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21.

Background

Complete encasement of the inferior vena cava by retroperitoneal tumors is rare. Although replacement of the vena cava has been considered for various conditions in adults, it is rarely used in children except for challenging resections and as a last chance approach – often aiming more at debulking than cure.

Materials and methods

From January 2009 to February 2017, 4 patients (2 adrenal neuroblastomas, 1 renal cell carcinoma, 1 infantile fibrosarcoma) underwent elective en-bloc resection of tumor and of the infrahepatic portion of the inferior vena cava (IVC), with planned IVC prosthetic replacement. In three cases a portion of the left renal vein had to be resected as well, with the vein reanastomosed onto the prosthesis, and a concomitant auto-transplantation of the right kidney was associated in one neuroblastoma patient.

Results

All patients had an uncomplicated postoperative course. In one patient, the prosthetic conduit is patent at long-term (43?months), while the middle portion of the prosthesis did eventually thrombose at mid-term after surgery in the three others – with no related symptoms. Interestingly, all renal venous reconstructions remain patent. Three patients (2 neuroblastomas and 1 infantile fibrosarcoma) are alive and disease-free at 43, 74 and 108?months after surgery, respectively. One patient with renal cell carcinoma died of recurrence of the disease 21?months after surgery.

Conclusion

Resection and reconstruction of the vena cava, including the renal vein portion, can be considered and planned electively in case of tumoral encasement. This strategy is associated with good tolerance of the operation, low morbidity and satisfactory long-term function, even in cases with progressive and/or secondary partial thrombosis.

Level of Evidence

IV  相似文献   
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Permissive beliefs relate to the acceptability of engaging in alcohol use in spite of obvious potential negative consequences. They are considered the most proximal and precipitating cognitive factor in the decision to use alcohol and/or the activation of strategies to obtain it. Recent research suggested that ‘desire thinking’ may be involved in the escalation of craving and addictive behaviours and can play a role in strengthening permissive beliefs. The current study tested whether the induction of desire thinking would have a stronger effect on rate of conviction in permissive beliefs compared to a control cognitive response in the form of neutral thinking and whether this effect would be specific for patients with alcohol use disorder (AUD). Thirty AUD patients and 30 social drinkers (SD) were randomly allocated to two thinking manipulation tasks (desire thinking and neutral thinking). Current permissive beliefs were measured before and after manipulation and after a resting phase. Findings showed that desire thinking increased the level of current permissive beliefs after manipulation relative to the neutral thinking condition for the AUD group but not for the SD group. This effect was not purely dependent on the concurrent level of perceived craving. This study supports a causal relationship between the induction of desire thinking and rate of conviction in permissive beliefs and highlights the relevance of targeting desire thinking in the treatment for AUD patients.  相似文献   
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Cognitive Therapy and Research - The Self Regulatory Executive Function (S-REF) model implicates maladaptive metacognitive beliefs and processes in the predisposition and/or maintenance of positive...  相似文献   
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Neurological Sciences - Maladaptive cognitive strategies and reduced autonomic flexibility have been reported in chronic pain conditions. No study to date addressed the effects of maladaptive...  相似文献   
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The cyclic adenosine monophosphate (cAMP) intracellular signaling pathway mediates the physiological effects of several hormones and neurotransmitters, acting by the activation of G‐protein coupled receptors (GPCRs) and several downstream intracellular effectors, including the heterotrimeric stimulatory G‐protein (Gs), the cAMP‐dependent protein kinase A (PKA), and cAMP‐specific phosphodiesterases (PDEs). Defective G‐protein–mediated signaling has been associated with an increasing number of disorders, including Albright hereditary osteodistrophy (AHO) and pseudohypoparathyroidism (PHP), a heterogeneous group of rare genetic metabolic disorders resulting from molecular defects at the GNAS locus. Moreover, mutations in PRKAR1A and PDE4D genes have been recently detected in patients with acrodysostosis (ACRDYS), showing a skeletal and endocrinological phenotype partially overlapping with AHO/PHP. Despite the high detection rate of molecular defects by currently available molecular approaches, about 30% of AHO/PHP patients still lack a molecular diagnosis, hence the need to screen patients negative for GNAS epi/genetic defects also for chromosomal regions and genes associated with diseases that undergo differential diagnosis with PHP. According to the growing knowledge on Gsα‐cAMP signaling‐linked disorders, we investigated our series of patients (n = 81) with a clinical diagnosis of PHP/AHO but negative for GNAS anomalies for the presence of novel genetic variants at PRKAR1A and PDE4D genes. Our work allowed the detection of 8 novel missense variants affecting genes so far associated with ACRDYS in 9 patients. Our data further confirm the molecular and clinical overlap among these disorders. We present the data collected from a large series of patients and a brief review of the literature in order to compare our findings with already published data; to look for PRKAR1A/PDE4D mutation spectrum, recurrent mutations, and mutation hot spots; and to identify specific clinical features associated with ACRDYS that deserve surveillance during follow‐up. © 2016 American Society for Bone and Mineral Research.  相似文献   
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So far, only three small outdated studies have investigated hepatitis C virus (HCV) incidence and risk factors among illicit drug users (DUs) in Italy. Thus, during 2007–2010, we conducted a prospective cohort study among DUs attending 17 Italian rehabilitation centers serving urban areas. Two hundred eighty-four HCV-uninfected DUs were prospectively followed by interview and anti-HCV antibody and RNA testing every 6 months. Incidence was calculated using the person-years method. Infection predictors were assessed by time-dependent Cox analysis. Participants were mostly male (83.4%), under opioid substitution therapy (OST) (78.9%), non-injecting DUs (67.9%), and with a mean age of 30.8. Ninety-one of 224 DUs initially under OST interrupted treatment during the follow-up. Overall HCV incidence was 5.83/100 person-years at risk (PYAR) [95% confidence intervals (CI), 3.63–9.38]. The incidence did not significantly differ according the participants’ sociodemographic characteristics or the degree of urbanization of the towns involved in the study. The incidence was higher for DUs under than for those not under OST (6.23 vs 4.50/100 PYAR; p = 0.681). Incidence was also higher for those with than for those without OST interruption (7.17 vs 5.04/100 PYAR; p = 0.55). However, all these differences were non-significant. At last follow-up visit, a significant decrease in frequency of sharing equipment for preparation/using drugs (by injection or not) was observed by analyzing either the whole cohort or DUs under OST only. Anti-HCV seroconversion resulted independently associated with sharing drug preparation/use equipment, backloading, having a HCV-positive sexual partner, or household and (marginally) intravenous injection. In this study, HCV incidence was non-negligible and OST seemed to lack effectiveness in reducing it. In Italy, implementation of combined harm reduction interventions and antiviral treatment of chronically infected DUs would be needed.  相似文献   
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