loading...| Preferred Name |
Congenital short bowel syndrome |
|
| Synonyms |
CSBS |
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| Definitions |
(CSBS) - A disease characterized by a shortened small intestine, intestinal malrotation, and malabsorption. The mean length of the small intestine in CSBS patients is approximately 50 cm, compared with a normal length at birth of 190-280 cm. Patients with CSBS may develop severe malnutrition as a result of the hugely reduced absorptive surface of the small intestine. Infants require parenteral nutrition for survival. However, parenteral nutrition itself causes life-threatening complications such as sepsis and liver failure which are associated with a high rate of mortality early in life. |
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| ID |
http://identifiers.org/omim/615237 |
|
| altLabel |
CSBS |
|
| definition |
(CSBS) - A disease characterized by a shortened small intestine, intestinal malrotation, and malabsorption. The mean length of the small intestine in CSBS patients is approximately 50 cm, compared with a normal length at birth of 190-280 cm. Patients with CSBS may develop severe malnutrition as a result of the hugely reduced absorptive surface of the small intestine. Infants require parenteral nutrition for survival. However, parenteral nutrition itself causes life-threatening complications such as sepsis and liver failure which are associated with a high rate of mortality early in life. |
|
| id |
OMIM:615237 |
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| notation |
OMIM:615237 |
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| prefLabel |
Congenital short bowel syndrome |
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| subClassOf |