In a prospective study, we have examined the effect of nutritional status, using anthropometric measurement, on outcome in 102 consecutive adult patients undergoing elective orthotopic liver transplantation. Mid-arm muscle circumference was calculated from these two measurements. Patient outcome variables were time spent in the intensive therapy unit, total time in hospital, infective complications and mortality within 6 months. Graft outcome variables were early graft function, peak aspartate transaminase, alkaline phosphatase, bilirubin and prothrombin time. Group A patients were below and group B patients above the 25th percentile for mid-arm circumference and triceps skin fold thickness. Eighty-four patients (79 %) were at or below the 25th percentile of anthropometric measurements and 30 patients (28 %) were below the 5th percentile. The median mid-arm muscle circumference in group A was 22.3 (range 16.4-28.9) cm and 25.7 (range 21.7-31.8) cm in group B. The medial albumin level was similar in the two groups. There were significantly more bacterial infec- tions in group A (27/84,32 YO) than in group B (2/22,8 %; x2 = 5.4, P = 0.02). There was a difference in mortality up to 6 months post-operatively that failed to reach statistical significance (Wilcoxon-Gehan statistic -199, P = 0.09). There were 11/84 (13 Yo) deaths in group A and no deaths in group B (x2 = 2.8, P = 0.09). Post transplantation, there were significant differences (Kruskal-Wallis Anova) between groups A and B for peak alkaline phosphatase (683 vs 334 IUD, P = 0.05) and peak prothrombin time [16 (range 13-25) vs 19.5 (range 12-65), P = 0.031. These data suggest that a significant proportion of patients undergoing liver transplantation are nutritionally compromised and that this has effects on patient infection, susceptibility, graft function and mortality, which may possibly be improved by nutritional intervention.

In a prospective study, we have examined the effect of nutritional status, using anthropometric measurement, on outcome in 102 consecutive adult patients undergoing elective orthotopic liver transplantation. Mid-arm muscle circumference was calculated from these two measurements. Patient outcome variables were time spent in the intensive therapy unit, total time in hospital, infective complications and mortality within 6 months. Graft outcome variables were early graft function, peak aspartate transaminase, alkaline phosphatase, bilirubin and prothrombin time. Group A patients were below and group B patients above the 25th percentile for mid-arm circumference and triceps skin fold thickness. Eighty-four patients (79 %) were at or below the 25th percentile of anthropometric measurements and 30 patients (28 %) were below the 5th percentile. The median mid-arm muscle circumference in group A was 22.3 (range 16.4-28.9) cm and 25.7 (range 21.7-31.8) cm in group B. The medial albumin level was similar in the two groups. There were significantly more bacterial infec- tions in group A (27/84,32 YO) than in group B (2/22,8 %; x2 = 5.4, P = 0.02). There was a difference in mortality up to 6 months post-operatively that failed to reach statistical significance (Wilcoxon-Gehan statistic -199, P = 0.09). There were 11/84 (13 Yo) deaths in group A and no deaths in group B (x2 = 2.8, P = 0.09). Post transplantation, there were significant differences (Kruskal-Wallis Anova) between groups A and B for peak alkaline phosphatase (683 vs 334 IUD, P = 0.05) and peak prothrombin time [16 (range 13-25) vs 19.5 (range 12-65), P = 0.031. These data suggest that a significant proportion of patients undergoing liver transplantation are nutritionally compromised and that this has effects on patient infection, susceptibility, graft function and mortality, which may possibly be improved by nutritional intervention.

Case reportA woman with type I11 glycogen storage disease (GSD) presented at the age of 26 with tiredness and episodes of pallor and sweating when fasting. GSD had been diagnosed in early life on the basis of liver histology, red cell glycogen level and a glucagon challenge test. Type I11 GSD had been confirmed at the age of 10 by measurement of white cell amylo-1,6-glucosidase, but she had taken no dietary precautions since the age of 13. Examination showed normal height and weight, and no peripheral stigmata of disease other than smooth hepatomegaly. Routine investigations including full blood count, coagulation studies, urea and electrolytes, liver function tests and fasting cholesterol and triglycerides were normal with the exception of an elevated aspartate transaminase of 127 U/L (normal < 40), and an elevated serum creatine kinase of 1817 U/L (normal < 150) suggesting the subtype IIIa GSD. Abdominal ultrasound revealed a large liver with bright echotexture, whilst electrocardiogram and echocardiogram appeared normal.After further dietary advice and implementation of cornstarch supplements (70g late evening, equating to 1 g/kg or approximately 4 mg/kg/min over a 4-hour period) her symptoms improved. Within a few months she became pregnant for the first time. At 18 weeks of gestation she was admitted for a two-day metabolic profile with and without late night cornstarch supplements. During the first night, without a cornstarch supplement, there was evidence of significant lipolysis and ketosis with peak free fatty acids of 1000 pmol/L and a plasma 3-hydroxybutyrate (30H-B) level of 1050 pmol/L (normal fasting values