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Turner syndrome (TS) complicated with Madelung deformity and complex brachydactyly are rare clinically, and it is even rarer to combine with ankylosing spondylitis (AS). This article reports a case of a 36 year-old female patient who was admitted to the hospital with "ankylosing spondylitis" due to repeated back, hip and knee pain for 11 years and aggravated for 1 month. After admission, the karyotype showed "46, X, i (Xq)" due to short stature, dysplasia of secondary sexual characteristics, limb deformity etc, thus confirming TS. Further examination revealed bilateral Madelung deformity and complex brachydactyly characterized by shortening of the fourth metacarpal, multiple missing and shortened middle phalanxes. By reviewing the relevant literature, we believe that autoimmune diseases such as AS should be excluded in TS patients. TS aggravates the inflammatory response of AS, but the combination of TS and AS is more likely to be an accidental event. Patients with Madelung deformity should be careful to exclude TS. Madelung deformity and shortening of the fourth metacarpal are mainly associated with haploinsufficiency of SHOX gene. Brachydactyly characterized by multiple loss and shortening of the middle phalanx may be a new clinical manifestation of TS, especially the karyotype of 46, X, i (Xq). It is possible that there are some genes on the short arm of X chromosome, which regulate the number, growth and development of fingers. Early estrogen replacement therapy can greatly benefit patients.
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Objective:To investigate the application value of esophagogastric anastomosis with double muscle flap plasty in total laparoscopic radical resection of proximal gastric cancer.Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 5 patients undergoing radical resection of proximal gastric cancer in Cancer Hospital of China Medical University from January to December 2020 were collected. All 5 cases were male, aged from 57 to 72 years, with a median age of 65 years. All 5 patients underwent total laparoscopic radical resection of proximal gastric cancer combined with esophagogastric anastomosis with double muscle flap plasty. Observation indicators: (1) operative situations; (2) postoperative situations; (3) follow-up. Follow-up was conducted using outpatient examination and telephone interview to detect postoperative anastomosis, esophageal reflux, nutritional status, quality of life, tumor recurrence and metastasis of patients up to February 2021. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers. Results:(1) Operative situations: all 5 patients underwent surgery successfully. The operation time, time of esophagogastric anastomosis with double muscle flap plasty, number of lymph node dissected, volume of intraoperative blood loss, length of surgical incision of 5 patients were (316±41)minutes,(109±11)minutes, 26±4, (48±12)mL, (3.4±0.4)cm, respectively. Results of intraoperative rapid frozen section pathological examination showed negative of esophageal margin. (2) Postoperative situations: the time to postoperative initial flatus, time to postoperative initial food intake, during of postoperative hospital stay, cost of treat-ment of 5 patients were (4.8±1.5)days, (5.8±1.5)days, (11.6±2.1)days and (5.5±0.4)×10 4 yuan, respectively. Results of postoperative pathological examination of 5 patients showed gastric adeno-carcinoma in all 5 patients including 4 cases with moderately and poorly differentiated adeno-carcinoma and 1 case with highly differentiated adenocarcinoma, with the TNM staging of pT1a-3N0-1 M0 stage. Of the 5 patients, 1 case underwent postoperative mild pneumonia and was cured by conservative treatment such as anti-infection and promotion of sputum evacuation. (3) Follow-up: all 5 patients were followed up for 2 to 12 months, with a median follow-up time of 6 months. Of the 5 patients, 4 cases underwent anastomotic patency and 1 case underwent mild anastomotic stenosis who was improved after endoscopic treatment. None of the 5 patients underwent reflux esophagitis. The body mass index, the score of nutritional risk screening 2002, the score of patient-generated subjective global assessment and the score of tumor patient quality of life of 5 patients were 21 kg/m 2(range, 19-27 kg/m 2), 2(range, 1-2), 2(range, 1-3) and 47(range, 42-52), respectively. None of the 5 patients underwent tumor recurrence or metastasis. Conclusion:Esophagogastric anas-tomosis with double muscle flap plasty can be used in total laparoscopic radical resection of proximal gastric cancer which will lead to satisfactory short-term efficacy.
