definitive treatment

确定性治疗
  • 文章类型: Journal Article
    外固定是复杂胫骨干骨折(TDF)最常用的治疗方法之一。这项研究的目的是评估单侧外固定器作为资源有限的复杂TDF的主要和确定性治疗的疗效。
    2016年6月至2021年3月的一项回顾性研究包括110名TDF受试者,他们在Ibb大学附属医院接受外固定器治疗作为确定性固定。病人的人口统计学特征,并发症,收集和分析结果。还研究了与针部位感染相关的因素。
    平均年龄为42.1±10.1岁,92.7%是男性。农村居民占22.7%。吸烟和糖尿病分别占27.3%和30.0%,分别。一般并发症发生率为12.0%,肺栓塞最常见,为4.5%。骨科并发症包括27.3%(30)的针迹感染和1.8%的骨髓炎(2)。销部位感染需要药物治疗21例,外固定器更换5例。2例骨髓炎和软组织各需要多次清创。在23.1±3.2周内,79.1%(87)发生完全愈合,在34.8±4.8周内,97.3%(107)发生最终对齐。在1.8%(2)中发生了马不通病,1例肥厚性骨不连。像农村居民这样的因素,吸烟,糖尿病,开放性骨折,最差骨折等级(Gustilo和AndersonC型),一般并发症的发生与针状部位感染显著相关(所有p值<0.05)。
    单侧外固定器作为主要和确定的治疗方法是可行的,简单,和TDF的有效选项,即使在资源有限的环境中也具有很高的成功率。在这项研究中,农村居民,吸烟,糖尿病,开放性骨折,最差断裂等级,一般并发症的发生与针脚部位感染的发生有关。
    UNASSIGNED: External fixation is one of the most often utilized treatment options for complicated tibial diaphyseal fractures (TDF). The purpose of this study was to assess the efficacy of unilateral external fixators as primary and definitive therapy for complex TDF in a resource-limited setting.
    UNASSIGNED: A retrospective study between June 2016 and March 2021 included 110 subjects with TDF who were treated with an external fixator as definitive fixation in hospitals affiliated with Ibb University. The patient\'s demographic characteristics, complications, and outcomes were gathered and analyzed. Factors associated with pin site infection were also investigated.
    UNASSIGNED: The mean age was 42.1 ± 10.1 years, with 92.7% being male. Rural residents accounted for 22.7%. Smoking and diabetes mellitus were present in 27.3% and 30.0%, respectively. General complications occurred in 12.0%, with pulmonary embolism being the most common at 4.5%. Orthopedic complications included pin-track infections in 27.3% (30) and osteomyelitis in 1.8% (2). Pin site infections required medical treatment in 21 cases and external fixator changes in five. Two cases each needed several debridements for osteomyelitis and soft tissue. Full union occurred in 79.1% (87) over 23.1 ± 3.2 weeks and final alignment in 97.3% (107) over 34.8 ± 4.8 weeks. Malunions occurred in 1.8% (2), and one case had hypertrophic nonunion. Factors like rural residency, smoking, diabetes, open fractures, worst fracture grade (Gustilo and Anderson type C), and general complications occurrence significantly correlated with pin site infection (all p-values < 0.05).
    UNASSIGNED: A unilateral external fixator as a primary and definitive treatment is a viable, simple, and effective option for TDF with a high success rate even in a resource-limited setting. In this study, residents in rural areas, smoking, diabetes, open fracture, worst fracture grade, and general complication occurrence were associated with pin site infection occurrence.
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  • 文章类型: Case Reports
    胸腺上皮肿瘤(TET),特别是B2型胸腺瘤,是罕见的肿瘤,主要见于前纵隔。目前的治疗方法包括手术,化疗,和放射治疗,但是关于放疗作为独立治疗的研究有限。本病例报告旨在阐明大分割放疗作为局部晚期B2型胸腺瘤的独立治疗的临床结果。提供对其潜在疗效和在临床实践中的作用的见解。
    Thymic epithelial tumors (TETs), particularly type B2 thymomas, are rare neoplasms primarily found in the anterior mediastinum. The current therapeutic approach includes surgery, chemotherapy, and radiotherapy, but there is limited research on radiotherapy as a standalone treatment. This case report aims to elucidate the clinical outcomes of hypofractionated radiotherapy as a standalone treatment for locally advanced type B2 thymoma, offering insights into its potential efficacy and role in clinical practice.
