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@#Objective To evaluate the survival results of surgical resection (SR) and CT-guided percutaneous ablation (PA) for stageⅠnon-small cell lung cancer (NSCLC). Methods The PubMed, Web of Science, EMbase, The Cochrane Library, CNKI, VIP, Wanfang databases from inception to June 2021 were searched to collect comparative studies on the survival results between SR and CT-guided PA treatment for stageⅠNSCLC. RevMan 5.3 software was used for statistical analysis of data. Results A total of 3 114 patients were included in 11 studies. The results of meta-analysis showed that compared with the PA group, the SR group had a higher 2-year postoperative overall survival (OS) rate (OR=1.44, 95%CI 1.00-2.06, P=0.05), 3-year postoperative OS rate (OR=2.37, 95%CI 1.47-3.81, P<0.001), 5-year OS rate (OR=1.64, 95%CI 1.19-2.28, P<0.01), 5-year progression-free survival rate after operation (OR=2.43, 95%CI 1.54-3.82, P<0.001) and lower local recurrence rate (OR=0.26, 95%CI 0.13-0.54, P<0.001). There were no statistical differences between the two groups in terms of 1-year postoperative OS rate, 1-year, 2-year, and 3-year tumor-related survival rates, 1-year, 2-year tumor-free survival rates, or distant postoperative recurrence rate (P>0.05). Conclusion For patients with stageⅠNSCLC with optimal basic conditions, surgery is a more appropriate treatment. For patients who cannot withstand surgical injuries or refuse surgery, CT-guided PA is also a potential alternative treatment. However, this conclusion needs to be verified by prospective controlled trials with larger sample sizes and a more rigorous design.
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Objective:To investigate the clinical characteristics of cerebral hemorrhage caused by tentorial dural arteriovenous fistula (TDAVF).Methods:An unusual TDAVF case admitted to the Department of Neurology, Qilu Hospital, Shandong University in March 2020, complicated with hypertension with successive bilateral basal ganglia hemorrhage in short term was reported. The characteristics of cerebral hemorrhage caused by TDAVF reported in the literature were summarized and analyzed.Results:Digital subtraction angiography (DSA) revealed that there was arteriovenous fistula in the tentorial foramen area of this patient (male, 33 years old), and the TDAVF was fed by the right meningohypophyseal trunk, bilateral middle meningeal artery and posterior cerebral artery. A shunted pouch was present in the tentorial foramen area, and retrograde reflux drainage was seen in the deep venous system, from the meningeal vein to superior sagittal sinus or sigmoid sinus. Transarterial embolization was performed and subsequently DSA showed obliteration of the fistula. This patient experienced no clinical decline or rehemorrhage during the 12 months follow-up period. Forty-one cases of TDAVF with hemorrhage of cerebral parenchyma which were reported before March 30, 2021 with detailed clinical and imaging data were summarized. The average age of onset of this group of patients was 57.2 years, and the ratio of male to female was about 3∶1. The hemorrhage was located in superior of the tentorium in 17 cases (41%), while in inferior of the tentorium in 24 cases (59%). Supratentorial intracerebral hemorrhage mainly occurred in occipital lobe and thalamus. DSA showed that the arteriovenous fistula was classified as Borden type Ⅲ or Cognard type Ⅳ in 36 cases (88%). Twenty-nine patients (71%) underwent a single surgical procedure, while 12 cases (29%) underwent combined surgical or other treatments. Overall, 37 patients (90%) achieved angiographically documented obliteration of the fistula and 39 patients (95%) experienced good or excellent outcomes.Conclusions:TDAVF often presents as cerebral parenchymal hemorrhage which is common in supratentorial region, but rare in basal ganglia region. The cause of cerebral hemorrhage in patients with hypertension may not be attributed to hypertension. Early diagnosis and intervention are of great significance to improve the prognosis of patients.
