Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia with enlarging vascular tumour,KMP usually develops in infancy and is associated with significant morbidity and mortality,the mortality rate is reported as high as 30％.It commonly reported sites of tumor include extremities,trunk,retroperitoneum and neck.There is no consensus in treatment and various regimens have been used by different authors.This report is aim to learn the pathophysiology of the KMP and its diagnosis and treatment.

OBJECTIVE We designed the overall layout,formulated the functional demand,and optimized the key design and operating parameters of a new-style asepsis clean ward.The new-style ward intends to provide a higher quality environment for patient infection control and all-around protection during treatment.METHODS We applied computational fluid dynamics(CFD)simulation to optimize the design scheme according to Chinese and international hospital design specifications.RESULTS The new-style asepsis ward reflected a new idea,new technology,high standards and created a comfortable and safe,high-quality environment for the treatment of patient.The Satisfaction of patients was more than 95% and workload of nurses was alleviated obviously.CONCLUSIONS The new-style asepsis ward can significantly control the endogenous infection of patients.

OBJECTIVE To compare the computational fluid dynamics(CFD) simulated results and field test data of the new-style asepsis ward.METHODS We used various equipments to test the asepsis ward and CFD software to simulate airflow of asepsis ward.RESULTS After calculating and comparing,we found the CFD simulation results agreed well with the tested data.Key design and operating parameters were up to the standard.The new-style of asepsis ward could improve the air quality and provide a comfortable environment for patient treatment and it was of great help to decrease the infection rate of patient.CONCLUSIONS The new-style asepsis ward can significantly control the endogenous infection of patients.

Objective To describe and examine the clinical characteristics and spectrum of tufted angiomas (TA) in infants so as to explore the therapeutic approaches to Kasabach-Merritt phenomenon (KMP).Methods The clinical and follow-up data of 24 patients with TA were retrospectively analyzed between Jan.2009 and Mar.2013.The median age of the patients was 7.5 months(ranged from 18 days to 2 years),including l0 male and 14 female.Surgical excision and observation were chosen according to the lesion and conditions of the patients.The mean follow-up period was 3.6 years (1.2 to 5.4 years).The changes in the patients' s condition were established by evaluating platelet counts,and the size of lesion.Results Common clinical features included dusky red or violaceous infiltrating cutaneous lesion,thrombocytopenia,pain or decreased function and hyperhidrosis or hypertrichosis.The following 3 clinical patterns of TA progress were described:spontaneous complete or partial regression (n =2,8.3％),TA without complications and persistence over the years(n =9,37.5％),and TA complicated by KMP(n =13,54.2％).The average interval of development of KMP for delayed cases was 45.2 days(ranged 0 d to 4 months).Each of the 13 patients who developed KMP subsequent to initial presentation was symptomatic at the time KMP was detected(enlarged lesion,n =8;increased lesion firmness with change in cutaneous stain,n =3 ;and respiratory distress,n =2).All of 13 patients were cured by surgery.Complete surgical resections were performed on 10 cases.The thrombocyte count was back to normal within 1-3 days post operation,and hemoglobin and blood coagulation function gradually returned to normal within 1 to 2 weeks.Other 3 cases received major resection surgery.The number of platelets in the patients were unstable,but significantly higher than that of preoperational stage.The platelet count remained above 60 × 109/L.The residue lesions in 2 cases disappeared gradually after the operation and medication were given within 3-6 months.And the other case died of multiple organ failure post-operation.Conclusions Surgical intervention can be applied to TA that severely makes damage to children's appearance or looks or may be complicated with KMP or functional abnormality.A closely monitored policy seems appropriate for the early small tumor without severe complications.And it is necessary to monitor the number of the platelet regularly and find the KMP by as early as possible.TA associated with KMP is vitol to infants.And surgical therapy after clear diagnosis should be done as early as possible.The surgical therapy is a reliable management with higher curative rate,short disease period and minimal side-effect.

Objective: To define a new subtype of vascular anomaly, named fibro-adipose vascular anomaly(FAVA)and to discuss the methods of diagnosis and treatment in order to decrease the misdiagnosis rate and improve the recovery rate. Methods: From Jan. 2011 to Dec. 2015, 34 cases with FAVA on the lower extremities received surgical treatment in our center. The clinical data were collected to summarize the imaging and pathological characteristics for diagnosis. Results: The misdiagnosis rate was 76.5%(26/34) in all these 34 patients. The cure rate was 100% after operation. The patients were followed-up for 1-3 years(average, 19 months) with normal function and no recurrence. Conclusions FAVA is usually misdiagnosed as hemangioma or vascular malformation. The disease should be well defined to help the diagnosis and treatment. Surgical excision is one of the optional treatments.

