05-02-2008, 02:07 PM
A 2-year-old white male with a history of recurrent respi-
ratory tract infections and infertility presented complaining
of chronic productive cough. A high resolution CT of the
chest showed widespread bronchiectasis, with pulmonary
function tests showing airflow obstruction. Considering the
most likely diagnosis, the following statements about this
condmon are correct except:
A. Meconium ileus suggests the diagnosis in a newborn.
B. It s an autosornal recessive genetic disease and the most
common mutation is a 3 base-pair deletion that causes
the loss of a phenylalanine at position 508.
C. A sweat chloride level more than 60 mEq/L associated
with typical clinical manifestations suggests the diagnosis.
D. Pancreatic insufficiency and progressive malnutrition
accounts for over 90% of the mortality related to this
disease.
E. Colonization and severe infection with Pseudomonas
aeruginosa are very frequent in more advanced stages.
ratory tract infections and infertility presented complaining
of chronic productive cough. A high resolution CT of the
chest showed widespread bronchiectasis, with pulmonary
function tests showing airflow obstruction. Considering the
most likely diagnosis, the following statements about this
condmon are correct except:
A. Meconium ileus suggests the diagnosis in a newborn.
B. It s an autosornal recessive genetic disease and the most
common mutation is a 3 base-pair deletion that causes
the loss of a phenylalanine at position 508.
C. A sweat chloride level more than 60 mEq/L associated
with typical clinical manifestations suggests the diagnosis.
D. Pancreatic insufficiency and progressive malnutrition
accounts for over 90% of the mortality related to this
disease.
E. Colonization and severe infection with Pseudomonas
aeruginosa are very frequent in more advanced stages.