Sucrose intolerance: Difference between revisions

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'''Sucrose intolerance''', also known as [[Congenital Sucrase-Isomaltase Deficiency]] (CSID), is a rare [[genetic disorder]] that affects a person's ability to digest certain sugars. People with this condition cannot break down the sugars [[sucrose]] and [[maltose]], or other carbohydrates made of these sugar molecules.
{{SI}}
 
{{Infobox medical condition
<youtube>
| name            = Sucrose intolerance
title='''{{PAGENAME}}'''
| image          = [[File:Sucrose-inkscape.svg|200px]]
movie_url=http://www.youtube.com/v/zcrcbbODYD4
| caption        = Structure of [[sucrose]]
&rel=1
| synonyms        = Congenital sucrase-isomaltase deficiency (CSID), sucrase-isomaltase deficiency
embed_source_url=http://www.youtube.com/v/zcrcbbODYD4
| field          = [[Gastroenterology]]
&rel=1
| symptoms        = [[Diarrhea]], [[abdominal pain]], [[bloating]], [[gas]]
wrap = yes
| onset          = Childhood
width=750
| duration        = Chronic
height=600
| causes          = Genetic mutation in the [[sucrase-isomaltase]] gene
</youtube>
| risks          = [[Family history]]
 
| diagnosis      = [[Breath test]], [[stool acidity test]], [[genetic testing]]
== Causes ==
| differential    = [[Lactose intolerance]], [[fructose malabsorption]], [[irritable bowel syndrome]]
 
| treatment      = [[Dietary management]], [[enzyme replacement therapy]]
Sucrose intolerance is caused by mutations in the [[SI gene]]. This gene provides instructions for producing an enzyme called sucrase-isomaltase, which is found in the small intestine. The enzyme is responsible for breaking down sucrose and maltose into simpler sugars, which can then be absorbed into the bloodstream. When the SI gene is mutated, the body produces little or no sucrase-isomaltase, leading to sucrose intolerance.
| medication      = [[Sacrosidase]]
 
| frequency      = Rare
}}
{{Short description|Overview of sucrose intolerance}}
'''Sucrose intolerance''', also known as '''congenital sucrase-isomaltase deficiency''' (CSID), is a condition where an individual lacks the enzyme needed to digest [[sucrose]], a common sugar found in many foods. This enzyme deficiency leads to gastrointestinal symptoms when sucrose is consumed.
== Pathophysiology ==
Sucrose intolerance is caused by a deficiency in the enzyme [[sucrase-isomaltase]], which is responsible for breaking down sucrose into [[glucose]] and [[fructose]]. This enzyme is located on the brush border of the [[small intestine]]. When sucrase-isomaltase is deficient or absent, sucrose cannot be properly digested, leading to symptoms such as [[diarrhea]], [[abdominal pain]], and [[bloating]].
== Symptoms ==
== Symptoms ==
 
The symptoms of sucrose intolerance can vary in severity and may include:
The symptoms of sucrose intolerance can vary widely, but they often include [[abdominal pain]], bloating, gas, and diarrhea. These symptoms typically occur after eating foods that contain sucrose or maltose. In severe cases, the condition can lead to malnutrition and weight loss.
* Chronic diarrhea
 
* Abdominal pain and cramping
* Bloating and gas
* Nausea
* Vomiting
These symptoms typically occur after the ingestion of foods containing sucrose.
== Diagnosis ==
== Diagnosis ==
 
Diagnosis of sucrose intolerance can be challenging and often involves a combination of dietary history, symptom assessment, and diagnostic tests. Common diagnostic methods include:
Sucrose intolerance is typically diagnosed through a combination of symptom history, physical examination, and medical tests. These tests may include a [[hydrogen breath test]], which measures the amount of hydrogen in a person's breath after they consume a sugar solution. High levels of hydrogen suggest that the sugar is not being properly digested.
* '''Breath hydrogen test''': Measures hydrogen in the breath after ingestion of sucrose.
 
* '''Sucrose tolerance test''': Monitors blood glucose levels after sucrose ingestion.
== Treatment ==
* '''Genetic testing''': Identifies mutations in the sucrase-isomaltase gene.
 