Objective: This study aims (1) to evaluate the surgical outcome of intramuscular venous malformations, and associated nerve and tendon encapsulated; (2) to summarize the surgical procedure, with the purpose of providing individualized surgical treatment. Methods: This is a retrospective study of 114 patients of intramuscular venous malformations with associated nerve and tendon encapsulated. All the patients were admitted in the Henan Provincial People′s Hospital from January 2012 to December 2014. The surgeries were produced, according to the patient′s condition. The surgical outcome was evaluated after 1-3 years follow up, by the overall evaluation of radiological imaging, symptoms and functions of limbs. The evaluation criteria: (1) Invalid: clinical symptoms or radiological imaging showed no improvement or even worsen; (2) Improvement: clinical symptoms or radiological imaging was significantly improved; (3) Cure: clinical symptoms were gone or the radiological imaging was normal. Results: All the 114 patients were followed up for 1-3 years, with the average of 1.8 years. Three cases were evaluated as invalid, 26 cases were improved, and 85 cases were cured. The effective rate was 97%, and the cure rate was 75% on 1 year after surgery. Conclusions It is difficult to treat intramuscular venous malformations as it often involves nerve and tendon. Universal treatment is few. Therefore, an individualized surgical plan is recommendable.

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.

To investigate the relationship between glucocorticoid receptor (GRα, GRβ) expression and hormone sensitivity in kaposiform hemangioendothelioma (KHE) patients complicated by Kasabach-Merritt phenomenon (KMP).Methods: In this study, 25 cases of KHE with KMP (mean age 2.4±1.5 months), including 9 males and 16 females at Henan Provincial People′s Hospital between May 2013 and May 2016 were included. All patients underwent surgical resection after being treated with hormone for one week, and were divided into sensitive group (10 cases) and resistance group (15 cases) according to the efficacy evaluation criteria. Normal tissues collected from 15 patients received surgical excision of lipoma was performed as a control group.Immunohistrochemical SP method was adopted in detecting GRα and GRβ expression in all groups. The expression intensity and positive rate were analyzed. Statistical significance was determined using the Wilcoxon rank sum test for the group samples and the Kruskal-Wallis test for multiple samples. Values of P<0.05 were considered statistically significant. Results: ①There was no significant difference among the control group, the resistance group and the sensitive group in expression intensity of GRα (control group 4.20±1.01, resistance group 4.10±0.99, sensitive group 3.53±0.52, P=0.632). The number of GRα positive cells in the sensitive group(46.20±2.57)was higher than that in the resistance group (40.93±5.18, P=0.032). ②The expression intensity of GRβ in the resistance group(5.40±0.51)was significantly higher than that in the sensitive group(2.60±0.52)and the control group(2.87±0.64, P=0.000, 0.002); there was significant difference among the sensitive group(29.70±2.50), the control group(36.07±3.47)and the resistance group (47.27±5.59)in the number of GRβ positive cells (P=0.000). ③The ratio of GRα/Grβ expression intensity was significantly lower in the resistance group than in the sensitive group and the control group; The ratio of GRα/GRβ positive cells was gradually decreased in the sensitive group, control group and resistance group, and the difference was statistically significant (P=0.000). Conclusions In the tumor tissue of KHE children with KMP, the increase of GRβ expression negatively regulats GRα, leading to imbalance of the expression ratio of the two, which may be an important cause for GC resistance.

Objective: To analyze the pain caused by intramuscular venous malformation, so as to avoid misdiagnosis. Methods: We retrospectively analyzed 173 patients who received surgical treatments in our department between Jan.2012 to Dec.2014, with the main complaint of local pain and were diagnosed as intramuscular venous malformation. The mechanisms of the local pain, based on the image data, intra-operative findings, pathology reports and the comparison of the Visual Analogue Scale(VAS) data before and after operation were summarized. The surgical interventions included simple excision or excision + adhesiolysis or excision + adhesiolysis + nerve decompression. Results: The reasons of local pain can be divided into 4 categories: ①lesion located in the tendon insertions; ②lesion involving the local nerve, inducing the thickening and tensing of its epineurium and the increasing of its diameter; ③lesion infiltrated to the periosteum; ④phlebolith in it. All the 173 patients received surgical treatments and got varying degrees of relieving from local pain. 63 patients got a decrease of the VAS by 5 or more, and 95 cases′ VAS number decreased by 3-4, the rest 15 patients′ VAS cut down by 1-2. Conclusions Intramuscular venous malformation is an important reason for local pain and should not be neglected. Surgical treatment can be an effective method to remove the lesion and relieve local pain.

Objective: To observe the effects of different concentrations and doses of urea on the proliferation and apoptosis of human hemangioma endothelial cells, in order to provide evidence for the further mechanism study of urea in the treatment of hemangioma. Methods: Human hemangioma endothelial cells (HemECs) and normal endothelial cells (VE) were cultured in vitro. Cell viability was detected by CCK-8 after invention with different concentrations(40%, 50%, 60%, 70%) and doses(3, 6, 9 μl/ml) of urea. The apoptosis of HemECs was detected by flow cytometry dual-dye and propidium lodide single dye. Results: The viability of HemECs was significantly lower than that of VE under different concentrations and doses of urea (P<0.05). The inhibition rate of 40% urea on HemECs increased with the increase of urea dose (P<0.05), and the inhibition effect was most obvious at 4 h and 12 h. The apoptosis of HemECs increased in a time and dosage dependent manner with the treatment of 40% urea. High dose(9 μl/ml)of 40% urea significantly promoted the apoptosis of HemECs(P<0.05). Conclusions Low dose of 40% urea significantly inhibited the proliferation of HemECs, and had no significant effect on VE. However, high doses of urea promoted apoptosis of HemECs.