Objective:To assess health-related quality of life and disease burden of Chinese Phenylketonuria(PKU)patients and inform optimized management and support strategies.Methods:A cross-sectional survey is conducted to explore questionnaires.The Delphi method was applied to form a standardized questionnaire.Results:A total of 263 valid questionnaires were collected.The average patient age was 7.6 years.Younger patients reported better quality of life.Mean total medical cost per patient was 238 461.9 yuan,exceeding the 2024 national per capita disposable income.Conclusion:PKU patients are facing significant challenges in both quality of life and economic burden.

Objective:To assess health-related quality of life and disease burden of Chinese Phenylketonuria(PKU)patients and inform optimized management and support strategies.Methods:A cross-sectional survey is conducted to explore questionnaires.The Delphi method was applied to form a standardized questionnaire.Results:A total of 263 valid questionnaires were collected.The average patient age was 7.6 years.Younger patients reported better quality of life.Mean total medical cost per patient was 238 461.9 yuan,exceeding the 2024 national per capita disposable income.Conclusion:PKU patients are facing significant challenges in both quality of life and economic burden.

Objective To observe the effect of transcatheter thrombectomy combined with catheter-directed thrombolysis(CDT)for treating acute medium-high and high risk pulmonary thromboembolism(PTE).Methods After placement of inferior vena cava filter,transcatheter thrombectomy combined with CDT were performed in 28 patients with acute medium-high or high risk PTE.After treatment,clinical symptoms improved or not was assessed,and interventional related complications were recorded.The outcomes of arterial blood gas analysis,coagulation function,blood routine test,pulmonary artery pressure(PAP)and right ventricular diameter/left ventricular diameter(RV/LV)were compared before and 72 h after treatment.Regular follow-up was performed,then PAP and the clearance of pulmonary arterial thrombosis were observed 1,3,6 months and 1 year after treatment during follow-up.Results Among 28 cases,significant improvement of clinical symptoms achieved in 26 cases after treatment,while 2 patients died of respiratory failure.Puncture site bleeding occurred in 4 cases and improved after conservative treatment.Compared with those before treatment,among 26 survived patients,blood pH,arterial oxygen pressure,fibrin degradation products and D-dimer increased while the heart rate,N-terminal pro-B-type natriuretic peptide,PAP and RV/LV decreased 72 h after treatment(all P＜0.05).During follow-up,compared with those before treatment,PAP decreased,while the clearance rate of pulmonary thrombosis increased 1,3,6 months and 1 year after treatment(all P＜0.05).No active bleeding nor recurrence of PTE happened.Conclusion Transcatheter thrombectomy combined with CDT was safe and effective for treating acute medium-high and high risk PTE.

Natural degeneration or trauma of articular cartilage all can lead to its structural and functional damage. Without blood supply and nerve innervation, chondrocytes in the matrix lacunae obtain essential nutrients and excrete metabolites mainly through osmosis, finally leads to its low metabolic activity and difficulty in self-repair after injury. At present, drug conservative treatment and surgical operation are the main clinical treatment, but both of them can't meet the clinical needs well. The development of cartilage tissue engineering provides a new direction for the repair of articular cartilage injury, in which growth factors plays a very important role. Growth factors, together with seed cells and cell scaffolds, constitute the three elements for the construction of tissue-engineered cartilage. Among them, Growth factors can significantly promote cell proliferation and differentiation and induce their functions. Various growth factors synergistically mediate the differentiation of seed cells into chondrocytes. In recent years, stem cell cartilage tissue engineering developed rapidly, which has opened a new way for repair of articular cartilage damage due to its abundant cell resources, small damage to body itself, strong ability of proliferation and directional differentiation, biological repair and other prominent advantages. Different types of hydrogels and stem cells show different abilities to support chondrogenesis and require different growth factors to induce chondrocyte differentiation. Traditional growth factors for tissue engineering include transcription growth factor β, insulin-like growth factors, bone morphogenetic proteins, fibroblast growth factors and cartilage derived morphogenetic protein. Recently, some scholars found that platelet-rich plasma, platelet-rich fibrin, Kartogenin and Mechano-growth factor can also effectively induce chondrogenic differentiation of stem cells and maintain chondrocyte phenotype. In addition, some synthetic compounds such as dexamethasone and inorganic particles can also promote the differentiation of stem cells into cartilage. This article systematically summarized the new progress of the traditional growth factors, emphatically introduced the new discovered growth factors and some synthetic compounds and inorganic particles, which can induce stem cells into cartilage. Finally classified the different sources of stem cells and its suitable growth factors, and gave an outlook of the next research direction of growth factors.

