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With the emergence of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), which causes coronavirus disease 2019 (COVID-19), a high-speed and convenient detection technology should be at the forefront of medical care worldwide. This study evaluated the usefulness of GeneSoC, a compact, high-speed reciprocal flow quantitative reverse transcription polymerase chain reaction system, for the detection of SARS-CoV-2. The results support the use of this system for the rapid identification of SARS-CoV-2. This approach can contribute to the strategic selection of initial management strategies for patients with COVID-19.

Duodeno-pancreatic and extrahepatic biliary tree injuries are rare in both adult and pediatric trauma patients, and due to their anatomical location, associated injuries are very common. Mortality is primarily related to associated injuries, but morbidity remains high even in isolated injuries. Optimal management of duodeno-bilio-pancreatic injuries is dictated primarily by hemodynamic stability, clinical presentation, and grade of injury. Endoscopic and percutaneous interventions have increased the ability to non-operatively manage these injuries. Late diagnosis and treatment are both associated to increased morbidity and mortality. Sequelae of late presentations of pancreatic injury and complications of severe pancreatic trauma are also increasingly addressed endoscopically and with interventional radiology procedures. However, for moderate and severe extrahepatic biliary and severe duodeno-pancreatic injuries, immediate operative intervention is preferred as associated injuries are frequent and commonly present with hemodynamic instability or peritonitis. The aim of this paper is to present the World Society of Emergency Surgery (WSES) and American Association for the Surgery of Trauma (AAST) duodenal, pancreatic, and extrahepatic biliary tree trauma management guidelines. © 2019 The Author(s).

SETTING National hospital for tuberculosis (TB) and rheumatoid arthritis (RA) in Japan. OBJECTIVE To compare two interferon-γ release assays (IGRAs), QuantiFERON®-TB Gold In-Tube (QFT) and T-SPOT®.TB (T-SPOT), in RA patients for detecting latent tuberculous infection (LTBI). DESIGN QFT and T-SPOT were conducted concurrently in 230 prospectively enrolled RA patients. RESULTS There were no active TB patients. The percentage of QFT- and T-SPOT-positive patients was respectively 8.3% and 5.7%. In patients aged ⩾60 years, these proportions were respectively 12.3% and 7.2%. The percentage of QFT positivity and T-SPOT positivity at age

The local structure of 10, 20, and 30-nm MgB2 thin films prepared by molecular beam epitaxy (MBE) is analyzed by polarized X-ray absorption fine structure (XAFS) of Mg K-edge, which is a powerful tool for structural analysis with element selectivity using a synchrotron radiation source. The structural parameters are obtained from extended X-ray absorption fine structure (EXAFS) analysis for 20-nm MgB2 film. X-ray near edge structure (XANES) analysis estimated the proportion of MgO inserted between the MgB2 film and ZnO substrate. The linear relation is found between the MgO proportion and T c.

National hospital for tuberculosis (TB) and rheumatoid arthritis (RA) in Japan.To compare two interferon-γ release assays (IGRAs), QuantiFERONQFT and T-SPOT were conducted concurrently in 230 prospectively enrolled RA patients.There were no active TB patients. The percentage of QFT- and T-SPOT-positive patients was respectively 8.3% and 5.7%. In patients aged ⩾60 years, these proportions were respectively 12.3% and 7.2%. The percentage of QFT positivity and T-SPOT positivity at age60 years was respectively 2.2% and 3.3%. After multivariate logistic analysis for QFT positivity, age ⩾60 years and TB suspected based on chest X-ray were selected as independent factors, with adjusted odds ratios of respectively 4.73 and 3.25. No factors were selected for T-SPOT positivity.QFT had a higher positivity rate. In the light of the previous estimated rate of LTBI in Japan, both IGRAs underestimate LTBI, and neither IGRA has enough capability to detect LTBI.

The present paper investigates an incentive problem (the method of providing incentives to members) in creative organizations, such as research and development departments, using a cybernetic model called the agency model. In such organizations, members appear to have their own individual objectives which are different from those defined by the organization, and so significant conflicts can exist. Therefore, the proposed model considers the objective of the individual, which is given no consideration in conventional models, and conflict between the objective of the individual and that of the organization. The principles of incentive system design (the effectiveness of the performance‐based wage system) are entirely different depending on whether the conflict exists.

