OBSOLETE: Congenital sucrase-isomaltase deficiency with minimal starch tolerance

Congenital sucrase-isomaltase deficiency (CSID) with minimal starch tolerance is a specific subtype of congenital sucrase-isomaltase deficiency, an inherited digestive disorder. This condition has been classified as 'OBSOLETE' in Orphanet, meaning it has been reclassified or merged into the broader category of congenital sucrase-isomaltase deficiency (also known as sucrose intolerance or sugar malabsorption). In this condition, the body lacks or has very low levels of two digestive enzymes — sucrase and isomaltase — that are needed to break down certain sugars and starches in the small intestine. Sucrase breaks down table sugar (sucrose), while isomaltase helps break down starches from foods like bread, rice, and potatoes. In this particular subtype, patients have very limited ability to tolerate starch in addition to sucrose, making the dietary restrictions more severe. When a person with this condition eats foods containing sucrose or starch, the undigested sugars pass into the large intestine where bacteria ferment them, causing symptoms such as watery diarrhea, bloating, gas, abdominal cramps, and nausea. Symptoms typically begin in infancy when babies are first introduced to formula, fruit juices, or solid foods containing these sugars and starches. In young children, chronic diarrhea and poor nutrient absorption can lead to failure to thrive and poor weight gain. Treatment focuses on strict dietary management — avoiding sucrose and limiting starch intake — along with enzyme replacement therapy using sacrosidase (brand name Sucraid), which is an FDA-approved oral enzyme supplement that helps break down sucrose. However, sacrosidase does not help with starch digestion, making this subtype particularly challenging to manage.

Bring these to your next appointment

• Q1.What specific foods should my child avoid, and are there any starches that might be tolerated in small amounts?,How do we determine my child's individual starch tolerance level?,Should we start sacrosidase (Sucraid), and how exactly should it be given?,How often should my child's growth and nutritional status be monitored?,Are there any nutritional supplements my child should take to prevent deficiencies?,Will my child's ability to tolerate starch improve as they get older?,Should other family members be tested for this condition?

Common questions about OBSOLETE: Congenital sucrase-isomaltase deficiency with minimal starch tolerance

Related conditions

Other rare diseases commonly researched alongside OBSOLETE: Congenital sucrase-isomaltase deficiency with minimal starch tolerance.

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• Trehalase deficiency→