Sections

Sections Glycogen Storage Diseases Types I-VII
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Medication

Medication Summary

No specific drug treatment is recommended for GSD type Ia. Appropriately treat concurrent infections with antibiotics. Allopurinol (Zyloprim), a xanthine oxidase inhibitor, therapy can reduce uric acid levels in the blood and prevent occurrence of gout and kidney stones in adult life.

Hyperlipidemia can be reduced by lipid-lowering drugs (eg, 3-hydroxy-3-methylglutaryl coenzyme A [HMG-CoA] reductase inhibitors, fibric acid derivatives). HMG-CoA reductase inhibitors of cholesterol biosynthesis in the liver are known as statins. Because of a high risk of myositis, the drugs may be recommended only after age 12 years. A new inhibitor of cholesterol absorption, ezetimibe, in a dose of 10 mg/d, can reduce low-density lipoprotein (LDL) cholesterol levels and has small triglyceride-lowering effects.

In patients with renal lesions, microalbuminuria can be reduced with angiotensin-converting enzyme (ACE) inhibitor therapy. In addition to their antihypertensive effects, ACE inhibitors are renoprotective and reduce albuminuria. Nephrocalcinosis and renal calculi can be prevented with citrate therapy.

Severe infection and chronic inflammatory bowel disease in patients with GSD type Ib should be treated with antibiotics and granulocyte-macrophage colony-stimulating factors (Neupogen).

Cardioglycosides and diuretics are prescribed for cardiovascular insufficiency in patients with GSD type II. Respiratory bacterial infections (aspiration pneumonia) in patients with GSD type II are treated with antibiotics. Enzyme replacement therapy has been approved as an orphan drug by the FDA.

No effective treatment is available for GSD types III, IV, V, and VII. Some patients with GSD type V may benefit from creatine supplement.

Class Summary

These agents correct iron deficiency.

Ferrous sulfate (Feosol, Feratab, Fer-Iron)

View full drug information

Ferrous sulfate is used to control anemia in patients with GSD types Ia or Ib.

Class Summary

These agents reduce production of uric acid without disrupting the biosynthesis of vital purines.

Allopurinol (Zyloprim)

View full drug information

Allopurinol is used to control elevated serum uric acid levels in patients with GSD types Ia or Ib.

Class Summary

These agents activate and stimulate production, maturation, migration, and cytotoxicity of neutrophils.

Filgrastim (Neupogen)

View full drug information

Filgrastim is a granulocyte colony-stimulating factor used in patients with GSD type Ib with severe infections, in those with pseudocolitis, and in patients as a preventive measure.

Class Summary

These agents elevate blood glucose levels.

Glucagon (GlucaGen)

View full drug information

Glucagon is used to treat GSD types V and VII. Pancreatic alpha cells of the islets of Langerhans produce glucagon, a polypeptide hormone. It exerts opposite effects of insulin on blood glucose. Glucagon elevates blood glucose levels by inhibiting glycogen synthesis and by enhancing the formation of glucose from noncarbohydrate sources such as proteins and fats (gluconeogenesis). It increases the hydrolysis of glycogen to glucose (glycogenolysis) in the liver in addition to accelerating hepatic glycogenolysis and lipolysis in adipose tissue. Glucagon also increases the force of contraction in the heart and has a relaxant effect on the GI tract.

Class Summary

These agents reduce microalbuminuria, have antihypertensive effects, and are renoprotective.

Lisinopril (Prinivil, Zestril)

View full drug information

Lisinopril prevents the conversion of angiotensin I to angiotensin II and lowers aldosterone secretion; in some patients, it may cause cough and angioedema.

Ramipril (Altace)

View full drug information

Ramipril prevents the conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

Captopril (Capoten)

View full drug information

Captopril prevents the conversion of angiotensin I to angiotensin II, resulting in lower aldosterone secretion.

