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39 Cards in this Set

  • Front
  • Back

When should a neonate be evaluated for cholestasis?

  • Those that have jaundice beyond 14-21 days after birth

Clinical features of biliary atresia
  • Neonatal cholestasis
  • Normal birth weight and postnatal weight gain
  • Normal stools changing to acholic stools
  • Bilirubin usually 6-12, only moderately elevated ALT/AST and alk phos

Why do newborns have substantially higher levels of bilirubin than adults?

  • Increased RBC mass (higher Hct)

  • Shortened RBC lifespan (70-90 vs 120 days in adults)

  • Degradation of extra hematopoietic tissue (liver, spleen, marrow)

  • Immature liver (decreased glucuronyl transferase)

  • Increased enterohepatic recirculation

What is Crigler-Najjar syndrome?

  • Rare, AR permanent deficiency of glucuronsyltransferase

  • Severe indirect hyperbilirubinemia

What is Gilbert disease?

  • Mutation of promoter region of glucuronosyltransferase

  • Mild indirect hyperbilirubinemia

What is breast milk jaundice?
  • Unconjugated hyperbilirubinemia without hemolysis
  • Breast milk may contain an inhibitor of bilirubin conjugation or may increase enterohepatic recirculation due to breast milk glucuronidase

How to treat jaundice on the first day of life

  • Always pathologic, require immediated attemption

  • Often a result of hemolysis, internal hemorrhage, or infection

Predominantly unconjugated hyperbilirubinemia, what next?

Check for red flags



  • Bilirubin >95th percentile for hours
  • Onset of jaundice on first day
  • Rapid rise of bilirubin (>.5mg/dL/hour)
  • Maternal blood O, Rh +

Predominantly unconjugated hyperbilirubinemia, red flags present, what now?

  • Coombs test

  • CBC with smear

  • Reticulocyte count

  • Infant and maternal blood type

Predominantly unconjugated hyperbilirubinemia, red flags present.




Coombs test positive.




What next?

  • Isoimmune hemolytic disease

  • Rh

  • ABO

  • KELL

Predominantly unconjugated hyperbilirubinemia, red flags present.



Coombs test negative




High hematocrit

  • Polycythemia

  • Diabetic mother

  • Delayed clamping

  • Fetal transfusion

  • Intrauterine hypoxia

Predominantly unconjugated hyperbilirubinemia, red flags present.




Coombs test negative




Hematocrit normal




What next?

  • Check reticulocyte count

Predominantly unconjugated hyperbilirubinemia, red flags present.




Coombs test negative




Hematocrit normal




Elevated reticulocyte count



What next?
  • Hb electrophoresis
  • G6PD Deficiency


RBC membrane defects


RBC enzyme deficiencies


Hemoglobinopathy

Predominantly unconjugated hyperbilirubinemia, red flags present.




Coombs test negative




Hematocrit normal




Reticulocytes normal




Diagnosis?

  • Breast feeding jaundice

  • Physiologic jaundice

  • Extravascular blood

  • Infection/sepsis

  • Hypothyroid

  • Drugs/toxins

  • Metabolic disease

  • Familial hyperbilirubinemia (Gilbert/Crigler)

Predominantly conjugated hyperbilirubinemia, what next?
  • CBC + differential
  • Blood and urine
  • Newborn screen
  • Serum glucose
  • Albumin
  • LFT's
  • PT time
  • TORCH titers
  • VDRL/PPR
  • Alpha 1 antitrypsin
  • Liver ultrasound

Dark urine and gray-white stools + jaundice after 2nd week of life suggests?

Biliary atresia

Symptoms of kernicterus

  • Lethargy

  • Hypotonia

  • Irritability

  • Poor feeding




Later = bulging fontanelle, pulmonary hemorrhage, fever, hypertonicity, paralysis of upward gaze, seizures

Therapy of indirect hyperbilirubinemia

  • Phototherapy

  • Bilirubin is transformed into isomers that are water soluble and easily excreted

  • Complications = increased water loss, diarrhea, dehydration, macular-papular rash, lethargy, and more

Treatment for greatly increased indirect bilirubin levels, risk for kernicturus.


