Objective To investigate the method and effectiveness of wide local excision combined with Mohs micrographic surgery for dermatofibrosarcoma protuberans (DFSP). Methods Between January 2007 and January 2010, 17 patients with DFSP were treated. There were 9 males and 8 females with an average age of 33.2 years (range, 16-55 years). Thelesions were located at head and neck (2 cases), trunk (12 cases), extremity (2 cases), and perineal region (1 case). There were 6 cases of primary DFSP and 11 cases of relapsed DFSP. The lesions presented as single or multitude nodules or fusion nodules with skin withering, scar, en plaque in the center and with ill-defined margins. The diameter of lesions ranged from 0.8 to 9.7 cm (mean, 4.3 cm). No distant metastasis or lymphatic metastasis occurred in all cases. After tumors resection by wide local excision combined with Mohs micrographic surgery, the wounds were repaired by direct suture in 3 patients, skin grafting in 9 patients, and local skin flap in 5 patients. Results Wide local excision and Mohs micrographic surgery were carried out once in 13 patients, twice in 3 patients, and three times in 1 patient with an average operation time of 98.6 minutes (range, 56-219 minutes). Primary heal ing of wound and donor site were achieved with no necrosis of skin grafting and skin flap. All patients were followed up 8-34 months (mean, 21.7 months) with no recurrence. Conclusion Wide local excision combined with Mohs micrographic surgery could treat DFSP, which has the advantages of shorter operation time, radical resection, and less injury.
Objective To evaluate the efficacy of statins pretreatment in patients before percutaneous coronary intervention (PCI). Methods Published literature on relevant randomized controlled trials (RCTs) were retrieved via electronic and handsearch in databases CNKI, CBM, MEDLINE and The Cochrane Library from January 1990 to May 2011. The references of these articles were also retrieved. Two reviewers independently identified articles according to the inclusion and exclusion criteria, extracted the data, assess the quality of the included studies, and then conducted meta-analysis using RevMan 5.0 software. Results A total of 10 trials involving 3 012 patients were included. The results of meta-analyses showed that: during the periprocedural period, the trial group had a lower incidence than the control group (98 of 1 514 cases, incidence 6.5%) in periprocedural myocardial infarction with a significant difference (OR=0.43, 95% CI 0.34 to 0.56, Plt;0.000 01). The composite of death, myocardial infarction, or target vessel revascularization in one month, essentially driven by periprocedural myocardial infarction, was reported 6.8% in the trial group and 15.1% in the control group (OR=0.41, 95% CI 0.32 to 0.53, Plt;0.000 01). Conclusion Current evidence supports the effectiveness of statin pretreatment used to reducing the rate of periprocedural myocardial infarction in patients before receiving PCI.
Objective To evaluate hepatic functional reserve and investigate the clinical value through measuring hepatic functional blood flow by D-sorbitol clearance rate and liver volume changes with CT. Methods Ninety-two patients with portal hypertension due to posthepatic cirrhosis were investigated (cirrhosis group). Twenty healthy volunteers were used as control group. D-sorbitol was infused intravenously at a steady rate. Blood and urine were collected and recorded once before infusion and at 120, 150 and 180 min after infusion, and their concentrations of D-sorbitol were examined by enzyme spectrophotometry. From pharmacokinetic equations, hepatic clearance rate of D-sorbitol (CLH) was calculated. Total hepatic blood flow (QTOTAL) was measured by Doppler sonography, intrahepatic shunt rate (RINS) was obtained. The liver volume change rate was obtained in patients with cirrhosis through the abdominal CT scan. The relations among the indicators, Child classification and postoperative complications were studied. Results After D-sorbitol was infused intravenously for 120 min, the plasma concentration was at the steady state. The plasma concentration was (0.189±0.05) mmol/L in control group and (0.358±0.06) mmol/L in cirrhosis group (Plt;0.01). CLH was (1 248.3±210.5) ml/min in control group and (812.7±112.4) ml/min in cirrhosis group (Plt;0.01). Although QTOTAL in cirrhosis group was declined, compared with the control group 〔(1 280.6±131.4) ml/min vs. (1 362.4±126.9) ml/min〕, Pgt;0.05, while RINS increased markedly 〔(36.54±10.65)% vs. (8.37±3.32)%, Plt;0.01〕. In cirrhosis group, the mean liver volume of Child A, B and C patients were (1 057±249) cm3, (851±148) cm3 and (663±77) cm3 respectively. There were significant differences among the mean liver volume (Plt;0.05). The liver volume was significantly smaller in Child B and C patients than that in Child A (Plt;0.05, Plt;0.01). When CLH was less than 600 ml/min, and liver volume decreased by more than 40%, postoperative complications increased significantly. CLH and the liver volume change rate were not in absolutely good accordance with Child classification. Conclusion The hepatic clearance of D-sorbitol and the quantitative determination of the liver volume with CT can be an objective evaluation of the liver metabolism of the inherent capacity and the hepatic functional blood flow changes. It contributes to the correct understanding of the hepatic functional reserve and lay the foundation for determining a reasonable treatment plan, surgical methods and time.
