Aphasia in Right Hemiparesis and Pain in Left Waist: A Deceiving Case in Vascular Ehlers-Danlos Syndrome / 罕见病研究

The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder. The prevalence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand. The vascular EDS(vEDS) are rare among the subtypes but are the worst in prognosis. The article reports a case of vEDS admitted to the hospital. The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons. The diagnosis was made after the genetic testing. The patient suffered from vEDS. Then, the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient. The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging. This article hopes to report this case and to share the experiences to the better understanding of this disease.

A Case Report of Blau Syndrome / 罕见病研究

Blau syndrome is a rare genetic disorder characterized by the a mix of granulomatous arthritis, uveitis, and dermatitis. Patients typically manifest multisystem involvement, including ocular, skin, and skeletal abnormalities. Blau syndrome is extremely rare, with a global incidence of less than one in a million among children. In this multidisciplinary consultation, we present a case of a 21-year-old young female patient having multisystemic involvement since early childhood. She was presented with multiple joint swelling, skin lesions, increased eye discharge, and accompanied by hypertension and arterial abnormalities, and received a diagnosis of uveitis. The patient had been receiving steroid treatment since the age of 6 and has tried various medications, with some improvement in joint swelling and ocular symptoms. Through this rare disease multidisciplinary consultation, we aim to provide guidance in the molecular diagnosis of the patient, multisystem assessment, and the selection and formulation of treatment plans. Additionally, we hope that by reporting this case, clinical physicians can gain a better understanding of the diagnosis and comprehensive treatment strategies for Blau syndrome, thereby improving the management and treatment of rare diseases.

Epidemiologic characteristics and drug resistance of isolated from blood culture escherichia coli in a hospital in Qinghai Province from 2016 to 2022 / 中华预防医学杂志

Objective: To explore the drug resistance of Isolated From Blood Culture Escherichia coli (E. coli) in a hospital in Qinghai over the past seven years, to evaluate the ability of matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) to analyze the homologous origin of E. coli, and to establish a protein fingerprint library to match with it, adjuvant clinical experience medication so as to provide the basis for the prevention and control of hospital-acquired infections. Methods: Retrospective analysis of blood cultures sent to hospitals from January 2016 to December 2022. Drug resistance and resistance changes in E. coli.A total of 1 841 E. coli strains were isolated from Qinghai Provincial People's Hospital from January 2016 to December 2022; all strains were identified by MALDI-TOF MS, and the VITEK2.0 drug sensitivity analyzer was applied for drug sensitivity analysis of the strains, and the mass spectrometry homology analysis and self-constructed protein fingerprint library were carried out by MALDI-Biotyper software; the protein fingerprint library was built by using WHONET5.6 software was used to statistically analyze the drug sensitivity results, SPSS23.0 software was used to analyze the relationship between fingerprint typing and drug sensitivity, and the χ2 test was used for intergroup comparisons. Results: A total of 1 841 strains of E. coli were detected in 4 582 positive blood culture specimens from January 2016 to December 2022, with a detection rate of 40.17%; the resistance rate of E. coli from blood sources to piperacillin/tazobactam and ceftriaxone was on the rise, and it was slightly decreased to cefepime, amikacin, levofloxacin, and sulfamethoxazole, and there was not much change to the rest of the drugs; After MALDI-Biotyper clustering analysis, the 1841 E. coli strains from Isolated From Blood Culture were classified into two major clusters and five subtypes, of which type Ⅰa1 accounted for about 40%, type Ⅰa2 accounted for about 2.7%, type Ⅰb accounted for about 3.8, type Ⅱa accounted for about 46%, and type Ⅱb accounted for about 7.5%. The detection rate of type Ⅰa1 E. coli was higher in general surgery (50.45%) and emergency surgery (50.92%), and the detection rate of type Ⅰb E. coli was higher in emergency medicine(10.05%)than in other departments. The drug sensitivity results of different subtypes were compared with each other, the resistance rate of type Ⅰa1 E. coli to cefepime was 21.3% higher than that of the remaining four types, and the difference was statistically significant (χ2=37.74,P=0.000); the resistance rate of type Ⅱ E. coli(>60%) to sulfamethoxazole was higher than that of type Ⅰ (<60%) as a whole, and the difference was statistically significant (χ2=15.248,P=0.004); and a preliminary database of homologous protein fingerprints of E. coli has been established E. coli homologous protein fingerprint library and validated. The drug susceptibility results of 1 288 E. coli strains in the validation set were statistically analyzed and compared with those in the training set. There was no significant difference(P>0.05). Conclusion: In recent years, the resistance rate of E. coli isolated from a hospital in Qinghai province to piperacillin/Tazobactam, cefepime, amicacin and other antibiotics has changed greatly. A fingerprint database of E. coli homologous protein was established, and it was found that the drug sensitivity data of E. coli were different among different fingerprint types. According to drug sensitivity, drug use could assist clinical experience and provide evidence for prevention and control of hospital illness.

