Tufted angioma (TA) is a rare vascular endothelial cell tumor that often occurs in infants and early childhood and is rare in adults. There are currently no unified treatment guidelines, and treatment options for children with TA are limited and challenging. This report introduces a case of a child diagnosed with superficial TA in the left popliteal fossa. Topical 0.1% sirolimus cream was given in the early stage and combined with pulsed dye laser treatment in the later stage. The child’s skin lesions recovered well. The author reviews relevant literature reports to summarize the treatment of TA in children and provide relevant ideas and new options for the diagnosis and treatment of the disease.

Tufted angioma (TA) is a rare vascular endothelial cell tumor that often occurs in infants and early childhood and is rare in adults. There are currently no unified treatment guidelines, and treatment options for children with TA are limited and challenging. This report introduces a case of a child diagnosed with superficial TA in the left popliteal fossa. Topical 0.1% sirolimus cream was given in the early stage and combined with pulsed dye laser treatment in the later stage. The child’s skin lesions recovered well. The author reviews relevant literature reports to summarize the treatment of TA in children and provide relevant ideas and new options for the diagnosis and treatment of the disease.

Objective:To evaluate the clinical efficacy and safety of a cream containing madecassoside, 5% panthenolon skin repairing after nonablative fractional 1 565 nm laser therapy.Methods:A total of 84 patients who received nonablative fractional laser surgery in our hospital from April 2017 to April 2018 were included as research objects. The patients were divided into observation group and control group by random number table method, 42 cases in each group. The control group was treated with routine facial intervention after operation, while the observation group was treated with a cream containing madecassoside, 5% panthenolon skin repairing on the basis of routine intervention. The postoperative skin barrier function of the two groups were recorded and compared through skin property system and VISIA complexion analysis system.Results:At 1 and 2 weeks after operation, the sebum content and cuticle water content in the observation group were higher than those in the control group, and the transdermal water loss in the observation group was less than that in the control group, and the differences were statistically significant ( t=4.927, 7.833, 12.430, 4.538, 10.083, 8.017, P<0.05). The erythema index (EI) and melanin index (MI) of the observation group were lower than those of the control group at 1 and 2 weeks after operation, and the incidence of complications was significantly lower than that of the control group ( t=2.392, 2.807, 3.485, 3.009, P<0.05). Conclusions:The application of a cream containing madecassoside, 5% panthenolon in skin repairing is helpful to enhance the moisturizing effect, reduce the complications and promote the early recovery of patients with skin trauma after fractional laser operation.

Objective:To improve the understanding of adult primary hemophagocytic syndrome (HPS) with aggressive natural killer cell leukemia (ANKL).Methods:The clinicopathological data of one adult patient with suspected primary HPS complicated with ANKL in Huiqiao Medical Center, Nanfang Hospital of Southern Medical University in October 2017 were retrospectively analyzed, and literatures were reviewed.Results:A 21-year-old male patient presented with persistent fever, hemocytopenia, splenomegaly, low fibrinogen, a significant increase in ferritin, hemophagocytes in bone marrow, decreased natural killer (NK) cell activity, and increased soluble CD25. Flow cytometry detection showed that the expression of NK cells was abnormal, and there were familial lysosomal trafficking regulator (LYST) and UNC13D gene defects. He was suspected of primary HPS complicated with ANKL. The patient was given 4 courses of EPOCH+PEG-Asp (etoposide, dexamethasone, vindesine, cyclophosphamide, doxorubicin hydrochloride liposome, pegaspargase) regimen chemotherapy, 20 mg of citalopidine twice a week maintenance therapy and matched unrelated hematopoietic stem cell transplantation. After 35 months of follow-up, he got sustained remission.Conclusions:Even if there are secondary causes of adult HPS, it is necessary to screen out related genes to avoid misdiagnosis. HPS patients with ANKL progress rapidly, and the early mortality is high. EPOCH+ PEG-Asp regimen induction therapy and allogeneic hematopoietic stem cell transplantation should be used as early as possible after diagnosis.

