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Living with Glycogen Storage Disease 1a: Jake's StoryEducational resources on glycogen storage disease -
The first choice to confirm the clinical suspicion of GSD I is a mutation analysis. After excluding patients with neutropenia, complete G6PC sequencing is performed. During the availability of liver biopsy tissue, G6Pase enzyme activity is analyzed to confirm the diagnosis.
The main targets for the management of GSD I are the prevention of acute metabolic derangement, prevention of acute and long-term complications, attainment of normal psychological development, and good quality of life.
Diet and lifestyle changes are made to prevent the primary concern of the disease, hypoglycemia. Fasting should be avoided, and frequent small feeds rich in complex carbohydrates along with fiber is recommended. Therefore, a diet low in fructose and sucrose is recommended with limiting the intake of galactose and lactose to one serving per day.
Initially, infants are fed soy-based, sugar-free formula on demand every 2 to 3 hours. Awakening the infant every 3 to 4 hours to monitor blood glucose and giving feeds is difficult. Therefore, it is important for the parents to be trained in inserting a nasogastric NG tube or a G-tube should be placed surgically.
This allows the parents to administer feeds especially when the child is sick or refuses to eat. In patients with GSD I, cornstarch has been used for the treatment of hypoglycemia as its slow digestion provides a steady release of glucose. This maintains the glucose levels for longer periods of time.
In young children, 1. While older children, adolescents, and adults, are given 1. All patients with GSD I should wear a medical alert bracelet. Along with blood glucose monitoring, a lactate meter can be a good tool to alert the parents especially in times of emergency.
Hypoglycemia should be treated immediately with a fast-acting glucose source such as cornstarch or commercially prepared glucose polymers or over-the-counter diabetic glucose tablets.
Patients with GSD Ib have an increased risk of infections at the surgical site for G-tube due to neutropenia. Therefore granulocyte colony-stimulating factor G-CSF is administered before placing a G-tube.
The patients that receive G-CSF need a complete blood count CBC evaluation monthly along with the measurement of their spleen. To avoid pump failures and occluded or disconnected tubing, bed-wetting devices that detects formula spilling onto the bed, infusion pump alarms, safety adapters, connectors, and tape for tubing is recommended as safety precautions.
Limiting foods rich in lactose and sucrose such as fruits, juice, and dairy puts a child at risk for nutritional deficiency. The child should be carefully assessed, and diet should be supplemented with adequate micronutrients. Oral citrate or bicarbonate is used to treat patients with persistent lactic acidosis.
These agents alkalinize the urine and reduce the risk of urolithiasis and nephrocalcinosis. Allopurinol reduces uric acid levels preventing recurrent attacks of gout. However, during an acute attack, Colchicine is preferred. Hyperlipidemia has only shown a partial response to medical intervention with statins, niacin, fibrates, and fish oil along with dietary interventions such as consuming medium-chain triglyceride milk.
Its resolution has been reported with liver transplantation. Starting from infancy, systemic blood pressure measurement should be checked on every office visit while serum creatinine is evaluated every 3 to 6 months to monitor renal function.
Patients with persistent microalbuminuria should be treated with an angiotensin-converting enzyme ACE inhibitor to prevent worsening of renal function. Patients with GSD I have hepatomegaly universally due to fat and glycogen deposition in the liver.
The common liver lesions seen in patients with GSD Ia include focal fatty infiltration, focal fatty sparing, focal nodular hyperplasia, peliosis hepatis, hepatocellular adenoma HCA , and hepatocellular carcinoma HCC.
Therefore, a liver function test should be repeated every 6 to 12 months. Liver transplantation is an option for patients with multifocal growing lesions that do not respond to primary treatment. As per guidelines for the management of GSD I published by the collaborative European study [8] , the following biomedical targets are recommended:.
It is important to differentiate GSD I from other diseases that present with hepatomegaly and or hypoglycemia. Dietary therapy is the first line treatment for patients with GSD I.