IGF-I is expressed in somatotrophs, and IGF-I receptors are expressed in most somatotrophs and some corticotrophs in the mouse pituitary gland. Our recent study demonstrated that IGF-I stimulates the proliferation of corticotrophs in the mouse pituitary. These results suggested that somatotrophs regulate corticotrophic functions as well as somatotrophic functions by the mediation of IGF-I molecules. The present study aimed to clarify factors regulating pituitary IGF-I expression and also the roles exerted by IGF-I within the mouse anterior pituitary gland. Mouse anterior pituitary cells were isolated and cultured under serum-free conditions. GH (0.5 or 1 microg/ml), ACTH (10(-8) or 10(-7) M), GH-releasing hormone (GHRH; 10(-8) or 10(-7) M), dexamethasone (DEX; 10(-8) or 10(-7) M) and estradiol-17beta (e2; 10(-11) or 10(-9) M) were given for 24 h. IGF-I mRNA levels were measured using competitive RT-PCR, and GH and pro-opiomelanocortin (POMC) mRNA levels were measured using Northern blotting analysis. GH treatment significantly increased IGF-I mRNA levels (1.5- or 2.1-fold). ACTH treatment did not alter GH and IGF-I mRNA levels. IGF-I treatment decreased GH mRNA levels (0.7- or 0.5-fold), but increased POMC mRNA levels (1.8-fold). GH treatment (4 or 8 microg/ml) for 4 days increased POMC mRNA levels. GHRH treatment increased GH mRNA levels (1.3-fold), but not IGF-I mRNA levels. DEX treatment significantly decreased IGF-I mRNA levels (0.8-fold). e2 treatment did not affect IGF-I mRNA levels. GH receptor mRNA, probably with GH-binding protein mRNA, was detected in somatotrophs, and some mammotrophs and gonadotrophs by in situ hybridization using GH receptor cDNA as a probe. These results suggested that IGF-I expression in somatotrophs is regulated by pituitary GH, and that IGF-I suppresses GH expression and stimulates POMC expression at the transcription level. Pituitary IGF-I produced in somatotrophs is probably involved in the regulation of somatotroph and corticotroph functions.

The present paper describes a new approach to the agency model, which is a cybernetic model designed to analyze the situation in which an economic actor (the principal) controls the behavior of another actor (the agent), by including the factor of non‐monetary utility (intrinsic motivation). The new model addresses an incentive problem in an organization. An organization is considered to be a system constituted by the principal and the agent. Two state variables of this system, namely productivity and intrinsic motivation, are the focus of the present study. The effect of these variables on how to offer incentive is analyzed. The following results were obtained: when productivity is high and the strength of intrinsic motivation and uncertainty of output are low, organizations should introduce performance‐based incentive system.

Objectives - To determine the frequencies of spinocerebellar ataxias (SCAs) in the Kinki district, the western part of the main island of Japan. Material and methods - One hundred and forty-three families with dominantly inherited ataxia and 220 patients with apparently sporadic cerebellar ataxia were examined for the SCA1, SCA2, SCA3/Machado--Joseph disease (MJD), SCA6, SCA7, SCA8, SCA12 and dentatorubral-pallidoluysian atrophy (DRPLA) mutations. Results - Among the dominant families, SCA1 accounted for 3%, SCA2 for 4%, SCA3/MJD for 24%, SCA6 for 31% and DRPLA for 12%. Neither SCA7 nor SCA12 mutations were detected. Among the apparently sporadic patients, 15% were found to have expanded triplet repeats. Of these, the SCA6 mutation was most frequently detected. Conclusion - SCA6 is the most common SCA in the Kinki district of Japan. Comparison of our results with those from other regions of Japan and different countries shows geographic and ethnic variation in the frequency of SCAs.

A resonant circuit is expected to be used as a heating source for hyperthermia implants, because the circuit can be effectively heated by an applied ac magnetic field. In order to realize a less invasive delivery of the implant through a catheter or injection needle, its miniaturization down to 1 mm or less is essential. However, the heating temperature of the resonant circuit is reduced by downsizing of the size of the circuit. A ferrite core in a coil is employed in order to obtain higher induced voltage in the inductor, resulting in the higher temperature rise of the resonant circuit. The resonant circuit implant consisting of the core coil has been prepared. Its diameter is 0.9 mm and the implant can be delivered through an 18 G needle.

