FAILURE IS THE WORST
Supportive measures in liver failure that can aid in clearance, fluid management, refractory coagulopathy.
What is CRRT and TPE?
This lab is cleared by the liver and can be elevated in multiple instances including urea cycle disorders, portosystemic shunting, urinary tract infection from urease-producing organisms, gastrointestinal bleeding, shock, renal disease, heavy exercise, smoking, parenteral nutrition, salicylate intoxication, medications
What is ammonia?
A 15-year-old girl presents with malaise, weight loss, and anorexia. She has occasional jaundice. She has anti-smooth muscle antibodies (ASMA).
What is type I autoimmune hepatitis?
This is a medication commonly used to suppress the immune system after liver transplantation
What is tacrolimus?
After transplant, patients are monitored for this serious complication where the body attacks the new liver
What is liver graft rejection?
Fluid overload in a patient with acute liver failure can lead to this potentially lethal sequelae?
What is cerebral herniation and death?
This lab study paired with abnormal hepatocellular labs suggests a patient may be in liver failure.
What is an INR?
This genetic syndrome can cause severe liver damage due to iron overload, sometimes requiring a liver transplant.
What is hereditary hemochromatosis?
What is the most common indication for pediatric liver transplant worldwide?
What is biliary atresia?
Long term immunosuppression can increase the risk of this type of infection.
What is cytomegalovirus (CMV) or other opportunistic infections?
Rapid decline in aminotransferases + rising bilirubin and coagulopathy in a patient who presented in liver failure?
What is massive hepatic necrosis with very poor prognosis?
Defined as: serum conjugated bilirubin of greater than 1 mg/dl when total is less than 5 mg/dL or over 20% of the total bilirubin concentration if the total is more than 5 mg/dL?
What is Cholestasis?
A patient presents with new onset hematemesis and ascites found to have liver disease that has progressed to this.
What is portal hypertension?
What is the most common cause of acute fulminant liver failure in children in the US?
What is Drug hepatotoxicity/Drug induced liver injury (DILI)?
Patients are counseled about this life-long risk in solid organ transplantation thought to be related to immunosuppression therapy (specifically tacrolimus) and is at times related to a viral infection.
What is PTLD?
Defined by coagulopathy not responsive to vitamin K, INR > 1.5 with presence of hepatic encephalopathy or INR > 2 without encephalopathy
What is pediatric acute liver failure?
Tests of liver biochemical activity
What are:
•Enzyme studies that measure liver cell injury (AST, ALT, LDH)
•Tests for impaired bile flow/cholestasis (GGT, alkaline phosphatase, bilirubin, bile acids)
This genetic disease causes excessive copper accumulation in the liver and presents with low ceruloplasmin.
What is Wilson disease?
This scoring system is used to prioritize liver transplant recipients based on the severity of their disease
What is the PELD score (Pediatric End-Stage Liver Disease)?
This is the most common vascular complication following a pediatric liver transplant
What is hepatic artery thrombosis?
Most common cause of neonatal liver failure?
What is gestational alloimmune liver disease (GALD)?
The most important initial step in evaluating a jaundiced infant is measuring what lab(s)?
What is serum total and direct (or conjugated) bilirubin?
Babies with this disorder have best outcomes if undergo this procedure before age 30-45 days of life.
What is a Kasai hepatic portoenterostomy (HPE)?
Goals of early evaluation for liver transplant
What is:
•Begins when liver disease that can progress to failure is identified
•Before life-threatening complications develop
•Before contraindications develop
After transplantation, children are monitored for this complication caused by immunosuppressant medications and can cause acute and long-term issues.
What is nephrotoxicity?