id	author	title	date	pages	extension	mime	words	sentence	flesch	summary	cache	txt
anesthj-126	Patson, Amber Lee Madrid	Carnitine Palmitoyl Transferase, Type 1A (CPT1A): The Arctic Variant	2019	2	.pdf	application/pdf	2434	108	47	The child may need hospital admission to correct metabolic status by receiving intravenous (IV) or nasogastric (NG) administration of glucose.13 A standard maintenance rate of dextrose containing IV fluids is usually sufficient.13 Intra-operatively: • Blood glucose levels should be checked and maintained.14 • Inpatient AV- CPT1A children should continue glucose- containing IV fluids intra-operatively. • CPT1A is the isoform of CPT1 found in the liver.1 • CPT1A catalyzes the rate limiting step that imports long chain fatty acids into the mitochondrion, allowing for the production of ketones and subsequent ATP energy from the breakdown of fat.2 • CPT1A Arctic Variant is an inherited autosomal recessive variant resulting in 80% decreased activity of the CPT1A enzyme.3 • The c.1436C>T variant occurs in 26% - 80% of Arctic populations including Alaska, Canada, Greenland and Siberia.4,5 Background Physiology: • Normally, during periods of fasting, the body initially breaks down the hepatic glycogen supply into glucose.4	cache/anesthj-126.pdf	txt/anesthj-126.txt