Apoptosis is found in labial salivary glands of patients with Sjogren's syndrome (SS). To analyze the pathogenesis of apoptosis in labial salivary glands of SS patients, we examined the expression of Fas Ag and Fas ligand (FasL) and TCR on T cells susceptible to anti-Fas mAbs (CH-11). Fas Ag is expressed on epithelial cells and mononuclear cells in the salivary glands as observed by an immunohistochemical method. FasL is over-expressed specifically on T cells infiltrating into the labial salivary glands as seen by an reverse transcription-PCR method. These results suggest that apoptosis in SS lips is mediated by a Fas/FasL pathway. PCR single-strand conformation polymorphism (SSCP) clearly demonstrated that more than 40% of the T cells accumulated in labial salivary glands are deleted by incubation with CH-11 for 24 h in vitro, indicating that these expanded cells are Fas sensitive. junctional sequence analysis showed that the same conserved amino acid motifs (LAGG, RLA, SLG, QGPG, PGG, GGE, RGR, KPG, AGD, and MLG) in complementarity determining region 3 (CDR3) are found in Fas-sensitive T cell clones, whereas they are not detected in Fas-resistant clones, suggesting that Fas-sensitive T cells recognize restricted T cell epitopes on autoantigens. In conclusion, the findings suggest that Fas-sensitive T cells in labial salivary glands of SS patients are generated by Ag stimulation and might function as autoreactive T cells.

To analyze the mechanism of interstitial nephritis in patients with Sjörgren's syndrome (SS), we examined the TCR repertoire of infiltration T cells in kidney, labial salivary glands, and PBLs using a PCR. The repertoire of the TCR V beta gene on infiltrating T cells from the kidneys of SS patients was more restricted than those on infiltrating T cells in labial salivary glands and PBL. The TCR V beta 2 gene was expressed predominantly in six of seven (86%) SS patients. Junctional sequences of cDNAs encoding the V beta 2 gene on infiltrating T cells in the kidneys of five SS patients showed that some of the cells expanded clonally, indicating Ag-driven stimulation rather than superantigen-induced proliferation. The same V beta 2 clones in the kidney were not detected in labial salivary glands of the same SS patients; the conserved amino acid (arginine at position 96) in the CDR3 region of the V beta 2 gene was found at a frequency of 48.0% in the kidney, whereas it was detected in only 15.4% of the clones in lips. In conclusion, these findings suggest the possibility that T cells that infiltrate the kidneys of SS patients with interstitial nephritis might recognize different autoantigens than those that infiltrate labial salivary glands.

The organization and evolution of the 0.8-Mb JH-proximal region in the human Ig VH locus were studied by mapping DNA fragments hybridized to non-repetitive intergenic probes and by determination of the content and distribution of repetitive sequences. Southern blot analysis of cloned DNA covering the 0.8-Mb region with intergenic probes allowed us to map two to seven cross-hybridizing fragments by each probe. Clusters of fragments detected by an identical set of probes appeared repeatedly within the 0.8-Mb JH-proximal region. Distantly located VH segments flanked by a cluster of DNA fragments hybridized by the same set of probes were highly homologous to each other, providing evidence for recent frequent duplication and translocation throughout the locus. DNA fragments detected by the same set of probes were orientated with the same 5' to 3' order within the cluster, suggesting little involvement of inversion upon recombination in the locus. The content of interspersed Alu and L1 sequences in the VH locus were not significantly greater than the average in the genome.

To clarify the efficacy and safety of anti-TNF-alpha therapy for intractable lupus nephritis.In nine patients with systemic erythematosus who presented with lupus nephritis resistant to steroids and immunosuppressants, 200 mg/body of infliximab was drip-infused three times. No changes were made to other treatments for three months after the start of anti-TNF-alpha therapy, and urinary findings, renal function, serum complement, anti-DNA antibody, SLE activity, and adverse events were examined for six months after the start of anti-TNF-alpha therapy.One of the nine patients developed pyelonephritis after the first infliximab injection and received no further injections. The remaining eight patients received 3 infliximab injections. Of the eight patients, urinary protein decreased after anti-TNF-alpha therapy in six patients, and the SLEDAI improved in five patients. Urinary findings and/or SLE activity improved in six patients. Of the patients whose urinary protein levels decreased after anti-TNF-alpha therapy, proteinuria recurred six months after anti-TNF-alpha therapy in one patient. After anti-TNF-alpha therapy, proteinuria and the SLEDAI improved significantly. With respect to adverse events, therapy was discontinued in one patient who developed pyelonephritis, and one patient developed decreased blood pressure due to infusion reactions. In one patient in whom the steroid dosage was increased due to poor response to anti-TNF-alpha therapy, brainstem infarction occurred four months later. In one patient, anti-DNA antibody levels increased after therapy, but none of the patients had decreased serum complement levels or increased SLE activity.In intractable lupus nephritis, anti-TNF-alpha therapy improved urinary protein levels and SLE activity. Although adverse events must be monitored cautiously, it may be possible to use anti-TNF-alpha therapy as a third-line treatment.

