ObjectiveTo analyze the risk factors of acute pancreatitis (AP) associated with hepatic dysfunction. MethodsWe collected the clinical data of AP patients from June 2012 to December 2014. According to the hepatic function, patients were divided into normal or mildly abnormal group (A), moderately abnormal hepatic function group (group B) and hepatic failure group (group C). We comparatively analyzed the relationship of abnormal hepatic function with causes (biliary, alcoholic, hypertriglyceridemia, and the others), age, gender, abdominal CT findings, laboratory test results, Ranson scores and the severity of acute pancreatitis bedside index (BISAP). ResultsA total of 117 AP patients were included in the study, including 75 males and 42 females aged from 28 to 71 years with the mean age of (53.21±1.35) years. There were 73 patients with mild AP (MAP), 28 with moderately severe AP (MSAP) and 16 with severe AP (SAP). There were 64 patients in group A, 37 in group B, and 16 in group C, in whom 73 patients were with hepatic dysfunction. The ratio of SAP and MSAP in group C was 75.0%, and MAP was 25.0%. There were 52 patients with bilious AP, 39 with alcoholic AP, 19 with hypertriacylglycerolemia AP, and 7 with others; the difference in causes among the three groups were not significant (P > 0.05). The differences in serum amylase, prothrombin time, white blood cells count, serum creatinine, triglycerides, total cholesterol, hemoglobin, and blood urea nitrogen among the three groups were not significant (P > 0.05). ConclusionsAP associated with hepatic dysfunction or failure may be caused by a various of factors. Whether the changes of serum amylase, prothrombin time, white blood cell count, serum creatinine, triglycerides, total cholesterol, hemoglobin, blood urea nitrogen, and BISAP scores can be the indexes of the judgment of AP with hepatic dysfunction needs more researches. Proper assessment of the related risk factors helps to prevent serious hepatic dysfunction or even failure.
【Abstract】ObjectiveTo explore the relationship between anomalous pancreaticobiliary ductal junction(APBDJ) and gallbladder carcinoma. MethodsThe current related literatures were reviewed.ResultsAPBDJ was associated with gallbladder carcinoma development. A proposed mechanism was free reflux of pancreatic juice into the gallbladder and molecular alterations of gallbladder epithelial cells.ConclusionAPBDJ is a high risk factor for gallbladder carcinoma. Prophylactic cholecystectomy is recommended for patients with APBDJ.
【摘要】 目的 探讨住院患者肝功异常病因及影像学诊断意义。方法 对2008年1月—12月住院的223例18～83岁肝功异常患者进行相关实验室检查,以及B超、CT和MRI检查。结果 肝脏本身疾病引起135例,占6054%,肝外疾病引起83例,占37.22%,原因不明5例,占2.24%。B超作为无创性检查,价格低亷,准确率高,可作为常规检查。结论 住院患者肝功损害病因复杂,以药物性肝炎、胆道系统疾病及脂肪肝较多见。
Objective To systematically review the clinical presentations and gene types of oculo-facio-cardio-dental (OFCD) syndrome and to provide a theoretical basis for future diagnosis, prevention, and treatment of the disease. Methods The PubMed, EMbase, The Cochrane Library, Web of Science, CBM, WanFang Data, and CNKI databases were electronically searched to collect studies on OFCD syndrome published from inception to March 1st, 2022. Two reviewers independently screened the literature, extracted data, and assessed the risk of bias of the included studies. A systematic review was then performed. Results A total of 19 studies involving 83 patients with OFCD syndrome were included. The patients had an average age of 15.95±16.03 years, including 5 males and 78 females. The clinical presentations mainly included ocular disorders, facial abnormalities, cardiac disorders, dental abnormalities, physical anomalies, and dysfunctions of other body systems. BCOR gene mutations were detected in 71 patients with OFCD syndrome (overall detection rate: 86%, 95%CI 78% to 93%), of whom five were males (detection rate: 6%, 95%CI 1% to 11%) and 66 were females (detection rate: 80%, 95%CI 71% to 88%). Patients were mostly treated using multidisciplinary symptomatic treatment approaches based on clinical presentations and imaging findings. Conclusion In addition to the typical clinical presentations, BCOR gene testing results should also be taken into consideration for the differential diagnosis of OFCD syndrome. Although symptomatic therapies in clinical practice are relatively mature, they do not address the underlying cause of the disease, i.e., BCOR gene mutations. In future research, greater attention should be diverted to gene therapy.
Object To evaluate the significance of double common bile duct （DCBD） in hepatobiliary surgery. Metheds The data of diagnosis and treatment of two patients with DCBD in our hospital between Jul. to Dec. 2010 were analyzed retrospective， and the related literatures were reviewed. Results The right hepatic bile duct of DCBD due to mistaking it for cystic duct in 1 case was accidental injuried during laparoscopic cholecystectomy. Another example，the DCBD was confirmed by intraoperative exploration and choledochoscopic examination， at the same time with chole-dochal cyst， anomalous pancreaticobiliary ductal junction （APBDJ）， primary hepatolithus， and choledocholith， and then operation was performed. Two cases were typeⅤb of DCBD. A total of 32 English literatures were reviewed. Since the beginning of 1932 English literature had reported 100 cases of DCBD. The type Ⅱand typeⅢwere the most common type of DCBD， and the typeⅤonly 10 cases. There were 27 cases of DCBD in twenty-five Chinese articles from 1994 to 2012. The typeⅤwas the most common type of DCBD. The accessory common bile duct （ACBD） opening in the duod-enum， gastric， and pancreatic duct were the most common. The common complications included stone， APBDJ， choled-ochal cyst， tumor etc. Conclusions DCBD is a very rare anatomic variation of extrahepatic bile duct， often accompanied by calculus of bile duct and common bile duct cyst， APBDJ， and other biliary anatomy abnormality， and potentially carci-nogenic potential. The existence of DCBD may increase the risk of iatrogenic bile duct injury and complexity of biliary operation. In view of this， this abnormality of extrahepatic duct should be paid with close attention during operation.
Congenital heart disease (CHD) is a series of structural cardiac abnormalities resulting from abnormal fetal heart development. With the prolongation of survival time, their cognitive function problems begin to be concerned. From fetus period to adulthood, people with complex CHD are more likely to have abnormalities in brains. Children with complex CHD have a significantly increased risk of developmental disorders in cognitive functions, such as intelligence, attention and psychosocial disorders. These diseases persist into their adulthood. Adults with CHD have earlier neurocognitive decline, poorer performance in intelligence, executive function, attention and academic achievement, and are more likely to have mood disorders, higher incidence of mental disorders and lower quality of life. This paper reviews the studies on cognitive function of adult patients with CHD from the dimension of the whole life cycle.
Objective To detect and analyse the mutations in rhodopsin gene of members in a family affected by autosomal dominant retinitis pigmentosa (ADRP). Methods Using the polymerase chain reaction (PCR), we amplified exon 1-5 of rhodopsin gene in patients with ADRP,and analyzed it with direct sequence measuement. Results The Gly-182-Asp mutation in the rhodopsin gene was detected in most of affected members of this ADRP family, but no mutation was detected in two affected members and the control ones. Conclusion We cannot regard the Gly-182-Asp mutation in the rhodopsin gene as the pathagenic factor of the ADRP family. It is likely there is a new gene next to the rhodopsin gene. (Chin J Ocul Fundus Dis, 2002, 18: 256-258)