ObjectiveTo investigate the relationship between PIGA gene mutation and early-onset epileptic encephalopathy (EOEE).MethodsThe clinical materials of a EOEE children with PIGA gene mutation who admitted to Guangdong Sanjiu Brain Hosipital Epilepsy Pediatric in March 2016 were retrospectively analyzed. The databases of Wanfang, CNKI and PubMed were also reviewed to give a summary.ResultsThe child’s onset age was before 1-year-old, who has a family history of epilepsy. Electrophysiological and clinical diagnosis were EOEE, auxiliary examination of genetic metabolism, urine organic acids, blood biochemistry and other tests showed no abnormalities. Epilepsy gene detection found that PIGA gene has a new missense mutation, in line with the X-linked inheritance. The mutations leading to EOEE has been reported in foreign literature, but rarely reported in China.ConclusionThe new mutations of X-linked PIGA gene are more likely to be the causative genes of some.
ObjectiveTo investigate the relationships between the onset age, genotype, clinical phenotype and the efficacy of Rapamycin in patients with tuberous sclerosis complex.MethodsRetrospectively analyze the clinical data of patients with tuberous sclerosis complex (TSC) who were diagnosed with epilepsy in Guangdong Sanjiu Brain Hospital from October 2013 to December 2018. Meanwhile, the relationships between the onset age of epilepsy and genotype, clinical phenotype and Rapamycin efficacy were analyzed comprehensively.ResultsTSC gene was detected in 104 patients with tuberous sclerosis complex, of which 85 (81.7%) were positive and 44 (51.8%) were males as well as 41 (48.2%) were females, with an average age of (4.0±4.9) years old. And there were 34 (40.0%) TSC1 mutations and 51 (60.0%) TSC2 mutations. The patients were divided into 3 groups according to their ages: ≤1 year old, 1 ~ 6 years old and ≥6 years old. Among them, 31 cases (36.5%) were in the ≤1 year old group, 31 cases (36.5%) in the 1 ~ 6 years old group and 23 cases (27.0%) in the ≥6 years old group. Through statistical analysis, we found that the onset age of epilepsy in patients with TSC1 and TSC2 gene mutations was statistically different (χ2=9.030, P=0.011). Further analysis of the relationship between the onset age of epilepsy and other clinical phenotypes showed that there were statistical differences in the probability of mental retardation and spasm seizure in different onset age groups of epilepsy (P<0.05). In addition, patients with epilepsy onset age ≤1 year old are more likely to have renal disease and patients with epilepsy onset age ≥6 years old are more likely to have SEGAs. There was no significant difference between the onset age of epilepsy and the efficacy of Rapamycin (P>0.05).ConclusionTSC2 mutation, mental retardation and spasm seizure are more likely to occur in patients with epilepsy onset age ≤1 year old. The study on multiple factors of epilepsy onset age may have a certain guiding role in judging the development and prognosis of TSC with epilepsy.
ObjectiveTo explore the efficacy of ketogenic diet on developmental and epileptic encephalopathy caused by PIGA gene mutation. Method A retrospective analysis was conducted on patients with developmental and epileptic encephalopathy admitted to Guangdong Sanjiu Brain Hospital from March 2016 to June 2020. Patients with positive PIGA gene mutations were selected, and their clinical characteristics and treatment effects were analyzed. ResultA total of 6 epilepsy patients with positive PIGA gene mutations were collected, all of whom were male. 5 cases were heterozygous mutations originating from the mother, and 1 case was a new mutation. All 6 patients were accompanied by varying degrees of psychomotor developmental delay, various types of epileptic seizures, multifocal discharge on EEG, and varying degrees of brain hypoplasia indicated by cranial MRI. All 6 patients met the criteria for drug-resistant epilepsy and were recommended to undergo ketogenic diet treatment, but three patients were discontinued in the early stages. Among them, Case 3 experienced hyperlipidemia on the fifth day of ketogenic diet and was discontinued, while Case 5 experienced transient hypoglycemia on the second day and the family refused to use it. Case 6: After one week of ketogenic diet, the family members voluntarily stopped using it. Only three patients adhered to a long-term ketogenic diet for more than 2 years. The efficacy of ketogenic diet treatment in cases 1 and 4 was very significant, reaching a seizure free state. Case 2 showed a 50% reduction in seizure frequency after ketogenic diet treatment. Case 4 developed hyperlipidemia after two years of ketogenic diet, and after discontinuing the ketogenic diet for about two months, the blood lipids returned to normal. Comparing patients in the ketogenic group with those in the non ketogenic group, it was found that the ketogenic group had a clear therapeutic effect after treatment. Among them, two patients had no seizures for more than a year and showed significant progress in development compared to before. Two years after ketogenic diet treatment, the EEG showed a significant decrease or disappearance of epileptic discharge compared to before. ConclusionPatients with developmental latency caused by PIGA gene mutations have an early only age, diverse types of sizes, varying degrees of psychomotor developmental delay, and some are compatible by von as possible.