Objective:To build a medical service experience system based on role play of hospital staff, and explore its contribution to upgrading medical service, elevating medical service quality, and improving patients′ satisfaction.Methods:An indicator system for medical service experience was developed via literature review. 20 newly recruited hospital employees in 2019 were sampled randomly to form a service experience team, who were arranged to experience the full medical service process of outpatients and inpatients based on role play. The questionnaire for service quality based on patient perception and expectation was used to survey the expectation and perception values of those role players on their medical service experiences, and summarize the defects found on such six dimensions as hospital reliability, responsiveness, tangibility, assurance, empathy and cost-effectiveness. After due corrections of these defects, further experiences and patient satisfaction survey were made to assess the effects of such actions taken. Paired t test was used respectively in statistical analysis of service experience value and actual perception value, as well as the perception values before and after the actions taken, and χ2 test was used for a statistical analysis of patient satisfaction. Results:Role players experienced respectively at the emergency, outpatient and inpatient departments of the hospital for a week in August 2019. Statistics of their experiences indicated≥0.50 points difference between the mean expectation and perception values on doctor-patient communication, patient help responsiveness, outpatient process, examination report delivery duration, service attitude, patient trust on medical workers, medical environment and patient privacy protection.Following a 3-month reform, role players found improvements in such aspects as hospital reliability, responsiveness, tangibility, assurance, empathy and cost-effectiveness; patient satisfaction improved sizably in December 2019 over January of the same year( P<0.05). Conclusions:The construction and application of the medical service experience system based on role play of hospital staff prove highly useful in upgrading medical service actions, improving medical service quality, and improving patient satisfaction.

Objective To analyze the treatment and follow-up of congenital coronary artery fistula (CAF) with giant coronary artery aneurysm (GCAA) in children.Methods The clinical data were analyzed retrospectively in 13 patients who were diagnosed as congenital CAF with GCAA between July 2009 and December 2016 in Guangzhou Women and Children's Medical Center.There were 8 boys and 5 girls.The median age was 18 months,ranging from 40 days to 12 years old.The body weight ranged from 3.8 kg to 29.0 kg with a median of 8.8 kg.Fistulas originated from right coronary artery accounted in 8 patients,with 5 from left coronary artery.Fistulas drained into right atrium in 3 patients,right ventricular in 8 patients and left ventricular in 2 patients.Single fistula occurred in 12 patients and multiple fistulas in 1 patient.The diameter of coronary artery aneurysm ranged from 8 mm to 16 mm with a median of 9.2 mm.Results One patient had tachypnea and growth retardation without heart murmur.The other 12 patients were asymptomatic with heart murmurs occasionally found in routine physical examination.One patient underwent fistula ligation without cardiopulmonary bypass (CPB).The remaining 12 cases received fistula correction with beating heart CPB.Direct suture was used in 10 patients and autologous pericardial patch in other 2 patients.Two patients were associated with atrial septal defect (ASD) and underwent repair of ASD concurrently.The coronary artery aneurysm remained original shape without any intervention during the operation.The mean hospital delay was (11.0 ± 2.5) days.Two patients had decreased ejection fraction as low as 38％ within 3 days after the operation,but went up to over 50％ in follow-up 1 month later.Transient T wave change occurred in 7 patients,and another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.All 13 patients had antiplatelet therapy with 12 taking Aspirin and one taking Dipyridamole.The dosage was 3-5 mg/(kg · d) with duration ranging from 3 days to 13 months with a median of 1 month.During the perioperative period and the follow-up period (3 months to 8 years),all patients were asymptomatically alive.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was not changed after the operation.There was no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusions GCAA can be combined with congenital CAF in children,so it needs early operation.The evidence-based intervention of coronary artery aneurysm and usage of anticoagulant and antiplatelet therapy in pediatric patients was still lacking,which needs long-term follow-up.

Objective To summarize the outcomes and experience of arterial switch operation (ASO) in the past 10 years in our center.Methods From September 2008 to July 2017,238 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.There were 193 male and 45 female.Median age at operation was 2 months (2 days to 10 years) and mean body weight was(4.2 ± 1.7) kg (1.8-20.6 kg).There were TGA and VSD 91 cases,TGA/IVS 110 cases,and Taussig-Bing anomaly 37 cases.Among them 24 patients had an aortic arch anomaly.Intramural coronary artery was found in 8 patients.Results All patients successfully completed the operation,one-stage ASO was perfonned in 232 patients.Two-stage ASO was performed in 6 patients.The mortality was 14.2％.The follow-up duration was 1-10 years (median time,46 mouths).There were 3 died.Two suffered sudden death,and another one arrhythmia.10-year survival rate was 92.8％.Conclusion The outcomes of ASO were satisfactory.The long-term reoperation rate was rare.

