Updated on 2025/07/28

写真a

 
NAGAYA Ken
 
Organization
Hospital Central Clinical Facilities Center for Maternity and Infant Care
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Papers

  • Can fetal ultrasound predict adrenal hypoplasia congenita caused by Xp21 deletion? Reviewed

    Yuko Yokohama, Yoshio Makita, Yuriko Oishi, Ken Nagaya, Shigeru Suzuki, Yasuhito Kato

    Journal of Obstetrics and Gynaecology Research   51 ( 4 )   2025.3

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    Publishing type:Research paper (scientific journal)   Publisher:Wiley  

    Abstract

    Xp21 deletion is a contiguous gene deletion syndrome characterized by adrenal hypoplasia congenita (AHC). If prenatal genetic diagnosis does not determine whether a male fetus at risk is affected, it is necessary to monitor him to diagnose adrenal insufficiency in the first few days of life to avoid a salt‐losing adrenal crisis. Whether AHC can be diagnosed by prenatal ultrasound is not known. Ultrasound findings of fetal adrenal glands were compared between healthy and affected siblings whose mother was a carrier of the Xp21 deletion. The couple did not want an invasive prenatal diagnosis during either pregnancy. The adrenal glands of a healthy fetus have a two‐layered structure, but this structure could not be confirmed in the affected male. We believe this is an important finding that suggests that steroid supplementation may be necessary immediately after birth.

    DOI: 10.1111/jog.16276

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  • Letter to the Editor

    Ken Nagaya

    Journal of Paediatrics and Child Health   2025.1

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    DOI: 10.1111/jpc.16741

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  • Early Transpyloric Tube Feeding in Preventing Adverse Respiratory Events in Extremely Low Birth Weight Infants Reviewed

    Shinya Tanaka, Fumihiko Namba, Ken Nagaya, Naohiro Yonemoto, Shinya Hirano, Itaru Yanagihara, Hiroyuki Kitajima, Masanori Fujimura

    Biomedicines   2024.12

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    DOI: 10.3390/biomedicines12122799

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  • Impact of visitation restrictions in neonatal intensive care units during the <scp>COVID</scp>‐19 pandemic on parents in northern Hokkaido, Japan Reviewed

    Ken Nagaya, Toshio Okamoto, Mitsumaro Nii, Aiko Aoyama‐Hashimoto, Tatsutoshi Sugiyama, Kenta Takahashi, Takashi Sato, Masaru Shirai

    Journal of Paediatrics and Child Health   60 ( 12 )   859 - 866   2024.10

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    Authorship:Lead author   Language:English   Publishing type:Research paper (scientific journal)   Publisher:Wiley  

    Aim

    Medical institutions restricted visitation to neonatal intensive care units (NICUs) during the coronavirus disease (COVID‐19) pandemic. Therefore, this study aimed to investigate the impact of COVID‐19 NICU visitor restrictions on parents.

    Methods

    We conducted a questionnaire of 378 parents of infants who were hospitalised for more than 1 week at two NICUs in our area and discharged between 1 April 2020 and 31 March 2022. While the visiting rules for NICUs during this period varied depending on the phase of the COVID‐19 epidemic, generally, only parents were allowed to visit the NICUs for a few hours daily.

    Results

    A total of 157 parents (A University Hospital (AMUH), n = 79; AK General Hospital (AKGH), n = 78) responded to the survey (41.3% response rate), with 40% stating that their emotions towards their infants were affected by restrictions due to the COVID‐19 pandemic. Parents' feelings towards their infants were influenced by mode of delivery, parity, duration of hospitalisation and opportunities to visit the NICU. More than 70% of respondents felt stressed due to COVID‐19 restrictions, and anxiety and emotional stress related to visitor restrictions were significantly affected by the number and duration of visits and allowability of family member visits. Parents felt that the restrictions had a negative impact on breastfeeding, overall infant care and infant–family bonding.

    Conclusion

    As parents felt an impact on the bond with their infant during visitation restrictions, it is essential for facilities to implement measures to protect infant–family bonding during potential future pandemics.

    DOI: 10.1111/jpc.16697

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  • Association between maternal multimorbidity and neurodevelopment of offspring: a prospective birth cohort study from the Japan Environment and Children's Study.

    Akagi T, Saijo Y, Yoshioka E, Sato Y, Nakanishi K, Kato Y, Nagaya K, Takahashi S, Ito Y, Iwata H, Yamaguchi T, Miyashita C, Ito S, Kishi R, JECS

    BMJ open   2024.8

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    DOI: 10.1136/bmjopen-2023-082585

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  • Non-progressive hepatic tumor with high levels of serum alpha-fetoprotein in two infants with trisomy 18.

    Okamoto T, Nagaya K, Nii M, Takahashi S

    Congenital anomalies   2024.8

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    DOI: 10.1111/cga.12582

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  • Null Association Between Isolated Orofacial Clefts and Sleep Duration: A Cohort Study From the Japan Environment and Children's Study. International journal

    Yukihiro Sato, Eiji Yoshioka, Yasuaki Saijo, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Sumitaka Kobayashi, Yu Ait Bamai, Keiko Yamazaki, Sachiko Itoh, Chihiro Miyashita, Atsuko Ikeda-Araki, Reiko Kishi

    The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association   61 ( 3 )   383 - 390   2024.3

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    Although children with orofacial clefts have an increased risk for sleep-disordered breathing, no studies have examined the association of sleep duration. Thus, this study aimed to examine associations between orofacial clefts and sleep duration at 1 month, 6 months, 1 year, and 3 years of age in Japan. A cohort study from the Japan Environment and Children's Study. This study consisted of 91 497 children, including ones with isolated cleft lip and palate (n = 69), isolated cleft lip only (n = 48), and isolated cleft palate only (n = 37), for which recruitment was undertaken during 2011 to 2014. Seep durations (hours per day) at 1 month, 6 months, 1 year, and 3 years of age, as reported by their mothers. In the control group, mean sleep durations and standard deviations at 1 month, 6 months, 1 year, and 3 years of age were 15.2 (2.5), 13.6 (1.9), 12.9 (1.6), and 11.6 (1.2) h, respectively. Compared to the control group, linear regression models reported effect sizes and 95% confidence intervals shorter than 1 h for sleep duration of each type of isolated orofacial cleft at each time point. This study suggested null associations between isolated orofacial clefts and sleep duration at 1 month, 6 months, 1 year, and 3 years of age. Children with isolated orofacial clefts had sufficient mean sleep duration.

    DOI: 10.1177/10556656221128425

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  • 2023年度研修開始専攻医の小児科領域専門研修プログラムに関するアンケート

    水野 克己, 飛彈 麻里子, 大西 聡, 荒堀 仁美, 落合 正行, 久保井 徹, 佐藤 義朗, 高橋 尚人, 戸石 悟司, 長屋 建, 福原 里恵, 松本 敦, 宮沢 篤生, 山田 恭聖, 山田 洋輔, 和田 友香, 日下 隆, 東海林 宏道, 森岡 一朗, 日本小児科学会新生児委員会

    日本小児科学会雑誌   128 ( 1 )   88 - 91   2024.1

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  • Maternal pre-pregnancy body mass index and related factors: A cross-sectional analysis from the Japan Environment and Children's Study. International journal

    Yasuaki Saijo, Eiji Yoshioka, Yukihiro Sato, Yuki Kunori, Tomoko Kanaya, Kentaro Nakanishi, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Hiroyoshi Iwata, Takeshi Yamaguchi, Chihiro Miyashita, Sachiko Itoh, Reiko Kishi

    PloS one   19 ( 6 )   e0304844   2024

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    Socioeconomic status and smoking are reportedly associated with underweight and obesity; however, their associations among pregnant women are unknown. This study aimed to investigate whether socioeconomic factors, namely educational attainment, household income, marital status, and employment status, were associated with pre-pregnancy body mass index (BMI) categories, including severe-moderate underweight (BMI ≤ 16.9 kg/m2), mild underweight (BMI, 17.0-18.4 kg/m2), overweight (BMI, 25.0-29.9 kg/m2), and obese (BMI ≥ 30.0 kg/m2) among Japanese pregnant women using data from the Japan Environment and Children's Study (JECS). In total, pregnant women were included 96,751. Age- and parity-adjusted multivariable multinomial logistic regression analyses assessed socioeconomic factors and smoking associations with falling within abnormal BMI categories (normal BMI as the reference group). Lower education and lower household were associated with overweight and obesity, and, especially, lowest education and household income had relatively higher point estimate relative ratios (RRs) of 3.97 and 2.84, respectively. Regarding the risks for underweight, however, only junior high school education had a significantly higher RR for severely to moderately underweight. Regarding occupational status, homemakers or the unemployed had a higher RR for severe-moderate underweight, overweight, and obesity. Unmarried, divorced, or bereaved women had significantly higher RRs for mildly underweight status. Quitting smoking early in pregnancy/still smoking had higher RRs for all four not having normal BMI outcomes; however, quitting smoking before pregnancy had a higher RR only for obese individuals. Lower educational attainment and smoking are essential intervention targets for obesity and severe-moderate underweight prevention in younger women. Lower household income is also a necessary target for obesity.

    DOI: 10.1371/journal.pone.0304844

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  • 先天性筋強直性ジストロフィー1型の患児の確定診断をめぐり両親への対応に苦慮した事例 根治的治療法がない疾患の発症前診断につながる可能性への配慮

    横浜 祐子, 蒔田 芳男, 長屋 建, 澤田 潤, 加藤 育民

    遺伝子医学   13 ( 3 )   135 - 139   2023.7

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  • A novel HECW2 variant in an infant with congenital long QT syndrome. International journal

    Rina Imanishi, Kouichi Nakau, Sorachi Shimada, Hideharu Oka, Ryo Takeguchi, Ryosuke Tanaka, Tatsutoshi Sugiyama, Mitsumaro Nii, Toshio Okamoto, Ken Nagaya, Yoshio Makita, Kumiko Yanagi, Tadashi Kaname, Satoru Takahashi

    Human genome variation   10 ( 1 )   17 - 17   2023.6

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    Pathogenic variants of HECW2 have been reported in cases of neurodevelopmental disorder with hypotonia, seizures, and absent language (NDHSAL; OMIM #617268). A novel HECW2 variant (NM_001348768.2:c.4343 T > C,p.Leu1448Ser) was identified in an NDHSAL infant with severe cardiac comorbidities. The patient presented with fetal tachyarrhythmia and hydrops and was postnatally diagnosed with long QT syndrome. This study provides evidence that HECW2 pathogenic variants can cause long QT syndrome along with neurodevelopmental disorders.

