Immune deficiency in familial duodenal atresia

SW Moore, G De Jongh, P Bouic, RA Brown… - Journal of pediatric …, 1996 - Elsevier
The familial occurrence of duodenal atresia is uncommon. This study evaluated the
inheritance patterns, the nature and associations, and the presence of immunologic deficits
in duodenal atresia recurring in at least three siblings each in two nonrelated families. In the
first family, an association with Fanconi's anemia was observed in three of seven
pregnancies (2 boys, 1 girl) suggesting an autosomal recessive mode of transmission.
Patients died as a result of overwhelming (fungal) septicemia in association with …