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WOS: 000512563000001, PubMed: 32013622, Aim of the study: Biologic reconstruction using tumor-bearing bone autografts devitalized by liquid nitrogen or extracorporeal irradiation (oncological sterilization) is a safe and effective method in musculoskeletal surgery. the purpose of this study was to examine the effects of these two oncological sterilization methods on nerve recovery. Methods: A total of 48 rats were randomly divided into 3 groups as autograft, irradiation and liquid nitrogen groups. A nerve defect created in the right sciatic nerve was reconstructed with an autograft obtained from the nerve itself. Group I underwent reconstruction with standard nerve autograft. Group II and Group III underwent reconstruction with devitalized nerve autograft treated through extracorporeal irradiation and liquid nitrogen, respectively. the left sciatic nerves of the rats served as control. Electromyography, motor function test and histomorphological analysis were performed to assess the nerve recovery on the 3rd (early stage) and 4th months (late stage). Results: Electrophysiological assessment revealed better results in irradiation group compared with liquid nitrogen group in terms of myelinization and axonal regeneration. Motor performance of the autograft group was slightly better than the other groups. Histologically, autograft group demonstrated better results compared with other groups. Late-stage assessments revealed high rates of myelinization in the graft segment in liquid nitrogen group and in the segment distal to the graft in irradiation group. Conclusions: This study has demonstrated that nerve autografts treated by oncological sterilization methods may be used for nerve reconstruction in limb salvage surgery. However, further studies are needed to clarify the applicability of these methods., Ege University Scientific Research FoundationEge University [15-TIP-013], This work was supported by Ege University Scientific Research Foundation (15-TIP-013).

© 2022 International Society of NephrologyIntroduction: Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disorder characterized by early onset fibrocystic hepatorenal changes. Previous reports have documented pronounced phenotypic variability even among siblings in terms of patient survival. The underlying causes for this clinical variability are incompletely understood. Methods: We present the longitudinal clinical courses of 35 sibling pairs included in the ARPKD registry study ARegPKD, encompassing data on primary manifestation, prenatal and perinatal findings, genetic testing, and family history, including kidney function, liver involvement, and radiological findings. Results: We identified 70 siblings from 35 families with a median age of 0.7 (interquartile range 0.1–6.0) years at initial diagnosis and a median follow-up time of 3.5 (0.2–6.2) years. Data on PKHD1 variants were available for 37 patients from 21 families. There were 8 patients from 7 families who required kidney replacement therapy (KRT) during follow-up. For 44 patients from 26 families, antihypertensive therapy was documented. Furthermore, 37 patients from 24 families had signs of portal hypertension with 9 patients from 6 families having substantial hepatic complications. Interestingly, pronounced variability in the clinical course of functional kidney disease was documented in only 3 sibling pairs. In 17 of 20 families of our cohort of neonatal survivors, siblings had only minor differences of kidney function at a comparable age. Conclusion: In patients surviving the neonatal period, our longitudinal follow-up of 70 ARPKD siblings from 35 families revealed comparable clinical courses of kidney and liver diseases in most families. The data suggest a strong impact of the underlying genotype.

OBJECTIVES Entire mitral valve reconstruction with an extracellular matrix tube graft is a potential candidate to overcome the current limitations of mechanical and bioprosthetic valves. However, clinical data have raised concern with respect to patch failure. The aim of our study was to evaluate the impact of extracellular matrix mitral tube graft implantation on mitral annular and subvalvular regional dynamics in pigs. METHODS A modified tube graft design made of 2-ply extracellular matrix was used (CorMatrix®; Cardiovascular Inc., Alpharetta, GA, USA). The reconstructions were performed in an acute 80-kg porcine model (N = 8), where each pig acted as its own control. Haemodynamics were assessed with Mikro-Tip pressure catheters and mitral annular and subvalvular geometry and dynamics with sonomicrometry. RESULTS Catheter-based peak left atrial pressure and pressure difference across the mitral and aortic valves in the reconstructions were comparable to the values seen in the native mitral valves. Also comparable were maximum mitral annular area (755 ± 100 mm2), maximum septal-lateral distance (29.7 ± 1.7 mm), maximum commissure–commissure distance (35.0 ± 3.4 mm), end-systolic annular height-to-commissural width ratio (10.2 ± 1.0%) and end-diastolic interpapillary muscle distance (27.7 ± 3.3 mm). Systolic expansion of the mitral annulus was, however, observed after reconstruction. CONCLUSIONS The reconstructed mitral valves were fully functional without regurgitation, obstruction or stenosis. The reconstructed mitral annular and subvalvular geometry and subvalvular dynamics were found in the same range to those in the native mitral valve. A regional annular ballooning effect occurred that might predispose to patch failure. However, the greatest risk was found at the papillary muscle attachments.

Objective: After a first episode of lower extremity deep venous thrombosis, post-thrombotic syndrome (PTS) develops in 20% to 50% of patients despite adequate anticoagulation. Symptoms of PTS can vary from leg swelling to venous ulceration with disabling venous claudication. It significantly affects the patient's quality of life and has considerable socioeconomic consequences. This review gives an update on diagnosis and current treatment strategies in patients with PTS due to chronic venous obstruction, in particular regarding the role of endovenous procedures.Methods: This review article is based on a selective literature search in PubMed and the Cochrane Library. The terms "postthrombotic syndrome," "post-thrombotic syndrome," "chronic venous obstruction," "venous outflow obstruction," and "venous stent" were used as keywords. Selected publications addressed the diagnosis of and therapy for PTS. Acute deep venous thrombosis, thrombolysis, case reports, complications as a result of caval vein filters, animal experiments, PTS of the upper extremity, and PTS in children were excluded.Results: In addition to conservative treatment of PTS, the following invasive procedures are also available: open surgical reconstructions, hybrid procedures, and endovenous recanalization of the occluded iliocaval venous tract with stent angioplasty. Since introduction of dedicated venous stents in 2012, technical success, patency rates, and improvement in quality of life have been at least as good as results of open surgical reconstruction if not better.Conclusions: First-line treatment should be conservative therapy. In case of therapy-resistant PTS with poor quality of life, the possibility of an invasive treatment should be evaluated. All invasive procedures are recommended with low levels of evidence. Therefore, deciding on an invasive treatment and type of procedure should be made individually. Because PTS is rarely a threat to life or limb, a minimally invasive treatment is preferred. Therefore, endovenous recanalization appears to be appropriate as the therapy of choice. In patients with involvement of the femoral confluence, endophlebectomy of the common femoral vein in addition to venous recanalization is inevitable to ensure an adequate inflow into the recanalized venous tract. It also secures a sufficient drainage of blood from the peripheral venous system. Because this hybrid procedure is burdened with a significantly higher risk of complications, strict criteria must be fulfilled to legitimize the indication for this procedure. For the best possible results to be achieved, the following perioperative and postoperative management must be considered: therapeutic anticoagulation, early mobilization, compression therapy, and systematic follow-up with duplex ultrasound.

