Good Morning! July 19, 2012

Semantic Qualifiers Symptoms Acute /subacuteChronic LocalizedDiffuse SingleMultiple StaticProgressive ConstantIntermittent Single EpisodeRecurrent AbruptGradual SevereMild PainfulNonpainful BiliousNonbilious Sharp/StabbingDull/Vague Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Localized problem Systemic problem AcquiredCongenital New problem Recurrence of old problem

 Predisposing Conditions  Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc)  Pathophysiological Insult  What is physically happening in the body, organisms involved, etc.  Clinical Manifestations  Signs and symptoms  Labs and imaging Illness Script

Predisposing Conditions Physiologic Jaundice**  Newborns in first full week of life…almost universal  Sibling with history of jaundice/requiring phototherapy  Asian ethnicity  Prematurity  Maternal diabetes  Breastfed infants**  Breast feeding is the most common cause of exaggerated unconjugated hyperbilirubinemia**  Breastfeeding (1 st week), Breast milk (6-14 days)**

Pathophysiology** Physiologic Jaundice

Clinical Manifestations Physiologic Jaundice  Peak bilirubin concentration at 3-5 days  Breast milk jaundice can persist for 1-3 months  Jaundice  Serum bilirubin concentrations of >4-5mg/dL in infants  >2-3mg/dL in older children  Scleral icterus  Elevated total bilirubin  Unconjugated hyperbilirubinemia  NOT conjugated/direct hyperbilirubinemia**  >2mg/dL direct or >20% total bilirubin level  This is PATHOLOGIC

Evaluation  After birth, infants should be assessed for jaundice every 8-12h (at least TcB)  Plot to determine what “risk-zone”  Obtain serum measurement if elevated  When to do more tests**  Jaundice in first 24 hours  Any infant receiving phototherapy  When TSB crosses percentiles on the nomogram  What tests  CBC with smear, reticulocyte count, Coombs test**  To detect hemolytic disease (ABO/Rh incomp, G6PD, etc.)**  Direct bilirubin concentration

Screening Prior to Discharge**

**Sepsis/UTI, metabolic disorders, and endocrine disorders can cause hyperbili…indirect or direct and should be screened for if clinically indicated!

Management  Helping mother’s breastfeed appropriately can decrease the likelihood of severe hyperbili**  Every 8-12 hours  Typically feed through breast milk/breastfeeding jaundice unless diagnosis in question or clinical reason not to!**  Lactation consultation when needed  Phototherapy**  Initiation based on TSB and age in hours**  Converts bilirubin into a water-soluble compound that can be excreted in urine or bile without conjugation  Blue lights in nm wavelength  More exposure = better  Expect a decrease of 0.5mg/dL/hr in first 4-8 hours  NOT for direct hyperbili (bronze infant syndrome)  Exchange transfusion

Phototherapy

Exchange Transfusion

Importance  Bilirubin crosses the BBB if unconjugated and unbound to albumin  Acute bilirubin encephalopathy**  Phase 1: first 1-2 days; poor suck, high-pitched cry, stupor, hypotonia, seizures  Phase 2: middle of 1st week; hypertonia of extensor mm, opisthotonus, retrocollis, fever  Phase 3: after 1 st week; hypertonia  Kernicterus**  1 st postnatal year: hypotonia, delayed motor skills  Later: choreoathetotic cerebral palsy, dental dysplasia, sensorineural hearing loss, cognitive impairment

Biliary atresia  Progressive and destructive inflammatory process that affects the extra and intra-hepatic biliary tree  Presentation**  Typically well-appearing  Jaundice 1-2 weeks after birth  Elevated direct bilirubin with mild elevation of total bilirubin (typically <12)  Increase AP or GGT  Imaging**  Abdominal US  Hepatobiliary Scintiscanning  Kasai Procedure*

FEVER WITHOUT A SOURCE, DR. HESCOCK Noon Conference