Liver cirrhosis and portal hypertension in cystic fibrosis
Introduction. As the expected survival improves in individuals with the cystic fibrosis (CF), so they may be faced with a number of medical complications. Objective. The aim of this study was to analyze the prevalence of liver cirrhosis in our CF population as well as the clinical and genet...
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| Format: | Article |
| Language: | English |
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Serbian Medical Society
2013-01-01
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| Series: | Srpski Arhiv za Celokupno Lekarstvo |
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| Online Access: | http://www.doiserbia.nb.rs/img/doi/0370-8179/2013/0370-81791312764F.pdf |
| _version_ | 1818591902471028736 |
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| author | Fuštik Stojka |
| author_facet | Fuštik Stojka |
| author_sort | Fuštik Stojka |
| collection | DOAJ |
| description | Introduction. As the expected survival improves in individuals with the
cystic fibrosis (CF), so they may be faced with a number of medical
complications. Objective. The aim of this study was to analyze the prevalence
of liver cirrhosis in our CF population as well as the clinical and genetic
characteristics of these patients. Methods. All patients older than 2 years
(n=96) were screened for liver disease. Liver cirrhosis was defined by
ultrasonographic findings of distinct heterogeneity of liver parenchyma and
nodular liver surface and/or by liver biopsy findings. Enlarged spleen,
distended portal vein and abnormal portal venous flow indicated portal
hypertension. Clinical and genotype data were analyzed. Results. Sixteen
patients were found to have liver cirrhosis, three of them with portal
hypertension. All patients had pancreatic insufficiency. Nutritional status
expressed as standard deviation score (Z score) for weight, height, and body
mass index was as follows: zW=-0.40±1.24, zH=-0.83±1.02, and BMI=20.1±2.3. CF
patients with liver cirrhosis generally had mild-to-moderate lung disease,
with average FVC and FEV1 values of 97.1±16.5% of predicted and 87.9±23.5% of
predicted, respectively. Genetic analysis showed high frequency of F508del
mutation in the group with cirrhosis (90.6%). Conclusion. The prevalence of
liver cirrhosis in our CF population older than 2 years was 16.6%. Patients
with pancreatic insufficiency and severe CFTR mutations, especially F508del,
were exposed to higher risk of developing liver cirrhosis. Liver cirrhosis
has no significant impact on the pulmonary function and the nutritional
status, until the end-stage liver disease. |
| first_indexed | 2024-12-16T10:19:49Z |
| format | Article |
| id | doaj.art-b674a9607f354b6197d60a59382971f2 |
| institution | Directory Open Access Journal |
| issn | 0370-8179 |
| language | English |
| last_indexed | 2024-12-16T10:19:49Z |
| publishDate | 2013-01-01 |
| publisher | Serbian Medical Society |
| record_format | Article |
| series | Srpski Arhiv za Celokupno Lekarstvo |
| spelling | doaj.art-b674a9607f354b6197d60a59382971f22022-12-21T22:35:21ZengSerbian Medical SocietySrpski Arhiv za Celokupno Lekarstvo0370-81792013-01-0114111-1276476910.2298/SARH1312764F0370-81791312764FLiver cirrhosis and portal hypertension in cystic fibrosisFuštik Stojka0University Children’s Clinic, Skopje, Republic of MacedoniaIntroduction. As the expected survival improves in individuals with the cystic fibrosis (CF), so they may be faced with a number of medical complications. Objective. The aim of this study was to analyze the prevalence of liver cirrhosis in our CF population as well as the clinical and genetic characteristics of these patients. Methods. All patients older than 2 years (n=96) were screened for liver disease. Liver cirrhosis was defined by ultrasonographic findings of distinct heterogeneity of liver parenchyma and nodular liver surface and/or by liver biopsy findings. Enlarged spleen, distended portal vein and abnormal portal venous flow indicated portal hypertension. Clinical and genotype data were analyzed. Results. Sixteen patients were found to have liver cirrhosis, three of them with portal hypertension. All patients had pancreatic insufficiency. Nutritional status expressed as standard deviation score (Z score) for weight, height, and body mass index was as follows: zW=-0.40±1.24, zH=-0.83±1.02, and BMI=20.1±2.3. CF patients with liver cirrhosis generally had mild-to-moderate lung disease, with average FVC and FEV1 values of 97.1±16.5% of predicted and 87.9±23.5% of predicted, respectively. Genetic analysis showed high frequency of F508del mutation in the group with cirrhosis (90.6%). Conclusion. The prevalence of liver cirrhosis in our CF population older than 2 years was 16.6%. Patients with pancreatic insufficiency and severe CFTR mutations, especially F508del, were exposed to higher risk of developing liver cirrhosis. Liver cirrhosis has no significant impact on the pulmonary function and the nutritional status, until the end-stage liver disease.http://www.doiserbia.nb.rs/img/doi/0370-8179/2013/0370-81791312764F.pdfcystic fibrosisCFTR geneliver cirrhosisportal hypertension |
| spellingShingle | Fuštik Stojka Liver cirrhosis and portal hypertension in cystic fibrosis Srpski Arhiv za Celokupno Lekarstvo cystic fibrosis CFTR gene liver cirrhosis portal hypertension |
| title | Liver cirrhosis and portal hypertension in cystic fibrosis |
| title_full | Liver cirrhosis and portal hypertension in cystic fibrosis |
| title_fullStr | Liver cirrhosis and portal hypertension in cystic fibrosis |
| title_full_unstemmed | Liver cirrhosis and portal hypertension in cystic fibrosis |
| title_short | Liver cirrhosis and portal hypertension in cystic fibrosis |
| title_sort | liver cirrhosis and portal hypertension in cystic fibrosis |
| topic | cystic fibrosis CFTR gene liver cirrhosis portal hypertension |
| url | http://www.doiserbia.nb.rs/img/doi/0370-8179/2013/0370-81791312764F.pdf |
| work_keys_str_mv | AT fustikstojka livercirrhosisandportalhypertensionincysticfibrosis |