Bone marrow failure in children with acute liver failure.

Background: Aplastic anemia is a rare but well-recognized complication of acute hepatitis and acute liver failure. The cause is unknown, and the condition is fatal without bone marrow recovery. Treatment includes immunosuppression regimens or bone marrow transplantation.

Use of cadaveric superior mesenteric artery as interpositional vascular graft in orthotopic liver transplantation.

Background: Hepatic artery thrombosis remains the most common technical complication that causes graft failure following orthotopic liver transplantation. The development of split liver and living related liver transplantation has led to the use of shorter and smaller arteries for arterial reconstruction to the graft. The present aim was to assess the effectiveness of the superior mesenteric artery as an interpositional graft in arterial reconstruction during liver transplantation.

Quality of life after liver transplantation for alcoholic liver disease.

There are few data on predictive factors for alcohol relapse or long-term functional outcome after liver transplantation for alcoholic liver disease (ALD). In all 56 surviving UK patients (47 men, 9 women; mean age: 51 years; range: 33 to 69 years) who underwent transplantation for ALD at King's College Hospital over a 10-year period, alcohol relapse and outcome were assessed by outpatient and case-note review and by postal questionnaire containing (1) the Nottingham Health Profile (NHP), (2) the Short-Form-36 (SF-36) Health Survey, and (3) a drug and alcohol questionnaire. At a median of 2.

Recurrence of Langerhans cell histiocytosis in the graft after pediatric liver transplantation.

Two girls were diagnosed with Langerhans cell histiocytosis (LCH) at the age of 16 and 7 months and developed end stage chronic liver disease related to LCH-induced sclerosing cholangitis at 28 and 8 months, respectively. They received liver transplants at 34 and 14 months of age. Five months post-orthotopic liver transplantation (OLT) one of the patients developed posttransplant lymphoproliferative disease, successfully treated with a combination of surgery and reduction of immunosuppression.

Liver transplantation for alpha-1-antitrypsin deficiency in children.

Alpha-1-antitrypsin (a1-AT) deficiency is an inborn error of metabolism, which can cause liver disease. The condition is one of the most common genetic disorders in the Caucasian population. Here we review our experience with 21 children suffering from end-stage liver disease due to a1-AT deficiency.

Mycobacterium tuberculosis infection in pediatric liver transplant recipients.

Objectives: To study the incidence, clinical presentation, management, complications and outcome of tuberculosis in pediatric liver transplant recipients.

Methods: A retrospective review of the medical records of children who underwent liver transplantation between 1991 and 1998.

Results: Mycobacterium tuberculosis infection occurred in 6 of 254 (2.

Prognosis of alpha-1-antitrypsin deficiency-related liver disease in the era of paediatric liver transplantation.

Background/aim: Alpha-1-antitrypsin deficiency (alpha1ATD) is the commonest metabolic disease leading to liver transplantation (LT) in children. Approximately 10-15% of the PiZZ population develops liver disease. Five percent of them will require LT within the first 4 years of life.

Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children.

Liver dysfunction is a well-recognized complication of intestinal failure in children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortunately TPN may lead to cholestatic liver disease particularly in the young children.

Curative hepatorenal transplantation in systemic amyloidosis caused by the Glu526Val fibrinogen alpha-chain variant in an English family.

A 53-year-old English woman who had been thought to have systemic monoclonal immunoglobulin light chain (AL) amyloidosis was investigated further because of her unusually long 17-year history and a suggestion of renal disease in the family. She was found to have the Glu526Val fibrinogen alpha-chain variant that causes autosomal dominant hereditary systemic amyloidosis. This has not previously been described in a British family.

Size reduction of small bowels from adult cadaveric donors to alleviate the scarcity of pediatric size-matched organs: an anatomical and feasibility study.

Background: Small bowel transplantation in children weighing less than 10 kg is hindered by the lack of size-matched donors. The ability to create reduced size small bowel grafts from adult cadaveric donors suitable for use in young children has been studied.

Methods: Volumetric assessment of computed tomography scans were used to evaluate abdominal cavity and small bowel volumes in children.

Oral absorption of tacrolimus in children with intestinal failure due to short or absent small bowel.

We describe two children with intestinal failure due to short or absent small bowel who underwent isolated liver transplantation for liver disease related to parenteral nutrition. Both received reduced-size liver grafts whilst awaiting a suitable small bowel donor. Immunosuppressive therapy was based on oral tacrolimus and intravenous steroids.

