Search Results - "Wens, C."

Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI by Mogalle, Katja, Perez-Rovira, Adria, Ciet, Pierluigi, Wens, Stephan C A, van Doorn, Pieter A, Tiddens, Harm A W M, van der Ploeg, Ans T, de Bruijne, Marleen

“…Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and…”
Get full text

Quality of life and participation in daily life of adults with Pompe disease receiving enzyme replacement therapy: 10 years of international follow-up by Güngör, Deniz, Kruijshaar, Michelle E., Plug, Iris, Rizopoulos, Dimitris, Kanters, Tim A., Wens, Stephan C. A., Reuser, Arnold J. J., van Doorn, Pieter A., van der Ploeg, Ans T.

“…Summary Background Pompe disease is an inheritable metabolic disorder for which enzyme replacement therapy (ERT) has been available since 2006. Effects of ERT…”
Get full text

Perioperative outcomes of revisional laparoscopic gastric bypass after failed adjustable gastric banding and after vertical banded gastroplasty: experience with 107 cases and subgroup analysis by Apers, J. A., Wens, C., van Vlodrop, V., Michiels, M., Ceulemans, R., van Daele, G., Jacobs, I.

“…Background A growing number of revision procedures are to be expected in bariatric surgery after failed restrictive procedures such as failed adjustable…”
Get full text

Lung MRI and impairment of diaphragmatic function in Pompe disease by Wens, Stephan C A, Ciet, Pierluigi, Perez-Rovira, Adria, Logie, Karla, Salamon, Elizabeth, Wielopolski, Piotr, de Bruijne, Marleen, Kruijshaar, Michelle E, Tiddens, Harm A W M, van Doorn, Pieter A, van der Ploeg, Ans T

“…Pompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine…”
Get full text

Safety and efficacy of exercise training in adults with Pompe disease: evalution of endurance, muscle strength and core stability before and after a 12 week training program by van den Berg, Linda E M, Favejee, Marein M, Wens, Stephan C A, Kruijshaar, Michelle E, Praet, Stephan F E, Reuser, Arnold J J, Bussmann, Johannes B J, van Doorn, Pieter A, van der Ploeg, Ans T

“…Pompe disease is a proximal myopathy. We investigated whether exercise training is a safe and useful adjuvant therapy for adult Pompe patients, receiving…”
Get full text

Phenotypical variation within 22 families with Pompe disease by Wens, Stephan C A, van Gelder, Carin M, Kruijshaar, Michelle E, de Vries, Juna M, van der Beek, Nadine A M E, Reuser, Arnold J J, van Doorn, Pieter A, van der Ploeg, Ans T, Brusse, Esther

“…Pompe disease has a broad clinical spectrum, in which the phenotype is partially explained by the genotype. The aim of this study was to describe phenotypical…”
Get full text

Increased aortic stiffness and blood pressure in non-classic Pompe disease by Wens, Stephan C. A., Kuperus, Esther, Mattace-Raso, Francesco U. S., Kruijshaar, Michelle E., Brusse, Esther, van Montfort, Kees C. A. G. M., de Boer, Marjan Scheltens, Sijbrands, Eric J. G., van der Ploeg, Ans T., van Doorn, Pieter A.

“…Vascular abnormalities and glycogen accumulation in vascular smooth muscle fibres have been described in Pompe disease. Using carotid-femoral pulse wave…”
Get full text

Long-term benefit of enzyme replacement therapy in Pompe disease: A 5-year prospective study by Kuperus, Esther, Kruijshaar, Michelle E, Wens, Stephan C.A, de Vries, Juna M, Favejee, Marein M, van der Meijden, Jan C, Rizopoulos, Dimitris, Brusse, Esther, van Doorn, Pieter A, van der Ploeg, Ans T, van der Beek, Nadine A.M.E

“…OBJECTIVE:To determine the effect of enzyme replacement therapy (ERT) after 5 years and to identify predictors for a favorable response because few data are…”
Get full text

Remarkably low fibroblast acid α-glucosidase activity in three adults with Pompe disease by Wens, Stephan C.A., Kroos, Marian A., de Vries, Juna M., Hoogeveen-Westerveld, Marianne, Wijgerde, Mark G.J.M., van Doorn, Pieter A., van der Ploeg, Ans T., Reuser, Arnold J.J.

“…Most adults with Pompe disease are compound heterozygotes in which one acid α-glucosidase (GAA) allele harbors the c.-32-13T>G mutation, causing partial loss…”
Get full text

Elevated Plasma Cardiac Troponin T Levels Caused by Skeletal Muscle Damage in Pompe Disease by Wens, Stephan C.A, Schaaf, Gerben J, Michels, Michelle, Kruijshaar, Michelle E, van Gestel, Tom J.M, in ‘t Groen, Stijn, Pijnenburg, Joon, Dekkers, Dick H.W, Demmers, Jeroen A.A, Verdijk, Lex B, Brusse, Esther, van Schaik, Ron H.N, van der Ploeg, Ans T, van Doorn, Pieter A, Pijnappel, W.W.M Pim

“…BACKGROUND—Elevated plasma cardiac troponin T (cTnT) levels in patients with neuromuscular disorders may erroneously lead to the diagnosis of acute myocardial…”
Get full text

Pompe disease in adulthood: effects of antibody formation on enzyme replacement therapy by de Vries, Juna M., Kuperus, Esther, Hoogeveen-Westerveld, Marianne, Kroos, Marian A., Wens, Stephan C.A., Stok, Merel, van der Beek, Nadine A.M.E., Kruijshaar, Michelle E., Rizopoulos, Dimitris, van Doorn, Pieter A., van der Ploeg, Ans T., Pijnappel, W.W.M. Pim

“…To determine the effect of antibodies against recombinant human acid α-glucosidase (rhGAA) on treatment efficacy and safety, and to test whether the GAA…”
Get full text

Exercise Training in Adults With Pompe Disease: The Effects on Pain, Fatigue, and Functioning by Favejee, Marein M., PT, MSc, van den Berg, Linda E.M., MD, Kruijshaar, Michelle E., PhD, Wens, Stephan C.A., MD, Praet, Stephan F.E., MD, Pim Pijnappel, W.W.M., PhD, van Doorn, Pieter A., MD, Bussmann, Johannes B.J., PhD, van der Ploeg, Ans T., MD, PhD

“…Abstract Objective To assess if a 12-week exercise intervention to improve aerobic fitness, muscle strength, and core stability also had an impact on fatigue,…”
Get full text

Response to Herbert et al by de Vries, Juna M, Kuperus, Esther, Hoogeveen-Westerveld, Marianne, Kroos, Marian A, Wens, Stephan C A, Stok, Merel, van der Beek, Nadine A M E, Kruijshaar, Michelle E, Rizopoulos, Dimitris, van Doorn, Pieter A, van der Ploeg, Ans T, Pijnappel, W W M Pim

Get full text