ObjectiveTo study the changes and clinical significance of serum heart-type fatty acid-binding protein (H-FABP) and soluble ST2 protein (sST2) in children with chronic heart failure (CHF).MethodsThirty-nine children with CHF and 30 healthy children were recruited. Serum levels of H-FABP and sST2 were determined by ELISA, The left ventricular ejection fraction (LVEF) and fractional shortening of the left ventricle (LVFS) were measured by two-dimensional echocardiog-raphy.ResultsIn 39 children with CHF, 15 males and 24 females, aged 2 months to 14 years, included 27 cases of endocardial ifbroelastosis (EFE) and 12 cases of dilated cardiomyopathy (DCM). According to the cardiac functional grading standard, the children with CHF were divided into 10 cases with cardiac function II, 15 cases with cardiac function III, and 14 cases with cardiac function IV. The mean levels of H-FABP, sST2 and NT-Pro-BNP in children with CHF at stage of heart failure and heart failure remission were statistically higher than those in the healthy children (allP<0.01). The serum H-FABP and sST2 levels had signiifcant differences among groups grouped according to cardiac functional grading standard (allP<0.05). The serum H-FABP and sST2 levels had no signiifcant difference between the EFE and DCM groups (allP>0.05). The Spearman correlation analysis showed that, in children with CHF at stage of heart failure, the serum H-FABP level was positively correlated with NT-Pro-BNP, sST2 and cardiac function (r=0.402、0.621、0.644,P<0.05). Serum sST2 level was positively correlated with NT-Pro-BNP and cardiac function (r = 0.501、0.678,P<0.05), and was negatively correlated with LVEF and LVFS (r=?0.340、?0.329, P<0.05).ConclusionsH-FABP and sST2 are involved in the development of heart failure. H-FABP and sST2 can be used as reference indices for clinical diagnosis and assessment of CHF.

Objective To explore the variation of serum galectin-3 and its correlation with ventricular remodeling in children with chronic cardiac failure (CHF). Methods Forty-ifve children with CHF were included and divided into cardiac function II group (n=10 ), III group (n=18 ), and IV group (n=17 ) according to the severity of CHF. The subjects were also divided into endocardial fibroelastosis (EFE) group (n=21 ) and dilated cardiomyopathy (DCM) group (n=24 ) according to primary disease. Thirty health children were included as control group. The level of serum galectin-3 was detected by ELISA. The level of serum NT-pro BNP was measured by radio immunoassay. The index of ventricular remodeling was detected by ultrasonic cardiogram. The correlation of the level of serum galectin-3 with ventricular remodeling and the level of serum NT-pro BNP were analyzed. Results In 45 children ( 19 males and 26 females) with CHF, the mean age was 3 . 42 ± 1 . 89 years. The levels of serum galectin-3 and NT-pro BNP were higher in cardiac function II group, III group, and IV group than those in control group (all P?0 . 05 ). Spearman rank correlation analysis showed that the level of serum galectin-3 was positively correlated with the left ventricular end diastolic diameter, the left ventricular mass, the left ventricular mass fraction, and the level of serum NT-pro BNP (all P?

Proper timing of repair is one of key factors predicting long-term prognosis of iatrogenic biliary injury.Local inflammation is proved related to long-term biliary stricture.This article introduces pathological procedure of biliary injury based on pathophysiological mechanism and animal model rescarch of wound healing,and how to increase intraoperative repair rate based on the clinical evidences.The preoperative active inflammation control and systemic management could create necessary conditions for the the subsequent early repair.At the same time,authors suggest to set individual strategy regarding timing of repair.Delayed repair is recommended for combined vascular injury or severe biliary injury with terrible contamination.

Objective To evaluate the clinical features and the key points in the diagnosis and management of splenic hemangioma.Methods The clinical presentations,laboratory tests,imaging and pathological results,treatment,and prognosis of 21 cases of splenic hemangioma admitted in Peking Union Medical College Hospital from April,1989 to July 2007 were retrospectively analyzed.Results The clinical presentations of splenic hemangiom are not specific which include left upper quadrant mass or discomfort,abdominal pain,etc.The diagnosis of imaging includes Doppler ultrasound,CT,MRI,DSA,etc.Splencetomy is recommended for all splenic hemangioma with severe symptoms or rupture.Conclusion Asymptomatic patients with small splenic hemangioma(＜4 cm)can be managed conservatively.Symptomatic large hamangioma may need a sp]enectomy.

