease (FD, acid ceramidase deficiency), Gaucher disease (GD), Niemann-Pick disease type C (NPDC), and GM1-gangliosidosis (GM1G), using delayed extraction matrix-assisted laser desorption ionization time-of-flight mass spectrometry (DE MALDI-TOF-MS). Crude lipids were extracted from about 100 mg wet weight of autopsied tissues, including liver, spleen, cerebrum or cerebellum. After mild alkaline treatment, a sphingolipid fraction was prepared from the crude lipids and analyzed by DE MALDI-TOF-MS. The results were as follows: (a) In FD liver both the ceramide/sphingomyelin and ceramide/monohexosylceramide ratios were significantly high; (b) in both liver and spleen from a GD patient, the glucosylceramide/sphingomyelin ratio was raised; (c) in liver from a NPDC patient, the monohexosylceramide/sphingomyelin ratio was markedly low, suggesting an increase of sphingomyelin; and (d) in all tissues examined in the GM1G patient, GM1-gangliosides or asialo-GM1-gangliosides, that are undetectable in a normal control, were increased. In conclusion, sphingolipids in human tissues could be directly determined by DE MALDI-TOF-MS, with only a small amount of specimens. This method will be useful for the diagnosis and biochemical evaluation of sphingolipidosis patients.
Child, Preschool↗
Pilot study of gas chromatographic-mass spectrometric screening of newborn urine for inborn errors of metabolism after treatment with urease.
Gas chromatographic-mass spectrometric (GC-MS) techniques for urinary organic acid profiling have been applied to high-risk screening for a wide range of diseases, mainly for inborn errors of metabolism (IEM), rather than to low-risk screening or mass screening. Using a simplified procedure with urease-pretreatment and the GC-MS technique, which allows simultaneous determination of organic acids, amino acids, sugars and sugar acids, we performed a pilot study of the application of this procedure to neonatal urine screening for 22 IEM. Out of 16,246 newborns screened, 11 cases of metabolic disorders were chemically diagnosed: two each of methylmalonic aciduria and glyceroluria, four of cystinuria, and one each of Hartnup disease, citrullinemia and alpha-aminoadipic aciduria/alpha-ketoadipic aciduria. The incidence of IEM was thus one per 1477, which was higher than the one per 3000 obtained in the USA in a study targeting amino acids and acylcarnitines in newborn blood spots by tandem mass spectrometry. Also, 227 cases were found to have transient metabolic abnormalities: 108 cases with neonatal tyrosinuria, 99 cases with neonatal galactosuria, and 20 cases with other transient metabolic disorders. Two hundred and thirty-eight cases out of 16,246 neonates (approximately 1/68) were thus diagnosed using this procedure as having either persistent or transient metabolic abnormalities.
Gas Chromatography-Mass Spectrometry↗
Screening for fatty acid beta oxidation disorders. Acylglycine analysis by electron impact ionization gas chromatography-mass spectrometry.
Urinary acylglycine analysis by chemical ionization (CI) GC-MS has been utilized for more than a decade to screen of fatty acid oxidation disorders. We have developed an alternative GC-MS method involving tert.-butyldimethylsilyl derivatization and standard electron impact ionization. Using six stable isotope labeled internal standards, this method allows the biochemical diagnosis of glutaric aciduria type II and medium chain acyl-CoA dehydrogenase deficiency, and could contribute to the diagnosis of other FAO disorders when used in combination with other biochemical investigations on blood and urine. This method can be conveniently applied to GC-MS system routinely used for organic acid analysis.
Dicarboxylic Acids↗
Convulsant actions of the neurosteroid pregnenolone sulfate in mice.