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Objective@#To investigate the clinical value of endoscopic ultrasonography (EUS) and Multi-slice Spiral CT (MSCT) in the preoperativestaging of tumor(T) and lymph node (N) metastasis in patients with SiewertⅡand Ⅲ typeadenocarcinoma of esophagogastric junction(AEG).@*Methods@#Clinical data of 145 Siewert Ⅱ and Ⅲ type AEG patientswithout preoperative chemoradiotherapy were retrospectively reviewed. Theyall received preoperative EUS and MSCT examination and underwent surgical resection, and the results of EUS and MSCT were compared with their postoperative pathologic staging.@*Results@#The sensitivity, specificity, and accuracy of EUS for T stage in Siewert Ⅱ and Ⅲ type AEG were higher than those of MSCT. The total accuracy of EUS and MSCT were 90.3% and 63.5%, respectively, and the difference was statistically significant (χ2=29.52, P<0.01). The sensitivity of EUS for T1, T2 and T3 were 89.5%, 91.1% and 85.2%, respectively, which were significantly higher than 42.1%, 66.7% and 29.6% of MSCT (χ2=9.47, P<0.01 for T1; χ2=8.07, P<0.01 for T2; χ2=17.40, P<0.01 for T3). In addition, the total accuracy of EUS and MSCT for lymph node metastasis status of Siewert Ⅱ and Ⅲ type AEG were 75.9% and 64.8%, respectively, showing a statistically significant difference(χ2=4.23, P=0.04). The sensitivity of EUS for N1 and N2 were 82.1% and 79.2%, respectively, which were significantly higher than 53.6% and 60.4% of MSCT (χ2=5.24, P=0.02; χ2=4.48, P=0.03). There was no statistical significance for sensitivity of EUS and MSCT in N0 and N3 (P>0.05).@*Conclusion@#EUS diagnosis of T and N staging in Siewert Ⅱ/Ⅲ type AEG showed significantly greater performance than MSCT.
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<p><b>OBJECTIVE</b>To retrospectively analyze the clinicopathology of patients with gastric gastrointestinal stromal tumor(gGIST) who underwent radical excision within 18 years in 10 domestic medical centers in order to understand the status of domestic surgical treatment of gGIST.</p><p><b>METHODS</b>Clinicopathological data of gGIST patients undergoing radical excision in 10 medical centers from January 1998 to January 2016 were collected, and their operational conditions, postoperative adjuvant therapy, gene detection and survival were analyzed retrospectively.</p><p><b>RESULTS</b>A total of 1 846 cases were recruited in this study, including 246 cases from Guangdong General Hospital, 331 cases from Sun Yat-sen University Cancer Center, 374 cases from Union Hospital, Tongji Medical College of Huazhong University of Science and Technology, 342 cases from Nanfang Hospital of Southern Medical University, 265 cases from Fujian Medical University Union Hospital, 148 cases from Fudan University Shanghai Cancer Center, 49 cases from West China Hospital of Sichuan University, 43 cases from Peking University Cancer Hospital and Institute, 28 cases from the 81st Hospital of Pepole's Liberation Army(PLA), 20 cases from Cancer Hospital of China Medical University, Liaoning Cancer Hospital and Institute. There were 918 male (49.7%) and 928 female patients (50.3%) with median onset age of 59(18 to 95) years old. Fundus(735 cases, 39.8%) and body (781 cases, 42.3%) of stomach were the common sites of lesions. The average size of tumor was (5.3±4.6) cm. There were 1 421 cases with mitotic count ≤5(77.0%). According to the operation procedure, 924 cases (50.1%) underwent laparoscopic surgery, 759 cases (41.1%) laparotomy, 120 cases (6.5%) endoscopic surgery, and 20 cases (1.1%) laparoscopic combined with endoscopic surgery, 6 cases (0.3%) laparoscopic excision surgery through gastric wall and cavity, and 17 cases (0.9%) laparoscopy and then were transferred to laparotomy. Wedge excision were performed in 1 308 cases (70.9%), proximal gastric excision in 226 cases(12.2%), distal gastric excision in 92 cases (5.0%), total gastrectomy in 94 cases (5.1%), and local gastrectomy in 126 cases(6.8%). Multi-visceral excision was performed in 138 cases, and the splenectomy was performed in 83 cases(60.1%)with the highest ratio. According to modified NIH classification, 399 cases(21.6%) were extreme low risk, 580 cases(31.4%) were low risk, 424 cases(23.0%) were moderate risk, 443 cases (24.0%) were high risk. A total of 461 cases received postoperative imatinib adjuvant therapy, accounting for 53.2%(461/867) of patients with moderate and high risk. Among 1 846 cases, 1 402 cases (75.9%) had complete follow-up data and the median follow-up time was 33.6 (0.1 to 158) months. The 5-year survival rates of extreme low risk, low risk, moderate risk and high risk were 100%, 98.5%, 92.5%, and 79.2% with significant difference(P=0.000).</p><p><b>CONCLUSIONS</b>Gastric GIST occurs mostly in fundus and body of stomach in China. Wedge excision is the main operational procedure and laparoscopic operation is over 50%. General prognosis of gastric GIST is quite good.</p>
Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young Adult , Antineoplastic Agents , Therapeutic Uses , China , Combined Modality Therapy , Gastrectomy , Gastrointestinal Neoplasms , Gastrointestinal Stromal Tumors , Pathology , General Surgery , Imatinib Mesylate , Therapeutic Uses , Laparoscopy , Laparotomy , Postoperative Period , Prognosis , Retrospective Studies , Splenectomy , Stomach Neoplasms , Pathology , General Surgery , Survival RateABSTRACT
Objective The purpose of this study is to investigate the expression of human epidermal growth factor receptor-2(HER-2)in gastric carcinoma tissue and normal gastric tissue of these patients,and its relationship with the clinical pathological characteristic,the microvessel density( MVD) and early post-operative recurrence,and to analyze the clinical significance of expression of HER-2 in gastric carcinoma,in order to lay the theoretical foundation of effective therapy for gastric carcinoma.Methods The clinical data of 398 cases of gastric cancer and 363 cases of their adjacent non tumorous gastric tissue of Liaoning Cancer Hospital from March 2012 to July 2013 were analyzed retrospectively.The expression of HER-2 gene was detected by IHC method and Fish method.The expressive rate of HER-2 in gastric carcinoma tissue and normal gastric tissue of these pa-tients were compared,and its relationship with the clinical pathological characteristic,MVD and early post opera-tive recurrence were also analyzed.Results (1)Expressive rate of HER-2 in 398 cases of gastric cancer tissue was 14.07%(56/398),which was higher than that in all the adjacent non tumorous gastric tissue presented none expression 0(0/363)(P0.05).Expres-sive rate of HER-2 in T4 stage was higher than( T1 +T2 +T3 ) stage,but the difference was not statistically sig-nificant( P>0.05).(3)The value of MVD in HER-2 expressive group(58.63 ±19.97)was significantly higher than those in HER-2 non expressive group(49.04 ±19.25).A positive significant correlation was found be-tween HER-2 and MVD expression using the rank correlation matrix(P0.05).Conclusion There is excessive expression of HER -2 in gastric cancer tissue,excessive expression of HER -2 patients withgastric cancer is correlated with Lauren type,tumor location,vessel invasion status,TNM stage,lymph node metastases;it displays no correlation with age,sex,tumor size,histological differentiation degree,growth patterns ofcancer.HER -2 expression is positively associated with MVD value.Excessive expression of HER -2 is possibleto be correlative with early post operative recurrence,and it must have further follow up to confirm.
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Objective To investigate the CT imaging features of urinary tract ceftriaxone-associated pseudolithiasis.Methods The CT imaging data of three patients with ceftriaxone-associated pseudolithiasis were collected from August,2008 to April,2012.Their data combined with related literatures were used to review CT imaging features of urinary tract pseudolithiasis.Those three patients,including 2 males and 1 female,were 9,21 and 55 years old,respectively.Ceftriaxone sodium was administrated to one patient due to appendicitis and the other two patients due to urinary tract infection.After ceftriaxone treatment,1 case began to have backache and abdominal pain on day 13,1 case presented backache with floc in the urine on day 2 and progressed to anuria,and the other one had cotton-like material in the urine on day 5.Urinary tract high density shadows were found in all patients by CT scan.The patient with anuria was treated with ureteroscopic lithotripsy and percutaneous nephrolithotomy.The other 2 cases were arranged to be followed up with CT scan.In those cases,the CT showed that the high density shadow in one patient disappeared 16 days after drug discontinuation.The high density shadow was significantly reduced 12 days after ceftriaxone withdrawal in the other one.However,no follow-up was performed afterwards.Results All the urinary tract ceftriaxone-associated pseudolithiasis presented the high density shadows on CT,with CT value 47-667 HU.The density of CT results is homogenic,except one ureteral stone.The pseudolithiasis,existed in kidney,ureter and bladder simultaneously,were found in two patients.And the other patient was found that the pseudolithiasis existed in both kidney and bladder.Two cases were accompanied with hydroureter and hydronephrosis and the other case were accompanied with cholecystolithiasis.The multiple renal pseudolithiasis was noticed in 2 cases,which exhibited the nodular shape.And one patient with hydronephrosis presented both sand-like and nodular stones.Ureteral pseudolithiasis was found in 1 case with 4 stones and the other one with 2 stones.In those pseudolithiasis,5 appeared column morphology and one showed nodular shape.The single nodular stone in bladder was found in one case.And the multiple bladder stones were found in 2 cases.Conclusions The CT imaging characters of urinary tract ceftriaxone-associated pseudolithiasis are high homogenic density shadows in multiple parts of bilateral urinary tracts.The ceftriaxone-associated pseudolithiasis is column shape in ureter and sand-like or nodular shape in kidney and bladder.