Ferroptosis is an iron-dependent programmed cell death associated with severe kidney diseases, linked to decreased glutathione peroxidase 4 (GPX4). However, the spatial distribution of renal GPX4-mediated ferroptosis and the molecular events causing GPX4 reduction during ischemia-reperfusion (I/R) remain largely unknown. Using spatial transcriptomics, we identify that GPX4 is situated at the interface of the inner cortex and outer medulla, a hyperactive ferroptosis site post-I/R injury. We further discover OTU deubiquitinase 5 (OTUD5) as a GPX4-binding protein that confers ferroptosis resistance by stabilizing GPX4. During I/R, ferroptosis is induced by mTORC1-mediated autophagy, causing OTUD5 degradation and subsequent GPX4 decay. Functionally, OTUD5 deletion intensifies renal tubular cell ferroptosis and exacerbates acute kidney injury, while AAV-mediated OTUD5 delivery mitigates ferroptosis and promotes renal function recovery from I/R injury. Overall, this study highlights a new autophagy-dependent ferroptosis module: hypoxia/ischemia-induced OTUD5 autophagy triggers GPX4 degradation, offering a potential therapeutic avenue for I/R-related kidney diseases.

对22例儿童急性痘疮样苔藓样糠疹(pityriasis lichenoides et varioliformis acuta,PLEVA)患儿的临床资料进行回顾分析.结果示22例患儿中,男女比例2.67:1,平均年龄(8.77±2.84)岁.皮损分布:弥漫型18例(81.82％),中心型3例(13.64％),外周型1例(4.55％).16例(72.73％)患儿发病前有上呼吸道或其他感染史.组织病理的共同特征是:基底细胞不同程度的液化变性,真皮浅层或深层血管周围炎.所有患儿均采取联合治疗,包括:系统使用免疫球蛋白、糖皮质激素、复方甘草酸苷、红霉素等并联合外用药物治疗,20例(90.91％)患儿治愈.

目的 分析术前血红蛋白与红细胞分布宽度比(HRR)对Ⅱ、Ⅲ期结直肠癌(CRC)术后患者预后的关系,评估其对预后的预测价值.方法 选取2014年1月—2015年12月苏州大学附属第一医院接受根治性手术的CRC患者149例.应用χ2检验、Kaplan-Meier检验、Log-rank检验、Cox风险回归模型分析患者术前HRR与预后的关系.结果 所有患者中位随访时间为41个月.单因素分析发现,ECOG、体重下降、N分期、TNM分期、癌结节和HRR与无病生存期(DFS)显著相关(P<0.05),年龄、ECOG、体重下降、N分期和HRR与总生存期(OS)显著相关(P<0.05).多因素分析显示,ECOG、体重下降和HRR是CRC术后患者DFS的独立预后危险因素,年龄、ECOG、体重下降是影响OS的重要因素.根据术前HRR水平将所有患者分为高HRR组和低HRR组,其3年DFS分别为56.1％、41.0％(P<0.01),3年OS分别为86.3％、73.4％(P<0.01).结论 术前HRR水平变化直接影响CRC患者的预后,高HRR预后较好.低HRR提示术后复发、转移风险增高,预后不佳.

Objective:To explore the clinical manifestations and treatment strategies of congenital intrahepatic portosystemic venous shunt (CIPSVS) in children with a review of literature.Methods:The clinical and postoperative follow-up data was retrospectively analyzed for a child with type V CIPSVS, namely, patent ductus venosus (PDV) with pulmonary hypertension as the first symptom, and the relevant literature was reviewed and analyzed.Results:The patient had repeated respiratory tract infection and pulmonary hypertension, and underwent venous catheter ligation.No respiratory tract infection occurred in the 6-month postoperative follow-up, with pulmonary hypertension lower than before.Relevant literature reports were analyzed simultaneously and 41 cases were included.There were 4 cases treated with interventional surgery, 17 cases with open surgery, and 3 cases with endoscopic surgery, all seeing clinical symptoms relieved after surgery.But there were 17 cases that did not report the treatment plan.Conclusion:CIPSVS is a clinically rare disease with its clinical manifestations highly diverse.In the cases of liver dysfunction and abnormal metabolism due to insufficient hepatic portal blood perfusion, and abnormal perfusion of cardiopulmonary system, CIPSVS may be the possible cause.Its treatment require individualization.

