Paediatric liver transplantation in Bergamo

A68

Abstracts / Digestive and Liver Disease 39 (2007) A49–A87

The lack of significant difference in survival rate with elective cases means that OLTx is a safe therapeutic option even in the most severe cases. doi:10.1016/j.dld.2007.07.098 PP 36 PAEDIATRIC LIVER TRANSPLANTATION IN BERGAMO M. Candusso, M.L. Melzi, P. Stroppa, M. Bravi, L. Cavalleri, M. Bosisio, F. Bruni, G. Torre, V. Corno, M. Guizzetti, A. Lucianetti, D. Pinelli, M. Zambelli, M. Colledan Pediatric Liver Transplantation Centre, Ospedali Riuniti, Bergamo, Italy We retrospectively studied 312 patients, undergone 350 OLTx from October 1997 till April 2007, at a median age of 3.5 years (range 29 days–17.3 years). Extrahepatic biliary atresia (EHBA) was present in 56% of cases (177 patients); Alagille’s syndrome in 8% (26 patients); primitive cirrhosis in 6% (18 patients); hepatic tumours in 4% (12 patients); metabolic diseases in 7% (22 patients); acute liver failure in 5% (17 patients); Byler’s disease in 4% (14 patients); secondary cirrhosis in 4% (11 patients); sclerosant cholangitis in 2% (5 patients); hepatic congenital fibrosis in 2% (5 patients); autoimmune hepatitis in 1% (4 patients) and graft-versus-host-disease in 1 patient. Immunusuppression therapy (IS) has been based on steroid and cyclosporine till 2000, and later on steroid for 4 months and tacrolimus (TAC). The median waiting time was 55 days (range 1–367 days). At transplant, the median weight was 14.3 kg (range 2–82 kg). The overall survival rate at 5 years is 86.2%; EHBA survival rate is 92%. The mean follow-up is 50.4 months. Split liver was performed in 75% of cases; whole liver in 20.8%; reduced liver in 4.2% of total cases. Kidney-liver transplants were needed in eight patients: for ossalosys oxaluria (one case), congenital hepatic fibrosis (two cases), familiar uraemic-haemolytic syndrome (two cases), methylmlonic acidaemia (one case) and for chronic renal insufficiency associated to biliary cirrhosis (two cases). Thirty-eight patients required re-transplant. Thirty-five patients needed 2 and 3 patients 3 grafts, with an overall survival rate of 60.5%. Biliary complications were reported in 34.3% of cases; vascular complications (stenosis or thrombosis of hepatic vessels) in 20.8%. Acute rejection was diagnosed in 33% of children, every case recovered after steroid bolus. Chronic rejection in 24.4%. PTLD was diagnosed in 13.7% of total cases; monomorphic form occurred in 17.9% of PTLD cases, after 24 months on average from OLTX; polymorphic PTLD was diagnosed in one case; in the majority of patients, only early lesions were reported. In each case of monoclonal PTLD, IS was stopped. Complete remission was reached in 97% of cases; survival rate at 41 months follow-up is 83.3%. doi:10.1016/j.dld.2007.07.099 PP 37 LIVER STEATOSIS IN PAEDIATRIC PATIENTS WITH CHRONIC HEPATITIS B: VIRAL AND HOST FACTORS A. Giannattasio a , F. Cirillo a , D. Liccardo a , V. Terlizzi a , G. Ranucci a , S. Rinaldi a , R. Vecchione b , R. Iorio a a b

Department of Pediatrics, University Federico II, Naples, Italy Department of Phatology, University Federico II, Naples, Italy

Background and aim. Hepatic steatosis is a common histological feature of chronic hepatitis C virus infection. The significance of liver steatosis in chronic hepatitis B (CHB) has not been clearly studied. Aim of the present study was to evaluate the prevalence of steatosis at liver biopsy in paediatric patients with CHB and to investigate factors associated to presence of steatosis. Patients and methods. The study included 56 (38 males; median age at liver biopsy 8 years, range 2.2–17.3) consecutive otherwise healthy children with CHB who underwent liver biopsy for diagnostic purposes at our Pediatric Liver Unit during a 14-year period. In all patients demographic data, clinical features, anthropometric and laboratory data (liver functional tests, fasting glucose, cholesterol and triglycerides) were evaluated at the time of

