Encephalopathy in type I hyperlipidemia.
Indian pediatrics, cilt.44, sa.4, ss.306-8, 2007 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 44 Sayı: 4
- Basım Tarihi: 2007
- Dergi Adı: Indian pediatrics
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.306-8
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
Özet
Familial chylomicronemia syndrome is a group of rare genetic disorders characterized by deficient activity of an enzyme lipoprotein lipase or apo-protein C-II deficiency. In this paper we present an infant with massive hyperchylomicronemia and severe pancreatitis. Exchange transfusion for controlling hypertriglyceridemia and pancreatitis led to an increase in hyperviscosity which resulted in encephalopathy.