Objective To formulate an individualized evidence-based treatment for a first-diagnosed patient with coronary artery-pulmonary artery fistula. Methods Aiming at the issue of whether interventional operation was necessary for first-diagnosed coronary artery-pulmonary artery fistula or not, the computer retrieval was conducted in the US National Guideline Clearinghouse, The Cochrane Library, PubMed and MEDLINE from 1990 to 2011, to collect and assess the best evidence of relevant systematic reviews, randomized controlled trials, controlled clinical trials and treatment guidelines, in order to be applied in clinical treatment. Results There were 1 clinical guideline for treating coronary artery fistula and 3 different high-quality evidence studies were retrieved. The results showed percutaneous coronary intervention was the best treatment currently. According to the obtained evidence and patient’s willingness, the relevant examinations were taken, and the preoperative preparation for percutaneous coronary intervention was done actively after the patient was admitted. Three days after hospitalization, the selective coronary angiography showed right coronary artery-pulmonary artery fistula and left coronary circumflexus artery-left atrial multiple fistulae, then the percutaneous coronary intervention spring coil embolization was successfully conducted for right coronary artery-pulmonary artery fistula. After operation, bayasprin enteric-coated tablets 0.1 g/d was taken for anti-platelet aggregation and preventing thrombotic diseases. The observation during operation and postoperative 5-day hospitalization showed no relevant complications. Conclusion Percutaneous coronary intervention is safe and effective for the symptomatic patients with coronary artery-pulmonary artery fistula.
Objective To compare two kinds of myofascial flap encapsulating adi pose-derived stromal cells (ADSCs) in adi pogenic efficacy in vivo, and to provide experimental basis for the efficient transplantation of free adi pose tissue. Methods ADSCs were isolated from the subcutaneous adipose tissue in the neck of 10 New Zealand rabbits (aged 3-4 months old, male and female, weighing 2.0-2.5 kg), and primary culture and subculture of ADSCs were conducted. When the cells at passage 3 covered 70%-80% of the bottom of the culture flask, BrdU (10 μg/mL) was appl ied to label the cells for 48 hours before performing immunofluorescence staining. Oil red O staining observation was conducted to thosecells 2 weeks after being induced towards adi pocyte, al izarin red staining observation was performed 3 weeks after being induced towards osteoblast, and alcian blue staining was conducted 2 weeks after being induced towards chondrocyte. Besides, after being induced towards adipocyte for 2 weeks, 1 × 107 ADSCs/piece at passage 3 labeled by BrdU was seeded into Col I (10 mm × 10 mm × 5 mm/piece) to prepare cell carrier complex. The experiment was divided into two groups: group A in which vascular pedicled dextral latissimus dorsi fascial flap was adopted to encapsulate the complex; group B in which dextral gluteus maximus fascial flap with no specific vessel pedicle was appl ied to encapsulate the complex. Rabbits in each group went through autogenous ADSCs transplant and self control. The implants were dislodged 8 weeks after operation, HE staining and immunohistochemistry staining were performed to testify cambium, the wet weight and micro vessel count of the cambium in each group were tested, immunofluorescence staining was performed to determine the origin of cambium and microvascular endothel ium. Results The nucleus of ADSCs positive for BrdU label ing showed green fluorescence under fluorescence microscope, with the positive label ing ratio of ADSCs above 90%. For ADSCs at passage 3, the formation of red l ipid droplets within cells was observed 2 weeks after being induced towards adipocyte, red calcium nodules were evident 3 weeks after being induced towards osteoblast, and highly congregated cell mass positive for alcian blue staining appeared 2 weeks after being induced towards chondrocyte. Eight weeks after operation, neogenetic blood vessel grew into scaffolds and no obvious fibreencapsulation was observed in group A, while few blood vessel grew into scaffolds in group B. The wet weight of cambium in group A and B was (0.149 5 ± 0.017 3) g and (0.095 3 ± 0.012 7) g, respectively, indicating there was a significant difference between two groups (P lt; 0.01). HE staining showed the formation of neogenetic adipose tissue and the growth of micrangium in the implant, and the degradation and absorption of scaffold. The micro vessel count of group A and B was 31.2 ± 4.5 and 19.3 ± 2.6, respectively, indicating there was a significant difference between two groups (P lt; 0.01). Eight weeks after operation, the immunofluorescence staining of cambium showed that the cell nucleus of regenerated adi pocytes and partial capillary endothel ium in groups A and B presented green fluorescence. Conclusion ADSCs encapsulated by vascular pedicled latissimus dorsi fascial flap and collagen protein scaffold complex has a higher adi pogenic efficacy in vivo than the gluteus maximus fascial flap with no specific vessel pedicle.
