Supportive pericardial suspension for surgical airway management of tracheobronchomalacia in unilateral pulmonary agenesis

Unilateral pulmonary agenesis, a rare developmental defect of the lung, is often accompanied by tracheobronchial stenosis or malacia due to displacement, distortion, and compression of the surrounding great vessels. We present two cases of unilateral pulmonary agenesis complicated by tracheobronchial problems that were successfully managed surgically with supportive pericardial suspension.Keywords: pericardial suspension, tracheobronchomalacia, unilateral pulmonary agenesi

Eccentric Figure-Eight Coils for Transcranial Magnetic Stimulation

Previously we proposed an eccentric figure-eight coil that can cause threshold stimulation in the brain at lower driving currents. In this study, we performed numerical simulations and magnetic stimulations to healthy subjects for evaluating the advantages of the eccentric coil. The simulations were performed using a simplified spherical brain model and a realistic human brain model. We found that the eccentric coil required a driving current intensity of approximately 18% less than that required by the concentric coil to cause comparable eddy current densities within the brain. The eddy current localization of the eccentric coil was slightly higher than that of the concentric coil. A prototype eccentric coil was designed and fabricated. Instead of winding a wire around a bobbin, we cut eccentric-spiral slits on the insulator cases, and a wire was woven through the slits. The coils were used to deliver magnetic stimulation to healthy subjects; among our results, we found that the current slew rate corresponding to motor threshold values for the concentric and eccentric coils were 86 and 78 A/µs, respectively. The results indicate that the eccentric coil consistently requires a lower driving current to reach the motor threshold than the concentric coil. Future development of compact magnetic stimulators will enable the treatment of some intractable neurological diseases at home. Bioelectromagnetics. 35:55–65, 2015. © 2014 Wiley Periodicals, Inc.ArticleBIOELECTROMAGNETICS. 36(1):55-65 (2015)journal articl

Combined effect of regulatory polymorphisms on transcription of UGT1A1 as a cause of Gilbert syndrome

<p>Abstract</p> <p>Background</p> <p>Gilbert syndrome is caused by defects in bilirubin UDP-glucuronosyltransferase (UGT1A1). The most common variation believed to be involved is A(TA)7TAA. Although several polymorphisms have been found to link with A(TA)7TAA, the combined effect of regulatory polymorphisms in the development of Gilbert syndrome remains unclear.</p> <p>Methods</p> <p>In an analysis of 15 patients and 60 normal subjects, we detected 14 polymorphisms and nine haplotypes in the regulatory region. We classified the 4-kbp regulatory region of the patients into: the TATA box including A(TA)7TAA; a phenobarbital responsive enhancer module including c.-3275T>G; and a region including other ten linked polymorphisms. The effect on transcription of these polymorphisms was studied.</p> <p>Results</p> <p>All haplotypes with A(TA)7TAA had c.-3275T>G and additional polymorphisms. In an <it>in-vitro </it>expression study of the 4-kbp regulatory region, A(TA)7TAA alone did not significantly reduce transcription. In contrast, c.-3275T>G reduced transcription to 69% of that of wild type, and the linked polymorphisms reduced transcription to 88% of wild type. Transcription of the typical regulatory region of the patients was 56% of wild type. Co-expression of constitutive androstane receptor (CAR) increased the transcription of wild type by a factor of 4.3. Each polymorphism by itself did not reduce transcription to the level of the patients, however, even in the presence of CAR.</p> <p>Conclusions</p> <p>These results imply that co-operation of A(TA)7TAA, c.-3275T>G and the linked polymorphisms is necessary in causing Gilbert syndrome.</p

Two cases of mandibular and masticatory reconstruction with vascularized fibra bone flaps and implants

　Defects of the jawbone have conventionally been repaired using free bone grafts and metallic plates, but patients undergoing reconstruction using these materials experience difficulty using dentures and restoring masticatory function. The use of vascularized bone grafts, which has recently been enabled, has improved bone graft survival rates, and its combination with dental implants has enabled both morphological and functional reconstruction, leading to a higher quality of life. 　We experienced two patients with tumors of the jaw that were treated by resecting and reconstructing it using a vascularized bone graft with the cooperation of the Department of Plastic Surgery in the School of Medicine. We subsequently inserted dental implants, and the superstructure was created by the Department of Prosthetics. We report the course of these patients, who achieved sufficient jaw morphology as well as masticatory function and who are presently satisfied overall three years to three years and nine months postoperatively

Hereditary Spherocytosis Coexisting with UDP-Glucuronosyltransferase Deficiency Highly Suggestive of Crigler-Najjar Syndrome Type II

