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See detailLong-term glycaemic effects of pioglitazone compared with placebo as add-on treatment to metformin or sulphonylurea monotherapy in PROactive (PROactive 18)
Scheen, André ULiege; Tan, M. H.; Betteridge, D. J. et al

in Diabetic Medicine : A Journal of the British Diabetic Association (2009)

Abstract Aims To assess the long-term glycaemic effects, concomitant changes in medications, and initiation of permanent insulin use (defined as daily insulin use for a period of ≥90 days, or ongoing use ... [more ▼]

Abstract Aims To assess the long-term glycaemic effects, concomitant changes in medications, and initiation of permanent insulin use (defined as daily insulin use for a period of ≥90 days, or ongoing use at death/final visit) with pioglitazone vs. placebo in diabetic patients receiving metformin or sulphonylurea monotherapy at baseline in the PROspective pioglitAzone Clinical Trial in macroVascular Events (PROactive). Methods In PROactive, patients with Type 2 diabetes and macrovascular disease were randomized to pioglitazone (force-titrated to 45 mg/day) or placebo, in addition to other existing glucose-lowering therapies. In a post-hoc analysis, we categorized patients not receiving insulin at baseline and treated by oral monotherapy into two main cohorts: addon to metformin alone (n = 514) and sulphonylurea alone (n = 1001). The follow-up averaged 34.5 months. Results There were significantly greater reductions in glycated haemoglobin (HbA1c) with pioglitazone than with placebo and more pioglitazone-treated patients achieved HbA1c targets, irrespective of the baseline oral glucose-lowering regimen and despite a decrease in the use of other glucose-lowering agents. Approximately twice as many in the placebo groups progressed to permanent insulin use than in the pioglitazone groups across the two cohorts: 3.4% for pioglitazone and 6.5% for placebo when added to metformin monotherapy and 6.3% and 14.8%, respectively, when added to sulphonylurea monotherapy. The overall safety of both dual therapies was good. Conclusions Intensifying an existing oral monotherapy regimen to a dual oral regimen by adding pioglitazone resulted in sustained improvements in glycaemic control and reduced progression to insulin therapy. The efficacy and safety of adding pioglitazone to either metformin monotherapy or sulphonylurea monotherapy were good. [less ▲]

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See detailLong-term glycaemic effects of pioglitazone in triple oral therapy: Results from PROactive
Charbonnel, B.; Scheen, André ULiege

in Diabetologia (2006, September), 49(Suppl. 1), 488-489

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See detailLong-term magnetic field monitoring of the Sun-like star \xi Bootis A
Morgenthaler, A.; Petit, P.; Saar, S. et al

in Astronomy and Astrophysics (2012), 540

Aims: We aim to investigate the long-term temporal evolution of the magnetic field of the solar-type star ξ Bootis A, both from direct magnetic field measurements and from the simultaneous estimate of ... [more ▼]

Aims: We aim to investigate the long-term temporal evolution of the magnetic field of the solar-type star ξ Bootis A, both from direct magnetic field measurements and from the simultaneous estimate of indirect activity indicators. Methods: We obtained seven epochs of high-resolution, circularly-polarized spectra from the NARVAL spectropolarimeter between 2007 and 2011, for a total of 76 spectra. Using approximately 6100 photospheric spectral lines covering the visible domain, we employed a cross-correlation procedure to compute a mean polarized line profile from each spectrum. The large-scale photospheric magnetic field of the star was then modelled by means of Zeeman-Doppler Imaging, allowing us to follow the year-to-year evolution of the reconstructed magnetic topology. Simultaneously, we monitored the width of several magnetically sensitive spectral lines, the radial velocity, the line asymmetry of intensity line profiles, and the chromospheric emission in the cores of the Ca II H and Hα lines. Results: During the highest observed activity states, in 2007 and 2011, the large-scale field of ξ Bootis A is almost completely axisymmetric and is dominated by its toroidal component. The toroidal component persists with a constant polarity, containing a significant fraction of the magnetic energy of the large-scale surface field through all observing epochs. The magnetic topologies reconstructed for these activity maxima are very similar, suggesting a form of short cyclicity in the large-scale field distribution. The mean unsigned large-scale magnetic flux derived from the magnetic maps varies by a factor of about 2 between the lowest and highest observed magnetic states. The chromospheric flux is less affected and varies by a factor of 1.2. Correlated temporal evolution, due to both rotational modulation and seasonal variability, is observed between the Ca II emission, the Hα emission and the width of magnetically sensitive lines. The rotational dependence of polarimetric magnetic measurements displays a weak correlation with other activity proxies, presumably due to the different spatial scales and centre-to-limb darkening associated with polarimetric signatures, as compared to non-polarized activity indicators. Better agreement is observed on the longer term. When measurable, the differential rotation reveals a strong latitudinal shear in excess of 0.2 rad d-1. [less ▲]