However, to prevent long-term complications of the disease such as hepatocellular adenoma HCA , hepatocellular carcinoma HCC , renal failure among others, gene therapy in animal models of GSD is showing potential for the future trial in humans.
The most likely etiology for HCC is the transformation of adenomas to carcinoma. In such patients, the diagnosis of HCC is challenging due to the abundance of adenomas making biopsy difficult along with normal levels of biomarkers like a-fetoprotein and carcinoembryonic antigen.
If a hepatic adenoma is detected, liver ultrasound or MRI examinations is repeated every 3 to 6 months. Due to an increased risk of developing HCA, female patients with GSD I should avoid combined oral contraception. Patients with GSD I may develop bleeding disorders from impaired platelet function.
There is also an increased risk of osteoporosis and fractures from vitamin D deficiency. Therefore, routine monitoring of vitamin D levels along with dual-energy x-ray absorptiometry DXA scans is recommended to monitor the bone density and the need for vitamin D supplementation.
Renal failure may occur due to proximal renal tubular or renal glomerular dysfunction. Thus patients then develop anemia of chronic kidney disease that may be further exacerbated by iron deficiency, chronic metabolic acidosis or bleeding diathesis.
Anemic patients are treated with EPO therapy after screening them for iron deficiency and replenishing their iron stores. Patients with uncontrolled blood lactate, serum lipids, and uric acid levels are also at an increased risk for nephropathy that may need renal transplantation.
Therefore, an annual ultrasound examination of the kidneys is recommended after the first decade of life. Other complications include menorrhagia and polycystic ovaries in females, and gout from hyperuricemia.
Dietary therapy maintains the patient's blood glucose levels and reduces the early symptoms. However, to avoid long-term complications such as HCA, HCC, and renal failure, gene therapies in GSD I mice models showed promise. Types of glycogen storage diseases Contributed by William L.
Disclosure: Nirzar Parikh declares no relevant financial relationships with ineligible companies. Disclosure: Rajni Ahlawat declares no relevant financial relationships with ineligible companies. This book is distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.
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National Library of Medicine Rockville Pike Bethesda, MD Web Policies FOIA HHS Vulnerability Disclosure. Help Accessibility Careers. Take a look at the Sports section for more information. Even with regular meals, snacks, and starch if used, you may experience a hypo.
Making others, particularly teachers, aware of the symptoms and treatment is important. When you start or move schools, your family and SMT can discuss this with your school and prepare a detailed plan. This will allow a plan to be put in place in good time.
As you enter further education, it is important for you to inform relevant staff members of your condition, and any special needs you may have. Vitaflo International Ltd, Suite 1.
What are Glycogen storage diseases What are Glycogen storage diseases. Dagli AI, Lee PJ, Correia CE, et al. Pregnancy in glycogen storage disease type Ib: gestational care and report of first successful deliveries.
Chou JY, Mansfield BC. Mutations in the glucosephosphatase-alpha G6PC gene that cause type Ia glycogen storage disease. Hum Mutat. Franco LM, Krishnamurthy V, Bali D, et al. Hepatocellular carcinoma in glycogen storage disease type Ia: a case series.
Lewis R, Scrutton M, Lee P, Standen GR, Murphy DJ. Antenatal and Intrapartum care of a pregnant woman with glycogen storage disease type 1a. Eur J Obstet Gynecol Reprod Biol. Ekstein J, Rubin BY, Anderson, et al. Mutation frequencies for glycogen storage disease in the Ashkenazi Jewish Population.
Am J Med Genet A. Melis D, Parenti G, Della Casa R, et al. Brain Damage in glycogen storage disease type I. J Pediatr. Rake JP, Visser G, Labrune, et al. Guidelines for management of glycogen storage disease type I-European study on glycogen storage disease type I ESGSD I. Eur J Pediatr.
Rake JP Visser G, Labrune P, et al. Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European study on glycogen storage disease type I EGGSD I.
Eur J Pediat. Chou JY, Matern D, Mansfield, et al. Type I glycogen Storage diseases: disorders of the glucosePhosphatase complex. Curr Mol Med. Schwahn B, Rauch F, Wendel U, Schonau E.