Liver transplantation with monosegments.

Background: Shortage of size-matched pediatric donors led to the development of surgical techniques to reduce or split livers and thus increase the potential pool of donors. Despite this, neonatal transplantation remains a problem because of the small size of the recipients. Further reduction of the left lateral segment is possible to provide a single segment graft (segment III).

Auxiliary partial orthotopic liver transplantation for Crigler-Najjar syndrome type I.

Objective: To determine if auxiliary partial orthotopic liver transplantation (APOLT) has the long-term potential to correct the underlying abnormality in Crigler-Najjar syndrome type 1 (CNS1) without the need for total liver replacement.

Background: Orthotopic liver transplantation has been used successfully to replace the defective enzyme in CNS1. Experimental studies have shown that only 1% to 2% of the normal hepatocyte mass is needed for bilirubin conjugation.

Assessment of potential donors for living related liver transplantation.

Background: Living related liver transplantation has been developed as an important potential source of organs for treatment of children with acute and chronic liver disease. A single UK centre performing living related liver transplantation was established in 1993.

Methods: Parents who were potential donors for their children for living related liver transplantation were assessed for suitability according to a protocol based on one developed and published by the University of Chicago Transplant Group.

Splenic artery aneurysm and orthotopic liver transplantation.

Splenic artery aneurysms are a rare but potentially fatal complication after liver transplantation. We report three cases presenting in a 12-month period in adult patients who underwent transplantation for chronic liver disease. Doppler ultrasound of the splenic artery should be performed in all patients with cirrhosis and portal hypertension who are being assessed for liver transplantation.

Laparoscopic cholecystectomy injury: an unusual indication for liver transplantation.

The introduction of laparoscopic cholecystectomy has been associated with a rise in the number of reported bile duct injuries (0.3%-0.8%).

Hemophagocytosis: a potential complication in small bowel transplantation.

Background: Acquired hemophagocytosis is a histiocytic proliferation associated with phagocytosis of the hemopoietic elements resulting in fever, profound pancytopenia, hepatosplenomegaly, lymphadenopathy, and coagulopathy. Acquired hemophagocytic syndrome is a specific entity that can be differentiated from malignant histiocytosis and familial hemophagocytosis. It has been associated with a variety of disorders, including immunodeficiency, infection, hemopathies, cancer, and autoimmune diseases, as well as with the period following solid organ and bone marrow transplantation.

Accessory right hepatic artery arising from the left: implications for split liver transplantation.

Background: Variant arterial anatomy must be recognized and appropriately managed during split liver transplantation to ensure complete vascular supply to both grafts. We describe an accessory posterior right hepatic artery, arising from the left and passing behind the portal vein bifurcation.

Methods: Thirty-seven consecutive livers were examined during ex vivo liver-splitting procedures.

Gender matching and outcome after pediatric liver transplantation.

Background: Gender is not a selection criterion for orthotopic liver transplantation (OLT), and reports in adults have shown a less favorable outcome for male recipients of female organs; the only pediatric study did not support this finding. The aim of the present study was to assess the impact of donor and recipient gender on graft and patient survival rates after pediatric OLT.

Methods: We have reviewed retrospectively 137 children (male=63; median age: 3.

Bowel perforation after paediatric orthotopic liver transplantation.

Bowel perforation is a well-recognized complication following orthotopic liver transplantation. Of 194 paediatric liver transplantations performed in our hospital, 13 patients (6.7%) developed bowel perforation post-transplantation.

Diaphragmatic nerve palsy in young children following liver transplantation. Successful treatment by plication of the diaphragm.

Diaphragmatic paralysis was identified in four children after liver transplantation. All presented with persistent right upper lobe atelectasis, pleural effusion and recurrent respiratory infections and could not be weaned from mechanical ventilatory support. Fluoroscopy and real-time ultrasound confirmed paradoxical right diaphragmatic movements.

Progressive cardiac amyloidosis following liver transplantation for familial amyloid polyneuropathy: implications for amyloid fibrillogenesis.

Background: Circulating transthyretin (TTR) is derived from the liver, and orthotopic liver transplantation (OLT) is widely performed for variant TTR-associated familial amyloid polyneuropathy (FAP). The effect of OLT on FAP-related cardiac amyloid is of particular interest because wild-type TTR can itself be deposited as senile cardiac amyloid.

Methods: Serial echocardiography was performed in 20 FAP patients, 14 of whom underwent OLT, and 10 other liver transplant patients.