Anesthetic management in a child with Glycogen Storage Disease IV.

De Armendi A; Patel V; Mayhew JF

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Anesthetic management in a child with Glycogen Storage Disease IV.

机译：糖原贮积病患儿的麻醉管理IV。

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We would like to share our experience anesthetizing a child with glycogen storage disease type 9. There are a total of nine types of this particular glycogen storage disease reported in the pediatric literature all of which involve a defect in the synthesis and or breakdown of glycogen within muscle and liver cells (1). Type 9 is as a result of a deficiency of phosphorylase kinase. The most common form is an X-linked liver phosphorylase kinase deficiency (1). Ketosis occurs with fasting associated with mild hypoglycemia. This particular type of GSD can improve as the child matures and prolong fasting may occur without hypoglycemia and ketosis (2).

机译：我们想分享一下麻醉9型糖原贮积病患儿的经验。儿科文献报道了总共9种类型的特殊糖原贮积病，其中涉及糖原合成和/或分解的缺陷。肌肉和肝细胞（1）。 9型是磷酸酶激酶缺乏的结果。最常见的形式是X连锁肝磷酸化酶激酶缺乏症（1）。空腹合并轻度低血糖会发生酮症。这种特殊类型的GSD可以随着孩子的成长而改善，并且可以在没有低血糖和酮病的情况下延长禁食（2）。

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《Paediatric anaesthesia》 |2010年第5期|共1页
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De Armendi A; Patel V; Mayhew JF;
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1. Anesthetic management in a child with Glycogen Storage Disease IV. [J] . De Armendi A, Patel V, Mayhew JF Paediatric anaesthesia . 2010,第5期

机译：糖原贮积病患儿的麻醉管理IV。
2. Glycogen-branching enzyme deficiency leads to abnormal cardiac development: novel insights into glycogen storage disease IV. [J] . Lee YC, Chang CJ, Bali D, Human Molecular Genetics . 2011,第3期

机译：糖原分支酶缺乏症会导致心脏异常发育：对糖原贮积病的新见解IV。
3. Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European Study on Glycogen Storage Disease Type I (ESGSD I). [J] . Rake JP, Visser G, Labrune P, European journal of pediatrics . 2002,第Suppla1期

机译：糖原贮积病I型：诊断，管理，临床过程和结果。欧洲糖原贮积病I型研究（ESGSD I）的结果。
4. GLYCOGEN STORAGE DISEASE IN A MEXICAN BABY GIRL.ULTRAESTRUCTURAL AND LIGHT MICROSCOPY FINDINGS [C] . Marco A. Ponce-Camacho, Juan Pablo Flores-Gutierrez, MC EnriqueRamirez-Bon, Microscopy Society of America annual meeting;Microscopy Society of Canada annual meeting;Societe de Microscopie du Canada annual meeting;Microbeam Analysis Society annual meeting;International Metallographic Society annual meeting . 2002

机译：墨西哥婴儿女孩的糖原贮积病超微结构和光学显微镜检查结果
5. Transition to self-management: Perspectives and experiences of glycogen storage disease type I and type III patients and their parents. [D] . King, Kellie L. 2009

机译：过渡到自我管理：I型和III型糖原贮积病患者及其父母的观点和经验。
6. Anesthetic Management for an Adult With Glycogen Storage Disease Type 0 [O] . Airi Sakamizu, Erika Yaguchi, Shinsuke Hamaguchi 2020

机译：具有糖原储存疾病的成年人的麻醉管理0
7. Glycogen storage disease type I:clinical, biochemical and genetic aspects, and implications for treatment and follow-up (management of glycogen storage disease type I) [O] . Rake Jan Peter 2003

机译：I型糖原贮积病：临床，生化和遗传方面及其对治疗和随访的意义（I型糖原贮积病的管理）

Anesthetic management in a child with Glycogen Storage Disease IV.

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