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Jaundice, Emergency Medicine

Basics

Description

- Conjugated (direct) hyperbilirubinemia: - Conjugated bilirubin is water soluble and measured as direct bilirubin. - In conjugated hyperbilirubinemia, bilirubin is returned to the bloodstream after conjugation in the liver instead of draining into the bile ducts. - Hepatocellular dysfunction: - Hepatitis - Cirrhosis - Tumor invasion - Toxic injury

- Cholestasis: - Pruritus - Pale stools - Dark urine

- Malignancy: - Anorexia - Weight loss - Malaise

- US: Most effective initial imaging technique: - >90% effective in identifying cholelithiasis - Ductal dilation is a reliable indicator of extrahepatic obstruction: - A dilated common bile duct (CBD) and gallbladder suggest distal obstruction, whereas dilation of the intrahepatic ducts (without CBD dilation) suggests proximal obstruction.

- Therapeutic: - Extraction of CBD stones and insertion of stents to bypass malignant obstructions - Biopsy under direct vision

- Prehepatic: - Hemolysis (sickle cell, other hemoglobinopathies) - Ineffective erythropoiesis - Drugs - Gilbert syndrome: Usually benign inherited form of unconjugated hyperbilirubinemia - Crigler " “Najjar syndrome - Prolonged fasting

- Hepatocellular: - Hepatitis (infectious, alcoholic, autoimmune, toxin, drug induced) - Cirrhosis - Postischemic - Hemochromatosis

- Isotonic IV fluid therapy if dehydrated - Toxic-appearing patients: - Supplemental oxygen, cardiac monitoring - Nasogastric suction and bladder catheterization

- Obstructive intrahepatic or nonobstructive jaundice: - Medical management: - Withdraw causative drug, ethanol - Interferon for chronic hepatitis B and C - Penicillamine and phlebotomy for Wilson disease and hemochromatosis - Corticosteroids for chronic hepatitis of autoimmune origin

Yellow pigmentation of tissues and body fluids due to hyperbilirubinemia, usually present at levels of >2.5 mg/dL ‚

Etiology

  • Unconjugated (indirect) hyperbilirubinemia: Unconjugated bilirubin is the direct breakdown product of heme, is water insoluble, and is measured as indirect bilirubin:Hemolytic:Excessive production of unconjugated bilirubinHepatic:Decreased hepatobiliary excretion of bilirubin by:Defective uptake (drugs, Crigler " “Najjar syndrome)Defective conjugation (Gilbert syndrome drugs)Defective excretion of bilirubin by the liver cell (drugs, Dubin " “Johnson syndrome)
  • Conjugated (direct) hyperbilirubinemia:Conjugated bilirubin is water soluble and measured as direct bilirubin.In conjugated hyperbilirubinemia, bilirubin is returned to the bloodstream after conjugation in the liver instead of draining into the bile ducts.Hepatocellular dysfunction:HepatitisCirrhosisTumor invasionToxic injuryIntrahepatic (nonobstructive) cholestasisExtrahepatic (obstructive) cholestasis

Diagnosis

Signs and Symptoms

History

  • Cholestasis:PruritusPale stoolsDark urine
  • Malignancy:AnorexiaWeight lossMalaise
  • Abdominal pain

Physical Exam

  • Icterus of sclera and tongue base (levels >2.5 mg/dL)
  • Right upper quadrant tenderness:Courvoisier rule:Painless jaundice and a palpable, nontender gallbladder represent malignant common duct obstruction.
  • Stigmata of cirrhosis:Abdominal collateral circulation including caput medusae, hepatosplenomegaly, or hepatic atrophyAscitesSpider telangiectasiaPalmar erythemaDupuytren contracturesAsterixisEncephalopathyGynecomastia
  • Palpable gallbladder
  • Hepatomegaly
  • Splenomegaly
  • Abdominal mass
  • Evidence of cachexia
  • Excoriations (primary biliary cirrhosis, obstruction)
  • Kayser " “Fleischer rings:

Essential Workup

  • History and physical exam, together with routine lab tests, will suggest the diagnosis in ¢ ˆ ¼80% of patients with jaundice.
  • Bilirubin level " ”severity may suggest cause:Malignancy causes highest levels (10 " “30 mg/dL).Choledocholithiasis rarely exceeds 15 mg/dL.

