Linoleate and possibly linolenate deficiency in a patient on long-term intravenous nutrition at home

A 39-year-old female with scleroderma was maintained on total parenteral nutrition (TPN) at home for four years. She received 2 units of a 10% fat emulsion per week in which 55% of the fatty acids were from linoleate and 7% from linolenate. She was initially placed on TPN because she had difficulty in swallowing due to scleroderma. At the end of four years she had a triene:tetraene ratio of greater than 1. There was evidence of alterations in membrane function due to essential fatty acid deficiency including CNS involvement (blindness, impaired hearing and disorientation) as well as respiratory insufficiency. The diagnosis of membrane involvement was made from gas chromatography (gc) and gc-mass spectroscopy (ms) analysis of red cell membranes which were deficient in linoleate. The patient's immediate cause of death was from respiratory insufficiency.