Objective To study the clinical features of intraductal papillary mucinous neoplasm of the biliary tract (BT-IPMN) and to analyse the diagnostic and surgical patterns.Method The data of 19 patients with BT-IPMN admitted from Jun.2012 to Jul.2016 were retrospectively analyzed.Results These 19 (2.3％) patients with BT-IPMN came from 815 patients with biliary tract tumors who were treated in our institution.There were 9 males and 10 females.The male to female ratio was 1.0∶ 1.1.The mean age was 60.6 ± 12.9 years with a range from 25 to 78 years.Jaundice (in 10 patients) and abdominal discomfort (in 6 patients) were the most common presenting symptoms.Bile duct dilatation and intraluminal mass were typical preoperative imaging findings.All these 19 patients were diagnosed to have BT-IPMN histopathologically.18 patients underwent surgery in our hospital.Left lateral hepatic sectionectomy or left hepatectomy was performed in 8 patients,pancreaticoduodenectomy in 6,local excision of bile duct and cholangiojejunostomy in 3 and central hepatectomy in 1.The average operating time was 280 minutes and the average amount of intra-operative bleeding was 515 ml.Fourteen patients underwent regional lymph node dissection and 3 positive lymph nodes were detected out of 94 resected lymph nodes.Sixteen patients were followed up from 1 to 51 months with a mean of (25.7 ± 19.5) months.Fourteen of these patients were still surviving.Two patients died 3 and 17 months after operation due to the tumor.Conclusions Intraductal papillary mucinous neoplasm of the biliary tract was very rare.Early diagnosis was not easy.There was a low percentage of lymphatic metastasis.Surgery was the first choice of treatment and complete resection of BT-IPMN was associated with good long-term survival.

Objective To evaluate clinical features of three different gallbladder projective lesions. Methods Statistic analysis was performed for clinical data obtained from 325 cases of gallbladder projective lesions. Results There were 308 patients of benign lesions, including cholesterol polyps in 278 cases, other polyps in 5 cases, and adenomas of 25 cases. Malignant lesions was found in 17 cases, including 2 adenomas with malignant changes and 15 adenocarcinomas. 36. 0% and 32. 0% patients with benign polyps and adenomas were over 50 years of age, while 82.4% patients with adenocarcinomas were over 50 years. Average size of benign polyps, adenomas and adenocarcinomas were 8. 5 mm, 11.7 mm and 31.1 mm in diameter. 1.5% of benign polyps, 0% of adenomas and 52. 9% of adenocarcinomas were of low echo. 42.9% of benign polyps, 68.0% of adenomas and 100% of adenocarcinomas were single polyp. Conclusion The nature of gallbladder projective lesions can be suggested by patient age, size, number, and intensity of gallbladder projective lesions under B-ultrasound.

IgG4-related sclerosing cholangitis (IgG4-SC) is characterized by biliary strictures and obstructive jaundice.Elevated levels of serum IgG4 and a multifocal IgG4-rich lymphoplasmacytic infiltration of affected organs are also the characteristics of IgG4-SC. Prednisone is recommended in the initial treatment of IgG4-SC. The differential diagnosis between IgG4-SC and cholangiocarcinoma is not easy. Comprehensively collection of medical history, paying attention to other organs involvement, and getting the pathologic diagnosis are important for diagnosis of this disease. In order to prevent misdiagnosis we should learn more about IgG4-SC. On the other hand, if we can not differentiate IgG4-SC and cholangiocarcinoma, operation may be recommended.

OBJECTIVETo describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China.

METHODSClinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were analyzed. Dilated internal jugular vein in the proband was removed by operation. The diagnosis, surgical treatment, and postoperative complications were retrospectively reviewed.

RESULTSVessels of the proband in the pedigree were crisp and easily lacinated during the procedure of removing his internal jugular vein. Repeating postoperative hematomas were found though complete stanching was achieved during the operation. The patient was successfully recovered by promptly debridgement and needle sucking. The other 5 members of the pedigree all had the triads of Ehlers-Danlos syndrome.

CONCLUSIONSThough it was of extremely low morbidity rate, the high mortality rate and complication of Ehlers-Danlos syndrome deserve great attention during surgical management, especially in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoperative vascular lascination and incontrollable hemorrhage.

China ; Ehlers-Danlos Syndrome ; genetics ; surgery ; Humans ; Jugular Veins ; surgery ; Male ; Middle Aged ; Pedigree

OBJECTIVETo report a case and a firstly described pedigree with Ehlers-Danlos syndrome in China.

METHODSClinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members with 4 generations were analyzed. Dilated internal jugular vein in the proband was removed operatively. The diagnosis, surgical treatment, and postoperative complications were retrospectively summarized. Etiology, clinical characteristics and classification of Ehlers-Danlos syndrome were also analysed.

RESULTSVessels of the proband in the pedigree were crisp and easily lacinated during the precedure for removal of his internal jugular vein. Repeat postoperative hemotomas were found though complete stanching was made during operation. The patient successfully recovered after prompt debridement in operative theatre and needle sucking. The other 5 members of the pedigrees all had the triads of Ehlers-Danlos syndrome, but no combined vascular diseases were found in them.

CONCLUSIONSThough the morbidity rate was extremely low, green attention should be paid to the high mortality rate and complication of Ehlers-Danlos syndrome during surgical management of in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoprative vascular lacination and incontrollable hamorrhage.

China ; Ehlers-Danlos Syndrome ; Humans ; Pedigree ; Retrospective Studies

Objective:To investigate the clinicopathological features and treatment strategies of intraductal papillary neoplasm of the bile duct (IPNB).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 40 patients with IPNB who were admitted to Peking Union Medical College Hospital from August 2000 to April 2020 were collected. There were 19 males and 21 females,aged (60±14) years. Patients underwent preoperative imaging examination and blood test for evaluation of tumor location, range and resectability. The treatment strategies of patients depended on preoperative examination and their own willingness. Observation indicators: (1) preoperative examinations and tests; (2) treatment; (3) pathological examination; (4) follow-up. Follow-up using outpatient examination, telephone interview and online diagnosis was performed to detect tumor recurrence and survival of patients up to April 2021. Count data were represented as absolute numbers and percentages. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distribution were represented as M(range). The Kaplan-Meier method was used to calculate the cumulative survival rate and draw survivla curve. Results:(1) Preoperative examinations and tests: 40 patients received preoperative imaging examination and blood test. Of 40 patients, 33 cases underwent abdominal ultrasonography, 31 cases underwent abdominal computed tomography (CT) examina-tion, 21 cases underwent magnetic resonance imaging (MRI), 15 cases underwent endoscopic retrograde cholangiopancreatography (ERCP), 8 cases underwent position emission tomography CT examination, 6 cases underwent endoscopic ultrasonography; some patients underwent multiple examinations. The main imaging features of IPNB were bile duct dilatation, and intraluminal tumor. Enhanced CT scan showed tumor reinforcement. Preoperative blood tests showed of the 40 patients, 21 cases with abnormal liver function, 17 cases with increased bilirubin, 9 cases with increased carcinoembryonic antigen, and 24 cases with increased CA19-9. (2) Treatment: 35 of 40 patients underwent surgery, 5 patients underwent ERCP and biopsy and didn′t undergo surgery based on their willings. Of 35 patients with surgeries,20 cases underwent hemihepatectomy or lobectomy, 8 cases underwent pancreatico-duodenectomy, 7 cases underwent bile duct tumor resection. The operation time was (262±91)minutes, and volume of intraoperative blood loss was 300 mL(range, 50?2 000 mL). Postopera-tive complications occurred in 6 of 35 patients, including 3 cases with Grade Ⅰ complications and 3 cases with Grade Ⅱ complication according to Clavien-Dindo classi-fication system. (3) Pathological examination: 40 patients were diagnosed as IPNB by pathological examinations. There were 19 and 21 patients with extrahepatic and intrahepatic lesions, respectively. There were 20 benign lesions (15 cases of low or intermediate-grade intraepithelial neoplasia and 5 cases of high-grade intraepithelial neoplasia) and 20 malignant lesions of invasive carcinoma. There were 18 cases with mucus secretion and 22 cases without mucus secretion or information. Five of 35 patients with surgeries had positive margin and the rest of 30 patients had negative margin. A total of 154 lymph nodes were dissected in 21 patients, including 3 positive lymph nodes. (4) Follow-up: 35 of 40 patients were followed up for (53±35)months. Seventeen of 35 patients survived without tumor, and 3 patients survived with tumor of which the time to tumor recurrence were 12, 17, 37 months. Fifteen patients died, with the time interval to death of (30±19)months. The 1-, 3-, and 5-year cumulative survival rates of 35 patients were 88.6%, 73.6%, and 50.7%, respectively.Conclusions:IPNB is rare, with the main imaging features as bile duct dilatation, and intraluminal tumor. The tumor is reinforce-ment after enhanced scan. Surgery is the main treatment for IPNB and lymph node metastasis is rare.