27 KB74 KB26 KB192920262003Logar-Car, GordanaMilivojevič, NatašaSedmak, Marjetaštevilka:4letnik:72str. 223-226ISSN:1318-0347COBISSID:16259801URN:URN:NBN:SI:doc-NSOMPRAOslSlovensko zdravniško društvoZdravniški vestnikCholestasisCistična fibrozaCystic FibrosisDiagnosisEtiologyHolestazaInfant, NewbornJaundice, NeonatalNovorojenčeknovorojenčkiPhysiopathologyUrsodeoxycholic AcidUrzodeoksiholna kislinazlatenicaZlatenica novorojenčkaZlatenica novorojenca - redka oblika pojavljanja cistične fibroze| Neonatal jaundice - a rare form of cystic fibrosis|Background. Cystic fibrosis is an autosomal recessive disorder that results indamage 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. 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. 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 consideredIzhodišča. Cistična fibroza je avtosomno recesivno dedna bolezen, ki povzroči motnjo v delovanju žlez z zunanjim izločanjem. Klinično se najpogosteje kaže sprizadetostjo dihal in trebušne slinavke. Pozno v poteku bolezni pogosto okvari tudi delovanje hepatobiliarnega sistema, izjemno redko pa povzroči nastanek zlatenice in druge znake zastoja žolča že v neonatalnem obdobju. Na Pediatrični kliniki v Ljubljani smo v desetletnem obdobju (1988-98) ugotovili dva primera, pri katerih je bila vzrok zlatenice novorojenca cistična fibroza.Bolniki in metode. Prispevek prikazuje deklici, pri katerih je bila zlatenica eden prvih znakov cistične fibroze. Prisotni so bili laboratorijski znaki zastoja žolča in prizadetosti jeter. Pri eni od deklic se je stanje popravilo spontano, pri drugi pa smo po zdravljenju z ursodeoksiholno kislino opazili pomembno izboljšanje kliničnega stanja in laboratorijskih kazalnikov zastoja žolča ter prizadetosti jeter. Zaključki. Zlatenica novorojenca s konjugirano hiperbilirubinemijo je v redkih primerih lahko posledica cistične fibroze. K zdravljenju je treba pristopiti konzervativno z ursodeoksiholno kislino, le v primeru, da se stanje ne izboljšuje, je treba razmišljati o kirurškem poseguTEXTznanstveno časopisjejournalsSlovenian National E-content AggregatorNational and University Library of SloveniaSlovensko zdravniško društvo