As the i-mode service grows in popularity, the i-mode traffic carried by the Personal Digital Cellular-Packet (PDC-P) mobile communication network is increasing rapidly. To meet this growing demand, the switching systems currently in place are being either increased in capacity or replaced by more powerful ones. To plan this effectively, it is necessary to make an accurate evaluation of the i-mode processing capacity. We have developed a new method of evaluating processing capacity using a formula for estimating the CPU occupancy by regression analysis, which takes account of the characteristics specific to the PDC-P network. This paper discusses the method of evaluating the processing capacity of switching systems used in the PDC-P mobile network. The discussion includes how to deal with differences between the CPU occupancy calculated using this technique and the actual CPU occupancy measured on a commercial switching system.

As the i-mode service grows in popularity, the i-mode traffic carried by the Personal Digital Cellular-Packet (PDC-P) mobile communication network is increasing rapidly. To meet this growing demand, the switching systems currently in place are being either increased in capacity or replaced by more powerful ones. To plan this effectively, it is necessary to make an accurate evaluation of the i-mode processing capacity. We have developed a new method of evaluating processing capacity using a formula for estimating the CPU occupancy by regression analysis, which takes account of the characteristics specific to the PDC-P network. The formula is to be applied to the commercial environment. It is important to maintain the accuracy of the evaluation above a certain level even when the traffic pattern changes greatly as a result of the future introduction of diverse services. This paper discusses the method of evaluating the processing capacity of switching systems used in the PDC-Packet mobile network. The discussion includes how to deal with differences between the CPU occupancy calculated using this technique and the actual CPU occupancy measured on a commercial switching system.

To determine the frequencies of spinocerebellar ataxias (SCAs) in the Kinki district, the western part of the main island of Japan.One hundred and forty-three families with dominantly inherited ataxia and 220 patients with apparently sporadic cerebellar ataxia were examined for the SCA1, SCA2, SCA3/Machado-Joseph disease (MJD), SCA6, SCA7, SCA8, SCA12 and dentatorubral-pallidoluysian atrophy (DRPLA) mutations.Among the dominant families, SCA1 accounted for 3%, SCA2 for 4%, SCA3/MJD for 24%, SCA6 for 31% and DRPLA for 12%. Neither SCA7 nor SCA12 mutations were detected. Among the apparently sporadic patients, 15% were found to have expanded triplet repeats. Of these, the SCA6 mutation was most frequently detected.SCA6 is the most common SCA in the Kinki district of Japan. Comparison of our results with those from other regions of Japan and different countries shows geographic and ethnic variation in the frequency of SCAs.

Tumor necrosis factor-related apoptosis inducing ligand (TRAIL) induces apoptosis of tumor cells but not normal cells; its role in normal non-transformed tissues is unknown.To evaluate the role of apoptosis mediated by TRAIL and TRAIL-receptor (TRAIL-R) system in lymphocytic sialadenitis in patients with Sjögren's syndrome.The expression of TRAIL and TRAIL-R1, 2, 3 and 4 in lymphocytic sialadenitis was examined by immunoperoxidase staining in patients with Sjögren's syndrome and in normal subjects. To elucidate the mechanism of de novo expression of TRAIL-R1 antigen, we quantitatively investigated its induction by cytokines in human salivary duct cell line (HSG) by cell enzyme-linked immunosorbent assay. In human salivary duct cells stimulated by cytokines, we investigated the induction of apoptotic cell death by recombinant TRAIL protein.In patients with massive mononuclear cell infiltration, some infiltrating cells showed TRAIL. In patients with severe lymphocytic sialadenitis, TRAIL-R1, TRAIL-R2, or both were strongly expressed on the ductal epithelial cells. Neither TRAIL-R3 nor R4 were observed on ductal epithelium. In contrast, TRAIL-R1 and R2 were not found in the minor salivary glands of normal subjects or patients with mild lymphocytic sialadenitis. Unstimulated HSG cells did not express TRAIL-R1. Interferon-gamma (IFN-gamma) consistently upregulated levels of TRAIL-R1. In contrast, tumor necrosis factor-alpha (TNF-alpha), interleukin 1-beta (IL-1 beta), IL-2, and IL-4 had no effect on TRAIL-R1 levels. HSG cells expressing TRAIL-R1 in response to IFN-gamma were susceptible to apoptosis by recombinant TRAIL protein.Our findings suggest that TRAIL and TRAIL-R system may play a role in the pathogenesis of lymphocytic sialadenitis in patients with Sjögren's syndrome.

