To establish an ultrasound-based radiomics model to differentiate fibro adipose vascular anomaly (FAVA) and intramuscular venous malformation (VM). The clinical data of 65 patients with VM and 31 patients with FAVA who were treated and pathologically confirmed were retrospectively analyzed. Dimensionality reduction was performed on these features using the least absolute shrinkage and selection operator (LASSO). An ultrasound-based radiomics model was established using support vector machine (SVM) and random forest (RF) models. The diagnostic efficiency of this model was evaluated using the receiver operating characteristic. A total of 851 features were obtained by feature extraction, and 311 features were screened out using the t-test and Mann-Whitney U test. The dimensionality reduction was performed on the remaining features using LASSO. Finally, seven features were included to establish the diagnostic prediction model. In the testing group, the AUC, accuracy and specificity of the SVM model were higher than those of the RF model (0.841 [0.815-0.867] vs. 0.791 [0.759-0.824], 96.6% vs. 93.1%, and 100.0% vs. 90.5%, respectively). However, the sensitivity of the SVM model was lower than that of the RF model (88.9% vs. 100.0%). In this study, a prediction model based on ultrasound radiomics was developed to distinguish FAVA from VM. The study achieved high classification accuracy, sensitivity, and specificity. SVM model is superior to RF model and provides a new perspective and tool for clinical diagnosis.

AIMS:Intramuscular hemangioma capillary type (IHCT) is a rare entity that refers to fast-flow vascular lesions. This study aims to elucidate the relationships between clinicopathological, radiological, and molecular characteristics in IHCT patients. METHODS AND RESULTS:We reviewed all IHCT cases which were treated surgically in our pathology database from 2014 to 2023. Ten cases were analyzed via next-generation sequencing (NGS) and Sanger sequencing. The cohort consisted of 10 patients (6 males, 4 females) with a median age of 18 years (range: 1-37). Disease lesions were located in the trunk (n = 4), upper extremity (n = 2), lower extremity (n = 2), shoulder (n = 1), and neck (n = 1). IHCT is most commonly a progressively increasing painless mass. Histopathologically, all lesions exhibited aggregates, lobules, and anastomosing cords of capillary-type vessels separating or infiltrating the skeletal muscles. Four cases exhibited irregularly dilated vessels with thick walls, such as arteriovenous malformations (AVM) in the lesion's periphery. MRI findings commonly demonstrated a well-delineated, homogeneous mass. Somatic mutations were detected in seven of the ten IHCT cases. Four cases harbored mutations in MAP2K1 (p.Q58_E62del, p.K57_G61del, p.K57 N), two cases harbored mutations in KRAS (p.Q61R and p.L56V, p.G13R), and one case harbored a mutation in HRAS (p.D69_Q70insRWYSAMRD). Mutant allele frequencies detected by sequencing ranged from 9.98% to 15.97%. CONCLUSIONS:The hemodynamic and molecular genetic phenotypes of IHCT closely resemble those observed in AVMs. Newly identified KRAS missense mutations, including cases with coexisting mutation types, and HRAS insertion mutations offer valuable insights into the genetic basis of vascular anomalies. These findings may also present potential targets for the development of novel pharmacotherapeutic interventions.

Kaposiform lymphangiomatosis(KLA)is an aggressive subtype of generalized lymphatic anomaly. It is difficult to diagnose because of the lack of specificity of early symptoms. Moreover, there is still a lack of effective treatment, so the prognosis of this disease is generally poor, with a 5-year survival rate of 51% and an overall survival rate of only 34%. This article reviews the progress of the genetic studies related to the development of KLA and summarizes potential drug targets. A review of the literature found that the mutations potentially associated with KLA pathogenesis included NRAS gene mutations and CBL gene mutations. The signaling pathways involved were the PI3K-AKT-mTOR pathway and the RAS-MAPK-ERK pathway. Therefore, the key molecules PI3K/AKT/mTOR and RAS/MEK in the above signaling pathway may be used as targets for KLA-targeted therapy and develop specific treatments.

