J Gastroenterol Hepatol Endosc | Volume 6, Issue 2 | Case Report | Open Access
Daniela Andrea Sturla Álvarez1, Julio Alberto Vázquez Gómez1*, Carolina Muñoz Codoceo2, Yurami Sánchez Santacruz3, Andrés Urquía Reinke4, José Ramón Villa Asensi4and Rosa Ana Muñoz Codoceo1
1Gastroenterology and Nutrition Section, Hospital Infantil Universitario Niño Jesús, Spain
2Digestive System Service, Hospital 12 de Octubre, Spain
3Department of Pathological Anatomy, Hospital Central de la Defensa Gómez Ulla, Spain
4Department of Pulmonology Section, Hospital Central de la Defensa Gómez Ulla, Spain
*Correspondance to: Julio Alberto Vázquez Gómez
Fulltext PDF"Cystic Fibrosis-Related Liver Disease" (CFLD) has a prevalence of 30% to 50% and is another consequence of CFTR dysfunction. Its spectrum can vary from an elevation of transaminases to cirrhosis with portal hypertension, constituting the second cause of death in these patients after pulmonary disease.
We present the case of two twin brothers diagnosed with CFLD with a torpid evolution of this complication. After searching for other liver diseases, they were diagnosed with Wilson's disease.
Although CFLD is a relatively common entity, it does not exclude that other concomitant processes may be associated. Wilson's disease is an autosomal recessive pathology in which there is an alteration of copper metabolism, depositing in the liver and frequently associating hepatic and neurological alterations.
In the literature, there is only one case like those described in this study, highlighting the importance of expanding the differential diagnosis when hepatic impairment is observed in CF patients.
Cystic fibrosis-related liver disease; Wilson&39;s disease; Hepatic impairment; Hepatic steatosis
Álvarez DAS, Gómez JAV, Codoceo CM, Santacruz YS, Reinke AU, Asensi JRV, et al. Wilson's Disease and Cystic Fibrosis. An Infrequently Recognized Association. J Gastroenterol Hepatol Endosc. 2021; 6(2): 1094..