Master Cori Disease with Picmonic for Medicine

With Picmonic, facts become pictures. We've taken what the science shows - image mnemonics work - but we've boosted the effectiveness by building and associating memorable characters, interesting audio stories, and built-in quizzing.

Cori Disease

Coral-reef Disease
Picmonic
Cori’s disease is a type III glycogen storage disease, and it results from a deficiency in the debranching enzyme alpha-1,6-glucosidase.  The cells are able to breakdown glycogen and mobilize it into glucose, but there is a limitation in breaking down the branched bonds.  This ultimately results in some glycogen build up due to the body’s inability to break down the alpha 1, 6 bond between glucose residues. The clinical presentation is similar to type 1, Von Gierke’s, but less severe. Patients still suffer from hypoglycemia, but not to the extent as seen in type 1.  Additionally, patients often have stunted growth due to abnormal glycogen metabolism.  
16 KEY FACTS
PATHOPHYSIOLOGY
Type III Glycogen Storage Disease
(3) Tree with Gliders

Cori's disease is also referred to as type III glycogen storage disease. This is an autosomal recessive disorder, where patients are unable to convert branched glycogen polymers to glucose due to a deficiency of debranching enzyme.

Deficiency of Alpha 1, 6 Glucosidase
Broken Afro-guy with (1) Wand and (6) Mask Stuck in Glue-daisies

Alpha-1,6 glucosidase or glucosidase is a component of debranching enzyme, and breaks the alpha 1, 6 bond in glycogen. Thus, this enzyme helps mobilize glucose.

Debranching Enzyme
Enzyme Cutting-branches

Branching bonds of glycogen are essential for effective storage. In Cori's disease, these bonds cannot be broken due to a deficiency in the debranching enzyme.

Limit-Dextrin Accumulation (In Cytosol Structures)
Limit-Desks Accumulating at Side-toe-sail Boat

In this disorder, patients have an inability to convert branched glycogen polmers to glucose. Because limit dextrin is a remaining polymer produced after hydrolysis of glycogen, patients develop limit dextrin accumulation in their cytosol.

SIGNS AND SYMPTOMS
Hypoglycemia
Hippo-glue-bottle

While there is glycogenolysis ability in Cori's disease, it is limited due to the inability to breakdown branched glycogen molecules. This limitation in the body's ability to acutely mobilize glucose stores can result in transient hypoglycemia.

Muscle Weakness
Droopy Muscle

Patients have abnormal glycogen accumulation in muscle tissues in Cori disease. The accumulated glycogen is structurally abnormal and impairs the function of muscles, leading to weakness. This side effect of Cori disease is especially pronounced in adult form.

Hypotonia
Floppy Hippo-baby

Patients have abnormal glycogen accumulation in Cori disease. This is structurally abnormal and impairs the function of muscles. Often, the first sign and of disease in infants is poor muscle tone, or hypotonia.

Stunted Growth
C-clamp Stunting Growth

Growth is limited because the body cannot mobilize glycogen sufficiently and sustain adequate glucose levels for the body.

Hepatomegaly
Liver-balloon

Due to the deficiency of the debranching enzyme, there is accumulation of residual glycogen in the liver, which leads to hepatomegaly.

DIAGNOSIS
Normal Blood Lactic Acid Levels
Normal-bell-curve at Lake Acidic-lemon

The cells are still able to undergo gluconeogenesis and create free glucose residues, so lactic acid levels are within normal limits.

Gluconeogenesis Intact
Glue-genie

Unlike in Von Gierke disease, in Cori disease gluconeogenesis is intact. This means that patients are able to generate glucose from non-carbohydrate substances like lactate, glycerol, and glucogenic amino acids.

Increased LFT's
Up-arrow Liver with Test-tubes

Patients with Cori disease can develop hepatomegaly from abnormal amounts of accumulating glycogen. If the liver is involved, patients can display elevations in liver function enzymes (LFTs) on lab testing.

Increased Creatinine Kinase (CK/CPK)
Up-arrow Calvin Klein Model

The abnormal glycogen accumulation in Cori disease is structurally abnormal and impairs the function of muscles. Additionally, in times of glucose need, muscle protein is metabolized as a glucose source. These factors lead to lab findings of increased creatine kinase, which is a marker of muscle breakdown.

TREATMENT
Continuous Feeding
Infinite Feeding

It is recommended to provide frequent daytime feedings, along with continuous nasogastric tube feedings at night to infants up until the age of 3 years old. This is done to ensure they maintain satisfactory blood glucose levels.

High Protein Diet
Protein Filled Meat

Because gluconeogenesis is intact and protein can be used as a source of glucose, patients are recommended to have a high protein diet. A high protein diet prevents breakdown of muscle protein in times of glucose need, preserving muscles.

Cornstarch (Uncooked)
Corn-star

Uncooked cornstarch is a recommended dietary supplement in patients because it is a slow-release form of glucose.

Unlock all 16 facts & definitions with Picmonic Free!

JOIN FREE

Take the Cori Disease Quiz

Picmonic's rapid review multiple-choice quiz allows you to assess your knowledge.

START QUIZ NOW

It's worth every penny

Our Story Mnemonics Increase Mastery and Retention

Memorize facts with phonetic mnemonics

Unforgettable characters with concise but impactful videos (2-4 min each)

Ace Your Medical (MD/DO) Classes & Exams with Picmonic:

Over 870,000 students use Picmonic’s picture mnemonics to improve knowledge, retention, and exam performance.

Choose the #1 Medical (MD/DO) student study app.

Picmonic for Medicine (MD/DO) covers information that is relevant to your entire Medical (MD/DO) education. Whether you’re studying for your classes or getting ready to conquer the USMLE Step 1, USMLE Step 2 CK, COMLEX Level 1, or COMLEX Level 2, we’re here to help.

Works better than traditional Medical (MD/DO) flashcards.

Research shows that students who use Picmonic see a 331% improvement in memory retention and a 50% improvement in test scores.