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1.
Andrea Ferrari MD Daniel Orbach MD Michela Casanova MD Max M. van Noesel MD Pablo Berlanga MD Bernadette Brennan MD Nadege Corradini MD Reineke A. Schoot MD Gema L. Ramirez-Villar MD Lisa Lyngsie Hjalgrim MD Rita Alaggio MD Gabriela Guillen Burrieza MD Akmal Safwat MD Alison L. Cameron MD Rick R. van Rijn MD Veronique Minard-Colin MD Ilaria Zanetti BSc Gianni Bisogno MD Julia C. Chisholm MD Johannes H. M. Merks MD 《Cancer》2023,129(16):2542-2552
Background
Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.Methods
This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.Results
The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.Conclusions
The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.Plain Language Summary
- Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
- Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
- This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.
2.
Maadrika M.N.P. Kanglie Nanko de Graaf Femke Beije Elise M.J. Brouwers Sabine D.M. Theuns-Valks Frits H. Jansen Diederick B.W. de Roy van Zuidewijn Bas Verhoeven Rick R. van Rijn Roel Bakx 《Journal of pediatric surgery》2019,54(3):500-506
Background
There is a lack of studies addressing the occurrence of negative intraoperative findings (that is the absence of intussusception) after an unsuccessful hydrostatic reduction of an ileocolic intussusception. The aim of this study is to determine the incidence of negative intraoperative findings after unsuccessful hydrostatic reduction of ileocolic intussusception.Methods
We conducted a multicentre retrospective study of all children aged 0–18?years treated for ileocolic intussusception from January 1, 2010 to December 31, 2015 in 9 Dutch hospitals. Primary outcome measure was the percentage of children without an intussusception during surgical exploration after unsuccessful hydrostatic reduction.Results
In the study period 436 patients were diagnosed with an ileocolic intussusception. Of these, 408 patients underwent hydrostatic reduction of an ileocolic intussusception. 112 patients (27.5%) underwent surgery after an unsuccessful hydrostatic reduction. In 13 (11.6%) patients no intraoperative evidence of intussusception was found. Patients who underwent surgical intervention after unsuccessful hydrostatic reduction were significantly younger than patients who had a successful hydrostatic reduction; there was no gender difference.Conclusion
A substantial number of children (11.6%) underwent a laparotomy after unsuccessful hydrostatic reduction in whom no intussusception was found intraoperatively. We suggest initiating laparoscopy instead of laparotomy when surgery is necessary.Level of evidence
Level II. 相似文献3.
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5.
Yang Yang Perkins D. Rick Stearns A. Elizabeth 《International journal of mental health and addiction》2020,18(4):1048-1066
International Journal of Mental Health and Addiction - While a plethora of studies indicating substance use treatment reduced measurable, pathological symptoms, research is still needed to explore... 相似文献
6.
Hao Cui Hartzell V. Schaff Joseph A. Dearani Brian D. Lahr Jason K. Viehman Jeffrey B. Geske Rick A. Nishimura Steve R. Ommen 《The Journal of thoracic and cardiovascular surgery》2021,161(3):997-1006.e3
ObjectiveTo evaluate the outcomes after septal myectomy in patients with obstructive hypertrophic cardiomyopathy according to atrial fibrillation and surgical ablation of atrial fibrillation.MethodsWe reviewed patients with obstructive hypertrophic cardiomyopathy who underwent septal myectomy at the Mayo Clinic from 2001 to 2016. History of atrial fibrillation was obtained from patient histories and electrocardiograms. All-cause mortality was the primary end point.ResultsA total of 2023 patients underwent septal myectomy, of whom 394 (19.5%) had at least 1 episode of atrial fibrillation preoperatively. Among patients with atrial fibrillation, 76 (19.3%) had only 1 known episode, 278 (70.6%) had recurrent paroxysmal atrial fibrillation, and 40 (10.2%) had persistent atrial fibrillation. Surgical ablation was performed in 190 patients at the time of septal myectomy, including 148 with pulmonary vein isolation and 42 with the classic maze procedure. Among all patients, operative mortality was 0.4%, and there were no early deaths in patients undergoing surgical ablation. Over a median follow-up of 5.6 years, patients with preoperative atrial fibrillation had increased mortality (hazard ratio, 1.36; 95% confidence interval, 0.97-1.91; P = .070) after multivariable adjustment for comorbidities. When considering the impact of atrial fibrillation with or without surgical treatment, the adjusted hazard ratio for mortality in patients undergoing ablation compared with no ablation was 0.93 (95% confidence interval, 0.52-1.69; P = .824).ConclusionsAtrial fibrillation is present preoperatively in one-fifth of patients with obstructive hypertrophic cardiomyopathy undergoing myectomy and showed a trend toward higher all-cause mortality. Survival of patients undergoing septal myectomy with preoperative atrial fibrillation was similar between those who did and did not receive concomitant surgical ablation. 相似文献
7.
