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20 Cards in this Set
- Front
- Back
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Unconjugated Hyperbilirubinemia
Seen clinically at > _ mg/dL |
Unconjugated Hyperbilirubinemia
Seen clinically at > 5 mg/dL |
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Free ______________ bilirubin can pass through the blood/brain barrier
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Free unconjugated bilirubin can pass through the blood/brain barrier
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Unconjugated Hyperbilirubinemia
Excreted primarily in the _____ |
Unconjugated Hyperbilirubinemia
Excreted primarily in the stool |
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RBC defects causing hemolytic anemia
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G6PD
Hereditary spherocytosis Thallasemias others |
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Immunologic cause of hemolytic anemia
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ABO or Rh incompatibility
Esp when mom is O and baby is A |
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What conjugation defect?
rare UDP glucuronyl transerase deficiency two types; autosomal recessive phenobarbital |
Crigler-Najjar
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What conjugation defect?
rather common mild UDPGT deficiency; autosomal dominant no treatment necessary |
Gilbert
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What conjugation defect?
peak in 2nd week resolves on interruption of breast feeding prolonged enterohepatic circulation |
Breast milk
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Metabolic disorders
causing Unconjugated Hyperbilirubinemia |
Galactosemia
Hypothyroidism |
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Labs ordered for suspected unconjugated hyperbilirubinemia
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Total & direct bili
CBC Retic Type maternal vs baby Direct Coombs Radiology – obstruction |
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Tx of unconjugated hyperbilirubinemia
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Phototherapy
Exchange transfusion Phenobarbital |
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Conditions causing Increased enterohepatic recirculation that cause unconj hyperbilirubinemia
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Cystic fibrosis
Pyloric stenosis |
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Altered binding to albumin
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Cephtriaxone (Rocephin)
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Physiology of Unconjugated Hyperbilirubinemia
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Indirect bili < 13 mg/dL
No jaundice in 1st 24 hours Conjugated bili < 2 mg/dL Common high levels 5-6 mg/dL in European/African, 10 mg/dL in Asian Mechanisms: Increased bili load with increased enterohepatic recirc Defective uptake Defective conjugation |
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What is direct bili of conjugated hyperbili
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Direct bili > 2 mg/dL or >20% total bili
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Extrahepatic causes of conj hyperbili
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Biliary atresia
Choledochal cysts Other obstructive processes (eg, neoplasm) |
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Intrahepatic causes of conj hyperbili
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Alagille syndrome – autosomal dominant
Progressive cholestasis – autosomal recessive Inspissated bile -- 2nd to hemolytic disease hepatosplenomegaly |
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Hepatocellular causes of conj hyperbili
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Alpha-1-antitrypsin deficiency
Cystic fibrosis Zellweger's (cerebrohepatorenal) syndrome Dubin-Johnson & Rotor autosomal recessive decreased rate of passage through liver Galactosemia Others Infection Hepatitis B, C Coxackievirus EBV Bacterial sepsis TPN Idiopathic |
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Dx of conjugated hyperbilirubinemia
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GI Consult
Labs Total & direct bili LFT PT/PTT CBC, retic Ammonia TORCH titers Many others Radiology Ultrasound Biopsy of liver |
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Management of Conjugated Hyperbilirubinemia
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Drugs
Phenobarbital, cholestyramine, Actigall Diet Medium-chain triglycerides in formula ADEK Surgery Kasai procedure (portoenterostomy) Liver transplant |
