Increased aortic stiffness and blood pressure in non-classic Pompe disease

Vascular abnormalities and glycogen accumulation in vascular smooth muscle fibres have been described in Pompe disease. Using carotid-femoral pulse wave velocity (cfPWV), the gold standard methodology for determining aortic stiffness, we studied whether aortic stiffness is increased in patients with Pompe disease. Eighty-four adult Pompe patients and 179 age- and gender-matched volunteers participated in this cross-sectional case-controlled study. Intima media thickness and the distensibility of the right common carotid artery were measured using a Duplex scanner. Aortic augmentation index, central pulse pressure, aortic reflexion time and cfPWV were assessed using the SphygmoCor® system. CfPWV was higher in patients than in volunteers (8.8 versus 7.4 m/s, p < 0.001). This difference was still present after adjustment for age, gender, mean arterial blood pressure (MAP), heart rate and diabetes mellitus (p = 0.001), and was shown by subgroup analysis to apply to the 40-59 years age group (p = 0.004) and 60+ years age group (p = 0.01), but not to younger age groups (p = 0.99)

Increased aortic stiffness and blood pressure in non-classic Pompe disease

Vascular abnormalities and glycogen accumulation in vascular smooth muscle fibres have been described in Pompe disease. Using carotid-femoral pulse wave velocity (cfPWV), the gold standard methodology for determining aortic stiffness, we studied whether aortic stiffness is increased in patients with Pompe disease. Eighty-four adult Pompe patients and 179 age- and gender-matched volunteers participated in this cross-sectional case-controlled study. Intima media thickness and the distensibility of the right common carotid artery were measured using a Duplex scanner. Aortic augmentation index, central pulse pressure, aortic reflexion time and cfPWV were assessed using the SphygmoCor® system. CfPWV was higher in patients than in volunteers (8.8 versus 7.4 m/s, p < 0.001). This difference was still present after adjustment for age, gender, mean arterial blood pressure (MAP), heart rate and diabetes mellitus (p = 0.001), and was shown by subgroup analysis to apply to the 40-59 years age group (p = 0.004) and 60+ years age group (p = 0.01), but not to younger age groups (p = 0.99). Except for a shorter aortic reflexion time (p = 0.02), indirect indicators of arterial stiffness did not differ between patients and volunteers. Relative to volunteers (20 %), more Pompe patients had a history of hypertension (36 %, p = 0.005), and the MAP was higher than in volunteers (100 versus 92 mmHg, p < 0.001). This study shows that patients with non-classic Pompe disease have increased aortic stiffness and blood pressure. Whether this is due to glycogen accumulation requires further investigation. To reduce the potential risk of cardiovascular diseases, we recommend that blood pressure and other common cardiovascular risk factors are monitored regularly

Adjuvant Hepatic Arterial Infusion Pump Chemotherapy After Resection of Colorectal Liver Metastases: Results of a Safety and Feasibility Study in The Netherlands

Background: The 10-year overall survival with adjuvant hepatic arterial infusion pump (HAIP) chemotherapy after resection of colorectal liver metastases (CRLMs) was 61% in clinical trials from Memorial Sloan Kettering Cancer Center. A pilot study was performed to evaluate the safety and feasibility of adjuvant HAIP chemotherapy in patients with resectable CRLMs. Study Design: A phase II study was performed in two centers in The Netherlands. Patients with resectable CRLM without extrahepatic disease were eligible. All patients underwent complete resection and/or ablation of CRLMs and pump implantation. Safety was determined by the 90-day HAIP-related postoperative complications from the day of pump placement (Clavien–Dindo classification, grade III or higher) and feasibility by the successful administration of the first cycle of HAIP chemotherapy. Results: A total of 20 patients, with a median age of 57 years (interquartile range [IQR] 51–64) were included. Grade III or higher HAIP-related postoperative complications were found in two patients (10%), both of whom had a reoperation (without laparotomy) to replace a pump with a slow flow rate or to reposition a flipped pump. No arterial bleeding, arterial dissection, arterial thrombosis, extrahepatic perfusion, pump pocket hematoma, or pump pocket infections were found within 90 days after surgery. After a median of 43 days (IQR 29–52) following surgery, all patients received the first dose of HAIP chemotherapy, which was completed un

Adjuvant hepatic arterial infusion pump chemotherapy and resection versus resection alone in patients with low-risk resectable colorectal liver metastases - the multicenter randomized controlled PUMP trial