Patients with co-existing hereditary spherocytosis (HS) and UDP-glucuronosyltransferase 1A1 (UGT1A1) deficiency as Gilbert's syndrome (GS) have been reported, and previous studies have demonstrated an increased risk for developing gallstones in patients with co-inheritance of GS and HS. We experienced an interesting case of HS showing persistent jaundice after splenectomy, and upon further evaluation, the 25-year-old female patient was found to have HS combined with UGT1A1 deficiency. Sequence analysis of the UGT1A1 gene revealed that she was a compound heterozygote with p.[G71R; Y486D] + [Y486D] mutations, which suggests Crigler-Najjar syndrome type II rather than GS. Careful evaluation of inappropriately elevated bilirubin level compared with the degree of hemolysis is important, reflecting the therapeutic implication of splenectomy and cholecystectomy

Effects of common genetic variants of human uridine diphosphate glucuronosyltransferase subfamilies on irinotecan glucuronidation.

The adverse effects (diarrhea and neutropenia) of irinotecan (7-ethyl-10-[4-(1-piperidino)-1-piperidino]carbonyloxycamptothecin) are associated with genetic variants of uridine diphosphate glucuronosyltransferase 1A subfamilies (UGT1As). UGT1As are enzymes that metabolize the active form of irinotecan, 7-ethyl-10 hydroxycamptothecin (SN-38), by glucuronidation in the liver. They are widely known as predictive factors of severe adverse effects, such as neutropenia and diarrhea. Some studies have suggested that variants of UGT1As affect SN-38 glucuronidation activities, thus exerting severe adverse effects. We aimed to identify UGT1A isoforms that show SN-38 glucuronidation activity and determine the relationship between UGT1A variants and SN-38 glucuronidation in vitro. We found that UGT1A1 and UGT1A6-UGT1A10 displayed SN-38 glucuronidation activity. Among these, UGT1A1 was the most active. Furthermore, the variants of these isoforms showed decreased SN-38 glucuronidation activity. In our study, we compared the different variants of UGT1As, such as UGT1A1.6, UGT1A1.7, UGT1A1.27, UGT1A1.35, UGT1A7.3, UGT1A8.4, UGT1A10M59I, and UGT1A10T202I, to determine the differences in the reduction of glucuronidation. Our study elucidates the relationship between UGT1A variants and the level of glucuronidation associated with each variant. Therefore, testing can be done before the initiation of irinotecan treatment to predict potential toxicities and adverse effects

Practical study on palliative medicine education through teachers for medicine-Religionist Collaboration

科学研究費補助金研究成果報告書研究種目: 基盤研究(C)研究期間: 2009～2011課題番号: 21590561研究代表者: 馬場 忠雄（滋賀医科大学・学長）研究分担者: 早島 理（滋賀医科大学・医学部・教授）研究分担者: 佐藤 浩（滋賀医科大学・医学部・教授）研究分担者: 丸尾 良浩（滋賀医科大学・医学部・講師）研究分担者: 長倉 伯博（滋賀医科大学・医学部・非常勤講師

Systemic adverse events after screening of retinopathy of prematurity with mydriatic.

Purpose:To evaluate systemic adverse events after screening for retinopathy of prematurity (ROP) performed with mydriatic.Methods:This was a retrospective case series study. Medical records of consecutive patients who underwent screening for ROP with 0.5% phenylephrine and 0.5% tropicamide eyedrops were retrospectively reviewed. The score of abdominal distention (0-5), volume of milk sucked and volume of stool, along with systemic details (pulse and respiration rates, blood pressure and number of periods of apnea) were collected at 1 week and 1 day before ROP examination, and at 1 day after examination. Results were compared between the days before and after examination. Correlation between body weight at the time of examination and the score of abdominal distention was examined. The numbers of infants with abdominal and/or systemic adverse events were compared between pre- and post-examination periods.Results:Eighty-six infants met the inclusion criteria. The score of abdominal distention increased from 2.0 at 1 day before examination to 2.3 at 1 day after examination (p = 0.005), and the number of infants who had worsened abdominal distension increased after examination (p = 0.01). Infants with lower body weight had a higher score of abdominal distention (p < 0.0001, r = -0.57). The number of infants with reduced milk consumption increased after examination (p = 0.0001), as did the number of infants with decreased pulse rate (p = 0.0008).Conclusions:Screening for ROP with mydriatic may have adverse effects on systemic conditions. Infants should be carefully monitored after ROP screening with mydriatic