Objective After a first episode of lower extremity deep venous thrombosis, post-thrombotic syndrome (PTS) develops in 20% to 50% of patients despite adequate anticoagulation. Symptoms of PTS can vary from leg swelling to venous ulceration with disabling venous claudication. It significantly affects the patient's quality of life and has considerable socioeconomic consequences. This review gives an update on diagnosis and current treatment strategies in patients with PTS due to chronic venous obstruction, in particular regarding the role of endovenous procedures. Methods This review article is based on a selective literature search in PubMed and the Cochrane Library. The terms “postthrombotic syndrome,” “post-thrombotic syndrome,” “chronic venous obstruction,” “venous outflow obstruction,” and “venous stent” were used as keywords. Selected publications addressed the diagnosis of and therapy for PTS. Acute deep venous thrombosis, thrombolysis, case reports, complications as a result of caval vein filters, animal experiments, PTS of the upper extremity, and PTS in children were excluded. Results In addition to conservative treatment of PTS, the following invasive procedures are also available: open surgical reconstructions, hybrid procedures, and endovenous recanalization of the occluded iliocaval venous tract with stent angioplasty. Since introduction of dedicated venous stents in 2012, technical success, patency rates, and improvement in quality of life have been at least as good as results of open surgical reconstruction if not better. Conclusions First-line treatment should be conservative therapy. In case of therapy-resistant PTS with poor quality of life, the possibility of an invasive treatment should be evaluated. All invasive procedures are recommended with low levels of evidence. Therefore, deciding on an invasive treatment and type of procedure should be made individually. Because PTS is rarely a threat to life or limb, a minimally invasive treatment is preferred. Therefore, endovenous recanalization appears to be appropriate as the therapy of choice. In patients with involvement of the femoral confluence, endophlebectomy of the common femoral vein in addition to venous recanalization is inevitable to ensure an adequate inflow into the recanalized venous tract. It also secures a sufficient drainage of blood from the peripheral venous system. Because this hybrid procedure is burdened with a significantly higher risk of complications, strict criteria must be fulfilled to legitimize the indication for this procedure. For the best possible results to be achieved, the following perioperative and postoperative management must be considered: therapeutic anticoagulation, early mobilization, compression therapy, and systematic follow-up with duplex ultrasound.

Purpose: To assess the efficacy and safety of a glaucoma procedure to control intraocular pressure (IOP) using the adjustable eyeWatch glaucoma drainage device compared with Ahmed glaucoma valve (AGV) in refractory glaucoma. Patients and Methods: Monocentric, retrospective, comparative clinical trial. Patients suffering from refractory glaucoma after failed surgeries and requiring a further glaucoma procedure including an aqueous shunt were enrolled in this study. The first group AGV included patients with an AGV. The second group eW-B included patients receiving an eyeWatch used in connection with a Baerveldt glaucoma implant. The primary outcome was the success rate, defined as an IOP 20% from baseline, and IOP >= 5 mm Hg. Secondary outcomes were mean IOP, number of antiglaucoma medications, visual acuity, number and type of complications. Results: Twenty-one patients were included. The mean follow-up time was 13.2 +/- 3.4 months. Mean IOP decreased from 24.8 +/- 9.0 mm Hg before surgery to 13.8 +/- 3.6 mm Hg at 12 months for group AGV, and 27.3 +/- 7.0 to 12.8 +/- 2.4 mm Hg for group eW-B, respectively (P

Background: There is considerable controversy about whether condylar fractures should be treated open or closed. Even when there is a clear indication for open treatment, the appropriate approach opens another debate.Purpose: To provide a clear overview on the complications of extraoral approaches to condylar fractures.Methods: Systematic literature search of all indexed years on PubMed, Medline, and Embase. For detailed analysis, we selected 70 studies.Results: Of the 2783 patients who were studied with respect to facial nerve function, 338 (12%) experienced some form of weakness. Most (95%) achieved full recovery. In 17 of the 338 patients (5%), these paralyses were permanent. A hematoma occurred in 1.7%, an infection of the wound in >= 2.9%, Frey syndrome in 1.1%, a sialocele in 2.6%, a salivary fistula in >= 4.8%, sensory disturbance of the great auricular nerve in 7.9%, and an unsatisfactory scar in >= 1.6% of the patients.Conclusion: Due to the great diversity in fractures, approaches, and surgical techniques, it is difficult to objectively compare surgical techniques for condylar fractures and their complications. Based on the literature studied in this review, we propose a treatment protocol with respect to open treatment approaches. (C) 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Background: There is considerable controversy about whether condylar fractures should be treated open or closed. Even when there is a clear indication for open treatment, the appropriate approach opens another debate. Purpose: To provide a clear overview on the complications of extraoral approaches to condylar fractures. Methods: Systematic literature search of all indexed years on PubMed, Medline, and Embase. For detailed analysis, we selected 70 studies. Results: Of the 2783 patients who were studied with respect to facial nerve function, 338 (12%) experienced some form of weakness. Most (95%) achieved full recovery. In 17 of the 338 patients (5%), these paralyses were permanent. A hematoma occurred in 1.7%, an infection of the wound in >= 2.9%, Frey syndrome in 1.1%, a sialocele in 2.6%, a salivary fistula in >= 4.8%, sensory disturbance of the great auricular nerve in 7.9%, and an unsatisfactory scar in >= 1.6% of the patients. Conclusion: Due to the great diversity in fractures, approaches, and surgical techniques, it is difficult to objectively compare surgical techniques for condylar fractures and their complications. Based on the literature studied in this review, we propose a treatment protocol with respect to open treatment approaches. (C) 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Since the introduction of rigid internal fixation devices, more and more surgeons favour an open approach to treating condylar fractures of the mandible in adult patients. Different indications for open treatment have been published. Open treatment is associated with surgical complications because of the technique employed. The aim of this systematic review was to provide an overview of the studies published exclusively on open treatment, and to summarize the existing open treatment modalities and their clinical outcomes. A total of seventy studies were selected for detailed analysis. Most studies reported good results with regard to the outcome measures of open treatment. Surgical complications including hematoma, wound infection, weakness of the facial nerve, sialocele, salivary fistula, sensory disturbance of the great auricular nerve, unsatisfactory scarring, and fixation failure were reported in the studies. This review suggests that because of the high level of methodological variance in the relevant studies published to date, among other factors, there are currently no evidence-based conclusions or guidelines that can be formulated with regard to the most appropriate open treatment. Establishment of such standards could potentially improve treatment outcomes.

Since the introduction of rigid internal fixation devices, more and more surgeons favour an open approach to treating condylar fractures of the mandible in adult patients. Different indications for open treatment have been published. Open treatment is associated with surgical complications because of the technique employed. The aim of this systematic review was to provide an overview of the studies published exclusively on open treatment, and to summarize the existing open treatment modalities and their clinical outcomes. A total of seventy studies were selected for detailed analysis. Most studies reported good results with regard to the outcome measures of open treatment. Surgical complications including hematoma, wound infection, weakness of the facial nerve, sialocele, salivary fistula, sensory disturbance of the great auricular nerve, unsatisfactory scarring, and fixation failure were reported in the studies. This review suggests that because of the high level of methodological variance in the relevant studies published to date, among other factors, there are currently no evidence-based conclusions or guidelines that can be formulated with regard to the most appropriate open treatment. Establishment of such standards could potentially improve treatment outcomes.

