Infantile onset myasthenia gravis with MuSK antibodies.
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Biomedical subjects
Publications and source records attributed to T Noda.
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The effect of disorder on the superconducting transition temperature T(c) of cuprate superconductors is examined. Disorder is introduced into the cation sites in the plane adjacent to the CuO2 planes of two single-layer systems, Bi(2.0)Sr(1.6)Ln(0.4)CuO(6+delta) and La(1.85-y)Nd(y)Sr0.15CuO4. Disorder is controlled by changing rare earth (Ln) ions with a different ionic radius in the former, and by varying the Nd content in the latter with the doped carrier density kept constant. We show that this type of disorder works as weak scatterers in contrast to the in-plane disorder produced by Zn, but remarkably reduces T(c), suggesting novel effects of disorder on high-T(c) superconductivity.
BACKGROUND: This study aims to demonstrate the relevance of temperament to job stress. METHOD: The subjects were 848 male and 366 female Japanese company employees. Temperament Evaluation of Memphis, Pisa, Paris and San Diego-Autoquestionnaire version (TEMPS-A) and Munich Personality Test (MPT) were administered to assess temperaments, and the NIOSH Generic Job Stress Questionnaire (GJSQ) to assess job stress. We used hierarchical multiple linear regression analysis in order to demonstrate whether temperament variables added any unique variance after controlling the effects of other predictors such as gender, age and job rank. RESULTS: In all subscales of the GJSQ, temperament predicted a large share of the variance in job stress. Remarkably, for interpersonal relationship stressors, the temperament variables added greater variance than that predicted by gender, age and job rank. Summary of the hierarchical linear regression analysis showed that the irritable temperament was associated with the most prominent vulnerability, followed by cyclothymic and anxious temperaments. The schizoid temperament had difficulty in the area of social support. On the other hand, the hyperthymic temperament displayed significant robustness in facing most job stressors; the melancholic type showed a similar pattern to a lesser degree. LIMITATION: The findings may be different in a clinical Japanese sample, or a cohort of healthy employees from a different cultural background. CONCLUSIONS: Temperament influences job stress significantly-indeed, it impacts on such stress with greater magnitude than age, gender and job rank in most areas examined. Temperament influences interpersonal relationship stressors more than workload-related stressors. Interestingly, in line with previous clinical and theoretical formulations, the hyperthymic and melancholic types actually appear to be "hyper-adapted" to the workplace.
Five comminuted and displaced fractures of the distal metaphysis of the radius were treated by a technique of minimally invasive plate osteosynthesis (MIPO) with the aim of minimizing soft tissue damage and devascularization of the fracture fragments. This technique used the small AO T-shaped locking compression plate (AO LCP T-plate) and left the pronator quadratus intact. Radial inclination, palmar tilt, and ulnar variance were restored without loss of reduction in all five cases and the fractures healed at an average of 10 weeks, with good to excellent clinical outcomes.
In the present study, we investigated whether Akt is involved in insulin-like growth factor-I (IGF-I)-stimulated activity of alkaline phosphatase, a marker of mature osteoblast phenotype, in osteoblast-like MC3T3-E1 cells. IGF-I induced the phosphorylation of Akt in these cells. Akt inhibitor significantly suppressed the IGF-I-stimulated alkaline phosphatase activity. The phosphorylation of Akt induced by IGF-I was reduced by the Akt inhibitor. LY294002 and wortmannin, inhibitors of phosphatidylinositol 3-kinase, significantly suppressed the IGF-I-induced alkaline phosphatase activity. The phosphorylation of Akt induced by IGF-I was markedly reduced by LY294002 and wortmannin. These results strongly suggest that phosphatidylinositol 3-kinase/Akt plays a role in the IGF-I-stimulated alkaline phosphatase activity in osteoblasts.