Background Recurrences are reported in 70% of all patients after resection of colorectal liver metastases (CRLM), in which half are confined to the liver. Adjuvant hepatic arterial infusion pump (HAIP) chemotherapy aims to reduce the risk of intrahepatic recurrence. A large retrospective propensity score analysis demonstrated that HAIP chemotherapy is particularly effective in patients with low-risk oncological features. The aim of this randomized controlled trial (RCT) --the PUMP trial-- is to investigate the efficacy of adjuvant HAIP chemotherapy in low-risk patients with resectable CRLM. Methods This is an open label multicenter RCT. A total of 230 patients with resectable CRLM without extrahepatic disease will be included. Only patients with a clinical risk score (CRS) of 0 to 2 are eligible, meaning: patients are allowed to have no more than two out of five poor prognostic factors (disease-free interval less than 12 months, node-positive colorectal cancer, more than 1 CRLM, largest CRLM more than 5 cm in diameter, serum Carcinoembryonic Antigen above 200 μg/L). Patients randomized to arm A undergo complete resection of CRLM without any adjuvant treatment, which is the standard of care in the Netherlands. Patients in arm B receive an implantable pump at the time of CRLM resection and start adjuvant HAIP chemotherapy 4–12 weeks after surgery, with 6 cycles of floxuridine scheduled. The primary endpoint is progression-free survival (PFS). Secondary endpoints include overall survival, hepatic PFS, safety, quality of life, and cost-effectiveness. Pharmacokinetics of intra-arterial administration of floxuridine will be investigated as well as predictive biomarkers for the efficacy of HAIP chemotherapy. In a side study, the accuracy of CT angiography will be compared to radionuclide scintigraphy to detect extrahepatic perfusion. We hypothesize that adjuvant HAIP chemotherapy leads to improved survival, improved quality of life, and

Problemen met hangouderen?: Ouderen signaleren tekorten in publieke ruimte

Plotseling leek er een nieuw maatschappelijk probleem te zijn: hangouderen. Ze zouden hinderlijk met hun rollators rondhangen in winkelcentra en vrouwen beschimpen. De vraag of hier werkelijk sprake was van een maatschappelijk probleem werd onderzocht door de berichtgeving in de media te analyseren, internetsearches te doen en telefonische interviews te houden

Van leugenbankje naar openluchtsocieteit: Hangouderen geven geen overlast

Plotseling leek er een nieuw maatschappelijk probleem te zijn: hangouderen. Ze zouden hinderlijk met hun rollators rondhangen in winkelcentra en vrouwen beschimpen. De vraag of hier werkelijk sprake was van een maatschappelijk probleem werd onderzocht door de berichtgeving in de media te analyseren, internetsearches te doen en telefonische interviews te houden

Interobserver variability of the International Federation of Gynecology and Obstetrics staging in cervical cancer

Objective: The objective of this study was to assess the interobserver variability of pelvic examination under anesthesia (EUA) in cervical cancer. Methods: Subsequent patients undergoing a staging procedure under anesthesia for primary cervical cancer were enrolled in the study. All clinicians assessed "blinded'' tumor size, and the involvement of vagina, parametria, sacrouterine ligaments, pelvic sidewalls, bladder, and/or rectum. Items were scored varying from 1 ("certainly no involvement''), 2 ("not sure about involvement''), to 3 (''involvement''). Each individual decided on the International Federation of Gynecology and Obstetrics (FIGO) stage; also, the urge for imaging and treatment proposal were accounted for. Final FIGO staging was obtained by consensus of the team. Investigators were classified as experienced after more than 50 EUAs. All others were classified less experienced. The free-marginal kappa values between experienced and less experienced investigators were calculated for all previously mentioned items. Results: Between February 2009 and December 2010, a total of 86 patients were enrolled. Among experienced investigators, a moderate interobserver agreement was found with regard to FIGO stage (free-marginal kappa value of 0.49) and an excellent interobserver agreement on their proposed therapy (free-marginal kappa value of 0.84). A lower level of agreement was found when comparing experienced with less experienced investigators: only a slight level of agreement on FIGO stage and a substantial agreement on their therapy proposal (free-marginal kappa values of 0.03 and 0.66). Conclusions: We describe only a moderate interobserver agreement on clinical staging of patients with cervical cancer. The interobserver agreement increases in the group of experienced doctors, indicating that EUA can be learned

Burgerkracht of spierballentaal?

Een pleidooi voor burgerkracht is te prijzen, maar de manier waarop Nico de Boer en Jos van der Lans dat hebben vormgegeven in hun rmo-essay schiet zijn doel voorbij.Het schikt zich naar populistisch vertoog en wakkert een gure wind aan