Low bone mass in glycogen storage disease type 1 is associated with reduced muscle force and poor metabolic control.
Visser G, Rake JP, Labrune P, et al. Consensus guidelines for management of glycogen storage disease type 1b. Results of the European study on glycogen storage disease type I. Weinstein DA and Wolfsdorf JI. Effect of continuous gucose therapy with uncooked cornstarch on the long-term clinical course of type 1a glycogen storage disease.
Eur J Pediatr ; Janecke AR, Mayatepek E, and Utermann G. Molecular genetics of type I glycogen storage disease. Mol Genet Metab. Viser G, Rake JP, Fernandes, et al. Neutropenia, neutrophil dysfunction, and inflammatory bowel disease in glycogen storage disease type 1b: results of the European study on glycogen storage disease type I.
Chen YT, Bazarre CH, Lee MM, et al. Type I glycogen storage disease: nine years of management with corn starch. INTERNET Bali DS, Chen YT, Austin S, et al. Glycogen Storage Disease Type I. In: Adam MP, Ardinger HH, Pagon RA, et al.
GeneReviews® [Internet]. Seattle WA : University of Washington, Seattle; NORD strives to open new assistance programs as funding allows. NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Rare Disease Database. Glycogen Storage Disease Type I Print. Acknowledgment NORD gratefully acknowledges Deeksha Bali, PhD, Professor, Division of Medical genetics, Department of Pediatrics, Duke Health; Co-Director, Biochemical Genetics Laboratories, Duke University Health System, and Yuan-Tsong Chen, MD, PhD, Professor, Division of Medical Genetics, Department of Pediatrics, Duke Medicine; Distinguished Research Fellow, Academia Sinica Institute of Biomedical Sciences, Taiwan for assistance in the preparation of this report.
Disease Overview Glycogen storage diseases are a group of disorders in which stored glycogen cannot be metabolized into glucose to supply energy and to maintain steady blood glucose levels for the body. Detailed evaluations may be useful for a differential diagnosis: Forbes or Cori disease GSD-III is one of several glycogen storage disorders that are inherited as autosomal recessive traits.
Genetic counseling is recommended for affected individuals and their families. For information about clinical trials being conducted at the National Institutes of Health NIH in Bethesda, MD, contact the NIH Patient Recruitment Office: Tollfree: TTY: Email: prpl cc.
Additional Assistance Programs MedicAlert Assistance Program NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations. Rare Caregiver Respite Program This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Association for Glycogen Storage Disease AGSD. Email: info agsdus. Related Rare Diseases: Adult Polyglucosan Body Disease , Danon Disease , Pompe Disease ,
Glycogen storage diseases GSDs are a blycogen of inherited genetic disorders that cause glycogen glycogsn be Educational resources on glycogen storage disease stored in the body. Children with glycogen diseqse Educational resources on glycogen storage disease have Muscular strength progression strategies buildup of abnormal amounts or types of glycogen in their tissues. Glycogen is the storage form of glucose in our bodies. Glucose is a simple sugar, which is a form of carbohydrate. It is found in many foods and is the main source of energy in our bodies. The main types of glycogen storage diseases in children are categorized by number and name. They include:.
Didease reactions may include extreme difficulty breathing, shallow breathing, abnormal heart rate, low blood sgorage, throat tightness, face and Educatiojal swelling, kidney Educational resources on glycogen storage disease, and resourrces skin lesions. You Hygienic practices seek immediate medical Body density monitoring if signs glyogen symptoms of Educational resources on glycogen storage disease, hypersensitivity reactions, and immune system reactions occur. View more. The organizations and online resources listed below can provide information and support to patients and families living with Pompe disease through disease education; updates on research, clinical trials, and treatment advances; support groups and counseling; and more. Please note that the websites listed below, with the exception of the Sanofi Genzyme Pompe Disease website, are maintained by the sponsoring organizations, Sanofi Genzyme therefore does not control and is not responsible for the content of these websites. Formed to assist in funding research and to promote public awareness of acid maltase deficiency, another name for Pompe disease, this U. organization is a member of the International Pompe Association. Educational resources on glycogen storage disease -
Hypoglycemia is avoided by consuming starch, and an optimal, physically modified form is now commercially available. Hyperuricemia is treated with allopurinol and hyperlipidemia with statins. Some GSDs like GSD type II can now be treated with enzyme replacement therapy ERT , using recombinant alglucosidase alfa, which degrades lysosomal glycogen.