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Objective:To summarize the infective characteristics of Fournier's gangrene (FG) and evaluate the effect of negative pressure wound therapy (NPWT).Methods:A retrospective case control study was conducted to analyze the clinical data of 31 patients with FG admitted to Peking University First Hospital from May 2010 to September 2020, including 29 males and 2 females, aged 21-78 years [(55.2±2.0)years]. A total of 29 patients were caused by infectious diseases of the perianal and urinary system, and the rest two patients were caused by vulvar infection and retroperitoneal abscess. A total of 23 patients were treated with NPWT (Group A) and 8 patients were treated with conventional dressing (Group B). Characteristics of pathogen, drug-resistance rate, medical treatment and prognosis for all patients were summarized. The hospitalization duration, numbers of operation and wound healing time were compared between two groups.Results:Monomicrobial infection was identified in 14 patients, while polymicrobial infection in 15 patients, fungal infection in 1 and culture-negative in 1. Escherichia coli, Enterococcus faecalis, Enterococcus faecium, Klebsiella pneumoniae and Staphylococcus haemolyticus were the most common pathogenic bacteria. The resistance rate of gram-negative bacilli to third-generation cephalosporins was 37%. Staphylococcus haemolyticus were methicillin-resistant Staphylococcus. The carbapenem antibiotics combined with vancomycin antibiotics were used for all patients as the empirical anti-infection treatment. Three patients died, and the rest 28 patients were followed up for 3 to 12 months [(10.8±2.6)months] after discharge. All the wounds were healed well without recurrence. In Group A and Group B, the hospitalization duration was (37.4±15.0)days and (47.0±16.0)days, respectively ( P>0.05); the number of operation was 3(3, 6) times and 13(4, 17)times, respectively ( P<0.05); the wound healing time was (38.9±17.8)days and (61.8±14.2)days, respectively ( P<0.05). Conclusions:Enterobacteriaceae, Enterococcus and Staphylococcus haemolyticus are the most common pathogenic bacteria for FG, among which the proportion of drug-resistant bacteria is relatively high. NPWT is an effective adjuvant therapy for wound management with reduced operation times and short wound healing time compared to conrentional method.
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To summarize the status of immediate breast reconstruction (IBR) after mastectomy in Beijing City, Tianjin City and Hebei Province. A retrospective analysis was made on the data of 382 cases with breast cancer who were treated and followed up successfully with immediate breast reconstruction after mastectomy from January 2012 to December 2016 in Beijing City, Tianjin City and Hebei Province. Clinic data of the followed-up 382 cases (all female, age (38.5±4.2) years (range: 24 to 70 years)), including general information, tumor information, sugery methods, and treatments after surgery were collected. The survival status, metastasis,complications and prognosis were followed up. Cosmetic effcet was evalated by Harris method, and life quality by Functional Assessment of Cancer Therapy-Breast scale (FACT-B). χ(2) test was used to compare the difference between year 2012 and year 2013 to 2016. Bonferroni method was used to correct the inspection level, which was 0.05/10=0.005. The trend of IBR rate (ratio of IBR to modified radical mastectomy) from 2013 to 2016 was analyzed by trend χ(2) test. There was 46 cases in stage 0, 152 cases in stage Ⅰ, 165 cases in stage Ⅱ, 19 cases in stage Ⅲ. Twenty-five cases was treated by neoadjuvant chemotherapy, 231 by chemotherapy and 35 by radiotherapy. The proportion of implant reconstruction was 48.7% (186/382), more than expanded of 21.5% (82/382), with latissimus dorsi of 12.0% (46/382), TRAM of 8.9% (34/382), DIEP of 2.1% (8/382), and latissimus plus implant of 6.8% (26/382). According to the Harris standard, the excellent and good rate of the cosmetic effect of the reconstructed breast was 93.7%. The score of FACT-B was 108.20±16.9 (range: 67 to 144) 1 year postoperatively. Compared with 2012, the IBR rate was significant increased, till 2015, the IBR rate was 153/10 000 cases (χ(2)=47.028, 0.000). There is a significant increase on IBR rate in Beijing City, Tianjin City and Hebei province by year. Most of cases received IBR is stage Ⅰ to Ⅱ. Implant reconstruction is the main reconstructive method. Postoperative cosmetic effects and quality of life are both meet patients' demon.
