Glycogen storage disease type III
(Redirected from Limit dextrinosis)
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
Glycogen storage disease type III | |
---|---|
Synonyms | Cori disease, Forbes disease, Limit dextrinosis |
Pronounce | |
Specialty | Endocrinology, Genetics |
Symptoms | Hepatomegaly, hypoglycemia, muscle weakness |
Complications | N/A |
Onset | Childhood |
Duration | Lifelong |
Types | N/A |
Causes | Genetic mutation in the AGL gene |
Risks | Family history |
Diagnosis | Genetic testing, liver biopsy |
Differential diagnosis | Glycogen storage disease type I, Glycogen storage disease type II |
Prevention | N/A |
Treatment | Dietary management, cornstarch therapy |
Medication | |
Prognosis | Variable, generally good with treatment |
Frequency | Rare |
Deaths |
Glycogen Storage Disease Type III (GSD III), also known as Cori disease or Forbes disease, is a rare genetic disorder that affects the metabolism of glycogen. It is one of the several types of glycogen storage diseases, which are characterized by the abnormal storage and accumulation of glycogen in the body.
Pathophysiology[edit | edit source]
GSD III is caused by a deficiency in the enzyme amylo-1,6-glucosidase, also known as the debranching enzyme. This enzyme is crucial for the proper breakdown of glycogen into glucose. In individuals with GSD III, the debranching enzyme is either absent or not functioning correctly, leading to the accumulation of abnormally structured glycogen in the liver and muscle tissues.
Clinical Features[edit | edit source]
The symptoms of GSD III can vary widely among affected individuals. Common clinical features include:
- Hepatomegaly (enlarged liver)
- Hypoglycemia (low blood sugar levels)
- Hyperlipidemia (elevated levels of lipids in the blood)
- Myopathy (muscle weakness)
- Growth retardation
Diagnosis[edit | edit source]
Diagnosis of GSD III is typically based on clinical evaluation, biochemical tests, and genetic testing. Liver biopsy may reveal the presence of abnormal glycogen deposits. Genetic testing can confirm mutations in the AGL gene, which encodes the debranching enzyme.
Treatment[edit | edit source]
There is currently no cure for GSD III, but treatment focuses on managing symptoms and preventing complications. Dietary management is crucial and may include:
- Frequent meals high in carbohydrates
- Avoidance of fasting
- Supplementation with cornstarch to maintain blood glucose levels
Prognosis[edit | edit source]
The prognosis for individuals with GSD III varies. With proper management, many individuals can lead relatively normal lives, although some may experience progressive muscle weakness and liver disease.
See also[edit | edit source]
Transform your life with W8MD's budget GLP1 injections from $125
W8MD offers a medical weight loss program NYC and a clinic to lose weight in Philadelphia. Our W8MD's physician supervised medical weight loss centers in NYC provides expert medical guidance, and offers telemedicine options for convenience.
Why choose W8MD?
- Comprehensive care with FDA-approved weight loss medications including:
- loss injections in NYC both generic and brand names:
- weight loss medications including Phentermine, Qsymia, Diethylpropion etc.
- Accept most insurances for visits or discounted self pay cost.
- Generic weight loss injections starting from just $125.00 for the starting dose
- In person weight loss NYC and telemedicine medical weight loss options in New York city available
- Budget GLP1 weight loss injections in NYC starting from $125.00 biweekly with insurance!
Book Your Appointment
Start your NYC weight loss journey today at our NYC medical weight loss, and Philadelphia medical weight loss Call (718)946-5500 for NY and 215 676 2334 for PA
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's NYC physician weight loss.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available. Call 718 946 5500.
Advertise on WikiMD
WikiMD's Wellness Encyclopedia |
Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates, categories Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD