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231224s2016 xx |||||o 00| ||chi c |
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|a 10.3760/cma.j.issn.0578-1310.2016.11.008
|2 doi
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|e rakwb
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|a chi
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| 100 |
1 |
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|a Xu, L L
|e verfasserin
|4 aut
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|a Clinical observation on human alpha glucosidase in treatment of five patients with glycogen storage disease Ⅱ
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|c 2016
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
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|a Date Completed 15.05.2017
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|a Date Revised 02.12.2018
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|a published: Print
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|a Citation Status MEDLINE
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|a Objective: To evaluate the effect of enzyme replacement therapy (ERT) on glycogen storage disease typeⅡ(GSDⅡ). Method: The clinical data of three juvenile onset and two infant onset GSDⅡpatients were collected from First Affiliated Hospital of Sun Yat-sen University in October 2015 to July 2016.Patient 1 was female, the age of onset was 15 months. Patient 2 was male, the age of onset was 20 months. Patient 3 was female, the sister of patient 2, the age of onset was 47 months. Patient 4 was male, the age of onset was 5 months. Patient 5 was male, the age of onset was 1 month.The age at the start of ERT of the 5 patients was 32, 31, 56, 34, and 3 months respectively and the duration of ERT was 19, 9, 4, 5, 5 doses respectively.ERT was administered at 20 mg/kg every 2 weeks.Dexamethasone was regularly given before each infusion. Result: ERT was well tolerated, only one time, Patient 1 developed tachycardia and hypertension without using dexamethasone.Patient 2 underwent successfully ventilator weaning.Patient 1 underwent a tracheotomy, also needed mechanical ventilation treatment.Patient 4 noninvasive ventilation was tried. Conclusion: Recombinant human alpha-glucosidase treatment was effective and well tolerated in patients with GSDⅡ
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|a Case Reports
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|a Journal Article
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|a alpha-Glucosidases
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|2 NLM
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|a Zhang, L D
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|a Liang, Y J
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|a Tang, W
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|a Huang, X Q
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|a Pei, Y X
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|a Cheng, Y C
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|a Huang, H M
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|a Zhang, C
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|t Zhonghua er ke za zhi = Chinese journal of pediatrics
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|g 54(2016), 11 vom: 02. Nov., Seite 829-833
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