Introduction: Vagus Nerve Stimulation (VNS) can be an efficacious add-on treatment in patients with drug-resistant epilepsy, who are not eligible for surgery. Evidence of VNS efficacy in children with intellectual disability (ID) is scarce.Objectives: The purpose of this study was to review all available VNS data in the pediatric population (Methods: Cochrane, EMBASE, PubMed and MEDLINE were used to collect all research associated to VNS and ID (or synonyms) leading to a total of 37 studies. Seven studies showed the results of patients with ID and those without separately; thereby only these studies were included in the VNS meta-analysis.Results: Our meta-analysis showedthat VNS was less effective in pediatric epilepsy patients with ID compared to those withoutID (Mantel-Haenszel meta- analysis; p = 0.028, OR 0.18 (CI 95% 0.039 0.84)). However, there were no prospective controlled studies. Numerous studies reported quality of life (QoL) improvements in this subpopulation. The most common adverse events were transient and well tolerated. Side effects on cognition and behavior were not reported.Discussion: These results might be a reason to consider VNS early on in the treatment of this subgroup. The significantly greater amount of retrospective studies, differences in followup (FU), lack of control data, heterogeneous series and limited number of patients could have biased the outcome measurements. Hence, current data do not exclude VNS for children with drug-resistant epilepsy and ID but should be interpreted with caution. (C) 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

ZusammenfassungHintergrund: Nach einer femoro-iliakalen und kavalen tiefen Venenthrombose (TVT) entwickeln bis zu 50 % der Patienten ein postthrombotisches Syndrom (PTS), was zu einer erheblichen Einschränkung der Lebensqualität führen kann.Ergebnisse: Die Behandlungsmöglichkeiten bei einem PTS sind die konservative Therapie, klassische offene Bypass-Operationen sowie die endovaskulären Verfahren.Zu den offenen Operationsverfahren liegen lediglich Studien mit geringer Fallzahl vor, deren Offenheitsrate 58–100 % beträgt. Die Komplikationen dieser invasiven Methode sind thrombotische Bypassverschlüsse, Hämatome und Wundheilungsstörungen.Mit dem endovaskulären Verfahren können Offenheitsraten von 73–100 % erreicht werden. Zu diesem Verfahren liegen Studien mit größeren Fallzahlen vor. Als Komplikationen kann man einen thrombotischen Verschluss und Hämatome nennen.Im Gegensatz zur konservativen Therapie gibt es zu den operativen Behandlungsmethoden keine randomisierten Studien. Aus diesem Grund kann für die operativen Verfahren nur eine schwache Empfehlung ausgespro-chen werden (Evidenzlevel II b).Schlussfolgerung: Bei Vorliegen eines post-thrombotischen Syndroms mit chronisch venöser Obstruktion sollten zunächst alle konservativen Behandlungsmöglichkeiten ausgeschöpft werden. Bei weiterhin bestehenden Beschwerden mit deutlicher Einschränkung der Lebensqualität sollte ein invasives Therapieverfahren erwogen werden. In diesem Fall stellt die endovaskuläre Rekanalisation die Therapiemethode der ersten Wahl dar. Randomisierte Studien sind notwendig, um einen aussagekräftigeren Empfehlungsgrad zu erreichen.

Attenuation correction is an essential component of the long chain of data correction techniques required to achieve the full potential of quantitative positron emission tomography (PET) imaging. The development of combined PET/magnetic resonance imaging (MRI) systems mandated the widespread interest in developing novel strategies for deriving accurate attenuation maps with the aim to improve the quantitative accuracy of these emerging hybrid imaging systems. The attenuation map in PET/MRI should ideally be derived from anatomical MR images; however, MRI intensities reflect proton density and relaxation time properties of biological tissues rather than their electron density and photon attenuation properties. Therefore, in contrast to PET/computed tomography, there is a lack of standardized global mapping between the intensities of MRI signal and linear attenuation coefficients at 511 keV. Moreover, in standard MRI sequences, bones and lung tissues do not produce measurable signals owing to their low proton density and short transverse relaxation times. MR images are also inevitably subject to artifacts that degrade their quality, thus compromising their applicability for the task of attenuation correction in PET/MRI. MRI-guided attenuation correction strategies can be classified in three broad categories: (i) segmentation-based approaches, (ii) atlas-registration and machine learning methods, and (iii) emission/transmission-based approaches. This paper summarizes past and current state-of-the-art developments and latest advances in PET/MRI attenuation correction. The advantages and drawbacks of each approach for addressing the challenges of MR-based attenuation correction are comprehensively described. The opportunities brought by both MRI and PET imaging modalities for deriving accurate attenuation maps and improving PET quantification will be elaborated. Future prospects and potential clinical applications of these techniques and their integration in commercial systems will also be discussed. (C) 2016 American Association of Physicists in Medicine.

ObjectivesTo analyse which mammographic and tumour characteristics led to concordant versus discordant recalls at blinded double reading to further optimise our breast cancer screening programme.MethodsWe included a consecutive series of 99,013 screening mammograms obtained between July 2013 and January 2015. All mammograms were double read in a blinded fashion. Discordant readings were routinely recalled without consensus or arbitration. During the 2-year follow-up, relevant data of the recalled women were collected. We compared mammographic characteristics, screening outcome and tumour characteristics between concordant and discordant recalls.ResultsThere were 2,543 concordant recalls (71.4%) and 997 discordant recalls (28.0%). The positive predictive value of a concordant recall was significantly higher (23.5% vs. 10.0%, p

OBJECTIVE: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. STUDY DESIGN: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. RESULTS: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. CONCLUSIONS: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD. ispartof: JOURNAL OF PEDIATRICS vol:199 pages:22-+ ispartof: location:United States status: published

Elbow dysplasia is an important cause of forelimb lameness in large breed dogs. The aim of this study was to report on the arthroscopic findings associated with medial compartment erosion (MCE) of the canine elbow joint. Retrospectively, records of 84 elbow joints from 66 dogs diagnosed arthroscopically with MCE were retrieved from a medical records database (2008 - 2012). The radiographic degree of osteoarthritis was determined. Arthroscopic images and videos were evaluated in detail. In nine joints (10.7%), MCE was the only pathological finding (= group 1). Group 2 (n = 50, 59.5%) consisted of elbows with MCE concomitant with medial coronoid process (MCP) pathology. In group 3 (n = 25 joints, 29.8%), MCE was diagnosed during a second- look arthroscopy in dogs presented with lameness after arthroscopic treatment for medial coronoid disease. There was a significant age difference (p < 0.001) between the groups, with dogs in group 1 being the oldest. Complete erosion of the medial compartment was most commonly found in group 1, whereas focal cartilage erosion was mostly identified in group 2. Overall, additional cartilage pathology of the lateral part of the humeral condyle and/or the radial head was recognized in 58.3% of the joints (49/84).

