Jaundice Flashcards

1
Q

What percentage of the total bilirubin needs to be conjugated to be concerning?

A

20%

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2
Q

Define Acute Bilirubin Encephalopathy

A
When the bilirubin crosses the BBB
Causing:
- Lethargy
- Hypotonia
- Poor feeding
- Fever
- Seizures
- Signs of increased intracranial pressure

Toxic levels of bilirubin: >20 mg/dL or 342 μmol/L
Associated with neurotoxicity, encephalopathy

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3
Q

Define kernicterus

A

The chronic and irreversible manifestations of bilirubin toxicity

  • Cognitive impairment
  • Cerebral palsy
  • SNHL
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4
Q

RF for Kernicterus

A
Sepsis
Hypoxia
Hypercarbia
Brisk hemolysis ( with RH incompatibility)
Hypoglycemia 
Prematurity
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5
Q

Indications that jaundice is pathologic

A
Jaundice in the first day of life
Bilirubin that increases rapidly
Conjugated bilirubin > 10% total
Jaundice beyond the first week of life
Jaundice with HSM and anemia
Jaundice in ill-appearing infants
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6
Q

Ix for jaundice

A

Total Bilirubin
Direct and Indirect levels

If Mom’s Blood type is incompatible
- CBCD, smear, retic, DAT
If evidence of hemolysis but DAT neg - could be an intrinsic RBC defect - G6PD
- should do a G6PD assay and RBC fragility test
Ill-aopearing infants - need a PSWU/FSWU

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7
Q

Define breast milk jaundice

A

Likely due to dehydration in the early stages as mom’s milk is coming in - low fluid intake, low caloric intake, decreased passage of mec stools

Later due to lipase and non-esterified long chain FA in the breast milk inhibit hepatic excretion of bilirubin
Beta-glucuronidase in breast milk increases enterohepatic circulation of bilirubin
Often when jaundice worsens from DOL 4 to 10

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8
Q

Define physiologic jaundice

A

Normal jaundice due to:

  • large RBC mass
  • decreased survival of RBCs
  • decreased hepatic uptake of bilirubin
  • decreased conjugation of bili
  • increased enterohepatic recirculation of bili

Usually peaks at DOL 3

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9
Q

Describe the physiology of bilirubin formation and removal from the body

A

Formed by breakdown of heme-containing proteins (primarily Hb)
Heme protoporphyrin is degraded into biliverdin and unconjugated bilirubin
Unconjugated bilirubin crosses the BBB
Unconjugated bilirubin is bound to albumin and carried to the liver
In the liver, bilirubin is conjugated by glucoronyl transferase
Conjugated bilirubin is then excreted into bile

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10
Q

RF for elevated bilirubin

A

MATERNAL
ABO or Rh incompatibility –> leads to hemolytic disease
Drugs – diazepam, oxytocin
Maternal illness – GDM
DifficultY with breastfeeding
Maternal age > 25
Ethnic background – Asian, European, Native American

NEONATAL
Birth trauma (instrumentation, development of a cephalohematoma)
Drugs – Pediazole, chloramphenicol, ceftriaxone
Excessive weight loss after birth
TORCH infections
Infrequent feedings and dehydration
Male gender
Polycythemia
Prematurity 
Previous sibling with hyperbilirubinemia
Delayed Meconium
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11
Q

DDx of Hyperbilirubinemia for infants

UNCONJUGATED

A

BENIGN/PHYSIOLOGIC
Physiologic Jaundice of the newborn
Breast milk jaundice

HEMOLYSIS
ABO incompatibility
Cephalohematoma (physiologic breadown of birth trauma hematoma)
Intraventricular or Intracranial hemorrhage
Spherocytosis
Elliptocytosis
Sickle cell anemia
Thalasesemia
G6PD deficiency
Pyruvate kinase deficiency

INFECTIONS
TORCH infections
UTI
Sepsis

OBSTRUCTIVE
Meconium Ileus
Hirschsprung’s disease
Duodenal atresia
Pyloric Stenosis
METABOLIC/GENETIC
Galactosemia
Congenital hypothyroidism
Crigler-Najjar syndrome
Gilbert’s syndrome
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12
Q