Epidemiologic characteristics and drug resistance of isolated from blood culture escherichia coli in a hospital in Qinghai Province from 2016 to 2022 / 中华预防医学杂志

Objective: To explore the drug resistance of Isolated From Blood Culture Escherichia coli (E. coli) in a hospital in Qinghai over the past seven years, to evaluate the ability of matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) to analyze the homologous origin of E. coli, and to establish a protein fingerprint library to match with it, adjuvant clinical experience medication so as to provide the basis for the prevention and control of hospital-acquired infections. Methods: Retrospective analysis of blood cultures sent to hospitals from January 2016 to December 2022. Drug resistance and resistance changes in E. coli.A total of 1 841 E. coli strains were isolated from Qinghai Provincial People's Hospital from January 2016 to December 2022; all strains were identified by MALDI-TOF MS, and the VITEK2.0 drug sensitivity analyzer was applied for drug sensitivity analysis of the strains, and the mass spectrometry homology analysis and self-constructed protein fingerprint library were carried out by MALDI-Biotyper software; the protein fingerprint library was built by using WHONET5.6 software was used to statistically analyze the drug sensitivity results, SPSS23.0 software was used to analyze the relationship between fingerprint typing and drug sensitivity, and the χ2 test was used for intergroup comparisons. Results: A total of 1 841 strains of E. coli were detected in 4 582 positive blood culture specimens from January 2016 to December 2022, with a detection rate of 40.17%; the resistance rate of E. coli from blood sources to piperacillin/tazobactam and ceftriaxone was on the rise, and it was slightly decreased to cefepime, amikacin, levofloxacin, and sulfamethoxazole, and there was not much change to the rest of the drugs; After MALDI-Biotyper clustering analysis, the 1841 E. coli strains from Isolated From Blood Culture were classified into two major clusters and five subtypes, of which type Ⅰa1 accounted for about 40%, type Ⅰa2 accounted for about 2.7%, type Ⅰb accounted for about 3.8, type Ⅱa accounted for about 46%, and type Ⅱb accounted for about 7.5%. The detection rate of type Ⅰa1 E. coli was higher in general surgery (50.45%) and emergency surgery (50.92%), and the detection rate of type Ⅰb E. coli was higher in emergency medicine(10.05%)than in other departments. The drug sensitivity results of different subtypes were compared with each other, the resistance rate of type Ⅰa1 E. coli to cefepime was 21.3% higher than that of the remaining four types, and the difference was statistically significant (χ2=37.74,P=0.000); the resistance rate of type Ⅱ E. coli(>60%) to sulfamethoxazole was higher than that of type Ⅰ (<60%) as a whole, and the difference was statistically significant (χ2=15.248,P=0.004); and a preliminary database of homologous protein fingerprints of E. coli has been established E. coli homologous protein fingerprint library and validated. The drug susceptibility results of 1 288 E. coli strains in the validation set were statistically analyzed and compared with those in the training set. There was no significant difference(P>0.05). Conclusion: In recent years, the resistance rate of E. coli isolated from a hospital in Qinghai province to piperacillin/Tazobactam, cefepime, amicacin and other antibiotics has changed greatly. A fingerprint database of E. coli homologous protein was established, and it was found that the drug sensitivity data of E. coli were different among different fingerprint types. According to drug sensitivity, drug use could assist clinical experience and provide evidence for prevention and control of hospital illness.

