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|Unpublished Registry Series** N=128|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Incidence histograms by Type versus Age are as follows (unpublished Registry and several literature cases):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 Unpublished Registry data: in 138 centrally reviewed cases, there were 72 females and 66 males.
Unpublished Registry data: in 134 centrally-reviewed Registry cases, disease was on the right side in 72 children (54%), left in 50 children (37%), and bilateral lung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%).