Objective     To analyze the clinical efficacy and survival outcome of totally thoracoscopic redo mitral valve replacement and evaluate its efficiency and safety. Methods     The clinical data of patients with totally thoracoscopic redo mitral valve replacement in Guangdong Provincial People’s Hospital between 2013 and 2019 were retrospectively analyzed. Survival analysis was performed using the Kaplan-Meier method. Univariate and multivariate Cox regression analyses were used to determine the risk factors for postoperative death. Results     There were 48 patients including 29 females and 19 males with a median age of 53 (44, 66) years. All the procedures were performed successfully with no conversion to median sternotomy. A total of 15, 10 and 23 patients received surgeries under non-beating heart, beating heart and ventricular fibrillation, respectively. The in-hospital mortality rate was 6.25% (3/48), and the incidence of early postoperative complications was 18.75% (9/48). Thirty-five (72.92%) patients had their tracheal intubation removed within 24 hours after the operation. The 1- and 6-year survival rates were 89.50% (95%CI 81.30%-98.70%) and 82.90%(95%CI 71.50%-96.20%), respectively. Age>65 years was an independent risk factor for postoperative death (P=0.04). Conclusion     Totally thoracoscopic redo mitral valve replacement is safe and reliable, with advantages of rapid recovery, reducing blood transfusion rate, reducing postoperative complications and acceptable long-term survival rate. It is worthy of being widely popularized in the clinic.

【Subjects】 To investigate the clinical application value of myocardial contrast echocardiography （MCE） in selecting CTO-PCI patients. MethodsFrom February 2019 to March 2020， a total of 50 patients with chronic coronary artery occlusion were consecutively selected as the research subjects. MCE and two-dimensional speck-tracking echocardiography were completed before and 12 months after interventional therapy. The primary end point was major adverse cardiovascular events. Patients were divided into groups according to the preoperative myocardial perfusion level of MCE. The improvement of left ventricular function was evaluated by two-dimensional echocardiography and left ventricular global longitudinal strain. ResultsCompared with the abnormal perfusion group， the improvement of GLS in the normal perfusion group was greater （P=0.028）. The wall motion score index （WMSI） of the abnormal perfusion group before PCI was higher than that of the normal perfusion group （P=0.002）. WMSI in the abnormal perfusion group was higher than that in the normal perfusion group one year after PCI （P<0.001）. The left ventricular GLS（P=0.008）.WMSI（P=0.016） and left ventricular end-diastolic volume（P=0.032） in the normal perfusion group were improved compared with those before operation； The postoperative perfusion score of patients with abnormal perfusion was significantly improved （ P=0.032）. ConclusionMCE has clinical application value in optimizing the selection of CTO-PCI patients. CTO patients with different myocardial perfusion types have different benefits after PCI.

【Objective】 To explore the methods and safety of autologous peripheral hematopoietic stem cells collection in patients with sequential double transplantation of solid tumors and conduct efficacy analysis. 【Methods】 Peripheral blood stem cells were collected from 27 patients with solid tumors after routine mobilization of rhG-CSF and rhGM-CSF.A specific program was made for the patients.The condition and cooperation degree of children were comprehensively evaluated before cell collection, and a femoral venous catheterization was inserted to ensure the cells collected smoothly.A mononuclear cell collection(MNC) program was selected, and machine parameters were set based on the patient's low body weight.The number of mononuclear cell (MNC) and the CD34⁺ cell was detected by flow cytometry for retrospective analysis. 【Results】 A total of 73 cell collections were performed in 27 patients, and the number of mononuclear cells and CD34⁺ cells was 12.586(10.22~19.586)×10⁸/kg and 13.575(7.275~23.825)×10⁶/kg, respectively, which can meet the requirement of sequential double transplantation. No intoxication of citrate and other serious adverse reactions occurred, and the follow-up was generally in good condition. 【Conclusion】 The method is effective and safe for pediatric patients, even for pediatric patients with low weight. Sufficient stem cells can be collected for patients with solid tumors by this method to meet the requirement of sequential double transplantation.