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  • 文章类型: Journal Article
    介绍经皮胆囊造口术(PC)是急性胆囊炎(AC)患者的一种治疗选择,或太病态腹腔镜胆囊切除术(LC)。一些患者有PC作为一种确定的治疗方法,而其他人则在LC之前将PC作为桥接治疗。这项研究的目的是调查接受PC作为确定性治疗与桥接治疗的患者的患者特征和死亡率。方法我们的研究回顾性回顾了2019年2月至2022年11月在Torbay和SouthDevonNHSFoundationTrust接受PC治疗的所有患者,托基,英格兰。50名患者接受了AC的PC治疗,48例患者有随访数据可供分析。其中,26名患者(54%)仅接受PC(最终PC),22例患者(46%)后来接受了LC(桥接LC).结果在这项研究中,68.8%的患者为男性,平均年龄为76±9岁。总体平均Charlson合并症指数(CCI)评分为4.96±1.12,平均美国麻醉医师协会(ASA)评分为2.83±0.36。PC排出时间的中位数为42天。6例患者(12.5%)的AC复发,在PC插入后平均发作57天。12例患者(25%)经历了PC并发症:11例(23%)是轻微的,涉及疼痛或脱落的管子,一个(2%)是主要的,导致肝下脓肿.从PC插入到LC手术的中位持续时间为50.5天。桥接LC队列的30天和1年死亡率为0%,而最终的PC队列30日死亡率为30.8%(8例),1年死亡率为46.1%(12例).与确定的PC队列相比,桥接LC队列的CCI显着降低(4.39vs5.57,p<0.05),ASA明显降低(2.61vs3.04,p<0.05)。1年生存队列与30天死亡率队列相比,ASA显著降低(2.71vs3.25p<0.05),和非显著较低的CCI(4.66vs5.86p=0.094)。呼吸功能障碍和高胆红素血症的阴性预测因素的存在具有较高的30天和90天死亡率,分别为31.3%和37.5%,相比之下,他们分别为9.4%和21.4%。结论我们的结果表明,PC是一种安全的手术,成功率高,并发症少。我们表明,PC是一种有效的治疗选择,可以桥接选定的患者队列以接受延迟LC。此外,数据提示ASA和CCI评分可作为临床辅助手段,用于评估从PC过渡到LC的患者是否合适.最后,ASA,呼吸功能障碍,和高胆红素血症可用作PC后死亡率的显著阴性预测因子。
    Introduction Percutaneous cholecystostomy (PC) is a treatment option for patients with acute cholecystitis (AC) who are too unwell, or too morbid for laparoscopic cholecystectomy (LC). Some patients have PC as a definitive treatment, whereas others have PC as a bridging treatment prior to LC. The aim of this study is to investigate patient characteristics and mortality among those who received PC as definitive treatment versus bridging treatment. Methods Our study retrospectively reviewed all patients treated with PC for AC from February 2019 to November 2022 at the Torbay and South Devon NHS Foundation Trust, Torquay, England. Fifty patients underwent PC for AC, with 48 patients having follow-up data available for analysis. Of these, 26 patients (54%) only received PC (definitive PC), and 22 patients (46%) later underwent LC (bridging LC). Results In this study, 68.8% of the patients were male, with a mean age of 76 ± 9 years. The overall mean Charlson Comorbidity Index (CCI) score was 4.96 ± 1.12, and the mean American Society of Anesthesiologists (ASA) score was 2.83 ± 0.36. The median PC drain duration was 42 days. Six patients (12.5%) had a recurrence of AC with a mean of 57 days onset after PC insertion. Twelve patients (25%) experienced PC complications: 11 (23%) were minor, involving pain or a dislodged tube, and one (2%) was major, resulting in a subhepatic abscess. The median duration from PC insertion to LC surgery was 50.5 days. The bridging LC cohort had a 30-day and one-year mortality of 0%, while the definitive PC cohort had a 30-day mortality of 30.8% (eight patients) and a one-year mortality of 46.1% (12 patients). The bridging LC cohort compared to the definitive PC cohort had a significantly lower CCI (4.39 vs 5.57, p<0.05), and a significantly lower ASA (2.61 vs 3.04, p<0.05). The one-year survival cohort compared to the 30-day mortality cohort had significantly lower ASA (2.71 vs 3.25 p<0.05), and a non-significantly lower CCI (4.66 vs 5.86 p=0.094). The presence of negative predictive factors of respiratory dysfunction and hyperbilirubinemia had higher 30-day and 90-day mortality rates of 31.3% and 37.5%, compared to their absence of 9.4% and 21.4% respectively. Conclusion Our results demonstrate that PC is a safe procedure with a high success rate and low complications. We showed that PC is an effective treatment option for bridging a select cohort of patients to receive a delayed LC. Furthermore, the data suggests ASA and CCI scoring can be used as clinical adjuncts to assess whether bridging patients from PC to LC is appropriate. Finally, ASA, respiratory dysfunction, and hyperbilirubinemia can be used as significant negative predictors of post-PC mortality.
