目的 汇总分析肝硬化患者消化道出血风险预测模型，为今后模型的建立和优化提供参考。方法   系统检索中国知网、维普、PubMed数据库在2025年4月22日前公开发表的所有肝硬化患者消化道出血风险预测模型，按纳入标准筛选文献，对最终纳入文章分析摘录并系统汇总，包括模型特征、危险因素及模型预测评估效果等信息。结果   共检索3 603篇预测模型相关研究论文，最终纳入30篇，其中中国27篇、韩国1篇、印度1篇、埃及1篇。22项研究收集了肝硬化病因，其中病毒性肝病最多（72.94%，2 922/4 006），药物性肝病及非酒精性脂肪性肝病最少（均为0.02%，1/4 006）。在研究类型上，有28篇单中心研究，2篇为多中心研究，其中有12个模型未进行验证，只有1个模型进行了外部验证，其余模型只进行了内部验证，曲线下面积（AUC）范围0.680~0.994。根据模型纳入因素特点，分为血常规指标、凝血指标、生化指标、影像学指标、复合指标、其他指标共6种，其中纳入因素最多为影像学指标，最少为凝血指标。在纳入危险因素中，第1位为门静脉直径，第2位为血小板计数，第3位为血红蛋白水平及脾脏硬度，所有因素中与脾脏相关的指标最多。结论   肝硬化患者消化道出血风险预测模型研究质量有待提升，影像学指标应用最广，脾脏相关指标重要性突出，门静脉直径、血小板计数、血红蛋白水平及脾脏硬度为最常用的危险预测因素。

       Objective  To  summarize and analyze the  prediction models for gastrointestinal  bleeding  risk in  patients with cirrhosis，providing references for the establishment and optimization of future models．Methods  A systematic search was conducted in CNKI，VIP，and PubMed for all published prediction models for gastrointestinal bleeding risk in patients with cirrhosis before April 22，2025．Articles were screened according to the inclusion criteria，and the finally included articles were analyzed and summarized，including model characteristics，risk factors，and model prediction evaluation effects．Results  A total of 3 603 related research papers on prediction models were initially retrieved，and 30 were finally included，with 27 from China，one from South Korea，one from India，and one from Egypt．Among the 22 studies that collected the etiology of cirrhosis，viral hepatitis was the most common（72.94%，2 922/4 006），while drug-induced liver disease and non-alcoholic fatty liver disease were the least common（0.02%，1/4 006）．In terms of study type，28 were single-center studies and two were multicenter studies．Among them，12 models were not validated，only one model was externally validated，and the rest were only internally validated，with an area under the curve range of 0.680-0.994．According to the characteristics of the factors included in the models，they were divided into six types of indicators：blood routine，coagulation，biochemistry，imaging，composite，and others，among which imaging indicators were the most common and coagulation indicators were the least．In the included risk factors，the first was portal vein diameter，the second was platelets count，and the third was hemoglobin level and spleen stiffness，with the most factors related to the spleen．Conclusions  The quality of studies on prediction models for gastrointestinal bleeding risk in cirrhosis patients needs to be improved．Imaging indicators are the most widely used，and spleen-related indicators are of prominent importance，with portal vein diameter，platelets count，hemoglobin level，and spleen stiffness being the most commonly used risk prediction factors．

       纤毛是细胞表面的重要细胞器，广泛参与细胞运动、感知外界信号和维持器官功能等生理过程。纤毛的形成，即纤毛发生（ciliogenesis）是一个高度复杂且受精密调控的过程，涉及大量与纤毛结构和功能相关基因的表达与调控。近年来，随着基因组学和发育生物学的发展，越来越多的研究揭示了多种关键转录因子在纤毛发生中的调控作用，包括RFX家族、FOXJ1、MCIDAS、GEMC1、MYB、E2F等。这些转录因子共同构成了一个多层次、多通路交织的调控网络，调控纤毛组装、基体复制、纤毛定位和功能维持等多个方面。本文系统综述了纤毛相关基因转录调控的研究进展，特别是关键转录因子的功能、相互作用及其在纤毛病中的作用，为深入理解纤毛的发育机制和疾病治疗提供参考。

