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1.
BackgroundAdjuvant chemotherapy, postoperative radiation (PORT), and prophylactic cranial irradiation (PCI) have been individually examined in limited-stage small cell lung cancer (SCLC). There is a paucity of data on the effectiveness of each adjuvant treatment modality when used in combination after surgical resection of SCLC.MethodsData were collected from 5 cancer centers on all patients with limited-stage SCLC who underwent surgical resection between 1986 and 2019. Univariate and multivariable models were conducted to identify predictors of long-term outcomes, focusing on freedom from recurrence and survival benefit of adjuvant chemotherapy, PORT, and PCI.ResultsA total of 164 patients were analyzed. Multivariable Cox regression analysis did not identify any adjuvant therapies to significantly influence recurrence in this cohort. Specifically, PORT was not associated with a significant influence on locoregional recurrence and PCI was not significantly associated with intracranial outcomes. Adjuvant chemotherapy improved survival in all stage I through III disease (hazard ratio, 0.49; 95% confidence interval, 0.29-0.81; P = .005) and even in pathologically node negative patients (hazard ratio, 0.49; 95% confidence interval, 0.27-0.91; P = .024). Although PCI was found to improve survival in univariate analysis, it was not significant in a multivariable model. PORT was not found to affect survival on either univariate or multivariable analysis.ConclusionsThis is among the largest multi-institutional studies on surgically resected limited-stage SCLC. Our results highlight survival benefit of adjuvant chemotherapy, but did not identify a statistically significant influence from mediastinal PORT or PCI in our cohort. Larger prospective studies are needed to determine the benefit of PORT or PCI in a surgically resected limited-stage SCLC population.  相似文献   

2.
PurposeThe purpose of this study was to evaluate the feasibility, safety and efficacy of percutaneous ablation (PA) of obscure hypovascular liver tumors in challenging locations using arterial CT-portography (ACP) guidance.Materials and methodsA total of 26 patients with a total of 28 obscure, hypovascular malignant liver tumors were included. There were 18 men and 6 women with a mean age of 58 ± 14 (SD) years (range: 37–75 years). The tumors had a mean diameter of 14 ± 10 (SD) mm (range: 7–24 mm) and were intrahepatic cholangiocarcinoma (4/28; 14%), liver metastases from colon cancer (18/28; 64%), corticosurrenaloma (3/28; 11%) or liver metastases from breast cancer (3/28; 11%). All tumors were in challenging locations including subcapsular (14/28; 50%), liver dome (9/28; 32%) or perihilar (5/28; 18%) locations. A total of 28 PA (12 radiofrequency ablations, 11 microwave ablations and 5 irreversible electroporations) procedures were performed under ACP guidance.ResultsA total of 67 needles [mean: 2.5 ± 1.5 (SD); range: 1–5] were inserted under ACP guidance, with a 100% technical success rate for PA. Median total effective dose was 26.5 mSv (IQR: 19.1, 32.2 mSv). Two complications were encountered (pneumothorax; one abscess both with full recovery), yielding a complication rate of 7%. No significant change in mean creatinine clearance was observed (80.5 mL/min at baseline and 85.3 mL/min at day 7; P = 0.8). Post-treatment evaluation of the ablation zone was overestimated on ACP compared with conventional CT examination in 3/28 tumors (11%). After a median follow-up of 20 months (range: 12–35 months), local tumor progression was observed in 2/28 tumours (7%).ConclusionACP guidance is feasible and allows safe and effective PA of obscure hypo-attenuating liver tumors in challenging locations without damaging the renal function and with acceptable radiation exposure. Post-treatment assessment should be performed using conventional CT or MRI to avoid size overestimation of the ablation zone.  相似文献   

