Jaundice/Hyperbilirubinemia

also see Neonatal jaundice 
 
Jaundice
- Yellow discoloring of skin & Sclera (Scleral Icterus)
- Serum bilirubin > 3.0
 Hemolytic anemia Extravascular Hemolysis
Unconjugated
(indirect bilirubin)
Conjugated (Direct bilirubin)
- Pre Hepatic Jaundice
- Urine = Urobilinogen Dark Urine
- Pigment Stones in biliary tree
- AST
- Direct Bilirubin, detectable in urine dipstick
- Dark Urine due to conjugated bilirubin
Production of Bilirubin uptake/Impaired conjugation Decreased Hepato-cellular Excretion Impaired Bowel Flow
RBC Distruction   Canalicular membrane defect Viral Hepatitis - Obstructive liver dz
- MOA: Gallstone blocking bile flow
- Clinical: Pale stool, Jaundice, Icterus, Dark urine, Cholestasis (ALK Phos)
Hemolytic Anemia Infective Erythropoiesis
(Sepsis)
Physiological Crigler-Najjer Syndrome Gilbert Syndrome Dubin-johnson Syndrome Rotor Syndrome - Liver cell & bile duct necrosis
- ↑ UCB in blood (serum)
- ALT > AST
Intra-hepatic Extra-hepatic
    - ↓ UGT 1A1 activity
-Kernicterus (Seizure, Hypotonia)
 
Type I
complete UGT deficiency
Fatal
Kernicterus
 
expression of UGT

Common/Benign

Fluctuation of unconjugated hyperbilirubinemia

Stress related
- Autosomal recessive
- Darkly pigmented liver
- Not compatible w/ life
- Autosomal recessive
- No Pigments on liver
- Drug induced
- Destruction of intrahepatic duct
 
DDx:
- Gallstone
- Cancer of the head of pancreas
- Liver-fluke infection
- Bile duct tumor
- Biliary atresia
- Primary Sclerosing Cholangitis
Graft vs Host Primary Sclerosing Cholangitis Primary Biliary Cirrhosis
Type II
Partial defect
Deep Yellow skin
Caused by bone marrow transplant - Chronic liver Dz
- Associated w/ Ulcerative Colitis
- risk of cholangiocarcinoma
- Dx: ERCP, P-ANCA, ALK Phos
- Micro: beaded apprearance
- Inflammation & granulomatous destruction.
- Xanthoma, Xanthelasma.
- serum Cholesterol
- Anti-mitochondrial Ab
- Association:

 Sicca syndrome (Sjogran)
 Scleroderma (DNA topoisomerase I)
 RA (Anti-CCP)
 SLE (ANA)
 Thryoiditis (Hypothyroid)


Major Lab Abnormality Etiology Examples Evaluation
None "Pseudo-Jaundice" - Carotenemia
- Normal Pigmentation
- Assess vegetable intake
Isolated unconjugated (indirect) bilirubin Production of Bilirubin - Hemolysis
- Transfusion
- Sepsis
 
- Transfusion load
- Blood smear
- Haptoglobin
- Direct Coomb's
- CT abd/pelvis for hematoma
uptake/Impaired conjugation - Hematoma resorption
- Physiological
- Crigler-Najjer Syndrome
- Gilbert Syndrome
direct (conjugated) bilirubin Transaminases Hepatocellular injury - Drug injury
- Hypotension
- Hypoxemia
- Acute Hepatitis (Viral, EtoH, autoimmune)
- Wilson's disease
- Non-alcoholic steatohepatits
- Veno-Occlusive
- Budd-Chiari
- Worsening of pre-existing liver dz
 
- R/O Drug injury
- Assess tissue perfusion (Doppler ultrasound)
- Hepatitis serologies
- Liver Biopsy
Alk Phos Cholestatic injury - Drug injury
- "Benign postoperative"
- Sepsis
- TPN
 
- R/O drug injury
- Amylase, Lipase
- US or CT scan
- ERCP or MRCP if ducts are dilated
- Liver biopsy
Biliary obstruction (intra- vs extra-hepatic) - Choledocholithiasis
- Pancreatitis
- Biliary structure (benign or malignant)
- Hepatic metastasis
- Sarcoidosis
- PBC, PSC
- AIDS cholangiopathy
 

 

Pathophysiology

History

Physical Exam

Labs:

Treatment

  1. Directed at cause
  2. Supportive treatment (fluids, etc)
  3. Viral
  4. Mechanical
  5. Neonatal
  6. Toxin

Disposition

  1. Admit
  2. Viral hepatitis (see Viral Hepatitis )
  3. Follow-up potential contacts