@entity8257 ( @entity8257 ) is an autosomal dominant condition comprising bilateral @entity6792 , @entity90 with a beaked nasal tip, and broad and medially deviated thumbs and great toes. It is a clinically variable disorder and has been divided into three subtypes [Cohen, 1993: Am J Med Genet 45:300-307]. Type 1 represents the less severe cases, while types 2 and 3 are the more severe cases. These latter types tend to have a higher risk for @entity245 and a reduced life expectancy. Here we review the clinical course of seven @entity1 with @entity8257 type 3. All of these @entity1 had severe manifestations of @entity8257 ; however, development was essentially normal in three, mild delay was noted in two, and moderate delay in one. Favorable outcomes in @entity1 with types 2 and 3 @entity8257 were also documented by Moore et al. [1995: Cleft Pal-Craniofac J 32:62-70]. These cases illustrate that while @entity1 with @entity8257 types 2 and 3 have an increased risk for @entity245 , a favorable outcome can be achieved in some cases with aggressive medical and surgical management. Finally, although such management should be the rule for @entity8257 types 2 and 3, it needs to be remembered that normal outcome is not the rule. The prognosis for favorable neurodevelopmental outcome and/or life expectancy remains guarded in most cases.

Favorable prognosis for XXXX with Pfeiffer syndrome types 2 and 3: implications for classification.

@entity1 :: ('9606', 'Species') :: ['children'] @entity90 :: ('MESH:C564570', 'Disease') :: ['midface hypoplasia'] @entity245 :: ('MESH:D002658', 'Disease') :: ['neurodevelopmental problems', 'neurodevelopmental difficulties'] @entity8257 :: ('MESH:C538582', 'Disease') :: ['Pfeiffer syndrome', 'PS'] @entity6792 :: ('MESH:C537369', 'Disease') :: ['coronal craniosynostosis']
@entity1 :: (9606,Species) :: ['children']