BACKGROUND:There is an increasing tendency to treat spinal dural arteriovenous fistulas (SDAVFs) endovascularly despite the lack of clear evidence favoring embolization over surgery.OBJECTIVE:To compare the initial failure and recurrence rates of primary treatment of SDAVFs by surgery and endovascular techniques.METHODS:A meta-analysis using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) standard was performed. All the English literature from 2004 onward was evaluated. From each article that compared the 2 treatment modalities, the odds ratio (OR) was calculated. Combined ORs were calculated with Review Manager 5.3 of The Cochrane Collaboration.RESULTS:A total of 35 studies harboring 1112 patients were assessed. Initial definitive fistula occlusion was observed in 588 of 609 surgical patients (96.6%; 95% confidence interval [CI], 94.8-97.8) vs 363 of 503 endovascularly treated patients (72.2%; 95% CI, 68.1-75.9; P

BACKGROUND:There is an increasing tendency to treat spinal dural arteriovenous fistulas (SDAVFs) endovascularly despite the lack of clear evidence favoring embolization over surgery.OBJECTIVE:To compare the initial failure and recurrence rates of primary treatment of SDAVFs by surgery and endovascular techniques.METHODS:A meta-analysis using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) standard was performed. All the English literature from 2004 onward was evaluated. From each article that compared the 2 treatment modalities, the odds ratio (OR) was calculated. Combined ORs were calculated with Review Manager 5.3 of The Cochrane Collaboration.RESULTS:A total of 35 studies harboring 1112 patients were assessed. Initial definitive fistula occlusion was observed in 588 of 609 surgical patients (96.6%; 95% confidence interval [CI], 94.8-97.8) vs 363 of 503 endovascularly treated patients (72.2%; 95% CI, 68.1-75.9; P

Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disease with a severe phenotype often presenting prenatally or in early childhood. With its obligate renal and hepatic involvement, ARPKD is one of the most important indications for liver and/or kidney transplantation in childhood. Marked phenotypic variability is observed, the genetic basis of which is largely unknown. Treatment is symptomatic and largely empiric as evidence-based guidelines are lacking. Therapeutic initiatives for ARPKD face the problem of highly variable cohorts and lack of clinical or biochemical risk markers without clear-cut clinical end points. ARegPKD is an international, multicenter, retro- and prospective, observational study to deeply phenotype patients with the clinical diagnosis of ARPKD. Initiated in 2013 as a web-based registry (www.aregpkd.org), ARegPKD enrolls patients across large parts of Europe and neighboring countries. By January 2017, more than 400 patients from 17 mostly European countries have been registered in the ARPKD registry study with significant follow-up data. Due to comprehensive retro- and prospective data collection and associated biobanking, ARegPKD will generate a unique ARPKD cohort with detailed longitudinal clinical characterization providing a basis for future clinical trials as well as translational research. Hence, ARegPKD is hoped to contribute to the pathophysiological understanding of the disease and to the improvement of clinical management. ispartof: Frontiers in Pediatrics vol:5 pages:18- ispartof: location:Switzerland status: published

Background— Pulmonary insufficiency is the nexus of late morbidity and mortality after transannular patch repair of tetralogy of Fallot. This study aimed to establish the feasibility of implantation of the novel Medtronic Harmony transcatheter pulmonary valve (hTPV) and to assess its effect on pulmonary insufficiency and ventricular function in an ovine model of chronic postoperative pulmonary insufficiency. Methods and Results— Thirteen sheep underwent baseline cardiac magnetic resonance imaging, surgical pulmonary valvectomy, and transannular patch repair. One month after transannular patch repair, the hTPV was implanted, followed by serial magnetic resonance imaging and computed tomography imaging at 1, 5, and 8 month(s). hTPV implantation was successful in 11 animals (85%). There were 2 procedural deaths related to ventricular fibrillation. Seven animals survived the entire follow-up protocol, 5 with functioning hTPV devices. Two animals had occlusion of hTPV with aneurysm of main pulmonary artery. A strong decline in pulmonary regurgitant fraction was observed after hTPV implantation (40.5% versus 8.3%; P =0.011). Right ventricular end diastolic volume increased by 49.4% after transannular patch repair (62.3–93.1 mL/m 2 ; P =0.028) but was reversed to baseline values after hTPV implantation (to 65.1 mL/m 2 at 8 months, P =0.045). Both right ventricular ejection fraction and left ventricular ejection fraction were preserved after hTPV implantation. Conclusions— hTPV implantation is feasible, significantly reduces pulmonary regurgitant fraction, facilitates right ventricular volume improvements, and preserves biventricular function in an ovine model of chronic pulmonary insufficiency. This percutaneous strategy could potentially offer an alternative for standard surgical pulmonary valve replacement in dilated right ventricular outflow tracts, permitting lower risk, nonsurgical pulmonary valve replacement in previously prohibitive anatomies.

International audience; Background: Post-thrombotic syndrome (PTS) is a frequent complication of deep vein thrombosis (DVT) despite adequate treatment. Venous angioplasty and stent placement has been progressively used to restore and maintain venous patency in PTS patients. This study reports our single-center experience with the use of endovascular treatment for chronic post-thrombotic symptomatic ilio-femoral venous obstructive lesions. Methods: A prospective mono-centric observational cohort study of PTS patients with chronic symptomatic ilio-femoral venous obstructive lesions referred for endovascular treatment was conducted from March 2012 to April 2016. Procedure consisted in recanalization, pre-dilation and self-expandable stenting of stenotic or occluded iliac and/or femoral veins. Severity of PTS, quality-of-life and treatment outcomes were assessed using Villalta scale and Chronic Venous Insufficiency Questionnaire (CIVIQ-20) at baseline and 3 months after the procedure. Imaging follow-up was based on duplex ultrasound (US) and computed tomography (CT). Results: Twenty-one patients (11 females, 10 males; median age, 41 years; range, 32-60) were included. Recanalization and stenting was successfully accomplished in all prime procedures, 4 patients benefitted from an additional procedure. Immediate technical success rate was 96% considering 25 procedures, performed without any complications. Median follow-up was 18 months (range, 6-30 months) with a 90.5% stent patency rate. Villalta score significantly decreased from baseline compared with 3 months after the procedure [14 (range, 11-22) and 5 (range, 1-10), respectively, P

Introduction Vagus Nerve Stimulation (VNS) can be an efficacious add-on treatment in patients with drug-resistant epilepsy, who are not eligible for surgery. Evidence of VNS efficacy in children with intellectual disability (ID) is scarce. Objectives The purpose of this study was to review all available VNS data in the pediatric population (≤18 years old) and focus on the subpopulation with ID since appropriate treatment of these children is often challenging and complex. Methods Cochrane, EMBASE, PubMed and MEDLINE were used to collect all research associated to VNS and ID (or synonyms) leading to a total of 37 studies. Seven studies showed the results of patients with ID and those without separately; thereby only these studies were included in the VNS meta-analysis. Results Our meta-analysis showed that VNS was less effective in pediatric epilepsy patients with ID compared to those without ID (Mantel-Haenszel meta-analysis; p = 0.028, OR 0.18 (CI 95% 0.039–0.84)). However, there were no prospective controlled studies. Numerous studies reported quality of life (QoL) improvements in this subpopulation. The most common adverse events were transient and well tolerated. Side effects on cognition and behavior were not reported. Discussion These results might be a reason to consider VNS early on in the treatment of this subgroup. The significantly greater amount of retrospective studies, differences in follow-up (FU), lack of control data, heterogeneous series and limited number of patients could have biased the outcome measurements. Hence, current data do not exclude VNS for children with drug-resistant epilepsy and ID but should be interpreted with caution.