    DOI: 10.1038/s41439-023-00245-w

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  • Severe tricuspid insufficiency with rupture of the papillary muscle secondary to premature closure of ductus arteriosus: a case report

    Izumi Kengo, Miyoshi Takamasa, Sugiyama Tatsutoshi, Aoyama Aiko, Nii Mitsumaro, Okamoto Toshio, Nagaya Ken

    Journal of Japan Society of Perinatal and Neonatal Medicine   59 ( 1 )   77 - 81   2023.5

  • Association between maternal multimorbidity and preterm birth, low birth weight and small for gestational age: a prospective birth cohort study from the Japan Environment and Children's Study. International journal

    Kentaro Nakanishi, Yasuaki Saijo, Eiji Yoshioka, Yukihiro Sato, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Sumitaka Kobayashi, Chihiro Miyashita, Atsuko Ikeda-Araki, Reiko Kishi

    BMJ open   13 ( 3 )   e069281   2023.3

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    OBJECTIVES: Multimorbidity is defined as the coexistence of two or more chronic physical or psychological conditions within an individual. The association between maternal multimorbidity and adverse perinatal outcomes such as preterm delivery and low birth weight has not been well studied. Therefore, this study aimed to investigate this association. METHODS: We conducted a prospective cohort study using data from the Japan Environment and Children's Study of pregnant women between 2011 and 2014. Those with data on chronic maternal conditions were included in the study and categorised as having no chronic condition, one chronic condition or multimorbidities. The primary outcomes were the incidence of preterm birth (PTB), low birth weight (LBW) and small for gestational age (SGA). Adjusted logistic regression was performed to estimate ORs (aORs) and 95% CIs. RESULTS: Of the 104 062 fetal records, 86 885 singleton pregnant women were analysed. The median maternal age and body mass index were 31 years and 20.5 kg/m2, respectively. The prevalence of pregnant women with one or more chronic conditions was 40.2%. The prevalence of maternal multimorbidity was 6.3%, and that of PTB, LBW, and SGA were 4.6%, 8.1%, and 7.5%, respectively. Pre-pregnancy underweight women were the most common, observed in 15.6% of multimorbidity cases, followed by domestic violence from intimate partner in 13.0%. Maternal multimorbidity was significantly associated with PTB (aOR 1.50; 95% CI 1.33-1.69), LBW (aOR 1.49; 95% CI 1.35-1.63) and SGA (aOR 1.33; 95% CI 1.20-1.46). CONCLUSION: Maternal multimorbidity was associated with adverse perinatal outcomes, including PTB, LBW and SGA. The risk of adverse perinatal outcomes tends to increase with a rise in the number of chronic maternal conditions. Multimorbidity becomes more prevalent among pregnant women, making our findings important for preconception counselling.

    DOI: 10.1136/bmjopen-2022-069281

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  • Associated congenital anomalies and syndromes of 248 infants with orofacial clefts born between 2011 and 2014 in the Japan environment and children's study. International journal

    Yukihiro Sato, Eiji Yoshioka, Yasuaki Saijo, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Sumitaka Kobayashi, Yu Ait Bamai, Keiko Yamazaki, Sachiko Itoh, Chihiro Miyashita, Atsuko Ikeda-Araki, Reiko Kishi

    Congenital anomalies   63 ( 1 )   9 - 15   2023.1

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    This study aimed to document the complication status of infants with orofacial clefts born between 2011 and 2014 in Japan. This was a descriptive study using data from the Japan Environment and Children's Study. Among 103 060 pregnancies, 248 infants with orofacial clefts were included (livebirth, 239; stillbirth, 4; miscarriage, 5). The items of interest were complication status of orofacial clefts: isolated (typical orofacial clefts only); multi-malformed (orofacial clefts with unrelated major defects); syndromic (orofacial clefts with a syndrome or a chromosomal defect). Regarding the cleft subtypes, of 248 infants with orofacial clefts, 104 had cleft lip with cleft palate (CLP) (41.9%), 68 had cleft lip without cleft palate (CL) (27.4%), 58 had cleft palate without cleft lip (CP) (23.4%), and 18 were nonclassified (7.3%). In infants with CLP, the proportions of isolated, multi-malformed, and syndromic phenotypes were 73.1%, 15.4%, and 11.5%, respectively. In infants with CL, the proportions were 79.4%, 16.2%, and 4.4%, respectively. In infants with CP, the proportions were 69.0%, 13.8%, and 17.2%, respectively. The most frequently associated congenital anomaly was congenital heart disease. In infants with syndromic CLP, 41.7% had trisomy 13. In infants with syndromic CP, 80.0% had the Pierre Robin sequence. Congenital heart disease could be the most frequently associated congenital anomaly. The most frequently associated syndrome could be trisomy 13 in those with CLP and Pierre Robin sequence in those with CP.

    DOI: 10.1111/cga.12496

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  • Mortality and morbidity of extremely low birth weight infants in Japan, 2015. International journal

    Tokuo Miyazawa, Hitomi Arahori, Satoshi Ohnishi, Hromichi Shoji, Atsushi Matsumoto, Yuka Sano Wada, Naoto Takahashi, Toshimitsu Takayanagi, Satoshi Toishi, Ken Nagaya, Hisaya Hasegawa, Masahiro Hayakawa, Mariko Hida, Rie Fukuhara, Yasumasa Yamada, Masahiko Kawai, Kusaka Takashi, Kazuko Wada, Ichiro Morioka, Katsumi Mizuno

    Pediatrics international : official journal of the Japan Pediatric Society   65 ( 1 )   e15493   2023.1

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    BACKGROUND: In Japan, the mortality rate of extremely low birth weight (ELBW) infants is notably low in comparison with other developed countries, but the prevalence of chronic lung disease (CLD) and retinopathy of prematurity (ROP) is relatively high. This study aimed to estimate the mortality and morbidity of ELBW infants born in 2015 who were admitted to neonatal intensive care units (NICUs) in Japan and to examine the factors that affected the short-term outcomes of these infants. We also compared the mortality of ELBW infants born in 2005, 2010, and 2015. METHODS: We analyzed the mortality, morbidity, and factors related to short-term outcomes of ELBW infants, using data from 2782 infants born in 2015 and registered at NICUs in Japan. RESULTS: The mortality rates during NICU stays were 17.0%, 12.0%, and 9.8% for ELBW infants born in 2005, 2010, and 2015, respectively. Among ELBW infants born in 2015, multiple logistic regression analysis showed that short gestational age and low birthweight Z-score contributed to the increased risk of death. Births by cesarean section and antenatal corticosteroid administration were significantly associated with a reduced risk of death. Among infants who survived, CLD was observed in 53.1% and ROP requiring treatment was observed in 30.4%. CONCLUSIONS: Mortality in ELBW infants decreased significantly from 2005 to 2015. As CLD and ROP may affect quality of life and long-term outcomes of infants who survived, prevention strategies and management for these complications are critical issues in neonatal care in Japan.

    DOI: 10.1111/ped.15493

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  • Evaluating association of smoking status during pregnancy with adverse birth outcomes using urinary cotinine concentration: The Japan environment and Children's study (JECS). International journal

    Yuki Kunori, Yasuaki Saijo, Eiji Yoshioka, Yukihiro Sato, Tomoko Kanaya, Kentaro Nakanishi, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Sachiko Itoh, Sumitaka Kobayashi, Chihiro Miyashita, Atsuko Ikeda-Araki, Reiko Kishi

    Environmental research   215 ( Pt 2 )   114302 - 114302   2022.12

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    Urinary cotinine concentration (UCC) reflects smoking status. However, in pregnant women, its association with adverse birth outcomes related to fetal growth is not widely known. Thus, we aimed to explore this relationship by focusing on dose-response relationships. We investigated 86,638 pregnant women enrolled between 2011 and 2014 in a prospective cohort study in Japan and observed three birth outcomes (preterm birth, low birth weight, and small-for-gestational age). We measured UCC in the second or third trimester, and categorized the participants using cut-off values (negative cotinine concentration, passive cotinine concentration, and active cotinine concentration corresponding to non-smokers, passive smokers, and active smokers, respectively). Logistic regression analyses were conducted to evaluate the risks, and dose-response relationships were visualized using restricted cubic spline curves. Analyses based on self-reported smoking status were also performed. We found that in low active and highly active cotinine concentrations, the adjusted odds ratios (aORs) of birth outcomes were significantly increased (preterm birth, 1.24 [95% CI 1.06-1.46], 1.39 [95% CI 1.19-1.62]; low birth weight, 1.40 [95% CI 1.24-1.58], 2.27 [95% CI 2.05-2.53]; small-for-gestational age, 1.35 [95% CI 1.19-1.52], 2.39 [95% CI 2.16-2.65]). Restricted cubic spline curves demonstrated risk elevations in the active cotinine concentration range. Our research revealed dose-response relationships between UCC during pregnancy and the risks of preterm birth, low birth weight, and small-for-gestational age. Measurement of UCC to ascertain smoking status during pregnancy may be a useful approach for predicting the risks of these birth outcomes.

    DOI: 10.1016/j.envres.2022.114302

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  • 北海道道北地域における新生児・乳児のビタミンD欠乏状況とビタミンD投与についての検討

    野原 史勝, 津田 淳希, 芳賀 俊介, 高橋 はるか, 浅井 霞, 山木 ゆかり, 高橋 健太, 高橋 弘典, 土田 悦司, 真鍋 博美, 竹田津 原野, 佐藤 敬, 白井 勝, 沖 潤一, 杉山 達俊, 青山 藍子, 二井 光麿, 岡本 年男, 長屋 建, 東 寛

    日本小児臨床薬理学会雑誌   35 ( 1 )   109 - 109   2022.9

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  • Severity of low pre-pregnancy body mass index and perinatal outcomes: the Japan Environment and Children's Study. International journal

    Kentaro Nakanishi, Yasuaki Saijo, Eiji Yoshioka, Yukihiro Sato, Yasuhito Kato, Ken Nagaya, Satoru Takahashi, Yoshiya Ito, Sumitaka Kobayashi, Chihiro Miyashita, Atsuko Ikeda-Araki, Reiko Kishi

