Faculty Profiles - TSUKAHARA Hirokazu

Papers - TSUKAHARA Hirokazu

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先天性心疾患術後に施行した冠動脈領域に対する介入についての検討評価・適応について

栗田佳彦, 馬場健児, 近藤麻衣子, 重光祐輔, 福嶋遥佑, 原真祐子, 平井健太, 大月審一, 塚原宏一, 笠原真悟

日本小児循環器学会総会・学術集会抄録集 59回 [I - 02] 2023.7

Quality of life and physical/psychosocial factors in children and adolescents with orthostatic intolerance. Reviewed International journal

Yoshie Shigeyasu, Ayumi Okada, Chikako Fujii, Chie Tanaka, Akiko Sugihara, Makiko Horiuchi, Takashi Yorifuji, Hirokazu Tsukahara

BioPsychoSocial medicine 17 ( 1 ) 23 - 23 2023.6

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Orthostatic intolerance (OI), which is common in children and adolescents, negatively impacts their quality of life (QOL) due to physical symptoms that limit work, school, and daily activities. The purpose of this study is to explore the association of physical and psychosocial factors with QOL scores in children and adolescents with OI. METHODS: A cross sectional observational study was conducted. The study participants included 95 Japanese pediatric patients of age 9-15 years who were diagnosed with OI from April 2010 to March 2020. The QOL scores and the QOL T-scores of children with OI assessed using the KINDL-R questionnaire at the initial visit were compared with conventional normative data. The associations of physical and psychosocial factors with the QOL T-scores were examined using multiple linear regression. RESULTS: Pediatric patients with OI had significantly lower QOL scores than healthy children in both elementary (50.7 ± 13.5 vs. 67.9 ± 13.4, p < 0.001) and junior high schools (51.8 ± 14.6 vs. 61.3 ± 12.6, p < 0.001). This finding was observed in the physical, mental, self-esteem, friends, and school domains. Total QOL scores were significantly associated with school nonattendance (β = - 3.2, 95% confidence interval [CI] = - 5.8 to - 0.5, p = 0.022) and poor relationship with school (β = - 5.0, 95% CI = - 9.8 to - 0.4, p = 0.035). CONCLUSIONS: These results suggest that the assessment of QOL, including both physical and psychosocial aspects, especially school factors, needs to be implemented earlier in children and adolescents with OI.

DOI： 10.1186/s13030-023-00278-1

Early childhood exposure to maternal smoking and obesity: A nationwide longitudinal survey in Japan. Reviewed International journal

Miho Yamashita, Takashi Yorifuji, Naomi Matsumoto, Toshihide Kubo, Hirokazu Tsukahara

Clinical obesity 13 ( 3 ) e12572 2023.6

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

Involuntary exposure to tobacco smoke is suspected to be one of the risks factors that are associated with obesity in children. The purpose of this study was to examine the relationship between early childhood exposure to tobacco smoke and the risk of obesity and overweight in Japan. This study utilized a nationwide, population-based longitudinal survey. The participants were restricted to 32 081 children who had available information on maternal smoking history as well as childhood height and weight. We conducted a binomial log-linear regression analysis with children of non-smoking mothers as the reference group. The children with mothers who were smokers had a higher risk of developing obesity or being overweight compared to the children with mothers who were nonsmokers. The risk ratios were 1.20 (95% confidence interval [CI]: 1.09-1.32) for overweight and 1.17 (95% CI: 0.95-1.44) for obesity. Early exposure to maternal smoking increases the risk of being overweight and having obesity during childhood. The increased risk is more pronounced among children with mothers, smoked heavily, or parents, who were smokers.