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See detailLong-term magnetic field monitoring of the sun-like star ξ Bootis A
Morgenthaler, A.; Petit, P.; Aurière, M. et al

in Boissier, S.; Heydari-Malayeri, M.; Samadi, R. (Eds.) et al SF2A-2010: Proceedings of the Annual meeting of the French Society of Astronomy and Astrophysics (2010, December 01)

Phase-resolved observations of the solar-type star ξ Bootis A were obtained using the Narval spectropolarimeter at Telescope Bernard Lyot (Pic du Midi, France) during years 2007, 2008, 2009 and 2010. The ... [more ▼]

Phase-resolved observations of the solar-type star ξ Bootis A were obtained using the Narval spectropolarimeter at Telescope Bernard Lyot (Pic du Midi, France) during years 2007, 2008, 2009 and 2010. The data sets enable us to study both the rotational and the long-term evolution of various activity tracers. Here, we focus on the large-scale photospheric magnetic field (reconstructed by Zeeman-Doppler Imaging), the Zeeman broadening of the FeI 846.84 nm magnetic line, and the chromospheric CaII H and Hα emission. [less ▲]

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See detailLong-term memory effects on verbal short-term memory : a replication study
Majerus, Steve ULiege; Van der Linden, Martial ULiege

in British Journal of Developmental Psychology (2003), 21(Part 2), 303-310

The influence of lexico-semantic language representations stored in long-term memory (LTM) on short-term memory (STM) performance has been studied extensively in adults. However, there are relatively few ... [more ▼]

The influence of lexico-semantic language representations stored in long-term memory (LTM) on short-term memory (STM) performance has been studied extensively in adults. However, there are relatively few data on lexico-semantic LTM effects on STM in children. On the other hand, the influence of phonological LTM effects on STM has been studied more extensively in children than in adults. In this study, we explored whether these different LTM effects on verbal STM could be replicated in both adults and children by administering immediate serial recall tasks (ISR) for high- and low-frequency words, for high- and low-imageability words, for words and non-words, and for high and low phonotactic frequency non-words to 6-, 8-, and 10-year-old children, to adolescents and to adults. Significant word frequency, lexicality and phonotactic frequency effects were observed in all age groups, as well as a word imageability effect which was, however, weaker than the other three effects. Our data suggest that LTM effects on STM are equivalent in both children and adults. [less ▲]

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See detailLong-term monitoring of European eels in the Belgian Meuse River basin. From the historical drastic decline to recent outcomes of restocking practices.
Nzau Matondo, Billy ULiege; Dierckx, Arnaud ULiege; Benitez, Jean-Philippe ULiege et al

Conference (2017, June)

Long-term monitoring of yellow- and glass eel stages were performed in upland areas (>300km from sea) in order (i) to quantify the decline of wild yellow eels entering in the Belgian Meuse from the ... [more ▼]

Long-term monitoring of yellow- and glass eel stages were performed in upland areas (>300km from sea) in order (i) to quantify the decline of wild yellow eels entering in the Belgian Meuse from the Netherlands; (ii) to follow their upstream individual colonization; and (iii) to analyze the adaptation of young eels stocked as glass eels imported from UK. By monitoring a fish pass from 1992 to 2016, we quantified that the number of ascending eels has declined from n=5613 in 1992 to n=21 in 2016 (3.99%/year) and the mean length of eels has increased (4.1mm/year). During 6-years, upstream individual colonization of eels (2010-2015, n=1371) was followed using fixed RFIDtracking system. Few eels continued to migrate 4 years after tagging (<0.3%) and at >20km upstream (3.7%); and velocity of eels varied between individuals (0.012- 3.1km/day). In a 4-year (2013-2016) monitoring study of restocked glass eels using electrofishing and mobile RFID-tracking campaigns, we observed that eels grew rapidly in upland small brooks and recruitment was better in rivers with high carrying capacity (>15.8%, 2years post-stocking). Restocked eels dispersed in up- and downstream directions with behaviors including sedentary, nomadic and intermediate lifestyles. This suggests stocking as potential management measure to enhance local eel stocks. [less ▲]