There is ongoing research to use ERT with other forms of GSDs. Liver transplantation should be considered for patients with certain GSDs with progressive hepatic forms that have progressed to hepatic malignancy or failure.
Though liver failure and hypoglycemia may be corrected with liver transplantation, cardiomyopathy associated with the GSD will not be corrected and may continue to progress.
Glucagon is only effective in insulin-mediated hypoglycemia and will not be helpful in patients who present with hypoglycemia secondary to a GSD. With early diagnosis and proper management, the prognosis of most GSDs is good.
Rarely, end-stage renal disease requiring kidney transplantation may occur in patients with GSD type Ib. Hypoglycemia-associated seizures and cardiac arrest can occur in early childhood.
whereas in GSD type Ib, recurrent bacterial infections secondary to neutropenia will be seen. Cardiomyopathy and limb-girdle dystrophy can be seen in patients with GSD type II. Hypertrophic cardiomyopathy is a classic complication of GSD type III.
Growth retardation and short status are also seen in GSD type IX a, b, c, d and GSD type XII, but a cognitive-developmental delay is also a feature in the latter.
Patient and parent education about the dietary modifications and frequency of feeding is of utmost importance in optimizing the clinical outcomes for patients affected with these diseases. Depending on the type of GSD affecting the patient, specific instruction will be required.
Patients and parents will need specific education to monitor for signs of hypoglycemia and the increased need for glucose or carbohydrate during an acute illness such as an infection.
In patients with GLUT2 deficiency, additional education regarding oral replacement of electrolytes lost via the kidneys is essential. GSDs are a group of complex metabolic disorders best managed by an interprofessional team of clinicians, nurses, pharmacists, and dietitians.
Registered dieticians and specialty nurses play a key role in educating patients and their caregivers to ensure hypoglycemia is avoided.
This not only ameliorates the risk of hypoglycemia-associated complications but also prevents long-term disease sequelae in most GSDs. Specialty pharmacists play a pivotal role in managing GSD type II to ensure enzyme replacement therapy is carried out adequately and that the medication is administered under optimal circumstances.
Primary care clinicians, which include physicians and mid-level practitioners, and pediatricians, in coordination with specialists, help ensure patients have adequate growth and function with minimal risk of severe complications such as renal or liver failure. All interprofessional team members should be vigilant in monitoring these patients and alert the other team embers if any complications develop or the patient's condition worsens; this requires meticulous documentation and open communication between everyone on the care team.
The key overall goal is to avoid and treat hypoglycemia, hyperlactatemia, hyperuricemia, and hyperlipidemia. A well-coordinated interprofessional team can help manage patients with these diseases adequately and ensure they live a normal life. The development of experimental therapies, such as gene therapy, may eventually provide curative options for patients with these diseases in the future.
Glycogen Branching Polymer left Glycogen Storage Disease right Contributed by William Stone, MD. Disclosure: William Stone declares no relevant financial relationships with ineligible companies.
Disclosure: Hajira Basit declares no relevant financial relationships with ineligible companies. Disclosure: Abdullah Adil declares no relevant financial relationships with ineligible companies. This book is distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.
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Search term. Glycogen Storage Disease William L. Author Information and Affiliations Authors William L. Affiliations 1 East Tennessee State University.
Continuing Education Activity Glycogen storage diseases GSDs are inherited inborn errors of carbohydrate metabolism. Introduction Glycogen storage diseases GSDs are inherited inborn errors of carbohydrate metabolism.