目的 了解儿童色素性玫瑰糠疹的临床表现及组织病理特点.方法 回顾性分析5例儿童色素性玫瑰糠疹患儿临床表现、组织病理及治疗效果等.结果 5例患儿中,男1例,女4例,男女比例1:4;发病年龄为5～16岁,平均9.4岁;病程1个月～6年,平均23.2个月.皮损表现为躯干及四肢散在分布淡褐色或黑褐色斑疹,皮疹长轴大致与皮纹方向一致.组织病理主要表现为表皮角化过度,基底层黑素细胞不同程度增多.采用维生素C、维生素E和抗组胺药物等以及外用润肤剂治疗后,所有患儿皮疹1～3个月后消退.结论 该病根据临床表现及组织病理变化可诊断,目前尚无特效疗法,临床以对症治疗为主.

目的 探讨操作技能直接观察评估(direct observation of procedural skills,DOPS)在小儿泌尿外科住院医师规范化培训中的应用效果.方法 选取2015年7月至2019年12月苏州大学附属儿童医院76名小儿外科住院医师为研究对象,2018年1月之后采用DOPS形成性评价的44名作为试验组,之前未采用DOPS形成性评价的32名作为对照组,试验组和对照组的培训教师和培训内容均相同.规范化培训结束前应用迷你临床演练评估(mini-clinical evaluation exercise,Mini-CEX)对学员的临床操作进行效果评价.采用SPSS 22.0统计学软件对相关数据进行统计分析.结果 试验组医师Mini-CEX考试的整体表现评分(6.55±0.95)分高于对照组(5.91±1.00)分,其差异有统计学意义(P<0.05).结论 应用DOPS形成性评价可以提高小儿泌尿外科规范化培训医师的Mini-CEX考试成绩,提高小儿泌尿外科规范化培训医师实际临床操作能力,改善小儿泌尿外科住院医师规范化培训的效果.

Objective:To explore the clinicopathological characteristics of pediatric ovarian tumors with endocrine disorders and to provide rationales for early interventions.Methods:Clinical data were retrospectively reviewed for 15 children with a diagnosis of ovarian tumors with endocrine disorders of precocious puberty (n=7) and irregular menstruation (n=8). The median age was 121 (47-197) months. The level of estradiol was elevated (n=8) .Results:After laparoscopic exploration, adnexectomy (n=2) and ovarian cystectomy (n=13) were performed. Histopathological diagnosis was epithelial ovarian tumors (n=5), germ cell tumors (n=4) and sex cord-stromal tumors (n=6). Three cases were malignant tumors, including juvenile granulosa cell tumor (n=2) and immature teratoma (n= 1). Two sex-cord tumors with annular tubules were borderline tumors. One case of immature teratoma received postoperative chemotherapy. During follow-ups, the symptoms of precocious puberty and irregular menstruation disappeared and there was no tumor recurrence.Conclusions:There is a high incidence of malignancy in pediatric ovarian tumors with endocrine disorders, especially sex cord-stromal tumors. Therefore preoperative examinations such as sex steroids, anti-Mullerian hormone (AMH), inhibin, human chorionic gonadotropin (HCG) and tumor markers should be performed along with frozensection pathology to evaluate the nature of tumor and determine the resection range. Moreover, follow-up treatments should be guided by a definite pathological diagnosis.

目的 探讨轻型凝血因子缺乏性疾病患者术后阴囊血肿的诊断和治疗.方法 通过分析苏州大学附属儿童医院2019年1月—2022年12月收治的2 例腹股沟斜疝及鞘膜积液合并轻型凝血因子缺乏性疾病的患者术后出血致阴囊血肿的诊疗过程,归纳其临床特点并对此类型患者围手术期管理及处理要点进行文献复习.结果 通过完善凝血因子全套检查明确患者所缺乏的凝血因子,补充相应凝血因子治疗,阴囊血肿逐步吸收.结论 轻型凝血因子缺乏性疾病患者由于出血症状隐匿,术前凝血常规常无明显异常,若术前没有输注凝血因子替代治疗,术后常发生持续而严重的出血,男性患儿会阴区手术术后出血则可能导致巨大阴囊血肿.为防止此类并发症的发生,在手术治疗前,除常规行出凝血及其他有关检查外,详细询问病史及家族史尤为重要.