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See detailLong-term multicolour photometry and high-resolution spectroscopy of the two gamma Doradus stars HD 12901 and HD 48501
Aerts, C.; Cuypers, J.; De Cat, P. et al

in Astronomy and Astrophysics (2004), 415

We gathered long-term multicolour Geneva UB[SUB]1[/SUB]BB[SUB]2[/SUB]V[SUB]1[/SUB]VG photometric and high-resolution (R=40 000) spectroscopic data of the two gamma Doradus stars HD 12901 and HD 48501. The ... [more ▼]

We gathered long-term multicolour Geneva UB[SUB]1[/SUB]BB[SUB]2[/SUB]V[SUB]1[/SUB]VG photometric and high-resolution (R=40 000) spectroscopic data of the two gamma Doradus stars HD 12901 and HD 48501. The photometry reveals three frequencies for each of the two stars: f[SUB]1[/SUB]=1.21563 c d[SUP]-1[/SUP], f[SUB]2[/SUB]=1.39594 c d[SUP]-1[/SUP] and f[SUB]3[/SUB]=2.18636 c d[SUP]-1[/SUP] for HD 12901 and f[SUB]1[/SUB]=1.09408 c d[SUP]-1[/SUP], f[SUB]2[/SUB]=1.29054 c d[SUP]-1[/SUP] and f[SUB]3[/SUB]=1.19924 c d[SUP]-1[/SUP] for HD 48501. The photometric amplitude is each time largest in the Geneva B[SUB]1[/SUB] filter and the variations in all the different filters are perfectly in phase within the measurement errors. Mode identification points out that the six modes are all l=1 modes and that the non-adiabatic temperature variations are extremely small, in contradiction to current theoretical predictions. Our spectra show that all the observed frequencies are intrinsic to the stars and cannot be due to binarity. We detect clear line-profile variations at low amplitude (<1 km s[SUP]-1[/SUP]) due to the oscillations of both targets. The estimated v sin i from the spectra are Ë 53 km s[SUP]-1[/SUP] for HD 12901 and Ë 29 km s[SUP]-1[/SUP] for HD 48501. It is at present unclear if the triplet-like structure for HD 48501 is the consequence of rotational splitting or of the large separation expected for high-order gravity modes in the asymptotic regime. Based on observations gathered with the Swiss 0.7 m telescope equipped with the photometer P 7 and with the Swiss 1.2 m Euler telescope equipped with the spectrograph CORALIE, both situated at La Silla, Chile. Reduced data available upon request from the first author. [less ▲]

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See detailLong-term neurodevelopmental outcome and exercise capacity after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy.
Hovels-Gurich, Hedwig H; Konrad, Kerstin; Skorzenski, Daniela et al

in Annals of Thoracic Surgery (2006), 81(3), 958-66

BACKGROUND: The purpose of this prospective study was to assess whether neurodevelopmental status and exercise capacity of children 5 to 10 years after corrective surgery for tetralogy of Fallot or ... [more ▼]