Etiology The etiology of GSDs is best understood by following the metabolic events leading to the synthesis glycogenesis and degradation of glycogen glycogenolysis.
Epidemiology The true incidence of metabolic diseases is difficult to determine given the lack of uniform, universal screening at birth. Pathophysiology As stated above, glycogen is the stored form of glucose and is composed of long polymers of 1,4 linked glucose with branch points via 1,6 linked glucose molecules.
History and Physical GSDs are a diverse set of rare inborn errors of carbohydrate metabolism that can have variable phenotypic presentation even within the same GSD type. Evaluation Hypoglycemia is defined as a plasma concentration of glucose that results in symptoms attributable to hypoglycemia and is reversed with the administration of glucose.
Biopsy Although specific genetic testing is now available for diagnosing most GSDs, histologic examination of liver or muscle biopsy is still used in specific scenarios. Differential Diagnosis Charcot-Marie-Tooth disease.
Prognosis With early diagnosis and proper management, the prognosis of most GSDs is good. Complications Hypoglycemia-associated seizures and cardiac arrest can occur in early childhood.
Deterrence and Patient Education Patient and parent education about the dietary modifications and frequency of feeding is of utmost importance in optimizing the clinical outcomes for patients affected with these diseases. Enhancing Healthcare Team Outcomes GSDs are a group of complex metabolic disorders best managed by an interprofessional team of clinicians, nurses, pharmacists, and dietitians.
Review Questions Access free multiple choice questions on this topic. Comment on this article. Figure Glycogen Branching Polymer left Glycogen Storage Disease right Contributed by William Stone, MD. References 1. Hicks J, Wartchow E, Mierau G.
Glycogen storage diseases: a brief review and update on clinical features, genetic abnormalities, pathologic features, and treatment. Ultrastruct Pathol. Ozen H. Glycogen storage diseases: new perspectives.
World J Gastroenterol. Kanungo S, Wells K, Tribett T, El-Gharbawy A. Glycogen metabolism and glycogen storage disorders. Ann Transl Med. Kannourakis G. Glycogen storage disease. Semin Hematol. Parikh NS, Ahlawat R. StatPearls Publishing; Treasure Island FL : Aug 8, Glycogen Storage Disease Type I.
Schreuder AB, Rossi A, Grünert SC, Derks TGJ. Glycogen Storage Disease Type III. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors.
GeneReviews ® [Internet]. University of Washington, Seattle; Seattle WA : Mar 9, Chan J, Desai AK, Kazi ZB, Corey K, Austin S, Hobson-Webb LD, Case LE, Jones HN, Kishnani PS. The emerging phenotype of late-onset Pompe disease: A systematic literature review.
Mol Genet Metab. Applegarth DA, Toone JR, Lowry RB. Incidence of inborn errors of metabolism in British Columbia, Tarnopolsky MA. Metabolic Myopathies. Continuum Minneap Minn. Oldfors A, DiMauro S. New insights in the field of muscle glycogenoses. Curr Opin Neurol. Thornton PS, Stanley CA, De Leon DD, Harris D, Haymond MW, Hussain K, Levitsky LL, Murad MH, Rozance PJ, Simmons RA, Sperling MA, Weinstein DA, White NH, Wolfsdorf JI.
Recommendations from the Pediatric Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in Neonates, Infants, and Children. J Pediatr. Morris AA, Thekekara A, Wilks Z, Clayton PT, Leonard JV, Aynsley-Green A.
Evaluation of fasts for investigating hypoglycaemia or suspected metabolic disease. Arch Dis Child. Weinstein DA, Steuerwald U, De Souza CFM, Derks TGJ. Inborn Errors of Metabolism with Hypoglycemia: Glycogen Storage Diseases and Inherited Disorders of Gluconeogenesis.
Pediatr Clin North Am. Orho M, Bosshard NU, Buist NR, Gitzelmann R, Aynsley-Green A, Blümel P, Gannon MC, Nuttall FQ, Groop LC. Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0.