Materials 9(4), 219 (2016). doi:10.3390/ma9040219, Published by MDPI, Basel

Aim. Bare metal stents have improved results of endovascular treatment of aortoiliac occlusive disease. Polytetrafluoroethylene covered stents may further improve patency rates by preventing tissue ingrowth, and might reduce complications. This study was conducted to assess possible assets or liabilities of covered stents used for aortoiliac occlusive disease and to conduct a comparison with bare metal stents.Methods. A review was performed of literature published until March 2012 for infrarenal aortic lesions, iliac lesions and complex aortoiliac lesions. Outcomes were technical success, patency rates, clinical success and complication rates.Results were addressed to the three anatomic regions: the infrarenal aorta, the aortoiliac bifurcation and iliac arteries. Results. A total of 51 articles were included in the study. Overall technical success varied between 73% and 100%. Randomized data have proven the superiority of covered stents in extensive iliac occlusive lesions. Case series of patients with iliac occlusive disease demonstrated a 1-year primary patency of bare metal stents between 76% and 100% with a 5-year primary patency rate of 63%-83%. One-year primary patency of covered stents varied between 70% and 100%, while no long term patency rates with covered stents have been reported so far. Reliable comparisons between groups cannot be made due to variances in patient and lesion characteristics. Covered stents seem to improve results of kissing stents and are related to excellent results in isolated aortic lesions. No difference in complication rate between bare metal and covered stents have been described, to date.Conclusion. Covered stents improve results of endovascular treatment of extensive iliac occlusive lesions and are related to excellent results in isolated aortic lesions. They may provide a valid alternative for surgery in patients with extensive aortoiliac disease.

Unilateral leg swelling is most often caused by deep vein thrombosis but other conditions may mimic this disorder. We describe the case of a patient with symptoms of unilateral lower extremity swelling caused by external compression of the iliac vein by a mass originating from the iliopsoas muscle. Initially this mass was diagnosed as an iliopsoas hematoma in a patient using anticoagulants. However, it proved to be B-cell non-Hodgkin lymphoma. Compression was relieved by placement of an endovenous stent in the left common iliac vein., Endovenous stenting is a relatively new treatment modality that is used to treat post-thrombotic venous occlusions and chronic venous insufficiency. Only a few, case series have been described of stenting of compressed pelvic veins by adjacent structures such as gynecological malignancies. Although stent patency lasted only four weeks in this patient, venous stent placement quickly reliefs symptoms and should therefore be considered as an option to bridge time to allow development of sufficient venous collaterals.

Purpose: To study the efficacy and tolerability of add-on levetiracetam in children and adolescents with refractory epilepsy.Methods: In this prospective multi-centre, open-label, add-on study, 33 children aged 4-16 years (median 8.5 years) with epilepsy refractory to at least two antiepileptic drugs were treated with levetiracetam in addition to their present treatment regimen with a follow-up of 26 weeks. The starting dose of 10 mg/kg/day was increased with 2-week steps of 10 mg/kg/day, if necessary, up to a maximum dose of 60 mg/kg/day.Results: Retention rate was 69.7% after 26 weeks on a median levetiracetam dosage of 22 mg/kg/day. Four children dropped-out because levetiracetam was ineffective, four because seizure frequency increased and/or seizures became more severe, and two because they developed aggressive behaviour. Compared to their baseline seizure frequency, 13 children (39.4%) had a > 50% seizure reduction 12 weeks after initiation of levetiracetam, and 17 children (51.5%) at 26 weeks. At 26 weeks, nine children (27.3%) had been seizure-free for at least the last 4 weeks, terminal remission ranged from 0 to 187 days (mean 46 days). Levetiracetam was effective in both partial and primary generalized seizures, but had most effect in partial seizures.Most reported side effects were hyperactivity (48.5%), somnolence (36.4%), irritability (33.3%) and aggressive behaviour (27.3%). Severity of most side effects was mild. Five children had a serious adverse event, which all concerned hospital admissions that were not related to levetiracetam use.Conclusion: Levetiracetam proved to be an effective and well-tolerated add-on treatment in this group of children with refractory epilepsy. (C) 2007 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

BACKGROUND: Recommendations on the use of I-123 metaiodobenzylguanidine (MIBG) scintigraphy in localising phaeochromocytomas vary. The accuracy of I-123 MIBG scintigraphy was determined by evaluating our own I-123 MIBG scans and performing a meta-analysis.MATERIALS AND METHODS: Between January 1992 and May 2002, the I-123 MIBG scans of consecutive patients suspected of a phaeochromocytoma were re-evaluated. For the meta-analysis, studies with more than 5 I-123 MIBG scans were selected.RESULTS: Thirty patients were evaluated. The sensitivity in our own population was 92% and the specificity was 100%. Twelve articles were selected for our meta-analysis. The overall sensitivity and specificity were 96% and 100%, respectively. The sensitivity and specificity for tumours in the adrenal gland was 98% for both. For tumours located outside the adrenal gland, the sensitivity was 98% and the sensitivity for malignancies was 79%.CONCLUSION: 1-123 MIBG scintigraphy has an excellent sensitivity and specificity in localising phaeochromocytomas, except for malignant tumours. 1-123 MIBG scintigraphy is superior in localising tumours outside the adrenal gland.

Background: The main aim of performing a vascular anastomosis is to achieve maximal patency rates. An important factor to achieve that goal is to minimize damage to the vessel walls. Sutures inevitably induce vascular wall damage, which influences the healing of the anastomosis. Over time, several alternatives to sutures have become available.Methods: A Medline literature search was performed to locate English, German and French language articles pertinent to non-suture methods of vascular anastomosis. Manual cross-referencing was also performed and many historical articles were included.Results and conclusion: The non-suture techniques can be categorized into five groups based on the materials used: rings, clips, adhesives, stents and laser welding. With all these techniques a faster and less traumatic anastomosis can be made compared with sutures. However, each device is associated with technique-related complications. As a consequence, suturing continues to be the standard approach. The disadvantages of the non-suture techniques include: rigidity and a non-compliant anastomosis with rings; toxicity, leakage and aneurysm formation with adhesives; early occlusion with stents; cost, reduced strength in larger-sized vessels and demand for surgical skills with laser welding. Further refinement is needed before widespread adoption of these techniques can occur. Clips, however, may be particularly promising but long-term evaluation is required.