Enalapril (Vasotec)

View full drug information

Enalapril is a competitive inhibitor of ACE. It reduces angiotensin II levels, decreasing aldosterone secretion.

Class Summary

Recombinant human enzyme glucosidase alfa has been designated an orphan drug for GSD type II (Pompe disease).

Alglucosidase alfa (Myozyme)

View full drug information

Alglucosidase alfa is a recombinant human enzyme alpha-glucosidase (rhGAA) indicated as an orphan drug for the treatment of Pompe disease. It replaces rhGAA, which is deficient or lacking in persons with Pompe disease. Alpha-glucosidase is essential for normal muscle development and function. Alglucosidase alfa binds to mannose-6-phosphate receptors and then is transported into lysosomes; it undergoes proteolytic cleavage that results in increased enzymatic activity and the ability to cleave glycogen. It improves infant survival without requiring invasive ventilatory support compared with historical controls without treatment.

von Gierke E. Hepato-nephromegalia glykogenica (Glykogenspeicherkrankheit der Leber und Nieren). Beitr Path Anat. 1929. 82:497-513.
Cori GT, Cori CF. Glucose-6-phosphatase of the liver in glycogen storage disease. J Biol Chem. 1952 Dec. 199(2):661-7. [QxMD MEDLINE Link].
Narisawa K, Igarashi Y, Otomo H, Tada K. A new variant of glycogen storage disease type I probably due to a defect in the glucose-6-phosphate transport system. Biochem Biophys Res Commun. 1978 Aug 29. 83(4):1360-4. [QxMD MEDLINE Link].
Gjorgjieva M, Raffin M, Duchampt A, et al. Progressive development of renal cysts in glycogen storage disease type I. Hum Mol Genet. 2016 Sep 1. 25 (17):3784-97. [QxMD MEDLINE Link].
Ding J, Huang Y, Yang H, Zhang Q, Hou X, Liu X, et al. [Analysis of clinical features of 6 patients with infantile type glycogen storage disease type II]. Zhonghua Er Ke Za Zhi. 2015 Jun. 53 (6):436-41. [QxMD MEDLINE Link].
Dagli A, Sentner CP, Weinstein DA, Pagon RA, Adam MP, Ardinger HH, et al. Glycogen Storage Disease Type III. March 9 2010. 1993-2017. [QxMD MEDLINE Link]. [Full Text].
Sentner CP, Hoogeveen IJ, Weinstein DA, Santer R, Murphy E, McKiernan PJ, et al. Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome. J Inherit Metab Dis. 2016 Sep. 39 (5):697-704. [QxMD MEDLINE Link].
Decostre V, Laforêt P, Nadaj-Pakleza A, De Antonio M, Leveugle S, Ollivier G, et al. Cross-sectional retrospective study of muscle function in patients with glycogen storage disease type III. Neuromuscul Disord. 2016 Sep. 26 (9):584-92. [QxMD MEDLINE Link].
Malfatti E, Barnerias C, Hedberg-Oldfors C, et al. A novel neuromuscular form of glycogen storage disease type IV with arthrogryposis, spinal stiffness and rare polyglucosan bodies in muscle. Neuromuscul Disord. 2016 Oct. 26 (10):681-7. [QxMD MEDLINE Link].
McArdle B. Myopathy due to a defect in muscle glycogen breakdown. Clin Sci. 1951. 10:13-33.