       Cilia are crucial cell-surface organelles involved in cell movement，signal sensing，and organ function maintenance．Their formation，or ciliogenesis，is a complex and  precisely controlled  process that  requires the expression and regulation of numerous cilia-related genes．Recent advances in genomics and developmental biology have uncovered the  regulatory roles of key transcription factors like the RFX family，FOXJ1，MCIDAS，GEMC1，MYB，and E2F in ciliogenesis．These factors form a multi-level，interconnected regulatory network that oversees cilium assembly，basal body replication，ciliary positioning，and function preservation．This review systematically examines current research on transcriptional regulation of ciliary genes，with a focus on the roles，interactions，and contributions of these key transcription factors to ciliopathies，offering insights into ciliary development and disease treatment．

       为规范广州地区“港澳药械通”伦理审查工作，满足临床用药用械需求，保障患者用药用械安全，广州市医学会医学伦理分会、广州市医院协会“港澳药械通”工作专业委员会组织行业专家，结合相关政策法规要求与本地实践经验，广泛征求专家意见，经多次讨论，形成《广州地区“港澳药械通”伦理审查共识》。共识涵盖适用范围、术语和定义、伦理审查的原则、伦理审查的类别、伦理审查的流程、伦理审查的方式及多医疗机构申请的伦理审查等方面。共识旨在为广州地区“港澳药械通”指定医疗机构的伦理审查工作提供指导，统一审查标准，提高审查效率与质量，切实保护患者的合法权益，促进临床急需进口港澳药械的安全合理使用，从而促进粤港澳大湾区医疗健康事业发展。

       To standardize the ethical review process of the “Hong Kong and Macao Drugs and Medical Devices Access” in Guangzhou，meet the clinical demand for drugs and medical devices，and ensure the safety of patients’ use of drugs and medical devices，the Medical Ethics Branch of Guangzhou Medical Association and the “Hong Kong and Macao Drugs and Medical Devices Access” Professional Committee of Guangzhou Hospital Association organized industry experts，combined with  relevant policy and regulatory requirements and local practical experience，widely solicited expert opinions，the “Consensus on Ethical Review of Drugs and Medical Devices for Hong Kong and Macao in Guangzhou” were formed after several discussions．The consensus content covers aspects such as the scope of application，terms and definitions，principles of ethical review，categories of ethical review，procedures of ethical review，methods of ethical review，and Ethical Review for Multi-institutional Applications．The consensus aims to provide guidance for the ethical review work of designated medical institutions for the “Hong Kong and Macao Drugs and Medical Devices Access” in the Guangzhou area，unify the review standards，improve the efficiency and quality of the review，effectively protect the legitimate rights and interests of patients，promote the safe and rational use of clinically urgently imported medicines and medical devices Hong Kong Macao，and thereby promote the development of medical and health care in the Guangdong-Hong Kong-Macao Greater Bay Area．

目的 通过学习1例少见的组织学特征与IgG4相关性淋巴结病类似的浆细胞型特发性多中心型Castleman病（PC-iMCD）,总结两种易误诊疾病的鉴别要点,提高病理诊断水平。方法 回顾性分析1例PC-iMCD患者临床资料,常规苏木素-伊红（HE）染色分析淋巴结组织结构及细胞形态,免疫组织化学染色及原位杂交分析免疫表型及EB病毒（EBV）感染状态,并结合文献分析讨论其与IgG4相关淋巴结病的鉴别诊断。结果 48岁女性患者,临床表现为口干、多饮、皮肤瘙痒伴全身多处淋巴结肿大。实验室检查血清IgG、IgA、IgM及IgE水平均升高,血清IgG4显著升高（14.7 g/L）,白介素- 6（IL-6）异常升高（150.84 pg/mL）。病理检查显示淋巴结生发中心萎缩,套区淋巴细胞呈“洋葱皮”样围绕生发中心排列,滤泡间区扩张,其内见大量成熟的浆细胞呈片状浸润,灶区见含铁血黄素沉积及血管增生;免疫组化染色显示IgG4阳性浆细胞数大于100/高倍视野,IgG4阳性细胞/IgG阳性细胞比值>40%,Kappa及Lambda轻链呈非限制性表达;EB病毒编码RNA原位杂交（EBER）阴性。结论 部分PC-iMCD与IgG4相关淋巴结病具有相似的组织病理学特征,单纯根据组织学及免疫表型难以将两者鉴别,正确诊断需结合IgG4相关病变诊断标准、排除性诊断标准、临床表现及实验室检查综合判断。

