Proteus syndrome is a rare complex disorder characterized by asymmetric and disproportionate overgrowth of multiple tissues,presence of cerebriform connective tissue nevi and epidermal nevi,vascular malformation and abnormality of fat tissues.In 2006,a patient with typical manifestation of Proteus syndrome was admitted to our hospital.By minimally invasive plastic surgery,we achieved good therapeutic results.

Objective:To introduce a novel method of endoscopic forehead lift and its follow-up results.Methods:In the past 7 years, 119 patients underwent endoscopic forehead lift. The parallel incisions were made on the frontalis muscle to relieve the wrinkles of the forehead. Glabella muscles were partially bluntly bitten by minimally invasive forceps. No fixator or suture was applied during the operation. Postoperative FACE-Q scales were issued to patients, and the changes of eyebrow position before and after operation were measured.Results:One week postoperatively, glabella frown and forehead wrinkles were relieved or eliminated as the swell gone. The eyebrow measurement outcome showed that the eyebrows of the patients were definitely raised after the operation. The results of FACE-Q scales showed that patients were highly satisfied with the improvement of frontal wrinkles and glabella frown wrinkles, and with the surgical outcomes and decisions. The patients thought they were averagely 7 years younger after operation. The most common complication was temporary frontal skin numbness, followed by hairline regression.Conclusions:The endoscopic forehead lift can effectively improve the forehead wrinkles and the glabella frown wrinkles which meets the demands of Chinese patients.

Objective To investigate the treatment effection of cranial decompression under temporal muscle in very-low position with large bone flap for severe cranial trauma.Methods 53 cases of severs cranial traumatic brain herniation were derided into two groups.22 cases with unilateral dilated pupil,and 31 cases of bilateral dilated pupil,all the patients were treated with cranial decompression under temporal muscle in very low position with large bone-flap.The ICP,appearance rate of cisternal,pupil contraction rate and GCS evaluation were observed,recorded and statistiely analysised.Results According to the intraeranial pressure monitoring,the rate of 24h ICP＜20mmHg was increased significantly,and the rate of 72h ICP＞40mmHg was decreased in both groups.The occurance rate of cisternal:81.82% in unilateral dilated pupil group,and 51.61% in bilateral dilated pupil group.The recoverance rate of pupil in 24h:77.2% in unilateral dilated pupil group,and 32.26% in bilateral dilated pupil group.GCS evaluation:All of the patient's average mark of GCS after operation wag(8.02±3.03)which increased(3.92±2.21)compared with the mark of GCS before operation,which was(4.10±0.82),with a significant difference(P＜0.01).Treat outcome:well/moderate disability:64.15%,severe/long-term coma:7.55%,and death:28.30%.Conclusion Cranial decompression under temporal muscle in very-low position with large bone-flap for severe cranial trauma can enlarge the cranial capacity effectively,increase the decompression space in dorsolateral cranium,which can alleviate the intracranial pressure in axial centre of brain truak,promote the effeetion of the brain herniation restore and the recover of the brain trauma.

ObjectiveTo explore the effect of different methods of drainage tube and catheter drainage in the treatment of intraventricular hematoma.MethodsThe analysis was conducted in 83 cases,who were randomly divided into two groups,the treatment group with 48 cases adopting lateral venticle catheterization combined with bulletshaped tube,and the control group with 35 cases adopting conventional catheterization.The contrasted study was conducted on the clearance rate of cerebroventricular hematoma and the effect of the different drainages.ResultsThe clearance rate in treatment group was 27.1％ ( 13/48 ),50.0％ (24/48),14.6％ (7/48),at the time of ＜ 24h,24 ～72h,＞ 72h after the operation,and 5.7 ％ ( 2/35 ),17.1％ ( 6/35 ),48.6％ ( 17/35 ) in the control group.It showed statistical significance(x2 =6.2425,9.4678,11.3757,all P ＜0.01 ).The cases with type Ⅰ of ADL stage in treatment group (47.9％)were more than control group( x2 =6.8347,P ＜0.01 ).The morbidity in treatment group was 12.5％,which was lower than control group( x2 =6.9636,P ＜0.01 ).ConclusionThe lateral venticle catheterization drainage combined with bullet-shaped tube could avoid the obstruction,reduce the morbidity,and improve the therapeutic effect.