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  • 文章类型: Journal Article
    目的:在一个种族不同的队列中,研究婚姻状况与前列腺癌预后的关系。
    方法:研究人群包括来自基线北卡罗莱纳州-路易斯安那州前列腺癌(PCaP)队列的男性(1010个黑人;1070个白人)。诊断时的婚姻状况和筛查史由自我报告确定。婚姻状况的二元衡量标准定义为已婚(包括已婚生活)与未婚(从未结婚,离婚/分居,或丧偶)。高侵袭性肿瘤是使用PSA的复合测量来定义的,格里森分数,和舞台。明确治疗定义为接受根治性前列腺切除术或放疗。多变量logistic回归用于检查婚姻状况与(1)高侵袭性肿瘤,(2)接受明确治疗,(3)黑人和白人前列腺癌患者的筛查史。
    结果:黑人男性结婚的可能性低于白人男性(68.1%vs.83.6%)。未结婚(与已婚)与整个研究人群中高侵袭性肿瘤的几率增加相关(校正赔率比(aOR):1.56;95%置信区间(CI):1.20-2.02)以及种族分层分析中的黑人和白人男性。在整个研究人群中,未婚男性接受明确治疗的可能性较小(aOR:0.68;95%CI:0.54-0.85)。在种族分层分析中,未婚的黑人男性不太可能接受明确的治疗。未婚的黑人和白人男性比已婚男性不太可能有前列腺癌筛查史。
    结论:黑人男性较低的结婚率可能表明对治疗决策的支持减少,症状管理,和可能导致前列腺癌差异的护理人员支持。
    OBJECTIVE: To examine the association of marital status with prostate cancer outcomes in a racially-diverse cohort.
    METHODS: The study population consisted of men (1010 Black; 1070 White) with incident prostate cancer from the baseline North Carolina-Louisiana Prostate Cancer (PCaP) cohort. Marital status at time of diagnosis and screening history were determined by self-report. The binary measure of marital status was defined as married (including living as married) vs. not married (never married, divorced/separated, or widowed). High-aggressive tumors were defined using a composite measure of PSA, Gleason Score, and stage. Definitive treatment was defined as receipt of radical prostatectomy or radiation. Multivariable logistic regression was used to examine the association of marital status with (1) high-aggressive tumors, (2) receipt of definitive treatment, and (3) screening history among Black and White men with prostate cancer.
    RESULTS: Black men were less likely to be married than White men (68.1% vs. 83.6%). Not being married (vs. married) was associated with increased odds of high-aggressive tumors in the overall study population (adjusted Odds Ratio (aOR): 1.56; 95% Confidence Interval (CI): 1.20-2.02) and both Black and White men in race-stratified analyses. Unmarried men were less likely to receive definitive treatment in the overall study population (aOR: 0.68; 95% CI: 0.54-0.85). In race-stratified analyses, unmarried Black men were less likely to receive definitive treatment. Both unmarried Black and White men were less likely to have a history of prostate cancer screening than married men.
    CONCLUSIONS: Lower rates of marriage among Black men might signal decreased support for treatment decision-making, symptom management, and caregiver support which could potentially contribute to prostate cancer disparities.
