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Prevention of parenteral nutrition-associated liver disease in children
1Pediatric Intestinal Transplantation Medicine, The Mount Sinai Hospital and Medical School, New York 10029-6574, USA. stuart.kaufman@mountsinai.org
Insights
Parenteral nutrition can cause liver injury, ranging from mild to severe liver failure, especially in patients with massive intestinal resection. Management involves enteral nutrition, sepsis control, and potentially restricting lipid emulsions.
Area of Science:
- Hepatology
- Gastroenterology
- Clinical Nutrition
Background:
- Parenteral nutrition (PN) is linked to liver injury, with severity varying widely.
- Severe PN-related liver disease often occurs in patients with massive intestinal resection.
- Sepsis and inflammation play a key role in the progression of PN-associated liver injury.
Purpose of the Study:
- To describe the spectrum of liver injury associated with parenteral nutrition.
- To outline the clinical presentation and management strategies for PN-related liver disease.
- To differentiate liver failure in short bowel syndrome from that in anatomically normal GI tracts.
Main Methods:
- Review of clinical presentations and outcomes in patients with PN-associated liver disease.
- Analysis of factors contributing to liver injury progression, including sepsis and inflammation.
- Comparison of clinical features of liver failure in short bowel syndrome versus normal GI tracts.
Main Results:
- Liver injury severity is not solely dependent on PN duration or delayed enteral feeding.
- Enteral nutrition, sepsis management, and bacterial overgrowth control are crucial treatments.
- Advanced liver failure in short bowel syndrome presents with hyperbilirubinemia and hepatosplenomegaly, differing from typical liver failure.
Conclusions:
- PN-related liver disease management requires a multifaceted approach.
- Early intervention for sepsis and inflammation is critical.
- Intestinal and/or liver transplantation may be the best option for long-term survival in severe cases.
Abstract:
Liver injury is associated with parenteral nutrition therapy. Severity of injury varies from minimal and transient increases in liver-related blood tests to biliary cirrhosis and liver failure. Severe parenteral nutrition-related liver disease is usually confined to patients who have undergone massive intestinal resection. In these patients, early sepsis appears to cause initial liver injury, and recurring sepsis and inflammation, local or systemic, may result in its perpetuation and progression. Liver disease associated with parenteral nutrition is not necessarily related either to duration of parenteral nutrition or to delayed intestinal feeding. However, treatment includes enteral nutrition to promote enterohepatic circulation of bile acids and management of inflammation and sepsis, including control of intestinal bacterial overgrowth. Restriction of intravenous lipid emulsions may be important. The clinical picture of advanced liver failure related to short bowel syndrome differs from liver failure with an anatomically normal gastrointestinal tract. In the former, hyperbilirubinemia, hepatosplenomegaly, and functional hypersplenism dominate the clinical picture, and severe ascites and esophageal variceal hemorrhage are unusual. Early referral of these patients for intestinal and/or liver transplantation may provide the best chance for long-term survival.
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