BACKGROUND: Hyaluronan-methylcellulose hydrogel cannot only be conjugated with short peptide sequences and growth factors to achieve sustained release, but also has a role in blocking dural defects and reducing inflammation. It is an ideal biomaterial for the treatment of spinal cord injury. OBJECTIVE: To investigate the effect of neurotrophin-3 modified hyaluronan-methylcellulose (HAMC-NT-3) hydrogel on the recovery of neurological function in rats with spinal cord injury. METHODS: Fifty-four female Sprague-Dawley rats (provided by the Experimental Animal Center of the Academy of Military Medical Sciences in China) were randomly divided into three groups (n=18 per group) . The sham group only underwent T10 laminectomy. In the model group and the experimental group, an aneurysm clip was used to establish spinal cord injury models after T10 laminectomy. The experimental group was locally injected with HAMC-NT-3 hydrogel. The Basso Beattie Bresnahan function scoring was performed at 1 day, 1, 2, 3, 4, 5, 6, 7, and 8 weeks after surgery. The inclined plane test was performed at 4, 6 and 8 weeks after surgery to evaluate the recovery of hindlimb motor function. ELISA was used to detect the concentrations of inflammatory factors in the spinal cord at 1 week after surgery. Immunohistochemical staining was used to observe the area of syringomyelia, glial fibrillary acidic protein expression and nerve regeneration at 8 weeks after surgery. RESULTS AND CONCLUSION: (1) The Basso Beattie Bresnahan scores of the model group and the experimental group were lower than those of the sham group at various time points after surgery (P < 0.05) . The Basso Beattie Bresnahan scores of the experimental group were higher than those of the model group at 4-8 weeks after surgery (P < 0.05) . (2) In the inclined plane test, the maximum inclined angles of the model group and the experimental group at each time point after surgery were lower than that of the sham group (P < 0.05) . The maximum inclined angles of the experimental group at 6 and 8 weeks after surgery were higher than those of the sham group (P < 0.05) . (3) The concentrations of tumor necrosis factor-α, interleukin-1β, interleukin-6 and interleukin-10 in the experimental group and the model group were higher than those in the sham group (P < 0.05) . The concentrations of tumor necrosis factor-α, interleukin-1β and interleukin-6 in the experimental group were lower than those in the model group (P < 0.05) . The concentration of interleukin-10 in the experimental group was higher than that in the model group (P < 0.05) . (4) Immunohistochemical staining showed that the expression levels of glial fibrillary acidic protein in the experimental group and the model group were higher than those in the sham group, while the expression of glial fibrillary acidic protein in the experimental group was lower than that in the model group. The area of syringomyelia in the experimental group was smaller than that in the model group (P < 0.05) . These results indicate that local injection of HAMC-NT-3 hydrogel can effectively inhibit inflammation as well as astrocyte activation and proliferation, reduce fibrous scar formation, and promote the protection of nerve tissue and the recovery of hindlimb motor function after spinal cord injury.