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4.
Does oligometastatic breast cancer (OMBC) deserve a dedicated treatment? Although some authors recommend multidisciplinary management of OMBC with a curative intent, there is no evidence proving this strategy beneficial in the absence of a randomized trial. The existing literature sheds little light on OMBC. Incidence is unknown; data available are either obsolete or biased; there is no consensus on the definition of OMBC and metastatic sites, nor on necessary imaging techniques. However, certain proposals merit consideration. Knowledge of eventual specific OMBC biological characteristics is limited to circulating tumor cell (CTC) counts. Given the data available for other cancers, studies on microRNAs (miRNAs), circulating tumor DNA (ctDNA) and genomic alterations should be developed Finally, safe and effective therapies do exist, but results of randomized trials will not be available for many years. Prospective observational cohort studies need to be implemented.  相似文献   

5.
BackgroundUntreated pediatric choledochal cyst (CC) is associated with complications including cholangitis, pancreatitis, and risk of malignancy. Therefore, CC is typically treated by surgical excision with biliary reconstruction. Both open and laparoscopic (lap) surgical approaches are regularly used, but outcomes have not been compared on a national level.MethodsThe Nationwide Readmissions Database was used to identify pediatric patients (age 0–21 years, excluding newborns) with choledochal cyst from 2016 to 2018 based on ICD-10 codes. Patients were stratified by operative approach (open vs. lap). Demographics, operative management, and complications were compared using standard statistical tests. Results were weighted for national estimates.ResultsCholedochal cyst excision was performed in 577 children (75% female) via lap (28%) and open (72%) surgical approaches. Patients undergoing an open resection experienced longer index hospital length of stay (LOS), higher total cost, and more complications. Anastomotic technique differed by approach, with Roux-en-Y hepaticojejunostomy (RYHJ) more often utilized with open cases (86% vs. 29%) and hepaticoduodenostomy (HD) more common with laparoscopic procedures (71% vs. 15%), both p < 0.001. There was no significant difference in post-operative cholangitis or mortality.ConclusionsAlthough utilized less frequently than an open approach, laparoscopic choledochal cyst resection is safe in pediatric patients and is associated with shorter LOS, lower costs, and fewer complications. HD anastomosis is more commonly performed during laparoscopic procedures, whereas RYHJ more commonly used with the open approach. While HD is associated with more short-term gastrointestinal dysfunction than RYHJ, the latter is more commonly associated with sepsis, wound infection, and respiratory dysfunction.Level of evidenceLevel III: Retrospective Comparative Study.  相似文献   

6.
ObjectiveThe incidence of atrial fibrillation increases with age, and therefore many elderly patients presenting for cardiac surgery have atrial fibrillation. In recent publications, increasing age has been recognized as a predictor for ablation failure. Furthermore, many surgeons are reluctant to perform a surgical ablation in elderly patients. We investigated the safety and efficacy of concomitant surgical atrial fibrillation ablation in elderly patients.MethodsBetween 2003 and 2013, 556 patients underwent concomitant surgical atrial fibrillation ablation at the University Heart Center Hamburg and served as our primary study cohort. During follow-up, rhythm monitoring was established by 24-hour Holter electrocardiogram (70.5%) or an implantable loop recorder (29.5%) at 3, 6, and 12 months postoperatively. The primary end point of the study was freedom from atrial fibrillation at 12 months follow-up and the detection of deviations from a linear association between age and risk of atrial fibrillation recurrence. A multiple logistic regression model including age as a linear term was used to identify predictors for rhythm outcome.ResultsMean patients’ age was 68.4 ± 9.07 years, and 67.3% of the patients were male. Mean duration of atrial fibrillation was 3.5 ± 3.3 years, and mean left atrium diameters were enlarged with 50.5 ± 8.8 mm. There were no major ablation-related complications. The 30-day and 1-year survivals were 97.7% and 95.8%, respectively. The overall rate of freedom from atrial fibrillation ranged from 62% to 72% and was independent of age. The age-dependent risk of atrial fibrillation at 12 months was significantly increased in elderly patients undergoing a concomitant coronary artery bypass grafting surgery. Multiple logistic regression model revealed double valve procedures (odds ratio, 3.48; P = .020), preoperative persistent atrial fibrillation (odds ratio, 2.43; P = .001), and coronary artery bypass grafting surgery in elderly patients (odds ratio, 2.03; P = .009) as risk factors for recurrence of atrial fibrillation. Sinus rhythm at discharge (odds ratio, 0.39; P < .001) and bipolar ablation (odds ratio, 0.32; P < .001) were significant predictors for successful ablation.ConclusionsSurgical atrial fibrillation ablation was safe and effective independently of age. Sinus rhythm at discharge and bipolar ablation were significant predictors for successful ablation, whereas double valve procedures, preoperative persistent atrial fibrillation, and coronary artery bypass grafting surgery in elderly patients were risk factors for recurrence of atrial fibrillation.  相似文献   

