Dupuis-Girod S.,Service de Genetique |
Dupuis-Girod S.,University of Lyon |
Chesnais A.-L.,Service de Genetique |
Ginon I.,Center Hospitalier Lyon Sud |
And 15 more authors.
Liver Transplantation | Year: 2010
Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to hepatic veins, hepatic artery to portal vein, and portal vein to hepatic vein. Hepatic involvement in HHT may lead to biliary ischemia, portal hypertension, or high-output cardiac failure (HOCF). Orthotopic liver transplantation (OLT) has been proposed as the only definitive curative treatment. The aim of this study was to evaluate the long-term outcome of patients with hepatic involvement due to HHT after OLT with respect to mortality, cardiac and hepatic status, epistaxis, and quality of life. Patients with HHT and severe hepatic vascular malformations who underwent OLT in the Lyon Liver Transplant Unit (LLTU) from 1993 to 2007 were followed at the LLTU and the French Reference Center for HHT. Quality of life was evaluated with the Short Form 36 questionnaire. There were 13 patients who fulfilled the entry criteria of the study (12 women and 1 man). The mean age at the time of OLT was 51.8 years (range = 33-65 years). Indications for OLT were cardiac failure (n = 9), biliary necrosis (n = 2), both cardiac failure and biliary necrosis (n = 1), and hemobilia (n = 1). The mean duration of follow-up was 109 months (range = 1-200 months). Twelve patients (92.3%) are still alive. For the 9 patients with HOCF, the mean cardiac index decreased from 5.4 L/minute/m2 before OLT to 3.0 L/minute/m2 after OLT. No severe hepatic complications were observed after OLT. Nine of the surviving patients (75%) experienced dramatic improvements in epistaxis and quality of life, including an ability to undertake more physical activity. In conclusion, OLT is an important therapeutic option for patients with HHT who have severe hepatic involvement. In the reported cohort, the mortality after OLT for this indication was low. © 2010 AASLD. Source
Huissoud C.,Hospices Civils de Lyon Service de Gynecologie Obstetrique |
Huissoud C.,French Institute of Health and Medical Research |
Dupont C.,Pole IMER Information Medicale |
Canoui-Poitrine F.,Pole IMER Information Medicale |
And 5 more authors.
European Journal of Obstetrics Gynecology and Reproductive Biology | Year: 2010
Objectives: To determine the interval between decision and delivery (DDI) for urgent and very urgent caesarean deliveries within a perinatal network, to compare the results according to maternity ward level and organisation, and to assess the impact of DDI on neonatal outcome. Study design: Prospective observational study in the 31 maternity units of the Aurore perinatal network (17 Level I, 12 Level II, and two Level III). The obstetric team defined the degree of urgency for the caesareans, measured the DDI, and reported neonatal outcome. Results: The study includes 666 unplanned caesarean sections. The median DDI for emergency caesareans (n = 365) was 48 min for Level I units, 40 min for Level II, and 22 min for Level III (P < 0.05). For the very urgent caesareans (n = 82), the median DDI was respectively 35, 24, and 13 min (P < 0.05) and the percentage with a DDI ≤ 30 min were 45%, 62%, and 100% (P < 0.05). The proportion of DDI ≤ 30 min was 0% in maternity units where obstetricians and anaesthetists were not always onsite, 67% when only the anaesthetist was always present (P < 0.05) and 88% for units where both were always present. The neonate's condition did not differ significantly according to DDI. Conclusions: DDI varies very substantially according to the level and organisation of the maternity units in the Aurore network. It was not significantly correlated with neonatal outcome in our population. © 2010 Elsevier Ireland Ltd. All rights reserved. Source