Nogales-Gadea G, Godfrey R, Santalla A, Coll-Cantí J, Pintos-Morell G, Pinós T, et al. Genes and exercise intolerance: insights from McArdle disease. Physiol Genomics. 2016 Feb. 48 (2):93-100. [QxMD MEDLINE Link].
Grünert SC, Hannibal L, Spiekerkoetter U. The Phenotypic and Genetic Spectrum of Glycogen Storage Disease Type VI. Genes (Basel). 2021 Aug 3. 12 (8):[QxMD MEDLINE Link]. [Full Text].
Hers HG. alpha-Glucosidase deficiency in generalized glycogenstorage disease (Pompe's disease). Biochem J. 1963 Jan. 86:11-6. [QxMD MEDLINE Link].
Tauri S, Okuno G, Ikura Y, Tanaka T, Suda M, Nishikawa M. Phosphofructokinase deficiency in skeletal muscle. A new type of glycogenosis. Biochem Biophys Res Commun. 1965 May 3. 19:517-23. [QxMD MEDLINE Link].
Derks TGJ, van Rijn M. Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions. Journal of Inherited Metabolic Disease. January 30 2015. 38(3):537-543. [Full Text].
Fuller M, Duplock S, Turner C, et al. Mass spectrometric quantification of glycogen to assess primary substrate accumulation in the Pompe mouse. Anal Biochem. 2012 Feb 15. 421 (2):759-63. [QxMD MEDLINE Link].
Cupler EJ, Berger KI, Leshner RT, et al. CONSENSUS TREATMENT RECOMMENDATIONS FOR LATE-ONSET POMPE DISEASE. 2012;45(3):319-333. doi:10.1002/mus.22329. Muscle & nerve. 2012. 45(3):319-333. [QxMD MEDLINE Link].
Ozen H. Glycogen storage diseases: New perspectives. World Journal of Gastroenterology. May 14 2007. 13:2541-2553. [Full Text].
Magoulas PL, El-Hattab AW. Glycogen Storage Disease Type IV. Available at https://www.ncbi.nlm.nih.gov/books/NBK115333/. 2013; Accessed: May 15 2017.
Martín MA, Lucía A, Arenas J, Andreu AL, Pagon RA, Adam MP, et al. Glycogen Storage Disease Type V. April 19 2006. [QxMD MEDLINE Link]. [Full Text].
Krishnamoorthy N, Santosh V, Yasha TC, Mahadevan A, Shankar SK, Jethwani D, et al. Glycogen storage disease type V (Mc Ardle's disease): A report on three cases. Neurol India. 2011 Nov-Dec. 59(6):884-6. [QxMD MEDLINE Link].
Musumeci O, Bruno C, Mongini T, Rodolico C, Aguennouz M, Barca E, et al. Clinical features and new molecular findings in muscle phosphofructokinase deficiency (GSD type VII). Neuromuscul Disord. 2011 Nov 29. [QxMD MEDLINE Link].
Ellingwood SS, Cheng A. Biochemical and clinical aspects of glycogen storage diseases. J Endocrinol. 2018 Sep. 238 (3):R131-R141. [QxMD MEDLINE Link]. [Full Text].
Beyzaei Z, Geramizadeh B. Molecular diagnosis of glycogen storage disease type I: a review. EXCLI J. 2019. 18:30-46. [QxMD MEDLINE Link].
Matern D, Seydewitz HH, Bali D, Lang C, Chen YT. Glycogen storage disease type I: diagnosis and phenotype/genotype correlation. Eur J Pediatr. 2002 Oct. 161 Suppl 1:S10-9. [QxMD MEDLINE Link].
Melis D, Fulceri R, Parenti G, et al. Genotype/phenotype correlation in glycogen storage disease type 1b: a multicentre study and review of the literature. Eur J Pediatr. 2005 Aug. 164(8):501-8. [QxMD MEDLINE Link].
Kuijpers TW, Maianski NA, Tool AT, et al. Apoptotic neutrophils in the circulation of patients with glycogen storage disease type 1b (GSD1b). Blood. 2003 Jun 15. 101(12):5021-4. [QxMD MEDLINE Link].