Roderick P.P.W.M. Maas MD Rick C.G. Helmich MD PhD Bart P.C. van de Warrenburg MD PhD 《Movement disorders》2020,35(2):215-227
Over the last three decades, measuring and modulating cerebellar activity and its connectivity with other brain regions has become an emerging research topic in clinical neuroscience. The most important connection is the cerebellothalamocortical pathway, which can be functionally interrogated using a paired-pulse transcranial magnetic stimulation paradigm. Cerebellar brain inhibition reflects the magnitude of suppression of motor cortex excitability after stimulating the contralateral cerebellar hemisphere and therefore represents a neurophysiological marker of the integrity of the efferent cerebellar tract. Observations that cerebellar noninvasive stimulation techniques enhanced performance of certain motor and cognitive tasks in healthy individuals have inspired attempts to modulate cerebellar activity and connectivity in patients with cerebellar diseases in order to achieve clinical benefit. We here comprehensively explore the therapeutic potential of these techniques in two movement disorders characterized by prominent cerebellar involvement, namely the degenerative ataxias and essential tremor. The article aims to illustrate the (patho)physiological insights obtained from these studies and how these translate into clinical practice, where possible by addressing the association with cerebellar brain inhibition. Finally, possible explanations for some discordant interstudy findings, shortcomings in our current understanding, and recommendations for future research will be provided. © 2019 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society. 相似文献
8.
Rachael Purri BA Laura Brennan PhD Jacqueline Rick PhD Sharon X. Xie PhD Benjamin L. Deck BS Lana M. Chahine MD Nabila Dahodwala MD Alice Chen-Plotkin MD John E. Duda MD James F. Morley MD PhD Rizwan S. Akhtar MD PhD John Q. Trojanowski MD PhD Andrew Siderowf MD MSCE Daniel Weintraub MD 《Movement disorders》2020,35(9):1618-1625
9.
Anita Nguyen Hartzell V. Schaff Dustin Hang Rick A. Nishimura Jeffrey B. Geske Joseph A. Dearani Brian D. Lahr Steve R. Ommen 《The Journal of thoracic and cardiovascular surgery》2019,157(1):306-315.e3
Objectives
In patients with hypertrophic cardiomyopathy, obstruction of the left ventricular outflow tract can be relieved by surgical septal myectomy or alcohol septal ablation, but uncertainty remains regarding long-term results and comparative effectiveness of alcohol septal ablation. This study aims to compare short- and long-term outcomes of the 2 procedures.Methods
Between December 1998 and September 2016, 2407 patients underwent septal myectomy and 211 patients underwent alcohol septal ablation at our institution. After 2:1 propensity score matching, the study cohort included 334 patients who underwent myectomy and 167 patients who underwent alcohol septal ablation.Results
Median (interquartile range) ages of patients in the myectomy and alcohol septal ablation groups were 65 (58-71) years and 64 (56-73) years (P = .9), respectively. After intervention, median resting left ventricular outflow tract gradient at predischarge transthoracic echocardiography was 0 (0-10) mm Hg in the myectomy group (n = 288) and 21 (10-60) mm Hg in the alcohol septal ablation group (n = 63) (P < .001, tested at baseline gradients of 30 and 50 mm Hg). There were no differences in survival between the 2 groups (risk of death for alcohol septal ablation vs myectomy, hazard ratio, 1.5; 95% confidence interval, 0.9-2.6; P = .1). Survival of patients undergoing septal myectomy was better than that of an age-, sex-, and race-matched US population (82% vs 75% at 12 years, P = .01). Reintervention for left ventricular outflow tract obstruction was more likely to occur in patients who received alcohol septal ablation (hazard ratio, 33.3; 95% confidence interval, 4.4-250.6; P < .001).Conclusions
There were no differences in survival of patients undergoing myectomy or alcohol septal ablation, but freedom from reintervention and early and late reduction of left ventricular outflow tract gradient are superior in patients undergoing septal myectomy. 相似文献10.
Heather K. Knych Rick M. Arthur Kirsten L. Kanarr Dan S. McKemie Philip H. Kass 《Drug testing and analysis》2019,11(9):1431-1443
Methamphetamine is a central and peripheral nervous system stimulant. There is only a single study that describes exposure to and disposition of this compound in horses. The potential for abuse and inadvertent exposure in equine athletes along with the limited data available necessitates further study. The objectives of the current study were to describe drug and metabolite concentrations, develop an analytical method that could be used to regulate its use, and describe selected pharmacodynamic effects. In phase 1, six horses were randomized into three transmucosal dose groups (n = 2/group; 0.5, 1.0 or 10 mg). In phase 2, horses received a single 10 mg intravenous dose. In phase 3, the effects of urinary pH on elimination were studied. Blood and urine samples were collected for up to 72 hours post drug administration. Concentrations of methamphetamine were measured using liquid chromatography–tandem mass spectrometry. Methamphetamine was below the limit of detection (LOD) in blood by 2, 4, and 18 hours following transmucosal administration of 0.5, 1, and 10 mg, respectively. Following intravenous administration, methamphetamine fell below the LOD between 12 and 18 hours. Following urinary acidification, methamphetamine fell below the limit of quantitation (LOQ) by 12 hours. In urine, methamphetamine was no longer detected at 48, 48, and 72 hours in the 0.5, 1, and 10 mg transmucosal groups and 18 hours in the intravenous group. Increased urinary pH resulted in urinary concentrations of methamphetamine falling below detectable levels by 48 hours post transmucosal administration. While the number of animals was small, behavioral, stimulatory, and cardiac effects were minimal. 相似文献