Background: Infusion of anti-tumour necrosis factor-alpha appears to be highly effective in patients with Crohn's disease. Aim: To assess the effect of infliximab on the quality of life in patients with active or fistulizing disease, as measured by the inflammatory bowel disease questionnaire, and to examine the impact on its four dimensions. Methods: An observational study was conducted in 65 patients. An infusion of 5 mg/kg infliximab was given at week 0 in patients with active disease and at week 0, 2 and 6 in fistulizing disease. Changes from baseline in the total and dimensional inflammatory bowel disease questionnaire scores were calculated and compared between the patient groups. Potential predictors of change in the quality of life were identified. Results: In the active disease group, at week 4, the mean total and dimensional inflammatory bowel disease questionnaire scores improved compared to baseline (P

Chronic venous insufficiency (CVI) as an advanced stage of chronic venous disease is a common problem that occurs in approximately 1-5 % of the adult population. CVI has either a nonthrombotic (primary) or postthrombotic (secondary) cause involving reflux, obstruction, or a combination of both. The role of venous obstruction is increasingly recognized as a major cause of CVI, with obstructive lesions in the iliocaval segment being markedly more relevant than lesions at the levels of the crural and femoral veins. Approximately 70-80 % of iliac veins develop a variable degree of obstruction following an episode of acute deep venous thrombosis. Nonthrombotic iliac vein obstruction also known as May-Thurner or Cockett's syndrome is the most common cause of nonthrombotic iliac vein occlusion. While compression therapy is the basis of therapy in CVI, in many cases, venous recanalization or correction of obstructive iliac vein lesions may result in resolution of symptoms. This document reviews the current evidence on iliocaval vein recanalization and provides standards of practice for iliocaval stenting in primary and secondary causes of chronic venous disease.

This study examined physicians’ motivation to engage in work-related learning and its contribution to expertise development beyond work experience. Based on deliberate practice theory, semi-structured interviews were conducted with 17 residents and 28 experienced physicians in internal medicine, focusing on the activities they engaged in during work that might contribute to professional development and the goals underlying this behavior. Learning motivation was also measured using a goal orientation questionnaire. Expertise was measured by a case test derived from the Medical Knowledge Self Assessment Program (MKSAP). The interviews showed that participants’ learning was largely embedded in everyday work; most of their learning activities were inherent to the job rather than motivated by competence improvement goals. The problems encountered in patient care played a key role in prompting learning. Role, work experience and work situation affected the type of activities engaged in, as well as the intensity of practice. Deliberate engagement in work-related learning activities was related neither to goal orientations nor to case test performance, except activities by the experienced physicians to keep up-to-date. Work experience, in contrast, showed a clear positive relationship with the performance of residents. Two main conclusions can be drawn from this study: (1) patient care induced relevant learning activities aimed at performance improvement for the patient’s sake; (2) deliberate investments in learning can be enhanced to promote expertise development. Self-regulated learning by physicians needs to be strengthened and the organization for which they work should facilitate and encourage learning in daily practice.

Made available in DSpace on 2019-09-12T16:53:31Z (GMT). No. of bitstreams: 0 Previous issue date: 2016 Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) BACKGROUND: A recent randomized clinical trial named Management of Myelomeningocele Study (MOMS trial) showed that prenatal correction of open spina bifida (OSB) via open fetal surgery was associated with improved infant neurological outcomes relative to postnatal repair, but at the expense of increased maternal morbidity. OBJECTIVE: We sought to report the final results of our phase I trial (Cirurgia Endoscopica para Correcao Antenatalda Meningomielocele [CECAM]) on the feasibility, safety, potential benefits, and side effects of the fetoscopic treatment of OSB using our unique surgical technique. STUDY DESIGN: Ten consecutive pregnancies with lumbosacral OSB were enrolled in the study. Surgeries were performed percutaneously under general anesthesia with 3 ports and partial carbon dioxide insufflation. After appropriate surgical positioning of the fetus, the neuro-placode was released with scissors and the skin was undermined to place a biocellulose patch over the lesion. The skin was closed over the patch using a single running stitch. Preoperative, postoperative, and postnatal magnetic resonance imaging were performed to assess hindbrain herniation. Neurodevelopmental evaluation was performed before discharge and at 3, 6, and 12 months. All cases were delivered by cesarean delivery, at which time the uterus was assessed for evidence of thinning or dehiscence. RESULTS: The median gestational age at the time of surgery was 27 weeks (range 25-28 weeks). Endoscopic repair was completed in 8 of 10 fetuses. Two cases were unsuccessful due to loss of uterine access. The mean gestational age at birth was 32.4 weeks with a mean latency of 5.6 weeks between surgery and delivery (range 2-8 weeks). There was 1 fetal and 1 neonatal demise, and 1 unsuccessful case underwent postnatal repair. Of the 7 infants available for analysis, complete reversal of hindbrain herniation occurred in 6 of 7 babies. Three babies required ventriculoperitoneal shunting or third ventriculostomy. Functional motor level was the same or better than the anatomical level in 6 of 7 cases. There was no significant maternal morbidity and no evidence of myometrial thinning or dehiscence. However, surgeries were complicated by premature rupture of membrane and prematurity. CONCLUSION: Our study suggests that the antenatal treatment of OSB using a fetoscopic approach and our unique surgical technique can result in a watertight seal, reversal of the hindbrain herniation, and better than expected motor function. Our technique differs substantially from the classic repair of OSB used in prior open fetal surgery and fetoscopic studies, in which the dura mater is dissected and the defect is closed in multiple layers. Instead, we use a biocellulose patch placed over the lesion and simple closure of the skin. As such, our technique is an alternative to the current paradigms in the antenatal treatment of OSB. Our clinical outcomes are in line with the results of our extensive prior animal work. Maternal benefits of our approach and technique include minimal morbidity and no myometrial legacy. Current limitations of the approach include potential loss of access, premature rupture of membranes, and attendant prematurity. Phase II trials are needed to prevent these complications and to further assess the risks and benefits of our distinct surgical approach and technique. [Pedreira, Denise A. L.] Albert Einstein Hosp, Fetal Therapy Program, Sao Paulo, Brazil [Pedreira, Denise A. L.] Inst Assistencia Ao Serv Publ Estado Sao Paulo, Dept Obstet, Sao Paulo, Brazil [Nishikuni, Koshiro] Inst Assistencia Ao Serv Publ Estado Sao Paulo, Dept Neurosurg, Sao Paulo, Brazil [Zanon, Nelci] Univ Fed Sao Paulo, Dept Neurosurg, Sao Paulo, Brazil [Moreira de Sa, Renato A.] Univ Fed Fluminense, Dept Obstet, Niteroi, RJ, Brazil [Acacio, Gregerio L.] Universidade de Taubaté (Unitau), Dept Obstet [Chmait, Ramen H.] Univ So Calif, Keck Sch Med, Dept Obstet & Gynecol, Los Angeles, CA 90033 USA [Kontopoulos, Eftichia V.; Quintero, Ruben A.] Jackson Fetal Therapy Inst, Div Maternal Fetal Med & Fetal Therapy, Miami, FL USA

Unilateral leg swelling is most often caused by deep vein thrombosis but other conditions may mimic this disorder. We describe the case of a patient with symptoms of unilateral lower extremity swelling caused by external compression of the iliac vein by a mass originating from the iliopsoas muscle. Initially this mass was diagnosed as an iliopsoas hematoma in a patient using anticoagulants. However, it proved to be B-cell non-Hodgkin lymphoma. Compression was relieved by placement of an endovenous stent in the left common iliac vein., Endovenous stenting is a relatively new treatment modality that is used to treat post-thrombotic venous occlusions and chronic venous insufficiency. Only a few, case series have been described of stenting of compressed pelvic veins by adjacent structures such as gynecological malignancies. Although stent patency lasted only four weeks in this patient, venous stent placement quickly reliefs symptoms and should therefore be considered as an option to bridge time to allow development of sufficient venous collaterals.