Cheung YY, Kim SY, Yiu WH, et al. Impaired neutrophil activity and increased susceptibility to bacterial infection in mice lacking glucose-6-phosphatase-beta. J Clin Invest. 2007 Mar. 117(3):784-93. [QxMD MEDLINE Link].
Janecke AR, Bosshard NU, Mayatepek E, et al. Molecular diagnosis of type 1c glycogen storage disease. Hum Genet. 1999 Mar. 104(3):275-7. [QxMD MEDLINE Link].
Shigeto S, Katafuchi T, Okada Y, Nakamura K, Endo F, Okuyama T, et al. Improved assay for differential diagnosis between Pompe disease and acid α-glucosidase pseudodeficiency on dried blood spots. Mol Genet Metab. 2011 May. 103 (1):12-7. [QxMD MEDLINE Link].
Nogales-Gadea G, Brull A, Santalla A, Andreu AL, Arenas J, Martín MA, et al. McArdle Disease: Update of Reported Mutations and Polymorphisms in the PYGM Gene. Hum Mutat. 2015 Jul. 36 (7):669-78. [QxMD MEDLINE Link].
Dimaur S, Andreu AL, Bruno C, Hadjigeorgiou GM. Myophosphorylase deficiency (glycogenosis type V; McArdle disease). Curr Mol Med. 2002 Mar. 2(2):189-96. [QxMD MEDLINE Link].
Howard TD, Akots G, Bowden DW. Physical and genetic mapping of the muscle phosphofructokinase gene (PFKM): reassignment to human chromosome 12q. Genomics. 1996 May 15. 34(1):122-7. [QxMD MEDLINE Link].
Jacoby JT, Bento Dos Santos B, Nalin T, Colonetti K, Farret Refosco L, F M de Souza C, et al. Bone Mineral Density in Patients with Hepatic Glycogen Storage Diseases. Nutrients. 2021 Aug 27. 13 (9):2987. [QxMD MEDLINE Link]. [Full Text].
Bali DS, Chen YT, Austin S, Goldstein JL, Pagon RA, Adam MP, et al. Glycogen Storage Disease Type I. GeneReviews. August 2016. [QxMD MEDLINE Link]. [Full Text].
Chen YT. Type I glycogen storage disease: kidney involvement, pathogenesis and its treatment. Pediatr Nephrol. 1991 Jan. 5 (1):71-6. [QxMD MEDLINE Link].
Mundy HR, Lee PJ. Glycogenosis type I and diabetes mellitus: a common mechanism for renal dysfunction?. Med Hypotheses. 2002 Jul. 59(1):110-4. [QxMD MEDLINE Link].
Papadimas GK, Spengos K, Papadopoulos C, Manta P. Late Onset Glycogen Storage Disease Type II: Pitfalls in the Diagnosis. Eur Neurol. 2011 Dec 15. 67(2):65-68. [QxMD MEDLINE Link].
Visser G, Rake JP, Kokke FT, Nikkels PG, Sauer PJ, Smit GP. Intestinal function in glycogen storage disease type I. J Inherit Metab Dis. 2002 Aug. 25(4):261-7. [QxMD MEDLINE Link].
Leslie N, Bailey L. Pompe Disease. Available at https://www.ncbi.nlm.nih.gov/books/NBK1261/. 2007 Aug 31 [Updated 2017 May 11].; Accessed: May 15 2017.
Pompe Disease Diagnostic Working Group., Winchester B, Bali D, Bodamer OA, et al. Methods for a prompt and reliable laboratory diagnosis of Pompe disease: report from an international consensus meeting. Mol Genet Metab. 2008 Mar. 93 (3):275-81. [QxMD MEDLINE Link].
Kishnani PS, Steiner RD, Bali D, Berger K, Byrne BJ, Case LE, et al. Pompe disease diagnosis and management guideline. Genet Med. 2006 May. 8 (5):267-88. [QxMD MEDLINE Link].