7.
PurposeTo retrospectively report on safety, pain relief and local tumor control achieved with percutaneous ablation of sacral bone metastases.Materials and methodsFrom February 2009 to June 2020, 23 consecutive patients (12 women and 11 men; mean age, 60 ± 8 [SD] years; median, 60; range: 48-80 years) with 23 sacral metastases underwent radiofrequency (RFA) or cryo-ablation (CA), with palliative or curative intent at our institution. Patients’ demographics and data pertaining to treated metastases, procedure-related variables, safety, and clinical evolution following ablation were collected and analyzed. Pain was assessed with numerical pain rating scale (NPRS).ResultsSixteen (70%) patients were treated with palliative and 7 (30%) with curative intent. Mean tumor diameter was 38 ± 19 (SD) mm (median, 36; range: 11-76). External radiation therapy had been performed on five metastases (5/23; 22%) prior to ablation. RFA was used in 9 (39%) metastases and CA in the remaining 14 (61%). Thermo-protective measures and adjuvant bone consolidation were used whilst treating 20 (87%) and 8 (35%) metastases, respectively. Five (22%) minor complications were recorded. At mean 31 ± 21 (SD) (median, 32; range: 2-70) months follow-up mean NPRS was 2 ± 2 (SD) (median, 1; range: 0–6) vs. 5 ± 1 (median, 5; range: 4–8; P < 0.001) at the baseline. Three metastases out of 7 (43%) undergoing curative ablation showed local progression at mean 4 ± 4 (SD) (median, 2; range: 1-8) months follow-up.ConclusionPercutaneous ablation of sacral metastases is safe and results in significant long-lasting pain relief. Local tumor control seems sub-optimal; however, further investigations are needed to confirm these findings due to paucity of data.  相似文献   

8.
ObjectiveIn an era of broader lung sharing, different-team transplantation (DT, procuring team from nonrecipient center) may streamline procurement logistics; however, safety and cost implications of DT remain unclear. To understand whether DT represents a safe means to reduce lung transplant (LTx) costs, we compared posttransplant outcomes and lung procurement and index hospitalization costs among matched DT and same-team transplantation (ST, procuring team from recipient center) cohorts at a single, high-volume institution. We hypothesized that DT reduces costs without compromising outcomes after LTx.MethodsPatients who underwent DT between January 2016 to May 2020 were included. A cohort of patients who underwent ST was matched 1:3 (nearest neighbor) based on recipient age, disease group, lung allocation score, history of previous LTx, and bilateral versus single LTx. Posttransplant outcomes and costs were compared between groups.ResultsIn total, 23 DT and 69 matched ST recipients were included. Perioperative outcomes and posttransplant survival were similar between groups. Compared with ST, DT was associated with similar lung procurement and index hospitalization costs (DT vs ST, procurement: median $65,991 vs $58,847, P = .16; index hospitalization: median $294,346 vs $322,189, P = .7). On average, procurement costs increased $3263 less per 100 nautical miles for DT versus ST; DT offered cost-savings when travel distances exceeded approximately 363 nautical miles.ConclusionsAt our institution, DT and ST were associated with similar post-LTx outcomes; DT offered cost-savings with increasing procurement travel distance. These findings suggest that DT may mitigate logistical and financial burdens of lung procurement; however, further investigation in a multi-institutional cohort is warranted.  相似文献   