Propranolol has been used in the first-line therapy of infantile hemangioma (IH) for a number of years; however, the mechanisms through which propranolol regulates IH are not yet fully understood. In the present study, microRNA (miRNA/miR) sequencing analysis was performed to identify differentially expressed miRNAs in human umbilical vascular endothelial cells (HUVECs) treated with propranolol. Cell viability and apoptosis were detected using CCK-8 assay and flow cytometry, respectively. Cell migration was assessed using wound healing, Transwell, and tube formation assays. Methylation-specific PCR was then used to investigate the promoter methylation status. The levels of oxidative stress indicators, including superoxide dismutase, glutathione, and malondialdehyde were also detected. Finally, cell cycle analysis was performed using flow cytometry and western blotting. It was observed that propranolol induced the upregulation of miR-206 in HUVECs, which was caused by demethylation of the miR-206 promoter. Moreover, propranolol significantly inhibited the proliferation of HUVECs by inducing apoptosis, while these phenomena were reversed by miR-206 antagomir. VEGFA was found to be a target gene of miR-206. In addition, propranolol notably inhibited the migration and induced G1 arrest of the HUVECs, whereas these results were eliminated by miR-206 antagomir. Collectively, the findings of the present study demonstrated that propranolol may inhibit the proliferation and migration in HUVECs via modulating the miR-206/VEGFA axis. These findings suggest a novel mechanism through which propranolol suppresses the progression of IH.

目的:观察0.1％糠酸莫米松乳膏在儿童特应性皮炎维持治疗中的临床效果.方法:将127例特应性皮炎患儿随机分为治疗组64例和对照组63例,两组患儿在相同治疗方案控制皮疹后,对照组患儿仅用润肤剂维持,治疗组患儿在润肤剂日常护理基础上继续予0.1％糠酸莫米松乳膏治疗,每周2次,分别在1～3周后观察治疗效果.结果:维持治疗后第1周治疗组复发2例,对照组4例,显效率分别为96.88％和93.65％,两组比较差异无统计学意义(P>0.05).维持治疗后第2周治疗组复发3例,对照组10例,显效率分别为95.31％和84.13％,两组比较差异有统计学意义(P<0.05).第3周治疗组复发5例,对照组18例,显效率分别为92.19％和71.43％,两组比较差异有统计学意义(P<0.01).结论:在儿童特应性皮炎的治疗中,润肤剂和外用激素的使用能较快改善患儿症状,每周2次使用0.1％糠酸莫米松软膏维持治疗能有效减少特应性皮炎病情的反复,建议维持治疗3～4周.

对792例我院确诊的儿童白癜风病例进行回顾性分析.792例患儿中,男405例(51.14％),女387例,3～8岁患儿589例(74.37％),6岁为发病高峰年龄.最常累及部位为面颈部,共284例(35.86％).102例(12.88％)有家族史,534例(67.42％)春夏季发病,242例(30.56％)发病前有外伤、精神因素、日晒等诱发因素.113例(14.27％)伴有其他疾病.实验室检查示白癜风组TPOAb阳性43例(9.31％),TGAb阳性24例(5.19％),显著高于正常对照组的1.65％和0.63％(均P<0.05);血清25-(OH)D水平为(37.64±13.62)nmol/L明显低于正常对照组的(62.87±12.38)nmol/L,差异具有统计学意义(P<0.05).治疗有效率为57.38％,面颈部治疗效果优于躯干及四肢.