    BMC pregnancy and childbirth   22 ( 1 )   121 - 121   2022.2

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    BACKGROUND: The extremes of maternal pre-pregnancy body mass index (BMI) are known to be risk factors associated with obstetric and adverse perinatal outcomes. Among Japanese women aged 20 years or older, the prevalence of underweight (BMI < 18.5 kg/m2) was 11.5% in 2019. Maternal thinness is a health problem caused by the desire to become slim. This study aimed to investigate the association between the severity of maternal low pre-pregnancy BMI and adverse perinatal outcomes, including preterm birth (PTB), low birth weight (LBW), and small-for-gestational age (SGA). METHODS: We conducted a prospective cohort study using data from the Japan Environment and Children's Study, which recruited pregnant individuals between 2011 and 2014. Pre-pregnancy BMI was categorized as severe-moderate underweight (BMI < 16.9 kg/m2), mild underweight (BMI, 17.0-18.4 kg/m2), low-normal weight (BMI, 18.5-19.9 kg/m2), high-normal weight (BMI, 20.0-22.9 kg/m2), overweight (BMI, 23.0-24.9 kg/m2), and obese (BMI ≥ 25.0 kg/m2). The high-normal weight group was used as the reference for statistical analyses. Adjusted logistic regression was performed to evaluate the association between pre-pregnancy BMI and PTB, LBW, and SGA. RESULTS: Of 92,260 singleton pregnant individuals, the prevalence was 2.7% for severe-moderate underweight, 12.9% for mild underweight, and 24.5% for low-normal weight. The prevalence of adverse outcomes was 4.6% for PTB, 8.1% for LBW, and 7.6% for SGA. The adjusted odds ratios (aORs) for PTB were 1.72 (95% confidence interval [CI], 1.46-2.03) for severe-moderate underweight and 1.26 (95% CI, 1.14-1.39) for mild underweight. The aORs of LBW were 2.55 (95% CI, 2.27-2.86) for severe-moderate underweight, 1.64 (95% CI, 1.53-1.76) for mild underweight, and 1.23 (95% CI, 1.16-1.31) for low-normal weight. The aORs of SGA were 2.53 (95% CI, 2.25-2.84) for severe-moderate underweight, 1.66 (95% CI, 1.55-1.79) for mild underweight, and 1.29 (95% CI, 1.21-1.38) for low-normal weight. CONCLUSIONS: A dose-response relationship was found between the severity of low pre-pregnancy BMI and PTB, LBW, and SGA. Even low-normal BMI (18.5-19.9 kg/m2) increased the risk of LBW and SGA. This study provides useful information for pre-conception counseling in lean individuals.

    DOI: 10.1186/s12884-022-04418-3

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  • Reticulocyte hemoglobin content changes after treatment of anemia of prematurity. International journal

    Mitsumaro Nii, Toshio Okamoto, Tatsutoshi Sugiyama, Aiko Aoyama, Ken Nagaya

    Pediatrics international : official journal of the Japan Pediatric Society   64 ( 1 )   e15330   2022.1

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    BACKGROUND: Iron deficiency during infancy is associated with poor neurological development, but iron overload causes severe complications. Appropriate iron supplementation is therefore vital. Reticulocyte hemoglobin content (RET-He) provides a real-time assessment of iron status and chracterezes hemoglobin synthesis in preterm infants. However, the existing literature lacks detailed reports assessing chronological changes in RET-He. The aim of this study was to assess the chronological changes in RET-He during oral iron dietary supplementation, and concomitant therapy with recombinant human erythropoietin (rHuEPO) in preterm very low birthweight infants. METHODS: Very low birthweight infants, admitted to our neonatal intensive care unit were analyzed retrospectively. Hemoglobin (Hb), reticulocyte percentage (Ret), mean corpuscular volume, RET-He, serum iron (Fe), and serum ferritin were recorded. Data at birth (T0), the initial day of rHuEPO therapy (T1), the initial day of oral iron supplementation (T2), 1-2 weeks (T3), 3-4 weeks (T4), 5-6 weeks (T5), and 7-8 weeks (T6) from the initial day of oral iron supplementation were extracted, and their changes over time were examined. RESULTS: Reticulocyte hemoglobin content was highest at birth and declined rapidly thereafter, especially after starting rHuEPO therapy. There was no upward trend in RET-He after the initiation of oral iron supplementation, with a slower increase during 5-6 weeks after the initiation of iron therapy. CONCLUSIONS: During the treatment of anemia of prematurity, low RET-He levels may be prolonged. Anemia of prematurity should therefore be assessed and treated on a case-by-case basis, while considering the iron metabolic capacity of preterm infants.

    DOI: 10.1111/ped.15330

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  • Severe hyponatremia in a neonate with Costello syndrome and CoA during PGE1 infusion. International journal

    Mitsumaro Nii, Hiroko Asai, Fumikatsu Nohara, Toshio Okamoto, Ken Nagaya

    Pediatrics international : official journal of the Japan Pediatric Society   64 ( 1 )   e14984   2022.1

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    DOI: 10.1111/ped.14984

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  • Medical care of newborns born to mothers with confirmed or suspected severe acute respiratory syndrome coronavirus 2 infections in Japan.

    Morioka I, Toishi S, Kusaka T, Wada K, Mizuno K, Committee of Neonatal Medicine in Japan Pediatric Society

    Pediatrics international : official journal of the Japan Pediatric Society   2021.11

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    DOI: 10.1111/ped.14855

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  • Neurally adjusted ventilatory assist in an infant with tracheobronchomalacia. International journal

    Hiroko Asai, Toshio Okamoto, Mitsumaro Nii, Fumikatsu Nohara, Ken Nagaya

    Pediatrics international : official journal of the Japan Pediatric Society   63 ( 11 )   1396 - 1398   2021.11

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    DOI: 10.1111/ped.14625

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  • Retrospective diagnosis of transient abnormal myelopoiesis by using preserved dried umbilical cord. International journal

    Toshio Okamoto, Ken Nagaya, Naohisa Toriumi, Takeo Sarashina, Hiroshi Azuma

    Pediatrics international : official journal of the Japan Pediatric Society   63 ( 10 )   1243 - 1245   2021.10

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    DOI: 10.1111/ped.14583

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  • Urinary Tetrahydrocortisol/Tetrahydrocortisone Ratio at Early Postnatal Period Predict Steroid Dependency and Neonatal Condition in Preterm Infant

    Ken Nagaya, Toshio Okamoto, Fumikatsu Nohara, Mitsumaro Nii, Aiko Aoyama, Tatsutoshi Sugiyama

    2021.6

  • ダイ45カイ ニホン ショウニ ガンカ ガッカイ ; ガクジュツ テンジ 「 シンケイ ガンカ 」

    14 ( 6 )   372 - 376   2021.6

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  • Breastfeeding in a patient with chronic myeloid leukemia during tyrosine kinase inhibitor therapy. International journal

    Ryuta Terao, Mitsumaro Nii, Hiroko Asai, Fumikatsu Nohara, Toshio Okamoto, Ken Nagaya, Hiroshi Azuma

    Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners   27 ( 3 )   756 - 760   2021.4

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    INTRODUCTION: Although imatinib is the first-line of therapy for Philadelphia chromosome (Ph)-positive chronic myeloid leukemia (CML), in Japan, it is recommended by the manufacturer that lactating women treated with imatinib mesylate for CML should discontinue breastfeeding their infants. CASE: A 32-year-old pregnant patient was diagnosed with Ph-positive CML at 13 weeks of gestation. She received imatinib (400 mg/day) after 28 weeks of gestation. A female infant was delivered at a gestational age of 35 weeks and 3/7 days after preterm premature rupture of membranes. It was decided to feed only colostrum to the infant and formula feeding was done subsequently because of the risk of the transfer of imatinib to breast milk. The milk/plasma (M/P) ratio and the relative infant dose (RID) for imatinib were calculated to be 0.35 and 1.4%, respectively at 5 days of life. Moreover, the serum level of imatinib in the child of age 5 days was 27 ng/mL, which was much lower than the target trough value for CML (1000 ng/mL). CONCLUSION: The M/P ratio and RID values for maternally administered imatinib were within the safe range for breastfeeding, as reported in previous studies. In addition, it was found that the serum concentration of imatinib in the child was relatively low during short-term breastfeeding.

    DOI: 10.1177/1078155220948942

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  • Two patients of trisomy 21 with transient abnormal myelopoiesis with hypereosinophilia without blasts in peripheral blood smears. International journal

    Toshio Okamoto, Ken Nagaya, Tatsutoshi Sugiyama, Aiko Aoyama, Mitsumaro Nii, Hiroshi Azuma

    Pediatric hematology and oncology   38 ( 2 )   168 - 173   2021.3

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    Clinical diagnosis of transient abnormal myelopoiesis (TAM) relies on the detection of characteristic blasts and leukocytosis in peripheral blood. We report two patients of trisomy 21 with TAM with hypereosinophilia, who had neither circulating blasts nor leukocytosis. Genetic testing of polymorphonuclear leukocytes isolated from whole blood revealed heterozygous mutations in GATA1, suggesting that the mutations were harbored in increased eosinophils. Both patients had direct hyperbilirubinemia and one died of liver fibrosis. Our findings emphasize the importance of screening for GATA1 mutations in neonatal infants with Down syndrome and hypereosinophilia even if blasts are not detected in peripheral blood smears.

    DOI: 10.1080/08880018.2020.1826070

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  • 非侵襲的陽圧換気療法が有効であった先天性両側声帯麻痺の1例

    今西 梨菜, 杉山 達俊, 二井 光麿, 岡本 年男, 長屋 建

    小児科診療   83 ( 12 )   1813 - 1816   2020.12

  • 北海道道北地域における新生児・乳児のビタミンD欠乏状況とビタミンD投与についての検討

    野原 史勝, 津田 淳希, 芳賀 俊介, 高橋 はるか, 浅井 霞, 山木 ゆかり, 高橋 健太, 高橋 弘典, 土田 悦司, 真鍋 博美, 竹田津 原野, 佐藤 敬, 白井 勝, 沖 潤一, 杉山 達俊, 青山 藍子, 二井 光麿, 岡本 年男, 長屋 建, 東 寛

    日本小児臨床薬理学会雑誌   33 ( 1 )   32 - 38   2020.12

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  • 9番染色体部分トリソミーの1例

    野原 史勝, 二井 光麿, 浅井 洋子, 岡本 年男, 長屋 建, 東 寛, 芳賀 俊介, 大久保 仁史

    日本小児科学会雑誌   123 ( 8 )   1343 - 1343   2019.8

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  • Congenital basal meningoceles with different outcomes: a case series. International journal

    Satomi Okano, Ryosuke Tanaka, Akie Okayama, Etsushi Tsuchida, Fumikatsu Nohara, Nao Suzuki, Toshio Okamoto, Ken Nagaya, Satoru Takahashi, Hiroshi Azuma

    Journal of medical case reports   11 ( 1 )   359 - 359   2017.12

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    BACKGROUND: Basal meningoceles are rare congenital defects and often clinically occult until they result in life-threatening complications. Therefore, it is important to know the diagnostic clues to early diagnosis. CASE PRESENTATION: We describe three cases of congenital basal meningocele in a 3-year-old Japanese boy, a 1-month-old Japanese baby boy, and a 10-month-old Japanese baby girl. One of our patients died of sepsis due to traumatic rupture of the meningocele during nasal suction. His meningocele remained undiagnosed until it resulted in the fatal complication. The other patients underwent surgical repair without any complications. Their meningoceles were complicated by midfacial anomalies including cleft palate and hypertelorism, or a sign of nasal obstruction such as snoring. CONCLUSIONS: These clinical features may be a clue to the early diagnosis of congenital basal meningocele, which enables its safe preoperative management and provides an opportunity for surgical repair before the condition results in serious complications.

