Differences between the pediatric and adult presentation of XXXX : results from the US Registry.

@entity1 @entity32 @entity101 @entity4267 @entity10
BACKGROUND: @entity4267 ( @entity4267 ) is a @entity32 that causes significant morbidity in @entity1 . METHODS: The clinical features, presenting symptoms, and vascular beds involved are reviewed in the first 33 @entity1 aged <18  years who are enrolled in the United States Registry for @entity4267 from five registry sites and compared with 999 adult @entity1 from 12 registry sites. RESULTS: Mean age at diagnosis was 8.4        4.8  years (16  days to 17  years). Compared with adults, @entity4267 occurs in more males (42.4 vs 6  %, p   <   0.001). @entity1 with @entity4267 have a stronger previous history of @entity101 (93.9 vs 69.9  %, p   =   0.002). @entity101 (100  %), @entity10 (55  %), and abdominal bruits (10.7  %) were the most common presenting signs and symptoms. @entity4267 affects renal vasculature in almost all @entity1 (97 vs 69.7  %, p   =   0.003). The extra-cranial carotid vessels are less commonly involved in @entity1 (23.1 vs 73.3  %, p   <   0.001). The mesenteric arteries (38.9 vs 16.2  %, p   =   0.02) and aorta (26.3 vs 2.4  %, p   <   0.001) are more commonly involved in @entity1 . CONCLUSIONS: In the United States Registry for @entity4267 , @entity4267 affects @entity1 from infancy throughout childhood. All @entity1 presented with @entity101 and many presented with @entity10 and abdominal bruits. In @entity1 , @entity4267 most commonly affects the renal vasculature, but also frequently involves the mesenteric arteries and abdominal aorta; the carotid vessels are less frequently involved.
