Case 8: 6-day old - Jaundice Flashcards
3wk old presents with jaundice .what things should you ask?
- OLDCARTS
- mother’s/baby’s blood type
- weight, energy, feeding
- stool color
==> too old for breastfeeding/physiologic jaundice
in which type of hyperbilirubinemia would you have a weird stool color
conjugated hyperbilirubinemia ==> hepatic / excretory cause
== pale stools b/c not being colored with bili
3wk old, yellow with elevated tbili 12, dbili 7. what do you want to do urgently next? what are you concerned about
RUQ ultrasound
- biliary cyst / stone
- biliary atresia /stricture (== Kesei procedure <6w of age = Roux-en-Y of the bile duct to remove the stricture)
outcomes of Kesei for biliary atresia
1/3 – transplant in first 1y of life
1/3 – transplant in school age (10y)
1/3 – long term Kesei survivors
What does elevated AST mean?
damage to hepatocytes, heart muscle [myositis], RBCs [hemolysis], skeletal muscles
AST > ALT == alcoholic cirhossis; otherwise not the liver
What does elevated ALT mean?
damage to hepatocyte
ALT > AST == hepatocyte death, hepatitis
What does elevated alk phos mean?
bone, osteblasts, cholangiocytes, brain
growth spurt in toddlers (3yo) and adolescents (13yo)
diffdx for large total protein (nml 7.5-8g/dL) and albumin (nml 4 g/dl)
== measure of immunoglobulins
multiple myeloma
HIV
diffdx low albumin
malnourished
chronic disease
glomerulonephritis
liver problem
Real liver function test ==> i.e., labs to get when concerned for liver damage / failure
- TB
- albumin
- PT/INR (b/c factor 7 fastest)
- ammonia
- glucose – d/t gluconeogenesis, glycogenolysis
Briar’s disease
familial progressive intrahepatic cholestasis
Type I, II == low GGT, elevated LFTs, cholestasis
Type III ==
what are the kdiseaes with low GGT, elevated LFTs
- mitochondrial bile salt conjugation defect
- Briar’s type I and II
define: jaundice
which is worse?
hyperbilirubinemia
unconjugated hyperbilirubinemia WORSE > conjugated hyperbilirubinemia
define: kernicterus
- etiology
- sxs
- sequelae:
- management:
1) staining of the basal ganglia & cranial nerve nuclei by bilirubin
2) clinical condition- toxic effects of high levels of unconjugated bilirubin
Etiology:
-Rh incompatibility -induced hemolysis (erythroblastosis fetalis) ==> hemolysis ==> unconjugated hyperbilirubinemia (tbili > 25mg/dL)
Sxs:
- severe anemia
- shock/acidosis
Sequelae: - lose suck reflex - become lethargic - hyperirritability, seizures - death (long-term) - opisthotonus = abnormal posturing that involves rigidity & severe arching of back + head thrown backward - rigidity - oculomotor paralysis - tremors - hearing loss - ataxia
management:
- screening: Rh incompatibility
- prevention: anti-Rh immunoglobulin (RhoGAM)
- treatment: phototherapy –> for unconjugated hyperbilirubinemia, goal bili < 20mg/dl
newborn bilirubin physiology
NEWBORNS: 75% of bilirubin from physiological breakdown of RBCs
1) PERIPHERY = RBC breakdown –> Hgb release –> UNCONJUGATED BILIRUBIN = insoluble in aqueous solutions, binds to albumin in blood stream
2) LIVER (hepatocytes) = extract bilirubin + cytosolic proteins ==> conjugated + glucuronide (by uridine diphosphate glucuronyl transferase == UDPGT, glucuronosyl transferase ==> CONJUGATED BILIRUBIN = water-soluble excreted into bile –> intestine
differentiate bile excretion in newborns v. adult
ADULT
- bile metabolized by intestinal flora to urobilin –> excreted in stool
==> reabsorb through bile salts
NEWBORN (enterohepatic circulation)
- lack GI flora to metabolize bile
==> beta-glucuronidase present in meconium hydrolyzes conjugated bilirubin –> back to unconjuated form
==> reabsorbed into bloodstream + bind to albumin (lots of bilirubin)
diffdx jaundice in the newborn
- PHYSIOLOGIC JAUNDICE: 1-2w (peak jaundice on day 3-4 of life)
- BREASTFEEDING “lack of breast-feeding” JAUNDICE: 1w of life
