S. Koppelmann et al., Association of cystic fibrosis, coeliac disease and alpha-1-antitrypsin deficiency in a child of 4 years, MONATS KIND, 149(5), 2001, pp. 489-492
Background. Cystic fibrosis associated diseases as coeliac disease or alpha
-1-antitrypsin deficiency may remain undetected for a long time,as the lea
ding symptoms may be explained by the underlying basic disorder CF.
Case report. We report on a 4 year old caucasian cystic fibrosis patient wi
th additional alpha -1-antitrypsin deficiency and coeliac disease. If a pat
ient with cystic fibrosis fails to thrive despite intensive treatment, addi
tional disorders,for example coeliac disease, must be excluded by means of
serology and histology. An alpha -1-antitrysin deficiency syndrom in cystic
fibrosis patients with hepatic manifestation may accelerate the progressio
n of liver cirrhosis and increase the risk of acquiring pseudomonas aerugin
osa.