We previously reported that Fas antigen was strongly expressed on salivary duct epithelial cells and that some salivary infiltrating cells showed the Fas ligand in patients with severe sialoadenitis due to Sjögren's syndrome (SS). Apoptotic changes were observed in ductal epithelial cells and some infiltrating cells by DNA nick end labeling methods. These findings suggest that the Fas-Fas ligand system may play a role in the pathogenesis of sialoadenitis in SS.To elucidate the mechanism of the de novo expression of ductal Fas antigen in sialoadenitis associated with SS, we investigated the induction of Fas antigen and apoptosis by cytokines in a human salivary duct cell line.Human salivary duct cell line (HSG) was cultured with interferon gamma (IFN-gamma), tumor necrosis factor alpha (TNF-alpha), interleukin 1 beta (IL-1 beta), interleukin 2 (IL-2), interleukin 4 (IL-4), and granulocyte monocyte colony stimulating factor (GM-CSF). The expression of Fas antigen in HSG was examined by immunoperoxidase cell ELISA. The appearance of DNA strand breaks during apoptosis induced by anti-Fas antibody was detected by DNA nick end labeling methods.Unstimulated HSG cells constitutively expressed low levels of Fas antigen. IFN-gamma and TNF-alpha consistently upregulated constitutive levels of Fas. In contrast, IL-1 beta, IL-2, IL-4, and GM-CSF had no effect on Fas levels. HSG cells expressing Fas antigen in response to IFN-gamma or TNF-alpha were susceptible to apoptosis by anti-Fas antibody.Our findings suggest that IFN-gamma or TNF-alpha secreted by infiltrating lymphocytes induces ductal Fas expression and ductal apoptosis in sialoadenitis associated with SS.

Agency theory has often been employed to discuss the incentive problems. Holmstrom (1979) noted that if there is a signal from the agent's behavior, the principal had a better design of incentive systems by considering this signal. In this paper, a model including intrinsic motivation which is given little considerations in the former models is developed. From the analysis, the following results are obtained: 1) when the agent is highly intrinsically motivated, the principal has to take not only the accurate signal but also the noisy signal into account in evaluating the agent's behavior; and 2) when the agent is not intrinsically motivated, the principal has to evaluate the agent's behavior based on only the signal which has few noises.

The agency theory has often been employed to discuss the incentive problems. In the standard models, the labourers have been assumed to devote their effort only to the objectives given by the organizations. However, in actual organizations, labourers seem to have individual objectives besides the ones assigned by the organizations. In this paper, a new model considering this individual objective is developed, and the question of how to provide incentives according to non-monetary productivity of the organizational and individual objectives is discussed. From the analysis, it is obtained that when the non-monetary productivity of the individual and the organizational objective are low, introducing the piece rate should be effective.

This paper proposes a new behavioral model of organizations based on garbage can model proposed by Cohen, March and Olsen (CMO) in 1972. It explains the choice processes of modern business organizations. From the analysis, the following results are obtained: 1) though CMO noted tight organizations like a bureaucrat is always competent in problem solving, there are some cases in which loose organizations are more competent; and 2) though CMO noted when there are many difficult problems the organizational choice processes do not function well, there are some cases in which difficult problems motivate the members to produce useful knowledge.

To evaluate the role of Fas-Fas ligand system-mediated apoptosis in the sialoadenitis and interstitial nephritis of Sjögren's syndrome.The expression of Fas antigen and Fas ligand in sialoadenitis and interstitial nephritis was examined by immunoperoxidase staining and the reverse transcriptase-polymerase reaction (RT-PCR) in patients with Sjögren's syndrome and in normal subjects. The appearance of DNA strand breaks during apoptosis was detected in the tissue by DNA nick end labeling methods.In patients with severe sialoadenitis, Fas antigen was strongly expressed on the ductal epithelial cells. In contrast, Fas antigen was not seen in the minor salivary glands of normal subjects nor in patients with mild sialoadenitis. In patients with massive mononuclear cell infiltration, some of the infiltrating cells showed the Fas ligand. In patients with interstitial nephritis associated with Sjögren's syndrome, Fas was expressed on the tubular epithelial cells, while such expression was not observed in control subjects without interstitial nephritis. In the patients with interstitial nephritis, some of the infiltrating cells showed the Fas ligand. Apoptotic changes were observed in the ductal epithelial cells, tubular epithelial cells and some infiltrating cells by DNA nick end labeling methods. mRNA for the Fas antigen and Fas ligand was found to be expressed in the labial salivary glands from all SS patients by RT-PCR.The findings of this study suggest that the Fas-Fas ligand system may play a role in the pathogenesis of the sialoadenitis and interstitial nephritis of Sjögren's syndrome.