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  • 文章类型: Observational Study
    背景:在巴基斯坦,医院特别是广谱的抗生素处方不当以及随后对抗生素耐药率的影响令人担忧。减少不适当处方的一种公认方法是根据培养敏感性报告的结果调整经验性治疗。目的:使用文化敏感性报告优化巴基斯坦一家教学医院的抗生素处方。方法:在GhurkiTrust教学医院进行回顾性观察性研究。在研究期间(2018年5月和2018年12月),总共从患者中采集了465个阳性培养物。评估了患者感染部位的病原体鉴定和敏感性测试的结果。从患者的医疗档案中收集其他数据。这包括人口统计数据,样品类型,致病微生物,抗菌治疗,以及经验性或确定性治疗以及药物成本。抗菌数据使用世界卫生组织定义的每日剂量方法进行评估。结果:从465个患者样本中检测到497个分离株,因为32个患者存在微生物,其中包括309克阴性杆菌和188克阳性球菌。在497个分离株中,最常见的革兰氏阳性病原菌是金黄色葡萄球菌(甲氧西林敏感金黄色葡萄球菌)(125)(25.1%),最常见的革兰氏阴性病原菌是大肠杆菌(140)(28.1%).发现大多数革兰氏阴性分离株对氨苄青霉素和co-amoxiclav具有抗性。大多数鲍曼不动杆菌对碳青霉烯类抗生素耐药。革兰阳性菌对利奈唑胺和万古霉素的敏感性最高。经验性治疗最广泛使用的抗生素是头孢哌酮加舒巴坦,头孢曲松,阿米卡星,万古霉素,和甲硝唑,而利奈唑胺的使用率高,克林霉素,美罗培南,哌拉西林+他唑巴坦在确定性治疗中可见。在220例(71.1%)革兰氏阴性感染和134例(71.2%)革兰氏阳性感染中调整了经验性治疗。与经验性治疗相比,在确定性治疗中,抗生素的使用数量减少了13.8%.确定性治疗中抗生素的平均费用低于经验性治疗(8.2%),住院时间也减少了。结论:培养敏感性报告有助于降低抗生素利用率和成本,并有助于选择最合适的治疗方法。我们还发现迫切需要在医院实施抗菌药物管理计划,并制定医院抗生素指南,以减少不必要的广谱抗生素处方。
    Background: There are concerns with inappropriate prescribing of antibiotics in hospitals especially broad spectrum in Pakistan and the subsequent impact on antimicrobial resistance rates. One recognized way to reduce inappropriate prescribing is for empiric therapy to be adjusted according to the result of culture sensitivity reports. Objective: Using culture sensitivity reports to optimize antibiotic prescribing in a teaching hospital in Pakistan. Methods: A retrospective observational study was undertaken in Ghurki Trust Teaching Hospital. A total of 465 positive cultures were taken from patients during the study period (May 2018 and December 2018). The results of pathogen identification and susceptibility testing from patient-infected sites were assessed. Additional data was collected from the patient\'s medical file. This included demographic data, sample type, causative microbe, antimicrobial treatment, and whether empiric or definitive treatment as well as medicine costs. Antimicrobial data was assessed using World Health Organization\'s Defined Daily Dose methodology. Results: A total of 497 isolates were detected from the 465 patient samples as 32 patients had polymicrobes, which included 309 g-negative rods and 188 g-positive cocci. Out of 497 isolates, the most common Gram-positive pathogen isolated was Staphylococcus aureus (Methicillin-sensitive Staphylococcus aureus) (125) (25.1%) and the most common Gram-negative pathogen was Escherichia coli (140) (28.1%). Most of the gram-negative isolates were found to be resistant to ampicillin and co-amoxiclav. Most of the Acinetobacter baumannii isolates were resistant to carbapenems. Gram-positive bacteria showed the maximum sensitivity to linezolid and vancomycin. The most widely used antibiotics for empiric therapy were cefoperazone plus sulbactam, ceftriaxone, amikacin, vancomycin, and metronidazole whereas high use of linezolid, clindamycin, meropenem, and piperacillin + tazobactam was seen in definitive treatment. Empiric therapy was adjusted in 220 (71.1%) cases of Gram-negative infections and 134 (71.2%) cases of Gram-positive infections. Compared with empiric therapy, there was a 13.8% reduction in the number of antibiotics in definitive treatment. The average cost of antibiotics in definitive treatment was less than seen with empiric treatment (8.2%) and the length of hospitalization also decreased. Conclusions: Culture sensitivity reports helped reduced antibiotic utilization and costs as well as helped select the most appropriate treatment. We also found an urgent need for implementing antimicrobial stewardship programs in hospitals and the development of hospital antibiotic guidelines to reduce unnecessary prescribing of broad-spectrum antibiotics.