Objective:To investigate the miRNA-1246 expression in photoaged human fibroblasts (HSFs) induced by ultraviolet A (UVA) , and to evaluate the effect of upregulating miRNA-1246 expression on its target gene MAPK14 and cell aging.Methods:HSFs were isolated from foreskins of healthy children after circumcision in Children′s Hospital of Soochow University, and irradiated with 10 J/cm 2 UVA once a day for 14 consecutive days. Real-time quantitative PCR was performed to determine the expression of miR-1246 immediately after the first irradiation and on days 3, 7 and 14 after the start of irradiation. Some HSFs were divided into 4 groups: blank control group receiving no treatment, UVA group irradiated with 10 J/cm 2 UVA for 14 days, miR-1246 group transfected with a lentiviral vector carrying miR-1246, and UVA + miR-1246 group transfected with a lentiviral vector carrying miR-1246 followed by irradiation with UVA. After treatment, the HSFs were collected, methyl thiazolyl tetrazolium (MTT) assay was performed to assess cellular proliferativy activity, β-galactosidase staining to detect senescent cells, RT-PCR and Western blot analysis were conducted to measure the mRNA and protein expression of MAPK14 and matrix metalloproteinase 1 (MMP-1) . One-way analysis of variance was used for comparison of means among multiple groups, and least significant difference (LSD) - t test was used for multiple comparisons. Results:On days 7 and 14, the relative expression of miR-1246 in HSFs was significantly lower in the UVA group (4.69 ± 0.85, 3.59 ± 0.45, respectively) than in the blank control group (8.42 ± 0.75, 7.61 ± 0.49, t = 29.84, 31.93, respectively, both P < 0.01) . After upregulation of miR-1246 and irradiation with UVA, MTT assay showed that the cellular proliferative activity significantly differed among the blank control group, UVA group, miR-1246 group, UVA + miR-1246 group (0.82 ± 0.03, 0.23 ± 0.02, 0.81 ± 0.02, 0.61 ± 0.02, respectively; F = 34.90, P < 0.05) , significantly lower in the UVA group than in the blank control group ( t = 28.14, P < 0.01) , lower in the UVA + miR-1246 group than in the miR-1246 group ( t = 10.61, P < 0.01) , but significantly higher in the UVA + miR-1246 group than in the UVA group ( t = 20.30, P < 0.01) . β-Galactosidase staining showed that the proportion of senescent cells significantly differed among the above 4 groups (3.93% ± 1.11%, 81.29% ± 2.53%, 5.50% ± 1.15%, 54.13% ± 2.09%, respectively; F = 16.14, P < 0.05) , significantly higher in the UVA group than in the blank control group ( t = 48.46, P < 0.01) , higher in the UVA + miR-1246 group than in the miR-1246 group ( t = 35.31, P < 0.01) , but significantly lower in the UVA + miR-1246 group than in the UVA group ( t = 14.32, P < 0.01) . Both RT-PCR and Western blot analysis showed that the mRNA and protein expression of MAPK14 and MMP-1 significantly differed among the above 4 groups (both P < 0.05) , significantly higher in the UVA group than in the blank control group ( P < 0.05) , higher in the UVA + miR-1246 group than in the miR-1246 group ( P < 0.05) , but significantly lower in the UVA + miR-1246 group than in the UVA group ( P < 0.05) . Conclusions:In the senescent HSFs induced by UVA, the expression of miR-1246 is suppressed. Upregulating the expression of miR-1246 can exert anti-photoaging effect by inhibiting the expression of its target gene MAPK14 and aging-related protein MMP-1.

目的 研究分析以皮疹为首发症状的儿童朗格汉斯细胞组织细胞增生症(LCH)的临床表现及组织病理特点,以提高LCH早期诊断率.方法 对26例以皮疹为首发症状就诊的LCH患者的临床资料、组织病理、免疫组化结果进行综合分析.结果 26例患儿中,男16例(61.54％),女10例(38.46％),男女比例1.6∶1,＜2岁22例.临床上以湿疹、脂溢性皮炎、紫癜样皮疹多见.24例患儿合并其他系统受累,最常见为骨10例(38.46％),脾9例(34.62％),肺8例(30.77％).免疫组织化学检查中,26例患儿CD1a、S-100均为阳性.结论 LCH发病越早,往往预后越差,皮疹可呈多种形态,提高皮肤活检率尽早完善各项系统检查,有助于儿童LCH的早期诊断,为其进一步的治疗提供依据.

