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[MACULOPAPULAR CUTANEOUS MASTOCYTOSIS HARBORING A KIT MUTATION (ASP419DEL): A CASE REPORT]. [携带 kit 突变(asp419del)的斑丘疹性皮肤肥大细胞增多症:病例报告]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.189
Akiko Fukaura, Takafumi Numata, Masako Chiyotanda, Tatsuo Maeda, Chizu Egusa, Tomonobu Ito, Kazutoshi Harada, Yukari Okubo

A 2-year-old, male patient presented with an 18-month history of scattered, brown macules and nodules up to 2 cm in size on his trunk and extremities. These macules were accompanied by pruritus and were positive for Darier's sign. A skin biopsy of a brown macule on the left thigh revealed a dense accumulation of CD117-positive, round or oval cells with amphophilic cytoplasm within the upper to middle dermis. The patient was otherwise healthy and had normal laboratory and imaging test results. Sequence analysis of genomic DNA from a skin biopsy demonstrated the presence of an Asp419del mutation in exon 8 of the KIT gene. Based on these findings, maculopapular cutaneous mastocytosis (MPCM) was diagnosed. The patient received H 1-antihistamine. Although the pruritus resolved, the brown macules remained for one year after the initial treatment. To the best of our knowledge, only three cases of cutaneous mastocytosis (CM) with an Asp419del mutation, including the present case, have been reported in the Japanese literature to date; moreover, while the previous two cases were of DCM, the present case was the first instance of MPCM. Normally, the symptoms of childhood-onset MPCM are dormant until puberty. However, a recent study reported that many MPCM patients may experience persistent or exacerbated symptoms. The present study therefore evaluated 53 Japanese cases of childhood onset MPCM with a KIT gene mutation and discussed the patients' clinical outcomes.

一名两岁的男性患者因躯干和四肢出现散在的棕色斑丘疹和结节已有 18 个月的病史,结节大小可达 2 厘米。这些斑丘疹伴有瘙痒,达里尔征阳性。对左大腿上的棕色斑丘疹进行皮肤活检后发现,真皮中上层有CD117阳性的圆形或椭圆形细胞密集聚集,细胞质呈嗜两性。患者其他方面健康,实验室和影像学检查结果正常。皮肤活检的基因组DNA序列分析表明,KIT基因第8外显子存在Asp419del突变。根据这些结果,确诊为斑丘疹性皮肤肥大细胞增多症(MPCM)。患者接受了 H 1-抗组胺治疗。虽然瘙痒症状缓解了,但棕色斑丘疹在最初治疗一年后仍然存在。据我们所知,包括本病例在内,迄今为止日本文献中仅报道过三例 Asp419del 突变的皮肤肥大细胞增多症(CM)病例;此外,前两例病例为 DCM,而本病例则是 MPCM 的首例病例。正常情况下,儿童期发病的 MPCM 症状在青春期前一直处于休眠状态。然而,最近的一项研究报告称,许多 MPCM 患者的症状可能会持续或加重。因此,本研究评估了日本 53 例 KIT 基因突变的儿童期发病的多发性骨髓瘤,并讨论了患者的临床预后。
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引用次数: 0
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.424
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引用次数: 0
[DEVELOPMENT AND VALIDATION OF QUALITY OF LIFE QUESTIONNAIRE FOR CHILDREN WITH FOOD ALLERGY REPORTED BY PARENTS]. [开发和验证由家长报告的食物过敏儿童生活质量问卷]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.406
Daisuke Yasunari, Chikako Motomura, Toshiaki Kawano, Akihiro Takase, Masanori Ikeda, Teruaki Matsui, Mika Ogata, Shuichi Suzuki, Junichiro Tezuka, Mizuho Nagao, Natsuko Masumoto, Noriyuki Yanagida, Yukihiro Ohya

Objective: No questionnaire aimed at evaluating the quality of life (QOL) of children with food allergies has been developed in Japan. Therefore, this study was aimed at developing a Japanese version of a QOL questionnaire for children with food allergies to be responded to by their parents.

Methods: A 59-question primary questionnaire was developed for parents of children aged 0 to 15 years who had food allergies. Responses to the primary questionnaire were collected, and question items were reduced using factor analysis. Then, a secondary questionnaire consisting of nine questions was developed. The secondary questionnaire, the Food Allergy Quality of Life Questionnaire - Parent Form (FAQLQ-PF), and the parent-reported questionnaire on health-related quality of life in children and adolescents (KINDL) were administered to parents of children aged 3 to 15 years who had food allergies.

Results: Overall, 342 parents completed all questionnaires. The QOL scores of the secondary questionnaire were significantly correlated to those of the FAQLQ-PF (r=0.765) and weakly correlated to those of the KINDL (r=-0.358). In addition, QOL scores were significantly worse in patients with a history of anaphylaxis, who were prescribed an adrenaline auto-injector, and who were allergic to hen's eggs, cow milk, or wheat.

Conclusion: We were able to develop a parent-reported validated Japanese version of the QOL questionnaire for children with food allergies.