Objective By studying a rare case of the plasma cell type idiopathic multicentric Castleman disease（PC-iMCD）with histological characteristics similar to IgG4-related lymphadenopathy,the differential points of the two easily misdiagnosed diseases were summarized to improve the level of pathological diagnosis．Methods The clinical data of one patient with PC-iMCD were collected,the structure alteration and cell morphology were observed by hematoxylin-eosin（HE）stains．The immunophenotype of cells was marked by immunohistochemical staining and the infections status of EB virus was detected by in situ hybridization．Besides,the differential diagnosis between IgG4-RD and PC-iMCD were analyzed and discussed based on literature analysis．Results This article reported a 48-year-old female who was admitted to the hospital with dry mouth,polydipsia,skin itching and multiple lymphadenopathy．The levels of various classes of serum immunoglobulin were all increased,such as IgG,IgA,IgM and IgE．Specially,the serum IgG4 was also significantly increased（14.7 g/L）and interleukin 6（IL-6）was abnormally raised（150.84 pg/mL）．The pathological examination indicated that the lymph node germinal center was atrophied and mantle zones were expanded which were composed of concentric rings of lymphocytes in an “onion skinning” appearance．Besides,the interfollicular area was expanded in which mature plasma cells were infiltrated in sheet-like,hemosiderin was deposited as well as the vessels were proliferated．Immunohistochemistry showed that the number of IgG4-positive plasma cells was >100/HPF,IgG4/IgG-positive cells ratio was >40%,and Kappa and Lambda light chains were expressed unrestrictedly．In situ hybridization revealed that the expression of EBER was negative．Conclusions PC-iMCD and IgG4-related lymphadenopathy shares similar histopathological characteristics and it’s challenging to distinguish these two diseases by their histology and immunophenotype．In conclusion,the correct diagnosis should be combined with the diagnostic criteria,exclusion diagnostic criteria,clinical manifestations and related laboratory examinations．

目的 分析我院羊水栓塞患者的临床资料及诊治经过,总结羊水栓塞致弥散性血管内出血（DIC）患者输血治疗救治经验。方法 回顾性分析2016年1月1日—2022年12月31日在广州医科大学附属第二医院番禺院区分娩并发羊水栓塞患者的症状、实验室辅助检查、用药诊治经过及妊娠结局。结果 在6 104次孕晚期分娩记录中,共发生3例羊水栓塞,高龄产妇1例、经产妇2例、多产1例。3例均并发DIC,产后出血1 500~3 000 mL。其中1例行子宫切除术,2例保留子宫,均抢救成功,无患者死亡。结论 根据有限数据推论羊水栓塞罕见、凶险,病情进展快,多继发产后出血、DIC,主要依靠临床表现进行诊断,早期识别诊断和处理,产科快速反应团队及多学科协作,给予高级生命支持,有效止血并科学地成分输血纠正凝血功能障碍及DIC,是改善孕产妇结局的关键。