Objective: To investigate the efficacy of haplotype hematopoietic stem cell transplantation in the treatment of acquired severe aplastic anemia (SAA) in children. Methods: The clinical characteristics of 59 pediatric patients with SAA, including 26 cases VSAA, 37males and 22 females, 47 cases typeⅠ and 12 cases typeⅡ, undrerwent haplo-HSCT in our hospital between December 1^st, 2011 and December 1^st, 2017 were retrospectively analyzed. Among 59 patients, 56 patients with a median age of 4.5 (1.2-14.8) years and median weight of 43 (12-80) kg underwent their first HSCT and 3 patients underwent their second HSCT. All patients received the following conditioning regimen: busulfan, cyclophosphamide, and rabbit ATG or Bu (–, CTX) , fludarabineand rabbit ATG. The prophylaxis of acute graft versus host disease (aGVHD) was cyclosporine (CsA) , MMF and methotrexate. All patients received bone marrow transfusion on day 01 and peripheral stem cell transfusion on day 02 from haploid donor. The median dose of donor mononuclear cell counts was 15.60 (7.74-21.04) ×10⁸/kg of recipient weight and CD34⁺ cell counts was 4.86 (3.74-7.14) ×10⁶/kg of recipient weight. Results: Neutrophils and platelets of all 59 children were implanted. The median implantation time of granulocytes and platelets were 13 (10-19) d, 19 (9-62) d, respectively. The incidence of grade Ⅰ-Ⅱ aGVHD was 45.76% (27 cases) and grade Ⅲ/Ⅳ 13.56% (8 cases) , The incidence of chronic GVHD was 8.47% (5 cases) , The incidences of CMV and EBV viremia were 59.32% (35 cases) and 28.81% (17 cases) , respectively. The median follow-up was 30 (8-80) months, 57 patients survived with disease free, 2 patients died of GVHD. Both of the estimated 5-year OS and DFS rates were (96.4±2.5) %. Conclusion Haplo-HSCT could improve the outcomes of SAA children.

Objective: To cross-cultural adapt the English version of obturator functioning scale (OFS) to form a simplified Chinese version, to preliminarily verify its reliability and validity in clinic, and to provide an effective tool for evaluating the oral function and quality of life of patients with palatal defect and restored with obturators in China. Methods: The English version of the OFS was taken for forward translation, synthesis, back-translation, and reviewed by expert committee to develop a pre-testing simplified Chinese version. This scale contained demographic data, basic information of diseases, eating problems dimensions (3 items), speech problems dimensions (5 items), and other problems dimensions (7 items). From December, 2016 to December, 2018, forty-two patients who were treated in the Department of Prosthodontics, Guanghua School of Stomatology, Hospital of Stomatology, Sun Yat-sen University with palatal defect and restored with obturators were evaluated with OFS. Among them, there were 26 males, and 21-84 years old, and 16 females, who were 24-80 years old.The reliability and validity of the data were examined and analyzed. Results: The results showed that Cronbach′s α coefficients of the overall scale and the three dimensions (eating problems, speech problems, and other problems) were 0.926, 0.938, 0.930, and 0.935, respectively. The internal consistency of the questionnaire was very good. The Spearman coefficients between each single dimension and the total score were 0.677, 0.792, and 0.860, respectively, suggesting that the scale convergence was good. The content validity index of 15 items was 0.905, indicating that the content validity was very good. Conclusions The Chinese version of the OFS is exhibiting high reliability and validity, providing an effective evaluation tool of oral function and quality of life for Chinese patients with obturator prostheses to restore palate defects.

Objective To investigate the curative effect and safety of human umbilical cord mesenchymal stem cells(UCMSCs)on the treatment of refractory acute graft versus host disease(aGVHD)of children after allogeneic hematopoietic stem cell transplantation.Methods Five children with refructory aGVHD who hospitalized at the De-partment of Pediatrics,the Navy General Hospital were treated with UCMSCs retrospectively.Among them,1 case was male and 4 cases were female,who aged from 18 months old to 15 years old.Two cases had aplastic anemia(AA),1 case with acute myeloblastic leukemia(AML-M2-CR2),1 case with acute lymphoblastic leukemia(ALL-CR1)and 1 case with myelodysplastic syndrome(MDS). Three cases received peripheral blood stem cell transplantation from HLA-matched sibling donor,1 case received mother′s peripheral blood and bone marrow stem cell transplantation from the haploid donor,and 1 case received father′s peripheral blood and bone marrow stem cell transplantation from the haploid donor(both 3/6 HLA locus matched).Prophylaxis for graft versus host disease(GVHD)was performed by using ciclosporin A and methotrexate in 1 case,others used anti-thymocyte globulin,ciclosporin A,mycophenolate mofetil and methotrexate for GVHD prophylaxis. All children developed refractory aGVHD,and they received 3-5 kinds of immunosuppressive agents to treat the refractory aGVHD,but the therapeutic effect was very poor.Then the children received UCMSCs infusion 2 or 3 times(once a week),the UCMSCs dose given was(1.5-2.0)×106per kg body weight,the curative effect and adverse reactions were apparent after infusion.Results All the children with re-fractory aGVHD were improved after treatment,the overall response was 100%,and 2 cases were healed and dis-charged,1 case suffered from relapsed of aGVHD and died,and the other 2 cases suffered from relapsed of aGVHD and died of thrombotic microvascular disease.On adverse reaction was monitored during infusion,and 2 cases had disease-free survival during 2 years follow-up,without tumour and primary disease recurrence.Conclusions UCMSCs is safe and effective for treatment of refractory aGVHD.In order to improve the curative effect and disease-free survival,the UCMSCs should be reduced early,which can reduce the application and side effects of the immunosuppressor.