Objective: To summarize the surgical results and experience of patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets. Methods: From April 2013 to June 2017, 10 patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets underwent biventricular repair at Guangzhou Women and Children Medical Center. Seven were male, and 3 were female. The age and body weight at surgery was 2 months to 13 years and 3.7-23.6 kg. Repair was performed with modified one-patch technique in 3 patients, modified two-patch technique in 6 patients, two-patch technique in 1 patient. Results: There was no hospital mortality. The ICU stay and hospital stay after operation were 2~5 days and 7~10 days. The follow-up duration was 16 to 65 months. All patients were alive and free from left ventricular outlet obstruction. The left atrioventricular valve function were normal in 2 patients, mild regurgitation in 6 patients, moderate regurgitation in 1 patient and severe regurgitation in 1 patient. Conclusion The outcomes of biventricular repair for patients with complete atrioventricular septal defect associated with Tetralogy of Fallot or double right ventricular outlets were satisfied, and long-term follow-up was demanded.

Objective Analyze the surgical and follow-up outcomes of congenital coronary artery fistula(CAF) in children and to discuss the treatment methods.Methods The clinical data were analyzed retrospectively in 22 patients who were diagnosed as congenital CAF and received surgical treatment between July 2008 and January 2017 in Guangzhou Women and Children's Medical Center.There were 14 boys and 8 girls.The median age was 17 months ranging from 14 days to 12 years old.The median weight was 8.8 kilograms ranging from 3.0 to 31.0 kilograms.Results Two patients had shortness of breath.Three had growth retardation with recurrent respiratory infection or tachypnea.The remaining 17 patients were asymptomatic.Twenty had heart murmurs.Fistulas originated from right coronary artery accounted for 11 patients,with 10 from left coronary artery,1 from both right and left coronary arteries.Fistulas drained into coronary sinus in 1 patient,right atrium in 6 patients,right ventricular in 13 patients and left ventricular in 2 patients.Single fistula occurred in 20 patients and multiple fistulas in 2 patients.The coronary arteries were obviously dilated in all patients with diameter from 4.0 to 12.0 millimeters.There were 8 cases with aneurysmal dilation of coronary arteries.Two patients underwent fistula ligation without cardiopulmonary bypass(CPB).The remaining 20 cases received fistula correction with beating heart CPB.Direct suture was used in 3 patients for closure of fistula inlet and 11 children for outlet.Both inlet and outlet were closed in 6 patients,and in 2 of them autologous pericardial patch was used for outlet.Other associated intra-cardiac anomalies in 7 children were corrected during the operation.The aneurysmal coronary arteries remained original shape without any intervention during the operation.The mean hospital delay was(12.3±3.2) days.Two patients appeared decreased heart function within 3 days after the operation,and recovered in return visit one month later.Another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.During the perioperative period and the follow-up period(3 months to 8 years),all 22 patients were asymptomatically alive without regular anticoagulation management.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was reduced over 6 months follow up after the operation.There were no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusion Clinical symptoms can be appeared in children with congenital CAF due to large shunt.Surgical correction is an effective method for the management of single CAF or CAF with coexisted intra-cardiac anomalies.The outcome was well.

Objective To summarize the revascularization surgery results of patients with unilateral absence of a pulmonary artery.Methods From November 2014 to July 2016,5 patients with unilateral absence of a pulmonary artery underwent surgical revascularization at Guangzhou Women and Children Medical Center.The age and body weight at surgery was 1-6 months and 3.9-5.8 kg.One patient was diagnosed with unilateral absence of left pulmonary artery.The other 4 patients were diagnosed with unilateral absence of right pulmonary artery.Three patients were diagnosed with isolated unilateral absence of a pulmonary artery.One was associated with Tetralogy of Fallot.One was associated with atrial septal defect.Revascularization was performed by connection of hilar artery and the main pulmonary artery,using direct anastomosis,Gore-Tex conduit or pericardial conduit.Results There was no mortality.The ICU stay after operation were 3-14 days.The follow-up duration was 14-34 months.All patients were alive and free from significant pulmonary stenosis.Conclusion Revascularization surgery for patients with unilateral absence of a pulmonary artery is effective and achieved satisfactory results.