ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.