Jaundice Flashcards
What percentage of the total bilirubin needs to be conjugated to be concerning?
20%
Define Acute Bilirubin Encephalopathy
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
Define kernicterus
The chronic and irreversible manifestations of bilirubin toxicity
- Cognitive impairment
- Cerebral palsy
- SNHL
RF for Kernicterus
Sepsis Hypoxia Hypercarbia Brisk hemolysis ( with RH incompatibility) Hypoglycemia Prematurity
Indications that jaundice is pathologic
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
Ix for jaundice
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
Define breast milk jaundice
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
Define physiologic jaundice
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
Describe the physiology of bilirubin formation and removal from the body
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
RF for elevated bilirubin
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
DDx of Hyperbilirubinemia for infants
UNCONJUGATED
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
DDx of Hyperbilirubinemia for infants
CONJUGATED
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
What are the TORCH infections?
T – Toxoplasma O – Other = HepB, syphilis, VZV, parvovirus B19 R – Rubella C – CMV (most common) H – HSV
When does jaundice become clinically apparent?
TSB > 85.5 μmol/L
5 mg/dL
DDx for jaundice in OLDER children
UNCONJUGATED
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
DDx for jaundice in OLDER children
CONJUGATED
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)
DDx of jaundice according to timing
<24h, 24-72h, 72h-96h, > 1 week
<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
What levels of bilirubin are concerning?
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
What is the workup for conjugated hyperbilirubinemia?
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
List the drugs that can cause cholestasis leading to conjugated hyperbilirubinemia
Anticonvulsants (phenobarb, phenytoin, carbamazepine, valproic acid)
Antibiotics (erythromycin, tetracycline, sulfonamides, isoniazid, rifampin, ketoconazole, griseofulvin)
Corticosteroids
OCP
Acetaminophen
Salicylates
Chlorpromazine
Cimetidine
Immunosuppressants (cyclosporine, azathioprine, methotrexate)
Indications for admission for a child with jaundice
Dehydration Hypoglycemia Active biliary tract disease Severe bacterial infection Ongoing hemolysis Signs of systemic disease Change in mental status Hepatic failure Diagnosis is uncertain