DOI： 10.1111/cob.12572

Other Link： https://onlinelibrary.wiley.com/doi/full-xml/10.1111/cob.12572

小児科医に潜在するワクチン忌避(Vaccine Hesitancy)に対する教育プログラム

勝田友博, 天羽清子, 荒木かほる, 石和田稔彦, 大宜見力, 神谷元, 越田理恵, 高野智子, 津川毅, 富樫篤生, 中野貴司, 西村直子, 野崎昌俊, 菱木はるか, 保科隆之, 細矢光亮, 前田明彦, 水野由美, 宮入烈, 宮崎雅仁, 吉川哲史, 和田泰三, 岩田敏, 尾内一信, 岡田賢司, 岡部信彦, 是松聖悟, 清水直樹, 菅秀, 宮崎千明, 森内浩幸, 大城誠, 藤岡雅司, 森岡一朗, 塚原宏一, 齋藤昭彦, 多屋馨子, 日本小児科学会予防接種・感染症対策委員会

日本小児科学会雑誌 127 ( 5 ) 787 - 794 2023.5

Language：Japanese Publisher：(公社)日本小児科学会

予防接種後有害事象(AEFI)および予防接種ストレス関連反応(ISRR)に関連する動画講義視聴が小児科医の予防接種に対する知識、態度、自信、役割認識、自己効力感、行動に与える影響を調査した。介入前評価(pre study)に回答した日本小児科学会会員368名をA群とB群に分けてA群にのみオンラインでISRRに関する約20分間の動画講義を配布し、pre studyから約1ヵ月後にA群・B群に介入後評価(Post study)を行った。Post studyの回答者A群74名、B群95名を対象に動画講義視聴による介入効果について検討した。その結果、AEFIおよびISRRに関連する調査によってA群・B群の知識レベルが向上したが、予防接種に対する態度、自信、役割認識、自己効力感、実際の接種行動に与える影響は限定的であった。

Decreased levels of histidine-rich glycoprotein and increased levels of high-mobility group box 1 are risk factors for refractory Kawasaki disease. Reviewed International journal

Takahiro Namba, Masato Yashiro, Yosuke Fujii, Mitsuru Tsuge, Keyue Liu, Masahiro Nishibori, Hirokazu Tsukahara

Modern rheumatology 33 ( 3 ) 599 - 607 2023.4

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

OBJECTIVES: Histidine-rich glycoprotein (HRG) and high-mobility group box 1 (HMGB1) regulate the activation of neutrophils and vascular endothelium. The aim of this study was to quantify HRG and HMGB1 levels in patients with Kawasaki disease (KD) and evaluate their use in the clinical management of KD. METHODS: This study was prospectively performed. Patients were divided into two groups and analyzed depending on whether KD symptoms improved by day 10 of illness. HRG, HMGB1, and other laboratory variables were measured before the first treatment in all cases and, in most cases, afterwards, for assessing trends. RESULTS: In this prospective study, we enrolled 60 patients with KD and 48 healthy controls. The HRG level in the KD group was significantly lower than that in the healthy control group; HMGB1 levels showed no obvious differences. In the KD group, HRG levels were negatively correlated with white blood cell and neutrophil counts. In the poor responders and responders groups, a tendency for a decrease in HRG and HMGB1 levels, respectively, was observed from pretreatment to post-treatment. CONCLUSIONS: HRG and HMGB1 are related to the pathogenesis of KD; low HRG and high HMGB1 levels cause resistance against KD treatment.

DOI： 10.1093/mr/roac040

Three Cases of Down Syndrome with Transient Abnormal Myelopoiesis who Underwent Liver Biopsy before Induction of Low-Dose Cytarabine. Reviewed

Kana Washio, Kosuke Tamefusa, Motoharu Ochi, Kiichiro Kanamitsu, Hisashi Ishida, Kaori Fujiwara, Kenji Nishida, Kei Tamai, Yosuke Washio, Junko Yoshimoto, Takuo Noda, Hirokazu Tsukahara

Acta medica Okayama 77 ( 2 ) 215 - 220 2023.4

Authorship：Last author Language：English

Among patients with transient abnormal myelopoiesis (TAM) associated with Down syndrome, approximately 20% die within 6 months from multiorgan failure, especially liver fibrosis. We experienced three children with TAM who had low white blood cell counts but increased bilirubin levels. Here, we discuss the detailed clinical courses of these patients, including the pathological findings of liver biopsies. Our cases, together with previous literature, suggest that liver biopsy can be performed safely and provides useful information, especially regarding disease activities, and that low-dose cytarabine is a reasonable option to prevent early death in TAM patients with liver dysfunction.