Pelvic peritoneum reconstuction with barbed suture combined with irrigation and negative pressure drainage in laparoscopic rectal anterior resection for rectal cancer / 中华普通外科杂志

Objective To evaluate pelvic peritoneum reconstruction with barbed suture and irrigation and negative pressure drainage in laparoscopy rectal anterior resection for locally advanced rectal cancer.Methods From 2014 to 2018 159 locally advanced rectal cancer patients treated by laparoscopy were derided into study group (n =76),and those received gravity drainage without pelvic peritoneum reconstruction (n =83).Results There were not significant difference in the operation time between the two groups [(165 ± 18) min vs.(160 ± 17) min,t =0.098,P =0.069].There were significant differences in postoperative drainage tube indwelling duration [(5.7 ± 2.4) d vs.(7.4 ± 3.7) d,t =3.309,P =0.001],intraabdominal infection (7％ vs.17％,x2 =3.991,P =0.046) and anastomotic leakage (4％ vs.13％,x/2 =4.280,P =0.039).There was no significant difference in anastomotic stenosis (5％ vs.10％,x2 =1.088,P =0.297) and intestinal obstruction (9％ vs.11％,x2 =0.117,P =0.732) between the two groups.Conclusion It is simple,safe and reliable to perform pelvic peritoneal reconstruction using barbed suture,combined with irrigation and negative pressure drainage,it can decrease drainage tube indwelling duration,reduce the incidence of intraabdominal infection and anastomotic leakage.

Clinical and ultrasound imaging features of pancreatic acinar cell carcinoma / 中华医学超声杂志（电子版）

Objective To explore the clinical and sonographic characteristics of pancreatic acinar cell carcinoma (PACC).Methods The clinical data and abdominal sonographic findings of 13 cases of PACC identified with pathology were reviewed,including contrast-enhanced ultrasound (CEUS) images.There were 9 males and 4 females with the average of 53.9 years old.Symptoms included upper abdominal pain,nausea,chest distress and weight loss.Results The serum level of tumor maker was elevated in only 5 cases.Eight masses (61.5％) were located in the pancreatic body-tail,4 (30.8％) in the head,the whole pancreas was involved in 1 case.The mean maximal diameter was 8.0 cm.Four lesions (30.8％) presented as solid-cystic mass.Well-defined border was showed in 6 cases (60.0％).Exophytic type was observed in 5 cases (38.5％).Pancreatic ductal dilation was seen in 3 cases (23.1％),in which 2 cases showed obstruction of common biliary duct.Liver metastasis was assessed in 4 patients (30.8％),and lymph node metastasis in 3 cases (23.1％).Vessels were invaded in 4 cases (30.8％).In 2 cases by CEUS,one showed arterial iso-enhancement,the other with strong enhancement.Conclusion Certain characteristic clinical and ultrasonographic features could be revealed in PACC,as a sizable,exophytic,solid-cystic and relatively hypervascular mass with well-definded border.

Neoadjuvant therapy of regional intra-arterial chemoembolization combined systemic vein chemotherapy for locally advanced gastric cancer / 中华普通外科杂志