9.
PurposeThe purpose of this study was to compare the diagnostic performance of ultra-low dose (ULD) to that of standard (STD) computed tomography (CT) for the diagnosis of non-traumatic abdominal emergencies using clinical follow-up as reference standard.Materials and methodsAll consecutive patients requiring emergency abdomen-pelvic CT examination from March 2017 to September 2017 were prospectively included. ULD and STD CTs were acquired after intravenous administration iodinated contrast medium (portal phase). CT acquisitions were performed at 125 mAs for STD and 55 mAs for ULD. Diagnostic performance was retrospectively evaluated on ULD and STD CTs using clinical follow-up as a reference diagnosis.ResultsA total of 308 CT examinations from 308 patients (145 men; mean age 59.1 ± 20.7 (SD) years; age range: 18–96 years) were included; among which 241/308 (78.2%) showed abnormal findings. The effective dose was significantly lower with the ULD protocol (1.55 ± 1.03 [SD] mSv) than with the STD (3.67 ± 2.56 [SD] mSv) (P < 0.001). Sensitivity was significantly lower for the ULD protocol (85.5% [95%CI: 80.4–89.4]) than for the STD (93.4% [95%CI: 89.4–95.9], P < 0.001) whereas specificities were similar (94.0% [95%CI: 85.1–98.0] vs. 95.5% [95%CI: 87.0–98.9], respectively). ULD sensitivity was equivalent to STD for bowel obstruction and colitis/diverticulitis (96.4% [95%CI: 87.0–99.6] and 86.5% [95%CI: 74.3–93.5] for ULD vs. 96.4% [95%CI: 87.0–99.6] and 88.5% [95%CI: 76.5–94.9] for STD, respectively) but lower for appendicitis, pyelonephritis, abscesses and renal colic (75.0% [95%CI: 57.6–86.9]; 77.3% [95%CI: 56.0–90.1]; 90.5% [95%CI: 69.6–98.4] and 85% [95%CI: 62.9–95.4] for ULD vs. 93.8% [95%CI: 78.6–99.2]; 95.5% [95%CI: 76.2–100.0]; 100.0% [95%CI: 81.4–100.0] and 100.0% [95%CI: 80.6–100.0] for STD, respectively). Sensitivities were significantly different between the two protocols only for appendicitis (P = 0.041).ConclusionIn an emergency context, for patients with non-traumatic abdominal emergencies, ULD-CT showed inferior diagnostic performance compared to STD-CT for most abdominal conditions except for bowel obstruction and colitis/diverticulitis detection.  相似文献   

10.
ObjectivesThoracic surgery can cause significant pain, and multiple strategies have been developed to control pain after surgery. We compared 2 bupivacaine formulations given intraoperatively: bupivacaine with epinephrine (1,200,000) or liposomal bupivacaine.MethodsThis was a randomized, open-label study (NCT03560362). Eligible patients were adults scheduled for a minimally invasive lung procedure. Incision sites were injected with bupivacaine with epinephrine or liposomal bupivacaine before incision, and each intercostal space was injected with 1 mL of bupivacaine with epinephrine or liposomal bupivacaine entering the thoracic cavity. Patient-controlled analgesia was initiated in the recovery room. Pain was recorded using a visual analog scale. The primary outcome was the amount of narcotics taken during the postoperative hospital stay.ResultsWe recruited 50 patients; 25 received bupivacaine with epinephrine, and 25 received liposomal bupivacaine. The treatment groups were similar in age, histology, and procedure performed. There were no statistical differences between the treatment groups in the amount of narcotics required during the hospital stay (36.3 mg for bupivacaine and 38 mg for liposomal bupivacaine) or in pain assessed the day of surgery (5 and 5), the first day (3.5 and 2.3), second day (3 and 2.6), 2 weeks (0 and 1), or 3 months (0 and 0) postoperatively. Hospital length of stay and complications were also similar.ConclusionsIn a small, randomized study, we did not find significant differences between bupivacaine with epinephrine or liposomal bupivacaine in mitigating pain after minimally invasive lung resection. We currently favor using the less expensive nonliposomal bupivacaine preparations until additional data are available.  相似文献   