AIMS:Fibroadipose vascular anomaly (FAVA) is a complex vascular malformation that is likely to be under-recognised. In this study we aimed to report the pathological features and somatic PIK3CA mutations associated with the most common clinicopathological features. METHODS AND RESULTS:Cases were identified by reviewing the lesions resected from patients with FAVA registered at our Haemangioma Surgery Centre and unusual intramuscular vascular anomalies in our pathology database. There were 23 males and 52 females, who ranged in age from 1 to 51 years. Most cases occurred in the lower extremities (n = 62). The majority of the lesions were intramuscular, with a few disrupting the overlying fascia and involving subcutaneous fat (19 of 75), and a minority of the cases had cutaneous vascular stains (13 of 75). Histopathologically, the lesion was composed of anomalous vascular components that were intertwined with mature adipocytic and dense fibrous tissues and vascular components with: (a) clusters of thin-walled channels, some with blood-filled nodules and others with thin walls resembling pulmonary alveoli; (b) numerous small vessels (arteries, veins and indeterminate channels) - proliferative small blood vessels were often mixed with adipose tissue; (c) larger abnormal venous channels usually irregularly and sometimes excessively muscularised; (d) lymphoid aggregates or lymphoplasmacytic aggregates were usually observed; and (e) lymphatic malformations were sometimes seen as minor elements. All patients had their lessons subjected to PCR, and 53 patients had somatic PIK3CA mutations (53 of 75). CONCLUSIONS:FAVA is a slow-flow vascular malformation with specific clinicopathological and molecular characteristics. Its recognition is fundamental for its clinical/prognostic implications and for targeted therapy.

ObjectiveTo investigate the value of ultrasonography as a diagnostic aid in differentiating intramuscular capillary-type hemangioma (ICTH) from fibro-adipose vascular anomaly (FAVA).MethodsA retrospective analysis was conducted of the clinical and ultrasound imaging data of 20 patients with ICTH and 45 patients with FAVA who were admitted to and pathologically confirmed in hospital between January 2013 and April 2023. The clinical and ultrasonographic appearances of the lesions in the two groups were compared and analyzed. A stepwise regression analysis was performed, and a joint diagnostic equation was constructed using the final variables selected. The receiver operating characteristic (ROC) curve and indicators, including sensitivity and specificity, were used to evaluate the efficacy of the joint diagnostic model.ResultsThe two groups of patients suffering from ICTH and FAVA presented a statistically significant difference (P< 0.05) in terms of ‘age’, ‘lesion size’, ‘fascial tail sign’, ‘presence of a fatty-tissue-like hyperecho around the lesion’, ‘blood flow’ and ‘presence of straight blood capillaries within the lesion’. Finally, the variables ‘fascial tail sign’ and ‘presence of straight blood capillaries within the lesion’ were selected to construct the model. The constructed joint diagnostic model had a sensitivity value of 70.0% (95% CI: 59.00–81.00), a specificity value of 98.0% (95% CI: 94.70–100.00) and a ROC curve value of 0.908, indicating the high efficacy of the combined diagnosis method.ConclusionsUltrasonography can be utilized to differentiate ICTH from FAVA, and the combined diagnosis method can further improve the technique’s diagnostic efficacy.