Diagnosis Tests & Interpretation

Lab

  • Urine dipstick is 74% sensitive for bilirubin.
  • Alkaline phosphatase:If no bone disease and not pregnant, then elevation suggests impaired biliary tract function.2X normal: Hepatitis and cirrhosis3X normal: Extrahepatic biliary obstruction (i.e., choledocholithiasis) and intrahepatic cholestasis (i.e., drug-induced and biliary cirrhosis)
  • Aminotransferases " ”provide evidence of hepatocellular damage:Alanine aminotransferase (ALT, SGPT): Primarily in the liverAspartate aminotransferase (AST, SGOT): Liver, heart, kidney, muscle, and brain
  • Ž ³-Glutamyl transpeptidase " ”throughout hepatobiliary system, pancreas, heart, kidneys, and lungs:May be the most sensitive indicator of biliary tract disease.Confirms hepatic origin of an elevated alkaline phosphatase.
  • 5 " ²-Nucleotidase " ”widespread tissue distribution:Confirms hepatic origin of an elevated alkaline phosphatase level.
  • Albumin: Decreased with severe liver disease
  • PT: Elevation is an important prognostic indicator in patients with acute hepatitis.

Imaging

  • US: Most effective initial imaging technique:>90% effective in identifying cholelithiasisDuctal dilation is a reliable indicator of extrahepatic obstruction:A dilated common bile duct (CBD) and gallbladder suggest distal obstruction, whereas dilation of the intrahepatic ducts (without CBD dilation) suggests proximal obstruction.
  • Tumors of the liver and head of pancreas are usually well visualized.
  • Distinguishes solid liver tumors from cystic structures.
  • Plain radiographs:May show evidence of hepatic and splenic enlargement or biliary calcifications
  • Hepatic nuclear scan (hepatobiliary iminodiacetic acid scan):Accurate method of diagnosing acute cholecystitis or cystic duct obstructionTime consuming (usually several hours)
  • CT:Superior to US in detecting pancreatic and intra-abdominal tumors.Can help differentiate fluid-containing structures.

Diagnostic Procedures/Surgery

Endoscopic retrograde cholangiopancreatography (ERCP): ‚

  • Diagnostic:Stones are seen as filling defects within bile duct lumen.Malignancies are seen as strictures.
  • Therapeutic:Extraction of CBD stones and insertion of stents to bypass malignant obstructionsBiopsy under direct vision

Differential Diagnosis

  • Prehepatic:Hemolysis (sickle cell, other hemoglobinopathies)Ineffective erythropoiesisDrugsGilbert syndrome: Usually benign inherited form of unconjugated hyperbilirubinemiaCrigler " “Najjar syndromeProlonged fasting
  • Hepatocellular:Hepatitis (infectious, alcoholic, autoimmune, toxin, drug induced)CirrhosisPostischemicHemochromatosis
  • Intrahepatic cholestasis:Idiopathic cholestasis of pregnancyDrugsDubin " “Johnson syndromeRotor syndromeBenign recurrent cholestasiaFamilial syndromesSepsisPostoperative jaundiceLymphoma
  • Extrahepatic obstruction:Common duct stoneBiliary strictureBacterial cholangitisSclerosing cholangitisCarcinoma (ampulla, gallbladder, pancreas), cholangiosarcomaPancreatitis, pancreatic pseudocystHemobiliaDuodenal diverticulaAscariasisPostlaparoscopic cholecystectomy complicationsCongenital biliary atresiaCongenital choledochal cyst

Intrahepatic cholestasis: ‚

  • Cardiovascular (congenital heart disease, congestive heart failure, shock, asphyxia)
  • Metabolic or genetic (α1-antitrypsin deficiency, trisomy 18 and 21, cystic fibrosis, Gaucher disease, Niemann " “Pick disease, glycogen storage disease type IV)
  • Infectious (bacterial sepsis, cytomegalovirus, enterovirus, herpes simplex virus, rubella, syphilis, TB, varicella, viral hepatitis)
  • Hematologic (severe isoimmune hemolytic disease)

Treatment

Initial Stabilization/Therapy

  • Isotonic IV fluid therapy if dehydrated
  • Toxic-appearing patients:Supplemental oxygen, cardiac monitoringNasogastric suction and bladder catheterization