11.
PurposeTo determine inter-reader agreement in categorizing hepatocellular carcinoma (HCC) treated with locoregional therapy using the Liver Imaging Reporting and Data System (LI-RADS) treatment response (LR-TR) algorithm.Materials and methodsA total of 93 patients with a total of 112 HCC nodules that were treated using thermal ablation or transarterial chemoembolization were prospectively included. There were 79 men and 14 women with a mean age of 55 ± 2.6 (SD) years (range: 48–63 years). All patients underwent magnetic resonance imaging (MRI) examination of the liver and MR images were analyzed by two independent observers. Treated HCC nodules were categorized into four groups according to LR-TR scoring system including: (i) LR-TR non-evaluable (treated, response not evaluable); (ii) LR-TR nonviable (treated, probably or definitively not viable); (iii) LR-TR equivocal (treated, equivocally viable) and (iv) LR-TR viable (treated, probably or definitively viable). The inter-observer agreement in LR-TR categorization was assessed using the kappa statistics.ResultsThere was excellent inter-observer agreement between the two reviewers for overall treated HCC according to LR-TR algorithm (kappa = 0.938; 95% CI: 0.89–1.00; P = 0.001) with 97.31% agreement. The LR-TR categories by both reviewers were non-viable (77/112; 69.6% and 76/112; 67.9%), viable (30/112; 26.8% and 32/112; 27.7%) and equivocal (5/112; 4.4% and 4/112; 3.6%). There was excellent inter-observer agreement for LR-TR nonviable (kappa = 0.938; 95% CI: 0.87–1.0; P = 0.001) with 97.3% agreement, LR-TR viable (kappa = 0.955; 95% CI: 0.89–1.00; P = 0.001) with 98.2% agreement and good inter-observer agreement for LR-TR equivocal (kappa = 0.700; 95% CI: 0.28–1.0; P = 0.001) with 97.3% agreement.ConclusionLR-TR algorithm conveys high degrees of inter-observer agreement for the evaluation of treatment response of HCC after thermal ablation and transarterial chemoembolization.  相似文献   

12.
《Neuro-Chirurgie》2021,67(4):350-357
IntroductionAnterior odontoid screw fixation is a valid surgical option for unstable odontoid fractures, as type II Anderson D’Alonzo fractures. Grauer further divided type II fractures in subtypes according to the fracture line, providing recommendations for implementation of screw fixation techniques.ObjectivePrimary endpoint of our study is to evaluate the postoperative results of minimally invasive odontoid screw insertion in terms of outcome, fusion rate and stability of cranio-cervical junction. Secondary endpoint was to investigate the influence of age or fractures’ features on outcome and fusion rate.Materials and methodsWe report the clinical and radiological features of 32 patients harbouring unstable type II fractures operated by a minimally invasive odontoid screw insertion technique. All patients underwent a high resolution multiplanar CT in order to assess fracture features according to Grauer's classification; the integrity of ligaments was investigated by MRI. In addition, a preoperative neurological performance (modified Rankin Scale, mRS) was evaluated for patients either directly or interviewing their families. Follow-up at one, three and six months and 1 year have been performed (averaging 13.5 months) by cervical CT (fusion rate and stability) and mRS update. In order to investigate the influence of age on postoperative neurological performance, two groups (≤ 50 yrs, 9 pts/ > 50 yrs, 23 pts) were separately considered and analysed. Overall, we observed no surgery related complications. We also analysed the fusion rate and its correlation with patient age and Grauer's subtype of fracture.ResultsAt last available clinical follow-up, the preoperative performance was preserved (mRS 0/1: 24, 75%; mRS 2–4: 9, 15%) although with slight reduction of intact patients (mRS 0: 22 vs. 19; 71.8 vs. 59.3%). Younger patients (≤ 50 yrs) fared significantly better than older ones, achieving a good clinical outcome (mRS 0/1) in 100% vs. 69.5% (9/9 vs. 16/23 pts). Statistical analysis showed a fair correlation between age and outcome. Other factors such as sex and Grauer's type did not influence significantly the clinical outcome. Nine patients did not complete a full radiological follow-up and were therefore excluded from analysis of radiological outcome. Among the remaining 23 patients, only 25% of those who were followed three months or less showed fusion; conversely, all patients who have been examined from 6 to 48 months fused. Among the non-union patients, two underwent a second surgery by posterior approach.ConclusionsIn our recent experience, the minimally invasive AOSF proved safe and effective in treating odontoid peg fractures. Selection based on Grauer's type is mandatory to achieve best results. While in the elderly, an anterior approach is well accepted as the first choice treatment, we recommend that this option should be offered as a suitable alternative to Halo or orthosis also in younger patients since it provides prompt, excellent clinical outcome and high fusion rate especially in this age group.  相似文献   