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Objective@#To summarize the status of immediate breast reconstruction (IBR) after mastectomy in Beijing City, Tianjin City and Hebei Province.@*Methods@#A retrospective analysis was made on the data of 382 cases with breast cancer who were treated and followed up successfully with immediate breast reconstruction after mastectomy from January 2012 to December 2016 in Beijing City, Tianjin City and Hebei Province. Clinic data of the followed-up 382 cases (all female, age (38.5±4.2) years (range: 24 to 70 years)), including general information, tumor information, sugery methods, and treatments after surgery were collected. The survival status, metastasis,complications and prognosis were followed up. Cosmetic effcet was evalated by Harris method, and life quality by Functional Assessment of Cancer Therapy-Breast scale (FACT-B). χ2 test was used to compare the difference between year 2012 and year 2013 to 2016. Bonferroni method was used to correct the inspection level, which was 0.05/10=0.005. The trend of IBR rate (ratio of IBR to modified radical mastectomy) from 2013 to 2016 was analyzed by trend χ2 test.@*Results@#There was 46 cases in stage 0, 152 cases in stage Ⅰ, 165 cases in stage Ⅱ, 19 cases in stage Ⅲ. Twenty-five cases was treated by neoadjuvant chemotherapy, 231 by chemotherapy and 35 by radiotherapy. The proportion of implant reconstruction was 48.7% (186/382), more than expanded of 21.5% (82/382), with latissimus dorsi of 12.0% (46/382), TRAM of 8.9% (34/382), DIEP of 2.1% (8/382), and latissimus plus implant of 6.8% (26/382). According to the Harris standard, the excellent and good rate of the cosmetic effect of the reconstructed breast was 93.7%. The score of FACT-B was 108.20±16.9 (range: 67 to 144) 1 year postoperatively. Compared with 2012, the IBR rate was significant increased, till 2015, the IBR rate was 153/10 000 cases (χ2=47.028, P=0.000).@*Conclusions@#There is a significant increase on IBR rate in Beijing City, Tianjin City and Hebei province by year. Most of cases received IBR is stage Ⅰ to Ⅱ. Implant reconstruction is the main reconstructive method. Postoperative cosmetic effects and quality of life are both meet patients′ demon.
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Objective@#To summarize the status of immediate breast reconstruction (IBR) after mastectomy in Beijing City, Tianjin City and Hebei Province.@*Methods@#A retrospective analysis was made on the data of 382 cases with breast cancer who were treated and followed up successfully with immediate breast reconstruction after mastectomy from January 2012 to December 2016 in Beijing City, Tianjin City and Hebei Province. Clinic data of the followed-up 382 cases (all female, age (38.5±4.2) years (range: 24 to 70 years)), including general information, tumor information, sugery methods, and treatments after surgery were collected. The survival status, metastasis,complications and prognosis were followed up. Cosmetic effcet was evalated by Harris method, and life quality by Functional Assessment of Cancer Therapy-Breast scale (FACT-B). χ2 test was used to compare the difference between year 2012 and year 2013 to 2016. Bonferroni method was used to correct the inspection level, which was 0.05/10=0.005. The trend of IBR rate (ratio of IBR to modified radical mastectomy) from 2013 to 2016 was analyzed by trend χ2 test.@*Results@#There was 46 cases in stage 0, 152 cases in stage Ⅰ, 165 cases in stage Ⅱ, 19 cases in stage Ⅲ. Twenty-five cases was treated by neoadjuvant chemotherapy, 231 by chemotherapy and 35 by radiotherapy. The proportion of implant reconstruction was 48.7% (186/382), more than expanded of 21.5% (82/382), with latissimus dorsi of 12.0% (46/382), TRAM of 8.9% (34/382), DIEP of 2.1% (8/382), and latissimus plus implant of 6.8% (26/382). According to the Harris standard, the excellent and good rate of the cosmetic effect of the reconstructed breast was 93.7%. The score of FACT-B was 108.20±16.9 (range: 67 to 144) 1 year postoperatively. Compared with 2012, the IBR rate was significant increased, till 2015, the IBR rate was 153/10 000 cases (χ2=47.028, P=0.000).@*Conclusions@#There is a significant increase on IBR rate in Beijing City, Tianjin City and Hebei province by year. Most of cases received IBR is stage Ⅰ to Ⅱ. Implant reconstruction is the main reconstructive method. Postoperative cosmetic effects and quality of life are both meet patients′ demon.