目的 探讨加速康复外科(ERAS)理念对下肢肌内静脉畸形(IMVM)患者术后康复的影响.方法 选择2018年1月至2019年12月河南省人民医院收治的下肢IMVM患者81例为研究对象,所有患者行IMVM切除术,根据围手术期护理措施将患者分为观察组(n=41)和对照组(n=40),对照组患者围手术期给予常规护理,观察组患者围手术期给予ERAS理念护理模式.术前、术后1周及术后1、3个月,采用视觉模拟评分(VAS)评估患者术区疼痛程度,采用角度尺测量踝关节活动度,采用美国矫形外科足踝协会踝-足功能量表(AOFAS-AHS)评估患者的踝-足功能,采用关节炎生活质量测量量表2-短卷(AIMS2-SF)评估患者的生活质量.观察2组患者跟腱挛缩、术区出血、术区感染等并发症的发生情况.结果 术前2组患者疼痛VAS评分比较差异无统计学意义(P>0.05);2组患者术后1周及1、3个月时疼痛VAS评分显著低于术前(P<0.05),2组患者术后1、3个月时疼痛VAS评分显著低于术后1周(P<0.05),2组患者术后3个月时疼痛VAS评分显著低于术后1个月(P<0.05);术后1周及1、3个月时,观察组患者疼痛VAS评分显著低于对照组(P<0.05).术前2组患者的踝关节背屈角度、跖屈角度比较差异无统计学意义(P>0.05);2组患者术后1周及1、3个月时踝关节背屈角度、跖屈角度显著大于术前(P<0.05),2组患者术后1、3个月踝关节背屈角度、跖屈角度显著大于术后1周(P<0.05),2组患者术后3个月时候踝关节背屈角度、跖屈角度显著大于术后1个月(P<0.05);术后1周及1、3个月时,观察组患者的踝关节背屈角度、跖屈角度显著大于对照组(P<0.05).术前2组患者AOFAS-AHS评分比较差异无统计学意义(P>0.05);2组患者术后1周及1、3个月时AOFAS-AHS评分显著高于术前(P<0.05),2组患者术后1、3个月时AOFAS-AHS评分显著高于术后1周(P<0.05),2组患者术后3个月时AOFAS-AHS评分显著高于术后1个月(P<0.05);术后1周及1、3个月时,观察组患者AOFAS-AHS评分显著高于对照组(P<0.05).术前2组患者AIMS2-SF评分比较差异无统计学意义(P>0.05);2组患者术后1周及1、3个月时AIMS2-SF评分显著高于术前(P<0.05),2组患者术后1、3个月时AIMS2-SF评分显著高于术后1周(P<0.05),2组患者术后3个月时AIMS2-SF评分显著高于术后1个月(P<0.05);术后1周及1、3个月时,观察组患者AIMS2-SF评分显著高于对照组(P<0.05).观察组和对照组患者并发症发生率分别为7.32％(3/41)、37.50％(15/40),观察组患者并发症发生率显著低于对照组(χ2=9.451,P<0.05).结论 在下肢IMVM术后康复训练中应用ERAS理念可有效减轻IMVM患者的疼痛,提高患者踝-足关节功能和生活质量,降低并发症发生率.

We have previously developed several kinds of rapamycin-encapsulated nanoparticles to achieve sustained release of rapamycin to treat hemangioma. However, lack of intrinsic targeting and easy clearance by the immune system are major hurdles that artificial fabricated nanoparticles must overcome. We constructed rapamycin-encapsulated macrophage-derived exosomes mimic nanoparticles-in-microspheres (RNM), to achieve the goal of continuous targeted therapy of hemangiomas. The rapamycin-encapsulated exosome mimic nanoparticles (RN) were firstly prepared by the extrusion-based method from the U937 cells (the human macrophage cell line). After then, RN was encapsulated with PLGA (poly(lactic-co-glycolic acid)) microspheres to obtain RNM. The release profile, targeting activity, and biological activity of RN and RNM were investigated on hemangioma stem cells (HemSCs). RN has a size of 100 nm in diameter, with a rapamycin encapsulation efficacy (EE) of 83%. The prepared microspheres RNM have a particle size of ~30 µm), and the drug EE of RNM is 34%. The sustained release of RNM can remarkably be achieved for 40 days. As expected, RN and RNM showed effective inhibition of cellular proliferation, significant cellular apoptosis, and remarkable repressed expression of angiogenesis factors in HemSCs. Our results showed that RNM is an effective approach for prolonged and effective delivery of rapamycin to hemangiomas.

目的 分析协同护理模式下早期运动干预应用于下肢肌内静脉畸形(VM)切除手术后的效果.方法 选取81例下肢肌内VM切除手术患者,依据随机数字表法分为对照组(40例)和观察组(41例),对照组实施术后常规护理,观察组在对照组基础上实施协同护理模式下早期运动干预.比较2组患者干预前后焦虑自评量表(SAS)评分、抑郁自评量表(SDS)评分、视觉模拟量表(VAS)评分、美国矫形足踝协会评分系统(AOFAS)评分以及患者满意度.结果 干预前2组患者SAS评分、SDS评分、VAS评分差异无统计学意义(P>0.05);干预后观察组SAS评分、SDS评分、VAS评分分别为(32.62±1.85)分、(31.52±1.05)分及(1.64±0.19)分,均低于对照组(46.38±4.82)分、(45.52±5.67)分及(3.95±1.27)分,差异有统计学意义(P<0.05).干预前2组AOFAS评分差异无统计学意义(P>0.05);干预后观察组AOFAS评分[功能评分(42.62±5.28)分、疼痛评分(36.82±4.52)分]高于对照组[功能评分(33.62±2.84)分、疼痛评分(24.34±2.07)分],差异有统计学意义(P<0.05).观察组患者满意度(95.12％)高于对照组(72.50％),差异有统计学意义(P<0.05).结论 协同护理模式下早期运动干预可有效减轻下肢肌内VM切除手术患者不良情绪、疼痛感,改善下肢运动功能,提高患者满意度.

