XXXX : a clinicopathologic survey of the "Harrisburg family" with a 49-year follow-up.

@entity1 @entity558 @entity5 @entity2838 @entity189 @entity344 @entity5277 @entity4607 @entity1586
Of the original Peutz-Jeghers families reported by Jeghers, the "Harrisburg Family" has now been followed for 49 yr. Their 12 affected family members comprise the largest Peutz-Jeghers kindred reported. The course of this family illustrates that @entity2838 is not a benign disease. One family member developed a @entity189 in a @entity4607 with adenomatous changes; this progression in the duodenum has not previously been reported. Ten @entity1 underwent 75 polypectomies. One @entity1 developed @entity5277 . Three @entity1 died in young adulthood. The development of @entity344 in 2 of 12 affected @entity1 suggests that @entity2838 may be a @entity558 . Consequently, even asymptomatic gastric, duodenal, and @entity1586 should be removed endoscopically. If surgical intervention is necessary, intraoperative endoscopy with polypectomy may prevent the development of a @entity5277 . Colonoscopic screening of @entity1 and their family members may be beneficial and surveillance for extraintestinal @entity5 appears to be warranted.
