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Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.
OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
Michael K. Farrell, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pediatric Gastroenterology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio,
John C. Bucuvalas, M.D., Professor, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Clinical Director, Medical Director of Liver Transplantation, Department of Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
The liver, the largest parenchymal organ in the body, receives 25% of the resting cardiac output . It is also a complex metabolic organ involved in a variety of synthetic and detoxification functions. By virtue of its size, multiple metabolic functions, and prominent position in the circulatory system, the liver is frequently involved in systemic, circulatory, and inflammatory diseases. It is often an “innocent bystander” during systemic diseases; conversely, hepatic dysfunction may be the first clue to a systemic disorder. This chapter reviews hepatic involvement in common childhood systemic diseases.
JAUNDICE IN THE CRITICALLY ILL CHILD
Hepatic dysfunction, manifest as jaundice, occurs in patients with systemic diseases associated with increased bilirubin production, ischemia, hypoxemia, or malnutrition (Table 38.1). Bilirubin production increases with hemolysis, blood transfusions, intraluminal bleeding, extracorporeal oxygenation, and resorption of blood from hematomas. The inflammatory cascade has multiple effects on hepatic function [2–9]. In the healthy patient, the liver has the capacity to conjugate and excrete bilirubin. However, with fasting, malnutrition, positive pressure ventilation, or ischemia, the liver's ability to process bilirubin is compromised and conjugated hyperbilirubinemia results [10, 11] (Figure 38.1). Liver dysfunction improves with correction of the primary disorder, but inadequate or unsuccessful treatment may result in progressive hepatic dysfunction.
Jaundice and conjugated hyperbilirubinemia occur more frequently in infants and children with sepsis, even in the absence of shock. Patients may have mildly elevated serum alkaline phosphatase and aminotransferase levels; isolated hyperbilirubinemia is uncommon [12, 13].
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