Objective:To evaluate the clinical efficacy of DSA-guided sclerotherapy with polidocanol injectable foam in treatment of Puig's Ⅰ and Ⅱ perianal venous malformations in children.Methods:A retrospective analysis was made of 38 children with Puig's Ⅰ and Ⅱ perianal venous malformations who were treated with DSA-guided percutaneous puncture of 3% polydocarol foam sclerotherapy under general anesthesia from March 2018 to June 2019 in Henan Provincial People's hospital.The curative effect, adverse reactions were observed in follow-up.Results:The 38 children were treated 76 times (1-3 times/case), and the average single application dose of 3% polydocarol foam sclerosant was (5.94±1.69) mL. 28 cases were cured, 9 cases were basically cured and 1 case was effective; the cure rate was 73.68% (28/38), and the total effective rate was 100%. Adverse reactions. There were mild swelling at the lesion in 76 cases after interventional sclerotherapy and tension blisters in the skin in 3 cases.One patient had mild skin ulceration, however, the ulcertion healed after 10 days of symptomatic treatment. After 14-28 months of follow-up (median follow-up time was 24 months), there was no significant recurrence of symptoms.Conclusions:3% polydocanol foam sclerotherapy is a minimally invasive, safe, effective and worth popularization treatment for Puig's Ⅰ and Ⅱ perianal venous malformations in children.

Objective:To investigate the clinical effect of supermicrosurgery combined with modified anterograde replantation in Yamano Ⅰ zone.Methods:To retrospect and analysis the data of replantation of amputated finger in Yamano Ⅰ in Xuzhou Renci Hospital from March 2016 to October 2019. All patients were treated by supermicrosurgery combined with modified anterograde replantation method. The modified anterograde replantation method was according to proportional anastomosis of arteries and veins, the proceed was artery and nerve → fixation of bone → anastomosis of subcutaneous vein → suturing of skin wound. In the procedure of anastomosis of arteries and nerves, the position of injured finger replantation was modified, the customary horizontal position was altered to vertical position, the severed finger was flipped to the palmar side which was taken as the rotation axis, and the anastomosis was performed through the dorsal approach. Both the proximal and distal sections was completely exposed in the position, so that the visual angle of the surgeon was changed from squint to direct vision, and which suitable for the observation and operation. Follow-up was performed in outpatient department and WeChat after surgery, and functional evaluation was recorded according to the trial standard for functional evaluation of replantation of severed finger of Hand Surgery Society of Chinese Medical Association.Results:All of 38 patients were involved, including 23 males and 15 females. The mean age was 27.3 years (ranged from 1 to 58 years). All of injured fingers were completely severed in Yamano Ⅰ zone by single finger. The causes of injuries included chainsaw injury( n=6), knife cutting injury ( n=5), crush injury ( n=19), and avulsion injury ( n=8). According to the classification of Yamano Ⅰ zone, there were 4 cases of type Ⅰ, 14 cases of type Ⅱ, 11 cases of type Ⅲ, 6 cases of type Ⅳ and 3 cases of type Ⅴ. There were 12 cases of thumb, 9 cases of index finger, 6 cases of middle finger, 7 cases of ring finger and 4 cases of little finger. The ischemia time was 1-12 h. The survival rate was 94.7% (36/38). Thirty-three patients were followed up for 6-12 months. The length and shape of the fingers were similar to the contralateral finger, the nail was intact, and the two-point discrimination was 3-5 mm. The hand function returned to normal. Conclusions:The supermicrosurgery combined with modified anterograde replantation in Yamano Ⅰ zone can be used for the replantation of fingertip with arterial and venous anastomosis. The replantation fingertip has a high survival rate, satisfactory function and appearance. It is an ideal choice for the treatment of amputated finger in Yamano Ⅰ.