BACKGROUND: The purpose of this prospective study was to assess whether neurodevelopmental status and exercise capacity of children 5 to 10 years after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy was different compared with normal children and influenced by the preoperative condition of hypoxemia or cardiac insufficiency. METHODS: Forty unselected children, 20 with tetralogy of Fallot and hypoxemia and 20 with ventricular septal defect and cardiac insufficiency, operated on with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass at a mean age of 0.7 +/- 0.3 years (mean +/- SD), underwent, at mean age 7.4 +/- 1.6 years, standardized evaluation of neurologic status, gross motor function, intelligence, academic achievement, language, and exercise capacity. Results were compared between the groups and related to preoperative, perioperative, and postoperative status and management. RESULTS: Rate of mild neurologic dysfunction was increased compared with normal children, but not different between the groups. Exercise capacity and socioeconomic status were not different compared with normal children and between the groups. Compared with the normal population, motor function, formal intelligence, academic achievement, and expressive and receptive language were significantly reduced (p < 0.01 to p < 0.001) in the whole group and in the subgroups, except for normal intelligence in ventricular septal defect patients. Motor dysfunction was significantly higher in the Fallot group compared with the ventricular septal defect group (p < 0.01) and correlated with neurologic dysfunction, lower intelligence, and reduced expressive language (p < 0.05 each). Reduced New York Heart Association functional class was correlated with lower exercise capacity and longer duration of cardiopulmonary bypass (p < 0.05 each). Reduced socioeconomic status significantly influenced dysfunction in formal intelligence (p < 0.01) and academic achievement (p < 0.05). Preoperative risk factors such as prenatal hypoxia, perinatal asphyxia, and preterm birth, factors of perioperative management such as cardiac arrest, lowest nasopharyngeal temperature, and age at surgery, and postoperative risk factors as postoperative cardiocirculatory insufficiency and duration of mechanical ventilation were not different between the groups and had no influence on outcome. Degree of hypoxemia in Fallot patients and degree of cardiac insufficiency in ventricular septal defect patients did not influence the outcome within the subgroups. CONCLUSIONS: Children with preoperative hypoxemia in infancy are at higher risk for motor dysfunction than children with cardiac insufficiency. Corrective surgery in infancy for tetralogy of Fallot or ventricular septal defect with combined circulatory arrest and low flow bypass is associated with reduced neurodevelopmental outcome, but not with reduced exercise capacity in childhood. In our experience, the general risk of long-term neurodevelopmental impairment is related to unfavorable effects of the global perioperative management. Socioeconomic status influences cognitive capabilities. [less ▲]

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See detailLong-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation.
Hovels-Gurich, Hedwig H; SEGHAYE, Marie-Christine ULiege; Schnitker, Ralph et al

in Journal of Thoracic and Cardiovascular Surgery (The) (2002), 124(3), 448-58

OBJECTIVE: Neurodevelopmental status of children between 8 and 14 years of age after neonatal arterial switch operation for transposition of the great arteries has not previously been systematically ... [more ▼]

OBJECTIVE: Neurodevelopmental status of children between 8 and 14 years of age after neonatal arterial switch operation for transposition of the great arteries has not previously been systematically evaluated. METHODS: Within a longitudinal study, 60 unselected children operated on as neonates with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass were reevaluated at the age of 7.9 to 14.3 years (mean +/- SD 10.5 +/- 1.6 years). Clinical neurologic status and standardized tests to assess gross motor function, intelligence, acquired abilities, language, and speech were carried out, and the results were related to preoperative, perioperative, and postoperative status, to management, and to neurodevelopmental status at a mean age of 5.4 years. RESULTS: Neurologic and speech impairments were evidently more frequent (27% and 40%, respectively) than in the general population. Intelligence and socioeconomic status were not different (P =.29 and P =.11), whereas motor function, acquired abilities, and language were reduced (P < or =.04 for each). Overall rate of developmental impairment in one or more domains was 55%, compared with 26% at age 5.4 years. Multivariable analysis showed that severe preoperative acidosis and hypoxia predicted reduced motor function (mean deficit 52.7 points, P <.001), whereas longer bypass duration predicted both neurologic (odds ratio per 10 minutes of bypass duration 1.8, P =.04) and speech (odds ratio per 10 minutes of bypass duration 1.9, P =.02) dysfunction, and perioperative and postoperative cardiocirculatory insufficiency predicted neurologic (odds ratio 6.5, P =.04) and motor (mean deficit 6.8 points, P =.03) dysfunction. CONCLUSIONS: The neonatal arterial switch operation with combined circulatory arrest and low-flow bypass is associated increasingly with age, with reduced neurodevelopmental outcome but not with cognitive dysfunction. In our experience, the risk of long-term neurodevelopmental impairment after neonatal corrective cardiac surgery is related to deleterious effects of the global perioperative management and to special adverse effects of prolonged bypass duration. Severe preoperative acidosis and hypoxia and postoperative hemodynamic instability must be considered as important additional risk factors. [less ▲]

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See detailLong-term optical spectrophotometric monitoring of comet C/1995 O1 (Hale-Bopp)
Rauer, H.; Helbert, J.; Arpigny, Claude ULiege et al

in Astronomy and Astrophysics (2003), 397

We observed comet C/1995 O1 (Hale-Bopp) at 4.6-2.9 AU pre-perihelion and 2.8-12.8 AU post-perihelion with optical long-slit spectroscopy. Emission bands of CN, C[SUB]3[/SUB], C[SUB]2[/SUB] and NH[SUB]2 ... [more ▼]