The main metabolites of o-xylene in urine are o-methylhippuric acid, o-toluic acid, o-toluic acid glucuronide, 3,4-dimethylphenol, 3,4-dimethylphenol conjugates and o-xylylmercapturic acid. The urinary excretion of o-toluic acid, o-toluic acid conjugates and o-xylene were increased by the prior administration of sodium benzoate. Conversely, the amounts of o-methylhippuric acid, 3,4-dimethylphenol conjugates and o-xylylmercapturic acid decreased by sodium benzoate pretreatment. In addition, the urinary excretion of o-methylhippuric acid was delayed by the pretreatment. The percentages of urinary excretion of the o-xylene metabolites were substantially changed by the pretreatment with sodium benzoate. These results therefore highlight a potential interaction of an air pollutant with a food additive, an interaction that remains to be established in man.
Many cases demonstrating the oral manifestations of Langerhans cell histiocytosis (LCH) have been reported; however, tooth development in jaw lesions has rarely been mentioned. This paper reports the case of a 3-year-old Japanese girl with LCH suffering from multiple osteolytic lesions of the skull and jaw bones. She was referred to our paediatric clinic because of swelling of the mucogingival folds in the upper and lower primary molar regions. The patient responded well to steroid therapy and the osteolytic lesions resolved. There was an accompanying development of permanent tooth germs included in the lesions. Langerhans cell histiocytosis in children usually has a long-term clinical course and paediatric dentists should be involved with oral health care for affected patients.
Retrospective investigations of odontomas in Japanese children and one recurrent case were carried out. Thirty-nine cases of odontoma in 38 children were treated in the Paediatric Dentistry Clinic of Niigata University Dental Hospital between September 1979 and December 2002. The patients consisted of 23 males and 15 females and their ages ranged from 1 year 2 months to 14 years old. The chief complaints were delayed tooth eruption in 19 cases (five: primary teeth, 14: permanent teeth), retention of primary teeth in 11, incidentally found on the radiographic examination in eight cases, and swelling of the jaw in one case. Thirty-four cases (87%) were associated with tooth eruption disturbances. The most frequently affected region was the maxillary anterior region. Treatment consisted of surgical removal of odontomas in all cases, after which if the impacted teeth did not erupt, exposure of the crown and/or orthodontic traction was performed. Pathological diagnoses were compound odontoma in 30 cases, complex odontoma (n = 7), and compound and complex odontoma (n = 2). A retrospective study of the radiographs revealed the developing process of odontomas in four cases and odontoma disturbed tooth eruption since the early uncalcified developing stage. A recurrent case was a boy aged 6 years 5 months in whom the first surgical removal of odontoma was performed at the age of 1 year 8 months. Recurrence of an odontoma is very rare, but in very young children odontomas are in the early developing stages, containing uncalcified portions, so it is important to perform periodical observations until the succedaneous teeth erupt.
The aim of the study was to detect patterns of spatial-current distribution in the late QRS and early ST-segments that distinguish Brugada-syndrome cases from complete right-bundle branch block (CRBBB). Magnetocardiograms (MCGs) were recorded from Brugada-syndrome patients (n = 6), CRBBB patients (n = 4) and the members of a control group (n = 33). The current distributions at six time points from Q-onset were estimated by producing current-arrow maps (CAMs). The angle of the current arrow of maximum amplitude at each time point was calculated. In the Brugada cases, the characteristic ST elevation was seen above the upper right chest, and abnormal currents appeared to be present in the right-ventricular outflow tract (RVOT). The angles of the abnormal arrows were -78 degrees +/- 51 degrees at 100 ms and -50 degrees +/- 61 degrees at 110 ms. In the cases of CRBBB, wide S- and R-waves were recorded above the upper right and lower right chest, respectively. The angles of the abnormal arrows for CRBBB were 152 degrees +/- 19 degrees at 100 ms, 159 degrees +/- 20 degrees at 110 ms, and 157 degrees +/- 19 degrees at 120 ms. The findings suggest that an abnormal current from the RVOT to the upper left chest may be a feature of the Brugada syndrome, and that the direction of this current is completely different from that seen in CRBBB.
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We have investigated the pressure effect on T(c) and the Hall coefficient in the static stripe-ordered phase of La(1.48)Nd(0.4)Sr(0.12)CuO(4) crystal under hydrostatic pressure. We found a dramatic change of the Hall coefficient and an abrupt increase of T(c) at low pressure of about 0.1 GPa. The results are indicative of a transition from one- to two-dimensional charge transport, associated with the suppression of low-temperature-tetragonal (LTT) phase. From the uniaxial pressure measurements it turns out that the observed critical change is induced primarily due to the in-plane compression of the CuO(2) planes which would make the pinning potential of the LTT lattice distortions weaker.