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ObjectiveGuidelines for treatment of non–small cell lung cancer identify patients with tumors ≤2 cm and pure carcinoma in situ histology as candidates for sublobar resection. Although the merits of lobectomy, sublobar resection, and lymphoid (LN) sampling, have been investigated in early-stage non–small cell lung cancer, evaluation of these modalities in patients with IS disease can provide meaningful clinical information. This study aims to compare these operations and their relationship with regional LN sampling in this population.MethodsThe National Cancer Database was used to identify patients diagnosed with non–small cell lung cancer clinical Tis N0 M0 with a tumor size ≤2 cm from 2004 to 2017. The χ2 tests were used to examine subgroup differences by type of surgery. Kaplan-Meier method and Cox proportional hazard model were used to compare overall survival.ResultsOf 707 patients, 56.7% (401 out of 707) underwent sublobar resection and 43.3% (306 out of 707) underwent lobectomy. There was no difference in 5-year overall survival in the sublobar resection group (85.1%) compared with the lobectomy group (88.9%; P = .341). Multivariable survival analyses showed no difference in overall survival (hazard ratio, 1.044; P = .885) in the treatment groups. LN sampling was performed in 50.9% of patients treated with sublobar resection. In this group, LN sampling was not associated with improved survival (84.9% vs 85.0%; P = .741).ConclusionsWe observed no difference in overall survival between sublobar resection and lobectomy in patients with cTis N0 M0 non–small cell lung cancer with tumors ≤2 cm. Sublobar resection may be an appropriate surgical option for this population. LN sampling was not associated with improved survival in patients treated with sublobar resection.  相似文献   

14.
ObjectiveThe study objective was to verify whether the Eurolung score was associated with long-term prognosis after lung cancer resection.MethodsA total of 1359 consecutive patients undergoing anatomic lung resection (1136 lobectomies, 103 pneumonectomies, 120 segmentectomies) (2014-2018) were analyzed. The parsimonious aggregate Eurolung2 score was calculated for each patient. Median follow-up was 802 days. Survival distribution was estimated by the Kaplan–Meier method. Cox proportional hazard regression and competing risk regression analyses were used to assess the independent association of Eurolung with overall and disease-specific survival.ResultsPatients were grouped into 4 classes according to their Eurolung scores (A 0-2.5, B 3-5, C 5.5-6.5, D 7-11.5). Most patients were in class A (52%) and B (33%), 8% were in class C, and 7% were in class D. Five-year overall survival decreased across the categories (A: 75%; B: 52%; C: 29%; D: 27%, log rank P < .0001). The score stratified the 3-year overall survival in patients with pT1 (P < .0001) or pT>1 (P < .0001). In addition, the different classes were associated with incremental risk of long-term overall mortality in patients with pN0 (P < .0001) and positive nodes (P = .0005). Cox proportional hazard regression and competing regression analyses showed that Eurolung aggregate score remained significantly associated with overall (hazard ratio, 1.19; P < .0001) and disease-specific survival after adjusting for pT and pN stage (hazard ratio, 1.09; P = .005).ConclusionsEurolung aggregate score was associated with long-term survival after curative resection for cancer. This information may be valuable to inform the shared decision-making process and the multidisciplinary team discussion assisting in the selection of the most appropriate curative treatment in high-risk patients.  相似文献   