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  • 文章类型: Journal Article
    背景:寡转移食管癌(EC)的研究相对较新。初步数据表明,在某些患者中更积极的治疗方案可能会提高寡转移型EC的生存率。然而,共识建议姑息治疗.我们假设,与单纯姑息治疗和历史对照相比,采用确定性方法(放化疗[CRT])治疗的寡转移食管癌患者的总体生存率(OS)有所提高。
    方法:诊断为同步寡转移的患者(任何组织学,对在单一学术医院治疗的≤5个转移灶)食管癌进行回顾性分析,并分为确定性和姑息性治疗组。最终CRT被定义为在≥40Gy和≥2个周期的化疗中对原发部位进行放射治疗。
    结果:78期IVB(AJCC第8版。)患者,36符合预先指定的寡转移定义。其中,19人接受了最终的CRT,17人接受姑息治疗。中位随访时间为16.5个月(范围:2.3-95.0个月),确定性CRT和姑息治疗组的中位OS分别为90.2和8.1个月(p<0.01),转换为50.5%的5年OS(95CI:32.0-79.8%)与7.5%(95CI:1.7-48.9%),分别。
    结论:接受确定性CRT治疗的低转移性EC患者受益于该方法,其5年生存率(50.5%)大大超过5%的转移性EC的历史标准。在我们的队列中,与仅接受姑息治疗的患者相比,接受确定性CRT治疗的低转移性EC患者的OS显着改善。值得注意的是,与接受姑息治疗的患者相比,接受明确治疗的患者通常更年轻,表现状况更好.有必要对寡转移EC的最终CRT进行进一步的前瞻性评估。
    BACKGROUND: The study of oligometastatic esophageal cancer (EC) is relatively new. Preliminary data suggests that more aggressive treatment regimens in select patients may improve survival rates in oligometastatic EC. However, the consensus recommends palliative treatment. We hypothesized that oligometastatic esophageal cancer patients treated with a definitive approach (chemoradiotherapy [CRT]) would have improved overall survival (OS) compared to those treated with a purely palliative intent and historical controls.
    METHODS: Patients diagnosed with synchronous oligometastatic (any histology, ≤5 metastatic foci) esophageal cancer treated in a single academic hospital were retrospectively analyzed and divided into definitive and palliative treatment groups. Definitive CRT was defined as radiation therapy to the primary site with ≥40 Gy and ≥2 cycles of chemotherapy.
    RESULTS: Of 78 Stage IVB (AJCC 8th ed.) patients, 36 met the pre-specified oligometastatic definition. Of these, 19 received definitive CRT, and 17 received palliative treatment. With a median follow-up of 16.5 months (Range: 2.3-95.0 months), median OS for definitive CRT and palliative groups were 90.2 and 8.1 months (p < 0.01), translating into 5-year OS of 50.5% (95%CI: 32.0-79.8%) vs. 7.5% (95%CI: 1.7-48.9%), respectively.
    CONCLUSIONS: Oligometastatic EC patients treated with definitive CRT benefited from that approach with survival rates (50.5%) that vastly exceeded historical standards of 5% at 5 years for metastatic EC. Oligometastatic EC patients treated with definitive CRT had significantly improved OS compared to those treated with palliative-only intent within our cohort. Notably, definitively treated patients were generally younger and with better performance status versus those palliatively treated. Further prospective evaluation of definitive CRT for oligometastatic EC is warranted.
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  • 文章类型: Journal Article
    未经评估:抗生素降级是限制耐药性传播和出现的抗菌药物管理计划的关键要素。进行这项研究以评估大肠杆菌或甲氧西林敏感金黄色葡萄球菌(MSSA)的阳性培养敏感性报告对抗生素治疗降阶梯的影响。
    UNASSIGNED:这项前瞻性观察性研究是对256名感染患者进行的。样品主要是从感染部位的脓液中获得的,用于鉴定病原体和培养敏感性测试。数据是从病人的医疗档案中收集的,其中包括他们的人口统计数据,样品类型,作为基于培养的经验性或确定性治疗的致病微生物和抗菌治疗。数据采用SPSS进行分析。
    未经证实:在256个分离的微生物中,138(53.9%)为MSSA,118为大肠杆菌(46.1%)。MSSA对头孢西丁100%敏感,苯唑西林,万古霉素,磷霉素,粘菌素和超过90%的利奈唑胺(95.3%),替加环素(93.1%),氯霉素(92.2%)和阿米卡星(90.2%)。大肠杆菌仅对磷霉素有100%的敏感性,对粘菌素有90%以上的敏感性(96.7%),多粘菌素B(95.1%)和替加环素(92.9%)。头孢哌酮+舒巴坦的高使用率(151),阿米卡星(149),头孢曲松(33),甲硝唑(30)和哌拉西林+他唑巴坦(22)见于经验性处方。在敏感性测试之后,大肠杆菌最常见的抗生素是美罗培南IV(34),阿米卡星(34),环丙沙星(29)和头孢哌酮+舒巴坦(25)。对于MSSA案例,利奈唑胺(48),克林霉素(30),头孢哌酮+舒巴坦IV(16)和阿米卡星(15)常用。总的来说,大肠杆菌的抗生素使用减少了23%,MSSA病例减少了43%.
