ABP Transplant Hepatology flashcards

32 cards. Read the term and the definition here. The deck at the top of this page is optional.

PALF coagulopathy criterion

Pediatric acute liver failure requires INR at least 1.5 with encephalopathy or INR at least 2.0 without encephalopathy in a child without known chronic liver disease.

Neonatal cholestasis definition

Conjugated bilirubin above 1 mg/dL, or above 20 percent of total bilirubin when total exceeds 5 mg/dL, defines neonatal cholestasis and warrants prompt evaluation.

Autoimmune hepatitis type 2

Type 2 autoimmune hepatitis is marked by anti-liver-kidney microsomal type 1 antibodies and often presents in younger children with severe or fulminant hepatitis.

Wilson disease screening

Low ceruloplasmin, elevated 24-hour urinary copper, low alkaline phosphatase, Coombs-negative hemolysis, and Kayser-Fleischer rings support Wilson disease; genotype or liver biopsy may confirm.

Alpha-1 antitrypsin testing

Measure serum alpha-1 antitrypsin level and phenotype or genotype; PiZZ is associated with neonatal cholestasis and early childhood cirrhosis.

Chronic hepatitis B phases

Chronic hepatitis B phases are defined by HBeAg, HBV DNA, and ALT: immune-tolerant, immune-active, inactive carrier, and HBeAg-negative reactivation; treatment depends on inflammation and fibrosis.

HVPG thresholds

Hepatic venous pressure gradient is normal at 1-5 mm Hg; varices develop above 10 mm Hg, and variceal bleeding risk rises above 12 mm Hg.

Pediatric variceal bleeding

For suspected pediatric variceal hemorrhage, give octreotide 1 mcg/kg bolus then 1 mcg/kg/hour, ceftriaxone prophylaxis, and arrange endoscopy within 12-24 hours after stabilization.

SAAG interpretation

Serum-ascites albumin gradient of at least 1.1 g/dL indicates portal hypertension; below 1.1 g/dL suggests peritoneal, pancreatic, or other nonportal causes.

Spontaneous bacterial peritonitis

Spontaneous bacterial peritonitis is diagnosed when ascitic fluid polymorphonuclear cells are at least 250 per cubic millimeter; treat with intravenous cefotaxime and albumin.

Hepatopulmonary syndrome

Hepatopulmonary syndrome combines liver disease, intrapulmonary vascular dilations, and hypoxemia with room-air PaO2 below 80 mm Hg or elevated alveolar-arterial gradient.

Biliary atresia Kasai timing

Kasai portoenterostomy for biliary atresia achieves best bile drainage when performed before 60 days of age; later surgery increases risk of progressive cirrhosis and transplant need.

Alagille syndrome features

Alagille syndrome, usually from JAG1 or NOTCH2 variants, causes bile duct paucity, cholestasis, butterfly vertebrae, peripheral pulmonary stenosis, and characteristic facies.

PFIC types

PFIC1 arises from ATP8B1, PFIC2 from ABCB11, and PFIC3 from ABCB4; PFIC1 and PFIC2 have low GGT, while PFIC3 has high GGT.

Tyrosinemia type 1

Tyrosinemia type 1 results from fumarylacetoacetate hydrolase deficiency, causes succinylacetone elevation, and is treated with nitisinone plus a low-tyrosine diet; HCC risk remains.

Galactosemia management

Classic galactosemia from GALT deficiency presents with jaundice, hepatomegaly, and Escherichia coli sepsis; lifelong galactose restriction prevents acute toxicity but not all long-term complications.

PELD score components

The Pediatric End-Stage Liver Disease score for children under 12 uses bilirubin, INR, albumin, growth failure below minus two standard deviations, and age under one year.

MELD 3.0 variables

MELD 3.0 for patients aged 12 and older includes bilirubin, INR, creatinine, albumin, sodium, and sex, with adjustment for female sex.

Transplant absolute contraindications

Absolute contraindications include active uncontrolled sepsis, extrahepatic malignancy, severe irreversible cardiopulmonary disease, and uncontrolled psychiatric illness or active nonadherence.

Living donor evaluation

Living liver donor evaluation requires normal liver anatomy and function, compatible blood type, adequate graft volume, and absence of significant medical, psychiatric, or psychosocial risk.

Pediatric waitlist exception

Pediatric waitlist exception requests use objective complications such as growth failure, intractable pruritus, recurrent cholangitis, portopulmonary hypertension, or hepatopulmonary syndrome and require regional review.

Early graft dysfunction

Early graft dysfunction shows rising transaminases, impaired synthetic function, and cholestasis; ultrasound excludes hepatic artery thrombosis and biliary obstruction, and biopsy assesses rejection.

Hepatic artery thrombosis

Hepatic artery thrombosis after pediatric liver transplant may present with fever, sepsis, biliary leak, or fulminant graft failure; Doppler ultrasound is first-line and urgent revascularization may be needed.

Biliary anastomotic stricture

Biliary anastomotic stricture causes cholestasis, rising bilirubin, and dilated ducts; diagnosis is by ultrasound, magnetic resonance cholangiopancreatography, or cholangiography, and treatment is endoscopic dilation or stenting.

Post-transplant CMV prophylaxis

CMV prophylaxis with valganciclovir is commonly given for 3-6 months after liver transplant, especially for CMV-seronegative recipients of seropositive grafts.

PTLD presentation

Post-transplant lymphoproliferative disorder often presents with fever, lymphadenopathy, tonsillar enlargement, or gastrointestinal symptoms; EBV viral load and biopsy establish diagnosis.

Acute cellular rejection

Acute cellular rejection shows mixed portal inflammation, bile duct injury, and endothelialitis; first-line treatment is high-dose intravenous methylprednisolone, followed by rebiopsy if response is incomplete.

Chronic rejection histology

Chronic liver allograft rejection is characterized by ductopenia, vanishing bile ducts, and arteriopathy; it is often irreversible and may require retransplantation.

Tacrolimus toxicity

Tacrolimus toxicity causes nephrotoxicity, hypertension, tremor, hypomagnesemia, hyperkalemia, and post-transplant diabetes; levels are monitored because CYP3A4 interactions are common.

Mycophenolate risks

Mycophenolate causes gastrointestinal intolerance, leukopenia, and anemia; it is teratogenic, so effective contraception is required for female recipients of childbearing potential.

mTOR inhibitor effects

Sirolimus and everolimus impair wound healing, raise lipids, and can worsen proteinuria; they are avoided early after transplant when anastomotic healing is critical.

Antibody-mediated rejection

Antibody-mediated rejection involves donor-specific antibodies, capillary injury, and C4d deposition; treatment includes intravenous immunoglobulin, plasmapheresis, and sometimes rituximab.