Safety and efficacy of N-acetylcysteine in children with non-acetaminophen-induced acute liver failure.

Acute liver failure (ALF) carries a high mortality in children. N-acetylcysteine (NAC), an antioxidant agent that replenishes mitochondrial and cytosolic glutathione stores, has been used in the treatment of late acetaminophen-induced ALF and non-acetaminophen-induced ALF. In our unit, NAC was introduced as additional treatment for non-acetaminophen-induced ALF in 1995.

Polyclonal T-cell responses to cytochrome P450IID6 are associated with disease activity in autoimmune hepatitis type 2.

Background & Aims: Autoimmune hepatitis type 2 (AIH-2), a severe juvenile liver disorder of unknown etiology and pathogenesis, is characterized by liver-kidney microsomal antibody type 1 targeting cytochrome P450IID6 (CYP2D6) and is associated to HLA DRB1*07. Although CYP2D6 B-cell reactivity has been extensively characterized, little is known about CYP2D6-specific T-cell responses. The aim of the present study was to characterize anti-CYP2D6 cellular immune responses and their possible pathogenic role in patients with AIH-2.

Neonatal hemochromatosis--medical treatment vs. transplantation: the king's experience.

The aim of our study was to compare the outcome of medical treatment vs. liver transplantation in infants with neonatal hemochromatosis (NH) referred to King's College Hospital from 1990-2002. We conducted a retrospective review of 19 children from 14 families.

Effect of CD4+ CD25+ regulatory T-cells on CD8 T-cell function in patients with autoimmune hepatitis.

Background And Aims: CD4 T lymphocytes constitutively expressing the IL-2-receptor alpha-chain (CD25) (T-regs) are central to self-tolerance maintenance, preventing the proliferation and effector function of autoreactive T-cells. In autoimmune hepatitis T-regs are defective in number but maintain the ability to suppress IFNgamma production by CD4+CD25- T-cells. We have studied the ability of CD4+CD25+ (T-regs) to regulate proliferation and cytokine production by CD8 T-cells in patients with autoimmune hepatitis at diagnosis and during remission.

Risk factors for fungal infection in paediatric liver transplant recipients.

Fungal infection (FI) is a major and potentially fatal complication in liver transplantation (LT). Published experience of FI in paediatric LT is limited. We therefore reviewed case records of 79 children, aged between 0.

Wilson's disease in children: 37-year experience and revised King's score for liver transplantation.

Wilson's disease (WD) is a rare liver-based disorder of copper metabolism. Prognostic criteria described by our group in 1986 to predict death without transplantation have not been universally validated. The clinical features of 88 children were reviewed, retrospectively in 74 and prospectively in 14.

Cystatin C, an easy and reliable marker for assessment of renal dysfunction in children with liver disease and after liver transplantation.

Renal dysfunction of variable severity is being increasingly recognized as a major complication of calcineurin inhibitors (CI), in some patients even necessitating renal transplantation. Close and effective monitoring of the renal function is indicated. Current methods for this monitoring are calculation of the glomerular filtration rate (GFR) based on creatinine or exogenous substances like 51Cr-EDTA.

Approaches to acute liver failure in children.

Acute liver failure (ALF) is a rare but often fatal disorder in childhood. Its aetiology includes infections, toxins, metabolic disorders, infiltrative diseases, autoimmune hepatitis, ischaemia, irradiation damage, but in a high proportion of cases it remains unknown. In contrast to adults, in children with ALF hepatic encephalopathy can be a late event, and may not develop at all, despite a lethal outcome, particularly in infants.

The mortality index for neonatal transportation score: a new mortality prediction model for retrieved neonates.

Objective: To develop a mortality prediction score for retrieved neonates based on the information given at the first telephone contact with a retrieval service.

Methods: Data from the New South Wales Newborn and Pediatric Emergency Transport Service database were examined. Analysis was performed with the results for 2504 infants (median gestational age: 36 weeks; range: 24-43 weeks) who were <72 hours of age at the time of referral and whose outcome (neonatal death or survival) was known.

Impairment of CD4(+)CD25(+) regulatory T-cells in autoimmune liver disease.

Background/aims: CD4(+) lymphocytes constitutively expressing the IL-2-receptor alpha-chain (CD25) regulate the activation of CD4 and CD8 autoreactive T-cells by suppressing their proliferation and effector function. The aim of this study is to: (1) measure the percentage of CD4(+)CD25(+) T-cells (T-regs) in patients with autoimmune liver disease at presentation and during remission, (2) correlate their frequency with disease activity, (3) determine their ability to expand and (4) to inhibit interferon-gamma (IFNgamma) production by CD4(+)CD25- T-cells.

Methods: 41 patients were studied.

Exhaled nitric oxide levels in infants with chronic lung disease.

Unlabelled: Chronic lung disease (CLD) is an inflammatory disorder; in patients with other inflammatory disorders exhaled nitric oxide (NO) levels are elevated. The aim of this study was to test the hypothesis that prematurely born infants with CLD would have elevated exhaled NO levels compared to those without CLD and healthy term-born infants. Ten infants with CLD (median gestational age 26 weeks; CLD group), ten infants without CLD (median gestational age 32 weeks; non-CLD group) and ten term-born infants (term group) were examined at post-conceptional ages between 36 and 45 weeks.

An illness severity score and neonatal mortality in retrieved neonates.

Unlabelled: The Clinical Risk Index for Babies (CRIB) score is a simple tool to measure clinical risk and illness severity in very low birth weight infants. The aim of this study was to determine if a modified CRIB score (MCRIB) used at first telephone contact with a transport service differentiated between retrieved infants who did or did not die in the neonatal period and hence might be a useful triage tool. A retrospective cohort study of 2504 infants, median gestational age 36 weeks and birth weight 2782 g, transported by the New South Wales Newborn and Paediatric Emergency Transport Service (NETS) was performed.

Can energy expenditure be predicted in critically ill children?

Objective: To determine whether critically ill children are hypermetabolic and to calculate whether predictive equations are appropriate for critically ill children.

Design: Prospective, clinical study.

Setting: Pediatric intensive care unit.

Bone mineral density and height gain in children with chronic cholestatic liver disease undergoing transplantation.

Background: Osteodystrophy is a well-described complication of chronic liver disease. Previous reports in adults and children undergoing liver transplantation (LT) were discordant, with the former showing no improvement of bone disease in the first year after transplantation and the latter demonstrating remarkable benefit from it. Our aim was to perform a pilot study on osteodystrophy in children undergoing LT and evaluate the contribution of growth on bone mineral density (BMD) changes.

Colloid infusion in the perinatal period and abnormal neurodevelopmental outcome in very low birth weight infants.

Unlabelled: In very low birth weight (VLBW) infants, colloid infusion is associated with impaired perinatal lung function and increased oxygen dependency duration. The aim of this study was to determine whether perinatal colloid infusion was associated with abnormal neurodevelopmental outcome. All perinatal fluid input (crystalloid and colloid) given to VLBW infants entered into a randomised trial was recorded.