Objective To investigate the efficacy and safety of preoperative regional intra-arterial chemoembolization combined with systemic vein chemotherapy in the treatment of locally advanced gastric cancer.Methods The clinical data of 78 locally advanced gastric cancer patients treated by radical D2 gastretomy from January 2010 to December 2012 were retrospectively analyzed.Patients were devided into treatment group receiving regional intra-arterial chemoembolization combined with systemic vein chemotherapy (n =36),and those with preoperative systemic vein chemotherapy (n =42) 3 to 4 weeks before surgery.Systemic chemotherapy with mFOLFOX6 was started after 5 weeks of surgery in the two groups.Results The incidence of toxicity reaction and postoperative anastomotic leakage were lower in treatment group than in control group,all P ＜ 0.05.The 1-year recurrence and overall survival rate were similar between the two groups (all P ＞ 0.05).The 3-year liver metastasis rate was higher in treatment group than in control group (x2 =4.063,P ＜ 0.05).The 3-year survival rate was higher than that of the control group(x2 =4.396,P ＜ 0.05).Conclusions Preoperative systemic vein chemotherapy combined with regional intra-arterial chemoembolization decreases toxicity reaction,anastomotic leakage and liver metastasis,while prolonging 3-year overall survival.

Impact of neoadjuvant chemotherapy on surgery and prognosis in patients with stage Ⅲ gastric cancer / 中华全科医师杂志

Objective To investigate the impact of neoadjuvant chemotherapy on surgery and prognosis in patients with stage Ⅲ gastric cancer.Methods The 90 cases staging Ⅲ gastric cancer were randomly divided into neoadjuvant chemotherapy group and surgery group.Both surgical complications and prognosis were compared.Results The overall effective rate of neoadjuvant chemotherapy was 53％ (24/45),disease control rate of neoadjuvant chemotherapy was 91％ (41/45).Blood loss (t =4.102,P =0.037),local adhesions surgery (x2 =19.756,P =0.000),local tissue necrosis (x2 =13.512,P =0.000),tissue fragility (x2 =12.870,P =0.000) number of cases found in neoadjuvant chemotherapy group were higher than surgery group,and the data were statistically significant.There were no statistically significance between two groups about operation time (t =2.391,P =0.129),tissue congestion (x2 =0.865,P =0.352),postoperative bleeding(x2 =0.720,P =0.396),postoperative fistula (x2 =1.047,P =0.306).The survival time of neoadjuvant chemotherapy group was longer than surgery group,but it was not statistically significant(t =1.086,P =0.372).Conclusions Neoadjuvant chemotherapy could reduce the stage of the gastric cancer and increase the complexity of surgery.Thus preoperative evaluation should be prepared before the surgery.

The value of endorectal elastography in the diagnosis of malignant rectal adenoma / 中华医学超声杂志（电子版）

Objective To assess the value of endorectal elastography in the diagnosis of malignant rectal adenoma. Methods Sixty patients referred to the department of basic surgery of Peking Union Medical College Hospital for the evaluation of rectal adenoma from January 2011 to May 2013 were included in this prospective cohort. All patients underwent elasticity score (ES) and strain ratio(SR) examine before operation. To evaluate the reproducibility of the strain-ratio measurements, the intraclass correlation coefficient (ICC) was calculated. Compared with final operational pathological results,the diagnostic values of elasticity score and strain ratio method were assessed. Results Histopathological result showed there were 20 adenomas and 40 adenocarcinomas. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of ERUS was 68%(27/40), 40%(8/20), 58%(35/60), 69%(27/39), 38%(8/21), respectively. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of elasticity score was 85%(34/40), 70%(14/20), 80%(48/60), 85%(34/40), 70%(14/20), respectively. The SR of adenocarcinomas was significantly higher than that of adenomas(2.82±1.81 vs 1.23±0.57, t=3.333, P < 0.05). The differences among the SR of benign adenoma, benign adenoma with severe atypical hyperplasia and completely malignant lesions were statistically significant (1.26±0.84 vs 2.94±1.95, 1.42±0.55 vs 2.94±1.95, t=2.619, 2.415, P=0.013, 0.021). When 1.240 was chosen as the best cut-off value of SR, the sensitivity, specificity, accuracy, positive predictive value and negative predictive value of strain ratio was 82%(33/40), 75%(15/20), 80%(48/60), 87%(33/38), 68%(15/22), respectively. Conclusion Endorectal elasotgraphy can accurately diagnose the malignant rectal adenoma, which can provide reliable preoperative imaging evidence for clinical operation mode.