The tissue distribution of cellular adhesion molecules (ICAM-1, ELAM-1, VCAM-1) was studied in specimens from six normal human kidneys and in six biopsies from kidneys with tubulointerstitial nephritis associated with Sjögren's syndrome. In addition, the expression of cellular adhesion molecules was examined both in four renal biopsies from cases of tubulointerstitial nephritis of diverse pathogenesis and in six lip biopsies from cases of Sjögren's syndrome. ICAM-1 was expressed on vascular endothelial cells in normal kidneys, in all specimens of tubulointerstitial nephritis and in salivary glands. On tubular epithelial cells, ICAM-1 appeared slightly in normal kidneys; otherwise tubular epithelial ICAM-1 was observed in and around the foci of cellular infiltration in all cases of tubulointerstitial nephritis. ELAM-1 and VCAM-1 were observed on the newly generated vessels in massive cellular infiltrates in some cases of tubulointerstitial nephritis associated with Sjögren's syndrome; by contrast, they were not seen in normal kidneys and in cases of tubulointerstitial nephritis of diverse pathogenesis. In the lip biopsies from salivary glands, ICAM-1 was observed on ductal epithelial cells in and around the foci of cellular infiltration, and ELAM-1 and VCAM-1 occasionally appeared on the newly generated vessels in massive cellular infiltrates. Chronic and progressive inflammation may be facilitated by such ELAM-1 and VCAM-1 expression on newly generated vessels. The adhesion molecules were thought to play a role in the pathogenesis of tubulointerstitial nephritis and sialoadenitis associated with Sjögren's syndrome. It was thus concluded that the same inflammatory process that took place in the salivary glands to induce the characteristic tissue change of Sjögren's syndrome likely was operative in the renal tubulointerstitial tissue as well.

We reported a sporadic case with late onset SCA1. There was no family history of neurological diseases. His parents had been healthy until they died at the age of 77 and 89 years, respectively. The patient noticed gait disturbance at age of 60. Thereafter, he gradually developed cerebellar ataxia, hyporeflexia, mild atrophy of the facial and limb muscles and moderate deep sensory disturbance. MRI of the brain showed moderate atrophy of the cerebellum and brainstem. Sequencing analysis of SCA1 gene demonstrated that the patient had an expanded allele with 40 CAG repeats and no CAT interruption. Consequently, he was diagnosed as having SCA1. These results suggest the possibility that among apparently sporadic cases with cerebellar ataxia, there are some cases of SCA1 with mild CAG repeat expansion.

We report a case of dystrophia myotonica (DM) in a 38-year-old man with homozygous DM kinase abnormality. His distal muscle strength was reduced moderately; muscle biopsy showed type 1 predominance and type 1 fiber atrophy. The patient's WAIS total IQ score was under 60. Since his childhood, his cognitive deficit has been more severe than his muscle weakness. MRI demonstrated many abnormal changes in the brain of this patient, but these changes were mild in comparison to the severity of his reduced cognition and low IQ. The relationship between mental dysfunction and DM kinase abnormalities is quite different from that seen in cases of heterozygous DM kinase abnormalities. This case demonstrated severe mental changes in spite of mild DM kinase abnormalities. We suspect the homozygous DM kinase abnormality to be a cause of the different clinical presentations of this patient.

A 45-year-old woman with rheumatoid arthritis(RA) who developed nephrotic syndrome and acute renal failure was reported. She first noticed polyarthritis in June 1990, and was diagnosed as RA. Since her RA was not controlled with nonsteroidal anti-inflammatory drugs (NSAID), she started taking prednisolone 10 mg daily and received 100 mg of D-penicillamine from October 1990 with improvement of the RA. In March 1991, she noticed edema of the face and legs, at which time massive proteinuria and hematuria were first noted. Because of her nephrosis, she was referred to our hospital for further evaluation. Laboratory investigations revealed 24-hour urine proteinuria of 37 g, serum creatinine, 2.7 mg/dl, blood urea nitrogen, 43.5 mg/dl, total protein, 4.1 g/dl, albumin, 1.5 mg/dl, and total cholesterol, 600 mg/dl. The rheumatoid factor and anti-nuclear antibody were positive. Renal biopsy showed focal segmental glomerulosclerosis (FSGS). Her nephrotic syndrome and renal dysfunction recovered after the administration of prednisolone at 60 mg/day. The possible pathogenesis of FSGS in patients with RA was discussed.