目的 了解神经科血管瘤患儿母亲抑郁现状及影响因素,为减轻患儿母亲抑郁情绪及干预提供理论支持.方法 选取2018-09—2020-09在河南省某三级甲等医院的神经科住院的198名血管瘤患儿母亲为研究对象,采用抑郁自评量表和匹兹堡睡眠质量指数量表进行调查.结果 调查结果显示,血管瘤患儿母亲抑郁的发生率为46.50％.二分类Logisitic回归分析显示,血管瘤患儿母亲抑郁影响因素包括学历(OR:12.18,95％CI:4.04～36.72)、医疗费用支付方式(OR:7.68,95％CI:1.29～45.66)及PSQI得分(OR:1.98,95％CI:1.39～2.82).结论 血管瘤患儿母亲的抑郁发生率较高,医护工作者应提高重视,通过加强患儿母亲血管瘤疾病相关的健康教育,及时发现其负性情绪并给予疏导,改善患儿母亲的睡眠质量以降低抑郁的发生.

目的 探讨综合性护理在颌面部血管畸形患者中的应用效果.方法 选取201 8年4月至2020年6月河南省人民医院收治的1 26例颌面部血管畸形患者作为对象,按照随机数字表法分为两组,各63例.对照组接受常规护理,观察组在对照组基础上接受综合性护理.护理3个月后,比较两组患者自我管理水平、口腔功能、满意度及依从性.结果 干预后,观察组异常情况处理、自我管理信心、自我责任感、自我管理水平评分均高于对照组(P＜0. 05 ).干预后,两组固位功能、语言功能、咀嚼功能及咬合功能评分均高于干预前,差异有统计学意义(P＜0. 05 );且观察组各项评分高于对照组,差异有统计学意义(P＜0. 05).观察组操作技术、服务态度、健康教育、心理干预情况及护理质量评分均高于对照组,差异有统计学意义(P＜0. 05).观察组患者的坚持用药、合理饮食、规律锻炼及定期复诊率均高于对照组,差异有统计学意义(P＜0. 05).结论 综合性护理可提高颌面部血管畸形患者自我管理水平,改善患者口腔功能,提高依从性,可获得较高的护理满意度.

Hydrogel-based local delivery systems provide a good delivery platform for cancer immunotherapy. Injectable hydrogels can directly deliver antitumor drugs to the tumor site to reduce systemic toxicity and achieve low-dose amplification immunotherapy. Therefore, it may overcome the problems of low drug utilization rate and the systemic side effects in cancer immunotherapy through systemic immune drugs, and it provides simple operation and little invasion at the same time. This study aimed to review the research progress of injectable hydrogels in tumor immunotherapy in recent years. Moreover, the local delivery of multiple drugs using injectable hydrogels in tumors is introduced to achieve single immunotherapy, combined chemo-immunotherapy, combined radio-immunotherapy, and photo-immunotherapy. Finally, the application of hydrogels in tumor immunotherapy is summarized, and the challenges and prospects for injectable hydrogels in tumor immunotherapy are proposed.

Objective:To identify the gene mutations associated with facial cleft-related orbital hypertelorism in 3 pairs of monozygotic twins with different phenotypes (with/without hypertelorism) and to investigate their mechanisms.Methods:From May 2014 to May 2019, 3 pairs of monozygotic twins, 2 males and 4 females, aged 5-18 years, were treated in Ninth People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, one with normal orbital distance and widening of orbital distance was caused by facial fissure. Among the twins, there was 1 case of orbital hypertelorism and the other case of without orbital hypertelorism, and the hypertelorism was caused by facial cleft. To screen for mutations in hypertelorism, whole genome sequencing was performed on 3 pairs of twins. The Sanger method was used to sequence the exons of 33 patients with facial fissure associated hypertelorism and 50 healthy individuals in the same period to identify the genes selected by the whole genome sequencing. The periosteal tissues were obtained from patients and healthy people during plastic surgery. The cells were cultured, the activity of alkaline phosphatase was measured, and the osteogenic differentiation was identified by alizarin red staining, real-time quantitative PCR and Western blotting were used to detect the mRNA and protein expression of signal transduction pathways in periosteal cells.Results:Whole genome sequencing analysis showed that in all three sets of twins, a new synonymous mutation (c.1479G>A, p. Q493Q) was found in the MAML3. In Sanger exon sequencing, 17(51.5%) of 33 patients with hypertelorism carried the mutation, while no mutation was detected in 50 normal controls. The result of periosteum-derived cytology showed that the expression of MAML3 mRNA and protein in the patient-derived cells was lower than that in the healthy-derived cells. Three, 7, 14 days after osteoinduction, the ALP activity in the cells from the patients was higher than that from the healthy subjects (8.540±1.450, 20.740±2.514, 24.090±3.213 vs. 5.268±0.482, 11.680±1.527, 13.200±0.592; all P<0.05). Fourteen days after osteoinduction, the result of alizarin red staining showed that there were more erythema formation in the cells from the patients than those from the healthy subjects, these result suggest that MAML3 mutation may lead to over-differentiation of human periosteal-derived cells. The mRNA and protein expression levels of hes1 and hes5 downstream of the Notch signal pathway were down-regulated in the periosteal cells of the patients, while Wnt3a and β-catenin mRNA and protein expression levels were up-regulated in the Wnt signal pathway. Conclusions:The MAML3 gene (c.1479G>A, p. Q493Q) mutation is one of the causative genes of facial cleft-related hypertelorism. Notch and Wnt/β-catenin signaling pathway play an important role in the pathogenesis of hypertelorism.