Objective To analyze the clinical data,diagnosis and treatment of patients with amniotic fluid embolism in our hospital,and to summarize the experience of blood transfusion treatment for disseminated intravascular coagulation（DIC） patients with amniotic fluid embolism. Methods From January 1,2016 to December 31,2022,the symptoms,laboratory tests,drug treatments and pregnancy outcomes of patients with amniotic fluid embolism during delivery in Panyu Hospital,the Second Affiliated Hospital of Guangzhou Medical University were retrospectively analyzed. Results There were 3 cases of amniotic fluid embolism in 6 104 late pregnancy records,accounting for 1/3 of the elderly parturients,2/3 of the multiparas and 1/3 of the prolific parturients．All three cases had DIC and postpartum hemorrhage of 1 500-3 000 mL．Among them,1 case underwent hysterectomy and 2 cases saved the uterus,all of which were successfully rescued without death. Conclusions Amniotic fluid embolism is rare,dangerous disease with rapid progression,often followed by secondary postpartum hemorrhage and DIC,and mainly relies on clinical manifestations for diagnosis．Early identification,diagnosis and treatment,rapid response team in obstetrics and multidisciplinary collaboration,advanced life support,effective hemostasis and scientific component blood transfusion to correct coagulation dysfunction and DIC,are the keys to improve maternal outcomes．

目的 探讨特发性间质性肺炎（IIPs）[非特发性肺间质性纤维化（IPF）型]合并弥漫性肺部出血综合征患者治疗方案以及应用价值。方法 报道1例IIPs（非IPF型）合并弥漫性肺部出血综合征患者的治疗经过以及结果,结合文献分析治疗IIPs（非IPF型）合并弥漫性肺部出血综合症临床应用价值。结果 该文报道 l 例特发性肺间质肺炎（非IPF型）伴弥漫性肺泡出血综合征的老年男性患者,合并呼吸、循环衰竭,启用静脉-静脉体外膜肺氧合（VV-ECMO）抢救并成功撤机,病情好转出院。结论 IIPs作为病因以及发病机制未明、临床表现多样的一类肺间质性疾病,需临床多学科协作,及早诊断、治疗,才能成功挽救患者。

Objective To explore the treatment plan and application value of idiopathic interstitial pneumonia（non IPF）complicated with diffuse alveolar hemorrhage syndrome．Methods A case of idiopathic interstitial pneumonia（non IPF）complicated with diffuse alveolar hemorrhage syndrome was reported．The clinical application value of treatment of idiopathic interstitial pneumonia（non IPF）complicated with diffuse alveolar hemorrhage syndrome was analyzed combined with the literature．Results A case of idiopathic interstitial pneumonia（non IPF type）with diffuse alveolar hemorrhage syndrome was reported in this paper． Combined with respiratory and circulatory failure,veno-venous extracorporeal membrane oxygenation was used to rescue and successfully wean,and the condition improved and discharged．Conclusions Through the curative effect evaluation of this patient,it is believed that idiopathic interstitial pneumonia,as a kind of pulmonary interstitial disease with unknown etiology and pathogenesis and diverse clinical manifestations,need clinical multidisciplinary cooperation,early diagnosis and treatment,in order to successfully save the patient．

缺牙区水平骨量不足一直是口腔种植的重要难题。骨劈开术是常用于解决此难题的手术方式之一,目前临床常用术式是采用超声骨刀沿牙槽嵴顶矢状劈开后,用骨凿将唇(颊)侧骨板向唇(颊)侧移位后增加牙槽骨水平宽度,达到同期种植的目的。骨劈开并同期植入种植体降低了困难病例的手术难度和风险,减少了患者就诊次数,降低患者成本。但若劈开后种植体周围的间隙大于1 mm或唇侧骨板分离,则需要同期进行植骨。但骨劈开术的使用也具有一定的适应症,要求缺牙区垂直骨高度足够,水平骨宽度不足(3～5 mm),骨质为Ⅲ ～ Ⅳ类骨,患者无种植手术的局部和全身禁忌证。近年来的研究表明骨劈开术具有创伤小、手术安全有效、不用开辟第二创口即可获得较好的骨增量效果等优点,一定程度上扩大了种植手术适应证。现本文对骨劈开术的翻瓣术式、非翻瓣术式及术后效果的进展进行综述。

