Jaundice resident survival guide (pediatrics)

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Synonyms and keywords:

Jaundice resident survival guide (pediatrics) Microchapters
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts

Overview

This section provides a short and straight to the point overview of the disease or symptom. The first sentence of the overview must contain the name of the disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.

• Biliary atresia
• Fulminant Liver failure

Common Causes

Causes of jaundice in children include:

Common causes of Jaundice in children

Unconjugated hyperbilirubinemia	Conjugated hyperbilirubinemia
	Hepatic etiology	Post-hepatic etiology
Breast milk jaundice Breast feeding failure jaundice Gilbert syndrome Excessive hemolysis Crigler-Najjar syndrome Hypothyroidism	Viral hepatitis Hepatitis A, B, C, D, and E EBV HSV Metabolic disorders Wilson disease Hepatotoxic drugs Acetaminophen Anticonvulsants Anesthetics Antituberculous drugs Chemotherapeutic agents Antibiotics Oral contraceptives Alagille syndrome Alpha-1-antitrypsin deficiency Cystic fibrosis Autoimmune hepatitis	Biliary atresia Choledochal cyst Gallstone Inspissated bile syndrome

Complete Diagnostic Approach

Shown below is an algorithm summarizing the diagnosis of jaundice in children.

Characterise jaundice ❑ Onset ❑ Duration (acute or chronic) ❑ Progression ❑ Location ❑ Precipitating events ❑ Dark urine ❑ Acholic stools

Ask about associated symptoms ❑ Abdominal pain ❑ Abdominal distension ❑ Nausea and Vomiting ❑ Dyspepsia ❑ Anorexia ❑ Weight gain or loss ❑ Fever and Chills ❑ Itching ❑ Steatorrhea ❑ Arthralgia ❑ Myalgia ❑ Urticaria ❑ Sore throat ❑ Rash ❑ Pale skin ❑ Fatigue ❑ Back pain ❑ Confusion ❑ Lethargy ❑ Poor feeding

Obtain a detailed history ❑ Age (causes of jaundice varies with age ❑ Race ❑ Past medical history ❑ Recurrent episodes of jaundice ❑ Hemolytic anemia ❑ Autoimmune disease ❑ Malignancy ❑ Past surgical history ❑ Family history ❑ Jaundice ❑ Liver disease ❑ Wilson disease ❑ Malignancy ❑ Anemia ❑ Autoimmune disease ❑ Travel history (poor sanitation) ❑ Medications or Herbal preparation ❑ Blood transfusion and Needle stick injury or unsterilized syringe use ❑ Sexual history ❑ Social history ❑ Smoking ❑ Alcohol ❑ Drug abuse ❑ Tattoo ❑ Occupational history ❑ Sewage worker (Leptospirosis) ❑ Health care worker (needle stick injury) ❑ Birth history (Neonatal jaundice) ❑ Maternal infection ❑ Maternal blood group ❑ Maternal Diabetes ❑ Medications during pregnancy ❑ Birth trauma ❑ Feeding history ❑ Breast milk ❑ Formula feeding ❑ Number of wet diapers per day

Examine the patient Check vitals ❑ Temperature ❑ Pulse ❑ Blood pressure ❑ Respiratory rate Perform a general physical exam ❑ HEENT examination ❑ Yellow sclera ❑ Cataract (TORCH infection) ❑ Hematoma (cephalhematoma) ❑ Lymphadenopathy ❑ Skin examination ❑ Yellow skin ❑ Pale skin ❑ Scratch mark (indicates pruritus) ❑ Bruise ❑ Spider nevi and palmar erythema (seen in liver disease) ❑ Abdominal examination ❑ Signs of previous surgery ❑ Right upper quadrant tenderness ❑ Murphy sign ❑ Courvoisier law ❑ Abdominal distension (hepatomegaly, enlarged gall bladder, splenomegaly, ascites, malignancy) ❑ CNS examination (Kernicterus) ❑ Altered mental status ❑ Hyoptonia ❑ Asterixis ❑ Lymph nodes (infection, malignancy) ❑ Extremities examination ❑ Lower limb edema (cirrhosis, malignancy) ❑ Dysmorphic features (Alagille syndrome)

Order serum bilirubin

Increased indirect bilirubin

Increased direct bilirubin

Unconjugated hyperbilirubinemia

Conjugated hyperbilirubinemia

Pathological jaundice

Physiological jaundice

Liver function test

Features of hemolysis

Increased ALT and AST

Increased ALP and GGT

Present

Absent

Hemolytic anemia ❑ Family history of anemia ❑ African-American or African race ❑ Pallor ❑ Fatigue ❑ Fever ❑ Chills ❑ Hemoglobinuria ❑ Splenomegaly ❑ Gallstone

Breast milk jaundice ❑ Breastfeeding history ❑ Healthy infant ❑ Absence of other abnormalities Breast feeding jaundice ❑ Inadequate breastfeeding ❑ Signs of dehydration Gilbert syndrome ❑ Mild jaundice seen in neonates ❑ Positive family history ❑ Aggravated by exertion, stress, illness, fasting, infection ❑ Recurrent episodes of jaundice Crigler-Najjar syndrome ❑ Neonatal jaundice ❑ Positive family history ❑ Recurrent episodes of jaundice

Hepatic etiology Viral hepatitis ❑ Viral prodrome ❑ Recent travel history ❑ Blood transfusion and needle stick injury ❑ Injection drug use ❑ Promiscuous sexual behavior ❑ Healthcare worker ❑ Family history of hepatitis Alcoholic hepatitis ❑ History of alcoholism ❑ Hepatomegaly (Liver span > 15cm) ❑ Leucocytosis ❑ Macrocytic anemia Hepatotoxic drugs ❑ History of drug exposure ❑ Fever ❑ Leucocytosis ❑ Eosinophilia Wilson disease ❑ Neuropsychiatric symptoms ❑ Kayser-Fleischer ring Metabolic disorders ❑ Failure to thrive ❑ Growth retardation ❑ Congenital malformations ❑ Hepatomegaly

