scholarly journals Reduced risk of recurrent pneumothorax for sirolimus therapy after surgical pleural covering of entire lung in lymphangioleiomyomatosis

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Teiko Sakurai ◽  
Toru Arai ◽  
Masaki Hirose ◽  
Kensuke Kojima ◽  
Tetsuki Sakamoto ◽  
...  

Abstract Background Patients with lymphangioleiomyomatosis (LAM) frequently experience pneumothorax. Although sirolimus is the standard therapy for LAM, its effect on pneumothorax is controversial. Recently, total pleural covering (TPC) and modified TPC (mTPC) were introduced as surgical treatment options for pneumothorax for patients with LAM. However, the effect of sirolimus on the recurrence of pneumothorax in patients who underwent the treatments is still uncertain. We hypothesized that some clinical factors including sirolimus treatment could predict postoperative recurrence of pneumothorax. In order to clarify this hypothesis, we retrospectively analyzed the clinical data from 18 consecutive patients with LAM who underwent 24 surgical pleural covering of entire lung (SPC) as 17 TPC and 7 mTPC against pneumothoraces from surgical database between January 2005 and January 2019, and we determined the predictors of postoperative recurrence. Results Of the 24 surgeries of SPC, 14 surgeries (58.3%) had a history of two or more ipsilateral pneumothoraces, and 11 surgeries (45.8%) had a history of ipsilateral pleural procedures before SPC. Sixteen surgeries (66.6%) in 12 patients received treatment of sirolimus after SPC (sirolimus group). With a median follow-up time of 69.0 months after SPC, four surgeries (16.6%) in three patients had a postoperative recurrence, and the 5-year recurrence-free survival (RFS) after SPC was 82.9%. In patients with postoperative recurrence, serum level of vascular endothelial growth factors D was significantly higher than that in those with non-recurrence (3260.5 vs. 892.7 pg/mL, p = 0.02), and the rate of sirolimus treatment in the recurrence group was significantly lower than that in the no-recurrence group (0 vs. 80%, p = 0.006). The log-rank test showed that the RFS of the sirolimus group (sirolimus use after SPC) was significantly better than that of the non-sirolimus group (p = 0.001), and no significant difference was observed for other factors. Conclusion We first reported sirolimus might effectively suppress the recurrence of pneumothoraces in LAM patients who received SPC. Sirolimus induction after SPC (TPC or mTPC) might be a feasible option for frequent pneumothorax in LAM.

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 22-22
Author(s):  
Allison Taylor ◽  
Kimberley Doucette ◽  
Bryan Chan ◽  
Xiaoyang Ma ◽  
Jaeil Ahn ◽  
...  

