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 To explore the therapeutic effect and the efficacy of external carotid artery catheterization and drug infusion therapy for severe infant hemangiomas in oral and maxillofacial regions.Methods The clinical and follow-up data of 126 cases with severe infant oral and maxillofacial hemangiomas were retrospectively analyzed in Henan Provincial People's Hospital between June 2004 and December 2009.The patients included 45 male and 81 female cases,and the average visiting age of the patients was 3.9 months (ranged 10 days to 1 year and 4 months).Among 126 patients,24 cases occurred with Kasabach-Merritte syndrome (KMS).The patients were treated with ligation of external carotid artery,insertion of a tube inside the lesion and injection of 400 g/L Carbonyldiamide after operation.Methylprednisolone was added in infusion if the patients were combined with KMS.Results The mean follow-up period was 6.1 years (ranged 4.3 to 10.2 years).All of 126 cases were cured through the management including external carotid artery catheterization and drug infusion therapy in 94 cases,and the combination treatment of the infusion and local Carbonyldiamide injection in 32 cases.The size of hemangiomas gradually shrank and disappeared after the therapy.The hemangiomas in 105 cases disappeared completely in 6-12 months,and 21 cases disappeared in 1-2 years.Twenty-four patients were complicated with KMS.The thrombocyte count and blood coagulation function also returned to normal after the therapy.Among the 24 cases,the thrombocyte count in 13 cases was back to normal within 3-7 days,and 7 cases within 8-14 days.In the remaining 4 cases,it normalized within 15-30 days.The weight,height,immunity of the patients were in keeping with the healthy counterparts after long term follow-up.And no serious adverse effect was observed.Conclusions For severe infant hemangiomas in oral and maxillofacial regions,the external carotid artery catheterization and drug infusion therapy can obviously shorten the treatment time,and reduce the drug side effects.It is a better and more reliable treatment method.

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 assess the psychometric properties of the Chinese Heartburn Version of Quality of Life in Reflux and Dyspepsia Questionnaire (QOLRAD) in patients with gastroesophageal reflux disease.Methods 130 patients with symptoms of heartburn completed the Chinese version of QOLRAD,the Short-Form-36 (SF-36).30 of them received proton pump inhibitors (PPI) for 8 weeks,which was used to test responsiveness of the Chinese heartburn version of QOLRAD.Results The Chinese version of QOLRAD had acceptable internal consistency.The overall Cronbach's alpha was 0.89 and the internal consistency of dimensions ranged from 0.70～0.90.Content validity index (CVI) was 0.82.Confirmation factor analysis revealed a 5 factor solutions accounting for 62.02％ and most of items in their dimensions had acceptable loads (＞0.4).There was acceptable concurrent validity with correlations between the Chinese heartburn version of QOLRAD and Short Form-36 health survey ranging from 0.172～0.613.As to responsiveness,after therapy of PPI for 8 weeks,except the dimension of sleep disturbance,scores for dimensions of vitality,food/drink problems,physical/social functioning,emotional distress had significant changes.Conclusions The Chinese version of QOLRAD has a good reliability,validity and responsiveness to therapy,which can be used to assess the quality of life in patients with gastroesophageal reflux disease.

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.

This study was aimed to compare and evaluate the diagnostic performance of non-contrast-enhanced MR angiography (NCE-MRA) with contrast-enhanced MR angiography (CE-MRA) in the anatomic assessment of hepatic portal vein. Thirty people, ten patients with hepatic cirrhosis without ascites and twenty normal physical examination donors as control group were included in the NCE-MRA and CE-MRA with the same 1.5T MR scanner. Anatomic angiographic images were reconstructed and their datasets available for analysis independently performed by two radiologists. Assessment of data quality of hepatic portal vein vessels was rated with a four-point scale. After consensus reading, a total 27 images (90%) scored more than 3 point were observed in NCE-MRA and 28 (93.3%) in CE-MRA, respectively. Segmental branch vessels were visualized on MR angiography in the majority of cases. Both NCE-MRA and CE-MRA correctly characterized the hepatic portal veins with grade 5 and without false positive cases. Only 4 false negatives with grade 6 were missed in NCE-MRA group. There were no statistically significant differences between NCE-MRA and CE-MRA for characterization of hepatic vasculature (P < 0.05). Kappa value was larger than 0.75 for both reviewers. A conclusion could be drawn that NCE-MRA is a non-invasive and effective method that provides a comprehensive assessment of the hepatic portal vein.
Adult ; Aged ; Contrast Media ; Female ; Humans ; Liver Cirrhosis ; pathology ; Magnetic Resonance Angiography ; methods ; Male ; Middle Aged ; Portal Vein ; pathology ; Young Adult