目的 探究在进行子宫肌瘤患者的子宫肌瘤剔除术时,选择经腹子宫肌瘤剔除术和腹腔镜下子宫肌瘤剔除术的效果,分析其临床可用价值.方法 将2017年4月-2019年6月作为研究时段,确认在其中存在资料登记的40例子宫肌瘤患者的个体资料,将患者一般资料录入Excel表格后,按照公式法进行两组均分,单组样本量设置为20.对照组患者应用经腹子宫肌瘤剔除术进行治疗,实验组患者则选择腹腔镜下子宫肌瘤剔除术进行治疗,对比两组患者的各项手术指标以及术后不良反应发生状况.结果 实验组患者的手术时间相较于对照组患者略显延长,但对比组间差异无统计学意义(P＞0.05).而实验组患者的术中出血量、住院时间均优于对照组,差异有统计学意义(P＜0.05).而在实验结果中,实验组患者并发症发生率为10.00％,低于对照组患者的55.00％,组间差异有统计学意义(x2=9.231,P=0.002).结论 在对子宫肌瘤患者进行手术治疗时,选择腹腔镜下子宫肌瘤剔除术,各项指标均能得到有效改善,患者的术后不良反应发生状况也能得到有效控制,具有较高的可应用价值.

目的 探究进行宫颈病变患者的治疗时,选择阴道镜联合利普刀作为治疗方案的效果.方法 将2017年9月-2019年5月作为研究时段,在该时段内该院数据库进行资料统计确认,在其中存在登记的100例宫颈病变患者的基本资料,将其录入Excel表格后进行公式法两组均分,单组样本量设置为50例,分别记名为对照组与实验组.对照组患者选择利普刀进行治疗,实验组患者则在利普刀的手术基础上,选择阴道镜进行引导,对比两组患者的各项手术指标以及治疗总有效率.结果 在该次研究结果中,两组患者在接受治疗后,临床症状均有所改善,但实验组患者治疗总有效率为45(90.00％),优于对照组患者的31(62.00％),组间差异有统计学意义(χ2=10.746,P=0.001);实验组患者的各项手术指标相较于对照组明显更优,数据对比差异有统计学意义(P<0.05).结论 在对宫颈病变患者进行实际治疗时,应用阴道镜联合利普刀开展手术操作,能够大大降低患者的手术创面大小以及愈合时间,能够有助于提高患者的治疗总有效率,对于患者的康复来说有积极意义,具有高度的可推广价值.

目的:探讨普萘洛尔治疗增生期婴幼儿血管瘤的疗效及血清细胞因子检测的临床意义.方法:选取2015年4月～2018年5月期间我院皮肤科诊治的76例增生期血管瘤患儿为治疗组,治疗组给予普萘洛尔治疗8周.同时选取同期来我院行健康体检的正常婴幼儿50例为对照组,检测并比较治疗组治疗期间以及对照组体检时血清血管内皮生长因子(VEGF)、基质金属蛋白酶-9(MMP-9)、缺氧诱导因子-1α(HIF-1α)以及表皮生长因子样结构域(EGFL7)水平,观察治疗组治疗后的疗效及不良反应发生情况,分析治疗8周后血清VEGF、EGFL7、HIF-1α、MMP-9下降水平与疗效等级的关系,分析治疗组血清VEGF、EGFL7、HIF-1α、MMP-9之间的相关性.结果:不同类型血管瘤患儿治疗4周后总有效率整体比较差异无统计学意义(P＞0.05),治疗8周后混合型、深部型血管瘤患儿总有效率高于治疗4周后,且混合型、深部型血管瘤患儿总有效率高于浅表型血管瘤患儿(P＜0.05).治疗组治疗期间未出现严重不良反应现象.与对照组比较,治疗组不同时间点血清VEGF、EGFL7、HIF-1α、MMP-9水平均升高(P＜0.05);随着时间的推移,治疗组血清VEGF、EGFL7、HIF-1α、MMP-9水平逐渐降低(P＜0.05).Spearman等级相关法分析显示,治疗组治疗8周后血清VEGF、EGFL7、HIF-1α、MMP-9下降水平与疗效等级呈正相关(P＜0.05).Pearson相关性分析显示,治疗组血清VEGF、EGFL7、HIF-1α以及MMP-9水平之间两两呈正相关(P＜0.05).结论:普萘洛尔治疗增生期婴幼儿血管瘤安全有效,其具体作用机制可能与下调血清VEGF、EGFL7、HIF-1α、MMP-9水平有关.

