Liver disease in children with cystic fibrosis: observational study
Introduction: Cystic fibrosis (CF) is a genetic disease of autosomal recessive inheritance, characterized by dysfunction of the exocrine secretion glands. The liver is an affected organ, which causes an increase in early morbidity and mortality. Objective: To evaluate liver disease in a group of...
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2025-04-01
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| Series: | Revista Médica del Hospital General de México |
| Online Access: | https://www.hospitalgeneral.mx/frame_eng.php?id=249 |
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| author | Alejandra Sabillón Flora Zárate-Mondragón Rubén Peña-Vélez Ana I. Quesada Jaime Ramírez |
| author_facet | Alejandra Sabillón Flora Zárate-Mondragón Rubén Peña-Vélez Ana I. Quesada Jaime Ramírez |
| author_sort | Alejandra Sabillón |
| collection | DOAJ |
| description | Introduction: Cystic fibrosis (CF) is a genetic disease of autosomal recessive inheritance, characterized by dysfunction of the exocrine secretion glands. The liver is an affected organ, which causes an increase in early morbidity and mortality.
Objective: To evaluate liver disease in a group of children with CF.
Material and methods: A total of 82 children with CF confirmed with genetic testing were included. Biochemical liver function tests and liver ultrasound were evaluated. The presence of fibrosis was estimated using the aspartato aminotransferasa to platelet ratio index (APRI) and correlation tests were performed.
Results: 59.8% (n = 49) of patients had elevated alanine aminotransferase. 30.5% (n = 25) showed an APRI suggestive of fibrosis. The correlation of APRI with alanino aminotransferase was 0.685 (p < 0.001) and with GGT 0.385 (p < 0.001). The prevalence of alterations in hepatic echogenicity was lower than biochemical alterations in transaminases.
Conclusions: There is a high prevalence of liver disease at the diagnosis of CF and even a third of children could present with liver fibrosis. In this study, we found no difference in liver function tests according to liver ultrasound.
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| format | Article |
| id | doaj-art-66d5456399754aa094e64848389a0250 |
| institution | DOAJ |
| issn | 0185-1063 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Permanyer |
| record_format | Article |
| series | Revista Médica del Hospital General de México |
| spelling | doaj-art-66d5456399754aa094e64848389a02502025-08-20T02:53:50ZengPermanyerRevista Médica del Hospital General de México0185-10632025-04-01882Liver disease in children with cystic fibrosis: observational studyAlejandra Sabillón0Flora Zárate-Mondragón1Rubén Peña-Vélez2Ana I. Quesada3Jaime Ramírez4Department of Gastroenterology and Nutrition, Intituto Nacional de Pediatría, Secretaría de Salud, Mexico City, MexicoDepartment of Gastroenterology and Nutrition, Intituto Nacional de Pediatría, Secretaría de Salud, Mexico City, MexicoDepartment of Gastroenterology and Nutrition, Intituto Nacional de Pediatría, Secretaría de Salud, Mexico City, MexicoDepartment of Gastroenterology and Nutrition, Intituto Nacional de Pediatría, Secretaría de Salud, Mexico City, MexicoDepartment of Gastroenterology and Nutrition, Intituto Nacional de Pediatría, Secretaría de Salud, Mexico City, MexicoIntroduction: Cystic fibrosis (CF) is a genetic disease of autosomal recessive inheritance, characterized by dysfunction of the exocrine secretion glands. The liver is an affected organ, which causes an increase in early morbidity and mortality. Objective: To evaluate liver disease in a group of children with CF. Material and methods: A total of 82 children with CF confirmed with genetic testing were included. Biochemical liver function tests and liver ultrasound were evaluated. The presence of fibrosis was estimated using the aspartato aminotransferasa to platelet ratio index (APRI) and correlation tests were performed. Results: 59.8% (n = 49) of patients had elevated alanine aminotransferase. 30.5% (n = 25) showed an APRI suggestive of fibrosis. The correlation of APRI with alanino aminotransferase was 0.685 (p < 0.001) and with GGT 0.385 (p < 0.001). The prevalence of alterations in hepatic echogenicity was lower than biochemical alterations in transaminases. Conclusions: There is a high prevalence of liver disease at the diagnosis of CF and even a third of children could present with liver fibrosis. In this study, we found no difference in liver function tests according to liver ultrasound. https://www.hospitalgeneral.mx/frame_eng.php?id=249 |
| spellingShingle | Alejandra Sabillón Flora Zárate-Mondragón Rubén Peña-Vélez Ana I. Quesada Jaime Ramírez Liver disease in children with cystic fibrosis: observational study Revista Médica del Hospital General de México |
| title | Liver disease in children with cystic fibrosis: observational study |
| title_full | Liver disease in children with cystic fibrosis: observational study |
| title_fullStr | Liver disease in children with cystic fibrosis: observational study |
| title_full_unstemmed | Liver disease in children with cystic fibrosis: observational study |
| title_short | Liver disease in children with cystic fibrosis: observational study |
| title_sort | liver disease in children with cystic fibrosis observational study |
| url | https://www.hospitalgeneral.mx/frame_eng.php?id=249 |
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