Pregnenolone sulfate (PS) is an endogenous neurosteroid known to antagonize GABA(A) receptor-mediated inhibitory responses and potentiate NMDA receptor-mediated excitatory responses in vitro. To assess the actions of the steroid as a modulator of seizure susceptibility in vivo, PS (30-300 nmol) was administered intracerebroventricularly in mice. At doses of 50 to 150 nmol, PS elicited seizures characterized by head jerks, rearing and falling, severe forelimb and hindlimb clonus, opisthotonos and explosive running. The seizures increased in severity and frequency with time and eventually progressed to status epilepticus, tonic hindlimb extension and death. The doses producing convulsions in 50% (CD(50)) and 97% (CD(97)) of animals were 92 and 205 nmol, respectively. A subconvulsant dose of PS (50 nmol) significantly increased the convulsant potencies of systemically administered pentylenetetrazol (30-50 mg/kg) and NMDA (50-100 mg/kg). Systemically administered PS at doses as high as 100 mg/kg failed to induce seizures or alter the convulsant potencies of pentylenetetrazol and NMDA. Protection against PS (205 nmol)-induced seizures and lethality was conferred by the GABA(A) receptor positive allosteric modulators clonazepam and allopregnanolone, and by the NMDA receptor antagonists dizocilpine and (R)-CPP. The overall pharmacological profile suggests that the convulsant actions of PS are mediated predominantly via its effects on GABA(A) receptors, and also possibly by effects on NMDA receptors.
Animals↗
Elevated circulating levels and cardiac secretion of soluble Fas ligand in patients with congestive heart failure.
The circulating levels of soluble Fas ligand was increased in patients with advanced congestive heart failure. This study also indicates that the failing heart may contribute to the increased concentration of soluble Fas ligand in patients with congestive heart failure.
Adult↗
Protection of chickens against very virulent infectious bursal disease virus (IBDV) and Marek's disease virus (MDV) with a recombinant MDV expressing IBDV VP2.
To develop a herpes virus vaccine that can induce immunity for an extended period, a recombinant Marek's disease (MD) virus (MDV) CVI-988 strain expressing infectious bursal disease virus (IBDV) host-protective antigen VP2 at the US2 site (rMDV) was developed under the control of an SV40 early promoter. Chickens vaccinated with the rMDV showed no clinical signs and no mortality and 55% of the chickens were considered protected histopathologically after challenge with very virulent IBDV (vvIBDV), whereas all of the chickens vaccinated with the conventional IBDV vaccine showed no clinical signs and were protected. Chickens vaccinated with the CVI-988 or chickens in the challenge control showed severe clinical signs and high mortality (70-75%) and none of them were protected. Also, the rMDV conferred full protection to chickens against vvMDV just as the CVI-988 strain did, whereas 90% of the challenge control chickens died of MD. Antibody levels against IBDV and MDV following the vaccination increased continuously for at least 10 weeks. No histopathological lesions in the rMDV-vaccinated chickens and no contact transmission of the rMDV to their penmates were confirmed. These results demonstrate that an effective and safe recombinant herpesvirus-based IBD vaccine could be constructed by expressing the VP2 antigen at the US2 site of the CVI-988 vaccine strain.
Animals↗
No ethnic difference between Caucasian and Japanese hepatic samples in the expression frequency of CYP3A5 and CYP3A7 proteins.
Ethnic differences in the pharmacokinetics of nifedipine, a substrate of CYP3A, and in CYP3A7 expression have been reported. The aim of the present study was to measure the protein levels of CYP3A4, CYP3A5, and CYP3A7 and nifedipine oxidation activity in hepatic microsomes from 15 Caucasian and 15 Japanese patients for comparison between the two ethnic groups. Nifedipine oxidation activity and CYP3A4 protein level were well correlated. No significant difference between Caucasian and Japanese microsomal samples was found in nifedipine oxidation activity or in the CYP3A4 protein level. CYP3A5 was detected in 6 of 15 Caucasian samples and in 5 of 15 Japanese samples, but no ethnic difference was found in either the frequency of expression or its protein level. CYP3A7 was found in 10 of 15 Caucasian samples and in 14 of 15 Japanese samples. Although the estimated CYP3A7 protein level was higher in the Japanese than in the Caucasian samples, its protein level was much lower than that of CYP3A4. These results imply that the contribution of CYP3A5 or CYP3A7 to the purported Caucasian-Japanese ethnic difference in the overall CYP3A activity seems to be small.
Adolescent↗