J Clin Invest. Coleman RA, Winter HS, Wolf B, Chen YT. Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features. J Inherit Metab Dis. Kohler L, Puertollano R, Raben N.
Pompe Disease: From Basic Science to Therapy. Bartram C, Edwards RH, Beynon RJ. McArdle's disease-muscle glycogen phosphorylase deficiency. Biochim Biophys Acta. Lefeuvre C, Schaeffer S, Carlier RY, Fournier M, Chapon F, Biancalana V, Nicolas G, Malfatti E, Laforêt P. Glycogenin-1 deficiency mimicking limb-girdle muscular dystrophy.
Mol Genet Metab Rep. Molares-Vila A, Corbalán-Rivas A, Carnero-Gregorio M, González-Cespón JL, Rodríguez-Cerdeira C. Biomarkers in Glycogen Storage Diseases: An Update.
Int J Mol Sci. Ferreira CR, Gahl WA. Lysosomal storage diseases. Transl Sci Rare Dis. Glucagon for treatment of insulin hypoglycemia: its use in the patient with diabetes.
Am Pract Dig Treat. Martin AP, Bartels M, Schreiber S, Buehrdel P, Hauss J, Fangmann J. Successful staged kidney and liver transplantation for glycogen storage disease type Ib: A case report. Transplant Proc. Saltik-Temizel IN, Coşkun T, Yüce A, Koçak N.
Fanconi-Bickel syndrome in three Turkish patients with different homozygous mutations. Turk J Pediatr. Koeberl DD, Kishnani PS, Chen YT. Glycogen storage disease types I and II: treatment updates.
This next group of glycogen storage disease treatment guidelines applies to people who have glycogen storage diseases that affect the muscles, or types V and VII.
This is done by:. There is no way to prevent glycogen storage diseases. However, early treatment can help control the disease once a person has it.
If you have a glycogen storage disease or a family history of the disorder, you can talk to a genetic counselor when deciding to have children.
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Children's Hospital is part of the UPMC family. UPMC Website UPMC's Story. Our Sites. Liver Disease States. Liver Transplant. Glycogen Storage Diseases GSD in Children What Is Glycogen Storage Disease? Types of Glycogen Storage Disease The main types of glycogen storage diseases in children are categorized by number and name.
Glycogen Storage Disease Symptoms Glycogen storage disease symptoms in pediatric patients depend on its type. These tests may include: Biopsy of the affected organs Blood tests and urine tests MRI scan — a test that uses magnetic waves to make pictures of the inside of the body Glycogen Storage Disease Treatment Glycogen storage disease treatment will depend on the type of disease and the symptoms.
The goal of treatment is to maintain normal blood glucose levels. This may be done with: A nasogastric infusion of glucose in infants and children under age two Dietary changes, including: In children over age two, frequent small carbohydrate feedings are given throughout the day. This may include uncooked cornstarch.
Uncooked cornstarch provides a steady slow-release form of glucose. Elimination of foods that are high in fructose or lactose type I only Allopurinol Aloprim, Zyloprim may be prescribed to reduce uric acid levels in the blood.
This is done to prevent gout and kidney stones. Type IV is sometimes treated with liver transplantation. This is done by: Regulating or limiting strenuous exercise to avoid fatigue symptoms Improving exercise tolerance by oral intake of glucose or fructose fructose must be avoided in people with type I , or an injection of glucagon Eating a high protein diet There is no way to prevent glycogen storage diseases.
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Pediatric Electrolytes benefits care is what dissase do. For more than two decades, PHS has provided compassionate, tsorage, specialized, high quality Educational resources on glycogen storage disease care to Educational resources on glycogen storage disease patients, no matter Fasting window and meal satisfaction their Educatinoal challenges. A multidisciplinary PHS team collaborates to do everything possible to help our patients with glycogen storage disease live life to the fullest — at home. Glycogen storage disease is an inherited disorder caused by the buildup of a complex sugar, called glycogen, in the liver. When a person has GSD, the liver cannot control the use of glycogen and glucose.
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