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Glycogen Storage Disease in Pediatric Population

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Last updated: 24 Dec 2024

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Abstract

Introduction: The pathway of glycogen metabolism is regulated by many hormones such as insulin, glucagon, and corticosteroids. Glycogen storage diseases (GSD) most commonly affect muscles, liver, or both and occur in each 20000 to 43000 live birth. They are classified into 12 subtypes, but types I, II, and IX are the most common.  Methodology: We conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE, from January 2001, through February 2017. The following search terms were used: glycogen storage diseases, Von Gierke disease, Pompe's disease, Cori Disease, Forbes disease, Andersen's disease, McArdle disease, neonatal hypoglycemia, neonatal hepatomegaly. Aim: In this review, we aim to study the genetic basis, diagnosis, presentation, and different management approach to various common types of glycogen storage diseases prevalent in pediatric population. Conclusion:  There are no cures for any type of glycogen storage diseases presently. Most treatments are designed to control signs and symptoms. The overall goals are primarily avoiding hypoglycemia, hyperlactatemia, hyperuricemia, and hyperlipidemia. Liver transplantation should be deliberated for patients with GSD type IV and for other progressive hepatic types of GSDs in order to avoid hepatic failure or malignancy. More research must be carried out to develop newer and more effective ways of management.

Keywords

neonatal hypoglycemia, glycogen storage diseases, Von Gierke disease, Pompe's disease, Cori Disease, Forbes disease, Andersen's disease, McArdle disease

Authors

First Name

Yara Mofarih

Last Name

Assiri

MiddleName

-

Affiliation

King Khalid University

Email

yara.mm.aa@gmail.com

City

-

Orcid

-

First Name

Marium Mohammed

Last Name

Iqbal

MiddleName

-

Affiliation

Batterjee Medical College

Email

-

City

-

Orcid

-

First Name

Reem Ali

Last Name

Almanie

MiddleName

-

Affiliation

King Khalid University

Email

-

City

-

Orcid

-

First Name

Atheer Eed

Last Name

Alotaibi

MiddleName

-

Affiliation

King Khalid University

Email

-

City

-

Orcid

-

First Name

Faris Abdullah S

Last Name

Alharbi

MiddleName

-

Affiliation

University of Gezira

Email

-

City

-

Orcid

-

First Name

Bayan Sultan

Last Name

Al Jobran

MiddleName

-

Affiliation

King Khalid University

Email

bayansu93@hotmail.com

City

-

Orcid

-

First Name

Huda F.

Last Name

Abbag

MiddleName

-

Affiliation

King Khalid University

Email

-

City

-

Orcid

-

First Name

Mohammed Younes

Last Name

Almoghrabi

MiddleName

-

Affiliation

Batterjee Medical College

Email

-

City

-

Orcid

-

First Name

Fahad Abdulaziz

Last Name

Jarad

MiddleName

-

Affiliation

King Khalid University

Email

-

City

-

Orcid

-

First Name

Ashwaq Y.

Last Name

Asiri

MiddleName

-

Affiliation

King Khalid University

Email

-

City

-

Orcid

-

First Name

Mohammad Abdullah

Last Name

Alotaibi

MiddleName

-

Affiliation

Imam Muhammad Bin Saud Islamic University

Email

-

City

-

Orcid

-

First Name

Wejdan Hamed

Last Name

Alshammari

MiddleName

-

Affiliation

Hail University

Email

-

City

-

Orcid

-

First Name

Hadil Mohammed

Last Name

Alenezi

MiddleName

-

Affiliation

Northern Border University

Email

-

City

-

Orcid

-

First Name

Badoor Abdrabalameer

Last Name

Alghafli

MiddleName

-

Affiliation

Arabian Gulf University

Email

-

City

-

Orcid

-

First Name

Saad Mahmoud

Last Name

Tobaigi

MiddleName

-

Affiliation

Batterjee Medical College

Email

-

City

-

Orcid

-

First Name

Amro Sulaiman

Last Name

AlJuhani

MiddleName

-

Affiliation

King Abdulaziz University

Email

-

City

-

Orcid

-

First Name

Maram Mohammed

Last Name

Bajoaifur

MiddleName

-

Affiliation

Ibn Sina National College

Email

-

City

-

Orcid

-

First Name

Emtenan Al

Last Name

Ansari

MiddleName

-

Affiliation

Ibn Sina National College

Email

-

City

-

Orcid

-

Volume

70

Article Issue

9

Related Issue

2036

Issue Date

2018-01-01

Receive Date

2018-08-08

Publish Date

2018-01-01

Page Start

1,539

Page End

1,543

Print ISSN

1687-2002

Online ISSN

2090-7125

Article File

EJHM_Volume 70_Issue 9_Pages 1539-1543.pdf

PDF . 245.7kB

Link

https://ejhm.journals.ekb.eg/article_10512.html

Detail API

https://ejhm.journals.ekb.eg/service?article_code=10512

Order

18

Type

Original Article

Type Code

606

Publication Type

Journal

Publication Title

The Egyptian Journal of Hospital Medicine

Publication Link

https://ejhm.journals.ekb.eg/

MainTitle

Glycogen Storage Disease in Pediatric Population

Details

Type

Article

Created At

22 Jan 2023