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車いす使用者は、座位姿勢による下半身の寸法変化が大きいため既製服が適合しにくい。ジーンズは、車いす使用者も外出着として着用したいと要望のあるボトムスの一つである。しかし、生地が硬く、スリムでスタイルのよいジーンズほどゆとりが少なく、身体への負荷が強いため、着用を諦めている人も多い。そこで後ろズボンの一部をツーウェイ伸縮性ニットに付け替えるリフォームジーンズを提案した。健康な女子大学生が着用して比較実験を行ったところ、衣服圧・拘束感の訴えが高かった膝前面や臀部の値はリフォームにより低下し、快適感を向上させることが確認された。 この結果をもとに、特別な縫製技術を必要とせず簡単にリフォームできる方法を考案し、着脱動作の検証を行った。ジーンズ着脱時の上半身の左右方向の移動距離、使用する筋肉群には個人差があったが、全体的にはリフォームジーンズの方が着脱時間は短く、着脱しやすいと評価された。脳波から算出したá 波出現率は高く、リフォームすることによりストレスなく着脱できることも確認された。日常的に車いすを使用している方からも着脱に要する時間が短く、拘束感が緩和されると評価された。以上より、今回提案した車いす使用者用ジーンズのリフォーム方法は有効であることが示唆された。

Background Pallister-Killian syndrome (PKS) is a rare disorder caused by the mosaic tetrasomy of chromosome 12p, and is characterized by facial dysmorphism, developmental delay, hypotonia and seizures. Results We report a patient with PKS showing unique polymicrogyria with calcification. He had delayed development and dysmorphic facial features including frontal bossing, hypertelorism, and high arched palate at 6 months of age. Neuroimaging revealed unilateral polymicrogyria with spot calcifications, which predominantly affected the right perisylvian region. Chromosome G-banding showed the karyotype 46,XY, however, array-based comparative genomic hybridization analysis showed mosaic duplication of chromosome 12p, in which CCND2, which encodes cyclin D2 and is a downstream mediator of PI3K-AKT pathway, is located. Supernumerary chromosome of 12p was detected in 58% of buccal mucosa cells by the interphase fluorescence in situ hybridization analysis using chromosome 12 centromere-specific D12Z3 probe. The diagnosis of PKS was made based on distinctive clinical features of our patient and the results of cytogenetic analyses. Conclusion This report is, to our knowledge, the first case of a patient with PKS who clearly demonstrates polymicrogyria colocalized with calcifications, as shown by CT scans and MRI, and suggests that a patient with PKS could show structural brain anomalies with calcification. We assume that somatic mosaicism of tetrasomy could cause asymmetrical polymicrogyria in our patient, and speculate that increased dosages of CCND2 at chromosome 12p might be involved in the abnormal neuronal migration in PKS.

BACKGROUND Both congenital heart disease (CHD) and very-low birthweight (VLBW) infants are at a very high risk of neurodevelopmental delay. We investigated neurological development at 3 years in pediatric patients with CHD after surgical intervention, those of VLBW, and healthy controls. METHODS We enrolled pediatric patients with CHD (n = 67), VLBW (n = 67), and healthy controls (n = 81). Infants with CHD were grouped into those with single ventricle and two ventricles, and infants with VLBW were grouped into those with birthweights of

To examine whether neurodevelopment at preschool age predicts IQ levels and needs for special education services in school-age children with single ventricle (SV) physiology. Thirty-five patients with SV physiology were assessed using the Bayley Scale of Infant and Toddler Development (BSID) II or III at 3 years and the Wechsler Intelligence Scale for Children-Fourth Edition (WISC) at 8 years. Whether the children were receiving special education services was also determined. Factors associated with the mental developmental index (MDI) of the BSID, the full-scale IQ (FSIQ) of the WISC, and special education services were also analyzed. The MDI and FSIQ of children with SV physiology were significantly lower than the values in healthy children (P0.01). The MDI at 3 years was moderately correlated with FSIQ at 8 years (P0.01, R

To define the correlation between neuroanatomic and developmental outcomes of children with single ventricle (SV) or transposition of the great arteries (TGA), a prospective longitudinal study was performed in preschool and school-age children. Twenty-seven children with congenital heart disease (9, TGA; 18, SV) were included. Participants underwent 3-dimensional magnetic resonance imaging (MRI) and neurodevelopmental assessment at around 3 years (preschool age) and at 9 years (school age), and 48 healthy controls underwent MRI, and their data were used to derive best-fit models for normal brain volumes for comparisons with congenital heart disease patients. Total brain volume (TBV) and regional brain volumes remained significantly smaller in SV children than in TGA children at both time points, though the growth slope of TBV was not significantly different between the SV and TGA groups. Although the psychomotor developmental index at preschool was significantly lower in SV patients, the full-scale IQ at school age was not significantly lower in SV patients. There was a strong correlation between full-scale IQ and TBV (r = 0.49, P = 0.005). Despite the current best practices, persistently lower TBV was seen in SV patients until 9 years of age. For both the SV and TGA groups, TBV at 3 years was a strong predictor of TBV at 9 years. Since there was a correlation between TBV and IQ at 9 years, identification of factors that affect brain growth until 3 years will be imperative to improve patients' cognitive function at school age.

Background and purpose Measuring head circumference (HC) in infants is an easy screening procedure with which to detect abnormalities in brain growth. It has been demonstrated that HC can predict total brain volume (TBV) in very-low-birth-weight (VLBW) infants. However, the correlation between HC and TBV was weaker than that observed in healthy term-born toddlers, suggesting that there are factors that influence the relationship between HC and TBV. The aim of this study was to identify the clinical risk factors that caused a deviation from the regression line obtained between HC and TBV. Methods The study population was based on 37 VLBW infants, who underwent a clinical magnetic resonance imaging (MRI) examination at a term-equivalent age, during 2013-2015, at Toyama University Hospital. The HC and the TBV were both adjusted for sex, multiple births, and postmenstrual age. The relationship between TBV/HC and clinical characteristics was evaluated. Results There was a positive correlation between HC and TBV (r = .58, P = .000168). Two clinical factors, the lower birth body weight (BBW) (r = .38, P = .02) and dolichocephaly (r = 0.46, P = .006), were identified as factors that negatively affected the TBV/HC ratio. After excluding infants with low BBW or with dolichocephaly, the correlation between HC and TBV was higher (r = .63). Conclusions Although HC has predictive value for TBV in VLBW infants, care should be taken in infants with low BBW (BBW less than 600 g) or dolichocephaly (MRI-based cranial index less than .68), which were related to overestimation of TBV.

A 79-year-old male was diagnosed as having a scirrhous cancer of the stomach. Carcinomatous peritonitis was suspected on abdominal CT examination and the CA19-9 showed a high level of 95 U/ml. The patient was treated with combined chemotherapy of TS-1 and CDDP. TS-1 (100 mg/day) was administered for 14 days followed by 14 days rest as one course. CDDP was administered in 24-h continuous intravenous infusion on day 8. This treatment was done every 4 weeks regularly. After 5 courses, X-ray and endoscopy examinations revealed disappearance of cancerous lesions in the stomach with an improvement in the extensibility. No cancer cell were confirmed by endoscopic biopsy, nor did a CT-scan detect carcinomatous peritonitis. The CA19-9 decreased within the normal limit. Ten months after chemotherapy was started, the patient was very healthy without a recurrence of cancer. This combined chemotherapy has administered in 8 courses, and during this period no high grade toxicities (WHO grade 3 or 4) occurred. This TS-1/CDDP chemotherapy was effective for scirrhous gastric cancer and might be administered safely even for aged patients.