Treatment of generalized infantile myofibromatosis with sorafenib and imatinib: A case report

Infantile myofibromatosis (IM) is characterized by solitary musculoskeletal nodules presenting during infancy but can manifest as multiple lesions with visceral involvement. Multicentric IM with visceral involvement carries a high risk of mortality and there is no consensus on treatment. We present a case of a patient with multicentric IM and pulmonary involvement who progressed on several chemotherapeutic regimens and subsequently had a complete response to sorafenib and later imatinib. This report describes the novel use of sorafenib and imatinib to treat generalized IM and the role of continued tyrosine kinase inhibitor therapy to maintain remission.     

微弧氧化技术与钛合金表面成骨细胞增殖及骨向分化能力

文题释义：微弧氧化技术：是金属材料表面处理的一种技术，将金属材料(如，镁、铝、钛等)置于特定电解液中，通过弧光放电过程中瞬时的高温和高压条件产生结合于金属表面的陶瓷膜层，该种方法制备的涂层材料具有高硬度、耐磨的特点。  种植体：是针对于人体自身牙齿缺失而植入上下颌骨内的人工牙根。性能优良的种植体需要同时具备高强度、耐降解、生物相容性好的特性。目前复合材料类如金属表面添加陶瓷涂层材料，具有上述多种材料特性而被临床选择。 背景：采用普通电化学法可在钛及其合金表面制备纳米级羟基磷灰石涂层，但该涂层吸收降解缓慢，需要8-12周的时间。而微弧氧化可在复杂表面形成均匀薄膜，有利于细胞黏附和骨组织长入。 目的：探索微弧氧化羟基磷灰石涂层钛合金对成骨细胞增殖及骨向分化能力的影响。 方法：采用电化学法与微弧氧化法分别制备羟基磷灰石涂层钛合金材料，检测两种材料表面的接触角。将成骨细胞系hFOB1.19接种于两组羟基磷灰石涂层钛合金材料表面，培养48 h时，采用扫描电镜观察细胞在材料上的形态变化；培养1，12，24，48，72 h时，采用MTT法检测细胞增殖；培养1，3，5 d时，比较两组材料表面细胞计数与碱性磷酸活性；培养第5天，采用Western Blotting检测细胞内骨形态发生蛋白2和骨形态发生蛋白4表达。 结果与结论：①微弧氧化组材料表面的接触角小于电化学组[(66.5±2.2)°，(52.8±2.1)°，P=0.001 5)]；②扫描电镜显示，电化学组成骨细胞形态不规则，胞体皱缩不饱满，与材料贴合较差；微弧氧化组细胞充分伸展，形态扁平，与材料贴合紧密；③微弧氧化组12-72 h的细胞增殖快于电化学组(P < 0.05)，培养3，5 d的细胞计数多于电化学组(P < 0.05)；④微弧氧化组培养1，3，5 d的细胞碱性磷酸酶活性高于电化学组(P < 0.05)；⑤微弧氧化组骨形态发生蛋白2和骨形态发生蛋白4表达均高于电化学组(P < 0.05)；⑥结果表明，微弧氧化羟基磷灰石涂层钛合金材料可促进成骨细胞的增殖及骨向分化能力。 ORCID: 0000-0003-2787-4667(王艳玲) 中国组织工程研究杂志出版内容重点：生物材料；骨生物材料; 口腔生物材料; 纳米材料; 缓释材料; 材料相容性；组织工程     

