NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS
<p>Background. Cystic fibrosis is an autosomal recessive disorder that results in damage to organs containing secretory epithelial cells. It predominantly affects respiratory tract and pancreatic function. While hepatobiliary system is frequently affected in older children with cystic fibrosis...
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| Format: | Article |
| Language: | English |
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Slovenian Medical Association
2003-04-01
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| Series: | Zdravniški Vestnik |
| Subjects: | |
| Online Access: | http://vestnik.szd.si/index.php/ZdravVest/article/view/1822 |
| _version_ | 1818573251344859136 |
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| author | Nataša Milivojevič Marjeta Sedmak Gordana Logar-Car |
| author_facet | Nataša Milivojevič Marjeta Sedmak Gordana Logar-Car |
| author_sort | Nataša Milivojevič |
| collection | DOAJ |
| description | <p>Background. Cystic fibrosis is an autosomal recessive disorder that results in damage to organs containing secretory epithelial cells. It predominantly affects respiratory tract and pancreatic function. While hepatobiliary system is frequently affected in older children with cystic fibrosis, in neonatal period jaundice caused by cystic fibrosis is a rare condition. Two cases were identified in the last ten years (1988–98) in the Department of Pediatrics University Medical Centre Ljubljana.</p><p>Patients and methods. We present two patients with neonatal jaundice as one of the rare features of cystic fibrosis. Laboratory evidences of cholestasis and liver involvement were present in both cases. In the first case, an improvement of a liver disease occured spontaneously and in the second case the resolution of clinical and laboratory evidence of liver disease followed after she had been treated with ursodeoxycholic acid.</p><p>Conclusions. Neonatal jaundice and cholestasis can be caused by cystic fibrosis. The treatment is conservative with ursodeoxycholic acid. Only in the cases when the disease is not improving conservatively, the surgical procedure can be considered.<br /><br /></p> |
| first_indexed | 2024-12-15T00:08:41Z |
| format | Article |
| id | doaj.art-ce1cf29257114b00967228df7282e4a8 |
| institution | Directory Open Access Journal |
| issn | 1318-0347 1581-0224 |
| language | English |
| last_indexed | 2024-12-15T00:08:41Z |
| publishDate | 2003-04-01 |
| publisher | Slovenian Medical Association |
| record_format | Article |
| series | Zdravniški Vestnik |
| spelling | doaj.art-ce1cf29257114b00967228df7282e4a82022-12-21T22:42:39ZengSlovenian Medical AssociationZdravniški Vestnik1318-03471581-02242003-04-017241335NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSISNataša Milivojevič0Marjeta Sedmak1Gordana Logar-Car2Inštitut za patološko fiziologijo Medicinska fakulteta Zaloška 4 1000 LjubljanaSlužba za gastroenterologijo Pediatrična klinika Klinični center Vrazov trg 1 1525 LjubljanaSlužba za gastroenterologijo Pediatrična klinika Klinični center Vrazov trg 1 1525 Ljubljana<p>Background. Cystic fibrosis is an autosomal recessive disorder that results in damage to organs containing secretory epithelial cells. It predominantly affects respiratory tract and pancreatic function. While hepatobiliary system is frequently affected in older children with cystic fibrosis, in neonatal period jaundice caused by cystic fibrosis is a rare condition. Two cases were identified in the last ten years (1988–98) in the Department of Pediatrics University Medical Centre Ljubljana.</p><p>Patients and methods. We present two patients with neonatal jaundice as one of the rare features of cystic fibrosis. Laboratory evidences of cholestasis and liver involvement were present in both cases. In the first case, an improvement of a liver disease occured spontaneously and in the second case the resolution of clinical and laboratory evidence of liver disease followed after she had been treated with ursodeoxycholic acid.</p><p>Conclusions. Neonatal jaundice and cholestasis can be caused by cystic fibrosis. The treatment is conservative with ursodeoxycholic acid. Only in the cases when the disease is not improving conservatively, the surgical procedure can be considered.<br /><br /></p>http://vestnik.szd.si/index.php/ZdravVest/article/view/1822cystic fibrosisneonatal jaundiceneonatal cholestasis |
| spellingShingle | Nataša Milivojevič Marjeta Sedmak Gordana Logar-Car NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS Zdravniški Vestnik cystic fibrosis neonatal jaundice neonatal cholestasis |
| title | NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS |
| title_full | NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS |
| title_fullStr | NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS |
| title_full_unstemmed | NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS |
| title_short | NEONATAL JAUNDICE – A RARE FORM OF CYSTIC FIBROSIS |
| title_sort | neonatal jaundice a rare form of cystic fibrosis |
| topic | cystic fibrosis neonatal jaundice neonatal cholestasis |
| url | http://vestnik.szd.si/index.php/ZdravVest/article/view/1822 |
| work_keys_str_mv | AT natasamilivojevic neonataljaundicearareformofcysticfibrosis AT marjetasedmak neonataljaundicearareformofcysticfibrosis AT gordanalogarcar neonataljaundicearareformofcysticfibrosis |