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Summary
A family affected by cystic fibrosis is described. One member had been under hospital
supervision for long periods since the age of 10 years without the disease being recognized
in her or her family.
Bowel symptoms were consistently absent but characteristic lung changes and progressive
dyspnoea appeared at age 47 years, leading to death from respiratory failure within
one year. At necropsy fatty replacement of the pancreas was found with preservation
of the islets of Langerhans.
The diagnosis of cystic fibrosis in the adult is discussed. Although the disease is
rare in adults, it is suggested that the family history should be carefully explored
in cases where the severity of symptoms leads to suspicion of bronchiectasis or tuberculosis
which cannot be confirmed.
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© 1970 Baillière, Tindall & Cassell. Published by Elsevier Inc.