DOI： 10.18926/AMO/65153

Evaluating the Coping Behavior of Children with Psychosomatic Disorders under Frustrating Situations Simulated Using the Rosenzweig Picture-Frustration Study. Reviewed

Akiko Sugihara, Ayumi Okada, Makiko Horiuchi, Mayumi Yabe, Yoshie Shigeyasu, Chikako Fujii, Chie Tanaka, Takashi Yorifuji, Hirokazu Tsukahara

Acta medica Okayama 77 ( 2 ) 185 - 192 2023.4

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

Psychosomatic disorders are influenced by psychosocial factors such as interpersonal relationships. Coping behaviors, especially in frustrating situations, reflect a patient's ability to cope with stress, and it is important to assess these behaviors for the treatment of psychosomatic diseases. This study aimed to clarify the interpersonal relationships and coping behaviors of pediatric patients with psychosomatic diseases during frustrating situations simulated using the Rosenzweig Picture-Frustration study. This retrospective study included 126 patients (41 male, 85 female) with an average age of 12.9 (6-16) years who were consulted at the Department of Pediatric Psychosomatic Medicine at Okayama University Hospital from 2013 to 2018 and underwent the P-F study. Each score was compared with a standardization sample. The mean group conformity rating did not differ significantly between the participants and healthy children. Compared with healthy children, those with psychosomatic diseases were less likely to explain their perspective. The children with psychosomatic disorders responded to frustrating situations in a sensible and age-appropriate manner. However, they were less likely to respond by explaining their perspective to protect themselves.

DOI： 10.18926/AMO/65148

Evaluation of the association of birth order and group childcare attendance with Kawasaki disease using data from a nationwide longitudinal survey. Reviewed International journal

Takahiro Namba, Akihito Takeuchi, Naomi Matsumoto, Mitsuru Tsuge, Masato Yashiro, Hirokazu Tsukahara, Takashi Yorifuji

Frontiers in pediatrics 11 1127053 - 1127053 2023.3

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Kawasaki disease (KD) is a form of pediatric systemic vasculitis. Although the etiology remains unclear, infections have been identified as possible triggers. Children with a later birth order and those who attend childcare are at a higher risk of infections due to exposure to pathogens from their older siblings and other childcare attendees. However, longitudinal studies exploring these associations are limited. Thus, we aimed to elucidate the relationship between birth order, group childcare attendance, and KD, using a nationwide longitudinal survey in Japan. METHODS: In total, 36,885 children born in Japan in 2010 were included. The survey used questionnaires to identify hospitalized cases of KD. We evaluated the relationship between birth order classification, group childcare attendance, and KD prevalence every year, from 6 to 66 months of age. For each outcome, odds ratios (ORs), and 95% confidence intervals (CIs) were estimated after adjusting for child factors, parental factors, and region of residence. RESULTS: Children with higher birth orders were more likely to be hospitalized with KD at 6-18 months of age (second child OR: 1.77, 95% CI: 1.25-2.51; third child OR: 1.70, 95% CI: 1.08-2.65). This trend was stronger for children who did not attend group childcare (second child OR: 2.51, 95% CI: 1.57-4.01; third child OR: 2.41, 95% CI: 1.30-4.43). An increased risk of KD hospitalization owing to the birth order was not observed in any age group for children in the childcare group. CONCLUSIONS: Children with higher birth orders were at high risk for hospitalization due to KD at 6-18 months of age. The effect of birth order was more prominent among the children who did not attend group childcare.

DOI： 10.3389/fped.2023.1127053

Adverse Reactions and Attitudes Toward the BNT162b2 COVID-19 Vaccine in Children 5 to 11 Years of Age in Japan. Reviewed

Naomi Matsumoto, Junya Shimizu, Yuji Yokoyama, Hirokazu Tsukahara, Takashi Yorifuji

Journal of epidemiology 33 ( 2 ) 110 - 111 2023.2

Longitudinal Measurement of Histidine-Rich Glycoprotein Levels in Bronchopulmonary Dysplasia: A Pilot Study. Reviewed International journal

Daisaku Morimoto, Yosuke Washio, Kei Tamai, Takeshi Sato, Tomoka Okamura, Hirokazu Watanabe, Yu Fukushima, Junko Yoshimoto, Misao Kageyama, Kenji Baba, Hirokazu Tsukahara