We describe the relation of the CAG repeat length to the genetic anticipation in a Japanese family with dentato-rubro-pallido-luysian atrophy (DRPLA). The proband, a 21-year-old woman, developed epilepsy at age 19. Her mother has displayed cerebellar ataxia, choreoathetosis, and dementia since age 40, and the grandmother has shown cerebellar ataxia since age 52. So a genetic anticipation was observed. The CAG repeat sizes of peripheral blood lymphocytes from the proband, mother, and grandmother were found to be 61, 59, and 60, respectively. Thus, the mother showed earlier onset but a smaller CAG repeat length as compared to the grandmother. This case is thought to be rare and valuable in respect that the intergenerational contraction of the CAG repeat was shown in spite of the genetic anticipation was observed.

We studied the relationship between muscle pathology and the number of trinucleotide (CTG) repeats observed in lymphocytes and biopsied muscle tissues from patients with dystrophia myotonica (DM). The diameter of type 1 muscle fibers was smaller than that of type 2 fibers in all patients. The diameter and proportion of each muscle fiber type were related to the patient's age, but not to the number of trinucleotide (CTG) repeats of DM kinase in biopsied muscles. On the other hand, the proportion of type 1 fibers with central nuclei was closely related to the number of trinucleotide (CTG) repeats in muscles. These findings suggest that DM kinase abnormalities influence the muscle nuclei, and increase the number of central nuclei.

A 31 year-old man treated with sulfasalazine for ulcerative colitis, developed nephrotic syndrome, photosensitivity, alopecia, lymphopenia and hypocomplementemia. Anti-nuclear antibody (speckled) and antibodies to single-stranded DNA and SS-A were positive, while those against native DNA and histon were negative. Renal biopsy revealed diffuse proliferative lupus nephritis. His nephrotic syndrome partially improved with corticosteroid therapy combined with cessation of sulfasalazine. His complement level became normal, however it decreased again during the gradual reduction of corticosteroid dosage. In conclusion, we diagnosed the patient's illness as an idiopathic syntemic lupus erythematosus rather than sulfasalazine-induced lupus.

The expression of Fas antigen on ductal epithelial cells of sialoadenitis was examined in patients with Sjögren's syndrome (SS) and in normal subjects.Minor salivary glands from the SS patients were examined by an immunohistochemical method using a new monoclonal antibody to the Fas antigen.In two patients with severe sialoadenitis, Fas was strongly expressed on the ductal epithelial cells. By contrast, the Fas antigen was not seen in the minor salivary glands of normal subjects nor in those with mild sialoadenitis.This finding suggests that the Fas antigen may play a role in the pathogenesis of sialoadenitis in SS by providing a specific target for cytotoxic T cells expressing the Fas ligand.

The expression of high-affinity IgE receptor (Fc epsilon RI) on alveolar macrophages was examined in order to determine the association of alveolar macrophages with IgE-dependent inflammation. Immunostaining showed that a 15-1 monoclonal antibody against Fc epsilon RI alpha chain reacted with alveolar macrophages from both atopic and non-atopic patients by immunostaining. In addition, preincubation of 15-1 monoclonal antibody specifically blocked monomeric IgE binding to alveolar macrophages. Reverse transcription polymerase chain reaction showed that CD14+ alveolar macrophages expressed Fc epsilon RI alpha, beta and gamma chain mRNA. These results show that alveolar macrophages express Fc epsilon RI and may participate in IgE-dependent inflammation mediated by Fc epsilon RI.

Histamine H1-receptor occupancy in the human brain was measured in healthy young volunteers by positron emission tomography (PET) using [11C]doxepin. d-Chlorpheniramine, a selective and classical antihistamine, occupied 76.8 +/- 4.2% of the averaged values of available histamine H1 receptors in the frontal cortex after its administration in a single oral dose of 2 mg. Epinastine, a non-sedative antihistamine, occupied 13.2 +/- 18.5% of the available H1 receptors in the human frontal cortex after its administration in a single oral dose of 20 mg. There was significant correlation between H1 receptor occupancy by epinastine and its plasma concentration in each subject. PET data on the human brain were essentially compatible with those on H1-receptor occupancy in the guinea pig brain as determined by an in vivo binding technique, although for the same H1-receptor occupancy, the dose was less in humans than in guinea pigs. Our PET studies demonstrated that receptor occupancy by a second-generation H1 antagonist, epinastine, was less than 20% of the total H1 receptors, and that the low receptor occupancy was closely related to the low incidence of central side effects.