针刀松解联合丹参注射液关节腔注射治疗骨性关节炎模型兔

文题释义： 针刀疗法：用一个完整的语言来概括应该是一种介于手术方法和非手术疗法之间的闭合性松解术。针刀疗法是将东方中医学的基本理论和西方医学的手术解剖基本理论融为一体，针刀疗法其操作的特点是在治疗部位刺入深部到病变处进行轻松的切割、剥离等不同的刺激，以达到止痛祛病的目的。 丹参注射液：其化学成分主要是水溶性酚酸类化合物，通过多种靶标起到促进血液循环和祛瘀的作用。动物研究表明，丹参注射液可通过抑制IκBα磷酸化来抑制NF-κB信号通路的激活。保护软骨下骨及软骨细胞，有效防治骨关节炎。 背景：丹参注射液与针刀松解术都是临床中治疗骨关节炎很常用的技术，目前缺乏对2种治疗方法联合使用的研究。 目的：对比单独使用针刀松解术、单独使用丹参注射液与两种方法联合使用治疗兔模型骨关节炎的效果。 方法：实验方案经黑龙江中医药大学动物实验伦理委员会批准。50只新西兰白兔分为5组：空白对照组；模型组；丹参注射液组；针刀组；针刀联合丹参注射液组。除空白对照组外，其他4组兔均制备膝关节骨关节炎模型。造模成功后，对针刀组兔内外侧支持带、内外侧膝眼，膝关节上方髌上囊、股四头肌各肌肌腹，膝关节内侧股薄肌肌腹及止点、收肌肌群肌腹及止点和胫骨平台附近部位进行针刀松解；空白对照组及模型组兔关节腔注射生理盐水0.3 mL；丹参注射液组兔关节腔每次注射0.3 mL丹参注射液；针刀联合丹参注射液组：每周进行1次针刀治疗同针刀组，每次针刀干预之后，关节腔注0.3 mL丹参注射液。上述干预每周1次，持续5周。对各组兔膝关节软骨进行大体观察及病理切片组织学观察，ELISA 检测关节液白细胞介素1、肿瘤坏死因子α水平。 结果与结论：①大体观察：模型组膝关节磨损较为严重，针刀组、丹参注射液组、针刀联合丹参注射液组膝关节轻度磨损；②病理切片评分：丹参注射液组评分略高于针刀组和针刀联合丹参注射液组差异无显著性意义；③ELISA 检测结果：针刀联合丹参注射液组白细胞介素1、肿瘤坏死因子α水平显著低于或针刀组或丹参注射液组(P < 0.05)；④结果说明，在兔膝关节骨关节炎模型中降低白细胞介素1、肿瘤坏死因子α水平方面，针刀松解术结合丹参注射液关节腔注射治优于单独使用针刀或单独使用丹参注射液关节腔注射。ORCID: 0000-0003-4119-633X(刘广宇) 中国组织工程研究杂志出版内容重点：组织构建；骨细胞；软骨细胞；细胞培养；成纤维细胞；血管内皮细胞；骨质疏松；组织工程     

Hypothesis of design of biological cell robot as human immunodeficiency virus vaccine

High genetic variability of human immunodeficiency virus (HIV) has been a major intractable challenge to the practical design of vaccines. But a recent pioneer study published in PNAS Xenobots, is likely to revolutionize HIV prevention as it presented the world's first living robot made of cells. In the advent of this discovery, we herein discuss the possibility of using living biological cell robots to target HIV-infected T lymphocytes, and the prospects of this approach being a new HIV vaccine. We capture the current research status and trend of advances in biological cell robots' design as a new HIV vaccine. The key differences between this novel vaccine and other HIV vaccines are highlighted.     

Clinical features of 22q11.2 deletion syndrome related to hearing and communication

Abstract

Background: Individuals with 22q11.2 deletion syndrome (22q11.2DS) exhibit various phenotypes.

Objective: To compare the clinical and otorhinolaryngological features of Japanese patients with 22q11.2DS with those of patients reported in Western literature.

Materials and methods: We retrospectively assessed the medical records of 17 Japanese patients with 22q11.2DS and compared our findings with previously reported findings in Western literature.

Results: Hearing loss was the most frequent complaint (n?=?8, 47%), followed by articulation disorders and/or nasopharyngeal closure failure (n?=?4, 24%) and language development delay (n?=?2, 12%). Ten patients (59%) had hearing loss regardless of the chief complaint (total 15 ears – mild, 9; moderate, 6). Four patients had bilateral hearing loss. One patient (6%) underwent tympanostomy tube placement for refractory exudative otitis media, another (6%) underwent myringoplasty, and three patients (18%) underwent tympanoplasties for chronic otitis media or middle ear malformation. Previous studies in Western countries reported similar results in terms of frequency of hearing loss, severity of hearing loss, and the percentage of middle ear malformations.

Conclusions: The otorhinolaryngological characteristics of Japanese patients with 22q11.2DS were similar to those in Western countries. Hearing loss was primarily caused by disorders like otitis media and middle ear malformation.

Significance: Our findings may aid treatment planning for Asian patients with 22q11.2DS.