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From July to August 2018, a survey on the awareness of diabetic foot screening was conducted using the online questionnaires posted on the WeChat group. From 107 trainees in General Practitioner Teacher Training Program of Beijing Xicheng District and 50 general practitioners working in Chinese Academy of Sciences, 145 valid questionnaires were received. All 145 participants worked in community health care settings, including 105 general practitioners, 76 had 10 to 20 years of service, and 98 had undergraduate degree. The survey showed that 75.9% (110/145) heard the diabetic foot screening and knew that screening should be done for all diabetic patients; 98.6% (142/145) knew that the screening should include medical history, foot skin, neuropathy, vascular status, and 73.1% (106/145) also knew that screening should include the foot bone, joint deformities and footwear selection; 21.4% (31/145) had participated in training related to diabetic foot screening. It is suggested that the awareness and knowledge of diabetic foot screening among Beijing community medical personnelare not sufficient, and more training opportunity is necessary for them.
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From July to August 2018, a survey on the awareness of diabetic foot screening was conducted using the online questionnaires posted on the WeChat group. From 107 trainees in General Practitioner Teacher Training Program of Beijing Xicheng District and 50 general practitioners working in Chinese Academy of Sciences, 145 valid questionnaires were received. All 145 participants worked in community health care settings, including 105 general practitioners, 76 had 10 to 20 years of service, and 98 had undergraduate degree. The survey showed that 75.9% (110/145) heard the diabetic foot screening and knew that screening should be done for all diabetic patients; 98.6% (142/145) knew that the screening should include medical history, foot skin, neuropathy, vascular status, and 73.1% (106/145) also knew that screening should include the foot bone, joint deformities and footwear selection; 21.4% (31/145) had participated in training related to diabetic foot screening. It is suggested that the awareness and knowledge of diabetic foot screening among Beijing community medical personnelare not sufficient, and more training opportunity is necessary for them.
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Patients?with?acute?myocardial?infarction?(AMI)?complicated?with?acute?Stanford?type?A?aortic?dissection?after?percutaneous?coronary?intervention?(PCI)?are?critically?ill,?with?a?very?high?fatality?rate,?and?few?cases?are?successfully?treated?clinically.?A?case?with?AMI?admitted?to?the?First?Affiliated?Hospital?of?Zhengzhou?University?complicated?with?acute?left?cardiac?insufficiency?after?PCI,?Stanford?type?A?aortic?dissection,?pericardial?and?pleural?infection,?recurrent?AMI?was?reviewed.?In?the?condition?of?coexistence?of?many?diseases,?through?timely?adjustment?of?treatment?strategy?and?exploratory?application?of?drugs?to?improve?cardiac?function,?the?patient?successfully?received?operation?and?discharged?from?the?hospital.?By?presenting?the?successful?treatment?experience?of?this?case,?the?author?aims?to?improve?the?overall?treatment?of?AMI?patients?with?acute?Stanford?type?A?aortic?dissection?after?PCI.
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Objective To investigate the prognosis predictors of anti-neutrophil cytoplasmic antibody(ANCA)-associated glomerulonephritis treated with glucocorticoid(GC).Methods The clinicopathological data of patients with biopsy-confirmed ANCA-associated glomerulonephritis were retrospective analyzed by retrieving the medical database in Peking Union Medical College Hospital from January 2000 to May 2015. Pathological categories were re-classified. Renal remission rates,infection rates,and death events were compared between intravenous glucocorticoid(GC)pulse therapy group and non-pulse group. Logistic regression analysis was performed to analyze factors influencing the short-term prognosis.Results Among the 81 patients with ANCA-associated glomerulonephritis,49(60.5%)received GC pulse therapy and 32(39.5%)did not. The GC pulse group had significantly lower estimated glomerular filtration rate at baseline(eGFR0)than the non-pulse group(t=3.003,P=0.015)but significantly higher 24-hour urinary protein(24 hUP)(t=2.394,P=0.002)and Birmingham Systemic Vasculitis Activity Score(BVAS)(t=0.049,P=0.013). There was no significant difference in the cumulative amount of cyclophosphamide(CTX)(t=1.336,P=0.245)between these two groups. The overall renal remission rate of GC pulse group in the 6 month was significantly lower(48.7% vs. 79.3%;χ =6.591,P=0.024). Univariate analysis showed that baseline 24 hUP(t=6.222,P=0.017),eGFR0(t=3.727,P=0.046),and pathological category(χ =7.654,P=0.045)were associated with the overall renal remission rate in the 6 month. Multivariate analysis showed the crescent category was an independent factor(OR=20.63,95%CI:2.217-191.973,P=0.008;compared with sclerotic category)for overall renal remission rate in the 6 month,while GC pulse therapy was not an predictor(OR=0.271,95%CI:0.062-1.179,P=0.082). A total of 37 patients experienced infections within 6 months. The infection rate in GC pulse group(55.1%,27/49)was significantly higher than that of non-pulse group(31.3%,10/32)(P=0.042). Univariate regression analysis showed that eGFR0(t=1.912,P=0.049),baseline BVAS(t=-3.360,P=0.001)and GC pulse(χ =6.249,P=0.014)were associated with infection events within 6 months. Multivariate analysis showed that the baseline BVAS was the only predictor with 1.089 times for every 1 point increase in BVAS(OR=1.089,95%CI:1.006-1.179,P=0.034). Conclusions Crescentic category favors renal remission independently compared with sclerotic category. Patients with crescentic category may benefit more from intensive treatment. BVAS acts as an independent risk factor of infection.