This report summarized the nursing experience of caring for twice skin grafts in a patient with enteroatmospheric fistula after trauma.Keys to nursing success including:monitoring vital signs closely to prevent septic shock,blocking enteroatmospheric fistula (EAF) and sucking overflowed intestinal juice timely,promoting the protection of the graft on abdominal wall wounds,strengthing drainage and lavage with the application of abdominal double cannula to control abdominal infection,early nutrion support with parenteral nutrition in combination with trophic enteral nutrition to improve intestinal immune function,and attaching importance to post-traumatic stress disorder.Timely blocking of EAF is the bases of skin graft healing.

OBJECTIVETo study the clinical efficacy of porcine pulmonary surfactant (PS) combined with budesonide suspension intratracheal instillation in the treatment of neonatal meconium aspiration syndrome (MAS).

METHODSSeventy neonates with MAS were enrolled for a prospective study. The neonates were randomly assigned to PS alone treatment group and PS+budesonide treatment group (n=35 each). The PS alone treatment group was given PS (100 mg/kg) by intratracheal instillation. The treatment group was given budesonide suspension (0.25 mg/kg) combined with PS (100 mg/kg).

RESULTSThe rate of repeated use of PS in the PS+ budesonide group was significantly lower than that in the PS alone group 12 hours after treatment (p<0.05). The improvement of PaO/FiO, TcSaO, PaO, and PaCOin the PS+ budesonide group was significantly greater than that in the PS alone group 6, 12, and 24 hours after treatment (p<0.05). The chest X-ray examination showed that the pulmonary inflammation absorption in the PS+ budesonide group was significantly better than that in the PS alone group 48 hours after treatment (p<0.05). The incidence of complications in the PS+budesonide group was significantly lower than that in the PS alone group (p<0.05), and the average hospitalization duration was significantly shorter than that in the PS alone group (p<0.01).

CONCLUSIONSPS combined with budesonide suspension intratracheal instillation for the treatment of neonatal MAS is effective and superior to PS alone treatment.

Animals ; Budesonide ; administration & dosage ; Female ; Humans ; Infant, Newborn ; Length of Stay ; Male ; Meconium Aspiration Syndrome ; complications ; drug therapy ; Prospective Studies ; Pulmonary Surfactants ; administration & dosage ; Suspensions ; Swine ; Trachea

Objective To explore the efficacy of unrelated cord blood transplantation treatment of mucopolysaccharidosis Ⅰ (MPS Ⅰ).Methods A 4-year-and-2-month-old boy with MPS Ⅰ who received treatment of human leucocyte antigen-mismatched unrelated cord blood stem cell transplantation after diagnosis was identified.The pre-treatment regimen was Busulfan + Cyclophosphamide + Fludarabine (Bu/Cy4 + Flud).Bu with the dosage of 1.2 mg/kg,once every 6 hours,4 days;Cy with the dosage of 50 mg/(kg · d) for 4 days and Flud with the dosage of 30 mg/(m2 · d) lasted for 4 days,respectively.The day that the graft was transplanted was defined as 0 day,days betore transplantation as negative days,days after transplantation as positive days.After pre-treatment,4.60 × 107/kg of cord blood nucleated cells and 3.05 × 105/kg CD34 positive cells were transplanted into the child.The combination of Antihuman thymocyte globulin,Cyclosporin A and Mycophenolate mofetil was administrated for prophylaxis of graft versus host disease(GVHD).After transplantation,the patient was given granulocyte colony stimulating factor to promote reconstitution of hematopoiesis.Results The myeloid and platelet engraftment time was respectively 15 days and 24 days after transplantation.Short tandem repeat (STR) DNA fingerprinting showed a full donor chimerism on day 21 after transplantation,and the full donor chimerism was stable afterwards.The peripheral-blood α-L-iduronidase (IDUA) activity returned to the normal value,and the IDUA gene sequencing did not demonstrate any mutation in 83 days after transplantation.On day 12 after transplantation,pulmonary infection with pulmonary hypertension occurred.Grade-Ⅱ acute intestinal GVHD occurred on day 15,Grade-Ⅱ acute cutaneous GVHD on day 51,and chronic GVHD (cutaneous,localized) on day 180.Otherwise,the patient complicated with hemorrhagic cystitis on day 35.These complications was cured favourably.In an 18-month-follow-up,the height of the boy increased by 3 cm,and his body weight had increased by 2.4 kg.His corneas regained clear,and his hepatosplenomegaly disappeared.The glycosaminoglycan of urine was negative.The neurocognitive performance of the boy had a little improvement.The abnormalities of fingers and other skeletons had no marked change.Conclusions Unrelated cord blood transplantation for MPS Ⅰ have definited effect.It is the first case report in China on treatment of MPS Ⅰ by unrelated cord blood transplantation.The researchers have accumulated some preliminary experience for future treatment of MPS Ⅰ by unrelated cord blood transplantation.