Chien YH, Lee NC, Thurberg BL, Chiang SC, Zhang XK, Keutzer J, et al. Pompe disease in infants: improving the prognosis by newborn screening and early treatment. Pediatrics. 2009 Dec. 124 (6):e1116-25. [QxMD MEDLINE Link].
Mechtler TP, Stary S, Metz TF, De Jesús VR, Greber-Platzer S, Pollak A, et al. Neonatal screening for lysosomal storage disorders: feasibility and incidence from a nationwide study in Austria. Lancet. 2012 Jan 28. 379 (9813):335-41. [QxMD MEDLINE Link].
Chien YH, Chiang SC, Zhang XK, Keutzer J, Lee NC, Huang AC, et al. Early detection of Pompe disease by newborn screening is feasible: results from the Taiwan screening program. Pediatrics. 2008 Jul. 122 (1):e39-45. [QxMD MEDLINE Link].
Martín MA, Lucía A, Arenas J, Andreu AL, Pagon RA, Adam MP, et al. Glycogen Storage Disease Type V. 1993. [QxMD MEDLINE Link]. [Full Text].
Kishnani PS, Austin SL, Abdenur JE, Arn P, Bali DS, Boney A, et al. Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics. Genet Med. 2014 Nov. 16 (11):e1. [QxMD MEDLINE Link].
Degrassi I, Deheragoda M, Creegen D, et al. Liver histology in children with glycogen storage disorders type VI and IX. Dig Liver Dis. 2021 Jan. 53 (1):86-93. [QxMD MEDLINE Link].
Bali DS, El-Gharbawy A, Austin S, et al. Glycogen Storage Disease Type I. Adam MP, Everman DB, Mirzaa GM, et al, eds. GeneReviews® [Internet]. Seattle, WA: University of Washington; 2021. 1993-2022. [Full Text].
Derks TGJ, Rodriguez-Buritica DF, Ahmad A, de Boer F, Couce ML, Grünert SC, et al. Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs. Nutrients. 2021 Oct 27. 13 (11):3828. [QxMD MEDLINE Link]. [Full Text].
Bhattacharya K, Orton RC, Qi X, et al. A novel starch for the treatment of glycogen storage diseases. J Inherit Metab Dis. 2007 Jun. 30(3):350-7. [QxMD MEDLINE Link].
Ross KM, Brown LM, Corrado MM, Chengsupanimit T, Curry LM, Ferrecchia IA, et al. Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I. JIMD Rep. 2016. 26:85-90. [QxMD MEDLINE Link].
Koeberl DD, Kishnani PS, Chen YT. Glycogen storage disease types I and II: treatment updates. J Inherit Metab Dis. 2007 Apr. 30(2):159-64. [QxMD MEDLINE Link].
Kishnani PS, Sun B, Koeberl DD. Gene therapy for glycogen storage diseases. Hum Mol Genet. 2019 Oct 1. 28 (R1):R31-R41. [QxMD MEDLINE Link]. [Full Text].
Chou, J.Y., Cho, JH., Kim, GY. et al. Molecular biology and gene therapy for glycogen storage disease type Ib. J Inherit Metab Dis. 2018. 41:1007-1014. [Full Text].
Zhou Z, Austin GL, Shaffer R, Armstrong DD, Gentry MS. Antibody-Mediated Enzyme Therapeutics and Applications in Glycogen Storage Diseases. Trends Mol Med. 2019 Dec. 25 (12):1094-1109. [QxMD MEDLINE Link]. [Full Text].
Zhang L, Lee C, Arnaoutova I, et al. Gene therapy using a novel G6PC-S298C variant enhances the long-term efficacy for treating glycogen storage disease type Ia. Biochem Biophys Res Commun. 2020 Jun 30. 527 (3):824-30. [QxMD MEDLINE Link]. [Full Text].
