Liver transplantation is an accepted modality of treatment in some patients with inborn errors of metabolism. We present a child with methylmalonic acidaemia who had successful liver transplantation. The pathophysiology, medical and anaesthetic managements are discussed.

The use of aprotinin to reduce blood loss by inhibiting fibrinolysis thereby decreasing transfusion requirements during orthotopic liver transplantation (OLT), is well-documented in adults. We set out to test the hypothesis that the prophylactic use of aprotinin reduced blood product requirements during pediatric OLT. A retrospective study was performed, reviewing data from 24 OLTs performed over a 4-yr period. Six patients did not receive aprotinin (group 1), while 18 (group 2) received a weight-based dose of aprotinin. Both groups were comparable with respect to demographics, baseline characteristics and surgical variables except for a significantly more prolonged activated partial thromboplastin time (APTT) in the aprotinin group (p = 0.015). Despite the fact that median values for transfused volumes of red blood cells (78.3 vs. 36.7 mL/kg) and fresh frozen plasma (51.9 vs. 23.7 mL/kg) were more than halved in the aprotinin group, there was no statistical difference demonstrated. The failure to reach statistical significance can probably be explained by the small number in group 1 and a high level of scatter. All patients in group 1 required intraoperative transfusion of RBC and fresh frozen plasma (FFP) while two patients in group 2 did not require RBC and seven received no FFP. There were four patients in group 1 and 17 in group 2 who did not receive platelets while five in group 1 and 12 in group 2 did not receive cryoprecipitate. The differences between the groups in avoidance of these blood products did not reach statistical significance. There was little difference between groups with respect to albumin and crystalloid requirements. No statistical difference was demonstrated in intraoperative hematologic profiles between the two groups except during the anhepatic phase of surgery when there was a statistically significant more prolonged prothrombin time (p = 0.04) and a greater international normalized ratio (p = 0.027) in group 2.

We report a case of superior vena cava obstruction in a child, which was probably secondary to long-term central venous cannulation. The obstruction was asymptomatic preoperatively, but became evident during liver transplantation, and complicated the intraoperative management. There is one other case report of this occurring in an adult in similar circumstances, and we believe that ours is the first report of such a presentation in the paediatric age group.

Like many medical texts offered in Australia, this book actually is published for a North American audience. Drug names may be unfamiliar, and some drugs may be unavailable in Australia. In addition, some of the contraindications reflect the litigationconscious North American approach to medicine. For example, the authors suggest that promethazine is contra-indicated in pregnancy, whereas this drug is used fairly frequently during labour in Australia. I can recommend this book to Departments of Anaesthesia and specialists as a useful manual, particularly with respect to non-anaesthetic drugs. K. LESLIE Melbourne, Vic.

No abstract