Showing papers by "Michael A. Heneghan published in 2009"

Spontaneous rupture of hepatocellular carcinoma: a Western experience.

Abstract: Background Spontaneous rupture of hepatocellular carcinoma (HCC) is a life-threatening presentation, with an incidence of Methods A retrospective review was performed of all patients who presented with ruptured HCC between 1995 and 2005. Data on clinical features, treatment strategies, and survival outcomes were collected. Statistical methods included univariate analysis and Kaplan-Meier survival estimates with log-rank test. Results A cohort of 21 patients (15 male and 6 female) was identified. Fourteen (66.6%) patients had histologic evidence of underlying cirrhosis, ad the median age at presentation was 68 years (interquartile range [IQR] 61 to 69). Ten of these patients (71.4%) were hemodynamically unstable at presentation. The mean tumor size was 8.5 cm (range 3 to 13), and there was multifocal disease in 6 (42.8%) patients. The etiology of cirrhosis was hepatitis B infection in 3, hepatitis C in 3, alcohol in 4, and cryptogenic in 4 patients. Initial bleeding control was attempted by transarterial embolization (TAE) in 7 (50%) and by emergency surgery in 7 patients (50%). Four of the operations were performed at referring hospitals, and 3 were performed at our institution. Two patients (14.2%) underwent palliative treatment only. Definitive treatment included resection at emergency surgery in 1, staged hepatectomy in 1, and transarterial chemoembolization in 2 patients. There were 7 patients who were noncirrhotic and had a median age of 51 years (IQR 42 to 60). Of these, 6 (87.5%) were hemodynamically unstable at presentation. Mean tumor size was 9 cm (range 6 to 18) and confined to right lobe in all patients. Primary hemostasis was successfully achieved by TAE in 2 and perihepatic packing in 1 patient. Definitive treatment was provided by emergency hepatectomy in 4 and staged hepatectomy in 3 patients. Patients with cirrhosis (n = 14) had a median survival rate of P Conclusions Spontaneous rupture of HCC is an uncommon presentation in Western countries. Primary hemostasis, followed by emergency or staged hepatic resection, is the treatment of choice. Median survival in patients initially treated with surgery was better than that observed in patients who underwent initial TAE, although this was not statistically significant. Patients who had no underlying liver disease had better prognosis than those who had cirrhosis.

Outcome of liver transplantation in hereditary hemochromatosis

Abstract: Summary Hereditary hemochromatosis (HH) is a genetic disorder of iron metabolism. It is an uncommon indication for liver transplantation (LT). It has been suggested that patients who undergo LT for cirrhosis related to HH have higher morbidity and mortality from cardiac, infectious and malignant complications. The purpose of this retrospective review was to determine whether these observations hold true in the current era. We analysed the data of 22 patients who had LT for HH from 1996 to 2007 at our center. Thirteen patients had LT for complications of end-stage liver disease, seven for hepatocellular carcinoma (HCC) and two for subacute liver failure. Cofactors promoting liver disease were identified in 15 patients. Ten patients had iron reduction with venesection before transplantation. Patient and graft survival at 1 and 5 years were 80.7%, and 74% respectively. There were seven deaths after a median follow up of 46 months either because of multiorgan failure, or caused by HCC recurrence. Bacterial infections were the commonest cause of morbidity. Patients with HH remain at a higher risk of developing HCC. Infectious complications are common. Iron reduction with preoperative venesection reduces the risk of cardiac and infection complications postoperatively. Improved survival post-LT reflects changes in selection, disease modification through venesection, and improvement in immunosuppression.

Normal neurological outcome in two infants treated with exchange transfusions born to mothers with Crigler-Najjar Type 1 disorder

Abstract: Patients with Crigler-Najjar Type 1 (CN-1) disorder have an unconjugated hyperbilirubinaemia due to the complete absence in activity of uridinediphosphate glucuronosyltransferase, a bilirubin-conjugating enzyme. In pregnant women with CN-1, the foetus is at high risk of being adversely affected by the bilirubin, as unconjugated bilirubin can cross the placenta and is potentially neurotoxic. We report the long-term outcomes of two infants born to women with CN-1. These infants had exchange transfusions soon after birth and have normal neurodevelopmental outcomes at 18 months and four years of age, respectively. We propose that this intervention might have improved the neurological outcome of these infants.