Incidence, prognosis, and etiology of end-stage liver disease in patients receiving home total parenteral nutrition

Background. Elevations in liver function tests have been reported in patien ts receiving total parenteral nutrition (TPN). The clinical aspects of end- stage liver disease (ESLD) associated with the prolonged use of home TPN ha ve not been fully clarified. In previous series patients with duodenocolost omies appeared to be at higher risk than persons with some jejunum or ileum remaining in situ. Methods. The records of 42 patients treated with home TPN for more than 1 y ear were examined. This constituted 283 person-years of home TPN. Patients with duodenocolostomies were examined as a separate group an the basis of t he the literature experience. Results. Six of 42 patients who received chronic home TPN had ESLD with 100 % subsequent mortality, at an average of 10.8 +/- 7.1 months after the init ial bilirubin elevation. Thirteen of 42 patients had superior mesenteric ar tery or vein thrombosis (SMT) leading to duodenocolostomy. In 8 of these 13 patients with SMT and underlying inflammatory or malignant disorder, 2 had ESLD. The remaining 5 SMT patients who had only minimal liver enzyme eleva tion over 13.6 +/- 6.7 (range 3 to 19) years of home TPN were significantly younger (36 +/- 7 years vs 64 +/- 13 years) and did not have underlying in flammation either by clinical diagnosis or as reflected in the high normal serum albumin level (greater than or equal to 4.0 g/dL). Despite their extr eme short bowel syndrome and long exposure to home TPN, ESLD did not develo p. Conclusions. Approximately 15% of patients who receive prolonged TPN have E SLD with a high rate of morbidity and mortality. The combination of chronic inflammation and the short bowel syndrome appears to be necessary for the development of ESLD with prolonged home TPN.