The insufficient horizontal bone mass in the missing tooth area has always been an important problem of dental implant. Bone splitting is one of the surgical methods commonly used to solve this problem. At present, the commonly used surgical method is to use ultrasonic bone knife to sagittal splitting along the crest of the alveolar ridge, and then use bone chisel to shift the lateral bone plate of the lip (buccal) to the lip (buccal) to increase the horizontal width of the alveolar bone, so as to achieve the purpose of simultaneous implantation. Bone splitting and implantation of implants in the same period reduced the surgical difficulty and risk of difficult cases, reduced the number of patient visits, and reduced the cost of patients. However, if the gap around the implant was more than 1 mm or the labial bone plate was separated after splitting, bone grafting should be performed simultaneously. But the use of bone splitting technique also has a certain indications, demanding sufficient vertical bone height in the missing area, inadequate width of horizontal bone (3 mm to 5 mm), Ⅲ~Ⅳ class bone, without local and systemic contraindicated of planting surgery. In recent years, the research shows that bone splitting surgery has the advantages of small trauma, safe and effective operation, and better bone increment effect obtained without opening the second wound. This article reviewed the progress of flap operation, flapless operation and postoperative effect of bone splitting.

目的 观察达雷妥尤单抗联合来那度胺及地塞米松(DRd)方案巩固治疗序贯达雷妥尤单抗和来那度胺两药维持治疗1例高龄高危初治多发性骨髓瘤患者的疗效、生存时间和不良反应。方法 回顾分析广州市第一人民医院老年病科血液肿瘤科2019年3月收治的1例高龄高危初治多发性骨髓瘤患者的临床资料,并复习相关最新文献。结果 患者应用伊沙佐米、来那度胺和地塞米松方案诱导治疗13疗程后只达到部分缓解的疗效,未能进一步缓解,且不良反应多且严重,后改为DRd方案巩固治疗2疗程后,达到完全缓解,继续使用达雷妥尤单抗联合来那度胺两药维持治疗,不良反应少,至随访结束总生存期和无进展生存期均为35个月。结论 含达雷妥尤单抗方案巩固和维持治疗可能会改善高龄高危初治多发性骨髓瘤患者的预后,延长生存时间,耐受性好。

Objective To observe the efficacy, survival time and adverse reactions of daratumumab combined with lenalidomide and dexamethasone (DRd) in the consolidation treatment of sequential daratumumab and lenalidomide maintenance treatment of an elderly patient with high-risk newly diagnosed multiple myeloma. Methods The clinical data of the elderly patient with newly diagnosed multiple myeloma treated in the Department of Geriatrics, Hematology & Oncology Ward, Guangzhou First People's Hospital in March 2019 were retrospectively analyzed, and the relevant latest literatures were reviewed. Results After 13 courses of induction treatment with isazomib, lenalidomide and dexamethasone, it only achieved partial remission, but failed to further remission, and there were many serious adverse reactions.Later, it was changed to DRd therapy to consolidate treatment.After 2 courses of treatment, it achieved complete remission.After that, we continued to use daratumumab combined with lenalidomide for maintenance treatment, with few adverse reactions.At the time of submission, the overall survival and progression free survival were 35 months. Conclusions Consolidation and maintenance therapy with daratumumab may improve the prognosis, prolong survival time and with good tolerance in elderly patients with high-risk newly diagnosed multiple myeloma.

目的 为临床合理使用替考拉宁以及更好地管理接受替考拉宁治疗的患者。方法 从药学角度对2022年日本《2022 JSC/JSTDM临床实践指南:替考拉宁治疗药物监测》(简称《指南》)涉及替考拉宁治疗的9个临床问题进行解读。结果 《指南》指出药-时曲线下面积/最小抑菌浓度是替考拉宁的关键药动学/药效学参数。替考拉宁治疗药物监测(TDM)的目的是明确目标谷浓度(C_min),对于严重或复杂的耐甲氧西林金黄色葡萄球菌(MRSA)感染,指南建议替考拉宁C_min为20~40 mg/L。肾功能正常或轻度受损的非复杂性的MRSA感染,目标C_min为15~30 mg/L。严重和/或复杂性MRSA感染,如感染性心内膜炎和骨髓炎,替考拉宁C_min为20~40 mg/L。结论 《指南》针对不同病理状态下患者替考拉宁目标C_min的确定,为临床治疗中替考拉宁TDM、个体化给药提供参考。

