ObjectiveTo analyze the prevalence and risk factors of metabolic syndrome (MS) after adult liver transplantation (LT) recipients. MethodsThe clinicopathologic data of patients with survival time ≥1 year underwent LT in the People’s Hospital of Zhongshan City from January 1, 2015 to August 31, 2020 were analyzed retrospectively. The logistic regression model was used to analyze the risk factors affecting MS occurrence after LT, and the receiver operating characteristic (ROC) curve was used to evaluate the optimal cutoff value of the index of predicting MS occurrence and its corresponding evaluation effect. ResultsA total of 107 patients who met the inclusion criteria were collected in this study. Based on the diagnostic criteria of MS of Chinese Medical Association Diabetes Association, the occurrence rate of MS after LT was 32.7% (35/107). Multivariate logistic regression analysis showed that the increased age of the recipient [OR (95%CI)=1.106 (1.020, 1.199), P=0.014], preoperative increased body mass index [OR (95%CI)=1.439 (1.106, 1.872), P=0.007] and blood glucose level [OR (95%CI)=1.708 (1.317, 2.213), P<0.001], and with preoperative smoking history [OR (95%CI)=5.814 (1.640, 20.610), P=0.006] and drinking history [OR (95%CI)=5.390 (1.454, 19.984), P=0.012] increased the probability of MS after LT. The areas under the ROC curve (AUC) corresponding to these five indexes were 0.666, 0.669, 0.769, 0.682, and 0.612, respectively. The corresponding optimal cutoff values of three continuous variables (recipient’s age, preoperative body mass index, and blood glucose level) were 53 years old, 23.1 kg/m2, and 6.8 mmol/L, respectively. The AUC of combination of the above five indexes in predicting occurrence of MS was 0.903 [95%CI (0.831, 0.952)], and the sensitivity and specificity were 80.0% and 90.3%, respectively. ConclusionsIncidence of MS after adult LT recipient is not low. For recipients with preoperative hyperglycemia, obese, elderly, histories of drinking and smoking before LT need to pay attention to the early detection and early intervention of MS.
Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic at the end of December 2019, more than 85% of the population in China has been infected. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mainly affects the respiratory system, especially the lungs. The mortality rate of patients with severe infection is high. A percentage of 6% to 10% of patients will eventually develop into COVID-related acute respiratory distress syndrome (CARDS), which requires mechanical ventilation and extracorporeal membrane oxygenation (ECMO) support. Some patients who survive acute lung injury will subsequently develop post COVID-19 pulmonary fibrosis (PCPF). Both fully treated CARDS and severe PCPF are suitable candidates for lung transplantation. Due to the special course, evaluation strategies are different from those used in patients with common end-stage lung disease. After lung transplantation in COVID-19 patients, special treatment is required, including standardized nucleic acid testing for the novel coronavirus, adjustment strategy of immunosuppressive drugs, and rational use of antiviral drugs, which is a big challenge for the postoperative management of lung transplantation. This consensus was evidence-based written and was reached by experts after multiple rounds of discussions, providing reference for assessment and postoperative management of patients with interstitial pneumonia after COVID-19 infection.
Objective To explore effects of edaravone on apoptosis and expressions of apoptotic proteins Smac and XIAP in hippocampal CA1 pyramidal cell of rats under intermittent hypoxia. Methods A total of 96 adult male Wistar rats were randomly divided into control group, 5% intermittent hypoxic group and edaravone group, and each group was divided into 4 time groups at 7 d, 14 d, 21 d and 28 d, respectively, with 8 rats in each subgroup. The content of reactive oxygen species (ROS) in hippocampal tissues of the experimental rats was detected by the reactive oxygen species detection kit. Immunohistochemistry and Western blot were used to detect the expressions of Smac and XIAP protein in hippocampal CA1 region. The Tunel method detected the apoptosis of neurons. Results Compared with the control group, the content of ROS, the expressions of Smac and XIAP proteins and the neuronal apoptosis index in the hippocampus were increased in the 5% intermittent hypoxia group and the edaravone group at each time point (all P<0.05). The content of ROS, the Smac protein expression and the neuronal apoptosis index in the edaravone group were significantly lower than those in the 5% intermittent hypoxia group (all P<0.05). The expression of XIAP protein in the edaravone group was significantly higher than that in the 5% intermittent hypoxia group (P<0.05). Conclusion Edaravone may improve the antioxidant capacity of the body by scavenging oxygen free radicals and regulate Smac and XIAP- mediated apoptosis, thus playing a protective role on neurons.
