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Lymphatic congestion is known to play an important role in the development of late Fontan complications. This study aimed to (1) develop a gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA) contrast three-dimensional heavily T2-weighed MR technique that can detect abnormal lymphatic pathway in the abdomen while simultaneously evaluating hepatocellular carcinoma (HCC) and to (2) propose a new classification of abnormal abdominal lymphatic pathway using a non-invasive method in adults with Fontan circulation. Twenty-seven adults with Fontan circulation who underwent Gd-EOB-DTPA abdominal MR imaging were prospectively enrolled in this study. We proposed MR lymphangiography that suppresses the vascular signal on heavily T2-weighted imaging after EOB contrast. The patients were classified as follows: grade 1 with almost no lymphatic pathway, grade 2 with a lymphatic pathway mainly around the bile duct and liver surface, and grade 3 with a lymphatic pathway mainly around the vertebral body and inferior vena cava. The grade 3 group showed the lowest oxygen saturation level, highest central venous pressure, highest incidence of massive ascites, HCC, and focal nodular hyperplasia. This group also tended to have patients with the oldest age and highest cardiac index; however, the difference was not statistically significant. As for the blood test, the grade 3 group showed the lowest platelet count and serum albumin level and the highest fibrosis-4 index. A novel technique, Gd-EOB-DTPA MR lymphangiography, can detect abnormal abdominal lymphatic pathways in Fontan circulation, which can reflect the severity of failing Fontan.

近年，風力発電導入加速のため環境アセスメントの迅速化目標が掲げられている。環境アセスメントの期間短縮が課題とされる中，四季変化に伴う風力発電施設の印象評価の基礎的データを得ることは重要な知見を得ることになると考えた。そこで本研究では，既存の風力発電施設サイトから規模別の9サイトを選定し，各サイトの四季変化に伴う印象評価実験を実施し，季節ごとの印象の特徴を明らかにした。その結果，春季・夏季・秋季と冬季の印象に差異があることが明らかとなった。またそれらは積雪や植生の状態が印象に影響を与えていることがわかった。, Recently the accelerated introduction of wind-generated electricity facilities has been demanded in Japan. In this regard, it is necessary to shorten the period of the Environmental Impact Assessment. We obtained the basic data of the impression evaluation of those wind-generated electricity facilities with change in the four seasons. In this study, we carried out an impression evaluation experiment of each facility. The results showed that the characteristic of the impressions were clarified every season. It became clear that spring, summer, fall and the impression of the winter season all showed a difference.

To use optical coherence tomography (OCT) and contrast letter acuity to characterize vision loss in Friedreich ataxia (FRDA). High- and low-contrast letter acuity and neurological measures were assessed in 507 patients with FRDA. In addition, OCT was performed on 63 FRDA patients to evaluate retinal nerve fiber layer (RNFL) and macular thickness. Both OCT and acuity measures were analyzed in relation to genetic severity, neurologic function, and other disease features. High- and low-contrast letter acuity was significantly predicted by age and GAA repeat length, and highly correlated with neurological outcomes. When tested by OCT, 52.7% of eyes (n = 110) had RNFL thickness values below the fifth percentile for age-matched controls. RNFL thickness was significantly lowest for those with worse scores on the Friedreich ataxia rating scale (FARS), worse performance measure composite Z2 scores, and lower scores for high- and low-contrast acuity. In linear regression analysis, GAA repeat length and age independently predicted RNFL thickness. In a subcohort of participants, 21% of eyes from adult subjects (n = 29 eyes) had macular thickness values below the first percentile for age-matched controls, suggesting that macular abnormalities can also be present in FRDA. Low-contrast acuity and RNFL thickness capture visual and neurologic function in FRDA, and reflect genetic severity and disease progression independently. This suggests that such measures are useful markers of neurologic progression in FRDA.