We observed comet C/1995 O1 (Hale-Bopp) at 4.6-2.9 AU pre-perihelion and 2.8-12.8 AU post-perihelion with optical long-slit spectroscopy. Emission bands of CN, C[SUB]3[/SUB], C[SUB]2[/SUB] and NH[SUB]2[/SUB] have been covered. Emission of C[SUB]3[/SUB] was detected up to 7.0 AU, and CN could be followed up to 9.8 AU post-perihelion. Spatial column density profiles of the radicals have been used to derive effective parent Haser scale lengths for heliocentric distances beyond 3 AU. Production rates were derived based on these Haser scale lengths. The observations of CN are in agreement with HCN as the major parent molecule of this radical at large distances from the Sun (i.e. beyond ~ 3 AU). We compare the measured CN production rate to sublimation rates of HCN from a simple nucleus sublimation model. The variation of CN production rates with changing heliocentric distance gives no indication for sublimation from the interior and is consistent with very little thermal lag of the nucleus. Based on observations collected at the European Southern Observatory, Chile, and the Observatoire de Haute Provence, France. [less ▲]

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See detailLong-term outcome after infliximab for refractory ulcerative colitis
Ferrante, M.; Vermeire, S.; Fidder, H. et al

in Journal of Crohn’s and Colitis [=JCC] (2008), 2(3), 219-225

Background and aims: Infliximab (IFX) has been shown efficacious for moderate-to-severe ulcerative colitis (UC), but data on long-term efficacy are tacking. We investigated long-term outcome including ... [more ▼]

Background and aims: Infliximab (IFX) has been shown efficacious for moderate-to-severe ulcerative colitis (UC), but data on long-term efficacy are tacking. We investigated long-term outcome including colectomy rates in outpatients treated with IFX for refractory UC in a single referral centre, and evaluated if predictors could be identified. Methods: The first 121 outpatients (median age 38.0 years) with refractory UC treated with IFX were included. The primary outcome was colectomy-free survival. Secondary measures were sustained clinical response and serious adverse events. Results: From the 81 patients (67%) with an initial clinical response to IFX, 68% had a sustained clinical response. No independent predictors of sustained clinical response could be identified. Over a median (IQR) follow-up period of 33.0 (17.0-49.8) months, 21 patients (17%) came to colectomy. Independent predictors of colectomy were absence of short-term clinical response [Hazard ratio 10.8 (95% Cl 3.5-32.8), p < 0.001], a baseline CRP level >= 5 mg/L [Hazard ratio 14.5 (95% Cl 2.0-108.6), p=0.006] and previous IV treatment with corticosteroids and/or cyctosporine [Hazard ratio 2.4 (95% Cl 1.1-5.9), p=0.033]. Six patients developed a serious infection, three a malignancy, two a post-operative complication and one patient died (suicide). Conclusions: With a median follow-upof 33.0 months after start of IFX, 17% of patients with refractory UC needed colectomy, while sustained clinical response was present in 68% of initial responders. (c) 2008 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved. [less ▲]

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See detailLong-term outcome and clinical spectrum of 73 pediatric patients with mitochondrial diseases.
DEBRAY, François-Guillaume ULiege; Lambert, Marie; Chevalier, Isabelle et al

in Pediatrics (2007), 119(4), 722-733

OBJECTIVES: We sought to determine the clinical spectrum, survival, and long-term functional outcome of a cohort of pediatric patients with mitochondrial diseases and to identify prognostic factors ... [more ▼]