    DOI: 10.1186/s13256-017-1497-7

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  • 胎児、胎盤11ベータハイドロキシステロイド脱水素酵素と新生児成熟に関する研究

    長屋 建

    旭川医科大学研究フォーラム   17   66 - 68   2017.3

  • 当院NICUにおけるHamilton C3の使用経験

    野原 史勝, 二井 光麿, 浅井 洋子, 岡本 年男, 長屋 建, 東 寛

    日本小児科学会雑誌   121 ( 2 )   478 - 478   2017.2

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  • 呼吸シミュレーターASL5000を用いた新生児人工呼吸器のトリガー性能とリーク補正性能の検討(Evaluation of trigger accuracy and leak compensation ability of six different ventilators using Active Servo Lung 5000 respiratory simulation system)

    野原 史勝, 二井 光麿, 浅井 洋子, 岡本 年男, 長屋 建

    日本新生児成育医学会雑誌   28 ( 3 )   525 - 525   2016.11

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  • 重症溶血性貧血を合併した孔脳症の1例

    野原 史勝, 土田 悦司, 岡本 年男, 長屋 建, 東 寛

    日本小児科学会雑誌   120 ( 2 )   477 - 477   2016.2

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  • Biliary atresia and stool: its consistency and fat content, another potentially useful clinical information. International journal

    Kazuki Okajima, Ken Nagaya, Hiroshi Azuma, Tatsuya Suzuki

    European journal of gastroenterology & hepatology   28 ( 1 )   118 - 118   2016.1

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  • Novel compound heterozygous mutations in DYNC2H1 in a patient with severe short-rib polydactyly syndrome type III phenotype. International journal

    Toshio Okamoto, Ken Nagaya, Yumi Kawata, Hiroko Asai, Etsushi Tsuchida, Fumikatsu Nohara, Kazuki Okajima, Hiroshi Azuma

    Congenital anomalies   55 ( 3 )   155 - 7   2015.8

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    Short-rib polydactyly syndrome type III is an autosomal recessive lethal skeletal ciliopathy, which is phenotypically similar to nonlethal asphyxiating thoracic dystrophy. Mutations in DYNC2H1 have been identified in both of these disorders, indicating that they are variants of a single disorder. However, short-rib polydactyly syndrome type III is the more severe variant. Here, we report novel compound heterozygous mutations in DYNC2H1 (p.E1894fsX10 and p.R3004C) in a patient with typical short-rib polydactyly syndrome type III phenotype. R3004 is located within the microtubule-binding domain of DYNC2H1, and its substitution is predicted to disrupt the interaction with microtubules. Considering the severe phenotype of our patient, our findings suggest that R3004 may be a key residue for the microtubule-binding affinity of dynein.

    DOI: 10.1111/cga.12098

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  • The temperature change in an endotracheal tube during high frequency ventilation using an artificial neonatal lung model with Babylog® 8000 plus. International journal

    Ken Nagaya, Etsushi Tsuchida, Fumikatsu Nohara, Toshio Okamoto, Hiroshi Azuma

    Pediatric pulmonology   50 ( 2 )   173 - 8   2015.2

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    OBJECTIVE: There is little available data on airway humidity during high-frequency ventilation (HFV). The purpose of this study is to evaluate the temperature drop in an endotracheal tube (ETT) during HFV. METHODS: We examined the airway temperature in a neonatal HFV system using Babylog® 8000 plus. We measured the temperature change of inspired gases in the ETT under various oscillatory frequencies and oscillatory volumes with a fixed base flow. The temperatures in the ETT during HFV were compared with the temperatures during conventional intermittent positive pressure ventilation (IPPV). RESULTS: As the oscillatory frequency was increased and the oscillatory volume (VThf) decreased, the difference in temperature between the Y piece and the inlet of an artificial lung in the ETT (ETT outside of body) increased. However, as the oscillatory frequency increased, there was no difference in the ETT temperature under constant oscillatory volume. In contrast, as the oscillatory volume was decreased, the difference in temperature in the ETT was greater under constant oscillatory frequency. Moreover, the temperature drop in the ETT with HFV was lower than that in the IPPV temperature with a similar respiratory volume. CONCLUSIONS: The temperature change in the ETT was not dependent on the oscillatory frequency when the oscillatory volume was fixed; however, the temperature was dependent on the oscillatory volume when the oscillatory frequency was fixed.

    DOI: 10.1002/ppul.22973

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  • 「致死性」とされる骨系統疾患の5例

    岡本 年男, 川田 友美, 浅井 洋子, 土田 悦司, 野原 史勝, 北村 宏之, 佐々木 吉明, 長屋 建, 東 寛

    日本小児科学会雑誌   118 ( 8 )   1229 - 1236   2014.8

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  • Developmental alterations of physical properties and components of neonatal-infantile stratum corneum of upper thighs and diaper-covered buttocks during the 1st year of life. International journal

    Masako Minami-Hori, Masaru Honma, Mizue Fujii, Wakana Nomura, Kyoko Kanno, Tokinaka Hayashi, Eiki Nakamura, Ken Nagaya, Yuki Miyauchi, Tsutomu Fujimura, Mitsuyuki Hotta, Yutaka Takagi, Takashi Kitahara, Yoshinori Takema, Hajime Iizuka

    Journal of dermatological science   73 ( 1 )   67 - 73   2014.1

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    BACKGROUND: Although physical properties of neonatal-infantile stratum corneum (SC) change drastically after birth, precise developmental alterations of specific sites have not been fully elucidated. OBJECTIVE: To determine the longitudinal alterations of neonatal-infantile SC functions and components of upper thighs and diaper-covered buttocks during the first year of life. The data were compared with those of adults. METHODS: Nineteen full-term neonates and their mothers were subjected to the measurements. Skin hydration, water sorption/retention capacity, TEWL were measured. Superficial SC analyses for NMF, ester binding sebum, and free fatty acids were performed by ATR-FTIR spectrometer. Total amount of ceramides (CERs) and CER subclasses were analyzed by NPLC-ESI-MS. RESULTS: SC hydration of neonatal thighs was lower than that of their mothers, which rapidly increased during the 1st month. Skin hydration of neonatal buttocks was similar to that of their mothers. This also rapidly increased during the 1st month. The neonatal TEWL was less than those of their mothers indicating more efficient barrier function at both sites, which significantly increased during the 1st year development. This was mostly correlated decreased in the ω-hydroxy fatty acid-esterified CERs. Superficial ester-binding sebum content of neonates was similar to that of their mothers, which significantly decreased during the measurement; the decrease was more marked on buttocks. Neither NMF nor FFA of the superficial SC showed significant alteration during the 1-year development. CONCLUSION: Our results indicate that physical functions and components of neonatal-infantile SC show considerable alterations between diaper-covered buttocks and upper thighs during the 1st year development.

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  • Thrombosed dural sinus malformation in a fetus: a case report. International journal

    Hiroko Asai, Toshio Okamoto, Etsushi Tsuchida, Fumikatsu Nohara, Ken Nagaya, Hiroshi Azuma

    Journal of neuroimaging : official journal of the American Society of Neuroimaging   24 ( 6 )   603 - 606   2014

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    BACKGROUND AND PURPOSE: A thrombosed dural sinus malformation (DSM) is a rare condition, the clinical features of which have not yet been completely characterized. Here, we describe the clinical course of a patient with a thrombosed DSM and discuss the outcomes in live birth cases from a review of the literature. CASE DESCRIPTION: An ultrasonography examination of a 32-year-old woman at 25 weeks' gestation indicated a fetal posterior fossa mass. The size of the intracranial mass remained constant during the second trimester and was observed to decrease from 33 weeks of gestation. A postnatal diagnosis of thrombosis in the dural sinus was established by magnetic resonance imaging and venography. No brain damage or hydrocephalus was noted. Although the circumference of the infant's head was enlarged at birth, her neurological outcome was normal at 1 year of age. CONCLUSIONS: Although normal cranial circumference is reportedly an essential factor for a favorable prognosis, the patient in this report with a cranial circumference at + 2.0 SD (35.6 cm) had a favorable prognosis. Further studies focused on improving clinical diagnostic accuracy in this rare entity will facilitate appropriate counseling.

    DOI: 10.1111/jon.12099

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  • Neonatal pleural empyema caused by emm type 6 group A streptococcus. International journal

    Fumikatsu Nohara, Ken Nagaya, Hiroko Asai, Etsushi Tsuchida, Toshio Okamoto, Tokitsugi Hayashi, Hiroshi Sakata, Yutaka Terao, Hiroshi Azuma

    Pediatrics international : official journal of the Japan Pediatric Society   55 ( 4 )   519 - 21   2013.8

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    Since the mid-1980s, there have been increasing reports of severe invasive group A streptococcal (GAS) disease in children and adults. There are few reports, however, of neonatal invasive disease, particularly neonatal pleural empyema caused by GAS. Although many mechanisms have been reported for the pathophysiology of invasive GAS infections, similar reports for neonates were unable to be located. Reported herein is the case of a 3-day-old girl with pleural empyema caused by GAS that demonstrated a high invasive capacity for human epithelial cells.