Post-hepatic etiology Biliary atresia ❑ Jaundice develops within 1-8 weeks after the birth ❑ Cholestatic jaundice ❑ Fat-soluble vitamin deficiencies ❑ Growth retardation Choledochal cyst ❑ Abdominal pain ❑ Palpable abdominal mass ❑ Jaundice ❑ Pancreatitis Inspissated bile syndrome ❑ Premature infants ❑ History of cystic fibrosis ❑ Prolonged TPN ❑ Fluconazole use ❑ Idopathic ❑ Symptoms of obstructive jaundice Gallstone ❑ History of hemolytic anemia ❑ Right upper quadrant pain ❑ Nausea and Vomiting ❑ Pancreatitis

Diagnostic work up ❑ Complete blood count with platelets ❑ Increased reticulocyte count ❑ Osmotic fragility test ❑ Direct and indirect Coombs test ❑ Serum haptoglobin ❑ Hemoglobin electrophoresis ❑ Increased serum lactate dehydrogenase ❑ Urine hemosiderin ❑ Urine hemoglobin ❑ Peripheral smear ❑ Schistocytes ❑ Spherocytes ❑ Target cell ❑ Bite cell ❑ Sickle shaped RBC

Diagnostic work up ❑ Normal blood profile ❑ Normal LFT ❑ Normal liver histology

Diagnostic work up Viral Hepatitis ❑ CBC with differentials ❑ Hepatitis serology ❑ Monospot test and Heterophile antibody test ❑ EBV serology ❑ HSV serology Wilson's disease ❑ Low serum ceruloplasmin ❑ High serum calcium ❑ 24-hour urinary copper excretion more than 100 μg/24h ❑ Coombs negative hemolytic anemia ❑ Liver bopsy Metabolic syndrome ❑ Newborn screening tests

Diagnostic work up Biliary atresia ❑ Cholestatic pattern on LFT ❑ Small or absent gall bladder on abdominal ultrasonography ❑ ERCP ❑ Hepatobiliary scintigraphy ❑ Intraoperative cholangiogram ❑ Liver biopsy Choledochal cyst ❑ LFT ❑ Abdominal ultrasonography ❑ ERCP and MRCP Inspissated bile syndrome ❑ Abdominal ultrasonography ❑ Intraperative cholangiogram Gallstone ❑ LFT ❑ Serum amylase and lipase ❑ CBC with differentials ❑ Transabdominal ultrasonography

Treatment

Shown below is an algorithm summarizing the treatment of jaundice in kids.

Jaundice

Unconjugated hyperbilirubinemia

Conjugated hyperbilirubinemia

Hemolytic anemia

Non-hemolytic causes of jaundice

Physiological jaundice ❑ Reassure

Hepatic etiology

Post-hepatic etiology

❑ Blood transfusion ❑ Erythropoietin ❑ Immunosuppression ❑ Treat the underlying cause

Breast milk jaundice ❑ Spontaneously resolves within 12 weeks ❑ Reassure and follow-up ❑ Continue breast feeding ❑ Phototherapy Breast feeding jaundice ❑ Increase the frequency of breast feeding ❑ Adequate hydration ❑ Monitor bilirubin levels ❑ Formula feeding if no improvement ❑ Phototherapy Gilbert syndrome ❑ No treatment ❑ Reassurance ❑ Patient education about the excellent prognosis Crigler-Najjar syndrome ❑ Phenobarbital ❑ Phototherapy ❑ Plasmapheresis ❑ Exchange transfusion ❑ Long term monitoring

Viral hepatitis ❑ Bed rest ❑ Symptomatic treatment ❑ Supportive care ❑ Observation ❑ Steroids ❑ Antiviral therapy Hepatotoxic drugs ❑ Discontinue the drug ❑ Supportive care ❑ Close monitoring for improvement or worsening of symptoms Alcoholic hepatitis ❑ Discontinue alcohol ❑ Supportive care ❑ Close monitoring for improvement or worsening of symptoms Wilson's disease ❑ Chelation therapy (D-penicillamine or Trientine hydrochloride) ❑ Zinc acetate to prevent copper reaccumulation ❑ Liver transplantation Metabolic syndrome ❑ Treat the underlying etiology ❑ Liver transplantation

Biliary atresia ❑ Hepatoportoenterostomy (Kasai procedure) ❑ Liver transplantation Choledochal cyst ❑ Complete cyst excision and hepaticojejunostomy Inspissated bile syndrome ❑ Hydration ❑ High-dose ursodeoxycholic acid ❑ Intraoperative cholangiogram ❑ Cholecystectomy Gallstone ❑ Hydration ❑ Cholecystectomy

Do's

• Do a thorough investigation and treat the underlying cause.
• Advice sunlight exposure in neonatal jaundice.
• Educate patients and parents about the excellent prognosis in Gilbert syndrome.
• Monitor for any signs of dehydration and provide adequate fluid support.
• In unconjugated hyperbilirubinemia above 20mg/dl, start phototherapy immeditely to prevent kernicterus.
• Take a detailed history of all the used medications to search for hepatotoxic agents.
• Administer vaccine to all the family members and close contacts of a hepatitis A patient.
• Always use sterilised syringes.

Don'ts

• Do not forget to take a detailed birth history, feeding history, travel history, sexual history, and social history.
• Do not forget to screen for alcohol use disorder in adolescents.
• Do not forget to screen newborns for metabolic disorders or inborn errors of metabolism.
• Do not discontinue breast feeding in neonates.