Literature DB >> 18930676

Improvement with ongoing Enzyme Replacement Therapy in advanced late-onset Pompe disease: a case study.

Laura E Case1, Dwight D Koeberl, Sarah P Young, Deeksha Bali, Stephanie M DeArmey, Joanne Mackey, Priya S Kishnani.   

Abstract

Benefits of enzyme replacement therapy with Myozyme (alglucosidase alfa), anecdotally reported in late-onset Pompe disease, range from motor and pulmonary improvement in less severely affected patients, to stabilization with minimal improvement in those with advanced disease. We report a case of a 63-year-old patient with significant morbidity who made notable motor and pulmonary function gains after two years on therapy. Thus, improvements in those with advanced disease may be possible after long-term treatment.

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Year:  2008        PMID: 18930676     DOI: 10.1016/j.ymgme.2008.09.001

Source DB:  PubMed          Journal:  Mol Genet Metab        ISSN: 1096-7192            Impact factor:   4.797


  12 in total

1.  36 months observational clinical study of 38 adult Pompe disease patients under alglucosidase alfa enzyme replacement therapy.

Authors:  Caroline Regnery; Cornelia Kornblum; Frank Hanisch; Stefan Vielhaber; Nicola Strigl-Pill; Birgit Grunert; Wolfgang Müller-Felber; Franz Xaver Glocker; Matthias Spranger; Marcus Deschauer; Eugen Mengel; Benedikt Schoser
Journal:  J Inherit Metab Dis       Date:  2012-01-31       Impact factor: 4.982

2.  Enzyme replacement therapy with alglucosidase alfa in 44 patients with late-onset glycogen storage disease type 2: 12-month results of an observational clinical trial.

Authors:  S Strothotte; N Strigl-Pill; B Grunert; C Kornblum; K Eger; C Wessig; M Deschauer; F Breunig; F X Glocker; S Vielhaber; A Brejova; M Hilz; K Reiners; W Müller-Felber; E Mengel; M Spranger; Benedikt Schoser
Journal:  J Neurol       Date:  2009-08-01       Impact factor: 4.849

3.  Effects of enzyme replacement therapy on five patients with advanced late-onset glycogen storage disease type II: a 2-year follow-up study.

Authors:  Yoshihiko Furusawa; Madoka Mori-Yoshimura; Toshiyuki Yamamoto; Chikako Sakamoto; Mizuki Wakita; Yoko Kobayashi; Yutaka Fukumoto; Yasushi Oya; Tokiko Fukuda; Hideo Sugie; Yukiko K Hayashi; Ichizo Nishino; Ikuya Nonaka; Miho Murata
Journal:  J Inherit Metab Dis       Date:  2011-10-07       Impact factor: 4.982

4.  Pathogenesis of growth failure and partial reversal with gene therapy in murine and canine Glycogen Storage Disease type Ia.

Authors:  Elizabeth Drake Brooks; Dianne Little; Ramamani Arumugam; Baodong Sun; Sarah Curtis; Amanda Demaster; Michael Maranzano; Mark W Jackson; Priya Kishnani; Michael S Freemark; Dwight D Koeberl
Journal:  Mol Genet Metab       Date:  2013-04-06       Impact factor: 4.797

5.  Hypertrophic cardiomyopathy in pompe disease is not limited to the classic infantile-onset phenotype.

Authors:  Dong-Hwan Lee; Wen-Juan Qiu; Jeongho Lee; Yin-Hsiu Chien; Wuh-Liang Hwu
Journal:  JIMD Rep       Date:  2014-09-12

6.  Cardiovascular abnormalities in late-onset Pompe disease and response to enzyme replacement therapy.

Authors:  Daniel Forsha; Jennifer S Li; P Brian Smith; Ans T van der Ploeg; Priya Kishnani; Sara K Pasquali
Journal:  Genet Med       Date:  2011-07       Impact factor: 8.822

7.  Targeted Therapies for Metabolic Myopathies Related to Glycogen Storage and Lipid Metabolism: a Systematic Review and Steps Towards a 'Treatabolome'.

Authors:  A Manta; S Spendiff; H Lochmüller; R Thompson
Journal:  J Neuromuscul Dis       Date:  2021

8.  The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patients.

Authors:  Erin J Feeney; Stephanie Austin; Yin-Hsiu Chien; Hanna Mandel; Benedikt Schoser; Sean Prater; Wuh-Liang Hwu; Evelyn Ralston; Priya S Kishnani; Nina Raben
Journal:  Acta Neuropathol Commun       Date:  2014-01-02       Impact factor: 7.801

9.  Standardized nursing management of enzyme replacement therapy for late-onset Pompe disease.

Authors:  Shan Tang; Jiachu Ma; Huaxing Meng; Junhong Guo; Shuyan Cao; Binquan Wang
Journal:  Medicine (Baltimore)       Date:  2021-01-22       Impact factor: 1.889

10.  Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy.

Authors:  Mauro Marzorati; Simone Porcelli; Giuseppe Bellistri; Lucia Morandi; Bruno Grassi
Journal:  Neuromuscul Disord       Date:  2012-12       Impact factor: 4.296

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