Arnaoutova I, Zhang L, Chen HD, Mansfield BC, Chou JY. Correction of metabolic abnormalities in a mouse model of glycogen storage disease type Ia by CRISPR/Cas9-based gene editing. Mol Ther. 2021 Apr 7. 29 (4):1602-10. [QxMD MEDLINE Link]. [Full Text].
Garbade SF, Ederer V, Burgard P, et al. Impact of glycogen storage disease type I on adult daily life: a survey. Orphanet J Rare Dis. 2021 Sep 3. 16 (1):371. [QxMD MEDLINE Link]. [Full Text].
Reuser AJ, Verheijen FW, Bali D, van Diggelen OP, Germain DP, Hwu WL, et al. The use of dried blood spot samples in the diagnosis of lysosomal storage disorders--current status and perspectives. Mol Genet Metab. 2011 Sep-Oct. 104 (1-2):144-8. [QxMD MEDLINE Link].
Kishnani PS, Corzo D, Leslie ND, Gruskin D, Van der Ploeg A, Clancy JP, et al. Early treatment with alglucosidase alpha prolongs long-term survival of infants with Pompe disease. Pediatr Res. 2009 Sep. 66 (3):329-35. [QxMD MEDLINE Link].
Kishnani PS, Goldenberg PC, DeArmey SL, Heller J, Benjamin D, Young S, et al. Cross-reactive immunologic material status affects treatment outcomes in Pompe disease infants. Mol Genet Metab. 2010 Jan. 99 (1):26-33. [QxMD MEDLINE Link].
Messinger YH, Mendelsohn NJ, Rhead W, Dimmock D, Hershkovitz E, Champion M, et al. Successful immune tolerance induction to enzyme replacement therapy in CRIM-negative infantile Pompe disease. Genet Med. 2012 Jan. 14 (1):135-42. [QxMD MEDLINE Link].
Martin-Touaux E, Puech JP, Chateau D, et al. Muscle as a putative producer of acid alpha-glucosidase for glycogenosis type II gene therapy. Hum Mol Genet. 2002 Jul 1. 11(14):1637-45. [QxMD MEDLINE Link].
Lim JA, Choi SJ, Gao F, Kishnani PS, Sun B. A novel gene therapy approach for GSD III using an AAV vector encoding a bacterial glycogen debranching enzyme. Mol Ther Methods Clin Dev. 2020 Sep 11. 18:240-9. [QxMD MEDLINE Link]. [Full Text].
Roe CR, Mochel F. Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential. J Inherit Metab Dis. 2006 Apr-Jun. 29(2-3):332-40. [QxMD MEDLINE Link].
Kinman RP, Kasumov T, Jobbins KA, Thomas KR, Adams JE, Brunengraber LN. Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. Am J Physiol Endocrinol Metab. 2006 Oct. 291(4):E860-6. [QxMD MEDLINE Link].
Reason SL, Godfrey RJ. The potential of a ketogenic diet to minimize effects of the metabolic fault in glycogen storage disease V and VII. Curr Opin Endocrinol Diabetes Obes. 2020 Oct. 27 (5):283-90. [QxMD MEDLINE Link].
Similä ME, Auranen M, Piirilä PL. Beneficial effects of ketogenic diet on phosphofructokinase deficiency (glycogen storage disease type VII). Front Neurol. 2020. 11:57. [QxMD MEDLINE Link]. [Full Text].
Martiniuk F, Mehler M, Tzall S, et al. Sequence of the cDNA and 5'-flanking region for human acid alpha-glucosidase, detection of an intron in the 5' untranslated leader sequence, definition of 18-bp polymorphisms, and differences with previous cDNA and amino acid sequences. DNA Cell Biol. 1999. 9:85. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