Objective To investigate the effect of acupuncture combined with hyperbaric oxygen therapy on neurological function and daily living ability of patients with cerebral hemorrhage. Methods From January 2014 to June 2017,one hundred and twenty-three patients with cerebral hemorrhage which hospitalized in the Department of Neurosurgery of Hebei Provincial Corps Hospital were selected and randomly divided into three groups: acupuncture group, acupuncture + hyperbaric oxygen group ( combined group ) and control group. The acupuncture group was given acupuncture on the basis of routine medical treatment after minimally invasive surgery treatment; the combined group was treated with acupuncture treatment as well as hyperbaric oxygen therapy after minimally invasive surgery; the control group was only treated with conventional medical therapy without any special treatment. The scores of nerve function defect,Fusl-Meyer motor function assessment scale,daily living ability score,cognitive function score,Glasgow coma scale at the 40th day of admission were evaluated and compared respectively,and the clinical effect were evaluated as well. Results After treatment, the NHISS score of the control group,the acupuncture group and the combined group decreased in turn. The score of Fusl-Meyer exercise function rating scale, the daily living ability score, the cognitive function score and the Glasgow coma scale were increased in turn, and the differences were statistically significant ( F=276. 620, 76. 050,31. 770,116. 598,33. 745,P<0. 01) . The total effective rate of the control group,acupuncture group and combined group increased sequentially (61％,76％,90％),and the difference was statistically significant (χ2=18. 547,P<0. 01) . Conclusion Acupuncture therapy for patients with cerebral hemorrhage can effectively promote the improvement of nerve function, daily living ability and prognosis. Acupuncture combined with hyperbaric oxygen can get more obvious therapeutic effect.

Objective To report the clinical efficacy of Kirschner wire combined with external fixator in the treatment of open comminuted distal tibiofibular fractures according to the concept of damage control orthopaedics.Methods A case series study was done on the clinical data of 15 open comminuted distal tibiofibular fractures which had been treated with kirschner wire combined with external fixation from January 2015 to August 2018 at Department of Orthopedics,Affiliated Hospital to Logistics College of Chinese People's Armed Police.They were 12 men and 3 women,aged from 27 to 62 years (mean,46.5 years).By the Gustilo classification,there were one case of type Ⅰ,4 cases of type Ⅱ,7 cases of type Ⅲ A,2 cases of type ⅢB and one case of type ⅢC.All the patients were treated with emergency debridement,tibial fixation using external fixator and fibular fixation using kirschner wire,followed by vacuum sealing drainage(VSD).Effective anti-inflammatory and other comprehensive treatments were given postoperatively.Regular follow-up was conducted to observe fracture healing and complications like osteomyelitis and bone disconnection.At the final follow-up,the American Orthopaedic Foot Ankle Society (AOFAS) ankle-hindfoot scale was used to evaluate the ankle function.Results All the patients were followed up for 12 to 18 months (mean,12.8 months).Primary bone union was achieved in 13 cases (86.7％),delayed healing observed in one case (6.7％) and bone nonunion in one case (6.7％).No osteomyelitis occurred.By the AOFAS ankle-hindfoot scale,the ankle function was rated as excellent in 9 cases,as good in 4,as fair in one and as poor in one.Conclusions For patients with open comminuted distal tibiofibular fracture,treatment should be conducted according to the concept of damage control orthopaedics.After early thorough debridement,the tibia should be fixated using external fixator and the fibula using kirschner wire,followed by VSD,leading to economical cost and satisfactory clinical efficacy.

Objective:To investigate the diagnosis and prognosis of one patient with carcinoma ex pleomorphic adenoma (CXPA)composed of three malignant components in parotid gland,and to raise the clinicians'awareness of the disease.Methods:A patient was presented to hospital because of the mass in left parotid gland region for more than 30 years and the accompanied pain lasted for one month.After color ultrasonography,the left parotid gland tumor resection was performed. Results: The operation was successful. The postoperative pathological diagnosis results comfirmed as CXPA,it was composed of three malignant components,including non-specific adenocarcinoma,ductal carcinoma,and myoepithelial carcinoma.Conclusion:CXPA composed of three malignant components at the same time are extremely rare.CXPA is difficult to diagnose and its prognosis is poor.The clinicians are supposed to improve the understanding of CXPA.