15.
《Neuro-Chirurgie》2023,69(3):101444
BackgroundAnnually, hundreds of thousands of patients undergo surgery for degenerative spine disease (DSD). This represents only a fraction of patients that present for surgical consideration. Procedures are often avoided due to comorbidities that make patients poor candidates for general anesthesia (GA) and its associated risks. With increasing interest in awake surgery under spinal anesthesia (SA), the authors have observed that SA may facilitate spine surgery in patients with relative contraindications to GA. With this in mind, the authors set out to summarize the outcomes of a series of highly comorbid patients who received surgery under SA.MethodsCase logs of a single surgeon were reviewed, and patients undergoing spine surgery under SA were identified. Within this group, patients were identified with relative contraindications to GA, such as advanced age and medical comorbidities. For these patients, for whom surgery was facilitated by SA, the medical records were consulted to report demographic information and patient outcomes.ResultsTen highly comorbid patients were identified who received lumbar spine surgery for DSD under SA. Comorbidities included octogenarian status, obesity, and chronic health conditions such as heart disease. The cohort had a mean age of 75.5 and a mean American Society of Anesthesiologists Physical Status (ASA-PS) score of 3.1. The patients were predicted to have a 2.74-fold increase of serious complications compared to the average patient. There were no adverse events.ConclusionFor patients with symptomatic, refractory DSD and relative contraindications to GA, SA may facilitate safe surgical intervention with excellent outcomes.  相似文献   

16.
Interstitial lung disease (ILD) in children (chILD) is a heterogeneous group of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. The pathogenesis of the various chILD is complex and the diseases share common features of inflammatory and fibrotic changes of the lung parenchyma that impair gas exchanges. The etiologies of chILD are numerous. In this review, we chose to classify them as ILD related to exposure/environment insults, ILD related to systemic and immunological diseases, ILD related to primary lung parenchyma dysfunctions and ILD specific to infancy. A growing part of the etiologic spectrum of chILD is being attributed to molecular defects. Currently, the main genetic mutations associated with chILD are identified in the surfactant genes SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3 and NKX2-1. Other genetic contributors include mutations in MARS, CSF2RA and CSF2RB in pulmonary alveolar proteinosis, and mutations in TMEM173 and COPA in specific auto-inflammatory forms of chILD. However, only few genotype-phenotype correlations could be identified so far. Herein, information is provided about the clinical presentation and the diagnosis approach of chILD. Despite improvements in patient management, the therapeutic strategies are still relying mostly on corticosteroids although specific therapies are emerging. Larger longitudinal cohorts of patients are being gathered through ongoing international collaborations to improve disease knowledge and targeted therapies. Thus, it is expected that children with ILD will be able to reach the adulthood transition in a better condition.  相似文献   

17.
《Neuro-Chirurgie》2022,68(3):273-279
ObjectiveTo define the prognostic factors for progression and to determine the impact of the histological grading (according to the World Health Organization) on the progression-free survival (PFS) of filum terminale ependymomas.MethodsA retrospective chart review of 38 patients with ependymoma of the filum terminale was performed, focusing on demographic data, preoperative symptoms, tumor size, quality of resection, presence of a tumor capsule, and histological grade.ResultsGross total resection (GTR) was achieved in 30 patients (78.9%). Histopathological analysis found 21 (55.3%) myxopapillary grade I ependymoma (MPE), 16 (42.1%) ependymoma grade II (EGII), and 1 (2.6%) ependymoma grade III. There was no significant difference between the mean ± SD volume of MPE (5840.5 ± 5244.2 mm3) and the one of EGII (7220.3 ± 6305.9 mm3, p = 0.5). The mean ± SD follow-up was 54.1 ± 38.4 months. At last follow-up, 30 (78.9%) patients were free of progression. In multivariate analysis, subtotal resection (p = 0.015) and infiltrative tumor (p = 0.03) were significantly associated with progression. The PFS was significantly higher in patients with encapsulated tumor than in patients with infiltrative tumor (log-rank p = 0.01) and in patients who had a GTR in comparison with those who had an incomplete resection (log-rank p = 0.05). There was no difference in PFS between patient with MPE and EGII (p = 0.1).ConclusionThe progression of ependymoma of the filum terminale highly depends on the quality of resection, and whether the tumor is encapsulated. Except for anaplastic grade, histopathological type does not influence progression.  相似文献   

