NURS 216 Lecture Notes - Lecture 24: Alipogene Tiparvovec, Kernicterus, Bilirubin
24 Jan 2020
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Document Summary
Caused by mutation in the lpl gene, which breaks down triglycerides. Glybera - gene therapy treatment for individuals with familial lpl deficiency delivers intact copy of lpl gene to the muscle cells via a viral vector. The lower the gestational age, the higher the risk. Unconjugated bilirubin is toxic to the cells. Onset within 2-3 days, peak within 3-5 days. Kernicterus: bilirubin encephalopathy bilirubin exceeds albumin binding capacity crosses bbb. Breastfeeding (although breastmilk can contribute to jaundice) Phototherapy bilirubin transformed by blue or blue-green light to photo bilirubins hydrophilic isomers that are easily excreted hydrate due to insensible water losses filtered sunlight also works. Iron supplements: ferrous sulphate po/iv/im, ferrous fumarate, ferrous gluconate. 90% bioavailability ideally taken on an empty stomach side effects: gi pain, constipation (from decreased peristalsis), drug to drug or food interaction. Deficiency: calculated based on lean body weight and hgb. Hemorrhage replacement: iron mg = ml (blood loss) x hct.