liver biopsy. As for liver histology, activity of necroinflammation and fibrosis staging were evaluated according to Ishak scoring system. Hepatic steatosis was graded semi-quantitatively by determining the percentage of affected hepatocytes (Brunt classification). Results. Fifty-four patients had no steatosis at liver biopsy. Steatosis of grade 1 (<33% of hepatocytes affected) was present in only 2 (3.6%) children (both males). The two groups were comparable for median age, sex, route of HBV infection. Mean relative BMI was significantly higher in the 2 patients with steatosis (132 ± 9) compared with the group without steatosis (109 ± 15) (P = 0.04). Among 54 patients without steatosis, 6 (11.1%) were overweight and 6 (11.1%) were obese. One of patients with steatosis was overweight and the other one was obese. Relative BMI did not statistically differ between the two patients with steatosis (126.4 ± 1.2) and 12 overweight/obese children without steatosis (125.7 ± 12.3). As for biochemical and virological parameters, no significant difference between patients with and without steatosis were found with regard to ALT, GGT, serum HBV DNA level. Further, cholesterol, triglycerides, fasting glucose serum levels were comparable between the two groups. Conclusions. Differently from chronic hepatitis C, histological evidence of steatosis is detectable only in a small percentage of paediatric patients with CHB. Although in CHB steatosis seems to be related to obesity rather than to viral factors, our data needed to be confirmed with larger scale studies. doi:10.1016/j.dld.2007.07.100 PP 38 IS THE EXCHANGE TRANSFUSION A POSSIBLE SPECIFIC TREATMENT FOR NEONATAL HAEMOCHROMATOSIS? E. Nicastro a , G. Timpani b , F. Foti b , A. Nicol`o b , M. Tufano a , A. Vicinanza a , R. Iorio a a b

Department of Pediatrics, University “Federico II”, Naples, Italy Neonatal Intensive Care Unit, Ospedali Riuniti, Reggio Calabria, Italy

Background. Neonatal haemochromatosis (NH) is a rare congenital disorder that affects the fetus in the late gestation, clinically defined as severe neonatal liver disease associated with extrahepatic siderosis. It is widely accepted that NH is the result of an alloimmune disorder that causes liver injury in the fetus: an alloantibody-mediated liver injury would lead to iron mishandling with resulting siderosis. Poor results have been observed with a treatment with antioxidants and an iron chelator. Although the effectiveness of the exchange transfusion (EXT) in the treatment of neonatal alloimmune diseases is well-known, the effectiveness of this approach in NH has never been evaluated. We report a second born of two siblings with NH successfully treated with EXT. Case report. The patient was a male born at 33 weeks of gestation by caesarean section. The familial anamnesis revealed a first sister died at 18 days for severe coagulopathy and acute renal failure. Apgar scores were 8 at 1 and 9 at 10 . He appeared to have mild generalized oedema and placental hydrops. Laboratory tests showed severe coagulopathy, hypoalbuminaemia and low platelet count. Aminotransferases and D-dimers were normal. The patient did not respond to the treatment with fresh frozen plasma, albumin, activated C protein and antithrombin III. Before achieving a definite diagnosis, the extremely severe coagulopathy and the unfavourable outcome of the elder sister led us to treat the child with an EXT as an intensive supportive care, with rapid clinical and laboratory improvement. Diagnosis of NH was based on elevated ferritin concentration of 2.219.25 ng/ml (N < 800 ng/ml); low transferrin concentration of 101 mg/dl (N 190–320 mg/dl); transferrin saturation 101% (N < 33%); serum AFP was 188,306.25; liver siderosis documented at MRI scanning. Placental biopsy revealed stainable iron in the specimens from either the patient and his elder sister, confirming the diagnosis of NH. Further supportive therapy consisted of continuative substitutive therapy, followed by a second EXT on the fifth day of life. The clear improvement of the patient at the moment of the diagnosis of NH, the lack of conclusive evidence about the efficacy of treatment with chelator/antioxidant cocktail and the risk of side effects associated to the iron-chelating therapy led us to treat the patient with n-acetylcysteine, vitamin E and selenium until a com-