Objective: To analyze the association of cytogenetic abnormalities with the prognosis of chronic myeloid leukemia (CML) patients in tyrosine kinase inhibitors (TKI) era. Methods: Karyotype analysis of chromosome G-banding was carried out in 387 newly diagnosed CML patients by short-term culture of bone marrow cells. The correlation of cytogenetic abnormalities and CML progression was explored in combination with ABL tyrosine point mutations. Result: Of 387 patients with positive BCR-ABL fusion gene assayed by fluorescence in situ hybridization (FISH) technique, 94.1% (364/387) patients were Ph positive and 5.9% (23/387) Ph negative; 320 patients (87.9%) had a translocation t (9;22) (q34;q11) and 5 (1.4%) a variant translocation t (v;22) . Additional cytogenetic aberrations (ACA) at diagnosis were found in 10.7% (39/387) Ph⁺ patients, major route ACA in 22 (56.4%) cases and minor route ACA in 15 (38.5%) cases and 2 patients (5.1%) lacked the Y chromosome (−Y) ; 23.4% (71/303) patients occurred ACA during TKI treatment and the most frequent abnormalities were abnormal chromosome numbersd, which were likely associated with high proportion of disease progression (χ²=168.21, P<0.001) and ABL tyrosine point mutations (χ²=29.04, P<0.001) . Newly diagnosed CML-CP patients with t (9;22) (q34;q11) had a longer event-free survival (EFS) and disease-free survival (DFS) rates than that of patients with ACA (P=0.037; P=0.003) , while the overall survival (OS) had no significant differences (P=0.209) . As for CML-CP patients that occurred ACA during TKI therapy would have a marked low OS, EFS and DFS (all P<0.001) compared with no ACA occurred patients. Survival of advanced patients that occurred ACA were dramatically reduced. Conclusion ACA often emerged during the disease progress in CML patients, regular and timely detection of chromosomes karyotype and ABL tyrosine point mutations during TKI treatment was important for therapeutic evaluation, progress and prognosis of CML.

Objective: To investigate the clinical implications of p16 gene deletion in adult Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph⁺ ALL) . Methods: Retrospective analysis of clinical, immunophenotypic, cytogenetics, molecular characteristics and prognosis of 80 newly diagnosed Ph⁺ ALL patients with p16 deletion. Results: Of 80 adult Ph⁺ ALL, the prevalence of p16 gene deletion was 31.3%. p16 gene deletion carriers frequently accompanied with high WBC counts (WBC≥30×10⁹/L) and CD20 expression. The incidence of complex chromosome abnormality in p16 gene deletion group was higher than that in non-deletion group, with alternations in chromosome 7, 8, 19 and der (22) more frequently observed. There was no difference occurred between patients with or without p16 gene deletion in complete remission (CR) rate following induction chemotherapy combined with tyrosine kinase inhibitors (TKIs) . However, after three cycles of chemotherapy, the MMR and CMR rate in the p16 gene deletion group was lower than patients with wild-type p16 gene (P=0.034, P=0.036) . The p16 gene deletion patients showed no significant differences in MMR, CMR and relapse rate between Imatinib or Dasatinib plus chemotherapy (P>0.05) . Deletion of p16 gene was significantly associated with poor outcomes including worse overall survival (OS) (37.1% vs 54.1%, P=0.037) , lower disease free-survival (DFS) (12.4% vs 45.9%, P=0.026) , and increased cumulative incidence of relapse (P=0.033) . Among the 25 patients with p16 deletion, 14 underwent allo-HSCT and the median survival was 21 months, better than that of patients received chemotherapy alone (12 months) (P=0.030) . Conclusion This study indicated that deletion of p16 was associated with poor prognosis in adult Ph⁺ ALL, and the utility of second-generation TKI (Dasatinib) does not necessarily have an edge on efficacy over Imatinib, but allo-HSCT has the potential of elongating life expectancy. It is an important significance to define the status of p16 in Ph⁺ ALL for predicting prognosis and guiding therapy decision-making.