Previous studies have shown that intracellular glutathione, a ubiquitous intracellular thiol, is related to cell proliferation and that cysteine or its disulphide form, cystine, also induces cell proliferation. Cysteine is a thiol containing amino acid and a rate-limiting precursor of glutathione. Therefore, it is still unresolved as to whether the proliferative effect of cysteine or cystine is entirely mediated by a change in the intracellular glutathione status. The objective of this study was to delineate the relationship among cysteine/cystine (thereafter referred to as cyst(e)ine), intracellular glutathione and cell proliferation in the human colon cancer CaCo-2 cell line. CaCo-2 cells were cultured in cyst(e)ine-free Dulbecco's Modified Eagle Medium without serum, and treated with 200 microm cysteine and/or 200-400 microm cystine for 24 h. In the presence of DL-buthionine-[S, R]-sulfoximine (BSO), a glutathione synthesis inhibitor, exogenously administered cyst(e)ine did not change the intracellular glutathione content, but increased the intracellular cysteine as well as cystine level. Addition of exogenous cyst(e)ine following 5 mm BSO treatment significantly increased cell proliferation as measured by 3H-thymidine incorporation and protein content. Cell cycle analyses revealed that cyst(e)ine promoted cell progression from the G1 phase to the S phase. Correspondingly, cyst(e)ine treatment induced expression of cyclin D1 and phosphorylation of retinoblastoma protein (Rb). In conclusion, these data indicate that both cysteine and cystine have proliferative effects in CaCo-2 cells independent of an increase in intracellular glutathione. Induction of cyclin D1, phosphorylation of Rb, and subsequent facilitation of G1-to-S phase transition were involved in the proliferative effect of exogenous cyst(e)ine.
A rare case of fusion between maxillary primary central incisors and supplemental teeth occurring bilaterally, accompanied by succedaneous supernumerary teeth, is presented. The patient was an 8.5-year-old Japanese boy. Intraoral examination revealed fusion of left and right maxillary primary central incisors to supplemental teeth, which had labial and lingual grooves. The maxillary primary lateral incisors were present. Radiographs showed that the fused teeth had separate roots, pulp chambers and root canals. There were two impacted supernumerary teeth and eruption of the permanent maxillary central incisors was delayed. Treatment was performed and the fused primary teeth and the impacted supernumerary teeth were extracted. After 6 months observation, surgical exposure of the two crowns of the permanent maxillary central incisors was performed. The teeth began to erupt and have since been kept under observation.
We present a rare case of an infant's palatal polyp associated with an impacted supernumerary tooth. We have previously reported three cases of palatal polyps in infants [1]. In one case, after surgical removal of the palatal polyp at the age of 1 year and 8 months, the lesion subsequently began swelling. A periapical radiograph at the age of 2 years and 9 months revealed a small calcified mass in the maxillary left incisor region. The swelling was kept under observation, the calcified mass developed gradually and was removed surgically at the age of 5 years and 5 months. The removed calcified mass was clinically diagnosed as an impacted supernumerary tooth and this was confirmed histologically. Histological findings did not indicate any relationship between the palatal polyp and the impacted supernumerary tooth.
OBJECTIVE: The aim of this study was to evaluate dental fear and cooperation during dental treatment using the Children's Fear Survey Schedule-Dental Subscale (CFSS-DS). METHODS: From the behaviour assessment of regular patients of the Pediatric Dental Clinic in Niigata University Dental Hospital (aged 5-12 years old), the patients were classified into two groups: uncooperative and cooperative. CFSS-DS questionnaires were sent to a total of 295 patients, 186 of which were returned, 61 from the uncooperative group and 125 from the cooperative group. RESULTS: In the uncooperative and the cooperative groups, the CFSS-DS mean total scores were 31.79 and 24.02 (P < 0.05), respectively, and the ratios of the patients whose scores were equal to or more than 35 were 36.1 and 11.2%, respectively. Fear of 'injections' and 'the dentist drilling' had high scores in both groups. CONCLUSIONS: The results indicated that there are many uncooperative patients with low dental fear and cooperative patients with high dental fear.