OBJECTIVETo explore the efficacy of unrelated umbilical cord blood transplantation (UCBT) in the treatment of Gaucher disease.

METHODThe clinical characteristics of three children with Gaucher disease underwent UCBT in our hospital between April 2013 and September 2014 were retrospectively analyzed. Literature on allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of Gaucher disease was searched at Wanfang and Pubmed databases between 1983 and 2015 and was reviewed and summaried.

RESULTThree children with Gaucher disease, all were female, received UCBT. These patients' age at receiving transplantation was 3.8 years, 7.1 years and 2.6 years, respectively. The second case received the second transplantation. The first and third case received splenectomy before UCBT. The pretreatment regimen was busulfan (Bu)/fludarabine (Flu)/cyclophosphamide (CTX)/antithymocyte globulin (ATG), and for the patient received the second transplantation melphalan was added to the myeloablative conditioning regimen of Bu/Flu/CTX/ATG. Cyclosporine and mycophenolate mofetil (MMF) wee used for prophylaxis of acute graft versus host disease (aGVHD). The dose of cord blood stem cell nucleated cell counts was 9.7 × 10⁷ /kg,11.9 × 10⁷ /kg and 7.6 × 10⁷/kg respectively. The dose of cord blood stem cell CD34⁺ cell counts was 5.4 × 10⁵/kg , 3.5 × 10⁵/kg and 3.2 × 10⁵/kg respectively. The day of granulocytes exceeding 0.5 × 10⁹/L was day 11, 12 and 19 after transplantation, respectively. The day of platelets exceeding 20 × 10⁹/L was day 14, 33 and 74 after transplantation, respectively. At one month after transplantation the rate of chimerism was over 95% and all patients got donor complete chimerism. The level of β-glucocerebrosidase recovered to normal at one month after transplantation. During transplantation, all patients developed cytomegalovirus (CMV) and Epstein-Barr virus (EBV) viremia. In case 1 immune thrombocytopenia occurred at five month after transplantation unresponding to steroids and mesenchymal stem cells infusion was administered and his platelet in routine blood test recovered to normal. But the patient died because she was infected with varicella-zoster virus out of hospital at nine month after transplantation and the level of β-glucocerebrosidase was normal before death and chronic GVHD (cGVHD) was not found. The case 2 is now in 19th month after transplantation and his level of β-glucocerebrosidase was normal. cGVHD was not found. The patient is currently free of disease. The case 3 was in 9th month after transplantation and his level of β-glucocerebrosidase was normal. cGVHD was found at 112 day after transplantation and was localized and could be controlled by hormonal therapy. The patient is currently free of disease. Three patients' size of liver was significantly reduced after their level of β-glucocerebrosidase ecovered. There were 50 cases with Gaucher disease who were treated with allo-HSCT in the literature and none of them were reported from China. Disease-free survival rate of patients treated with allo-HSCT for Gaucher disease was 85%. In all reports, there were 31 cases who had information of typing of Gaucher disease, of whom 22 cases had type 1 and 9 cases had type 3. Twenty-nine cases had information of survival, of whom 24 cases survived and 5 cases died of infection. Fifteen cases had data of engraftment, 2 of whom had graft failure and one had late graft failure.Glucocerebrosidase recovered to normal in 25 of 31 cases who had relevant data, in one of whom with late graft failure the enzyme recovered to normal 3 month after transplantation, but his enzyme decreased to the initial level 9 month after transplantation. Along with enzyme level's recovery to normal, in a part of cases bone pain and hepatomegaly were relieved and growth delay was improved.

CONCLUSIONThe unrelated UCBT may be a form of treatment that offers the potential of permanent cure and a procedure with possible long-term benefits in patients with Gaucher disease.

Antilymphocyte Serum ; therapeutic use ; Busulfan ; therapeutic use ; Child ; Child, Preschool ; China ; Cyclophosphamide ; therapeutic use ; Cyclosporine ; therapeutic use ; Disease-Free Survival ; Female ; Gaucher Disease ; therapy ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Mycophenolic Acid ; analogs & derivatives ; therapeutic use ; Retrospective Studies ; Transplantation Conditioning ; Vidarabine ; analogs & derivatives ; therapeutic use