DDx of Hyperbilirubinemia for infants

CONJUGATED

A
INFECTIONS
TORCH infections
UTI
Gram negative sepsis
Tuberculosis
Hepatitis B
HIV infection
Varicella
Coxsackievirus
Echovirus
Listeriosis
OBSTRUCTIVE
Biliary atresia
Choledochal cyst
Bile duct stricture
Congential Hepatic fibrosis
Inspissated bile syndrome
Alagille syndrome
Byler’s disease
METABOLIC/GENETIC
Galactosemia
Tyrosinemia
Glycogen storage disease Type IV
α-antitrypsin deficiency
Cystic fibrosis
Dubin-Johnson syndrome
Neonatal hypopituitarism
Zellweger’s syndrome
Donohue syndrome (Leprechaunism)
Rotor syndrome
Niemann-Pick disease
Woleman’s disease
Gaucher’s disease
Cholesterol ester storage disease

MISCELLANEOUS
Drugs and toxins
Parenteral nutrition

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13
Q

What are the TORCH infections?

A
T – Toxoplasma
O – Other = HepB, syphilis, VZV, parvovirus B19
R – Rubella
C – CMV (most common)
H – HSV
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14
Q

When does jaundice become clinically apparent?

A

TSB > 85.5 μmol/L

5 mg/dL

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15
Q

DDx for jaundice in OLDER children

UNCONJUGATED

A
GENETIC
Sickle cell anemia
Thalassemia
Spherocytosis, elliptocytosis
G6PD deficiency
Pyruvate kinase deficiency
Crigler-Najjar syndrome
Gilbert’s syndrome
OTHER
Drug-induced hemolytic anemia
Auto-immune hemolytic anemia
Microangiopathic hemolytic anemia
Hypersplenism
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16
Q

DDx for jaundice in OLDER children

CONJUGATED

A
OBSTRUCTIVE
Gallstones
Tumor
Choledochal cyst
Bile duct stricture

INFECTIONS
Hepatitis
Sepsis
UTI

GENETIC
Cystic Fibrosis
Wilson’s disease
Dubin-Johnson syndrome
Rotor syndrome
Glycogen storage disease Type IV
α-antitrypsin deficiency
OTHER
Cirrhosis
Sclerosing cholangitis
Cholestatic jaundice of pregnancy
Drugs and toxins (tylenol, estrogens, Abx, amanita mushrooms, antiepileptic drugs)
17
Q

DDx of jaundice according to timing

<24h, 24-72h, 72h-96h, > 1 week

A

<24h
ALWAYS PATHOLOGIC
Hemolysis - Rh or ABO incompatibility
Sepsis - GBS, Congenital infection (TORCH)

24-72h
Physiologic, polycythemia
Dehydration/ breast feeding jaundice
Hemolytic
-G6PD deficiency aka Mediterranean jaundice; weakness in structure of RBC’s (oxidative damage) causes them to burst
-Pyruvate kinase deficiency
-Spherocytosis
-Trauma from delivery
Sepsis/ “TORCH”

72h - 96h
Physiologic +/- breast feeding jaundice
Sepsis

> 1 week
Breast milk jaundice
Prolonged physiologic jaundice
Hypothyroidism
Neonatal hepatitis
Conjugation dysfunction (Gilbert’s syndrome, Crigler-Najjar syndrome)
Inborn errors of metabolism
Obstruction eg. Biliary atresia
18
Q

What levels of bilirubin are concerning?

A

Severe Hyperbilirubinemia: TSB > 340 μmol/L at any time during 1st 28 d of life

Critical Hyperbilirubinemia: TSB > 425 μmol/L at any time during 1st 28 d of life

19
Q

What is the workup for conjugated hyperbilirubinemia?

A
AST,  ALT,  ALP, GGT
INR/PTT
Ammonia, Glucose,  Albumin
Gas
Sepsis workup
Alpha-1 antitrypsin levels, Copper, ceruloplasmin
Abdo US
Urine reducing substances
Consult GI
20
Q

List the drugs that can cause cholestasis leading to conjugated hyperbilirubinemia

A

Anticonvulsants (phenobarb, phenytoin, carbamazepine, valproic acid)
Antibiotics (erythromycin, tetracycline, sulfonamides, isoniazid, rifampin, ketoconazole, griseofulvin)
Corticosteroids
OCP
Acetaminophen
Salicylates
Chlorpromazine
Cimetidine
Immunosuppressants (cyclosporine, azathioprine, methotrexate)

21
Q

Indications for admission for a child with jaundice

A
Dehydration
Hypoglycemia
Active biliary tract disease
Severe bacterial infection
Ongoing hemolysis
Signs of systemic disease
Change in mental status
Hepatic failure 
Diagnosis is uncertain