Aim. Bare metal stents have improved results of endovascular treatment of aortoiliac occlusive disease. Polytetrafluoroethylene covered stents may further improve patency rates by preventing tissue ingrowth, and might reduce complications. This study was conducted to assess possible assets or liabilities of covered stents used for aortoiliac occlusive disease and to conduct a comparison with bare metal stents. Methods. A review was performed of literature published until March 2012 for infrarenal aortic lesions, iliac lesions and complex aortoiliac lesions. Outcomes were technical success, patency rates, clinical success and complication rates. Results were addressed to the three anatomic regions: the infrarenal aorta, the aortoiliac bifurcation and iliac arteries. Results. A total of 51 articles were included in the study. Overall technical success varied between 73% and 100%. Randomized data have proven the superiority of covered stents in extensive iliac occlusive lesions. Case series of patients with iliac occlusive disease demonstrated a 1-year primary patency of bare metal stents between 76% and 100% with a 5-year primary patency rate of 63%-83%. One-year primary patency of covered stents varied between 70% and 100%, while no long term patency rates with covered stents have been reported so far. Reliable comparisons between groups cannot be made due to variances in patient and lesion characteristics. Covered stents seem to improve results of kissing stents and are related to excellent results in isolated aortic lesions. No difference in complication rate between bare metal and covered stents have been described, to date. Conclusion. Covered stents improve results of endovascular treatment of extensive iliac occlusive lesions and are related to excellent results in isolated aortic lesions. They may provide a valid alternative for surgery in patients with extensive aortoiliac disease.

Mechanical circulatory support (MCS) devices provide both short and long term hemodynamic support for advanced heart failure patients. Unfortunately these devices remain plagued by thromboembolic complications associated with chronic platelet activation - mandating complex, lifelong anticoagulation therapy. To address the unmet need for enhancing the thromboresistance of these devices to extend their long term use, we developed a universal predictive methodology entitled Device Thrombogenicity Emulation (DTE) that facilitates optimizing the thrombogenic performance of any MCS device - ideally to a level that may obviate the need for mandatory anticoagulation. DTE combines in silico numerical simulations with in vitro measurements by correlating device hemodynamics with platelet activity coagulation markers before and after iterative design modifications aimed at achieving optimized thrombogenic performance. DTE proof-of-concept is demonstrated by comparing two rotary Left Ventricular Assist Devices (LVADs) (DeBakey vs HeartAssist 5, Micromed Houston, TX), the latter a version of the former following optimization of geometrical features implicated in device thrombogenicity. Cumulative stresses that may drive platelets beyond their activation threshold were calculated along multiple flow trajectories and collapsed into probability density functions (PDFs) representing the device 'thrombogenic footprint', indicating significantly reduced thrombogenicity for the optimized design. Platelet activity measurements performed in the actual pump prototypes operating under clinical conditions in circulation flow loops - before and after the optimization with the DTE methodology, show an order of magnitude lower platelet activity rate for the optimized device. The robust capability of this predictive technology - demonstrated here for attaining safe and cost-effective pre-clinical MCS thrombo-optimization - indicates its potential for reducing device thrombogenicity to a level that may significantly limit the extent of concomitant antithrombotic pharmacotherapy needed for safe clinical device use. PLoS One

To describe the conjunctival epithelial features seen with in vivo confocal microscopy (IVCM) after gold micro shunt (GMS) implantation in the suprachoroidal space, in patients with uncontrolled glaucoma.This was an observational case series study. Fourteen eyes of 14 consecutive glaucomatous patients with a history of multiple failed incisional surgeries followed by GMS implantation were evaluated with a digital confocal laser-scanning microscope (HRT II Rostock Cornea Module). Patients were divided into two groups: successful implantations (Group 1: eight patients, eight eyes), defined as a one-third reduction in preoperative intraocular pressure (IOP) with or without antiglaucoma medications and failed implantations (Group 2: six patients, six eyes) as a less than one-third reduction in preoperative IOP with maximal tolerated medical therapy. The examination was performed from 3 to 20 months (mean 15.4±5.4) postoperatively. Conjunctival mean microcyst density (MMD: cysts/mm(2)) and mean microcyst area (MMA: μm(2)) were the main outcome measurements.The mean postoperative IOP was statistically different between the two groups (p0.05), with the values of 14.3±2.77 and 32.3±8.01 mm Hg in Groups 1 and 2, respectively. When comparing successful with failed implantation, the IVCM analysis showed a greater MMD (p0.01) and MMA (p0.01). Clinical evidence of filtering bleb was not found in any of the patients.Successful GMS implantation significantly increased conjunctival microcysts density and surface at the site of the device insertion. These findings suggest that the enhancement of the aqueous filtration across the sclera may be one of the possible outflow pathways exploited by the shunt.

Background: Percutaneous valve replacement is a relatively new treatment modality that is rapidly growing in application. Aim&Objectives: Percutaneous treatment of valve diseases has been an alternative to open surgery since the early 1980's. In patients with mitral and aortic stenosis, use of percutaneous catheter-based techniques as balloon valvuloplasty has been a good therapeutic option. Later on, percutaneous aortic valve replacement was demonstrated in the 1990s in vitro, followed by successful in vivo applications. Although these techniques have difficulties such as paravalvular leakage difficulty in deployment; migration of valved stent; percutaneous valve replacement is currently one of the most researched and actively debated subjects in interventional cardiology. In this study we are aiming to review the current state and evaluate the pros and cons of this new treatment modality. Methods/Study Design: We enumerated the pros and cons from different perspectives, referenced and discussed them from articles that were obtained through Pubmed. Results and Findings: Percutaneous valve replacement procedures offer substantial advantages to patients. The reducing of surgical risks, the fact that the procedures are less invasive in comparison to open heart surgery, and that rehabilitation times are shorter are positive aspects [2]. However, there are complications such as paravalvular leakage difficulty in deployment; migration of valved stent, that needed to be taken into consideration and the selection of patients for the procedure needs improvement. Study Limitations: Percutaneous valve replacement is a relatively new treatment modality, few publications exist for consideration. Conclusion: With 12 different kinds of valves available for use, and improvement in patient selection, we expect to see an increase in percutaneous valve replacements worldwide.