- BREAST MILK JAUNDICE: 4-7d (peak at 10-14d)
- HEMOLYSIS = ABO/Rh incompatibility, G6PD deficiency
- NON-HEMOLYTIC RED CELL BREAKDOWN
- extensive bruising from birth trauma
- large hemorrhage (ex. cephalohematoma, intracranial) = reabsorption of blood & metabolism of RBCs
- polycythemia
- swallowed blood during delivery
- METABOLIC ERROR (neonatal screening) = liver dysfunction + jaundice + seizures + sepsis + ascites
1) Crigler-Najjar syndrome–> deficiency / absent UDPGT –> decrease bili clearance (high indirect bili)
2) Gilbert syndrome –> decreased glucuronyltransferase activity; harmless (jaundice alone) (high indirect bili)
3) Galactosema
4) hypothyroidism
5) urea cycle defects
INTRINSIC LIVER DISEASE
- ETHNICITY: Asian > Caucasian > black
- SEPSIS = jaundice + other clinical signs; esp. if not breastfeeding ==> losing out on colostrum-provided preformed Abx, cells & other anti-infective substances
- TORCH infection== jaundice + hepatosplenomegaly + microcephaly and/orrash
- prematurity
- bowel obstruction
- birth at high altitude (erythropoiesis)
Jaundice in a baby: PHYSIOLOGIC JAUNDICE
- timing:
- labs:
- who’s at risk:
- prognosis:
- pathophys:
PHYSIOLOGIC JAUNDICE
- timing: 1-2w (peak jaundice on day 3-4 of life)
- labs: tbili = 15
- who’s at risk: full term,otherwise healthy
- prognosis: benign, self-limited
- pathophys: increased enterohepatic circulation:
1) increased short-lived fetal RBCs –> increased bilirubin production
2) immature liver –> deficiency of hepatocyte proteins and UDPGT
3) lack of intestinal flora to metabolize bile
4) high levels of beta-glucuronidase in meconium
5) minimal oral intake in first 2-4d of life –> slow excretion of meconium (esp. in breastfed infants)
Jaundice in a baby: BREASTFEEDING “lack of breast-feeding” JAUNDICE
- timing:
- labs:
- who’s at risk:
- prognosis:
- pathophys:
BREASTFEEDING “lack of breast-feeding” JAUNDICE
- timing: 1w of life
- who’s at risk: healthy babies, where “milk let down” has not yet occured in mom
- prognosis: if persistent, neonate can become dehydrated & malnourished
- pathophys: low milk supply –> limited oral intake –> decreased GI motility –> meconium retention, where beta-gluruonidase in meconium deconjugates bilirubin –> ENTEROHEPATIC CIRCULATION –> reabsorption, elevated serum bili levels
Jaundice in a baby: BREAST MILK JAUNDICE
- timing:
- labs:
- who’s at risk:
- prognosis:
- pathophys:
- tx
BREAST MILK JAUNDICE (lots of breast milk)
- timing: 4-7d (peak at 10-14d)
- labs: diagnosis of exclusion (normal physical exam, no hemolysis, normal TSH, adequate milk supply)
- who’s at risk: 60% of newborns can become jaundiced.
- prognosis: can persist for 12w without problems; likely doesn’t reach critical levels
- pathophys: beta-gluruonidase inn breast milk deconjugates bilirubin –> ENTEROHEPATIC CIRCULATION –> reabsorption, elevated serum bili levels
- tx: continue breast-feeding and recheck total bilirubin level in 24h (only if VERY BAD would you interrupt for 24-48h)
Jaundice in a baby: HEMOLYSIS
- timing:
- labs:
- who’s at risk:
- prognosis:
- pathophys:
- pathophys: reakdown of RBCs –> released Hgb —> elevated unconjugated bilirubin = jaundice
1) Antibody-positive hemolysis = blood group incompatibility (lab = direct Coombs positive)
2) Antibody-negative hemolysis = RBC membrane defects (spherocytosis); RBC enzyme deficits (G6PD / pyruvate kinase deficiency)
most common forms of antibody-positive hemolysis
- Rh incompatibility = mother is Rh-negative, baby is Rh-positive
- ABO incompatibility = mother is type O, baby is A or B
- incompatibilities in minor blood group antigens
typical breastfeeding pattern
8-12x every day
1) initially = lasts for 60min
2) ultimately = 10-15min at each breast
if sessions last for longer, without infant gaining weight ==> THIS IS A PROBLEM.