  • Indirect bilirubin 20mg/dL, infant with hemolysis, greater than 2000g
  • Exchange transfusion usually through umbilical venous cather to IVC

Requirement for conjugated hyperbilirubinemia

  • Direct bilirubin level > 2 mg/dL or > 20% of total bilirubin

How to distinguish a toddler with jaundice versus one with cartotenemia?

The one without jaundice will have white sclera.

In what way does neonatal jaundice progress?

  • Cephalocaudal progression

  • Icteric sclera visible at 5-7 mg/dl

  • Head/neck at 4-8 mg/dl

  • Upper body at 5-12 mg/dl

  • Lower body/thighs at 8-16 mg/dl

  • Palms/soles > 15 mg/dl

Criteria for pathologic jaundice

  • Jaundice in first 24 hours

  • Conjugated > 2

  • Bilirubin increasing by > 5mg/dl/day or >.5mg/dL/hr

  • Total bili > 5 on day 1, or >15 after day 1

  • Visible jaundice on day 1 or after 2 weeks

Non-hemolytic causes of increased bilirubin production in newborn

  • Hematoma or bruising

  • Hemorrhage

  • Excess RBC load (polycythemia) (Hct >60)

Treatment for biliary atresia

Kasai procedure (portoenterostomy)




Removes blocked duct and gallbladder and creates a bypass with small intestine.




Achieves biliary drainage, 10 year survival of 30% without transplant


  • Onset 2-4 days, peak 2-8 and decreases
  • Normal physical
  • Yellow coloration starting on face and progressing to abdomen then feet
  • Yellow sclera



  • Bilirubin rising at a rate of less than 5mg/dL/day
  • Direct bilirubin < 2 mg/dL
  • Hb normal
  • Retic normal
  • Coombs negative
  • CBC normal


Diagnosis?
  • Physiologic Jaundice

Treatment of physiologic jaundice
  1. Parent education
  2. Increase feeds to 10/24 hours
  3. Follow up with total and direct bili every 12-24 hrs
  4. Indirect beyond 2 weeks = abnormal
  5. Monitor for other signs/symptoms
  • Onset after 7th day, gradually decreases
  • Feeding: Breast milk
  • May persist 3-10 weeks, serum bilirubin gradually decreases if breast-feeding continued, rapidly falls with discontinuation




  • Normal physical
  • Yellow coloration of skin starting on face, progressing to abdomen and then feet
  • Yellow sclera





  • Bilirubin rising < 5mg/dL/day
  • Direct < 2 mg/dL
  • Hb normal
  • Retic normal
  • Coombs normal
  • CBC normal


Diagnosis?

Jaundice associated with breast-feeding (breast milk jaundice)

Treatment for jaundice associated with breast-feeding (breast milk jaundice)?


  1. Frequent breast-feeding
  2. Ongoing lactation support
  3. Breast-feeding should be continued
  4. Frequent feeding and supplementation with formula
  5. OR temporarily interrupt breast feedings and substitute formula for a day or two




  • Parent education
  • Follow up with total and direct bili every 12-24
  • Monitor baby
  • Phototherapy if indirect does not trend down

What makes breast-feeding jaundice different than breast milk jaundice?


  • Onset during 1st week (earlier)
  • Reduced urine output and BM
  • Decreased milk production and decreased intake




  • Excessive weight loss
  • Signs and symptoms of dehydration
  • Jaundice at birth or within 24 hours
  • Splenectomy or sibling with neonatal jaundice in history
  • Maternal history of oxytocin, sulfonamides, antimalarials, or nitrofurantoins



  • Pallor
  • Yellow coloration of skin, face -> feet
  • Yellow sclera
  • Cephalohematoma
  • Hepatosplenomegaly



  • Bilirubin rising >5mg/dL/day
  • Direct bilirubin < 2 mg/dL
  • Hb normal
  • Retic high
  • Coombs test positive
  • Smear = spherocytes, hemolysis, pyknocytes
  • May be ABO incompatibility or Rh incompatibility



Diagnosis?