BACKGROUND: Recommendations on the use of I-123 metaiodobenzylguanidine (MIBG) scintigraphy in localising phaeochromocytomas vary. The accuracy of I-123 MIBG scintigraphy was determined by evaluating our own I-123 MIBG scans and performing a meta-analysis. MATERIALS AND METHODS: Between January 1992 and May 2002, the I-123 MIBG scans of consecutive patients suspected of a phaeochromocytoma were re-evaluated. For the meta-analysis, studies with more than 5 I-123 MIBG scans were selected. RESULTS: Thirty patients were evaluated. The sensitivity in our own population was 92% and the specificity was 100%. Twelve articles were selected for our meta-analysis. The overall sensitivity and specificity were 96% and 100%, respectively. The sensitivity and specificity for tumours in the adrenal gland was 98% for both. For tumours located outside the adrenal gland, the sensitivity was 98% and the sensitivity for malignancies was 79%. CONCLUSION: 1-123 MIBG scintigraphy has an excellent sensitivity and specificity in localising phaeochromocytomas, except for malignant tumours. 1-123 MIBG scintigraphy is superior in localising tumours outside the adrenal gland.

A patient with a partially thrombosed fusiform giant basilar trunk aneurysm presented with devastating headache and symptoms of progressive brain stem compression. Having an aneurysm inaccessible for endovascular treatment, and after failing a vertebral artery balloon occlusion test, he was offered bypass surgery in order to exclude the aneurysm from the cerebral circulation and relieve his symptoms. A connection between the intracranial internal carotid artery and the superior cerebellar artery was created whereupon the basilar artery was ligated just distally to the aneurysm. The proximal anastomosis on the internal carotid artery was made using the excimer laser-assisted non-occlusive anastomosis (ELANA) technique, while a conventional end-to-side anastomosis was used for the distal anastomosis on the superior cerebellar artery. Intra-operative flowmetry showed a flow through the bypass of 40 ml/min after ligation of the basilar artery. An angiogram 24 hours later showed normal filling of the bypass and the vessels supplied by it, but also disclosed a subtotal occlusion of the proximal ipsilateral middle cerebral artery with delayed filling distally. The patient, who had a known thrombogenic coagulopathy, died the following day. Autopsy showed no signs of ischemia in the territories supplied by the bypass, but a thrombus in the proximal middle cerebral artery and massive acute hemorrhagic infarction with swelling in its territory and uncal herniation. Multiple fresh thrombi were found in the lungs. The ELANA anastomosis showed re-endothelialisation without thrombus formation on the inside.

Item does not contain fulltext RATIONALE AND OBJECTIVES: To evaluate 4 doses of gadobenate dimeglumine (Gd-BOPTA) for contrast-enhanced magnetic resonance angiography (CE-MRA) of the pelvic arteries and to compare CE-MRA with unenhanced time-of-flight MRA (2D-TOF-MRA). METHODS: A multicenter Phase II dose-finding study was performed in 136 patients with Gd-BOPTA doses of 0.025, 0.05, 0.1, and 0.2 mmol/kg bodyweight. Evaluation of CE-MRA images and comparison with 2D-TOF-MRA images was performed onsite and by 2 blinded offsite reviewers in terms of subjective image quality, number of lesions detected, and confidence in lesion characterization. RESULTS: Significant (P < 0.05) improvements over unenhanced findings were observed for CE-MRA at all dose levels. For reviewer 1 and the onsite investigators, the overall image quality increased up to a dose of 0.1 mmol/kg and then plateaued. For reviewer 2, increased image quality was noted up to a dose of 0.2 mmol/kg. Significant (P < 0.005) increases in diagnostic confidence on CE-MRA versus unenhanced MRA was observed for all dose groups by reviewer 1 and the onsite investigators and for the 0.1 and 0.2 mmol/kg dose groups by reviewer 2. No serious adverse events were recorded that were attributable to the study drug and no trends in laboratory parameters, vital signs, or electrocardiogram recordings were observed. CONCLUSIONS: Gadobenate dimeglumine-enhanced MRA is safe and significantly more effective than unenhanced 2D-TOF-MRA for imaging the pelvic arteries. A dose of 0.1 mmol/kg appears the most appropriate dose for subsequent Phase III clinical evaluation.

Background The main aim of performing a vascular anastomosis is to achieve maximal patency rates. An important factor to achieve that goal is to minimize damage to the vessel walls. Sutures inevitably induce vascular wall damage, which influences the healing of the anastomosis. Over time, several alternatives to sutures have become available. Methods A Medline literature search was performed to locate English, German and French language articles pertinent to non-suture methods of vascular anastomosis. Manual cross-referencing was also performed and many historical articles were included. Results and conclusion The non-suture techniques can be categorized into five groups based on the materials used: rings, clips, adhesives, stents and laser welding. With all these techniques a faster and less traumatic anastomosis can be made compared with sutures. However, each device is associated with technique-related complications. As a consequence, suturing continues to be the standard approach. The disadvantages of the non-suture techniques include: rigidity and a non-compliant anastomosis with rings; toxicity, leakage and aneurysm formation with adhesives; early occlusion with stents; cost, reduced strength in larger-sized vessels and demand for surgical skills with laser welding. Further refinement is needed before widespread adoption of these techniques can occur. Clips, however, may be particularly promising but long-term evaluation is required.

Background: Infusion of anti-tumour necrosis factor-alpha appears to be highly effective in patients with Crohn's disease. Aim: To assess the effect of infliximab on the quality of life in patients with active or fistulizing disease, as measured by the inflammatory bowel disease questionnaire, and to examine the impact on its four dimensions. Methods: An observational study was conducted in 65 patients. An infusion of 5 mg/kg infliximab was given at week 0 in patients with active disease and at week 0, 2 and 6 in fistulizing disease. Changes from baseline in the total and dimensional inflammatory bowel disease questionnaire scores were calculated and compared between the patient groups. Potential predictors of change in the quality of life were identified. Results: In the active disease group, at week 4, the mean total and dimensional inflammatory bowel disease questionnaire scores improved compared to baseline (P

Clinically used drugs and chemical agents may potentially cause adverse effects to the human auditory and vestibular systems. Many of them, such as aminoglycosides and cisplatin, can play a critical role in the treatment of serious or life-threatening diseases; others, like loop diuretics or salycilates, offer such important therapeutical effects compared to the ototoxic side effects that the ototoxicity risk can be considered to be of minor importance. The problem of ototoxic side effects is more acute in developing countries, where highly effective and low-cost drugs are more easily prescribed without adequate monitoring. Medical awareness of doses, forms of administration, populations at risk, and possible synergism is necessary in order to develop appropriate care in the prescription of drugs with ototoxic side effects. Relatively recent issues such as risk-benefit analysis, patient-informed consent, and quality-of-life considerations, particularly when life expectancy can be low, are also to be considered. At present, a uniform method of monitoring for all potentially ototoxic therapeutics does not seem reasonable or practical. It is recommended, however, that individual auditory function be noted for a particular drug being employed. Protocols and exams should be easy, quick, sensitive, reliable, and as objective as possible. Benefits of audiological monitoring include the opportunity to change the patient's treatment course, improvement of patient and family awareness of the impact of hearing impairment, and timely prescription of amplification devices. Finally, particular attention should be paid to high-risk populations such as neonatal intensive care unit patients.

Purpose - This paper aims to describe a numerical approach to the fragmentation of kidney stones by direct impact.