Background Although Axis I hypochondriasis is closely related to certain personality characteristics, the nature and extent of personality dysfunction in these patients still needs clarification. This study assessed the prevalence of personality disorders observed in hypochondriacal patients, described the types and comorbidity of personality disorders, and compared the psychological distress of patients with and without the most common comorbid personality disorder. Method One hundred fifteen patients meeting Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, criteria for hypochondriasis completed self-administered assessments, including the Personality Diagnostic Questionnaire-4+ (PDQ-4+), the Hopkins Symptom Checklist-90-Revised (SCL-90-R), the Whiteley Index, and the Somatic Symptom Inventory. These data were taken from a study conducted between September 1997 and November 2001. Results Eighty-eight patients (76.5%) had 1 or more personality disorders, whereas 27 patients (23.5%) had no personality disorders. Fifty-one patients (44.3%) had more than 3 personality disorders. The most common personality disorder in the hypochondriacal patients was obsessive-compulsive personality disorder (OCPD; 55.7%), followed by avoidant personality disorder (40.9%). The comorbidity of OCPD and avoidant personality disorder was 53.1% (34 of 64 patients with OCPD). The total PDQ-4+ score of the 64 patients with OCPD was significantly higher than that of the 51 patients without OCPD. On the SCL-90-R, the 64 patients with OCPD showed significantly higher scores on all of 3 global indices and 7 of 10 primary symptom dimensions (paranoid ideation, depression, anxiety, phobic anxiety, obsessive-compulsive, interpersonal sensitivity, and psychoticism) on the SCL-90-R compared to the 51 patients without OCPD. Conclusions The high prevalence of personality disorders, particularly OCPD, among patients with hypochondriasis suggests that consideration of personality features is important in assessment and therapeutic interventions for hypochondriasis.

To reveal the biological and pathological roles of anti-GM1 antibody in Guillain–Barré syndrome (GBS), we examined its effects on nerve growth factor (NGF) induced TrkA autophosphorylation (NGF-TrkA signaling) in PC12 cells, a sympathetic nerve cell line. The NGF-TrkA signaling is enhanced by exogenous GM1 ganglioside and this phenomenon is regarded as one of the functional aspects of GM1. The IgGs purified from patients' sera inhibited the NGF-TrkA signaling in GM1 pre-incubated PC12 cells. The degrees of inhibition by IgGs from patients paralleled their immunological reactivity to GM1. In addition, the IgGs also inhibited the neurite outgrowth of NGF-treated PC12 cells. Immunoglobulins in the rabbit sera, which were immunized by GM1, also caused a similar suppressive phenomenon. These results suggested that the anti-GM1 antibody could play roles in pathophysiology in anti-GM1 antibody positive GBS through interfering with the neurotrophic action of NGF and GM1 mediated signal modulation including NGF-TrkA signaling. It is suggested that the modulation of GM1 function is one important action of antibodies and could be one of the important mechanisms in GBS.

We examined natural killer (NK) activity in 128 patients with atopic dermatitis (AD) to investigate the relationships between NK activity and severity of dermatitis, duration of disease, and mental states. The results showed the following: 1) No relationship was shown between severity of dermatitis and NK activity, neither between NK activity nor eosinophilic counts nor serum IgE. 2) Patients with longer duration of AD lesions showed significantly lower NK activity (P=0.036). The significant relationship was recognized between severity of dermatitis and the duration of disease (P=0.014). 3) No relationships were recognized between NK activity and mental states evaluated using the Profile Of Mood States (POMS) questionnaire, as tension-anxiety, depression-dejection, anger-hostility, vigor, fatigue and confusion. From a psychoneuroimmunological viewpoint, chronic stress as having AD might influence the lower NK activity of patients with longer duration of AD.

CTG triplet repeats of "normal" length in the myotonic dystrophy protein kinase (DMPK) gene have been previously believed to be stable and new pathological expansion was not believed to occur. Here we report possible de novo CTG repeat expansion in the DMPK gene in a patient with cardiomyopathy, who was not diagnosed as having myotonic dystrophy type 1 (DM1) by conventional genetic tests.

We investigated whether dehydroepiandrosterone (DHEA) or DHEA-sulfate (S) affected the activities of nuclear receptors, with special reference to constitutive androstane receptor beta (CARbeta). Administration of DHEA or DHEA-S enhanced the DNA binding of hepatic nuclear extracts to responsive elements for the retinoic acid receptor, the retinoic acid receptor beta 2 and the peroxisome proliferator activated receptor. The bound complexes were shown to be the CARbeta-RXR heterodimer by antibody-supershift assays. The expression of a target gene of CARbeta, Cyp2b10, was increased in liver by DHEA or DHEA-S treatment, suggesting that DHEA or DHEA-S actually activated CARbeta in vivo. It was suggested that the metabolic conversion of DHEA, DHEA-S to CARbeta ligands could occur in vivo and the metabolites could regulate the expression of CARbeta target gene expression. Our results provide new insights into the in vivo relationship between DHEA/DHEA-S and CARbeta activation.