    UNASSIGNED:文化敏感性报告有助于抗菌治疗的降级,减少特别是广谱抗生素的处方。因此,建议根据当地抗菌药物敏感性模式制定当地医院指南,同时防止不必要地使用广谱抗生素进行经验性治疗.
    UNASSIGNED: Antibiotic de-escalation is a key element of antimicrobial stewardship programs that restrict the spread and emergence of resistance. This study was performed to evaluate the impact of positive culture sensitivity reports of E. coli or Methicillin sensitive Staphylococcus aureus (MSSA) on de-escalation of antibiotic therapy.
    UNASSIGNED: This prospective observational study was performed on 256 infected patients. The samples were obtained principally from the pus of infected sites for the identification of pathogens and culture-sensitivity testing. The data were collected from patient medical files, which included their demographic data, sample type, causative microbe and antimicrobial treatment as empiric or definitive treatment based on cultures. Data were analyzed using SPSS.
    UNASSIGNED: Of 256 isolated microbes, 138 (53.9%) were MSSA and 118 were E. coli (46.1%). MSSA showed 100% sensitivity to cefoxitin, oxacillin, vancomycin, fosfomycin, colistin and more than 90% to linezolid (95.3%), tigecycline (93.1%), chloramphenicol (92.2%) and amikacin (90.2%). E. coli showed 100% sensitivity to only fosfomycin and more than 90% to colistin (96.7%), polymyxin-B (95.1%) and tigecycline (92.9%). The high use of cefoperazone+sulbactam (151), amikacin (149), ceftriaxone (33), metronidazole (30) and piperacillin + tazobactam (22) was seen with empiric prescribing. Following susceptibility testing, the most common antibiotics prescribed for E. coli were meropenem IV (34), amikacin (34), ciprofloxacin (29) and cefoperazone+sulbactam (25). For MSSA cases, linezolid (48), clindamycin (30), cefoperazone+ sulbactam IV (16) and amikacin (15) was used commonly. Overall, there was 23% reduction in antibiotic use in case of E. coli and 43% reduction in MSSA cases.
    UNASSIGNED: Culture sensitivity reports helped in the de-escalation of antimicrobial therapy, reducing the prescribing of especially broad-spectrum antibiotics. Consequently, it is recommended that local hospital guidelines be developed based on local antimicrobial susceptibility patterns while preventing the unnecessary use of broad-spectrum antibiotics for empiric treatment.
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  • 文章类型: Case Reports
    先天性肾病可在新生儿期间出现严重的T细胞淋巴细胞减少症(TCL),可以通过新生儿筛查(NBS)中T细胞受体切除环(TRECs)的减少来检测。导致选择性TCL的最常见胸腺基质缺损是22q11.2缺失综合征(22q11.2DS)。T-box转录因子1(TBX1),存在于22号染色体上,负责胸腺上皮发育。TBX1中导致单倍体功能不全的单个变体引起模拟22q11.2DS的临床综合征。先天性无精症的决定性治疗是同种异体胸腺移植。然而,这种疗法的普遍可用性是有限的。我们介绍了一名因TBX1单倍功能不全而早期诊断为先天性异常的患者。在评估胸腺移植时,她出现了Omenn综合征(OS)和危及生命的腺病毒血症。尽管用抗病毒药物和细胞毒性T淋巴细胞(CTL)治疗,危及生命的腺病毒血症持续存在。鉴于迫切需要快速建立T细胞免疫和病毒清除,患者接受了未经处理的同胞供体(MSD)造血细胞移植(HCT),最终实现胸腺后供体来源的移植,病毒清除,和免疫重建。该病例说明,由于胸腺移植后免疫恢复较慢,而且胸腺移植在全球范围内的可用性有限,临床医生可考虑CTL治疗和HCT治疗严重感染的先天性无创性患者.