Ed Treatment/Procedures

  • For bacterial cholangitis/sepsis, obtain blood cultures and administer parenteral antibiotics:Ampicillin, gentamicin, and metronidazole orTicarcillin, or piperacillin, and metronidazole orCefoxitin and tobramycin
  • Obstructive extrahepatic jaundice:
  • Choledocholithiasis:ERCP papillotomy, balloon or basket retrieval, or open surgery
  • Obstructive intrahepatic or nonobstructive jaundice:Medical management:Withdraw causative drug, ethanolInterferon for chronic hepatitis B and CPenicillamine and phlebotomy for Wilson disease and hemochromatosisCorticosteroids for chronic hepatitis of autoimmune origin
  • Exchange transfusion:Emergent treatment of markedly elevated bilirubin (>20 mg/dL in full-term infants) and for correction of anemia caused by isoimmune hemolytic disease
  • Phototherapy " ”for neonatal jaundice when bilirubin = 17 mg/dL:Measure bilirubin once to twice daily and stop when bilirubin has been reduced by about 4 " “5 mg/dL.
  • Phenobarbital: In sepsis and drug-induced causes; decreases conjugated bilirubin.
  • Metalloporphyrins: Investigational inhibitors of heme oxygenase

Medication

  • Ampicillin: 2 g IV q6h (peds: 25 mg/kg IV q6 " “8h)
  • Cefoxitin: 2 g IV q6h (peds: 40 " “160 mg/kg/d div. q6 " “12h)
  • Gentamicin: 5 " “2 mg/kg IV q8h
  • Metronidazole: 7.5 mg/kg IV q6h (peds: Same)
  • Piperacillin/tazobactam: 3.375 g IV q6h (peds: 300 mg/kg/d div. q6h [>2 mo of age])
  • Ticarcillin/clavulanate: 3.1 g IV q6h (peds: 75 " “100 mg/kg/d div. q6h)
  • Tobramycin: 1 mg/kg IV q6h (peds: Same)

Follow-Up

Disposition

Admission Criteria

  • Bacterial cholangitis
  • Intractable pain
  • Intractable emesis
  • Associated pancreatitis
  • Elevated PT

Discharge Criteria

  • No evidence of infection (evaluate as outpatient)
  • Tolerating liquids

Additional Reading

  • Addley ‚ J, Mitchell ‚ RM. Advances in the investigation of obstructive jaundice. Curr Gastroenterol Rep. 2012;14:511 " “519
  • Maisels ‚ MJ, McDonagh ‚ AF. Phototherapy for neonatal jaundice. N Engl J Med. 2008;358:920 " “928.
  • Roche ‚ SP, Kobos ‚ R. Jaundice in the adult patient. Am Fam Physician. 2004;69:299 " “304.
  • Udell ‚ JA, Wang ‚ CS, Tinmouth ‚ J, et al. Does this patient with liver disease have cirrhosis? JAMA. 2012;307(8):832 " “842.
  • Wang ‚ Q, Gurusamy ‚ KS, Lin ‚ H, et al. Preoperative biliary drainage for obstructive jaundice. Cochrane Database Syst Rev. 2008;16(3):CD005444.

Codes

ICD9

  • 277.4 Disorders of bilirubin excretion
  • 774.6 Unspecified fetal and neonatal jaundice
  • 782.4 Jaundice, unspecified, not of newborn

ICD10

  • E80.6 Other disorders of bilirubin metabolism
  • P59.9 Neonatal jaundice, unspecified
  • R17 Unspecified jaundice
  • E80.5 Crigler-Najjar syndrome
  • E80.4 Gilbert syndrome

SNOMED

  • 18165001 Jaundice (finding)
  • 14783006 Hyperbilirubinemia (disorder)
  • 281610001 neonatal hyperbilirubinemia (disorder)
  • 28259009 Crigler-Najjar syndrome (disorder)
  • 27503000 Gilberts syndrome (disorder)
  • 44553005 Dubin-Johnson syndrome (disorder)
  • 7752002 Unconjugated hyperbilirubinemia (disorder)
  • 9326001 Conjugated hyperbilirubinemia (disorder)