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Study objectivePrevious studies have shown that prophylactic norepinephrine infusion is superior to intermittent bolus administration in preventing post-spinal hypotension. Nevertheless, it is still controversial whether manually-controlled variable-rate infusion is more effective than fixed-rate infusion. The purpose of the present study was to compare the efficacy of variable-rate infusion and fixed-rate infusion of norepinephrine for prophylaxis against maternal hypotension and maintaining hemodynamic stability during spinal anesthesia for cesarean delivery to determine more effective mode for clinical practice.DesignA prospective randomized, controlled study.SettingOperating room, Women's Hospital, Zhejiang University School of Medicine.PatientsA total of 161 parturients scheduled for elective cesarean delivery with spinal anesthesia were randomized into Group F (fixed-rate infusion) and Group V (variable-rate infusion).InterventionsParturients received prophylactic norepinephrine infusion concurrent with the intrathecal injection at rate started at 0.05 μg/kg/min. In Group F, norepinephrine was administered continuously at a fixed (on-off) rate, and a bolus of norepinephrine 5 μg or 10 μg was given when systolic blood pressure (SBP) decreased by 20% or more of baseline. In Group V, manually adjusted norepinephrine infusion within the range 0–0.14 μg/kg/min, according to SBP at 1-min intervals until delivery, aim to maintain values close to the baseline.MeasurementsDuring the study period, the incidence of maternal hypotension, hemodynamic performance, the number of physician interventions, reactive hypertension, bradycardia, nausea, vomiting, norepinephrine cumulative dose (before delivery), and neonatal outcomes were recorded.Main resultsThe incidence of maternal hypotension was significantly lower in Group V than that in Group F (9% versus 30%) (P < 0.001). No significant difference was found in the serial changes in SBP and heart rate (HR) for the first 15 min. Group V showed higher frequency of physician interventions compared with the Group F (P < 0.001). The incidence of hypertension, severe hypotension, nausea, vomiting, bradycardia, norepinephrine cumulative dose, and neonatal outcome were comparable between the two groups.ConclusionWhen norepinephrine was infused at an initial dose of 0.05 μg/kg/min for preventing hypotension during spinal anesthesia for cesarean delivery, due to technical limitations of inadequate dose design in this study, neither a variable-rate infusion (need more physician intervention) nor a fixed-rate infusion regimen (experience more transient hypotension) was optimal. However, in terms of clinical importance, how to prevent the parturients from experiencing more incidence of hypotension might be a greater concern for anesthesiologists.  相似文献   

20.
To date, conservative management including physical and/or systemic pharmacologic therapy is considered as the first line approach for the management of neuropathic pain syndromes. In the era of an opioid overdose crisis with an increased concern upon the risks and harms arising from the misuse of medicines for pain management, percutaneous minimally invasive techniques such as nerve infiltrations as well as neurolysis or neuromodulation techniques can be proposed to control pain and improve life quality. Computed tomography can serve as an ideal guiding technique due to its specific characteristics including precise anatomic delineation, high spatial resolution and good tissue contrast. The purpose of this review is to make the reader familiar with the most common indications for minimally invasive imaging-guided techniques in patients with neuralgia and provide current evidence regarding technical considerations.  相似文献   

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