目的 分析婴幼儿阴囊急症的病因和临床特征并探讨其临床诊断和处理方法 .方法回顾性分析我院2010年1月至2018年12月收治的53例婴幼儿阴囊急症患儿的临床资料,分析婴幼儿阴囊急症病因和临床表现并探讨其临床诊断和处理方法.结果 53例阴囊急症患儿年龄1 d至10个月27 d,平均年龄4个月.其中急性附睾炎41例(77.4％),睾丸扭转10例(18.9％),阴囊脓肿2例(3.8％).患儿均出现阴囊红肿症状,就诊时阴囊红肿出现时间1 h至3 d.急性附睾炎经彩色多普勒超声确诊33例(80.5％),超声提示附睾体积增大,附睾血流增多;8例(19.5％)经手术证实,超声提示睾丸/附睾血流不均.睾丸扭转和阴囊脓肿均为超声确诊.急性附睾炎患儿实验室检查血常规异常31例(75.6％),C反应蛋白升高35例(85.4％),应用抗生素前尿常规异常17例(41.5％),其中尿培养阳性5例,均为大肠埃希菌.术中取患儿脓液进行培养,阳性3例,1例链球菌,2例大肠埃希菌.睾丸扭转患儿入院后行手术探查,切除坏死睾丸.阴囊脓肿患儿入院后行脓肿切开引流术.结论 婴幼儿阴囊急症中急性附睾炎和睾丸扭转较为常见,两者鉴别诊断主要靠彩色多普勒超声,对不能及时确诊的患儿需行手术探查.

目的 分析翻转课堂结合情景教学法在皮肤科教学中的应用效果.方法 选取皮肤科2018年1月—2018年12月本科生74人,数字随机法进行分组,分为观察组和对照组,对照组37人采用常规教学方法带教,观察组37人采用翻转课堂结合情境教学法带教,对比两组的考核成绩和教学反馈情况.结果 观察组的理论知识评分(87.51±9.49)分、实践技能评分(86.56±10.32)分、综合成绩评分(88.73±11.52)分、优良率94.59％ 均优于对照组(82.32±8.26)分、(80.61±9.77)分、(81.62±10.71)分、78.38％,组间差异具有统计学意义(P<0.05).结论 在皮肤科教学中实施翻转课堂结合情境教学法可有效提升教学质量和学生授课满意度.

留学生不仅在文化背景、生活学习习惯、教育经历等方面与国内学生存在差异,还存在不同程度的语言交流障碍.本文总结留学生实习带教经验,找出留学生在儿童骨科临床实习期间存在的问题,听取留学生反馈意见,通过合理组建带教队伍,改革教学方式等手段,完善儿童骨科实习带教方案,提高留学生带教质量.

住院医师规范化培训是医学毕业生从学生成长为合格医生的重要阶段.当前我国面临儿科医师短缺的困境,儿科住院医师规培是培养儿科医学专业人才的重要措施,任重而道远.规培医院需要合理安排选择不同的教学形式,争取做到优势互补,促进教学培训效果的进一步优化,培养更多优秀的小儿外科医师.以问题为基础的PBL教学法和以案例为基础CBL教学法是两种注重学生参与的教学模式.文章探讨PBL联合CBL教学模式在儿外科住院医师规范化培训中的应用,并且对两种教学模式在案例及问题设计、教师、学生、评价体系和配套资源等方面出现的问题进行分析并提出改进意见.