Jaundice due to hemolytic state or hematoma (Hereditary spherocytosis, pyknocytosis, pyruvate kinase deficiency, G6PD deficiency, Thalassemia, Vit-K induced) (Acquired from ABO or Rh incompatibility or drugs)

Treatment for hemolytic state/hematoma jaundice


  1. Phototherapy with supportive care
  2. Phenobarbital (better if given to mother 1-2 weeks before delivery in a hemolytic baby)
  3. Metalloporphyrins
  4. Exchange transfusion (when kernicterus risk is significant)--> cuts bili in about half
  • Onset 2-4 days
  • DM in mother
  • Hypoxia + respiratory distress after birth




  • Yellow coloration face -> feet
  • Yellow sclera
  • Macrosomia
  • Apnea, tachypnea, temp instability
  • Hepatosplenomegaly




  • Bilirubin rising < 5mg/dL/day
  • Direct < 2mg/dL
  • Hb normal
  • Retic high
  • Coombs test negative
  • Smear normal
  • Hypoglycemia + acidosis



Diagnosis?

Jaundice due to metabolic disorder (hypoxia, resp distress, hypothyroid, DM mother)

Treatment for jaundice due to metabolic state


  1. Phototherapy + supportive care
  2. Correct abnormal chemistry
  3. Exchange transfusion (when kernicterus risk is significant)
  • Onset 2-4 days
  • Peak 1 week
  • Duration variable
  • Could have severe jaundice with yellow stool if severe




  • Yellow coloration face -> feet
  • Yellow sclera




  • Bilirubin rising < 5mg/dL
  • Direct < 2mg/dL
  • Hb normal
  • Retic normal
  • Coombs negative
  • Peripheral blood smear normal
  • Bilirubin in bile could be as low as <10mg/dL depending on severity
  • MEASURE GLUCURONYL TRANSFERASE activity and get liver specimen for closed biopsy

Jaundice due to genetic disorder (Crigler Najjar Type I + II and Gilbert Syndrome)

Treatment for jaundice due to genetic disorder


  1. Phototherapy + supportive care
  2. Exchange transfusion (if risk of kernicterus is significant)
  3. CN I = bad prognosis, kernicterus
  4. CN II = phenobarbital

  • May be in first 24 hours or first 4-7 days

  • Maternal Hx of TORCH infection

  • History of intrauterine transfusion

  • Poor feeding





  • Yellow discoloration face -> feet
  • Yellow sclera
  • Microcephaly / hydrocephalus
  • Lethargy
  • Petechiae
  • Hepatosplenomegaly
  • Chorioretinitis/cataract
  • Pallor
  • Failed hearing screen




  • Bilirubin rising > 5mg/dL/day
  • Direct bilirubin < 2mg/dL
  • Hb normal
  • Retic high
  • Coombs test negative
  • Smear shows hemolysis
  • Thrombocytopenia
  • Intracranial calcification



Diagnosis?

Jaundice due to mixed hemolytic and hepatotoxic (sepsis, pyelonephritis, toxoplasmosis, cytomegalic inclusion disease, rubella, syphillis, drugs, vit k)

Treatment of miixed hemolytic and hepatotoxic jaundice


  1. Phototherapy + supportive care
  2. Correct anemia
  3. Treat cause
  4. Exchange transfusion (if kernicterus risk significant)
  • Onset 2-3 days, maybe by 2nd week
  • May be premature
  • Light or acholic stool, dark urine




  • Yellow discoloration face -> feet
  • Yellow sclera
  • Microcephaly/hydrocephalus
  • Lethargy
  • Petichiae
  • Hepatosplenomegaly
  • Chorioretinitis/cataract
  • Pallor
  • Failed hearing




  • Bilirubin rising >5mg/dL/day
  • Direct > 2mg/dL
  • Coombs negative
  • Assess stool color
  • Urine/serum bile acid measurement
  • Albumin + coagulation profile
  • Alpha antitrypsin
  • Thyroxine and TSH
  • Sweat chloride
  • Urine/serum AA's
  • US of abdomen
  • Hepatobiliary scinography
  • Liver biopsy



Diagnosis?

Jaundice due to hepatocellular damage (biliary atresia, familial cholestatsis, galactosemia, hypothyroidism, hepatitis + infection)