    DOI: 10.1111/ped.12061

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  • 新生児期に血便・胆汁性嘔吐で発症しアレルゲン特異的リンパ球刺激試験(ALST)で診断した新生児-乳児消化管アレルギーの1症例

    佐藤 祐子, 坪田 朋佳, 新宅 茂樹, 堀井 百祐, 真鍋 博美, 中村 英記, 平野 至規, 室野 晃一, 川田 友美, 浅井 洋子, 土田 悦司, 野原 史勝, 岡本 年男, 長屋 建

    名寄市立病院医誌   21 ( 1 )   43 - 45   2013.7

  • Evaluation of glycated hemoglobin and fetal hemoglobin-adjusted HbA1c measurements in infants. International journal

    Shigeru Suzuki, Masafumi Koga, Noriyasu Niizeki, Akiko Furuya, Kumihiro Matsuo, Yusuke Tanahashi, Etsushi Tsuchida, Fumikatsu Nohara, Toshio Okamoto, Ken Nagaya, Hiroshi Azuma

    Pediatric diabetes   14 ( 4 )   267 - 72   2013.6

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    BACKGROUND: As the presence of fetal hemoglobin (HbF) affects the accuracy of hemoglobin A1c (HbA1c) analysis methods, HbA1c measurement may not be a good indicator for patients with neonatal diabetes mellitus, whereas glycated albumin (GA) may be a good indicator. OBJECTIVE: To investigate whether total glycated hemoglobin (GHb) or HbF-adjusted HbA1c (adj-HbA1c) can act as a glycemic control marker in infants. SUBJECTS AND METHODS: Plasma glucose (PG), GA, HbF, GHb measured using the affinity method, and HbA1c measured using the latex-immunoturbidimetry (LA) or the high-performance liquid chromatography (HPLC) methods were determined in 26 full-term newborn infants aged 4-234 d. Adj-HbA1c was calculated as HbA1c/(total Hb - HbF). RESULTS: GHb, adj-HbA1c measured using the LA and the HPLC methods were 4.8 ± 0.5%, 4.5 ± 0.5%, and 4.7 ± 0.6%, respectively. GA was most positively correlated with PG (r = 0.696, p < 0.0001). GHb was positively correlated with both PG (r = 0.479, p = 0.013) and GA (r = 0.727, p < 0.0001). Adj-HbA1c measured using the LA method was positively correlated with GA (r = 0.465, p = 0.017), but not PG (r = 0.304, p = 0.132). Adj-HbA1c measured using the HPLC method was correlated with neither PG (r = -0.077, p = 0.710) nor GA (r = 0.360, p = 0.071). CONCLUSIONS: GHb measured using the affinity method may be a useful glycemic control marker in infants. Although adj-HbA1c measured using the LA method was correlated with GA, it may not be a practical measure because it was not correlated with PG and determining HbF levels using HPLC method can be troublesome. Adj-HbA1c measured using the HPLC method should not be used as a glycemic marker in infants.

    DOI: 10.1111/pedi.12013

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  • 先天性食道閉鎖症を合併したPOR欠損症の1例

    野原 史勝, 川田 友美, 浅井 洋子, 土田 悦司, 岡本 年男, 長屋 建, 東 寛

    日本周産期・新生児医学会雑誌   49 ( 2 )   784 - 784   2013.6

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  • Glycated albumin is lower in infants than in adults and correlated with both age and serum albumin. International journal

    Shigeru Suzuki, Masafumi Koga, Noriyasu Niizeki, Akiko Furuya, Hironori Takahashi, Kumihiro Matsuo, Yusuke Tanahashi, Yumi Kawata, Hiroko Asai, Etsushi Tsuchida, Fumikatsu Nohara, Toshio Okamoto, Ken Nagaya, Hiroshi Azuma

    Pediatric diabetes   14 ( 1 )   25 - 30   2013.2

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    BACKGROUND: Glycated albumin (GA) reflects glycemic control in patients with neonatal diabetes mellitus (NDM). However, GA in NDM patients is apparently low in relation to glycemia. OBJECTIVE: To establish the reference intervals for GA in healthy infants. SUBJECTS AND METHODS: Fifty-eight healthy, full-term newborn infants were used to define the GA reference values and to investigate its relationship to plasma glucose (PG) and serum albumin. The infants were categorized into three groups according to age: group A, 5 (4-6) median (range) d: n = 18; group B, 33 (30-38) d: n = 19; and group C, 181 (50-352) d: n = 21. We also studied 212 non-diabetic adults [group D, 53 (28-78) yr old] and the 5 NDM patients previously reported for GA comparisons. RESULTS: In the infants, GA was strongly positively correlated with logarithmic transformation of age [log (age)] (p = 0.831, p < 0.0001). The GA in groups A, B, C, and D were 7.3 ± 1.0%, 8.6 ± 1.1%, 10.9 ± 0.8%, and 14.0 ± 1.1%, respectively. The GA was more strongly positively correlated with serum albumin (r = 0.768, p < 0.0001) than with PG (r = 0.596, p < 0.0001). When GA levels were compared with the age-dependent reference values, GA in the transient NDM patient was normalized although GA in the four permanent NDM patients decreased but remained high after insulin therapy. CONCLUSIONS: This study showed that the reference range for GA in infants is lower than that of adults and increases with age, with which we confirmed that GA in the NDM patients reflected the clinical course. Consequently, GA in NDM patients should be compared with the age-based reference values to assess the accurate glycemic status.

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  • Platyspondylic lethal dysplasia torrance type with a heterozygous mutation in the triple helical domain of COL2A1 in two sibs from phenotypically normal parents. International journal

    Toshio Okamoto, Ken Nagaya, Hiroko Asai, Etsushi Tsuchida, Fumikatsu Nohara, Tokitsugi Hayashi, Akiko Yamashita, Gen Nishimura, Hiroshi Azuma

    American journal of medical genetics. Part A   158A ( 8 )   1953 - 6   2012.8

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    Heterozygous COL2A1 mutations create a group of skeletal dysplasias collectively termed type II collagenopathies. Sporadic cases of type II collagenopathies are almost exclusively caused by de novo mutations. Very few cases with intrafamilial recurrence due to germinal mosaicism have been known. We report here on a family in which a severe form of skeletal dysplasia was recurrent in two sibs whose phenotype was most consistent with platyspondylic lethal skeletal dysplasia Torrance type (PLSD-T). A COL2A1 analysis showed that the two sibs had a heterozygous mutation in the encoded triple helical region of COL2A1, c.3545G>A (p.Gly1182Asp) in exon 50. The parents did not consent to a molecular analysis; however, the presence of the same mutation in the two sibs is proof of germinal mosaicism in one of the parents. PLSD-T has been shown to arise from a heterozygous dominant negative COL2A1 mutation in the encoded C-propeptide region. However, our observation suggests that the phenotype is also caused by a COL2A1 mutation in the encoded C-terminal triple helical region.

    DOI: 10.1002/ajmg.a.35509

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  • Human parechovirus 3型による新生児脳症の1例

    野原 史勝, 浅井 洋子, 土田 悦司, 岡本 年男, 長屋 建, 東 寛

    日本周産期・新生児医学会雑誌   48 ( 2 )   505 - 505   2012.6

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  • Sequential changes in pathophysiology of systemic inflammatory response in a disseminated neonatal herpes simplex virus (HSV) infection. International journal

    Tsunehisa Nagamori, Shin Koyano, Yoko Asai, Fumikatsu Nohara, Toshio Okamoto, Ken Nagaya, Tokitsugi Hayashi, Yurika Miura, Naoya Tsuda, Kenichi Iseki, Hiroshi Azuma

    Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology   53 ( 3 )   265 - 7   2012.3

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    BACKGROUND: Disseminated neonatal herpes simplex virus (HSV) infection causes a typical systemic inflammatory response syndrome and has a high mortality rate. However, the validity of anti-inflammatory intervention against this condition remains unknown. OBJECTIVES: We sought to demonstrate the sequential changes in the pathophysiology of disseminated neonatal HSV infections. STUDY DESIGN: The HSV serum copy number as well as high-mobility group box 1 (HMGB1) and cytochrome c concentrations, which predict the severity and mortality rate of sepsis, were sequentially evaluated in a patient with disseminated neonatal HSV infection caused by HSV-2. RESULTS: As the patient presented with evidence of hyper-inflammation and severe illness, we empirically undertook anti-inflammatory intervention that included the administration of prednisolone, high-dose immunoglobulin, and blood exchange therapy in addition to high-dose acyclovir (ACV) therapy. The patient survived without significant neurological sequela. We found that (1) the serum concentrations of both HMGB1 and cytochrome c were extremely high, (2) temporal increases in these biomarkers were observed after admission, and (3) interestingly, the increase in HMGB1 level preceded that of cytochrome c. These results suggested that the pathophysiology of this condition changed sequentially in a dramatic manner, and the timing of our anti-inflammatory intervention was prior to the transition of pathological status from hyper-inflammation to massive apoptosis. CONCLUSIONS: Anti-inflammatory intervention may only be effective if it is undertaken during the early phase of disseminated neonatal HSV infections.

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  • Maternal deletion allele of Angiotensin-converting enzyme gene is associated with fetal growth restriction. International journal

    Eiki Nakamura, Toshio Okamoto, Ken Nagaya, Tokitsugi Hayashi

    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society   15 ( 2 )   114 - 7   2012

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    To investigate the correlation between maternal and fetal angiotensin-converting enzyme gene insertion or deletion polymorphism and birth size, angiotensin-converting enzyme gene insertion or deletion polymorphisms of 470 Japanese pairs of mothers and infants were genotyped. The relationships between maternal and fetal angiotensin-converting enzyme gene insertion or deletion polymorphisms and birth sizes (weight, length, head circumference, and the incidence of small-for-gestational age status) were analyzed. No relationship between fetal angiotensin-converting enzyme genotype and birth size was observed. In contrast, the incidence of small-for-gestational age status in babies whose mothers had 1 or 2 deletion alleles was greater than that in babies whose mothers had 2 insertion alleles (7% vs 3%, P  =  0.045). Maternal deletion allele of angiotensin-converting enzyme is associated with fetal growth restriction in the Japanese normal population.

    DOI: 10.2350/11-09-1092-OA.1

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  • 脳室内出血・出血後水頭症を合併した先天性ネフローゼ症候群の1例

    高橋 弘典, 青山 藍子, 浅井 洋子, 鈴木 滋, 松尾 公美浩, 棚橋 祐典, 野原 史勝, 岡本 年男, 長屋 建, 林 時仲, 柿崎 秀宏, 中西 浩一, 佐々木 聡, 森田 研, 野々村 克也

    日本小児腎不全学会雑誌   31   162 - 164   2011.7

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  • 膿胸を呈した新生児A群溶連菌感染症における重症化因子の検討

    野原 史勝, 土田 悦司, 岡本 年男, 長屋 建, 林 時仲, 坂田 宏

    日本周産期・新生児医学会雑誌   47 ( 2 )   483 - 483   2011.6

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  • 5.78 Mb terminal deletion of chromosome 15q in a girl, evaluation of NR2F2 as candidate gene for congenital heart defects. International journal

    Eiki Nakamura, Yoshio Makita, Toshio Okamoto, Ken Nagaya, Tokitsugi Hayashi, Masaya Sugimoto, Hiromi Manabe, Genya Taketazu, Hiroki Kajino, Kenji Fujieda

    European journal of medical genetics   54 ( 3 )   354 - 6   2011

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    All patients with terminal deletion of chromosome 15q have been reported to show intrauterine growth retardation, postnatal growth retardation, abnormal facial appearance and developmental delay. Haploinsufficiency of IGF1R was considered to be responsible for these symptoms. However, it is difficult to explain other symptoms seen in some of the patients, such as congenital heart defects by the absence of IGF1R alone. Here, we reported a patient with congenital heart defects and a 5.78 Mb terminal deletion of chromosome 15q detected by array-CGH. Among the patients reported to share congenital heart defects and terminal deletion of chromosome 15q, our patient had the smallest deletion. Evaluating the deletion map, NR2F2 was considered a candidate gene contributing to congenital heart defects in patients with terminal deletion of chromosome 15q.

