Objective To evaluate the role of echocardiography in diagnosis and post-operative evaluation of infants and children with double aortic arch. Methods Echocardiography was performed in 6 patients with double aortic arch from January 2006 to June 2008. The outcome of postoperative follow-up was also evaluated. Results Six patients ranged in age from 3 months to 4 years and averaged 18 months. There were 2 boys and 4 girls. Echocardiography diagnosed double aortic arch in 4 patients, one balanced arch and 3 right arch dominant, and was confirmed by surgical observation in each patient. The size of the two arches were almost same in balanced arch,and the diameter of right arch was bigger than that of left arch in right arch dominant. Echocardiography failed to diagnose in 2 patients with balanced arch. Three patients had associated heart defect, such as patent ductus arteriosus and ventricular septal defect. Five of the 6 patients underwent surgical division of the left arch in order to relieve esophageal and tracheal compression. At about 2 years postoperative follow-up,echocardiography can clearly show the flow of aortic arch and it's branches. Conclusions It is an important clue to double aortic arch if only two branches of aortic arch was shown on supersternal view by echocardiography. As a non-invasive techniqe, echocardiography is useful for diagnosing and post-operative evaluating of patients with double aortic arch.

Objective Molecular biological methods were used to classify and analyze the isolated Brucella strains,and to understand the geographical distribution characteristics,genetic types and regional distribution characteristics of Brucella in Qinghai Province.Methods Molecular biology typing of species of isolated Brucella strains in Qinghai was studied using Multiple Locus Variable-number tandem repeat Analysis (MLVA) technology.The classification results were described by geographical information system (GIS).Results There were 3 species Brucella melitensis,Brucella abortus,and Brucella suis among the 65 strains of Brucella in Qinghai.Brucella melitensis was the dominant species.The genotypes of MLVA were 42,43,47,28,36,112 and 6.The geographical distribution features showed that the 42 belonged to the evolutionary branches of A and B,which was widely distributed.The 43 of the C evolutionary branch and 47 of the E evolution branch were mainly in the hinterland of the Qinghai-Tibet Plateau.When searching in the Brucella2012 MLVA database,none of the genotypes obtained in this study were identical to those in the database.Conclusions The MLVA genotypes of Brucella are varied in Qinghai Tibet Plateau.They are widely distributed,completely different from those in other areas,and different genetic variations are found in different places.

The construction of health file of patients with brucellosis in Qinghai Province was based on the Qinghai Provincial Public Health Network Information Platform. It was jointly participated and implemented by animal husbandry and veterinary departments, animal disease control centers, disease control centers and hospitals at all levels, to achieve standardized management of early detection, early diagnosis, early treatment, as well as follow-up, review, and efficacy evaluation. After analyzing the health file of brucellosis patients, we proposed to strengthen the propaganda of brucellosis, collect information from multiple diseases, share resources with multiple departments, integrate human resources and exert management responsibilities, and explore opinions and countermeasures suitable for local working conditions. The establishment of a health file for patients with brucellosis could fully grasp the diagnosis and treatment of brucellosis patients, facilitate the management of follow-up and review, and it was helpful to understand the distribution and prevalence of brucellosis in Qinghai Province, and to warn the epidemic trend.

Objective:To analyze the surgical methods of operable breast cancer and analyze the follow-up results.Methods:A retrospective analysis of the clinical and pathological data of 636 operable breast cancer patients admitted to Zibo First Hospital from July 2008 to April 2018, including the clinical stage, pathological staging. Analyze of the proportion of four surgical methods, and through follow-up, analyze the treatment effect of different surgical methods.Results:All patients are female, aged 26-80 years, the clinical stage of 636 patients: Tis 18 cases, stage Ⅰ 143 cases, stage Ⅱ 354 cases, stage Ⅲ 114 cases, stage Ⅳ 7 cases. There are four types of surgery: ① breast conserving surgery + sentinel lymph node biopsy in 124 cases (19.50%); ② breast conserving surgery + axillary lymph node dissection in 39 cases (6.13%); ③ mastectomy + sentinel lymph node biopsy in 163 cases (25.63%); ④ modified radical surgery in 310 cases (48.74%). Sentinel lymph node biopsy in 427 cases (67.14%), success in 404 patients (94.61%); all patients with lymph node negative 384 cases (60.38%). Follow-up for 1 to 9 years, 11 cases of local recurrence after breast-conserving surgery, It accounted for 6.75% of breast-conserving surgery; 43 cases of local recurrence of chest wall after mastectomy, accounting for mastectomy 9.09%; 33 cases of recurrence and metastasis of axillary lymph nodes and supraclavicular lymph nodes, 4 cases of axillary recurrence after sentinel lymph node biopsy.Conclusions:The proportion of breast-conserving surgery in this group of patients was high and the local recurrence rate of breast-conserving surgery was less than that of mastectomy group; the proportion of simple modified radical surgery declined further; patients with axillary lymph node metastasis were less in the whole group. The choice of reasonable operation method is an important factor to improve the prognosis of breast cancer.