Objective:To evaluate the improvement of nasal appearance, symptoms and psychological burden in patients with secondary cleft rhinoplasty by using retrospective research methods, through preoperative and postoperative questionnaires.Methods:Twenty-three cleft lip patients that underwent surgery in our unit since April 2016 were enrolled in this study. The self-designed questionnaire was used to explore the changes of patients before and after the operation. The questionnaire star was utilized to collect the data, and after the data was sorted and summarized, SPSS 20.0 was used for analysis and statistics.Results:The average follow-up time was 28.9 months. The total score of ROE satisfaction before surgery was 46.74 points, while after surgery it reached 63.04 points ( t=4.10, P<0.01); the self-scores of nasal shape were significantly improved after surgery; but there was no significant change in the ventilation function in the VAS scale before and after the operation; the nasal symptoms and psychological status in the SNOT-22 questionnaire, such as, nasal congestion, embarrassment, depression, restlessness and irritability, were significantly improved ( P<0.05). Conclusions:Our semi-autonomous designed questionnaire can be used to effectively evaluate the satisfaction, nasal shape, and symptoms of patients with nasal deformity secondary to cleft lip. Data analysis shows that our secondary cleft lip rhinoplasty for cleft lip and palate can improve patients′ satisfaction after the repair, as well as nose shape and function, at the same time the psychological burden caused by it.

Objective To investigate the clinical features and surgical treatment of cerebral amyloid angiopathy hemorrhage (CAAH) caused by cerebral vascular amyloidosis.Methods The clinical data,radiological diagnoses,pathology results and treatment efficacies of 76 patients with CAAH,admitted to and performed surgery in our hospital from August 2010 to September 2015,were retrospectively analyzed.Results The first preoperative CT indicated that prompt hematomas were located in the cerebral hemisphere brain cortex,forming lobulated or irregular shape;2 or more lesions of recurrent hemorrhage were noted in 14 (18.4％);34 (44.7％) were with subarachnoid hemorrhage,16 (21.1％) were with intraventricular hemorrhage.Histopathological examination supported the diagnosis.Sixteen patients (21.1％) underwent second operation during hospitalization due to large amount of bleeding.During hospitalization,18 (23.7％) died,and 20 (26.3％) had long-term coma.During the follow-up period,23 patients (30.3％) had recurrent intracranial hemorrhage,of which,6 patients (7.9％) underwent surgical treatment,and 17 patients (22.4％) died.Conclusion The bleeding sites of CAAH mainly locate in the lobes of the brain surface,with lobulated or irregular shape,which is easily complicated with subarachnoid hemorrhage or intraventricular hemorrhage,with multiple features;diagnostic rate of CAAH can be improved by pathological examination of brain tissues and blood vessels;CAAH after surgery has high relapse rate and poor surgical results.

Objective:To attain comprehensive insight into the diagnostic X-ray equipment and examination frequency in radiological diagnosis and treatment institutions in Wuhan, with the purpose of assisting the health administration department in formulating medical exposure protection strategies and efficiently allocating radiological diagnosis and treatment resources.Methods:Using the census method, questionnaires on the basic information on diagnostic X-ray equipment and the annual number of examinations filled out in 2022 by the 1 030 radiological diagnosis and treatment institutions (excluding military and armed police hospitals) were collected through the Wuhan Prevention and Treatment Information Management Platform for Occupational Diseases. To obtain the data on number and frequency of diagnostic X-ray examinations, the different types of diagnostic X-ray examinations were divided by the total number of permanent residents by the end of 2021 in Wuhan.Results:In Wuhan, 1 030 radiological diagnosis and treatment institutions had 7 062 radiation workers and 2 540 diagnostic X-ray units of various types. 37.76% of units and 75.01% of radiation workers were concentrated in tertiary hospitals. The number of diagnostic X-ray units per million population was 186.10, with the top two being 48.65 DR machines per million population and 31.21 intraoral dental machines per million population. The total number of diagnostic X-ray examinations was 11 884 582, with plain radiographs and computed tomography (CT) examinations accounting for 43.61% and 43.59% of the total, respectively. The annual frequency of examinations was 379.75 and 379.52 per 1 000 population, respectively. Radiodiagnosis and treatment resources were higher in central urban areas than that in remote urban areas.Conclusions:The development of diagnostic X-ray equipment in Wuhan was experiencing rapid growth, with potential for further expansion, and the frequency has not yet recovered to the level before the COVID-19 pandemic. The allocation of radiodiagnosis and treatment resources between central urban areas and remote urban areas needs to be coordinated and the management of medical radiation protection should be continuously strengthened, so as to promote the sustainable development of inter-regional radiodiagnosis and treatment, and ensure the health and safety of examinaed patients and indivuduals.