Objective To investigate the expression of human leukocyte antigen-G （HLA-G） in hepatocellular carcinoma （HCC） tissues， and to evaluate the prognosis of patients after liver transplantation. Methods The clinical data of 83 patients with HCC who underwent liver transplantation from January 2004 to May 2008 in the Liver Transplantation Center of the Third Affiliated Hospital of Sun Yat-sen University were analyzed retrospectively. The expression of HLA-G in HCC tissues was detected by using immunohistochemical analysis. The Kaplan-Meier method was used to evaluate the cumulative survival rate and tumor-free survival rate. Log-rank test and Cox regression model were used to analyze the single and muti-factor for tumor-free survival rate, respectively. Results Among the 83 patients，there were tumor recurrence in 35 patients （42.2%）. The 1-year，3-year, and 5-year of cumulative survival rate was 97.2%，89.8%, and 43.1%, respectively. The 1-year，3-year， and 5-year of tumor-free survival rate was 93.6%，68.9%, and 38.7%, respectively. The positive rate （68.7%） of HLA-G expression in HCC tissues was significantly higher than that in paracancerous tissues （15.7%）, P＜0.01. A significant association was found between expression of HLA-G and tumor size， vascular invasion, and pathology differentiation （P＜0.05）. Single factor analysis showed that the expression of HLA-G （P＜0.01）， tumor size （P＜0.05）， vascular invasion （P＜0.01）， and pathology differentiation （P＜0.01） effected on tumor-free survival rates of HCC patients after liver transplantation. The tumor-free survival rate in positive expression of HLA-G group was significantly lower than that in negative expression of HLA-G group （P＜0.01）. Cox regression model analysis showed that the expression of HLA-G （P＜0.05）， vascular invasion （P＜0.01）， and pathology differentiation （P＜0.05） were independent risk factors that affected the tumor-free survival rate of HCC patients after liver transplantation. Conclusions There is expression of HLA-G in HCC tissues. The independent risk factors that affecting the tumor-free survival rate of HCC patients after liver transplantation include the expression of HLA-G， vascular invasion, and pathological differentiation. Taking interferential measures for patients with positive expression of HLA-G and strict selection of indication of liver transplantation for HCC can reduce the recurrence rate of tumor.
Objective To study the effect of anti-CD40L monoclonal antibody on the rejection of rat pancreatic islet xenografts and its mechanism. Methods The animal models of human-rat pancreatic islet xenografts were established and were treated with anti-CD40L monoclonal antibody. The levels of blood glucose of transplantation rats were measured and the survival of grafts and transplantation rats were observed after transplantation. The morphological changes of grafts were observed and the levels of cytokines (IL-2 and TNF-α) were quantified by ELISA. Results ①Level of blood glucose in all the rats with diabetes decreased to normal on day (2.3±0.2) after transplantation. The average level blood glucose of control group began to increase on day (8.1±0.6), while the treatment group began to increase on day (18.5±1.2) after transplantation, which was significantly postponed compared with control respectively (P＜0.01). ②Grafts of treatment group and control group survived for (22±8.2) and (10±2.1) days respectively. Survival of grafts in treatment group was significant longer than that in control group (P＜0.01). ③Survival of transplantation rats were (35±6.5) and (21±5.7) days in treatment group and control group respectively. The survival of transplantation rats in treatment group was significant longer than that in control group (P＜0.05). ④Levels of serum IL-2 and TNF-α in control group increased dramatically within (3.2±0.3) days and reached peak within (7.3±0.5) days after transplantation, which were significantly higher than those measured before transplantation (P＜0.01); While in treatment group, the levels of serum IL-2 and TNF-α began to increase on day (22.6±1.7) after transplantation, and reached peak on day (28.5±2.2), which was significantly postponed than those in control group (P＜0.01). Conclusion Anti-CD40L monoclonal antibody can inhibit the rejection of rat pancreatic islet xenografts and prolong the survival time of transplantation rats and grafts.