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Humans , Antibodies, Antineutrophil Cytoplasmic , Glomerulonephritis , Drug Therapy , Glucocorticoids , Therapeutic Uses , Prognosis , Retrospective StudiesABSTRACT
Lipid storage myopathy (LSM) is an etiologically heterogeneous group of lipid metabolic disorders characterized by accumulation of light microscopic lipid droplets in muscle fibers.This disease seems to be more common in Chinese population accounting for 3％-5％ of total muscle biopsies in several large neuromuscular centers in China.The pathogenesis of LSM is the impairment of fatty acid oxidation in muscle fibers.Late-onset multiple acyl-coenzyme A dehydrogenase deficiency (MADD) caused by electron transfer flavoprotein dehydrogenase (ETFDH) gene mutation has been demonstrated to be the main molecular defect in China.Three frequent ETFDH mutations were identified:c.250G＞A in patients from South China,and c.770A＞G and c.1227A＞C in those from both South and North China.More importantly,almost all late-onset MADD are dramatically responsive to riboflavin supplementation.Neutral lipid storage disease with myopathy (NLSDM) caused by mutations in PNPLA2 gene is the second common cause of Chinese LSM.Distal muscle involvement and asymmetrical muscle weakness and atrophy are common in primary symptoms of NLSDM which may be the first clue indicating the diagnosis of NLSDM.There were also a few case reports showing that LSM may be caused by carnitine transport defect and other deficiencies of acyl-coenzyme A dehydrogenase involved in fatty acid beta oxidation.Increased lipid droplets accumulation in muscle fibers may also be a secondary consequence of mitochondrial myopathy (mtDNA depletion syndrome or MELAS),dermatomyositis and steroid treatment.
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Objective To evaluate the effect of different gargling liquid on colony number in air during oral ultrasound scaling for patients with periodontitis.Methods Patients with mild (n=54), moderate (n=54), and severe chronic periodontitis (n=54) were selected, then patients with same degree of periodontitis were randomly divided into three groups, with 18 in each group. Before scaling, patients in each group of mild, moderate, and severe periodontitis were given honeysuckle liquid (gargle A), 3％ hydrogen peroxide (gargle B), and normal saline (gargle C) respectively. Bacterial culture and identification of air specimens were conducted at 0 and 30 minutes after the beginning of ultrasonic scaling as well as 10, 20 minutes after the end of ultrasonic scaling.Results Variance analysis of three-factor factorial design data showed that degree of periodontitis, types of gargling liquid, and sampling time had interaction (F=2.666, P=0.002). Comparisons of air colonies: At the beginning of scaling, there was no significant difference in colony number of air around patients using different gargling liquid in mild, moderate and severe groups (all P＞0.05). Among patients using the same gargling liquid at 30 minutes after the beginning of scaling, colony number of air was the lowest around patients with mild periodontitis and the highest around patients with severe periodontitis, colony numbers of air around patients with gargle A and B were lower than that of patients with gargle C. Comparison of each sampling points showed that colony number of air around the right side of patients' head was highest, differences were all statistically significant (all P＜0.05). 20 minutes after the end of scaling, there was no significant difference in colony number among groups compared before scaling (all P＞0.05). The main isolated strains in the air were Streptococcus viridans (32.53％), coagulase negative staphylococcus (24.56％), and filamentous fungi (18.48％).Conclusion There are variety of opportunistic pathogens in the air during oral ultrasound scaling, and the number of colonies is positively correlated with the degree of periodontitis. honeysuckle liquid has a good effect on reducing the number of air colonies during and after scaling.
