Research Article| Volume 64, ISSUE 1, P33-36, January 1970

Adult cystic fibrosis (Mucoviscidosis)

Fatty replacement of the pancreas in a woman aged 47 years
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      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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