    DOI: 10.1016/j.ejmg.2010.12.004

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  • Patient reports: Two novel frameshift mutations in the SOX9 gene in two patients with campomelic dysplasia who showed long-term survival. International journal

    Toshio Okamoto, Eiki Nakamura, Ken Nagaya, Tokitsugi Hayashi, Tokuo Mukai, Kenji Fujieda

    Journal of pediatric endocrinology & metabolism : JPEM   23 ( 11 )   1189 - 93   2010.11

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    Campomelic dysplasia (CD) is a rare and usually fatal congenital skeletal disorder with respiratory failure. The SOX9 gene has been cloned as a candidate gene for CD. Here, we report the cases of 2 Japanese patients with CD who have survived for over 5 years. Molecular investigations revealed novel frameshift mutations in SOX9 in these patients; a single G insertion in 1 allele at nucleotide 261 (261-262insG) and a single C insertion in 1 allele at nucleotide 888 (888-889insC). The predicted protein of 261-262insG may lack more than 80% composition of the normal SOX9 protein, including the SRY high mobility group (HMG) domain and the transactivation (TA) domain; the predicted protein of 888-889insC may not contain the normal TA domain. Although it has been reported that most patients with CD die during the neonatal period, our patients have survived for a long time, despite putative severely impaired SOX9 proteins.

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  • 商用FISHプローブにてモザイクが検出されたMiller-Dieker症候群の1例

    蒔田 芳男, 長屋 建, 林 時仲, 高橋 悟, 藤枝 憲二, 林 深, 井本 逸勢, 稲澤 譲治

    日本小児科学会雑誌   114 ( 7 )   1087 - 1088   2010.7

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  • Increased expression of matrix metalloproteinase-9 and hepatocyte growth factor in the cerebrospinal fluid of infants with posthemorrhagic hydrocephalus. International journal

    Toshio Okamoto, Satoru Takahashi, Eiki Nakamura, Ken Nagaya, Tokitsugi Hayashi, Masaru Shirai, Kenji Fujieda

    Early human development   86 ( 4 )   251 - 4   2010.4

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    BACKGROUND: In approximately 60% of infants with posthemorrhagic hydrocephalus (PHH), ventricular dilation resolves by unknown intrinsic mechanisms, without the need for a shunt operation. A pathological hallmark of PHH is extensive deposition of extracellular matrix (ECM) proteins in the subarachnoid space. Our previous study revealed that matrix metalloproteinase (MMP)-9, which degrades ECM proteins, may play an important role in the resolution of ventricular dilation. MMP-9 is known to be induced by hepatocyte growth factor (HGF) in various cell lines. AIMS: The aim of this study is to confirm our earlier finding that MMP-9 contributes to the resolution of PHH, and to investigate whether HGF also contributes to this process. STUDY DESIGN: Cerebrospinal fluid (CSF) samples were collected from 13 infants who developed ventricular dilation after intraventricular hemorrhage (IVH). Of these infants, 9 exhibited resolution of ventricular dilation without shunt operation; however, 4 infants had to be treated with shunt operation. The CSF levels of MMP-9 and HGF were measured using an enzyme immunoassay. RESULTS: Significantly higher CSF levels of MMP-9 and HGF were detected in patients in whom the ventricular dilation resolved without shunt operation than in those with progressive ventricular dilation (MMP-9: median, 128ng/ml; range, 47-900ng/ml vs median, 50ng/ml; range, 12-110ng/ml; p<0.05; HGF: median, 2.42ng/ml; range, 0.81-7.04ng/ml vs median, 1.42ng/ml; range, 0.67-3.87ng/ml; p<0.05). CONCLUSIONS: Our results indicate that MMP-9 and HGF may participate in the resolution of ventricular dilation following IVH.

    DOI: 10.1016/j.earlhumdev.2010.03.007

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  • 胎児発育障害とインスリン様成長因子(IGFs)の役割

    長屋 建, 藤枝 憲二

    日本小児科学会雑誌   113 ( 8 )   1195 - 1204   2009.8

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  • Paternal allele of IGF2 gene haplotype CTG is associated with fetal and placental growth in Japanese. International journal

    Ken Nagaya, Yoshio Makita, Genya Taketazu, Toshio Okamoto, Eiki Nakamura, Tokitsugi Hayashi, Kenji Fujieda

    Pediatric research   66 ( 2 )   135 - 9   2009.8

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    IGF-II associates with feto-placental growth in rodent and human. We determined three tag-single nucleotide polymorphisms (SNPs) to investigate haplotype frequency of IGF2 relative to size at birth in 134 healthy Japanese infants. In addition, a total of 276 healthy infants were investigated to determine whether common genetic variation of IGF2 might contribute to feto-placental growth using haplotype analysis. Further, quantitative methylation analysis of the IGF2/H19 was performed using the MassARRAY Compact system. In the initial study, the frequency of haplotype CTG from the paternal allele in small for date (SFD) infants was significantly higher than that in non-SFD infants (p = 0.03). In a second study, the CTG haplotype infants exhibited significantly lower birth length, weight, and placental weight compared with non-CTG infants. Further, the number of infants less than -1.5 SD (SD) birth weight in CTG haplotype was higher than those in non-CTG infants. There was no significant difference in the methylation status of H19/IGF2 in the two haplotypes. In conclusion, inheriting the IGF2 CTG haplotype from a paternal allele results in reduced feto-placental growth, but it is not associated with the methylation status of IGF2/H19.

    DOI: 10.1203/PDR.0b013e3181a9e818

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  • Transforming growth factor-beta1 induces matrix metalloproteinase-9 expression in human meningeal cells via ERK and Smad pathways. International journal

    Toshio Okamoto, Satoru Takahashi, Eiki Nakamura, Ken Nagaya, Tokitsugi Hayashi, Kenji Fujieda

    Biochemical and biophysical research communications   383 ( 4 )   475 - 9   2009.6

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    Transforming growth factor (TGF)-beta1, a cytokine released into the cerebrospinal fluid (CSF) after intraventricular hemorrhage (IVH), stimulates the expression of the components of the extracellular matrix (ECM), which causes progressive ventricular dilatation by impaired CSF absorption. Matrix metalloproteinase-9 (MMP-9), a proteinase involved in the removal of ECM proteins, has been shown to contribute to the resolution of progressive ventricular dilation after IVH. The aim of this study is to clarify the mechanism by which MMP-9 is expressed following IVH. Cultured human meningeal cells were treated with human recombinant TGF-beta1. RT-PCR demonstrated that TGF-beta1 induced MMP-9 expression in the meningeal cells in a dose-dependent manner. The TGF-beta1-induced MMP-9 expression was attenuated in the presence of either MEK or Smad 3 inhibitor. Our data indicated that MMP-9 is released into the CSF from meningeal cells in response to TGF-beta1, most probably through the activation of ERK and Smad pathways.

    DOI: 10.1016/j.bbrc.2009.04.038

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  • Airway humidification with a heated wire humidifier during high-frequency ventilation using Babylog 8000 plus in neonates. International journal

    Ken Nagaya, Toshio Okamoto, Eiki Nakamura, Tokitsugi Hayashi, Kenji Fujieda

    Pediatric pulmonology   44 ( 3 )   260 - 6   2009.3

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    OBJECTIVE: Little data are available on airway humidity during high-frequency ventilation (HFV). Our purpose is to evaluate the airway humidification during HFV. METHODS: We examined the airway humidification and temperature in a neonatal HFV system using Babylog 8000 plus. The absolute humidity (AH), relative humidity (RH), and temperature at different sites and under different HFV conditions were compared with those during conventional intermittent positive pressure ventilation (IPPV). RESULTS: The mean AH and RH at the patient end of the respiratory circuit under 37 degrees C in the humidification chamber (HC) during HFV were less than 35 mg/L and 65%, respectively, while those during IPPV were 42.3 mg/L and 96.8%, respectively. The humidification at the outlet of the HC was similar results. Moreover, during HFV an increase in the bias-flow of ventilator led to a further decrease in the humidity at the patient end of respiratory circuit and the outlet of HC. It was necessary to set the temperature in the HC at >39 degrees C to maintain adequate humidity at the HC and the patient end of respiratory circuit during HFV. An increase in the incubator temperature led to an increase in the temperature at the patient end of the respiratory circuit. The temperature at the patient end of the respiratory circuit was about 39-40 degrees C when the incubator temperature was 35-37 degrees C. CONCLUSIONS: The airway humidification at the patient end of respiratory circuit and the outlet of HC in HFV were poorer than those in IPPV. However, the adequacy of humidification and safety in HFV remain to be demonstrated in clinical practice.

    DOI: 10.1002/ppul.20990

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  • 胎児・新生児期の副腎機能

    藤枝 憲二, 長屋 建

    日本未熟児新生児学会雑誌   21 ( 1 )   33 - 38   2009.2

  • 多血症に対する部分交換輸血療法の再評価 生理食塩水と5%アルブミンの比較

    中村 英記, 岡本 年男, 長屋 建, 林 時仲, 藤枝 憲二

    日本周産期・新生児医学会雑誌   44 ( 3 )   702 - 705   2008.8

  • ボセンタン投与によりECMOとNO吸入療法から離脱できた新生児遷延性肺高血圧症の1例

    中村 英記, 岡本 年男, 中右 弘一, 真鍋 博美, 杉本 昌也, 長屋 建, 林 時仲, 梶野 浩樹, 藤枝 憲二

    周産期医学   38 ( 3 )   369 - 373   2008.3

  • Matrix metalloproteinases in infants with posthemorrhagic hydrocephalus. International journal

    Toshio Okamoto, Satoru Takahashi, Eiki Nakamura, Ken Nagaya, Tokitsugi Hayashi, Masaru Shirai, Kenji Fujieda

    Early human development   84 ( 2 )   137 - 9   2008.2

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    The cerebrospinal fluid matrix metalloproteinase (MMP) activities were measured in infants with posthemorrhagic hydrocephalus to elucidate the intrinsic mechanism for the resolution of ventricular dilation. Increased MMP-9 activities were observed in the patients who escaped a shunt operation, suggesting its potential contribution to the resolution of ventricular dilation.