Objective:To investigate clinical stages and surgical effect during the treatment extremities for intramuscular venous malformations.Methods:A retrospective study was conducted on 336 cases diagnosed with intramuscular venous malformations of extremities from January 2016 to December 2018 in Henan Provincial People’s Hospital, including 163 male and 173 female patients with an average age of 16.7 years (range, 5-65 years). The intramuscular venous malformations of the extremities were diagnosed based on patients’ complaints, physical and magnetic resonance imaging (MRI) examinations. The extents of intramuscular venous malformations were ranged from stage Ⅰ through stage Ⅳ according to preoperative clinical symptoms, the degree of movement of joints, X-ray plain film and MRI examination. The 4 phases are stage Ⅰ painful stage, stage Ⅱ reversible joint dysfunction stage, stage Ⅲ joint dysfunction stage, stage Ⅳ ossification stage. Venous malformation resection was performed in stage Ⅰ and stage Ⅱ patients, while venous malformation resection combined with extended Z-type tendon anastomosis were performed in stage Ⅲ and stage Ⅳ patients. All patients received systematic rehabilitation exercises after operation. The surgical effects were evaluated according to MRI results, visual analogue scales of pain, changes of joint range of motion and patient’s satisfaction levels before operation and at the last time of follow-up.Results:Among 336 cases, 255 cases were treated with simple venous malformation resection and 81 cases were treated with venous malformation resection combined with extended Z-type tendon anastomosis. There were 173, 82, 75, 6 cases in stage Ⅰ, Ⅱ, Ⅲ, Ⅳ, respectively. Among 336 cases, clinical cure was obtained in 330 and improved in 6 after surgical treatment. The operation recovery rate was 98.3%, and the patient satisfaction rate was 100%.Conclusion:The clinical staging before venous malformation resection is helpful for accurately choosing surgical methods, improving operation recovery rate, and achieving optimal therapeutic results.

Background The clinical features and surgical outcomes of patients with Kaposiform hemangioendothelioma (KHE) is not fully clear. The purpose of this study is to better understand the therapeutic effect and hematological indices of surgical therapy for KHE. Procedure A retrospective study was conducted to review the medical documents of 43 patients with KHE who were treated with surgical resection at our hospital between February 2016 and November 2019. Multiple anatomical sites were involved. The curative effect was evaluated according to the tumor volume shrinkage, color shade, and blood examinations including platelet count, hemoglobin level, red blood cell number, clotting time and D-dimer concentration. Results For all cases underwent surgical treatment, the curative effects accounted for 100%. At the end of the treatment, 9 children were followed up for 6 months, and no evidence of the recurrence of KHE was not found in any case. The number of red blood cells and clotting time were maintained at normal levels during different stage of treatment. The concentrations of hemoglobin and fibrinogen and the average number of platelets increased significantly after surgical treatment. The concentrations of D-dimer were much higher than normal value during admission with a high variability, and significantly decreased after surgery treatment. Further analysis found that the changing trends of D-dimer was positively correlated with fibrinogen protein or platelet number, but not clotting time. Conclusions The surgical treatment method is highly effective and of great significance for KHE therapy in pediatric clinic.