An infant with glycogen storage disease type Ia. Note the typical facial aspect resembling a doll's face.
Glycogen storage disease type I. Abdominal sonogram showing large nodules in the liver.
A child with glycogen storage disease type Ia.
Glycogen storage disease type II. Photomicrograph of the liver. Note the intensively stained vacuoles in the hepatocytes (periodic acid-Schiff, original magnification X 27).
Glycogen storage disease type II. Photomicrograph of the liver. Note the regular reticular net and hepatocytes vacuolization (Gordon-Sweet stain, original magnification X 25).

of 5

Author

Catherine Anastasopoulou, MD, PhD, FACE Associate Professor of Medicine, The Steven, Daniel and Douglas Altman Chair of Endocrinology, Sidney Kimmel Medical College of Thomas Jefferson University; Einstein Endocrine Associates, Einstein Medical Center

Catherine Anastasopoulou, MD, PhD, FACE is a member of the following medical societies: American Association of Clinical Endocrinologists, American Society for Bone and Mineral Research, Endocrine Society, Philadelphia Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Dipa Avichal, DO Attending Physician, Department of Endocrinology, Diabetes and Metabolism, CHS Physician Partners, PC

Dipa Avichal, DO is a member of the following medical societies: American Association of Clinical Endocrinologists, American Thyroid Association, Endocrine Society

Disclosure: Nothing to disclose.

Daniela F de Lima Corvino, MD Resident Physician, Department of Internal Medicine, Einstein Medical Center

Daniela F de Lima Corvino, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD Professor Emeritus of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for Physician Leadership, American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical and Translational Research, Endocrine Society, International Society for Clinical Densitometry, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Djordjije Karadaglic, MD, DSc Professor, School of Medicine, University of Podgorica, Podgorica, Montenegro

Djordjije Karadaglic, MD, DSc is a member of the following medical societies: American Academy of Dermatology, European Academy of Dermatology and Venereology, Serbian Association of Dermatorvenerologists

Disclosure: Nothing to disclose.

Ljubomir Stojanov, MD, PhD Lecturer in Metabolism and Clinical Genetics, University of Belgrade School of Medicine, Serbia

Disclosure: Nothing to disclose.

Acknowledgements

Milos D Pavlovic, MD, PhD Head of Immunodermatology, Professor, Department of Dermatology and Venereology, Military Medical Academy, Belgrade, Serbia

Milos D Pavlovic, MD, PhD is a member of the following medical societies: European Academy of Dermatology and Venereology

Disclosure: Nothing to disclose.

Sections Glycogen Storage Diseases Types I-VII
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Glycogen Storage Diseases Types I-VII Medication

Medication Summary

Iron salts

Class Summary

Ferrous sulfate (Feosol, Feratab, Fer-Iron)

Ferrous sulfate (Feosol, Feratab, Fer-Iron)

Uricosuric agents

Class Summary

Allopurinol (Zyloprim)

Allopurinol (Zyloprim)

Growth factors

Class Summary

Filgrastim (Neupogen)

Filgrastim (Neupogen)

Glycogenolytic agents

Class Summary

Glucagon (GlucaGen)

Glucagon (GlucaGen)

Angiotensin-converting enzyme (ACE) inhibitors

Class Summary

Lisinopril (Prinivil, Zestril)

Lisinopril (Prinivil, Zestril)

Ramipril (Altace)

Ramipril (Altace)

Captopril (Capoten)

Captopril (Capoten)

Enalapril (Vasotec)

Enalapril (Vasotec)

Enzyme replacements

Class Summary

Alglucosidase alfa (Myozyme)

Alglucosidase alfa (Myozyme)

Glycogen Storage Diseases Types I-VII Medication

Medication Summary

Iron salts

Class Summary

Ferrous sulfate (Feosol, Feratab, Fer-Iron)

Uricosuric agents

Class Summary

Allopurinol (Zyloprim)

Growth factors

Class Summary

Filgrastim (Neupogen)

Glycogenolytic agents

Class Summary

Glucagon (GlucaGen)

Angiotensin-converting enzyme (ACE) inhibitors

Class Summary

Lisinopril (Prinivil, Zestril)

Ramipril (Altace)

Captopril (Capoten)

Enalapril (Vasotec)

Enzyme replacements

Class Summary

Alglucosidase alfa (Myozyme)

References

Tables

Contributor Information and Disclosures

What would you like to print?