Biomedicines 11 ( 1 ) 2023.1

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

Histidine-rich glycoprotein (HRG) has been reported to inhibit signaling leading to the release of high mobility group box 1 protein, a damage-associated molecular pattern. The present study aimed to determine the longitudinal change in HRG levels in extremely preterm infants and assess whether complications such as bronchopulmonary dysplasia (BPD) were associated with differences in HRG levels. In this multicenter, prospective, observational study, we measured serum HRG levels every 2 weeks from birth to 8 weeks of age. Serum HRG was measured using an enzyme-linked immunosorbent assay. We included 19 extremely preterm infants in the study and 74 samples were analyzed. The median gestational age was 26.0 weeks, and the median birth weight was 858 g. Serum HRG levels showed a significant upward trend after birth (p < 0.001); median HRG concentrations at birth and at 2, 4, 6, and 8 weeks of age were 1.07, 1.11, 2.86, 6.05, and 7.49 µg/mL, respectively. Onset of BPD was not associated with differences in serum HRG levels. Further, the serum HRG levels increased significantly after birth in extremely preterm infants.

DOI： 10.3390/biomedicines11010212

Prediction model for nonopiate-induced neonatal abstinence syndrome. Reviewed International journal

Daisaku Morimoto, Yosuke Washio, Takeshi Sato, Tomoka Okamura, Hirokazu Watanabe, Junko Yoshimoto, Hirokazu Tsukahara

Pediatrics international : official journal of the Japan Pediatric Society 65 ( 1 ) e15435 2023.1

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Neonatal abstinence syndrome (NAS) induced by opiate use is common worldwide. While psychiatric drugs are a more common cause of NAS in Japan, infants of mothers taking psychiatric medications do not always develop NAS. The purpose of this study was to develop a practical model for predicting the onset of nonopiate-induced NAS using variables available at birth. METHODS: In this diagnostic study, prediction models were developed using multivariable logistic regression with retrospective data collected at our hospital between 2010 and 2019. NAS diagnosis was based on the Isobe score, and maternal medications were converted to dose equivalents. RESULTS: A total of 164 maternal and infant dyads met the inclusion criteria; 91 were included in the analysis, of whom 29 infants (32%) were diagnosed with NAS. Final models were created with and without the drug indices. The model without the drug indices consisted of neonatal head circumference in z-score and Apgar score at 5 minutes < 9, and the model with the drug indices included these, as well as antipsychotics and hypnotics indices. The C-statistics were 0.747 (95% CI: 0.638-0.856), and 0.795 (95% CI: 0.683-0.907), respectively, indicating good predictive accuracy of NAS onset for the models. CONCLUSIONS: This study developed models that predicted nonopiate-induced NAS with good accuracy, which may be further improved through the use of drug indices.

DOI： 10.1111/ped.15435

Need for re-evaluating the risk of coronavirus disease 2019 transmission to neonates. Reviewed International journal

Kazuhiro Uda, Mitsuru Tsuge, Masato Yashiro, Tomoyuki Honda, Hirokazu Tsukahara

Pediatrics international : official journal of the Japan Pediatric Society 65 ( 1 ) e15460 2023.1

Adverse reactions in young children receiving the coronavirus disease 2019 vaccine. Reviewed International journal

Naomi Matsumoto, Junya Shimizu, Yuji Yokoyama, Hirokazu Tsukahara, Takashi Yorifuji

Pediatrics international : official journal of the Japan Pediatric Society 65 ( 1 ) e15696 2023.1

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

Abstract

Background

We sought to investigate the occurrence of adverse reactions in Japanese children aged 6 months to 4 years who received the BNT162b2 coronavirus disease 2019 (COVID‐19) vaccine, to examine parental considerations, and to evaluate potential risk factors associated with post‐vaccination fever.

Methods

This cross‐sectional survey study targeted 1617 children aged 6 months to 4 years who received their primary doses of BNT162b2 from November 10, 2022, to April 30, 2023, in Okayama Prefecture. We surveyed the occurrence of local and systemic reactions within 1 week after vaccination, and described the incidence proportions of adverse reactions for 515 participants overall and by age group. The study also examined the impact of previous COVID‐19 infection and co‐administration of the seasonal influenza vaccine on post‐vaccination fever. A survey also assessed parents' reasons for vaccinating their children and the sources of information they used.