Objective:To analyze efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon (KMP) .Methods:Clinical data were collected from 18 patients with KMP who underwent surgical treatment in Department of Hemangioma, Henan Provincial People′s Hospital from November 2017 to March 2019, and analyzed retrospectively. Preoperative treatment included preoperative use of glucocorticoids and immunoglobulins to increase platelet counts in patients. For patients irresponsive to glucocorticoids, a single large-dose of platelets was infused 12-24 hours before surgery. The platelet dose for infusion should be 0.3 and 0.2 therapeutic dose (TD) /kg respectively for patients with platelet counts lower than 30 × 10 9/L and those with platelet counts higher than 30 × 10 9/L, and the total infusion dose should be no more than 1 TD (containing about 2.5 × 10 11 platelets in 200-250 ml of infusion solution) . During the operation, tumor tissues were removed as much as possible; if there were difficulties in suturing in patients with large tumors, in situ autologous skin grafting would be performed; for children with potential postoperative functional dysfunction, a clinical consultation with rehabilitation specialists was given, and individualized functional exercises were prescribed to promote functional restoration. Results:Eighteen patients were enrolled, including 9 males and 9 females, with an average age of 73 days (range, 7-354 days) . Skin lesions were located on the extremities in 3 cases, on the trunk in 11, and on the head, face and neck in 4. Surgeries were successful in 17 patients, but 1 died. Among the 17 patients with successful operation, platelet counts and coagulation function returned to normal within 1 week after the surgery in 16, and did not returned to normal until after regular oral administration of sirolimus in 1. Favorable movement ability was maintained in all the children.Conclusion:Comprehensive surgical treatment shows rapid efficacy with less adverse reactions in the therapy of KMP.

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.

PHACE syndrome is a syndrome of multiple organ and multisystem abnormalities associated with infantile segmental hemangioma, characterized by abnormal posterior fossa development, infant hemangioma, aortic abnormalities, aortic coarctation and heart defects, eye anomalies and other symptoms. The incidence of the disease is low, but there exist life-threatening factors. Once clinically diagnosed, it should be highly valued and multidisciplinary consultation must be conducted. This article reviews the diagnostic criteria of PHACE syndrome and its associated facial segmental hemangioma, as well as the treatment and prognosis of brain abnormalities.

Objective: To explore the feasibility of application of multi-point puncture immunohistochemical to determine the invasion extent of the epidermis in Kaposiform hemangioendothelioma before surgery and choose the surgical method. Methods: From July 2013 to April 2016, 17 patients with Kaposiform hemangioendothelioma were admitted to the People′s Hospital of Zhengzhou University, including 7 males and 10 females. The first consultation age was 75.60±31.55 days. Preoperative multi-point pathological puncture D2-40, CD31, CD34 immunohistochemical staining was to test the invasion extent of the epidermis in Kaposiform hemangioendothelioma and to determine whether undergo autologous skin grafting. Skin graft survival areas were recorded postoperatively. Platelet values were examined after 1 week, 4 weeks, 12 weeks and 24 weeks. Results: In these 17 cases, 15 cases with epidermis and dermis unaffected was performed in situ grafting. 2 cases with the tumor invasion, superficial skin grafting were repaired. In these 15 cases of in situ grafting, platelet values returned to normal at 1 week after the operations, and platelet values were higher than 100×10⁹/L at 4 weeks, 12 weeks and 24 weeks. Among them, 12 cases of epidermal survival area were greater than 90%, 2 cases were 70%-90%, and 1 cases was about 50%. Conclusions During the complete resection of Kaposiform hemangioendothelioma, the multi-puncture D2-40, CD31 and CD34 staining are used to determine invasion extent of the tumor to the skin, which can maximize the retention of normal tissue surface, reduce postoperative complications. This is a new multi-disciplinary surgical approach.