肉芽肿性血管瘤亦称化脓性肉芽肿、毛细血管扩张性肉芽肿、分叶状毛细血管瘤，并非真性肿瘤，临床上较为少见。具有生长迅速，质脆，易发生溃疡、出血等特点，临床上易被误诊为恶性肿瘤。本文报道一例软腭特殊肉芽肿性血管瘤。

Objective:To investigate features and surgical results of Kasabach-Merritt phenomenon (KMP) with failed drug therapy.Methods:Forty-seven children with KMP who failed drug treatment in Henan Provincial People’s Hospital from January 2012 to December 2017, 26 males and 21 females, aged 1 to 11 months, underwent simple hemangiomas or hemangiomas resection + skin graft in situ. The clinical manifestations, intraoperative and postoperative conditions of them were analyzed.Results:Forty-seven children were younger. The time from hemangioma to KMP was 0 to 11 months. The tumor was deep and large. Forty-one patients underwent simple hemangiomas resection and 6 patients underwent hemangioma resection and skin graft. The operation time was 35 to 260 minutes, and the bleeding volume was 20 to 380 ml. The platelet count returned to normal on the 1 to 9 days after surgery, the peak appeared at 2 to 60 days, and the hospitalization time was 14 to 72 days. No recurrence or dysfunction patients was found after follow-up for 24 to 78 months.Conclusions:The progression time of KMP is short, and the patients who fail in conservative treatment of medicine can get better treatment effect.

We previously developed propranolol-encapsulated liposomes-in-microspheres (PLIM) to realize the sustained propranolol release for the treatment of hemangiomas. However, the liposomes released from the microspheres still lacked specificity for CD133-positive hemangioma-derived stem cells (HemSCs) which are considered to be the seeds of hemangiomas. Therefore, we hereby encapsulated propranolol-loaded CD133 aptamers conjugated liposomes in poly(lactic-co-glycolic acid (PLGA) microspheres to develop propranolol-loaded CD133 aptamers conjugated liposomes-in-microspheres (PCLIM), to realize the aim of the sustained and targeted therapy of hemangiomas. The evaluation of the release of propranolol from PCLIM was carried out, and the cytotoxic effect and angiogenic growth factor expression inhibitory ability of PCLIM were performed in HemSCs. The in vivo hemangioma inhibitory ability of PCLIM was also investigated in nude mice with subcutaneous human hemangiomas. PCLIM possessed a desired size of 29.2 μm, drug encapsulation efficiency (25.3%), and a prolonged drug release for 40 days. Importantly, PCLIM could inhibit HemSCs proliferation and the protein expression of basic fibroblast growth factor (bFGF) and vascular endothelial growth factor-A (VEGF) in HemSCs to a greater extent compared with PLIM. In nude mice bearing hemangioma xenograft, PCLIM showed the best therapeutic efficacy towards hemangiomas, as reflected by remarkably decreased hemangioma volume, weight and microvessel density (MVD). Thus, our results demonstrated that PCLIM realized the sustained and targeted treatment of hemangiomas, resulting in remarkable inhibition of hemangiomas.

Objective To assess the safety and effectiveness of sufficient,short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon,who were insensitive to glucocorticoids.Methods The infantile cases were retrospectively analyzed during May 2011 to December 2016,who were clinically diagnosed as KMP and insensitive to glucocorticoids,received PLT transfusion and surgical resection.PLT transfusion in patients whose PLTC was less than 30 × 109/L,was 0.3 therapeutic dose (TD)/kg,and 0.2 TD/kg in PLTC ≥ 30 × 109/L group.The maximum was 1 TD.Criteria of the PLT transrusion:1 hour after the transfusion,the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated.PLTC ≥ 100 × 109/L,CCI ＞ 7.5 × 109/L and PPR ＞ 30％ were defined as effective;while PLTC =(50-99) × 109/L,CCI ＞7.5 × 109/L and PPR ＞30％ as partial effective;PLTC ＜50 × 109/L,or CCI≤7.5 × 109/L,or PPR ≤30％ were defined as ineffective.By reviewing the method and response of their PLT infusions,to figure out the most effective way in rising PLT,as a part of pre-operation treatment.Results There were 46 cases in the research.Based on the PLTC,CCI and PPR 1 hour after PLT transfusion,there were 44 effective transfusion,2 patients with partial effectiveness,and no ineffective case.There was no allergic or heart failure happened in any cases.No critical potential complications of PLT transfusion occurred,including fluid and iron overload,alloimmunization to human leukocyte antigen and/or PLT antigen.Conclusions Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones.They can create a more optimistic opportunity for surgical resections.