Spontaneous perforation of the bile duct in infancy: a rare but important cause of irritability and abdominal distension.
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Ascites↗
Biomedical subjects
Frederick C Ryckman
Publications and source records attributed to Frederick C Ryckman.
Genetic induction of proinflammatory immunity in children with biliary atresia.
BACKGROUND: Biliary atresia is the commonest cause of pathological jaundice in infants and the leading indication for liver transplantation in children worldwide. The cause and pathogenesis remain largely unknown. Because of clinical heterogeneity and experimental difficulties in addressing molecular mechanisms underlying multifactorial disorders in human beings, we searched for genomic signatures of biliary atresia in affected infants. METHODS: We generated pools of biotinylated cRNA from livers of 14 infants with biliary atresia and six with neonatal intrahepatic cholestasis (diseased controls) and hybridised the cRNA against oligonucleotide-based gene chips. Immunohistochemistry and reverse transcriptase (RT)-PCR were used to assess the specificity of the findings and functional commitment of lymphocytes in affected livers. FINDINGS: Data filtering, to identify genes that are differentially expressed, and cluster analysis revealed a predominant and coordinated activation of immunity/inflammation genes within the livers of infants with biliary atresia. Most of the genes showed differential lymphocyte function, with activation of osteopontin, a regulator of cell-mediated (T-helper 1 [Th-1]) immunity in T-helper lymphocytes, and suppression of immunoglobulin genes in early stages of disease. These findings were associated with production of interferon gamma in 65% of infants with biliary atresia and no diseased control. However, histologically similar inflammatory infiltrates were present in livers of both groups, implying differential activation states of similar cell types. INTERPRETATION: Livers of infants with biliary atresia have a coordinated activation of genes involved in lymphocyte differentiation. Among these genes, the overexpression of osteopontin and interferon gamma points to a potential role of Th-1-like cytokines in disease pathogenesis.
Biliary Atresia↗
Long-term outcome after liver transplantation in children.
Children (defined as under 18 yr of age) account for approximately 12.5% of all liver transplants in the United States. Even though the annual number of liver transplantation procedures remains relatively constant, the population of long-term survivors of liver transplantation has grown. Presently, the population of long-term survivors of liver transplantation is 10-fold greater then the number of transplantations carried out each year. For long-term survivors of liver transplantation, the goal is to maintain graft function and wellness while decreasing the morbidity associated with long-term immunosuppression. The primary diagnosis leading to liver transplantation in children do not recur in the allograft. Consequently, many of the complications of liver transplantation, both early and long term, relate to the need for immunosuppression. Children may be at increased risk to develop significant end-organ damage as a result of increased serum lipid levels, elevated blood pressure, altered glucose metabolism, decreased renal function, cancer, and diminished bone accretion that occur as a result of immunosuppressive therapy or complications of therapy. As survival rates have increased, health care providers have begun to assess health-related quality of life. We will review our current knowledge of long-term outcome following pediatric liver transplantation in children.
Adaptation, Physiological↗