Glycogen Storage Disease in Pediatric Population
Assiri, Y., Iqbal, M., Almanie, R., Alotaibi, A., Alharbi, F., Al Jobran, B., Abbag, H., Almoghrabi, M., Jarad, F., Asiri, A., Alotaibi, M., Alshammari, W., Alenezi, H., Alghafli, B., Tobaigi, S., AlJuhani, A., Bajoaifur, M., Ansari, E. (2018). Glycogen Storage Disease in Pediatric Population. EKB Journal Management System, 70(9), 1539-1543.
Yara Mofarih Assiri; Marium Mohammed Iqbal; Reem Ali Almanie; Atheer Eed Alotaibi; Faris Abdullah S Alharbi; Bayan Sultan Al Jobran; Huda F. Abbag; Mohammed Younes Almoghrabi; Fahad Abdulaziz Jarad; Ashwaq Y. Asiri; Mohammad Abdullah Alotaibi; Wejdan Hamed Alshammari; Hadil Mohammed Alenezi; Badoor Abdrabalameer Alghafli; Saad Mahmoud Tobaigi; Amro Sulaiman AlJuhani; Maram Mohammed Bajoaifur; Emtenan Al Ansari. "Glycogen Storage Disease in Pediatric Population". EKB Journal Management System, 70, 9, 2018, 1539-1543.
Assiri, Y., Iqbal, M., Almanie, R., Alotaibi, A., Alharbi, F., Al Jobran, B., Abbag, H., Almoghrabi, M., Jarad, F., Asiri, A., Alotaibi, M., Alshammari, W., Alenezi, H., Alghafli, B., Tobaigi, S., AlJuhani, A., Bajoaifur, M., Ansari, E. (2018). 'Glycogen Storage Disease in Pediatric Population', EKB Journal Management System, 70(9), pp. 1539-1543.
Assiri, Y., Iqbal, M., Almanie, R., Alotaibi, A., Alharbi, F., Al Jobran, B., Abbag, H., Almoghrabi, M., Jarad, F., Asiri, A., Alotaibi, M., Alshammari, W., Alenezi, H., Alghafli, B., Tobaigi, S., AlJuhani, A., Bajoaifur, M., Ansari, E. Glycogen Storage Disease in Pediatric Population. EKB Journal Management System, 2018; 70(9): 1539-1543.

Glycogen Storage Disease in Pediatric Population

The Egyptian Journal of Hospital Medicine
Article 18, Volume 70, Issue 9, January 2018, Page 1539-1543  XML PDF (239.9 K)
Document Type: Original Article
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Authors
Yara Mofarih Assiri email 1; Marium Mohammed Iqbal2; Reem Ali Almanie1; Atheer Eed Alotaibi1; Faris Abdullah S Alharbi3; Bayan Sultan Al Jobran1; Huda F. Abbag1; Mohammed Younes Almoghrabi2; Fahad Abdulaziz Jarad1; Ashwaq Y. Asiri1; Mohammad Abdullah Alotaibi4; Wejdan Hamed Alshammari5; Hadil Mohammed Alenezi6; Badoor Abdrabalameer Alghafli7; Saad Mahmoud Tobaigi2; Amro Sulaiman AlJuhani8; Maram Mohammed Bajoaifur9; Emtenan Al Ansari9
1King Khalid University
2Batterjee Medical College
3University of Gezira
4Imam Muhammad Bin Saud Islamic University
5Hail University
6Northern Border University
7Arabian Gulf University
8King Abdulaziz University
9Ibn Sina National College
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
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