loading...| Preferred Name | atresia of small intestine | |
| Synonyms |
familial apple peel jejunal atresia congenital atresia of the small intestine apple peel small bowel syndrome apple-peel intestinal atresia APSB Jejunoileal atresia jejunal atresia small intestinal atresia small intestine atresia congenital small intestine atresia apple peel syndrome Jejunal Atresia atresia of the small intestine intestinal atresia type IIIb |
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| Definitions |
Atresia of small intestine is a special form of intestinal atresia with absence of mesentery, which is most likely due to an intrauterine intestinal vascular accident. Newborns are usually preterm infants with low birth-weights, that encounter feeding difficulties (including vomiting with initial feeds, which may later worsened and the abdomen becomes progressively distended) as well as failure to thrive. Affected children present disrupted bowel loops assuming a spiral configuration resembling an 'apple peel' and may have less than half of the normal length of the small bowel and a physiologically short bowel. Atresia of small intestine is characterized by jejunal atresia near the ligament of Treitz, foreshortened bowel, and a large mesenteric gap. The bowel distal to the atresia is precariously supplied. Atresia of small intestine may be a manifestation of cystic fibrosis. The most important cause of mortality is short bowel syndrome, encountered in 65% of cases. |
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| ID |
http://purl.obolibrary.org/obo/MONDO_0009476 |
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| closeMatch | ||
| database_cross_reference |
UMLS:C0266172 NCIT:C98828 Orphanet:1201 MESH:C538260 MedDRA:10010626 icd11.foundation:1949256262 OMIM:243600 MEDGEN:78590 GARD:140 NORD:1309 |
|
| definition |
Atresia of small intestine is a special form of intestinal atresia with absence of mesentery, which is most likely due to an intrauterine intestinal vascular accident. Newborns are usually preterm infants with low birth-weights, that encounter feeding difficulties (including vomiting with initial feeds, which may later worsened and the abdomen becomes progressively distended) as well as failure to thrive. Affected children present disrupted bowel loops assuming a spiral configuration resembling an 'apple peel' and may have less than half of the normal length of the small bowel and a physiologically short bowel. Atresia of small intestine is characterized by jejunal atresia near the ligament of Treitz, foreshortened bowel, and a large mesenteric gap. The bowel distal to the atresia is precariously supplied. Atresia of small intestine may be a manifestation of cystic fibrosis. The most important cause of mortality is short bowel syndrome, encountered in 65% of cases. |
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| exactMatch |
http://www.orpha.net/ORDO/Orphanet_1201 http://identifiers.org/mesh/C538260 http://identifiers.org/medgen/78590 |
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| has_exact_synonym |
Jejunoileal atresia jejunal atresia small intestinal atresia small intestine atresia congenital small intestine atresia apple peel syndrome Jejunal Atresia atresia of the small intestine intestinal atresia type IIIb |
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| has_related_synonym |
familial apple peel jejunal atresia congenital atresia of the small intestine apple peel small bowel syndrome apple-peel intestinal atresia APSB |
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| IAO_0000233 | ||
| id |
MONDO:0009476 |
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| in_subset |
http://purl.obolibrary.org/obo/mondo#ordo_disorder http://purl.obolibrary.org/obo/mondo#rare http://purl.obolibrary.org/obo/mondo#ordo_morphological_anomaly http://purl.obolibrary.org/obo/mondo#nord_rare http://purl.obolibrary.org/obo/mondo#orphanet_rare |
|
| label |
atresia of small intestine |
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| notation |
MONDO:0009476 |
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| preferred label |
atresia of small intestine |
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| prefLabel |
atresia of small intestine |
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| subClassOf |