Age at Diagnosis, Gender, Laterality

Age at Diagnosis

Overall Type I Type II Type III
Published Registry Series** N=50
   Age Range** (months) 0-147* 0-28 15-64 31-147
   Median Age** (months) 38 10 34 44
Unpublished Registry Series** N=128
   Age Range** (months) 0-431 0-32 6-431*** 15-147
   Median Age** (months) 10 36 44

These age differences by Type are statistically significant.

*The disease has rarely been reported in adults.

** Data from Registry publication of 50 cases [Priest,1997]

*** One 36 yr-old with documented PPB in the Registry series [see Registry Publications, Hill 1999].

Incidence histograms by Type versus Age are as follows (unpublished Registry and several literature cases):



males approximately equal females. Unpublished Registry data: in 138 centrally reviewed cases, there were 72 females and 66 males.


Unpublished Registry data: in 134 centrally-reviewed Registry cases, disease was on the right side in 72 children (54%), left in 50 children (37%), and bilateral lung disease (bilateral PPB or PPB one side and lung cysts other side) in 12 children (9%).