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Fibronectin glomerulopathy is a rare autosomal dominant inherited glomerular disease associated with massive deposition of fibronectin. We recently diagnosed fibronectin glomerulopathy in a 29-year-old woman presenting nephrotic syndrome. Genetic analysis of fibronectin 1 gene showed heterozygosity for the Y973C mutation. However, this mutation was not found in her parents. She had stable renal function but persistent nephrotic proteinuria after one-year follow-up.
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Objective To explore a potentially effective treatment of postoperative deep infection after spinal instrumentation without removal of implants. Methods A total of 4 patients with postoperative deep infection after spinal instrumentation were treated at our hospital between January 2015 and May 2016. They were 2 men and 2 women, aged from 62 to 75 years (mean, 69.2 years). They were treated with ag-gressive surgical debridement under the guidance of methylene blue and negative pressure wound therapy. Reversed latissimus dorsi muscle flap was used to obturate the wound when the bacterial culture of the wound was negative and the relevant inflammatory indicators returned to normal. Negative pressure treatment con-tinued over the closed incision. Results The devices for negative pressure wound therapy were removed in the 4 patients one week after surgery. All the wounds were healed well by first intention 2 weeks after surgery without removal of their implants. The patients were discharged after their examinations for blood routine, CRP, ESR and PCT turned to be normal. Follow-ups for more than one year revealed no re-infection, implants loosening or other adverse effects. Conclusions Negative pressure wound therapy combined with reversed latissimus dorsi muscle flap is a new attempt to treat postoperative deep infection after spinal instru-mentation without implant removal. It may increase the rate of implants reserved, accelerate wound healing and raise the curative rate of deep infection.
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Objective@#Reconstruction of defects after wide resection of tumor in perineal region is challenging because the defects are extensive and complex. In this report, we retrospectively analyzed the clinical experience of perineal reconstruction in last 6 years, to evaluate the value of clinical application of perforator flaps in defects of perineal region.@*Methods@#From January 2011 to December 2016, 32 cases of perineal cancer were treated by radical extensive excision, with defects repaired with perforator flaps. The size of perineal defects was between 8 cm×4 cm-20 cm× 20 cm, 52 perforator flaps were used for perineum reconstruction.@*Results@#All of the defects achieved tension-free closure. All the flaps were survived and healed with first intention except two infection and minor wound dehiscence cases. Both cases healing after dressing and secondary suture. There were no donor-site complications. During an average follow-up of 11.1 months (range, 10-12 months), the reconstructed areas achieved good functional and cosmetic outcomes.@*Conclusions@#Perineum reconstruction with perforator flaps following total radical extensive excision leads to good result in patients with perineal cancer. It can achieve tension-free closure and produces almost normal appearance and function of the perineum.
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Objective To report four patients with secondary α-dystroglycanopathy caused by guanosine diphosphate-mannose pyrophosphorylase-B ( GMPPB ) gene mutations and review the literature aiming to analyze the clinical manifestations , muscle image , molecular pathology and genetic characteristics of the disease.Methods The medical history , physical examination , electromyography and other clinical data of four patients with secondary α-dystroglycanopathy from two families were collected and retrospectively reviewed from 2009 to 2017.Case 1 ( proband of pedigree 1) and case 2 ( proband of pedigree 2) were then further analyzed with muscle imaging , muscle pathology and targeted next generation gene sequencing (NGS).Results Four patients came from two families (three from the same pedigree), two males and two females, with an onset age of 17 -18 years.All four cases presented as limb-girdle muscular dystrophy (LGMD) overlapping with congenital myasthenic syndrome (CMS) characterized by evident proximal limb weakness in early adulthood and fluctuating muscle weakness .They all had delayed motor milestone and did not perform well in physical education since childhood . Serum creatine kinase was elevated markedly (1877-5275 U/L).Myogenic changes on electromyography and marked attenuation on three Hz repetitive nerve stimulation were observed in all patients .Muscle MRI showed prominent involvement of bilateral hamstrings in case 1 and case 2.Muscular dystrophic patterns were demonstrated on muscle biopsies . Targeted NGS revealed two compound heterozygous missense mutations in GMPPB for each case .Case 1 carried c.860G>T (p.R287L)/c851T>C (p.V284L).Case 2 and his two affected sisters (case 3 and case 4) carried c.1097A >G ( p.N366S)/c.589G >T ( p.V197F) .All of these mutations were novel variants and pedigree analysis suggested that the two mutations were from parents .Compared with normal controls, immunohistochemistry and Western blotting showed significantly decreased expression of α-dystroglycan in the muscle tissue from case 1 and case 2.The myasthenic symptoms of all four patients were improved to varying degrees after treatment with pyridostigmine bromide . Conclusions Mutations in GMPPB can lead to dysfunction both in muscle and in neuromuscular transmission causing overlapping between LGMD and CMS phenotypes . Cholinesterase inhibitors can partly improve the symptoms of myasthenia in such patients .
