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Babelyn T. Dimabayao
Jaundice
Hyperbilirubinemia
to an excessive level of accumulated bilirubin in the blood Characterized by jaundice, a yellowish discoloration of the skin, sclerae, mucous membranes and nails Total serum bilirubin (TSB) of >5 mg/dL
Refers
Significance
in up to 60% of term and 80% of preterm Severe complications possible
Present
kernicterus
Neonatal Jaundice
Increased rbcs Shortened rbc lifespan Immature hepatic uptake & conjugation
Bilirubin
Ligandin (Y - acceptor) Bilirubin glucuronidase
Intestine
Bil glucuronide
Bilirubin
Stercobilin
Area of body Face Upper trunk Lower trunk & thighs Arms and lower legs Palms & soles
Bilirubin levels
mg/dl (*17=umol)
Neonatal Jaundice
Differential Diagnosis
1st 24 hours
Erythroblastosis fetalis Concealed hemorrhage Sepsis or congenital infections (syphilis, cytomegalovirus, rubella, and toxoplasmosis) Extensive ecchymosis or blood extravasation (premature infants)
Differential Diagnosis
3rd day and within the 1st wk
Bacterial sepsis or urinary tract infection Other infections (syphilis, toxoplasmosis, cytomegalovirus, or enterovirus)
Differential Diagnosis
1st month of life
Hyperalimentation-associated cholestasis Hepatitis Cytomegalic inclusion disease Syphilis Toxoplasmosis Familial non-hemolytic icterus Congenital atresia of the bile ducts Galactosemia Inspissated bile syndrome following hemolytic disease of the newborn
Physiologic Jaundice
Appears after 24 hours Maximum intensity by 4th-5th day in term & 7th day in preterm Serum level less than 15 mg/dl Disappears without any treatment Increased bilirubin production from the breakdown of fetal RBCs combined with transient limitation in the conjugation of bilirubin by the immature liver
Physiologic Jaundice
Breastmilk Jaundice
Elevated unconjugated bilirubin Prolongation of physiologic jaundice
Slower
66% of breastfed babies jaundiced May persist up to 3 months
May
Average max TSB = 10-12 mg/dL TSB may reach 22-24 mg/dL Milk factor (?)
Beta
Breastfeeding Jaundice
Elevated unconjugated bilirubin Benign or pathologic
Elevated
bilirubin in the 1st week of life tends to worsen breast milk jaundice during later weeks
Risk Factors
Pathologic Jaundice
Features
in 1st 24 hrs Rapidly rising TSB (> 5 mg/dL per day) TSB > 17 mg/dL Direct bilirubin >2mg/dL
Jaundice
Erythroblastosis Fetalis
Develops in an unborn infant when the mother and baby have different blood types Mother produces antibodies that attack the developing baby's red blood cells
ABO Incompatibility
Occurs when the major blood group antigens of the fetus are different from those of the mother Most common is between a type O mother and type A or B infant May occur in the first pregnancy
Rh Incompatibility
Rh-negative pregnant mother is exposed to Rhpositive fetal red blood cells
y
Fetomaternal hemorrhage during the course of pregnancy from spontaneous or induced abortion, trauma, invasive obstetric procedures, or normal delivery
Rh-negative female receives an Rh-positive blood transfusion Usually manifests in second/succeeding pregnancies
Infections
Congenital TORCH Infection Hepatitis Sepsis UTI Pneumonia Partly due to inadequate liver functioning but majority is due to plugging and subsequent bile stasis Intracellular accumulation of unconjugated bilirubin and by "toxic" cellular alterations, such as giant-cell transformation
Infections
G6PD Deficiency
A cause of kernicterus in up to 35% of cases Always suspect if severe hyperbilirubinemia or poor response to phototherapy Ethnic origin
11-13% of African Americans Mediterranean, Middle East, Arabian peninsula, SE Asia, Africa
Levels may be normal or elevated early Especially in presence of hemolysis Repeat level at 3 months
G6PD Deficiency
G6PD:
Oxidation
of glucose-6-phosphate to 6phosphogluconate while concomitantly reducing the oxidized form of nicotinamide adenine dinucleotide phosphate (NADP+) to nicotinamide adenine dinucleotide phosphate (NADPH)
RBCs
rely on enzyme activity as a source of NADPH that protects the cells against oxidative stresses
G6PD Deficiency
Erythrocytes produce ATP thru glycolysis PK potentiates the last step of glycolysis (phosphoenolpyruvate converted to pyruvate) Hampers Na-K ATPase Echinocytes Crigler-Najjar Type I and II Gilberts Syndrome
UGT Deficiency
Drug-Induced Jaundice
Competitive Binders
Aspirin Ceftriaxone Sulfisoxazole
Prevention
Breastfeeding
Should
be encouraged for most women 8-12 times/day for 1st several days Assistance and education Avoid supplements in non-dehydrated infants
Do
Prevention
Ongoing assessments for risk of developing severe hyperbilirubinemia
Monitor
Mom not tested, Rh (-): Coombs, ABO, Rh Mom O or Rh (+): optional to test cord blood
Laboratory Investigation
Indicated (if bilirubin concentrations reach phototherapy levels)
Serum total or unconjugated bilirubin concentration Serum conjugated bilirubin concentration Blood group with direct antibody test (Coombs test) Hemoglobin and hematocrit determinations
Discharge
Assess risk
bilirubin Use nomogram to determine risk zone And/or Assessment of risk factors
Predischarge
Discharge
Close follow-up necessary
Individualize based on risk Weight, % change from BW, intake, voiding habits, jaundice
Phototherapy
Mechanism: converts bilirubin to water soluble form that is easily excreted Forms
Fluorescent lighting Fiberoptic blankets
Goal is to decrease TSB by 4-5 mg/dL or < 15 mg/dL total Breastfed infants are slower to recover
Phototherapy
Bilirubin levels > 20 mg/dL, phototherapy should be administered continuously until levels fall below 20 mg/dL, upon which it can be administered intermittently
Exchange Transfusion
Mechanism: removes bilirubin and antibodies from circulation and correct anemia Most beneficial to infants with hemolysis
Indications
Jaundiced
infant with clinical signs of BIND (lethargy, hypotonia, poor-sucking, high pitched cry) Infants with a TB level greater than the threshold defined by the AAP
After a successful procedure, TB initially falls to half its pre-transfusion value and eventually equilibrates at two-thirds of this initial level
Pharmacologic Treatment
IVIG: can reduce the need for exchange transfusion in infants with hemolytic disease caused by Rh or ABO incompatibility Phenobarbital: increases the conjugation and excretion of bilirubin Ursodeoxycholic acid: increases bile flow and is useful in the treatment of cholestatic jaundice
Complications
Toxicity to basal ganglia and brainstem nuclei 2 terms
Acute bilirubin encephalopathy Kernicterus
Multiple phases
Risk of Kernicterus
TSB level > 25-30 mg/dl Acidosis Increased free bilirubin Low albumin, drug displacement Blood-brain barrier disruption Prematurity, sepsis, ischemia
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