Re-evaluation of the diagnostic criteria for Wilson disease in children with mild liver disease.

Unlabelled: The diagnosis of Wilson disease (WD) is challenging, especially in children. Early detection is desirable in order to avoid dramatic disease progression. The aim of our study was to re-evaluate in WD children with mild liver disease the conventional diagnostic criteria and the WD scoring system proposed by an international consensus in 2001.

Cholestasis in neonatal intensive care unit: incidence, aetiology and management.

Aim: Prevalence, aetiology, management and outcome of cholestasis were evaluated in infants admitted to neonatal intensive care unit (NICU).

Methods: Medical records of all infants admitted to two Italian level III NICUs from January 2005 to August 2007 were retrospectively reviewed. The role of ursodeoxycholic acid (UDCA) therapy was also investigated.

Genotype-phenotype correlation in Italian children with Wilson's disease.

Background/aims: Wilson's disease phenotype is very variable for clinical and laboratory features. Our aim was to assess the role of the type of ATP7B disease causing mutations on Wilson's disease phenotype.

Methods: We retrospectively evaluated the data of children with Wilson's disease from eight pediatric departments.

Long-term outcome in children with chronic hepatitis B: a 24-year observation period.

Background: Chronic hepatitis B seems to manifest as mild disease in children and young adults. However, data regarding the long-term course of hepatitis B in untreated and interferon-treated children are still scarce. This study investigates the long-term outcome of disease in a large series of untreated and treated children with hepatitis B virus (HBV) infection.

Is HCV infection associated with liver steatosis also in children?

Background/aims: Prevalence and significance of steatosis in children with chronic hepatitis C are not well defined. We analysed the prevalence of steatosis in children with chronic hepatitis C and its relationship with clinical, laboratory features and response to interferon.

Methods: Sixty-four consecutive children with CHC undergoing liver biopsy were retrospectively evaluated.

Evolution of hepatitis C viral quasispecies and hepatic injury in perinatally infected children followed prospectively.

Perinatal infection with hepatitis C virus (HCV) is characterized by a wide range of alanine aminotransferase (ALT) levels. The mechanisms responsible for this variability are unknown. We examined whether the evolution of the HCV quasispecies was associated with different ALT profiles in perinatally infected children.

Long-term lamivudine therapy for children with HBeAg-positive chronic hepatitis B.

One year of lamivudine treatment results in increased hepatitis B e antigen (HBeAg) seroconversion and serum hepatitis B virus (HBV) DNA negativity in children with chronic hepatitis B and high serum alanine aminotransferase concentrations. Two hundred seventy-six children who participated in a 1-year randomized, placebo-controlled study of lamivudine were enrolled in a 24-month, open-label extension. Patients were stratified into two groups based on HBeAg status at week 48 of the previous study: 213 HBeAg-positive children were entered into a treatment arm, and 63 HBeAg-negative children were entered into an observation arm to evaluate durability of HBeAg loss.

Immune phenotype and serum leptin in children with obesity-related liver disease.

Context: Little is known about pathogenesis of obesity-related liver disease in childhood. Data on the relationship among leptin, immunological parameters, and liver disease in obese children are lacking.

Objective: Thus, the objective of this study was to evaluate immune phenotype and leptin serum levels in obese children with and without obesity-related liver disease.

Chronic hepatitis C in childhood: an 18-year experience.

Background: The long-term outcome of chronic hepatitis C (CHC) has not been well studied, both for untreated and interferon-treated children. The aim of this study was to evaluate the long-term outcome of disease in a large series of children with CHC.

Methods: Clinical, biochemical, virological, and histological features were evaluated in all children (age, 2-18 years) with CHC who did not have concomitant disease and who attended at our hospital's liver unit during the period of 1986-2004.

Hypertransaminasemia in childhood as a marker of genetic liver disorders.

Background: The widespread use of routine biochemical assays has led to increased incidental findings of hypertransaminasemia. We aimed to evaluate the prevalence of different causes of raised aminotransferase levels in children referred to a university department of pediatrics.

Methods: We investigated 425 consecutive children (age range, 1-18 years) with isolated hypertransaminasemia.

Serum transaminases in children with Wilson's disease.

Objectives: The response of serum transaminase levels to penicillamine and zinc treatment in Wilson's disease is poorly understood. The aim of this multicenter retrospective study was to evaluate transaminase levels after penicillamine and zinc treatment in children with Wilson's disease.

Patients And Methods: One hundred and nine patients with Wilson's disease (median age at diagnosis, 7.

Severe Raynaud's phenomenon with chronic hepatis C disease treated with interferon.

Severe Raynaud's phenomenon developed in a 5-year-old girl with chronic hepatitis C infection at the fifth month of interferon therapy in the absence of cryoglobulinemia and other conditions commonly associated with secondary Raynaud's phenomenon. Although interferon therapy was promptly discontinued, Raynaud's phenomenon persisted for 4 months with appearance of necrotic-ulcerous lesions at the tips of fingers.

Noninvasive investigation of hepatopulmonary syndrome in children and adolescents with chronic cholestasis.

Early detection of hepatopulmonary syndrome (HPS) may be delayed because of invasiveness of the diagnostic procedures. In this pilot study, we prospectively investigated the usefulness of determining transcutaneous O(2) tension after 100% O(2) (TcPO(2)100) breathing using a transcutaneous hyperoxia test (THT) in 11 children with chronic cholestasis and without primary cardiopulmonary disease. These patients also underwent alveolar-arterial O(2) gradient testing (AaDO(2)) at an inspired oxygen fraction (FiO(2)) of 0.