Introduction The literature suggests a widespread reduction in the availability and accessibility of newer treatment options among marginalized groups in AML. Studies from large national databases point to lower socio-economic status, Hispanic and African American race, Medicare or no insurance, being unmarried, treatment at non-academic centers, and rural residence as negatively impacting overall survival (OS) and rates of chemotherapy utilization in AML patients (Patel et al. 2015, Jaco et al. 2017, Bhatt et al. 2018, Master et al. 2016). We hypothesized that facility affiliation and pt volume would also have important effects on time to treatment (TTT) and OS in AML, even when these socioeconomic disparities were accounted for. Methods For this retrospective analysis, we used NCDB data that included 124,988 pts over the age of 18 with AML between the years 2004-2016. Variables analyzed included facility types described as community cancer programs (CP), comprehensive community cancer programs (CCP), academic/research center cancer programs (AC) and integrated network cancer programs (IN), and volume of facilities defined as high volume (HV) and low volume (LV). HV facilities had case volumes of ≥ 99th percentile and all other facilities were classified as LV. Multivariate analyses (MVA) included demographic and socioeconomic covariables. We used Cox proportional hazard analysis for both TTT and OS MVA. The Kaplan-Meier method was used to estimate median TTT and OS, and the log rank test used to compare TTT and OS across predictor variables. Results The median age of AML patients was 63 yrs (range 18-90) with 54% males, and 86% Caucasian. Five percent of patients were treated at CP, 30% at CCP, 44% at AC, and 10% at IN. 21% at HV facilities and 79% at LV facilities. Median TTT in days at CP facilities was 7, compared to 5 days in CCP and AC facilities versus 4 days at IN (p<0.0001). TTT was 5 days at HV facilities versus 4 days at LV facilities (p<0.0001). Kaplan-Meier curves showed that TTT was similar between HV and LV facilities(figure 1). The median OS was 3.25 months in CP compared to 4.34 months at CCP, 5.06 months at IN and 9.53 months at AC (p<0.0001). For facility volume, the median OS was 13.11 months in HV facilities compared to 6.93 months in LV facilities (p<0.0001). When sex, race, age, Hispanic Origin, education, urban/rural residence, Charlson-Deyo Comorbidity score and Great Circle Distance were adjusted for in MVA (table 1), the OS was higher in AC versus CP facilities (hazard ratio [HR] of 0.90 (0.87-0.93, p<0.0001), and there was no statistically significant difference with comparison of other facility types to CP. Similarly, there was a lower OS at LV versus HV facilities with a HR of 1.14 (1.12-1.16, p<0.0001). CCP facilities had a shorter TTT compared to CP with a HR of 1.21 (1.17-1.26, p<0.0001). AC had a shorter TTT than CP with a HR of 1.17 (1.13-1.22, p<0.0001), and IN had a shorter TTT compared to CP with a HR of 1.29 (1.24-1.34, p<0.0001). Additionally, TTT in the MVA for facility volume was shorter in LV facilities compared to HV facilities with HR of 1.05 (1.04-1.07, p<0.0001) [table 1]. Conclusion When adjusting for various socioeconomic factors, we found that TTT was longest in CP compared to CCP, AC, and IN. Treatment at a LV facility resulted in a decreased overall survival. LV facilities may be less familiar with treatment regimens for AML, less likely to use novel treatment options, and be less familiar with the disease. We showed that treatment at an AC compared to CP, CCP and IN facilities improved survival. Given poor outcomes for AML, these results show the importance of going to AC and HV facilities with more experience in treating AML for improved outcomes. Disclosures Lai: Astellas: Speakers Bureau; Jazz: Speakers Bureau; Abbvie: Consultancy; Agios: Consultancy; Macrogenics: Consultancy.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e15587-e15587
Author(s):  
Irem Bilgetekin ◽  
Mehmet Dogan ◽  
Cengiz Karacin ◽  
Fatma Bugdayci Basal ◽  
Ece Esin ◽  
...  

e15587 Background: Expanded RAS analysis is essential for the selection of biologic agents in mCRC. RAS mutations indicates anti-EGFR unresponsiveness. In this study, we aimed to investigate RAS and BRAF mutations by liquid biopsy at progression in patients with RAS mutant mCRC. Methods: Sixty patients with mCRC who harbored tissue RAS mutations were prospectively analyzed between July 2019 and April 2020. All the patients treated with chemotherapy plus bevacizumab combinations . The plasma samples of the patients were analyzed after progression of bevacizumab combinations. RAS mutation profile was evaluated in plasma using Idylla PCR-based molecular diagnostics method, which enables rapid detection of common mutations in RAS and BRAF genes in circulating tumor DNA (ctDNA). Kaplan-Meier method was used for survival analysis and log-rank test was performed for comparison of groups. Results: The median age of the patients was 60 years (IQR:35-83 years) and female was (n=23, 38.3%). Primary tumor was located in the left colon in 81.7% of all patients. There were 95.0% KRAS and 5% NRAS mutations in baseline tissue biopsy. As a result of liquid biopsy after progression, 55.0% of the patients had KRAS, 3.3% NRAS and 3.3% had BRAF mutations. The RAS mutation detected in 58.3% of the patients. While there was no significant difference in terms of clinicopathological features between wild type (RAS/BRAF) and mutant type (RAS/BRAF) determined by liquid biopsy, the overall survival (OS) of the wild type group was significantly longer than mutant group (43.8 vs. 20.4 months, p= 0.002). Conclusions: This study demonstrated that there may be changes in RAS/BRAF mutation from plasma analysis after progression in patients with mCRC. Since better survival in the patient group with wild type was detected compared to the RAS concordance group, the evaluation of RAS mutation status at the time of progression may be important in terms of disease prognosis and treatment options.