Objective To make rational use of teicoplanin and better management of patients treated with teicoplanin. Methods Nine clinical issues related to the treatment of teicoplanin in Clinical practice guidelines for therapeutic drug monitoring of teicoplanin: a consensus review by the Japanese Society of Chemotherapy and the Japanese Society of Therapeutic Drug Monitoring (Japan, 2022) were interpreted from the perspective of pharmacy. Results The guidelines indicated that the area under drug-time curve/minimum inhibitory concentration was the key pharmacokinetic/pharmacodynamic parameters of teicoplanin.The purpose of therapeutic drug monitoring (TDM) of teicoplanin is to specify the target trough concentration (C_min), which guidelines recommend for severe or complex methicillin-resistant Staphylococcus aureus (MRSA) infection is 20-40 mg/L.The target C_min for uncomplicated MRSA infection with normal or mildly impaired renal function is 15-30 mg/L.For severe and/or complex MRSA infections, such as infective endocarditis and osteomyelitis, the C_min of teicoplanin was 20-40 mg/L. Conclusions The guidelines are aimed at the determination of target C_min of teicoplanin in patients with different pathological conditions, and provide reference for individual drug administration and teicoplanin TDM in clinical treatment.

目的 学习母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床病理及免疫表型特征,总结该少见肿瘤的病理诊断经验。方法 回顾分析2例BPDCN患者临床资料,通过苏木素-伊红(HE)染色分析肿瘤组织及细胞形态,通过免疫组织化学染色分析肿瘤免疫表型,通过流式细胞学检测骨髓有无肿瘤侵犯,并结合文献分析。结果 本报道中1例为97岁女性,临床以皮肤瘀斑结节为首发症状,肿瘤细胞真皮内弥漫浸润,不侵犯表皮,细胞中等大小,核形不规则,核仁不明显。另1例为69岁男性,临床以淋巴结肿大为首发症状,淋巴结结构完全破坏,肿瘤细胞弥漫浸润,细胞呈中等大小的母细胞样,核仁明显。2例免疫表型均表达CD123、CD4、CD56、TDT,不表达B系、T系淋巴细胞及髓系标志物,肿瘤均累及骨髓。结论 BPDCN是一种罕见的淋巴造血肿瘤,临床常以皮肤病变或淋巴结肿大为首发症状,临床过程具高度侵袭性,通常伴有骨髓侵犯。该肿瘤需与具有母细胞形态的淋巴系肿瘤和白血病相鉴别,诊断需结合临床信息、HE形态及免疫组化结果综合判断。

Objective To summarize the diagnostic experiences of blastic plasmacytoid dendritic cell neoplasm (BPDCN) based on the study of its clinicopathological features and immunophenotypes. Methods The clinical data of 2 patients with BPDCN were collected, the structure alteration and cell morphology were observed by HE staining, the immunophenotype of tumor cells were studied by immunohistochemistry staining and flow cytometry was adopted to confirm the bone marrow involvement. Results Two patients, one of whom was a 97 year-old female, presented with cutaneous ecchymosis nodules as the first symptom. The epidermis, but not the dermal, was diffusedly infiltrated by tumor cells, which were medium-sized with irregular nuclei without prominent nucleoli. The other case was a 69 year-old male with lymph node enlargement as the first symptom. The skin was normal, but the lymph nodes were invasively destroyed by tumor cells, which were medium-sized blast-like with prominent nucleoli. The immunophenotypes of the two patients were both positive for CD123, CD4, CD56 and TDT, but negative for B, T lymphocyte derived and myeloid origin markers, both of which involved bone marrow. Conclusions BPDCN is a rare form of hematological neoplasm, skin symptoms or lymph node enlargement may be presented as the initial symptom, the clinical course were highly aggressive with high frequency of bone marrow involvement. The blastic-like lymphoma and leukemia entities should be considered into account for differential diagnose. The precise diagnosis of BPDCN should be established by integrating histomorphology, immunophenotype and clinical presentation information comprehensively.