ABP Transplant Hepatology cheat sheet

Neonatal cholestasis is conjugated bilirubin >1 mg/dL or >20% of total bilirubin when total is >5 mg/dL; initial evaluation includes fractionated bilirubin, GGT, ultrasound, and metabolic or infection testing.

Pediatric acute liver failure is defined by INR ≥1.5 with encephalopathy or INR ≥2.0 without encephalopathy in a child without known chronic liver disease.

ALT predominance suggests viral hepatitis, autoimmune hepatitis, fatty liver disease, or drug injury; an AST:ALT ratio >2 suggests Wilson disease, alcohol exposure, or advanced chronic liver disease.

Autoimmune hepatitis type 1 shows ANA and/or SMA; type 2 shows anti-LKM1 and often anti-LC1; first-line therapy is prednisone with azathioprine.

Wilson disease findings include low ceruloplasmin, 24-hour urine copper >100 mcg/day, low alkaline phosphatase, Coombs-negative hemolysis, and Kayser-Fleischer rings; ATP7B testing or liver copper confirms.

Alpha-1 antitrypsin deficiency is evaluated with serum level plus phenotype or genotype; PiZZ is linked to neonatal cholestasis, early cirrhosis, and hepatocellular carcinoma.

Chronic hepatitis B monitoring includes HBeAg, HBV DNA, and ALT every 6-12 months; the immune-active phase has elevated ALT with high HBV DNA and warrants antiviral consideration.

Cystic fibrosis liver disease surveillance includes annual GGT, ultrasound, and physical examination; ursodeoxycholic acid is used for cholestasis, and portal hypertension may prompt transplant evaluation.

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 hemorrhage management includes octreotide 1 mcg/kg bolus then 1 mcg/kg/hour, ceftriaxone prophylaxis, and endoscopy within 12-24 hours after stabilization.

Ascitic fluid serum-ascites albumin gradient ≥1.1 g/dL indicates portal hypertension; SAAG <1.1 g/dL suggests peritoneal tuberculosis, malignancy, pancreatic ascites, or nephrotic syndrome.

Spontaneous bacterial peritonitis is diagnosed when ascitic polymorphonuclear cells are ≥250/mm3; treat with intravenous cefotaxime and add albumin for renal protection.

Hepatic encephalopathy is managed with lactulose titrated to 2-3 soft stools per day; precipitating factors include gastrointestinal bleeding, infection, dehydration, constipation, and sedating drugs.

Hepatorenal syndrome is functional renal failure in cirrhosis with ascites, no response to volume challenge, low urine sodium, and absence of shock or structural kidney disease.

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

Portopulmonary hypertension is mean pulmonary artery pressure >20 mm Hg with pulmonary vascular resistance >3 Wood units and pulmonary artery wedge pressure ≤15 mm Hg; severe disease contraindicates transplant.

Biliary atresia presents with acholic stools and direct hyperbilirubinemia; ultrasound may show the triangular cord sign, and Kasai portoenterostomy before 60 days improves bile drainage.

Alagille syndrome is caused by JAG1 or NOTCH2 variants and includes bile duct paucity, cholestasis, butterfly vertebrae, peripheral pulmonary stenosis, posterior embryotoxon, and pruritus.

PFIC1 is due to ATP8B1, PFIC2 to ABCB11, and PFIC3 to ABCB4; PFIC1 and PFIC2 have low GGT, while PFIC3 has high GGT.

Tyrosinemia type 1 results from fumarylacetoacetate hydrolase deficiency; succinylacetone is elevated, and treatment is nitisinone plus a low-tyrosine diet with HCC surveillance.

Classic galactosemia from GALT deficiency causes neonatal jaundice, hepatomegaly, and Escherichia coli sepsis; lifelong galactose restriction prevents acute toxicity.

Urea cycle disorders cause hyperammonemia with low BUN and respiratory alkalosis; acute therapy includes nitrogen scavengers, arginine, and dialysis for severe crisis.

Mitochondrial hepatopathy may show lactic acidosis, hypoglycemia, and elevated lactate-to-pyruvate ratio; valproate should be avoided because it can trigger liver failure.

Neonatal hemochromatosis causes severe neonatal liver failure with high ferritin and extrahepatic siderosis; IVIG and exchange transfusion are used, and transplant is considered in survivors.

PELD for children under 12 uses bilirubin, INR, albumin, growth failure below -2 standard deviations, and age under 1 year; exception points may be requested.

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

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

Pretransplant evaluation includes vaccination, nutrition, variceal screening, dental review, cardiac and pulmonary assessment, renal function, psychosocial assessment, and financial planning.

Living donor evaluation requires blood type compatibility, adequate graft volume, normal donor liver anatomy, absence of significant steatosis, and medical and psychosocial clearance.

Pediatric status 1A includes acute liver failure, primary graft nonfunction, hepatic artery thrombosis within 7 days of transplant, or acute decompensation of Wilson disease.

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

HCC transplant criteria include a single lesion ≤5 cm or up to three lesions each ≤3 cm, without vascular invasion or extrahepatic spread; imaging and AFP are repeated while waiting.

Early graft function shows falling transaminases, improving INR, and bile production; Doppler ultrasound is used urgently when hepatic artery thrombosis or biliary obstruction is suspected.

Hepatic artery thrombosis may present with fever, sepsis, biliary leak, or fulminant graft failure; Doppler ultrasound is first-line, and urgent revascularization or retransplantation may be required.

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

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

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

Live attenuated vaccines are generally contraindicated after liver transplant; inactivated vaccines are safe but may produce weaker responses, so pretransplant vaccination is preferred.

Long-term follow-up after pediatric liver transplant monitors renal function, hypertension, diabetes, bone health, growth, adherence, and annual age-appropriate cancer surveillance.

Acute cellular rejection most often occurs from 1 week to 3 months after transplant; chronic rejection usually develops later and is associated with ductopenia and arteriopathy.

Tacrolimus is a calcineurin inhibitor metabolized by CYP3A4; toxicity includes nephrotoxicity, hypertension, tremor, hypomagnesemia, hyperkalemia, and post-transplant diabetes.

Cyclosporine binds cyclophilin and causes hypertension, gingival hyperplasia, hirsutism, and dyslipidemia; like tacrolimus, it has narrow therapeutic indexing and many CYP3A4 interactions.

Mycophenolate inhibits inosine monophosphate dehydrogenase and causes gastrointestinal intolerance, leukopenia, and anemia; it is teratogenic and requires effective contraception.

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

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

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

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

CYP3A4 inducers such as rifampin, phenytoin, and carbamazepine lower calcineurin inhibitor levels; azole antifungals, macrolides, and grapefruit raise levels and toxicity risk.