OBJECTIVES: We sought to determine the clinical spectrum, survival, and long-term functional outcome of a cohort of pediatric patients with mitochondrial diseases and to identify prognostic factors. METHODS: Medical charts were reviewed for 73 children diagnosed between 1985 and 2005. The functional status of living patients was assessed prospectively by using the standardized Functional Independence Measure scales. RESULTS: Patients fell into 7 phenotypic categories: neonatal-onset lactic acidosis (10%), Leigh syndrome (18%), nonspecific encephalopathy (32%), mitochondrial (encephalo)myopathy (19%), intermittent neurologic (5%), visceral (11%), and Leber hereditary optic neuropathy (5%). Age at first symptoms ranged from prenatal to 16 years (median: 7 months). Neurologic symptoms were the most common (90%). Visceral involvement was observed in 29% of the patients. A biochemical or molecular diagnosis was identified for 81% of the patients as follows: deficiency of complex IV (27%), of pyruvate dehydrogenase or complex I (25% each), of multiple complexes (13%), and of pyruvate carboxylase (5%) or complexes II+III (5%). A mitochondrial DNA mutation was found in 20% of patients. At present, 46% of patients have died (median age: 13 months), 80% of whom were <3 years of age. Multivariate analysis showed that age at first symptoms was a major independent predictor of mortality: patients with first symptoms before 6 months had a highly increased risk of mortality. Cardiac or visceral involvement and neurologic crises were not independent prognostic factors. Living patients showed a wide range of independence levels that correlated positively with age at first symptoms. Among patients aged >5 years (n = 32), 62% had Functional Independence Measure quotients of >0.75. CONCLUSIONS: Mitochondrial diseases in children span a wide range of symptoms and severities. Age at first symptoms is the strongest predictor mortality. Despite a high mortality rate in the cohort, 62% of patients aged >5 years have only mild impairment or normal functional outcome. [less ▲]

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See detailLong-term outcome and complication rates in patients with macroprolactinomas treated with different therapeutic approaches : a comparative study of 184 patients
Elenkova, A.; PETROSSIANS, Patrick ULiege; Vasilev, V. et al

in ENEA Munich - abstract book (2011, December)

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See detailLong-term outcome and use of 6-Minute Walk test in West Highland white Terriers with idiopathic pulmonary fibrosis
Lilja-Maula, LIO; Laurila, HP; Syrjä, P et al

in Journal of Veterinary Internal Medicine (2014), 28(2), 379-385

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See detailLong-term outcome in dogs with sinonasal aspergillosis treated with intranasal infusions of enilconazole
Schuller, S.; Clercx, Cécile ULiege

in Journal of the American Animal Hospital Association (2007)

Long-term outcomes (mean 38+/-17 months) were evaluated in 27 dogs with sinonasal aspergillosis after successful medical treatment using intranasal infusions of 1% or 2% enilconazole (1%, n=15; 2%, n=12 ... [more ▼]

Long-term outcomes (mean 38+/-17 months) were evaluated in 27 dogs with sinonasal aspergillosis after successful medical treatment using intranasal infusions of 1% or 2% enilconazole (1%, n=15; 2%, n=12). Long-term outcomes with both treatment protocols were good, with half of the dogs being asymptomatic throughout the follow-up period. The remaining dogs showed mild clinical signs compatible with chronic rhinitis/sinusitis. These clinical signs were interpreted as chronic lymphoplasmacytic rhinitis/sinusitis and episodes of bacterial rather than fungal infection. Three dogs had confirmed reinfection or relapse 2 to 36 months after clinical resolution [less ▲]

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See detailLong-term outcome of Epilepsy surgery among 399 patients with non-lesional pathology including mesial temporal lobe sclerosis.
Scholsem, M.; Cohen-Gadol, A.; Martin, Didier ULiege et al

Conference (2006, March)

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See detailLong-term outcome of Leigh syndrome caused by the NARP-T8993C mtDNA mutation.
DEBRAY, François-Guillaume ULiege; Lambert, Marie; Lortie, Anne et al

in American Journal of Medical Genetics. Part A (2007), 143A(17), 2046-51

Mutations at mitochondrial DNA (mtDNA) nucleotide 8993 can cause neurogenic weakness, ataxia and retinitis pigmentosa (NARP syndrome), or maternally inherited Leigh syndrome (LS), with a correlation ... [more ▼]

Mutations at mitochondrial DNA (mtDNA) nucleotide 8993 can cause neurogenic weakness, ataxia and retinitis pigmentosa (NARP syndrome), or maternally inherited Leigh syndrome (LS), with a correlation between the amount of mutant mtDNA and the severity of the neurological disease. The T8993C mutation is generally considered to be clinically milder than the T8993G mutation but when the level of heteroplasmy exceeds 90%, progressive neurodegeneration has been found. We report on a long-term follow-up of a patient who presented at 4 years of age with typical LS but showed an unexpected resolution of his symptoms and a favorable outcome. At 18 years of age, his neurological examination was near normal, with neither peripheral neuropathy nor retinopathy. mtDNA analysis identified the presence of T8993C mutation at high level (>95%) in the patient's blood leukocytes. This case report and literature review emphasizes the variability of the phenotypic expression of the T8993C mutation and the need for caution in predictive counseling in such patients. (c) 2007 Wiley-Liss, Inc. [less ▲]