Results

Adverse reactions were infrequent (5.2%, with fever ≥37.5°C; no cases exceeded 39°C) and did not increase with vaccine doses administered. The risk of post‐vaccination fever was not statistically associated with a history of COVID‐19—the adjusted risk ratio (aRR) was 0.99, and the 95% confidence interval (CI) was 0.41–2.39—but was associated with co‐administration of the seasonal influenza vaccine (aRR 3.24, 95% CI 1.14–9.18). Parental decisions regarding vaccination were influenced by official government guidelines and primary care physicians' opinion.

Conclusion

This study provides valuable insight into the safety profile of the BNT162b2 vaccine in Japanese children aged 6 months to 4 years. Further research involving larger cohorts and appropriate control groups is needed.

DOI： 10.1111/ped.15696

Evaluation of the association of birth order and group childcare attendance with Kawasaki disease using data from a nationwide longitudinal survey. Reviewed International journal

Takahiro Namba, Akihito Takeuchi, Naomi Matsumoto, Mitsuru Tsuge, Masato Yashiro, Hirokazu Tsukahara, Takashi Yorifuji

Frontiers in pediatrics 11 1127053 - 1127053 2023

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Kawasaki disease (KD) is a form of pediatric systemic vasculitis. Although the etiology remains unclear, infections have been identified as possible triggers. Children with a later birth order and those who attend childcare are at a higher risk of infections due to exposure to pathogens from their older siblings and other childcare attendees. However, longitudinal studies exploring these associations are limited. Thus, we aimed to elucidate the relationship between birth order, group childcare attendance, and KD, using a nationwide longitudinal survey in Japan. METHODS: In total, 36,885 children born in Japan in 2010 were included. The survey used questionnaires to identify hospitalized cases of KD. We evaluated the relationship between birth order classification, group childcare attendance, and KD prevalence every year, from 6 to 66 months of age. For each outcome, odds ratios (ORs), and 95% confidence intervals (CIs) were estimated after adjusting for child factors, parental factors, and region of residence. RESULTS: Children with higher birth orders were more likely to be hospitalized with KD at 6-18 months of age (second child OR: 1.77, 95% CI: 1.25-2.51; third child OR: 1.70, 95% CI: 1.08-2.65). This trend was stronger for children who did not attend group childcare (second child OR: 2.51, 95% CI: 1.57-4.01; third child OR: 2.41, 95% CI: 1.30-4.43). An increased risk of KD hospitalization owing to the birth order was not observed in any age group for children in the childcare group. CONCLUSIONS: Children with higher birth orders were at high risk for hospitalization due to KD at 6-18 months of age. The effect of birth order was more prominent among the children who did not attend group childcare.

DOI： 10.3389/fped.2023.1127053

A randomized controlled trial of teprenone in terms of preventing worsening of COVID-19 infection. Reviewed International journal

Eiki Ichihara, Kou Hasegawa, Kenichiro Kudo, Yasushi Tanimoto, Kazuhiro Nouso, Naohiro Oda, Sho Mitsumune, Haruto Yamada, Ichiro Takata, Hideharu Hagiya, Toshiharu Mitsuhashi, Akihiko Taniguchi, Shinichi Toyooka, Kohei Tsukahara, Toshiyuki Aokage, Hirokazu Tsukahara, Katsuyuki Kiura, Yoshinobu Maeda

PloS one 18 ( 10 ) e0287501 2023

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Some COVID-19 patients develop life-threatening disease accompanied by severe pneumonitis. Teprenone induces expression of heat-shock proteins (HSPs) that protect against interstitial pneumonia in preclinical models. We explored whether teprenone prevented worsening of COVID-19 infections. METHODS: This open-label, randomized, pilot phase 2 clinical trial was conducted at five institutions in Japan. We randomized patients hospitalized for COVID-19 with fever to teprenone or no-teprenone groups in a 1:1 ratio. We stratified patients by sex, age < and ≥ 70 years and the existence (or not) of complications (hypertension, diabetes, ischemic heart disease, chronic pulmonary disease and active cancer). No limitation was imposed on other COVID-19 treatments. The primary endpoint was the intubation rate. RESULTS: One hundred patients were included, 51 in the teprenone and 49 in the no- teprenone groups. The intubation rate did not differ significantly between the two groups: 9.8% (5/51) vs. 2.0% (1/49) (sub-hazard ratio [SHR] 4.99, 95% confidence interval [CI]: 0.59-42.1; p = 0.140). The rates of intra-hospital mortality and intensive care unit (ICU) admission did not differ significantly between the two groups: intra-hospital mortality 3.9% (2/51) vs. 4.1% (2/49) (hazard ratio [HR] 0.78, 95%CI: 0.11-5.62; p = 0.809); ICU admission 11.8% (6/51) vs. 6.1% (3/49) (SHR 1.99, 95%CI: 0.51-7.80; p = 0.325). CONCLUSION: Teprenone afforded no clinical benefit. TRIAL REGISTRATION: Japan Registry of Clinical Trials jRCTs061200002 (registered on 20/May/2020).

