Effect of Plasmapheresis on Treatment of Acute Pancreatitis in Infant with Familial Chylomicronemia
Published on: 2017-01-26
Familial chylomicronemia is a rare genetical disorder. (autosomal recessive)with incidence 1/ 1000,000 that there is difficulty in clearance serum content of triglyceride & cholesterol due to deficiency of Apo-B-lipoprotein lipase or lipoprotein lipase(LPL) or cofactor Apo-C II. Different between them is decreased the level of serum TG after prescription FFP in Apo-CII and without response in LPL deficiency. Clinical features are Hepatosplenomegaly, Pancreatitis attack, Xantem eruptions on limbs & buts, Paleness of retina (libemia reticolaris). We introduce a female baby 41 days old that referred by hyperlipidemia (TG=25000mg/dl & cholesterol=1500mg/dl) and acute pancreatitis attack and signs & symptoms denoting to familial chylomicronemia who treated by plasmapheresis.