Presentation
It presents at birth and the prognosis is very poor with almost all those diagnosed with this condition dying with one month. It may be associated with combined immunodeficiency. [en.wikipedia.org]
This report presents a case of a surviving monochorionic twin with multiple intestinal atresia and congenital bilateral perisylvian syndrome, which developed after the intrauterine death of the cotwin. [ncbi.nlm.nih.gov]
Entire Body System
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Sepsis
Postoperatively, he developed several episodes of sepsis caused by persistent enterostasis, but was able to be weaned from total parenteral nutrition (TPN) by postoperative day (POD) 106. [ncbi.nlm.nih.gov]
Gastrointestinal
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Intestinal Atresia
The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. [ncbi.nlm.nih.gov]
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Abdominal Distension
Initial clinical examination was normal but abdominal distension developed during the first day. At laparotomy, prepyloric septal atresia, a distal duodenal membrane, and multiple intestinal atresia were found. The baby died aged 4 days. [ncbi.nlm.nih.gov]
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Vomiting
She developed intractable vomiting in the first hours of life. An upper gastrointestinal series and barium enema showed jejunal atresia and rectal atresia. A laparotomy was performed on the fourth day of life. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
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Jaundice
After the operation, the patient's stool remained acholic and her jaundice did not resolve. On the 58th day of life, another laparotomy was performed with the presumptive diagnosis of biliary atresia. [ncbi.nlm.nih.gov]
Musculoskeletal
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Short Arm
This gene is located on the short arm of chromosome 2 (2p16). This disorder was first described in 1971. [en.wikipedia.org]
Psychiatrical
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Suggestibility
This is characteristic of the hereditary atresias and suggests development of a defect in (re)canalization during embryogenesis. The association with omphalocele indicates a common developmental defect may be present. [ncbi.nlm.nih.gov]
Prognosis
It presents at birth and the prognosis is very poor with almost all those diagnosed with this condition dying with one month. It may be associated with combined immunodeficiency. [en.wikipedia.org]
Etiology
Multiple intestinal atresia is a rare disorder with vascular or hereditary etiology. So far, the occurrence of this malformation along with omphalocele has not been reported. [ncbi.nlm.nih.gov]
Prevention
Thus, multiple anastomoses may be the appropriate procedure to prevent short-gut syndrome for congenital multiple intestinal atresia, even in premature infants. [ncbi.nlm.nih.gov]