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10.1007/s00415-021-10475-z http://scihub22266oqcxt.onion/10.1007/s00415-021-10475-z 33625582!7903209!33625582     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       J+Neurol 2021 ; 268 (8): 2943-2950 Nephropedia Template TP {{tp|p=33625582|t=2021. The impact of interrupting enzyme replacement therapy in late-onset Pompe disease |pdf=|usr=}}{{33625582}} gab.com Text The impact of interrupting enzyme replacement therapy in late-onset Pompe disease JO: J Neurol http://www.kidney.de/pget.php?&tw=1&pmid=33625582 #FOAvip BACKGROUND: Late-onset Pompe disease (LOPD) is a rare autosomal recessive disorder caused by mutations in the GAA gene, leading to progressive weakness of locomotor and respiratory muscles. Enzyme replacement therapy (ERT), administered every second week, has been proven to slow down disease progression and stabilize pulmonary function. Due to the COVID-19 pandemic in Germany, ERT was interrupted at our centre for 29 days. As reports on ERT discontinuation in LOPD are rare, our study aimed to analyse the impact of ERT interruption on the change in clinical outcome. METHODS: We performed a prospective cohort study in 12 LOPD patients. Clinical assessments were performed after ERT interruption and after the next three consecutive infusions. We assessed motor function by muscle strength testing, a 6-minute-walk-test, pulmonary function tests, and adverse events. For statistical analysis, an estimated baseline was calculated based on the individual yearly decline. RESULTS: The mean time of ERT interruption was 49.42 days (SD +/- 12.54). During ERT interruption, seven patients reported 14 adverse events and two of them were severe. Frequent symptoms were reduced muscle endurance/increased muscle fatigability and shortness of breath/worsening of breathing impairment. After ERT interruption, significant deterioration was found for MIP(%pred) (p = 0.026) and MRC(%pred), as well as a trend to clinical deterioration in FVC(%pred) and the 6MWT(%pred). CONCLUSION: Interruption of ERT was associated with a deterioration in the core clinical outcome measures. Therefore, an interruption of ERT should be kept as short as possible. Twit Text FOAVip The impact of interrupting enzyme replacement therapy in late-onset Pompe disease ????J Neurol http://www.kidney.de/pget.php?&tw=1&pmid=33625582 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=33625582 #FOAvip English Wikipedia <ref>{{Cite pmid|33625582}}</ref> The impact of interrupting enzyme replacement therapy in late-onset Pompe disease #MMPMID33625582 Wenninger S; Gutschmidt K; Wirner C; Einvag K; Montagnese F; Schoser B J Neurol 2021[Aug]; 268 (8): 2943-2950 PMID33625582show ga BACKGROUND: Late-onset Pompe disease (LOPD) is a rare autosomal recessive disorder caused by mutations in the GAA gene, leading to progressive weakness of locomotor and respiratory muscles. Enzyme replacement therapy (ERT), administered every second week, has been proven to slow down disease progression and stabilize pulmonary function. Due to the COVID-19 pandemic in Germany, ERT was interrupted at our centre for 29 days. As reports on ERT discontinuation in LOPD are rare, our study aimed to analyse the impact of ERT interruption on the change in clinical outcome. METHODS: We performed a prospective cohort study in 12 LOPD patients. Clinical assessments were performed after ERT interruption and after the next three consecutive infusions. We assessed motor function by muscle strength testing, a 6-minute-walk-test, pulmonary function tests, and adverse events. For statistical analysis, an estimated baseline was calculated based on the individual yearly decline. RESULTS: The mean time of ERT interruption was 49.42 days (SD +/- 12.54). During ERT interruption, seven patients reported 14 adverse events and two of them were severe. Frequent symptoms were reduced muscle endurance/increased muscle fatigability and shortness of breath/worsening of breathing impairment. After ERT interruption, significant deterioration was found for MIP(%pred) (p = 0.026) and MRC(%pred), as well as a trend to clinical deterioration in FVC(%pred) and the 6MWT(%pred). CONCLUSION: Interruption of ERT was associated with a deterioration in the core clinical outcome measures. Therefore, an interruption of ERT should be kept as short as possible. |*COVID-19[MESH] |*Glycogen Storage Disease Type II/drug therapy[MESH] |Enzyme Replacement Therapy[MESH] |Germany[MESH] |Humans[MESH] |Pandemics[MESH] |Prospective Studies[MESH] |SARS-CoV-2[MESH]The impact of interrupting enzyme replacement therapy in late-onset Po(epmc).pdf DeepDyve Pubget Overpricing