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  • The corrected blood urea nitrogen predicts the developmental quotient of extremely low-birth-weight infants at the corrected age of 36 months. International journal

    Ken Nagaya, Shinya Tanaka, Hiroyuki Kitajima, Masanori Fujimura

    Early human development   83 ( 5 )   285 - 91   2007.5

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    BACKGROUND: Currently, there are no nutritional indices to predict cognitive function in extremely low-birth-weight (ELBW) infants. OBJECTIVE: To assess the neonatal blood urea nitrogen (BUN) values in ELBW infants according to their cognitive function at the corrected age of 36 months. METHODS: This was a retrospective study that assessed the neonatal factors affecting the developmental outcome in two groups "developmental quotient (DQ)> or =80" and "DQ<80", the groups were divided based on the DQ at the corrected age of 36 months. Between 1996 and 1999, 178 ELBW infants born at <28 weeks of gestation were admitted to our neonatal intensive care unit (NICU), of these, 32 died. Of the surviving 146 infants, 37 infants without any exclusion criteria (that would affect the cognitive function and BUN) except the nutritional factor, were assessed. Area under the curve (AUC) of corrected BUN (CBUN: BUN x 0.5/serum creatinine) from 28 to 84 days of life was used as an index of protein intake. RESULTS: No significant differences were observed between the two groups with regard to the gestational age, birth weight, Z score of birth weight, and sex. However, compared to 15 infants with DQ<80, 22 infants with DQ> or =80 had significantly shorter duration of artificial ventilation and O(2) supplementation, a higher Apgar score at 5 min, and a higher AUC of CBUN. On multiple regression analysis, DQ> or =80 was observed to be significantly correlated with the AUC of CBUN (Odd's ratio 1.03, 95% confidence interval: 1.002-1.06). CONCLUSION: The CBUN level would provide an estimate of adequate protein intake and the subsequent development of an ELBW infant.

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  • 胎児発育とIGF

    ナガヤ, ケン, タケタズ, ゲンヤ, フジエダ, ケンジ

    55 ( 4 )   335 - 341   2007.4

  • 北海道における脊髄髄膜瘤患者数の推移(1995~2004)

    中村 英記, 長屋 建, 竹田津 原野, 林 時仲, 藤枝 憲二

    臨床小児医学   54 ( 5-6 )   133 - 135   2006.12

  • 臍帯潰瘍を合併した先天性上部消化管閉鎖症の2例

    長屋 建, 中村 英記, 竹田津 原野, 林 時仲, 藤枝 憲二

    日本周産期・新生児医学会雑誌   42 ( 3 )   684 - 688   2006.8

  • 母親による母乳注入が母乳分泌に与える有効性の検討

    栗原 かおる, 阿部 明美, 本村 勅子, 久保 治美, 中村 英記, 長屋 建, 竹田津 原野, 林 時仲, 藤枝 憲二

    近畿新生児研究会会誌   ( 14 )   13 - 18   2005.11

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  • ハイリスク児のRSウイルス下気道感染症に対するパリビズマブの予防効果

    林 時仲, 岡本 年男, 中村 英記, 長屋 建, 竹田津 原野, 藤枝 憲二

    日本小児科学会雑誌   109 ( 3 )   359 - 362   2005.3

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  • 新生児慢性肺疾患に対する十二指腸チューブ栄養の効果

    長屋 建, 田中 真也, 北島 博之, 藤村 正哲

    大阪府立母子保健総合医療センター雑誌   20 ( 2 )   51 - 54   2005.3

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  • 【内分泌 興味ある症例】SOX9遺伝子に新規変異を同定したcampomelic dysplasiaの1例

    岡本 年男, 長屋 建, 竹田津 原野, 林 時仲, 向井 徳男, 藤枝 憲二

    ホルモンと臨床   52 ( 冬季増刊 )   140 - 143   2004.12

  • 早産児に対する母親による母乳注入の有効性

    長屋 建, 林 時仲, 竹田津 原野, 藤枝 憲二

    日本周産期・新生児医学会雑誌   40 ( 3 )   539 - 543   2004.8

  • Neonatal level of blood urea nitrogen in extremely low birth weight infants, and later growth and development at a corrected age of 18 months

    NAGAYA Ken, TANAKA Shinya, KITAJIMA Hiroyuki, FUJIMURA Masanori

    14 ( 2 )   85 - 91   2002.6

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  • 超低出生体重児のBUN値と修正1歳6ヵ月時の発育・発達

    長屋 建, 田中 真也, 北島 博之, 藤村 正哲

    日本未熟児新生児学会雑誌   14 ( 2 )   193 - 199   2002.6

  • FGFR2遺伝子にW290C変異を認めたAntley-Bixler症候群の1例

    侭田 光和, 上松 あゆ美, 室井 純子, 河井 昌彦, 依藤 亨, 中畑 龍俊, 白石 淳, 長屋 建, 岡本 伸彦

    日本小児科学会雑誌   105 ( 5 )   626 - 627   2001.5

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  • マイコプラズマ肺炎の経過中に急性膵炎を合併した小児例

    坂田 宏, 長屋 建, 岡本 年男, 中村 英記, 小久保 雅代, 梶野 真弓, 白井 勝, 丸山 静男

    旭川厚生病院医誌   9 ( 2 )   135 - 137   1999.12

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  • 最近10年間の当院における小児の急性脳炎34例の臨床的検討

    坂田 宏, 石井 朋子, 中村 英記, 長屋 建, 立花 幸晃, 小久保 雅代, 白井 勝, 丸山 静男

    旭川厚生病院医誌   9 ( 1 )   3 - 6   1999.6

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  • A Child Case of Bacteremia due to Rahnella aquatilis

    NAGAYA Ken, SAKATA Hiroshi, SHIRAI Masaru, MARUYAMA Shizuo

    103 ( 5 )   559 - 562   1999.5

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  • 肺炎球菌による菌血症の5例

    岡本 年男, 中村 英記, 長屋 建, 小久保 雅代, 梶野 真弓, 白井 勝, 坂田 宏, 丸山 静男

    日本小児科学会雑誌   103 ( 3 )   355 - 356   1999.3

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  • 免疫グロブリン大量投与中に無菌性髄膜炎を起こした特発性血小板減少性紫斑病の女児例

    長屋 建, 岡本 年男, 中村 英記, 小久保 雅代, 梶野 真弓, 白井 勝, 坂田 宏, 丸山 静男

    旭川厚生病院医誌   8 ( 2 )   135 - 138   1998.12

  • 共通肺静脈閉鎖(Common pulmonary vein atresia)の1例

    梶野 真弓, 岡本 年男, 中村 英記, 長屋 建, 小久保 雅代, 白井 勝, 坂田 宏, 丸山 静男, 津田 尚也, 林 憲一, 郷 一知

    旭川厚生病院医誌   8 ( 2 )   122 - 124   1998.12

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  • 大腸に主病変を認めた好酸球性胃腸炎の小児例

    長屋 建, 片野 俊英, 竹田津 原野

    小児科   39 ( 7 )   859 - 863   1998.6

  • 脳・神経疾患 後遺症なく治癒した急性壊死性脳症の7歳女児例

    片野 俊英, 池上 規恵, 長屋 建

    小児科臨床   51 ( 6 )   1091 - 1095   1998.6

  • 嚢胞性脳室周囲白質軟化症の成因と対策

    白井 勝, 岡本 年男, 中村 英記, 長屋 建, 竹田津 原野, 小久保 雅代, 梶野 真弓, 坂田 宏, 丸山 静男

    旭川厚生病院医誌   8 ( 1 )   9 - 13   1998.6

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  • 新生児単純ヘルペスII型脳炎の1例

    白井 勝, 岡本 年男, 中村 英記, 長屋 建, 小久保 雅代, 梶野 真弓, 坂田 宏, 丸山 静男

    旭川厚生病院医誌   8 ( 1 )   49 - 54   1998.6

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  • 肺炎球菌による菌血症の5例

    坂田 宏, 岡本 年男, 中村 英記, 長屋 建, 小久保 雅代, 梶野 真弓, 白井 勝, 丸山 静男

    旭川厚生病院医誌   8 ( 1 )   43 - 45   1998.6

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  • Propionibacterium acnesによる髄膜炎の1例

    坂田 宏, 池上 和洋, 長屋 建, 白井 勝, 丸山 静男

    臨床小児医学   46 ( 2 )   77 - 79   1998.4

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  • Bacillus cereus敗血症の小児例

    坂田 宏, 長屋 建, 白井 勝, 丸山 静男, 須貝 理香, 池上 和洋, 竹田津 原野

    臨床小児医学   46 ( 2 )   67 - 68   1998.4

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  • Yersinia enterocolitica 0:3腸炎による腸重積の1例

    池上 和洋, 長屋 建, 須貝 理香

    臨床小児医学   46 ( 1 )   15 - 18   1998.2

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  • 臍湿潤,臍周囲皮膚炎を契機に発見された尿膜管遺残症の新生児例

    竹田津 原野, 池上 和洋, 長屋 建, 須貝 理香, 白井 勝, 坂田 宏, 丸山 静男, 宮本 和俊

    旭川厚生病院医誌   7 ( 2 )   145 - 147   1997.12

  • 新生児早期の直接血圧と間接血圧の比較

    白井 勝, 長屋 建, 竹田津 原野, 坂田 宏, 丸山 静男

    旭川厚生病院医誌   7 ( 2 )   116 - 121   1997.12

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  • 診断確定前に腰椎穿刺で脊柱管内出血を起こした血友病Aの乳児例

    長屋 建, 池上 和洋, 須貝 理香

    臨床小児医学   45 ( 6 )   283 - 286   1997.12

  • 低出生体重児の無呼吸発作におけるドキサプラム投与の再検討

    長屋 建, 片野 俊英, 竹田津 原野

    日本新生児学会雑誌   33 ( 3 )   298 - 301   1997.9

  • 低出生体重児におけるarbekacin投与時の腎機能の変動

    坂田 宏, 松原 央, 長屋 建

    日本小児臨床薬理学会雑誌   10 ( 1 )   81 - 82   1997.9

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  • Severe Infection due to Adenovirus Type 7 in Children

    SAKATA Hiroshi, TAKEDA Takahiro, NAGAYA Ken, KATANO Toshihide, TAKETAZU Genya, SHIRAI Masaru, ISHIOKA Toru, MARUYAMA Shizuo

    101 ( 8 )   1291 - 1295   1997.8

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  • 当院NICUにおける新生児仮死症例の短期予後

    石岡 透, 長屋 建, 片野 俊英

    旭川厚生病院医誌   7 ( 1 )   8 - 11   1997.6

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  • Pharmacokinetics of Arbekacin in Low-Birth-Weight Infants

    SAKATA Hiroshi, MARUYAMA Shizuo, ISHIOKA Toru, SHIRAI Masaru, TAKETAZU Genya, KATANO Toshihide, NAGAYA Ken

    101 ( 5 )   930 - 933   1997.5

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  • 脊髄障害を呈した胸椎発生の好酸球性肉芽腫の1例

    坂田 宏, 雨宮 聡, 長屋 建

    小児がん   34 ( 1 )   55 - 57   1997.5

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  • 尿路感染症から菌血症をきたした乳幼児の臨床的検討

    坂田 宏, 長屋 建, 片野 俊英

    臨床小児医学   45 ( 2 )   93 - 95   1997.4

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  • 小児十二指腸潰瘍におけるHelicobacter.pylori除菌療法