Objective:To investigate the method of arthroplasty for reconstructing femporomandibular joint (TMJ) after the establishment of TMJ articular defect models in the rabbits,and to clarify the replacement and repair effects of carboh fiber reinforced polyetheretherketone (CFR-PEEK) material TMJ prostheses,and to provide an experimental basis for the clinical application of CFR-PEEK artificial joints to replace TMJ.Methods:Thirteen healthy adult Japanese rabbits were randomly divided into experimental group (n=6),positive control group (n=4) and negative control group (n =3).The right TMJ articular process of the rabbits in experimental group were resected to establish the joint defect models,and then the CFR-PEEK artificial joints were impanted.The right TMJ articular process of the rabbits in positive control group were resected,and the joint defect models were established without the CFR-PEEK artificial joint implantation.The rabbits in negative control group didn't receive any treatment.The body weights of the rabbits in each group were measured in 13 weeks.The effects of fixation and replacement after artificial joint implantation were assessed by CT imaging.Results:The TMJ articular process defect model was successfully implanted with the CFR-PEEK artificial joint.The CT imaging results after threedimensional reconstruction and CT images by contrast in vitro showed that the CFR-PEEK-implanted artificial joint was fixed well,it could be instead of the normal joint function.Compared with positive control group,the weights of rabbits in experimental group were significantly increased (P＜ 0.05) at 13 weeks;compared with positive control group,the weights of rabbits in experimental group and negative control group were both significantly increased (P＞0.05) at 13 weeks,but there was no significant difference between them (P＞0.05).The weights of rabbits in experimental group and negative control group kept normal growth;the weights of rabbits in positive control group was increased slowly,and even stopped growing or was negative.Conclusion:The CFR-PEEK artificial joint can be successfully implanted into the TMJ defect model and be used to repair the defect which help to restore chewing function.The CFR-PEEK is expected to be an ideal material for reconstructing TMJ.

Objective To explore the surgical feasibility and clinical efficacy of one-stage anterior-posterior approaches in treatment of severe fracture and dislocation of lower cervical spine in "beach chair position".Methods Sixteen male cases of severe fracture and dislocation of lower cervical spine and with a mean age of 49.8 years (range,36-78 years) treated surgically from May 2012 to May 2016 were analyzed retrospectively by using case series study.The segment of injury was C4-5 in 4 cases,C5-6 in 7 and C6-7 in 5.The degree of spinal cord injury according to the American Spine injury Association (ASIA) score was Grade A in 4 cases,Grade B in 7 and Grade C in 5.Sub-axial injury classification (SLIC) score was 8 points in 9 cases and 9 points in 7.After a general anesthesia,a ring with a hole was hanged on patient's head before the operation.Then,under the protection of hole traction,the upper of operating bed was swung up slowly,so that the patient was restricted in vertical "beach chair position" with traction on the halo in order to immobilize the head and partially reduce the kyphotic deformity.Routine cervical anterior-posterior approach was done with the exposure of damaged section of the front and rear structure.Pedicle screw system or lateral mass screw displacement was conducted.Anterior intervertebral discectomy or fracture vertebral was performed,using collaborative reset prying method before and after the road.In the front of intervertebral cage or titanium net support bone graft,rear pedicle screws or lateral mass screws fixation and bone graft fusion were implemented.The operation time and blood loss were recorded.The healing of the wound was observed.The recovery of neurological function was evaluated according to the ASIA grade.Postoperative review X-ray,CT and MRI were done to evaluate the reset and bone graft in position and fusion.Results All the surgeries were done well without aeroembolism and other related complications.The mean operative time was 153 minutes (range,150-180 minutes),and the mean amount of blood loss was 543 ml (range,400-800 ml).Sixteen cases were followed-up from 6 to 24 months (mean 13.7 months).All the incision were healed at Ⅰ stage.Spinal cord function did not aggravate.The ASIA grade was improved with an average of one to two Grades 6 months after surgery.Postoperative X-ray and CT confirmed that graft object position was favorable and cervical sequence was recovered well.The Cobb angle decreased from (23.6 ± 5.3) ° preoperatively to (4.0 ± 0.4)°postoperatively,and the translational displacement of vertebral body was restored into (2.7 ±0.4) mm (P ＜ 0.01) from (10.9 ± 1.6) mm before operation.The cervical spinal canal was not obstructed and the cervical spinal cord was relieved,showed by MRI.Conclusions One-stage anterior-posterior approaches for severe fracture and dislocation of lower cervical spine circumferential reconstruction in "beach chair position" is a beneficial and effective method,without the need of changing positions in a collaborative reduction and fixation.The method can reduce the interference of spinal cord,shorten the operation time and save anterior extra fixation.