atresia of small intestine https://github.com/monarch-initiative/mondo/issues/6743 https://github.com/monarch-initiative/mondo/issues/6876 https://www.malacards.org/card/jejunal_atresia GARD:0000140 jejunal atresia atresia of the small intestine Jejunal Atresia MEDGEN:78590 Jejunoileal atresia icd11.foundation:1949256262 small intestinal atresia apple-peel intestinal atresia Orphanet:1201 MESH:C538260 OMIM:243600 MedDRA:10010626 familial apple peel jejunal atresia UMLS:C0266172 congenital small intestine atresia MONDO:0009476 apple peel syndrome 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. NORD:1309 small intestine atresia APSB congenital atresia of the small intestine NCIT:C98828 intestinal atresia type IIIb disorder of development or morphogenesis small intestine disorder