目的:日本尚未开发出旨在评估食物过敏儿童生活质量(QOL)的问卷。因此,本研究旨在为食物过敏儿童编制一份日语版的生活质量问卷,供其父母回答:方法:为 0 至 15 岁食物过敏儿童的父母编制了一份包含 59 个问题的初级问卷。收集了对初级问卷的答复,并通过因子分析减少了问题项目。然后,编制了由九个问题组成的二级问卷。我们向 3 至 15 岁食物过敏儿童的家长发放了二级问卷、食物过敏生活质量问卷--家长表(FAQLQ-PF)和家长报告的儿童和青少年健康相关生活质量问卷(KINDL):共有 342 位家长填写了所有问卷。二次问卷的 QOL 分数与 FAQLQ-PF 的 QOL 分数呈显著相关(r=0.765),与 KINDL 的 QOL 分数呈弱相关(r=-0.358)。此外,有过敏性休克病史、使用肾上腺素自动注射器以及对鸡蛋、牛奶或小麦过敏的患者的 QOL 评分明显较低:结论:我们能够为食物过敏患儿编制一份由家长报告的、经过验证的日语版 QOL 问卷。
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引用次数: 0
[KEY RESIDUES IN DRUG-INDUCED IgE-INDEPENDENT PSEUDO-ALLERGIC REACTIONS VIA MAS-RELATED G-PROTEIN COUPLED RECEPTOR X2]. [药物诱导的不依赖 IgE 的 PSEUDO 过敏反应中的关键反应物通过 MAS-RELATED G-PROTEIN COUPLED RECEPTOR X2 起作用]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.313
Eri Hamamura-Yasuno, Takashi Shimada, Kazunori Fujimoto, Kazuhiko Mori
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引用次数: 0
[BASIC KNOWLEDGE FOR ALLERGIST ON MOLECULAR TARGETED THERAPY]. [过敏症专家分子靶向治疗基本知识]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.323
Makoto Nagata
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引用次数: 0
[INSTRUCTIONS ON ALLERGEN IMMUNOTHERAPY FOCUSING ON ALLERGEN IMMUNOTHERAPY FOR BRONCHIAL ASTHMA]. [过敏原免疫疗法说明,重点是支气管哮喘的过敏原免疫疗法]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.1133
Kazuyuki Nakagome
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引用次数: 0
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.1177
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引用次数: 0
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.357
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引用次数: 0
[RECURRENT AND VIRTUAL EDUCATION FOR ALL DISCIPLINES AND OCCUPATIONS IN THE ALLERGY REALM : A SURVEY OF THE PARTICIPANTS FROM THE INITIATIVE 'OUTREACH LECTURES' TO CREATE EDUCATIONAL OPPORTUNITIES]. [过敏领域所有学科和职业的经常性和虚拟教育:对 "外联讲座 "倡议参与者的调查,以创造教育机会]。
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.329
Katsunori Masaki, Masafumi Sakashita, Yasushi Ogawa, Takenori Inomata, Keigo Kainuma, Keiko Kan-O, Sakura Sato, Mayumi Tamari, Saeko Nakajima, Hideaki Morita, Yosuke Kurashima, Masaki Futamura, Koichiro Takahashi, Junji Haruta, Misa Hyakutake, Toshiaki Monkawa, Tamotsu Ishizuka, Yoshimasa Imoto, Noritaka Oyama, Sho Kanzaki, Masanori Kidoguchi, Atsuki Fukushima, Koichi Fukunaga, Shigeharu Fujieda, Motoko Yasutomi, Takeya Adachi

Background: In the enhancement of allergy care involving multidisciplinary and multiple medical departments, there is a perceived need for education that targets not only specialists but also non-specialists. However, research on the need for and methods of such education remains inadequate.

Objective: To design a remote allergy care education program for all medical practitioners and to validate its necessity and utility.

Methods: The Empowering Next Generation Allergist/immunologist toward Global Excellence Task Force (ENGAGE-TF), supported by the Japanese Society of Allergology, initiated a virtual educational program called 'Outreach Lectures' in collaboration with Keio University and Fukui University. This initiative was widely promoted through social media and various institutions, and a survey was conducted through its mailing list.

Results: 1139 responses were obtained. More than half were physicians from non-allergy specialties, representing a diverse range of healthcare professions. Over 70% expressed being 'very satisfied,' and over 60% found the difficulty level 'appropriate.' Free-form feedback revealed differences in learning focus based on profession and learning approach based on years of experience.

Conclusion: The high participation rate (90%) of non-specialist physicians underscores the demand for addressing allergic conditions in primary care. The effectiveness of virtual / recurrent education, particularly for healthcare professionals with over 11 years of experience, was implied. Further follow-up investigation focusing on quantitative and objective assessment of educational effectiveness is indispensable.

背景:在加强涉及多学科和多医疗部门的过敏护理方面,人们认为不仅需要针对专科医生的教育,还需要针对非专科医生的教育。然而,有关此类教育的需求和方法的研究仍然不足:目的:为所有医疗从业人员设计一项远程过敏护理教育计划,并验证其必要性和实用性:方法:在日本过敏学会的支持下,"增强下一代过敏/免疫医师能力,实现全球卓越 "工作组(ENGAGE-TF)与庆应义塾大学(Keio University)和福井大学(Fukui University)合作,发起了一项名为 "外联讲座 "的虚拟教育计划。该计划通过社交媒体和各种机构进行了广泛宣传,并通过其邮件列表进行了调查:结果:共收到 1139 份回复。超过一半的受访者是非过敏专业的医生,代表了医疗保健行业的不同领域。超过 70% 的人表示 "非常满意",超过 60% 的人认为难度 "适当"。自由反馈显示,不同专业的学习重点和不同年限的学习方法存在差异:非专科医生的高参与率(90%)强调了在初级保健中应对过敏性疾病的需求。虚拟/经常性教育的有效性,尤其是对有 11 年以上经验的医疗保健专业人员的有效性,已得到暗示。必须进一步开展后续调查,重点对教育效果进行量化和客观评估。
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引用次数: 0
Q4 Medicine Pub Date : 2024-01-01 DOI: 10.15036/arerugi.73.426
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引用次数: 0
期刊
Japanese Journal of Allergology
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