== Management ==
There is currently no cure for sucrose intolerance, but the condition can be managed through dietary changes. People with this condition are typically advised to avoid foods that contain sucrose and maltose. In some cases, they may also need to take enzyme replacement therapy to help their bodies break down these sugars.
Management of sucrose intolerance primarily involves dietary modification to limit or avoid sucrose intake. Patients may benefit from:
 
* '''Sucrose-free diet''': Avoiding foods high in sucrose such as sweets, certain fruits, and processed foods.
* '''Enzyme replacement therapy''': Use of enzyme supplements to aid in the digestion of sucrose.
== Prognosis ==
With appropriate dietary management and enzyme supplementation, individuals with sucrose intolerance can lead normal, healthy lives. Early diagnosis and intervention are crucial to prevent complications and improve quality of life.
== See also ==
== See also ==
* [[Lactose intolerance]]
* [[Lactose intolerance]]
* [[Fructose malabsorption]]
* [[Fructose malabsorption]]
* [[Glucose-galactose malabsorption]]
* [[Carbohydrate metabolism]]
 
[[Category:Digestive diseases]]
== References ==
 
{{reflist}}
 
[[Category:Genetic disorders]]
[[Category:Genetic disorders]]
[[Category:Digestive diseases]]
[[Category:Rare diseases]]
{{genetic-disorder-stub}}
{{digestive-system-disease-stub}}
{{stub}}

Latest revision as of 15:48, 12 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Sucrose intolerance
Synonyms Congenital sucrase-isomaltase deficiency (CSID), sucrase-isomaltase deficiency
Pronounce N/A
Specialty N/A
Symptoms Diarrhea, abdominal pain, bloating, gas
Complications N/A
Onset Childhood
Duration Chronic
Types N/A
Causes Genetic mutation in the sucrase-isomaltase gene
Risks Family history
Diagnosis Breath test, stool acidity test, genetic testing
Differential diagnosis Lactose intolerance, fructose malabsorption, irritable bowel syndrome
Prevention N/A
Treatment Dietary management, enzyme replacement therapy
Medication Sacrosidase
Prognosis N/A
Frequency Rare
Deaths N/A


Overview of sucrose intolerance


Sucrose intolerance, also known as congenital sucrase-isomaltase deficiency (CSID), is a condition where an individual lacks the enzyme needed to digest sucrose, a common sugar found in many foods. This enzyme deficiency leads to gastrointestinal symptoms when sucrose is consumed.

Pathophysiology[edit]

Sucrose intolerance is caused by a deficiency in the enzyme sucrase-isomaltase, which is responsible for breaking down sucrose into glucose and fructose. This enzyme is located on the brush border of the small intestine. When sucrase-isomaltase is deficient or absent, sucrose cannot be properly digested, leading to symptoms such as diarrhea, abdominal pain, and bloating.

Symptoms[edit]

The symptoms of sucrose intolerance can vary in severity and may include:

  • Chronic diarrhea
  • Abdominal pain and cramping
  • Bloating and gas
  • Nausea
  • Vomiting

These symptoms typically occur after the ingestion of foods containing sucrose.

Diagnosis[edit]

Diagnosis of sucrose intolerance can be challenging and often involves a combination of dietary history, symptom assessment, and diagnostic tests. Common diagnostic methods include:

  • Breath hydrogen test: Measures hydrogen in the breath after ingestion of sucrose.
  • Sucrose tolerance test: Monitors blood glucose levels after sucrose ingestion.
  • Genetic testing: Identifies mutations in the sucrase-isomaltase gene.

Management[edit]

Management of sucrose intolerance primarily involves dietary modification to limit or avoid sucrose intake. Patients may benefit from:

  • Sucrose-free diet: Avoiding foods high in sucrose such as sweets, certain fruits, and processed foods.
  • Enzyme replacement therapy: Use of enzyme supplements to aid in the digestion of sucrose.

Prognosis[edit]

With appropriate dietary management and enzyme supplementation, individuals with sucrose intolerance can lead normal, healthy lives. Early diagnosis and intervention are crucial to prevent complications and improve quality of life.

See also[edit]