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Item does not contain fulltext OBJECTIVE: Randomized trials have shown an initial survival benefit of endovascular over conventional open abdominal aortic aneurysm repair but no long-term difference up to 6 years after repair. Longer follow-up may be required to demonstrate the cumulative negative impact on survival of higher reintervention rates associated with endovascular repair. METHODS: We updated the results of the Dutch Randomized Endovascular Aneurysm Management (DREAM) trial, a multicenter, randomized controlled trial comparing open with endovascular aneurysm repair, up to 15 years of follow-up. Survival and reinterventions were analyzed on an intention-to-treat basis. Causes of death and secondary interventions were compared by use of an events per person-year analysis. RESULTS: There were 178 patients randomized to open and 173 to endovascular repair. Twelve years after randomization, the cumulative overall survival rates were 42.2% for open and 38.5% for endovascular repair, for a difference of 3.7 percentage points (95% confidence interval, -6.7 to 14.1; P = .48). The cumulative rates of freedom from reintervention were 78.9% for open repair and 62.2% for endovascular repair, for a difference of 16.7 percentage points (95% confidence interval, 5.8-27.6; P = .01). No differences were observed in causes of death. Cardiovascular and malignant disease account for the majority of deaths after prolonged follow-up. CONCLUSIONS: During 12 years of follow-up, there was no survival difference between patients who underwent open or endovascular abdominal aortic aneurysm repair, despite a continuously increasing number of reinterventions in the endovascular repair group. Endograft durability and the need for continued endograft surveillance remain key issues.

Contains fulltext : 215386.pdf (Publisher’s version ) (Open Access) BACKGROUND: Autoimmune hepatitis requires long-term therapy, and systemic corticosteroids are the backbone of therapeutic management. Prolonged use of corticosteroids may lead to adverse events but data from long-term studies are mainly derived from studies in rheumatic diseases. AIM: To assess cataract, diabetes and fractures in relation to corticosteroid doses in the long-term maintenance treatment of patients with autoimmune hepatitis. METHODS: We retrospectively collected data on 476 patients (77% women) with an established diagnosis of autoimmune hepatitis. Binary logistic regression with a generalised estimating equation was used to analyse the association between current corticosteroid use and the incidence of cataract, diabetes and fractures with onset after autoimmune hepatitis diagnosis. We corrected for sex, age, cirrhosis at diagnosis and predniso(lo)ne use in the prior 3 years to account for possible ongoing effects. RESULTS: A total of 6634 years, with a median of 13 (range 1-40) per patient were recorded. The median age at diagnosis was 44 years (range 2-88). Adverse events were documented in 120 (25%) patients. Low-dose predniso(lo)ne (0.1-5.0 mg/d) increased the odds of fractures whereas higher doses (>5.0 mg/d) increased the odds of cataracts and diabetes. Budesonide increased the odds of cataract and fractures; this effect was independent of predniso(lo)ne use in the prior 1, 2 or 3 years. CONCLUSIONS: Even low doses of corticosteroids frequently lead to substantial adverse events refuting the assumption that adverse events are prevented by administering low doses.

Background Pruritus (itch) is a frequent, burdensome and difficult-to-treat symptom in patients with cholestasis. Fibrates are currently under investigation for the treatment of primary biliary cholangitis in patients with a suboptimal response to ursodeoxycholic acid. Moreover, there is empirical evidence for a possible antipruritic effect. We aim to prove this in a randomized controlled trial, including patients with cholestatic liver diseases other than primary biliary cholangitis that are accompanied by pruritus. Methods A multicenter investigator-initiated, double-blind, randomized placebo-controlled trial to evaluate the effect of bezafibrate on cholestatic pruritus in 84 adult patients with primary biliary cholangitis or primary/secondary sclerosing cholangitis. Primary outcome is the proportion of patients with a reduction of itch intensity of 50% or more (measured on a Visual Analog Scale) after 21 days of treatment with bezafibrate 400 mg qid or placebo. Secondary outcomes include the effect of bezafibrate on a five-dimensional itch score, liver disease-specific quality of life, serum liver tests and autotaxin activity. Safety will be evaluated through serum parameters for kidney function and rhabdomyolysis as well as precise recording of (serious) adverse events. We provide a schematic overview of the study protocol and describe the methods used to recruit and randomize patients, collect and handle data and perform statistical analyses. Discussion Given its favorable safety profile and anticholestatic properties, bezafibrate may become the new first-line treatment option for treating cholestatic pruritus. Trial registration Netherlands Trial Register, ID: NCT02701166. Registered on 2 March 2016; Netherlands Trial Register, ID: NTR5436. Registered on 3 August 2015. Electronic supplementary material The online version of this article (doi:10.1186/s13063-017-1966-8) contains supplementary material, which is available to authorized users.