Objective:To construct a three-dimensional imaging model of the pancreatic head based on the embryological fusion plane, and to provide morphological parameters of the pancreatic head for future developments of basic and clinical researches on the pancreas.Methods:Histologic cross-sections of the pancreatic head with its adjacent structures were made from healthy cadavers. Immunohistochemical analysis of pancreatic polypeptide antibody was then performed to verify the existence and location of the embryological fusion plane reported previously. The histologically positioning method of the embryological fusion plane was then applied to the corresponding sections on computed tomography (CT). Based on the results of the above work, volunteers from the Affiliated Changzhou No. 2 People's Hospital of Nanjing Medical University were then used as research objects. A three-dimensional visualization reconstruction software was used to perform CT image-based structures to include the abdominal pancreas, dorsal pancreas head, and embryo fusion surface. Three-dimensional reconstruction of the pancreatic head, and morphological measurements of the relevant structures of the pancreatic head were then made.Results:Immunohistochemical analysis verified the existence and the position of the embryological fusion plane. The histologically positioning method was then successfully applied to the CT sections. The three-dimensional imaging model of the pancreatic head containing the embryological fusion plane, dorsal segment and ventral segment of head were built based on CT images. A total of 35 volunteers were included, including 19 males and 16 females, aged (48.26±8.26) years, and with a BMI of (22.29±1.78) kg/m 2. The morphological results showed that the volume of the pancreatic head, dorsal pancreas and abdominal pancreas were (32.80±8.15) cm 3, (22.21±6.94) cm 3, (10.59±3.87) cm 3, and the area of the embryo fusion surface was (12.46±3.20) cm 2. All volunteers were then grouped according to gender. Statistical analysis showed that there were no significant differences in the total pancreatic head volume, dorsal pancreatic head volume, abdominal pancreatic volume, and embryo fusion area among the groups ( P>0.05). Conclusions:It was feasible and practical to build a three-dimensional imaging model of the pancreatic head based on the embryological fusion plane by using a 3D computer system. This model and its morphological parameters could provide a new tool for research on pancreas.

Objective:To analyze the causes of postoperative stricture of biliary-enteric anastomotic for congenital choledochal cysts.Methods:These 28 patients underwent salvage operation on an average 15 years (0.2-25 years) after initial surgeries at the Department of Hepatobiliary Surgery, Hunan Provincial People's Hospital from Jan 2014 to Jun 2018.Results:In 26 patients the biliary-enteric anastomotic stenosis was benign, and in 2 the stricture was caused by cancerration. In 26 cases the Roux-en-Y hepaticojejunostomy was redone,among them 8 cases underwent concurrent hepatectomy for a better exposure of the intrahepatic bile duct. In 2 cases the anastomotic stenosis was found to be caused by canceration with extensive intraabdominal metastasis ,an external drainage was adopted. There were no inhospital deaths, and no serious complications. The postoperative follow-up time was 6-67 months. Two cancerated patients died within half a year, and the remaining patients had no long-term complications.Conclusions:Biliary-enteric anastomotic stenosis is one of the serious complications in postoperative patients for congenital choledochal cysts. Hence a wide, tension free biliary-enteric anastomosis performed by a experienced hand is necessary.

Objective:To evaluate partial ventral hepatectomy in the treatment of patients with complicated iatrogenic high bile duct injury.Methods:The clinical data of 8 cases of complicated iatrogenic high bile duct injury treated with the assistance of hepatic ventral segmentectomy from Mar 2013 to May 2020 at Hunan Provincial People's Hospital was retrospectively analyzed.Results:Among the 8 patients, 5 patients underwent partial Ⅳb lobectomy, and 3 patients received partial Ⅳb and Ⅴ segmentectomy of the liver. All the operation was successful without death in hospital. One case developed subphrenic infection and seroperitoneum, which was healed by anti-infection treatment and abdominocentesis. The postoperative follow-up time was 5-90 months, and all of patients are doing well. There was no stenosis in intrahepatic bile duct by postoperative cholangiography or MRI.Conclusions:Quadrate lobe hepatectomy provides a wide view for the treatment of complicated iatrogenic high bile duct injury by fully opening the first porta hepatis and exposing the primary and secondary bile duct branch helping establish a wide patent tension free bile duct-jejunostomy.

Hereditary gingival fibromatosis (HGF) is a rare inherited condition with fibromatoid hyperplasia of the gingival tissue that exhibits great genetic heterogeneity. Five distinct loci related to non-syndromic HGF have been identified; however, only two disease-causing genes, SOS1 and REST, inducing HGF have been identified at two loci, GINGF1 and GINGF5, respectively. Here, based on a family pedigree with 26 members, including nine patients with HGF, we identified double heterozygous pathogenic mutations in the ZNF513 (c.C748T, p.R250W) and KIF3C (c.G1229A, p.R410H) genes within the GINGF3 locus related to HGF. Functional studies demonstrated that the ZNF513 p.R250W and KIF3C p.R410H variants significantly increased the expression of ZNF513 and KIF3C in vitro and in vivo. ZNF513, a transcription factor, binds to KIF3C exon 1 and participates in the positive regulation of KIF3C expression in gingival fibroblasts. Furthermore, a knock-in mouse model confirmed that heterozygous or homozygous mutations within Zfp513 (p.R250W) or Kif3c (p.R412H) alone do not led to clear phenotypes with gingival fibromatosis, whereas the double mutations led to gingival hyperplasia phenotypes. In addition, we found that ZNF513 binds to the SOS1 promoter and plays an important positive role in regulating the expression of SOS1. Moreover, the KIF3C p.R410H mutation could activate the PI3K and KCNQ1 potassium channels. ZNF513 combined with KIF3C regulates gingival fibroblast proliferation, migration, and fibrosis response via the PI3K/AKT/mTOR and Ras/Raf/MEK/ERK pathways. In summary, these results demonstrate ZNF513 + KIF3C as an important genetic combination in HGF manifestation and suggest that ZNF513 mutation may be a major risk factor for HGF.
Animals ; Humans ; Mice ; Fibromatosis, Gingival/pathology* ; Gingiva ; Kinesins/genetics* ; Mutation/genetics* ; Phosphatidylinositol 3-Kinases/genetics*