ObjectiveTo investigate therapeutic method, curative effect, and prognosis of inferior vena cava (IVC) blocking Budd-Chiari syndrome (BCS) with thrombosis. MethodsClinical data of 128 BCS patients with membranous or short-segment occlusion of IVC as well as IVC thrombosis, who accepted interventional treatment in The Affiliated Hospital of Zhengzhou University from Apr. 2004 to Jun. 2012, were retrospectively analyzed. Comparison of the difference on effect indicators between predilation group and stent filter group was performed. ResultsThereinto, 9 patients with fresh IVC thrombosis were treated with agitation thrombolysis (agitation thrombolysis group), 56 patients were predilated by small balloon (predilation group), for the rest 63 patients, a stent filter was deployed (stent filter group). Besides 1 stent filter fractured during the first removal attempt and had to be extracted surgically in the stent filter group (patients suffered with sent migration), in addition, the surgeries of other patients were technically successful without procedure-related complication. effect indicators were satisfactory in all patients, and there were no statistical differences between predilation group and stent filter group in dosage of urokinase, urokinase thrombolysis time, hospital stay, and incidence of complication (P > 0.05), but the cost of predilation group was lower than that of stent filter group (P < 0.01). All of the 128 patients were followed-up postoperation, and the duration range from 18 to 66 months with an average of 44.2 months. During the follow-up period, reobstruction of the IVC was observed in 13 patients without thrombosis, of which 1 patient in agitation thrombolysis group, 6 patients in predilation group, and 6 patients in stent filter group. There was no significant difference in recurrence rate between predilation group and stent filter group (P > 0.05). Patients with recurrence got re-expansion treatment, and no stenosis or thrombogenesis recurred. ConclusionsAgitation thrombolysis for fresh IVC trombosis in the patients with BCS is safe and effective. Predilation and stent filter techniques are all effective in the treatment of BCS with chronic IVC thrombosis, but the former technique seems to be more economic.
ObjectiveTo study SCN1A gene mutations and their inheritance in patients with Dravet syndrome (DS), and to analyze the phenotypes of their family members. MethodsGenomic DNA was extracted from peripheral blood samples from DS patients and their parents. SCN1A gene mutations were screened using PCR-DNA sequencing and multiplex ligation-dependent probe amplification (MLPA). Results547 DS patients were collected, SCN1A gene mutations were identified in 379 patients (69.3%), which included 179 missense mutations (47.2%), 78 nonsense mutations (20.6%), 77 frameshift mutations (20.3%), 37 splice site mutations (9.8%), and 8 cases with SCN1A gene fragment deletions or duplications (2.1%). Of 379 DS patients, the parents of 354 DS patients were further analyzed, the de novo mutations accounted for 92.9%, inherited mutations accounted for 7.1%, and in 5 of the latter families, the SCN1A-positive parent carried a somatic mutations mosaicism. For the 25 parents carrying SCN1A mutations, 1 had DS, 11 had febrile seizures plus, 9 had febrile seizures, whilst 4 were normal. ConclusionsThe mutation rate of SCN1A in DS patients is high. Most mutations are of missense and truncation mutations (including nonsense mutation and frameshift mutation). Only a few patients have carried fragment deletions or duplications. Most SCN1A mutations are de novo, only a few are inherited from the parents. SCN1A mutations carried by the parents can be in the form of mosaicism. The phenotypes of parents with SCN1A mutations can be severe, mild or normal, and a mosaic transmitting parent always shows mild or normal.