AIMS: Differences in the sensitivity of the genotype of the congenital long QT syndrome to sympathetic stimulation have been suggested. This study compared the influence of sympathetic stimulation on continuous corrected QT (QTc) intervals between LQT1, LQT2 and LQT3 forms of the congenital long QT syndrome. METHODS AND RESULTS: We recorded a 12-lead electrocardiogram continuously before and after bolus injection (0.1 microg x kg(-1)) of epinephrine followed by continuous infusion (0.1 microg x kg(-1) min(-1)) in 12 LQT1, 10 LQT2, 6 LQT3, and 13 control patients. The QT intervals and previous RR intervals of all beats were measured semi-automatically, and the QTc intervals of all beats were calculated by Bazett's method. The dynamic response of the RR interval to epinephrine was no different between the four groups. The QTc was prolonged remarkably (477+/-42 to 631+/- 59 ms; P<0.0005, % delta prolongation =+32%) as the RR was maximally decreased (at peak of epinephrine), and remained prolonged at steady state conditions of epinephrine (556+/-56 ms; P<0.0005 vs baseline, +17%) in LQT1 patients. Epinephrine also prolonged the QTc dramatically (502+/-23 to 620+/-39 ms; P<0.0005, +24%) at peak of epinephrine in LQT2 patients, but this shortened to baseline levels at steady state (531+/-25 ms; P=ns vs baseline, +6%). The QTc was much less prolonged at peak of epinephrine in LQT3 (478+/-44 to 532+/-41 ms; P<0.05, +11%) and controls (394+/-21 to 456+/-18 ms; P<0.0005, +16%) than in LQT1 and LQT2 patients, and shortened to the baseline levels (LQT3; 466+/-49 ms, -3%, controls; 397+/-16 ms, +1%; P=ns vs baseline) at steady state. CONCLUSION: Our data suggest that the dynamic response of ventricular repolarization to sympathetic stimulation differs between LQT1, LQT2 and LQT3 syndromes, and may explain why the trigger of cardiac events differs between the genotypes.
To investigate the functional role of Ca(v)2.3 channel in glucose homeostasis, we performed in vivo glucose tolerance and insulin tolerance tests together with stress-induced glucose release tests using mice deficient in Ca(v)2.3 channel (Ca(v)2.3-/-). The Ca(v)2.3-/- mice were significantly heavier than wild-type mice. In glucose tolerance and insulin tolerance tests, Ca(v)2.3-/- mice showed a significantly higher blood glucose level compared to wild-type mice. However, stress-induced blood glucose changes in Ca(v)2.3-/- mice were similar to those in wild-type mice. These results suggest that Ca(v)2.3 channel plays a role in glucose homeostasis by reducing insulin sensitivity and that Ca(v)2.3-/- mice exhibit symptoms resembling non-insulin-dependent diabetes mellitus.
In mammalian male germ-line cells, low-voltage-activated (LVA) Ca(2+) current has been identified and its electrophysiological properties have been studied. To investigate whether alpha(1)2.3 (alpha(1E)) subunit of the voltage-dependent Ca(2+) channel codes for the LVA current, whole-cell patch clamp and following reverse transcription-polymerase chain reaction (RT-PCR) experiments were performed in pachytene spermatocytes from Ca(v)2.3+/+ and Ca(v)2.3-/- mice. Whole-cell current in acutely dissociated pachytene spermatocytes from Ca(v)2.3+/+ and Ca(v)2.3-/- mice displayed a typical profile of LVA Ca(2+) currents and kinetics with no significant differences. Single-cell RT-PCR revealed the expression of Cacna1g in the pachytene spermatocytes from Ca(v)2.3+/+ and Ca(v)2.3-/- mice in which LVA Ca(2+) currents were actually recorded. These results suggest that the Ca(v)2.3 channel makes no detectable contribution to the LVA Ca(2+) current in the pachytene spermatocyte. Instead, Ca(v)3 family such as Ca(v)3.1 may be the likely candidates responsible for the LVA currents in pachytene spermatocytes.