    片野 俊英, 松原 央, 長屋 建

    日本小児栄養消化器病学会雑誌   11 ( 1 )   33 - 36   1997.4

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    Language:Japanese   Publisher:(一社)日本小児栄養消化器肝臓学会  

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  • 胎児エコーで異常嚢胞像を呈した7症例の臨床的検討

    竹田津 原野, 長屋 建, 片野 俊英

    旭川厚生病院医誌   6 ( 2 )   131 - 138   1996.12

  • ガストログラフィンによる胃十二指腸造影後に腎盂が描出された1例

    石川 丹, 長屋 建, 公文 和子

    臨床小児医学   44 ( 6 )   295 - 297   1996.12

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  • 気道閉塞で発症した長期停留食道異物の1例

    公文 和子, 長屋 建, 小川 弥生

    臨床小児医学   44 ( 5 )   259 - 263   1996.10

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  • 先天性筋性線維性食道狭窄症の6歳児例

    石川 丹, 公文 和子, 長屋 建

    臨床小児医学   44 ( 4 )   215 - 217   1996.8

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  • Hydrocortisone投与後,グラム陰性桿菌敗血症を発症した小児喘息の2例

    坂田 宏, 松原 央, 長屋 建

    旭川厚生病院医誌   6 ( 1 )   49 - 51   1996.6

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  • 羊水のエリスロポエチンが高値で,一過性の眼球運動異常,筋緊張亢進をみとめた男児例

    佐々木 吉明, 長屋 建, 高瀬 雅史

    日本新生児学会雑誌   31 ( 3 )   557 - 559   1995.9

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    Language:Japanese   Publisher:(一社)日本周産期・新生児医学会  

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    CiNii Research

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  • 生後緊急ドレナージを行い救命しえた巨大嚢腫状胎便性腹膜炎の1例

    長屋 建, 佐々木 吉明, 高瀬 雅史

    臨床小児医学   43 ( 4 )   197 - 200   1995.8

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Books

  • 専門医を目指す周産期診療ワークブック

    日本周産期・新生児医学会周産期診療ワークブック作成委員会(新生児編「発達予後 2.発達に影響を与える病態」、「発達予後 3.脳性麻痺」、「発達予後 4.発達遅滞、神経発達症」)

    メジカルビュー社  2024.6  ( ISBN:9784758321310

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    Total pages:xv, 559p   Language:Japanese  

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MISC

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Research Projects

  • 在宅移行後1年間の医療的ケア児と親を支援する遠隔看護システムの構築

    Grant number:22K11054  2022.4 - 2025.3

    日本学術振興会  科学研究費助成事業  基盤研究(C)

    森 浩美, 矢田 しずえ, 長屋 建

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    Grant amount:\2,600,000 ( Direct Cost: \2,000,000 、 Indirect Cost:\600,000 )

    医療的ケア児は家族と暮らし、社会とつながることにより成長・発達が促進される。一方、親は一緒に暮らせることに喜びを感じつつ、家庭内でのケアは原則、親が行うため不安や負担が大きい。在宅で生活する医療的ケア児の親をサポートする遠隔看護システムを構築する必要があると考えた。本研究の目的は、在宅で生活する医療的ケア児の親をサポートする遠隔看護システムを構築することである。
    令和4年度は、在宅医療的ケア児と同居し、昼夜の医療的ケアを実施する親5~10名を対象に、在宅移行後一年間における医療的ケア児と親の思いとニーズの実態を明らかにする計画であった。現在、対象者1名の面接調査を終了し、そのデータを質的記述的に分析している最中である。対象者は在宅移行直後から現在まで訪問看護ステーションを利用し、困ったことや相談したことがあるときは、電子メール等で連絡を取っていた。訪問看護師からの応答もスムーズであり、いつでも連絡が取れ、相談できる相手がいることは対象者にとってとても心強いことであった。そして、訪問看護師への信頼も深められていることが明らかとなった。これらは、遠隔看護の重要性を示唆するものであると考える。
    また、令和4年12月から小児を対象とする訪問看護ステーションの看護師や病院NICUの看護師等と在宅医療的ケア児とその親を支援する遠隔看護の可能性について、月に一度の意見交換会を開催している。この会を継続し、具体的な方策を検討していきたいと考える。

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  • Feto-Placental 11beta-hydroxysteroid dehydrogenase2 activity affect fetal growth

    Grant number:24591596  2012.4 - 2016.3

    Japan Society for the Promotion of Science  Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

    NAGAYA Ken

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    Grant amount:\5,200,000 ( Direct Cost: \4,000,000 、 Indirect Cost:\1,200,000 )

    Feto-Placental 11 β HSD2 controls glucocorticoid disclosure to fetus. We investigated the influence by which these give it to fetus growth. Placental HSD11B2 mRNA was employed as a placental 11βHSD2 activity. Although urinal cortisone/cortisol metabolism ratio(u-F/E) didn't correlate with placental HSD11B2 mRNA, we thought that u-F/E could estimate fetal 11βHSD2 activity.
    The placental HSD11B2 mRNA in SGA infants was significantly low compared with AGA infants. And, The placental HSD11B2 mRNA was correlated with SDS of the birth weight and birth head circumference respectively. u-F/E in SGA infants was significantly low compared with AGA infants. However, there was no correlation between urine F/E and fetal growth. These relations were admitted in girls.

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  • 候補遺伝子解析並びにハプロタイプ解析による成長障害の成因の同定

    Grant number:21591307  2009 - 2011

    日本学術振興会  科学研究費助成事業  基盤研究(C)

    藤枝 憲二, 長屋 建, 松尾 公美浩

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    Grant amount:\3,120,000 ( Direct Cost: \2,400,000 、 Indirect Cost:\720,000 )

    <CPHD症例において同定したPIT1β変異体の機能解析>
    ヒト野生型PIT1β並びに変異PIT1βcDNAの作成と、それらの発現ベクターへのサブクローニングを終了した。今後、供与していただいた野生型および変異型PIT1cDNA発現ベクター、さらにその標的遺伝子であるPRL、GHのプロモーターと併せて、COS7細胞並びにGH4細胞を用いてルシフェラーゼアッセイを行う予定である。なお、他のCPHD3症例に対して同様にPit1β変異解析を行ったが、いずれにおいても変異は認められなかった。
    <GH不応症例に同定したGH1変異体の機能解析>
    ヒト成長ホルモン受容体を発現したBaF/GM細胞を用いたヒトGH生物学的活性の測定を行った。低身長児群と正常群の間で生物学的活性/免疫学的活性比に有意な差は認められなかった。今後、この系を用いてGH1変異を有するGH不応症例患者血清中のGH生物学的活性の測定を行い、機能解析を進める予定である。
    <低身長児を対象とした成長関連遺伝子解析>
    -2SD未満の低身長児100例を対象にGH1,GHR,IGF1,IGF1R,NPR2についてPCR-ダイレクトシークエンス法を用いて遺伝子解析を行った。その結果、GH1については一例のヘテロ接合性ミスセンス変異(未報告)、GHRについては二例同一の既報のヘテロ接合性ミスセンス変異、IGF1Rでは一例の一塩基挿入(未報告)、そしてNPR2では一例のヘテロ接合性ミスセンス変異(未報告)を同定した。

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  • Impact of glucocorticoid exposure for fetus in fetal development and neonatal disease

    Grant number:20591295  2008 - 2010

    Japan Society for the Promotion of Science  Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

    NAGAYA Ken, FUJIEDA Kenji, MAKITA Yoshio, HAYASHI Tokitugi

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    Grant amount:\4,680,000 ( Direct Cost: \3,600,000 、 Indirect Cost:\1,080,000 )

    The urinary steroid of 61 preemies was measured by using LC-MS/MS. In current study, the index of placental 11ss-hydroxysteroid dehydrogenase type2 (11ssHSD2) substituted for urinary (THF+allo-THF)/THE within 48 hours of life. The infants with low activity of 11ssHSD2 had severer illness than those with high or middle activity of 11ssHSD2. It was suggested that the infants with low placental 11ssHSD2 activity were insufficient for adrenal maturity because they were dependent on their mothers in adrenal function.
    Moreover, we were examined the impact of intrauterine infection (IUI) for neonatal adrenal function in 30 preemies with less than 30 weeks of gestational age. The adrenal fetal zone product (DHEA) for 1 and 2 weeks of life was high in infants with IUI, and the metabolites of cortisol were not difference regardless of IUI. It was suggested that IUI did not lead regression of adrenal fetal zone of neonates but the activity of hypothalamic-pituitary-adrenal axis was increased in infants with IUI.

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  • The association between fetal and postnatal growth and polymorphisms of insulin-like growth factors

    Grant number:17591137  2005 - 2007

    Japan Society for the Promotion of Science  Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

    NAGAYA Ken, MAKITA Yoshio, HAYASHI Tokitsugu, FUJIEDA Kenji

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    Grant amount:\3,200,000 ( Direct Cost: \2,900,000 、 Indirect Cost:\300,000 )

    Objective Insulin like growth factors (IGF1 and IGF2) and their receptors (IGF1R and IGF2R) associate with feto-placental growth. We hypothesized that polymorphism of IGFs gene could alter birth weight and placental weight inhuman.
    Subject and methods: The healthy 287 infants without congenital anomaly, who were born at〓35 weeks of gestational agebetween October 2004 and September 2007 in our hospital and Mori obstetrics gynecology hospital and their mothers were examined about IGFs and their receptors genes. We carried out haplotype analysis between feto-placental growth and IGF1, IGF2, IGF1R and IGF2R. Moreover, we analyzed methylation status of infants IGF2 gene using Mass ARRAY. Moreover, we compared their anthropometric status at 18 month of age according to haplotypes of IGF2.
    Result: Frequency of IGF1 haplotype TGG in heavy for date (HFD) infants was significantly higher than in appropriate for date (AFD) or small for date (SFD) infants. Infants with IGF1 haplotype TGG were significantly heavier birth weight compared with that of non-TGG infants. Hence, frequency of haplotype CTG of paternal IGF2 allele in SFD infants was significantly higher than in AFD or HFD infants. Infants with IGF2 haplotype CTG were significantly shorter birth length, lighter birth weight and placental weight compared with those of non-CTG infants. There was no significant difference in cord serum IGF-2 level regardless of haplotypes. Moreover, haplotype CTG on IGF2 gene did not associate with its methylation status. The infants with IGF2 haplotype CTG caught up their anthropometric status as much as that of non-CTG infants.
    Conclusion: IGF1 haplotype TGG may associate with increase of fetal growth. Haplotype CTG of paternal allele on IGF2 gene may reduce feto-placental growth regardless of its mathylation status, but not associate postnatal growth.

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