目的探讨Mirizzi综合征的诊断和治疗选择。方法对1982～2000年经手术证实的36例Mirizzi综合征进行回顾性分析。结果术前确诊仅8例(22.2%)，其中5例经ERCP确诊。36例分别选择性地施行了胆囊切除术、胆囊大部切除术、胆管瘘口修补术以及胆肠RouxenY吻合术。32例术后一期愈合，4例出现并发症，其中2例并发胆管狭窄而再次手术。结论B超结合ERCP检查可以提高Mirizzi综合征的术前确诊率， 手术治疗是其主要治疗方法，手术方式取决于局部病理损伤程度和解剖变异。
ObjectiveTo evaluate the safety and mid-to-long term outcomes of catheter-directed thrombolysis (CDT) in combination with percutaneous mechanical thrombectomy (PMT) followed by stent placement treatment for acute proximal deep vein thrombosis (DVT) complicated by iliac vein compression syndrome (IVCS), and to identify risk factors relevent to primary stent restenosis. MethodsA retrospective study was conducted. The patients diagnosed with acute proximal DVT and concurrent IVCS who underwent CDT in combination with PMT followed by stent placement at the First Affiliated Hospital of Chongqing Medical University from January 2018 to December 2021 were included. The demographics, clinical history, and procedural data were collected. The postoperative follow-up using color Doppler ultrasound were scheduled at 3, 6, and 12 months, and annually thereafter. The primary and secondary stent patency rates were evaluated. The univariate and multivariate Cox proportional hazards regression models were employed to assess risk factors for primary stent restenosis. ResultsA total of 188 patients who met the inclusion and exclusion criteria were enrolled, underwent CDT combined with PMT and stent implantation, and completed follow-up. During the follow-up, the restenosis occurred in 26 patients. The cumulative primary patency rates at 3, 6, 12, 24, 36, and 48 months after surgery were 100%, 98.9%, 92.5%, 88.3%, 86.7%, and 86.2%, respectively. The multivariate Cox proportional hazards regression analysis confirmed that a history of previous DVT [HR (95%CI)=4.21 (1.73, 10.28), P=0.002], implantation of two or more stents [HR (95%CI)=11.85 (1.66, 84.63), P=0.014], stent crossing the inguinal ligament [HR (95%CI)=9.92 (1.87, 52.78), P=0.007], and stent length [HR (95%CI)=0.98 (0.97, 0.99), P=0.003] were the affecting factors for primary restenosis. ConclusionsThe findings of this study suggest that CDT combined with PMT and stent implantation is a safe and effective strategy for treating acute proximal DVT complicated by IVCS. Close attention should be paid to the occurrence of restenosis in patients with two or more stents, stent crossing the inguinal ligament, and a history of previous DVT.
ObjectiveTo recognize and carry out early diagnosis for Cockayne syndrome (CS) as it is an extremely rare auto-recessive genetic syndrome characterized by multiple symptoms including growth failure and impaired development of the nervous system. MethodsHere we reported a case of typical CS with an unusual appearance. The 19-year-old young male patient was referred to West China Hospital on December 24th 2012. We analyzed the clinical characteristics of the patient and followed the literature review to help improve the knowledge on CS for clinicians. ResultsThe patient's parents were cousins. Laboratory data showed that lipoprotein profile, blood glucose and electrolytes, liver and renal function, as well as hormones (thyroxin, para-thyroxin, growth hormones, adrenocorticotropic hormone, corticosteroid) were all within normal limit. Electronic hearing examination showed moderate neural hearing loss. CT scan indicated multiple intracranial calcifications. The patient was definitely diagnosed with CS. He received nutritional support and symptomatic treatment but discharged due to lack of effective treatment. ConclusionCS is a progressive multisystem disorder characterized by a specific cellular defect in transcription-coupled repair. Typical features include developmental delay and impaired development of the nervous system. Typical clinical manifestations and imaging changes are helpful for clinical diagnosis of CS. Genotyping is necessary for patients with CS. Unfortunately, there is no ideal treatment for CS. Most of the patients with CS have poor prognosis.