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Objective:To study influence of amlodipine combined enalapril antihypertensive therapy on renal function in aged patients with essential hypertension (EH) complicated coronary heart disease (CHD).Methods:A total of 120 aged EH + CHD patients in our hospital from Feb 2014 to Apr 2016 were enrolled.Patients were randomly and equally divided into amlodipine group,enalapril group and combined treatment group (received amlodipine com-bined enalapril treatment ),all groups were treated for 12 weeks.Total effective rate,standard-reaching condition of blood pressure,urinary albumin excretion rate (UAER),levels of serum creatinine (Scr),cystatin C (CysC) and blood urea nitrogen (BUN) before and after treatment,and incidence of adverse reactions were measured and com-pared among three groups.Results:There was no significant difference in total effective rate among three groups,P＝0.139.Compared with amlodipine group and enalapril group,there was significant reduction in standard-reaching time of blood pressure [ (10.84 ± 2.79) months vs.(10.75 ± 3.31) months vs.(8.20 ± 1.46) months] in com-bined treatment group,P＝0.001 all.Compared with before treatment,after 12-week treatment,there were sig-nificant reductions in UAER,levels of Scr,serum CysC and BUN in amlodipine group and combined treatment group,P＜0.05 or ＜ 0.01;compared with amlodipine group and enalapril group after 12-week treatment,there were significant reductions in UAER [(130.55 ± 12.72) μg/min vs.(135.63 ± 17.64) μg/min vs.(112.25 ± 13.34) μg/min],levels of Scr [ (79.32 ± 6.13) μmol/L vs.(80.25 ± 5.97) μmol/L vs.(68.04 ± 5.56) μmol/L],serum CysC [ (1.14 ± 0.23) mg/L vs.(1.21 ± 0.26) mg/L vs.(0.76 ± 0.17) mg/L] and BUN [ (5.16 ± 1.13) mmol/L vs.(5.79 ± 1.03) mmol/L vs.(4.23 ± 0.56) mmol/L] in combined treatment group,and BUN level of amlodip-ine group was significantly lower than that of enalapril group,P＜0.05 or ＜0.01.There was no significant differ-ence in incidence rate of adverse reactions during treatment among three groups,P＝0.757.Conclusion:Small dose amlodipine combined enalapril is effective on controlling blood pressure in aged EH + CHD patients.Compared with monotherapy,it possesses better protection on renal function with high safety,which is worth extending.
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Objective@#To explore effects of perforator flaps combined with muscle flaps for repairing grade Ⅳ pressure ulcers in ischial tuberosity of elderly patients.@*Methods@#Nine elderly patients with grade Ⅳ pressure ulcers in ischial tuberosity were hospitalized in our burn ward from April 2014 to April 2017. Size of wounds ranged from 5 cm×3 cm to 12 cm×7 cm, and depth of sinus ranged from 6 to 22 cm. After admission, emergency debridement or debridement in selective time was performed. After debridement, the wounds were treated with continuous vacuum assisted closure therapy. After the treatment for 1 to 2 weeks, tissue flaps repair operations were performed. Four patients were repaired with inferior gluteal artery perforator flaps combined with long head of biceps femoris muscle flaps. Three patients were repaired with inferior gluteal artery perforator flaps combined with semimembranous muscle flaps. One patient was repaired with inferior gluteal artery perforator flap combined with gracilis muscle flap. One patient was repaired with femoral profound artery perforator flap combined with gluteus maximus muscle flap, and the distal area of femoral profound artery perforator flap of the patient which showed intraoperative cyanosis of 6 cm×4 cm was thinned to medium thickness skin to cover the muscle flap. The other eight patients showed no abnormality during operation. Size of perforator flaps ranged from 7 cm×5 cm to 14 cm×12 cm, and size of muscle flaps ranged from 11 cm×4 cm to 24 cm×6 cm. The donor sites of flaps were all sutured directly.@*Results@#The tissue flaps and skin graft of all patients survived well after operation. During follow-up of 8 to 35 weeks, operative area of all patients showed good shape and texture, with no local diabrosis or recurrence of pressure ulcers.@*Conclusions@#The combination of perforator flaps and muscle flaps is effective in repairing and reducing recurrence of grade Ⅳ pressure ulcers in ischial tuberosity of elderly patients.
