Differentiate between physiological and pathological jaundice.
State causes of hyperbilirubinemia.
Describe the most dangerous complication of hyperbilirubinemia.
Discuss the management of hyperbilirubinemia
Definition: Hyperbilirubinemia Hyperbilirubinemia: excessive level bilirubin in the blood characterized by jaundice, a yellowish discoloration of the skin, sclerae, mucous membranes and nails Typically seen at bili levels of: 85-120 Unconjugated bilirubin = Indirect bilirubin. Conjugated bilirubin = Direct bilirubin.
Why am I learning this? Is it important?
Why? Jaundice is quite common Full term infants: at least 60% Preterm infants: over 80%
Most Importantly… Kernicterus: unconjugated bilirubin deposits in the brain yellow staining + degenerative lesions Phase 1: decreased alertness Hypotonia Poor feeding Phase 2: Hypertonia, Retrocollis, opisthotonus Phase 3: Hypotonia
Source Of Bilirubin
85% from old RBC , the rest
from non haem proteins
Hb is degraded to Haem
Iron is extracted from Haem
Rest is converted to bilirubin
Bilirubin travels to liver bound
Journey through the liver Bilirubin taken up Conjugated to form water soluble conjugate Conjugate secreted into bile
In The Gut Bilirubin diglucuronide may be Deconjugated or Metabolised by bacteria to urobilinogenpartially reabsorbed (remainder makes the stool brown)
So where can things go wrong?
Pathophysiology Of Jaundice Hyperbilirubinemia is due to:
Back to our table..let’s break things down into basics..
Now that you’re a pro.. You’re called by a nurse for a new admission regarding a baby with elevated bili..what do you want to know
Approach to jaundiced baby Get age of baby (hours), gestational age, pregnancy hx, septic risk factors, complications with delivery Assess clinical condition (well or ill) Decide whether jaundice is physiological or pathological Look for evidence of kernicterus* in deeply jaundiced NB *review..what do you look for?
Approach to jaundiced baby Get age of baby (hours), gestational age, pregnancy hx, septic risk factors, complications with delivery Assess clinical condition (well or ill) Decide whether jaundice is physiological or pathological Look for evidence of kernicterus* in deeply jaundiced NB *Lethargy and poor feeding, poor or absent Moro's, opisthotonus or convulsions
Why does the age (hours) of baby matter?
Causes of jaundice Appearing within 24 hours of age Hemolytic disease of NB : Rh, ABO Infections: TORCH, malaria, bacterial Appearing between 24-72 hours of life Physiological G6PD deficiency Dehydration (breast feeding jaundice) Sepsis Polycythemia Concealed hemorrhage Intraventricular hemorrhage Increased entero-hepatic circulation Appearing beyond 1 week Breast milk jaundice Prolonged physiologic jaundice in preterm Hypothyroidism Neonatal hepatitis Conjugation dysfunction - e.g. Gilbert syndrome, Crigler-Najjarsyndrome Inborn errors of metabolism - e.g. galactosemia Biliarytract obstruction - e.g. biliaryatresia
What workup/labs do you order
Workup Initial laboratory tests Total & direct bilirubin Blood group and Rh for mother and baby CBC/d, reticcount and peripheral smear Coombtest TSH, G6PD screen Conjugated hyperbilirubinemia: AST, ALT, PT, PTT, serum albumin, ammonia, TSH, TORCH screen, septic work-up
Treatment During pregnancy (if severe) Intrauterine blood transfusion Early delivery After pregnancy Increase feeds (especially in breast feeding jaundice) Phototherapy Exchange transfusion (if severe)
Side effects of phototherapy Increased insensible water loss Loose stools Skin rash Bronze baby syndrome Hyperthermia Upsets maternal baby interaction