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See detailLong-term outcome of patients with acromegaly and congestive heart failure
Bihan, Hélène; Espinosa, Consuelo; Valdes Socin, Hernan Gonzalo ULiege et al

in Journal of Clinical Endocrinology and Metabolism (2004), 89(11), 5308-5313

Cardiovascular complications are a major cause of morbidity and mortality in patients with acromegaly. Normalization of GH secretion is associated with an improvement in structural and functional cardiac ... [more ▼]

Cardiovascular complications are a major cause of morbidity and mortality in patients with acromegaly. Normalization of GH secretion is associated with an improvement in structural and functional cardiac abnormalities. However, the long-term cardiac effects of treatment for acromegaly have not been studied in patients who have already developed chronic congestive heart failure (CHF). We reviewed the charts of 330 consecutive patients with acromegaly treated in two French and Belgian centers since 1985. Ten patients with both acromegaly and CHF (eight men, two women, mean age 49.7 yr) were studied retrospectively. One of them was excluded because CHF was due to severe aortic stenosis. CHF ( New York Heart Association stages III-IV and echocardiography showing dilated hypokinetic cardiomyopathy with left ventricular systolic dysfunction and a left ventricular ejection fraction less than 45%) was diagnosed before, concomitantly, or after acromegaly in, respectively, two, five, and two patients. Three patients were referred with terminal heart failure requiring transplantation. One patient had transient CHF associated with a hypertensive crisis. The other eight patients had symptomatic chronic CHF. Control of GH hypersecretion failed, totally or partially, in three patients: one had a long-term survival, and the two others died at 1 and 5 yr. Good GH control was achieved in five patients: four of these are still alive 2-16 yr after diagnosis of CHF, their clinical status is stable or improved, and their quality of life is good. Overall, the 1- and 5-yr mortality ( or transplantation) rates for patients with chronic symptomatic CHF were 25% ( 2 of 8 patients) and 37.5% ( 3 of 8 patients), respectively. In conclusion, less than 3% of acromegalic patients developed CHF in this study. Although effective treatment of acromegaly improved short-term cardiovascular status, its impact on long-term survival is questionable. [less ▲]

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See detailLong-term outcome of patients with heart failure and dynamic functional mitral regurgitation
Lancellotti, Patrizio ULiege; Gerard, P. G.; Ancion, A. et al

in European Heart Journal (2005, September), 26(Suppl. 1), 539

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See detailLong-term outcome of patients with heart failure and dynamic functional mitral regurgitation.
LANCELLOTTI, Patrizio ULiege; Gérard, Paul ULiege; Pierard, Luc ULiege

in European Heart Journal (2005), 26(15), 1528-32

AIMS: In patients with heart failure and chronic ischaemic mitral regurgitation (MR), the mortality risk is related to the quantified severity of MR at rest and its dynamic changes during exercise. The ... [more ▼]

AIMS: In patients with heart failure and chronic ischaemic mitral regurgitation (MR), the mortality risk is related to the quantified severity of MR at rest and its dynamic changes during exercise. The impact of dynamic MR on long-term mortality, hospital admission for heart failure, and major adverse cardiac events has never been investigated. METHODS AND RESULTS: We prospectively studied 161 patients with chronic ischaemic left ventricular (LV) dysfunction and at least mild MR who underwent quantitative measurement of the effective regurgitant orifice (ERO) of MR at rest and during semi-supine exercise test and who were followed up for 35+/-11 months. The 20 patients who underwent surgery were censored at the time of operation. Of the 141 patients who were treated medically, 23 died, 22 required hospitalization for heart failure, 4 had nonfatal myocardial infarction, and 11 developed unstable angina. By multivariate analysis, an exercise-induced increase in ERO by > or =13 mm(2) and a greater increase in transtricuspid pressure gradient during exercise emerged as predictors of mortality and of hospital admission for heart failure. MR severity under basal conditions (ERO> or =20 mm(2)) was an independent predictor of only cardiac death. Greater LV volumes at rest and lack of contractile reserve during exercise were additional independent markers of poor outcome. CONCLUSION: In patients with ischaemic heart disease and LV dysfunction, large exercise-induced increases in MR identify patients at high risk of morbidity and of death. [less ▲]

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