2019 ◽  
Vol 19 (1) ◽  
pp. 79
Author(s):  
Ria Febrina

In the Special Region of Yogyakarta (DIY), the Infant Mortality Rate (IMR) is 25 per 1000 live births, the most common causes of infant mortality in DIY are low birth weight (LBW) and sepsis. LBW is most commonly found in Sleman Regency as many as 823 babies. One of the management of LBW babies is exclusive breastfeeding. Exclusive breastfeeding coverage in Yogyakarta City in 2015 was 71.62%. The purpose of this study is to obtain information on the differences in the success of exclusive breastfeeding between counseling and education. In addition, it is to understand the relationship of extraneous variables (age, education, occupation, parity) with the success of exclusive breastfeeding in mothers who breastfeed a baby with a history of LBW.The type of this research was Quasi-Experimental, Posttest Only Control Design, using breastfeeding mother respondent who had a 4-months-old baby with a history of LBW as much as 51 people, sample as much as 30 people based on minimum sample size for the experiment. The location of the research was in 14 working area of community health center in Sleman Regency. The determination of the samples in each location was determined based on Proportional Random Sampling. The instrument used in determining the success of exclusive breastfeeding was a questionnaire. The data obtained were analyzed using Wilcoxon Sign Rank Test and Chi-square test with a significance level of 0.05.The results showed no significant difference in the success of exclusive breastfeeding between the groups of counseling and education (p-value 0.414). Education and parity had a significant relationship to the success of exclusive breastfeeding (p-value 0.003 and p-value 0-016).


2021 ◽  
Vol 8 ◽  
Author(s):  
Lun Xu ◽  
Wending Huang ◽  
Weiluo Cai ◽  
ZhengWang Sun ◽  
Meng Fang ◽  
...  

Objective: This study aimed to compare the outcomes between piecemeal spondylectomy and separation surgery for patients with spinal metastasis.Summary of Background Data: Piecemeal spondylectomy and separation surgery are two widely-used treatment options for spinal metastasis. However, no studies have compared the surgical outcomes between both treatment modalities.Methods: Patients with spinal metastasis who underwent piecemeal spondylectomy or separation surgery between August 2017 and April 2020 at our spine center were recruited. Demographic, preoperative, perioperative, and follow-up data were collected and analyzed. Kaplan–Meier analysis and the log-rank test were used to analyze overall survival (OS) and progression-free survival (PFS) in patients with spinal metastasis.Results: Overall, 26 patients were treated with piecemeal spondylectomy, and 29 underwent separation surgery with postoperative stereotactic radiosurgery. Both groups showed significant postoperative improvements in neurological status. The piecemeal spondylectomy group had significantly more blood loss (1784.62 ± 833.64 vs. 1165.52 ± 307.38 ml) and required longer operative time (4.76 ± 0.93 vs. 3.73 ± 1.15 h) than the separation surgery group. No significant difference in OS was found between the groups (P = 0.064); however, patients in the separation surgery group experienced less local recurrence than those in the piecemeal spondylectomy group (P = 0.0014). Notably, significant differences were detected in the development of complications between the groups (P = 0.029).Conclusion: Separation surgery led to less blood loss and reduced complications and had shorter operation time than piecemeal spondylectomy. Although no significant differences were found in OS between the groups, separation surgery was associated with better PFS compared with piecemeal spondylectomy. These findings suggest that separation surgery has some advantages over piecemeal spondylectomy for patients with spinal metastatic disease.


2015 ◽  
Vol 33 (3_suppl) ◽  
pp. 357-357 ◽  
Author(s):  
Madappa N. Kundranda ◽  
Donald Peter Braun ◽  
Kim Kramer ◽  
Jizhou Ai ◽  
Bradford A. Tan ◽  
...  

357 Background: Even with the advent of newer systemic therapies; long term survival in advanced PDAC is dismal. Hence there is an urgent need to use technologies such as NGS to identify potential therapeutic targets. Methods: A retrospective analysis was performed of all advanced PDAC patients (pts) evaluated with NGS through Foundation One (Foundation Medicine Inc., MA). DNA was extracted from biopsy specimens and sequencing was performed of 315 cancer-related genes plus select introns. Statistical analysis including Kaplan Meier survival analysis along with the log-rank test was used to compare the differences between groups. Results: 53pts were analyzed between Nov 2012 - Sep 2014; 25 (47%) were females; median age was 59 yrs (range: 33-77). 178 genomic alterations (GAs) were identified in 52 pts (average 3.43 GAs/pt). The GAs included mutations 140 (78%), amplifications 25 (14%), loss/deletions 13 (7%) and 1 pt had no GAs. Most frequent GAs and associated survival are listed in Table 1. There was no statistically significant difference in median overall survival (mOS) between the groups with or without KRAS (22.1 vs. 21 months; p=0.95) and with or without TP53 (21 vs. 23.4 months; p=0.76). 6/52 pts (12%) who had received at least 3 prior lines of therapy (range 3-4) and had an ECOG performance status <2; were treated off label with trametinib. The median PFS on the last therapy prior was 5 months (range 1.6-7). On trametinib, the median PFS was 1.9 months (range 1.1-7.1) with mOS of 5.1 months. Conclusions: Our data demonstrate that there are a range of GAs that are eligible for investigational targeted therapies. Secondly, the choice of agents (eg. Trametinib - targets downstream RAS effectors ) based on individual genomic profile can be considered when an appropriate clinical trial is not available or inaccessible even in heavily pretreated patients with a good performance status. Tumor profiling should be utilized in drug development to personalize treatment options in patients with advanced PDAC. [Table: see text]