DOI： 10.1371/journal.pone.0287501

Novel and recurrent COMP gene variants in five Japanese patients with pseudoachondroplasia: skeletal changes from the neonatal to infantile periods. Reviewed

Kosei Hasegawa, Natsuko Futagawa, Yuko Ago, Hiroyuki Miyahara, Daisuke Harada, Mari Miyazawa, Junko Yoshimoto, Kenji Baba, Tadashi Moriwake, Hiroyuki Tanaka, Hirokazu Tsukahara

Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology 32 ( 4 ) 221 - 227 2023

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

Pseudoachondroplasia (PSACH) is an autosomal dominant skeletal dysplasia caused by pathogenic variants of cartilage oligomeric matrix protein (COMP). Clinical symptoms of PSACH are characterized by growth disturbances after the first year of life. These disturbances lead to severe short stature with short limbs, brachydactyly, scoliosis, joint laxity, joint pain since childhood, and a normal face. Epimetaphyseal dysplasia, shortened long bones, and short metacarpals and phalanges are common findings on radiological examination. Additionally, anterior tonguing of the vertebral bodies in the lateral view is an important finding in childhood because it is specific to PSACH and normalizes with age. Here, we report five Japanese patients with PSACH, with one recurrent (p.Cys351Tyr) and four novel heterozygous pathogenic COMP variants (p.Asp437Tyr, p.Asp446Gly, p.Asp507Tyr, and p.Asp518Val). These five pathogenic variants were located in the calcium-binding type 3 (T3) repeats. In four of the novel variants, the affected amino acid was aspartic acid, which is abundant in each of the eight T3 repeats. We describe the radiological findings of these five patients. We also retrospectively analyzed the sequential changes in the vertebral body and epimetaphysis of the long bones from the neonatal to infantile periods in a patient with PSACH and congenital heart disease.

DOI： 10.1297/cpe.2023-0035

研修医報告/Resident Report 化学療法中の食事制限をきっかけに摂食障害を来した10歳男児例

明井孝弘, 藤井智香子, 重安良恵, 岡田あゆみ, 椙原彰子, 堀内真希子, 塚原宏一

子どもの心とからだ 31 ( 3 ) 407 - 413 2022.11

Language：Japanese Publisher：(一社)日本小児心身医学会

小児の摂食障害では,明らかなやせ願望を呈さない回避・制限性食物摂取症の占める割合が,成人と比較して多いことが特徴である.今回われわれは,化学療法中に厳格な食事制限を行ったことを契機に摂食障害を来した男児例を経験した.急性白血病に対する化学療法の副作用のために著明な高脂血症が出現し,脂質制限食を開始したところ食事を摂らなくなった.ボディイメージの障害ややせ願望は認めなかった.厳しい食事制限が行われたことが大きな心理的ストレスとなり,食事は嫌なものという反応が形成され,条件付けが起こったと考え,回避・制限性食物摂取症と診断した.親子が達成できる緩やかな枠組みの食事療法に変更し,改善した.親子が主体的に食事療法に関われるよう介入したことなどが健康な食欲や食事療法に取り組む意欲を生んだと考えた.化学療法中には,現病や治療に伴う食欲低下だけでなく,心理面に留意した対応が重要であると考えた.(著者抄録)

