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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this retrospective study carried out covering the period, 1978-1991, 62 neonates were seen, diagnosed and treated for intestinal atresia which included: duodenal atresia and stenosis, small bowel atresia and atresia of large bowel. Locations of obstruction were duodenal in 17 patients, jejunal in 25 patients, jejuno-ileal in 5 and colon in two. Duodenal atresia was noted in 9 infants and duodenal stenosis due to annular pancreas, Ladd's bands with malrotation of bowel in 8. Associated anomalies which were observed were anorectal malformations in 2 and malrotation in 2 infants. Birth weights ranged from 1450 gm to 3000 gm. Prematurity was recorded in 11 infants. Diagnosis of intestinal atresia in our patients was made clinically and radiologically. Intestinal atresia in neonates was differentiated from other causes of obstruction such as Meconium Ileus, Hirschsprung's disease, neonatal volvulus, rectal atresia in anorectal malformations. Treatment of infants with intestinal atresia was surgical. Surgical techniques used depended on pathological findings. In 36 patients, complications such as functional obstructions with vomiting and failure to thrive, malabsorption, aspiration, bronchopneumonia, sepsis were observed. Overall mortality rate in our cases was 25 (41.9%) out of 62 patients.
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PMID:Intestinal atresia and stenosis as seen and treated at Kenyatta National Hospital, Nairobi. 818 36

Intestinal atresias are a common cause of newborn bowel obstruction (Dalla Vecchia LK, Grosfeld JL, West KW, et al, Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133[5]:490-496). Hereditary multiple intestinal atresias, first reported by Guttman et al in 1973, is the rarest form of multiple atresias (Guttman FM, Braun P, Garance PH, et al, Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg 1973;8:633-640; Bass J, Pyloric atresia associated with multiple intestinal atresias and immune deficiency. J Pediatr Surg 2002;37:941-942.). It has been proposed to be autosomal recessive, to involve atresias in a variable combination of sites from stomach to rectum, and to be universally fatal (Bilodeau A, Prasil P, Cloutier R, et al, Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004;39:726-730; Moreno LA, Gottrand F, Turck D, et al, Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet 1990;37:143-146). Patients have significant intestinal dysfunction and unrelenting sepsis stemming from a poorly defined, severe immunologic defect. Our case report presents 2 full siblings to nonconsanguineous parents with pyloric atresia, multiple small bowel and colonic atresias, and severe immune dysfunction. Care was withdrawn within 3 months of life on both siblings after multiple bouts of sepsis. Data suggest that the immune defect may not be primary, but in fact be secondary to intestinal dysfunction. Although the subjects in this article ultimately had fatal outcomes, a comprehensive immunologic/physiologic picture is presented in hopes of furthering the understanding of this grave disease.
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PMID:Hereditary multiple intestinal atresias: 2 new cases and review of the literature. 2038 66