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Objective:To investigate the clinical biological characteristics of EVI1 positive acute myeloid leukemia (AML) and its effect on early chemotherapy.Methods:The clinical and biological characteristics of 33 AML patients with EVI1 positive were retrospectively analyzed in 361 AML patients who were diagnosed and treated in our institute from March 2015 to July 2016,and the clinical and biological features,and rates of the induced remission were compared between the intermediate risk and poor risk with EVI1 positive AML,moreover,the influential factors on complete remission (CR) were analyzed.The expression of EVI1/ABL was tested in 32 healthy donors to confirm the abnormal threshold of EVI1 expression.Results:The definition of EVI1 positive was that the quantitative expression of EVI1/ABL was more than 8.0％.The 33 AML patients with EVI1 positive were found in 361 newly diagnosed AML patients,in which the female and male patients were 17 and 16 respectively,the median age was 45 (18-67) years,with a median follow-up of 6.6 (0.7-13.2) months.Intermediate karyotype was found in 17 patients (including 9 patients with normal karyotypes,1 patient with + 8);unfavorable karyotype was found in 14 patients [including 7 patients with-7/7q-,4 patients with t (v;11q23),3 patients with inv (3)/t (3;3),and 2 patients without mitotic figures].The rate of CR in the first induction chemotherapy was 42.4％,and the rate of total CR was 60.6％.According to the NCCN,16 intermediate risk patients and poor risk patients were divided,without favorable risk patients.The rate of CR in the first induction chemotherapy were 68.8％ and 17.6％ (P =0.005) in the intermediate risk and poor risk respectively,that of total CR were 81.3％ and 41.2％ (P =0.032),and the rates of relapse were 7.7％ and 14.3％.Univariable analysis revealed that unfavorable karyotype could affect the rate of CR in the first reduction chemotherapy and that of total CR (P =0.004,0.029).The poor risk patients had higher mortality (41.2％ vs.6.3％,P =0.039) and lower overall survival (OS) (P =0.012).Conclusion:EVI1 may be not an independent prognostic factor for the AML patients considering the appearance in the intermediate and poor risk patients.It predicts poor outcome in the EVI1 positive AML patients who have unfavorable karyocytes,such as-7/7q-,t (v;11 q23),and inv (3)/t (3;3),and also a low rate of both CR in the first induction chemotherapy and total CR.It also has a low rate of long-term survival and high mortality in the AML patients with EVI1 positive,who may benefit from allogeneic bone marrow transplantation as soon as possible.
ABSTRACT
·AIM: To study the clinical efficacy of integrative therapy in the treatment of non-proliferative diabetic retinopathy.·METHODS: Ninety patients ( 90 eyes ) in our hospital with non - proliferative diabetic retinopathy were randomly divided into three groups. All three groups were treated with diabetes drugs to control blood sugar. The first group was treated with western medicine, the second group was treated with Chinese medicine decoction Traditional Chinese Medicine ( TCM ) treatment, and the third group was treated with the combination of those two methods. All patients were recorded and analyzed changes of clinical effects after 6 courses of treatment.·RESULTS: After 6 courses of treatment, the total efficacy rate of the third group was 86%, markedly higher than that of the first group (57%, P<0. 05) as well as the second group (60%, P<0. 05). The integrative group improved more markedly in terms of vision, macular edema absorption, and ERG b-wave amplitude restoration, the difference being statistically significant (P<0. 05) when compared to the first and the second group.· CONCLUSION: Integrative treatment of diabetic retinopathy could effectively improve the therapeutic effect in patients with non-proliferative retinopathy.