研修医報告/Resident Report チームアプローチによって疼痛が改善した消化管移植片対宿主病の1例

藤本耕慈, 藤井智香子, 岡田あゆみ, 重安良恵, 椙原彰子, 堀内真希子, 塚原宏一

子どもの心とからだ 31 ( 3 ) 414 - 419 2022.11

Language：Japanese Publisher：(一社)日本小児心身医学会

症例は14歳男性で,骨髄異形成症候群に対し造血幹細胞移植を受けた.その後,腸管の移植片対宿主病による腹痛が持続し,各種鎮痛薬を用いても改善が乏しいため心因の関与が疑われ,小児心身医療科に紹介された.移植片対宿主病による腸管狭窄から腹痛が生じていると考えられ,狭窄部切除術を実施されたが,さらに痛みの訴えが増強した.痛みや合併症の再発に対する不安,スタッフに十分に傾聴されていないという不満などの心因の関与により症状が遷延したと考えられたため,支持的カウンセリングに加えて多職種カンファレンスを行い,痛みの訴えへの対応方法を統一した.また,リハビリや食事の際に『できたこと』を肯定し行動を活性化させることにより,腹痛の訴えは軽減した.慢性の痛みがあると,痛みだけでなく痛みにまつわる気分や考えにより行動が左右され痛みの悪循環が発生することがある.この痛みの悪循環を断つためには,基礎疾患に対する身体的治療だけでなく,心理的サポートを行う必要がある.慢性疼痛には,多職種が協力し,心理的アセスメントとそれに基づいた一貫した対応によってチームアプローチを行うことが有効である.(著者抄録)

Neonatal hemochromatosis with εγδβ-thalassemia: a case report and analysis of serum iron regulators. Reviewed International journal

Mitsuru Tsuge, Aya Kodera, Hiromi Sumitomo, Tooru Araki, Ryuichi Yoshida, Kazuya Yasui, Hiroki Sato, Yosuke Washio, Kana Washio, Kenji Shigehara, Masato Yashiro, Takahito Yagi, Hirokazu Tsukahara

BMC pediatrics 22 ( 1 ) 622 - 622 2022.10

Authorship：Last author Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Neonatal hemochromatosis causes acute liver failure during the neonatal period, mostly due to gestational alloimmune liver disease (GALD). Thalassemia causes hemolytic anemia and ineffective erythropoiesis due to mutations in the globin gene. Although neonatal hemochromatosis and thalassemia have completely different causes, the coexistence of these diseases can synergistically exacerbate iron overload. We report that a newborn with εγδβ-thalassemia developed neonatal hemochromatosis, which did not respond to iron chelators and rapidly worsened, requiring living-donor liver transplantation. CASE PRESENTATION: A 1-day-old Japanese boy with hemolytic anemia and targeted red blood cells was diagnosed with εγδβ-thalassemia by genetic testing, and required frequent red blood cell transfusions. At 2 months after birth, exacerbation of jaundice, grayish-white stool, and high serum ferritin levels were observed, and liver biopsy showed iron deposition in hepatocytes and Kupffer cells. Magnetic resonance imaging scans showed findings suggestive of iron deposits in the liver, spleen, pancreas, and bone marrow. The total amount of red blood cell transfusions administered did not meet the criteria for post-transfusion iron overload. Administration of an iron-chelating agent was initiated, but iron overload rapidly progressed to liver failure without improvement in jaundice and liver damage. He underwent living-donor liver transplantation from his mother, after which iron overload disappeared, and no recurrence of iron overload was observed. Immunohistochemical staining for C5b-9 in the liver was positive. Serum hepcidin levels were low and serum growth differentiation factor-15 levels were high prior to living-donor liver transplantation. CONCLUSIONS: We reported that an infant with εγδβ-thalassemia developed NH due to GALD, and that coexistence of ineffective erythropoiesis in addition to erythrocyte transfusions may have exacerbated iron overload. Low serum hepcidin levels, in this case, might have been caused by decreased hepcidin production arising from fetal liver damage due to neonatal hemochromatosis and increased hepcidin-inhibiting hematopoietic mediators due to the ineffective hematopoiesis observed in thalassemia.

DOI： 10.1186/s12887-022-03706-3

新生児ヘモクロマトーシスを発症したεγδβ-サラセミアの1例

津下充, 塚原宏一

日本小児栄養消化器肝臓学会雑誌 36 ( Suppl. ) 94 - 94 2022.9

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