Abdullah ÖZYURT, Özge PAMUKÇU, Nazmi NARİN, Kazım ÜZÜM, Sadettin SEZER, Mustafa ARGUN, Ali BAYKAN

Cutting balloon angioplasty and stent implantation for left pulmonary artery stenosis in a case with Alagille syndrome

Alagille sendromu intrahepatik safra kanallarının gelişim bozukluğu, kalp, iskelet, göz, böbrek anormallikleri ve karakteristik yüz görünümü ile ilişkili otozomal domi- nant geçişli nadir bir hastalıktır. Alagille sendromlu hastalarda en sık görülen kalp anomalisi periferik pul- moner arter darlığıdır. Bu yazıda, yenidoğan döneminde kolestatik sarılık, posterior embriyotakson, karakteristik yüz görünümü ve periferik pulmoner arter darlığı eşlik etmesi nedeniyle Alagille sendromu tanısı konulan bir olgu sunuldu. Dört yaşında sol periferik pulmoner arter darlığının derecesinin artması üzerine yapılan kesici balon anjiyoplasti işlemi ve stent yerleştirme ile sempto- matik iyileşme sağlandı

Alagille sendromlu bir olguda sol pulmoner arter darlığına kesici balon anjiyoplasti ve stent yerleştirilmesi

Alagille syndrome is a rare autosomal dominant disorder associated with an impaired development of intrahepatic bile ducts, cardiac, skeletal, eye, kidney disorders, and characteristic facial appearance. Peripheral pulmonary stenosis is the most frequently seen cardiac anomaly in patients with Alagille syndrome. In this article, we report a case who was diagnosed with Alagille syndrome due to the coexistence of neonatal cholestatic jaundice, posterior embryotoxon, characteristical facial appearance, and peripheral pulmonary stenosis. Symptomatic relief was achieved by cutting balloon angioplasty and stent implantation applied when the degree of the left pulmonary stenosis increased at four years of age

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