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when they begin to have linear growth failure and the first complications of portal hypertension. Patients with familial cholestatic syndromes, which ultimately lead to cirrhosis, may have a less predictable course. Growth failure is characteristic of these syndromes even when liver function is preserved. Signs of advancing portal hypertension and liver synthetic failure are the earliest indications for transplant in this group. Children with metabolic defects, which are corrected by transplantation, are approached with a different strategy. In this setting, the goal should be to perform the transplant before the patient develops significant complications from the metabolic defect. The child with fulminant hepatic failure should undergo transplant as soon as a suitable organ is available, since fewer than 25% of these patients will survive without transplant. Table 12C.1 summarizes the medical complications that indicate the need to proceed with transplant.

The preoperative evaluation of a child awaiting liver transplantation includes, establishing the etiology, predicting the timing of the need for transplant, and identifying anatomic abnormalities or other organ system impairment, which would complicate the surgical procedure. Children with cirrhosis should show signs of hepatic insufficiency, such as growth failure or coagulopathy, or have significant complications of portal hypertension, such as ascites or variceal bleeding before liver transplant is performed. A child who has not developed these complications may have many years of good quality of life prior to the need for liver transplant.

Table 13C.1. Medical complications indicating the need for liver transplantation in pediatric patients

1) Biliary Atresia a) Status post failed Kasai procedure b) Recurrent ascending cholangitis c) Complications of cirrhosis as listed below

2) Cirrhosis of any etiology with the following complications a) Growth failure b) Ascites which is refractory to medical management c) Episodes of variceal bleeding which are refractory to sclerotherapy and/or TIPS

d) Hypersplenism causing thrombocytopenia e) Liver synthetic failure f) Other major systemic complications

3) Fulminant hepatic failure

4) Neonatal liver failure

5) Inborn errors of metabolism a) Tyrosinemia b) Glycogen storage disease c) Crigler-Nijjar Syndrome d) Ornithine transcarbamylase deficiency e) Other defects with the potential to cause neurologic or other major systemic complications

6) Unresectable hepatic tumors without extension

Blood Pressure Health

Blood Pressure Health

Your heart pumps blood throughout your body using a network of tubing called arteries and capillaries which return the blood back to your heart via your veins. Blood pressure is the force of the blood pushing against the walls of your arteries as your heart beats.Learn more...

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