    Congenital athymia can present with severe T cell lymphopenia (TCL) in the newborn period, which can be detected by decreased T cell receptor excision circles (TRECs) on newborn screening (NBS). The most common thymic stromal defect causing selective TCL is 22q11.2 deletion syndrome (22q11.2DS). T-box transcription factor 1 (TBX1), present on chromosome 22, is responsible for thymic epithelial development. Single variants in TBX1 causing haploinsufficiency cause a clinical syndrome that mimics 22q11.2DS. Definitive therapy for congenital athymia is allogeneic thymic transplantation. However, universal availability of such therapy is limited. We present a patient with early diagnosis of congenital athymia due to TBX1 haploinsufficiency. While evaluating for thymic transplantation, she developed Omenn Syndrome (OS) and life-threatening adenoviremia. Despite treatment with anti-virals and cytotoxic T lymphocytes (CTLs), life threatening adenoviremia persisted. Given the imminent need for rapid establishment of T cell immunity and viral clearance, the patient underwent an unmanipulated matched sibling donor (MSD) hematopoietic cell transplant (HCT), ultimately achieving post-thymic donor-derived engraftment, viral clearance, and immune reconstitution. This case illustrates that because of the slower immune recovery that occurs following thymus transplantation and the restricted availability of thymus transplantation globally, clinicians may consider CTL therapy and HCT to treat congenital athymia patients with severe infections.
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  • 文章类型: Journal Article
    Background Anal squamous cell carcinoma accounts for less than 2-3% of all digestive system carcinomas. The present study aimed to determine the clinical characteristics, treatment patterns, and treatment outcomes of patients at our institution. Methodology We reviewed the clinical data of all consecutive patients with anal squamous cell carcinoma who were treated with definitive radiotherapy in our department between July 2009 and July 2020. Radiotherapy was delivered in 1.8-2 Gy daily fractions to a whole pelvic dose ranging from 45 to 50 Gy, followed by boost radiotherapy of 10-15 Gy, resulting in a total dose of approximately 60 Gy. Concurrent chemotherapy with radiotherapy included 5-fluorouracil/mitomycin C or 5-fluorouracil/cisplatin. Results A total of 14 patients with a median age of 61.5 years (range: 45-85 years) were analyzed. There were nine women and five men. The clinical T stage was T1 in two patients, T2 in six patients, T3 in two patients, and T4 in four patients. The clinical N stage was N0 in four patients and N1 in 10 patients. Patients with clinical stage III disease comprised 79% of the entire study population. For the entire cohort, the five-year overall survival rate was 83.3% and the five-year progression-free survival rate was 48.5%. One patient experienced grade 3 fecal incontinence, and the others experienced no radiation-induced severe delayed adverse events. Conclusions The results of our study demonstrated that definitive radiotherapy with or without chemotherapy for patients with anal squamous cell carcinoma is an effective and feasible treatment.
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  • 文章类型: Case Reports
    BACKGROUND: Endobronchial metastasis is a very rare type of recurrence after lung cancer surgery. Surgical intervention may be difficult to perform due to the postoperative reduction in the activities of daily living (ADL) and the invasiveness associated with redo surgery. In such cases, endobronchial brachytherapy (EBBT) plays an important role not only as a palliative treatment, but also as a definitive treatment with curative intent.
    METHODS: Three men (64, 69, and 74 years old) underwent combination therapy of external beam radiation therapy (EBRT) and EBBT for endobronchial metastasis after lobectomy of stage I-II non-small cell lung cancer (NSCLC): 2 cases of squamous cell carcinoma and 1 of adenocarcinoma. We used a special source-centralizing applicator for EBBT to avoid eccentric distribution of the radiation dose. Follow-up was considered to start from the end of brachytherapy. None of our patients experienced severe adverse events, and none needed extensive outpatient treatment. Local control was achieved in all cases by a bronchoscopic evaluation. All patients were alive after 31, 38, and 92 months of follow-up, respectively. In the adenocarcinoma patient, two metastases to the lung were discovered 3 years after EBBT, and the patient underwent partial wedge resection.
    CONCLUSIONS: EBBT may be a promising treatment with curative intent for endobronchial metastasis after surgery of NSCLC.
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