1997 ◽  
Vol 77 (05) ◽  
pp. 0822-0824 ◽  
Author(s):  
Elvira Grandone ◽  
Maurizio Margaglione ◽  
Donatella Colaizzo ◽  
Marina d'Addedda ◽  
Giuseppe Cappucci ◽  
...  

SummaryActivated protein C resistance (APCR) is responsible for most cases of familial thrombosis. The factor V missense mutation Arg506>Gln (FV Leiden) has been recognized as the commonest cause of this condition. Recently, it has been suggested that APCR is associated with second trimester fetal loss. We investigated the distribution of FV Leiden in a sample (n = 43) of Caucasian women with a history of two or more unexplained fetal losses. A group (n = 118) of parous women with uneventful pregnancies from the same ethnical background served as control. We found the mutation in 7 cases (16.28%) and 5 controls (4.24%; p = 0.011). A statistically significant difference between women with only early fetal loss vs those with late events (p = 0.04) was observed. Our data demonstrate a strong association between FV Leiden and fetal loss. Furthermore, they indicate that late events are more common in these patients.


1997 ◽  
Vol 77 (05) ◽  
pp. 0955-0958 ◽  
Author(s):  
Carole A Foy ◽  
Peter J Grant

SummaryPAI-2 is a fibrinolytic inhibitor produced predominantly by monocytes. Most PAI-2 is intracellular making study in clinical conditions difficult. Abnormalities in production may be associated with inflammation and fibrinolysis at sites of tissue damage such as the atherosclerotic plaque.PAI-2 gene variants have been described: variant A consists of Asn120, Asn404 and Ser413 and variant B consists of Asp120, Lys404 and Cys413. We designed a PCR-RFLP assay using primers spanning the region containing Asn/Lys404 and Ser/Cys413. Variant B contains an Mwol restriction site. We analysed 302 Pima Indians and 286 healthy Caucasian volunteers. To investigate relationships between genotype and vascular disease we analysed 333 Caucasian patients undergoing coronary angiography.Gene variant B was more common in the Pimas than in Caucasians (p <0.0001). There was no significant difference in genotype distribution between the volunteers and patients. In the patients there was no association between genotype and either a history of MI or extent of coronary atheroma.


2008 ◽  
Vol 149 (17) ◽  
pp. 801-805
Author(s):  
Péter Rajnics ◽  
László Krenács ◽  
András Kenéz ◽  
Zoltán Járay ◽  
Enikő Bagdi ◽  
...  

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein–Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.


2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Farokh Saljughi ◽  
Mitra Savabi-Esfahani ◽  
Shahnaz Kohan ◽  
Soheila Ehsanpour

Mother-infant attachment is an intimate, lasting and satisfying relationship that leads to better cognitive, emotional and social growth of the infant. The aim of this study was to determine the effects of breastfeeding training by role-play on mother-infant attachment behaviours. This research was a randomised clinical trial (parallel design). Inclusion criteria were: no history of mental disorders; ability to read and write the Persian language to complete the questionnaire; no history of drug and tobacco intake in primigravida women. The sample comprised 100 pregnant women (in 2 groups), selected through simple random sampling at healthcare centres. The researcher reviewed prenatal care registries of selected healthcare centres and extracted the names of pregnant women in their early third trimester. The data were imported into randomisation software. The control group received routine breastfeeding training, while the intervention group received routine training together with training through role-play. The data collection tool was the Maternal Behaviour Inventory Questionnaire. Consequently 75 samples were analysed in SPSS16. Independent t-tests and chi-square tests were used to examine the difference between the two groups. Results showed that the mean score of mother-infant attachment one week after delivery was significantly higher in the intervention group in comparison to that in the control group (p<0.001). No significant difference was observed between the two groups in maternal age, age of marriage, neonatal gender, maternal employment and education, number of parity, and number of abortions (P>0.05). Since breastfeeding training through role-play could affect mother-infant attachment, it is suggested that this type of training should be provided for pregnant women to promote mother-infant attachment and exclusive breastfeeding.


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