Using monoclonal antibodies, Immunoperoxidase analysis was performed on renal biopsy samples obtained from patients with Sjögren's syndrome associated with tubulo-interstitial nephritis. In all cases the interstitial infiltrates were predominantly CD5-positive T cells (mean 66%), whereas both B cell (CD19-positive) and monocyte (CD15-positive) populations participated to a lesser degree. CD16-positive NK/K cells were rarely encountered. The CD4/CD8 ratio was consistently higher than 1.0 (mean 2.08). The lymphocyte which invaded the tubular epithelial cells to present a feature of tubulitis were CD8 positive, suggesting that they were cytotoxic T cells. These results were in general accord with those obtained in the salivary glands of patients with Sjögren's syndrome. It was thus concluded that the same immunological process was probably operative in the renal tubulointerstitial tissue as in the salivary glands to induced the characteristic tissue changes of Sjögren's syndrome.

The expression of Fas antigen on ductal epithelial cells of sialoadenitis was examined in patient with Sjögren's syndrome and normal subject. In two patients with severe sialoadenitis, Fas antigen was strongly expressed on the ductal epithelial cells. In contrast, Fas antigen was not seen in minor salivary gland of normal subjects and of mild sialoadenitis cases. This finding suggests that Fas antigen may play a role in the pathogenesis of sialoadenitis in Sjögren's syndrome by providing a specific target for cytotoxic T cells expressing Fas ligand.

A 46-year-old woman was admitted to our hospital because of effort angina. Electrocardiogram on exercise showed a significant ST depression in I, II, III, aVF, and V3-6 201Thallium myocardial scintigram demonstrated a low uptake lesion on effort and redistribution in anterior wall of left ventricle. Coronary angiography showed a 50% stenosis only in the left coronary ostium. Operation was carried out by transaortic patch angioplasty using autologous pericardium fixed with 0.25% glutaraldehyde. Her postoperative course was uneventful. Anginal discomfort disappeared, and favorable results was obtained. Recent reports suggest that an isolated coronary ostial stenosis is a distinct clinical entity which is different from the usual atherosclerotic ischemic heart disease. The transaortic patch angioplasty might be useful operative method for the isolated coronary ostial stenosis.

A 56-year-old woman was admitted to our department because of congestive heart failure. Chest X-ray showed dextrocardia with situs inversus. The echocardiography revealed severe mitral regurgitation due to prolapse of posterior leaflet and secondary Venography demonstrated the bilateral superior vena cava, huge coronary sinus and hemiazygos continuation with hypoplastic inferior vena cava. Mitral valve regurgitation was repaired by Carpentier's and Burr's technique, and tricuspid valve regurgitation was repaired by Kay's technique. Her postoperative course was uneventful. Valve surgery for acquired valvular lesion in dextrocardia with situs inversus is rare. Although valve repair is easily performed even in mirror-image heart as well as in normal, levo-position heart, preoperative evaluation of systemic venous return is important in the case of dextrocardia with systemic venous anomalies.

A successful case of left heart bypass (LHB) for severe heart failure after mitral commissurotomy is presented. A 44-year-old woman underwent surgery for mitral and tricuspid regurgitation, then could not be weaned from cardiopulmonary bypass because of low cardiac output and intractable ventricular arrhythmia. The LHB from right-sided left atrium to ascending aorta was instituted with anti-thrombogenic centrifugal pump system (Carmeda). During initial 6 hours of LHB, any anticoagulant drugs were not employed, thereafter the local administration of heparin into left ventricle was started with a dose of 200 unit per hour. The LHB was successfully weaned at 26 hours after institution. Her postoperative course was uneventful. The left heart bypass without systemic heparinization utilizing anti-thrombogenic material might be a safe and useful method for postcardiotomy cardiogenic shock.

Successful thrombectomy for left atrial ball thrombus without mitral stenosis is presented. A 62-year-old man was admitted to our department because of intermittent claudication. Arteriography demonstrated complete occlusion in bilateral superficial femoral arteries, however, the arterial wall was much smooth, suggesting thrombo-embolism rather than arteriosclerosis obliterans. The transthoracic echocardiography revealed no abnormal lesion in mitral apparatus and in left atrial cavity. The bypass operation was carried out for arterial occlusion, when the intraoperative transesophageal echocardiography showed the ball thrombus in left atrial appendage. The urgent operation was successfully carried out on 14th postoperative day after bypass operation. The thrombus occupied almost all the left atrial appendage, and its size was 24 x 19 x 13 mm. The transesophageal echocardiography was useful in diagnosis for left atrial appendage thrombus. Once the diagnosis was made for large and movable left atrial thrombus, immediate surgical therapy should be recommended to avoid serious complications and sudden death.