<abstract xmlns="http://www.w3.org/1999/xhtml">
<p>Cystic fibrosis (CF) represents a multisystemic condition, due to the transmembrane conductance regulatory protein (CFTR gene), involving both gastrointestinal tract and lungs. The prevalence of CF associated liver disease is related with increased life expectancy and it is rare in childhood or puberty. Liver disease (LD) is often preceded by complications associated with pulmonary damage. We present a case of a 13 year- old girl, known with cystic fibrosis since the age of 4 months, diagnosed with cholecyst hypoplasia and cholestasis, severe malnutrition progressive extension of bronchiectasis, recurrent respiratory infections in childhood, and, later, in puberty, liver disease with third stage fibrosis (F3) revealed by Fibroscan. Hepatobiliary damage is a late consequence, which is precisely why not performing a liver biopsy, especially in infants and young children, can be justified. We highlight the value of Fibroscan, a non-invasive imaging technique capable to reveal liver fibrosis and to stage it.</p>
</abstract>

Cystic fibrosis (CF) represents a multisystemic condition, due to the transmembrane conductance regulatory protein (CFTR gene), involving both gastrointestinal tract and lungs. The prevalence of CF associated liver disease is related with increased life expectancy and it is rare in childhood or puberty. Liver disease (LD) is often preceded by complications associated with pulmonary damage. We present a case of a 13 year- old girl, known with cystic fibrosis since the age of 4 months, diagnosed with cholecyst hypoplasia and cholestasis, severe malnutrition progressive extension of bronchiectasis, recurrent respiratory infections in childhood, and, later, in puberty, liver disease with third stage fibrosis (F3) revealed by Fibroscan. Hepatobiliary damage is a late consequence, which is precisely why not performing a liver biopsy, especially in infants and young children, can be justified. We highlight the value of Fibroscan, a non-invasive imaging technique capable to reveal liver fibrosis and to stage it.

Cystic Fibrosis Associated Liver Disease and Bronchiectasis in Puberty. Case Report and Literature Review

ARS Medica Tomitana

This work is licensed under the Creative Commons Attribution 4.0 International License.

Cystic fibrosis (CF) represents a multisystemic condition, due to the transmembrane conductance regulatory protein (CFTR gene), involving both gastrointestinal...

Cystic Fibrosis Associated Liver Disease and Bronchiectasis in Puberty. Case Report and Literature Review

Online veröffentlicht: 05. Sept. 2023

Seitenbereich: 108 - 112

DOI: https://doi.org/10.2478/arsm-2022-0023

Schlüsselwörter
liver disease, bronchiectasis, fibroscan, cystic fibrosis

© 2023 Cristina-Laura Peptisor et al., published by Sciendo

This work is licensed under the Creative Commons Attribution 4.0 International License.

Cystic Fibrosis Associated Liver Disease and Bronchiectasis in Puberty. Case Report and Literature Review

Online veröffentlicht: 05. Sept. 2023

Seitenbereich: 108 - 112

DOI: https://doi.org/10.2478/arsm-2022-0023

Schlüsselwörterliver disease, bronchiectasis, fibroscan, cystic fibrosis

© 2023 Cristina-Laura Peptisor et al., published by Sciendo

This work is licensed under the Creative Commons Attribution 4.0 International License.

Schlüsselwörter
liver disease, bronchiectasis, fibroscan, cystic fibrosis