Background: Identifying patients at risk for aneurysm rupture and sac expansion after open and endovascular abdominal aortic aneurysm (AAA) repair (EVAR) may help to attenuate this risk by intensifying follow-up and early detection of problems. The goal of this study was to validate the St George’s Vascular Institute (SGVI) score to identify patients at risk for a secondary intervention after elective aneurysm repair. Methods: A post hoc on-treatment analysis of a randomized trial comparing open AAA repair and EVAR was performed. In this multicenter trial, 351 patients were randomly assigned to undergo open AAA repair or EVAR. Information on survival and reinterventions was available for all patients at 5 years postoperatively, for 79% at 6 years, and for 53% at 7 years. Open repair was completed in 173 patients and EVAR in 171, based on an on-treatment analysis. Because 17 patients had incomplete anatomic data, 327 patients (157 open repair and 170 EVAR) were available for analysis. During 6 years of follow-up, 78 patients underwent at least one reintervention. The SGVI score, which is calculated from preoperative AAA morphology using aneurysm and iliac diameter, predictively dichotomized patients into groups at highrisk or low-risk for a secondary intervention. The observed freedom from reintervention was compared between groups at predicted high-risk and predicted low-risk. Results: The 20 patients in the high-risk group were indeed at higher risk for a secondary intervention compared with the 307 patients predicted to be at low risk (hazard ratio [HR], 3.82; 95% confidence interval [CI], 2.05-7.11; P < .001). Discrimination between high-risk and low-risk groups was valid for EVAR (HR, 4.06; 95% CI, 1.93-8.51; P < .001) and for open repair (HR, 3.41; 95% CI, 1.02-11.4; P [ .033). Conclusions: The SGVI score appears to be a useful tool to predict reintervention risk in patients after open repair and EVAR.

The purpose of this case report is to describe a rare case of a patient with a phaeochromocytoma with several cardiovascular complications, which can be attributed to the tumour. Detection of a phaeochromocytoma sometimes needs a 'Sherlock Holmes spirit' or simply time.

Contains fulltext : 79676.pdf (Publisher’s version ) (Closed access) BACKGROUND: Patients with inflammatory bowel disease (IBD) and concurrent primary sclerosing cholangitis (PSC) have a higher risk of developing colorectal cancer (CRC) than IBD patients without PSC. The aim of this study was to investigate potential clinical differences between patients with CRC in IBD and those with CRC in IBD and PSC, as this may lead to improved knowledge of underlying pathophysiological mechanisms of CRC development. METHODS: The retrospective study from 1980-2006 involved 7 Dutch university medical centers. Clinical data were retrieved from cases identified using the national pathology database (PALGA). RESULTS: In total, 27 IBD-CRC patients with PSC (70% male) and 127 IBD-CRC patients without PSC (59% male) were included. CRC-related mortality was not different between groups (30% versus 19%, P = 0.32); however, survival for cases with PSC after diagnosing CRC was lower (5-year survival: 40% versus 75% P = 0.001). Right-sided tumors were more prevalent in the PSC group (67% versus 36%, P = 0.006); adjusted for age, sex, and extent of IBD, this difference remained significant (odds ratio: 4.8, 95% confidence interval [CI] 2.0-11.8). In addition, tumors in individuals with PSC were significantly more advanced. CONCLUSIONS: The right colon is the predilection site for development of colonic malignancies in patients with PSC and IBD. When such patients are diagnosed with cancer they tend to have more advanced tumors than patients with IBD without concurrent PSC, and the overall prognosis is worse. Furthermore, the higher frequency of right-sided tumors in patients with PSC suggests a different pathogenesis between patients with PSC and IBD and those with IBD alone.

This paper describes the design of an autopilot for rudder roll stabilization for ships. This autopilot uses the rudder not only for course keeping but also for reduction of the roll. The system has a series of properties which make the controller design far from straightforward: the process has only one input (the rudder angle) and two outputs (the heading and the roll angle); the transfer from rudder to roll is non-minimum-phase; because large and high-frequency rudder motions are necessary, the non-linearities of the steering machine cannot be disregarded; the disturbances caused by the waves vary considerably in amplitude and frequency spectrum. In order to solve these problems a new approach to the LQG method has been developed. The control algorithms were tested by means of computer simulations, scale-model experiments and full-scale trials at sea. The results indicate that a rudder roll stabilization system is able to reduce the roll as well as a conventional fin stabilization system, while it requires less investments. Based on the results obtained in this project the Royal Netherlands Navy has decided to implement rudder roll stabilization on a series of ships under construction at this moment.

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