Module 8: PEDS: Jaundice, SIDS, Infections, CF,PKU, Gala, Dubin and Rotor Flashcards
What is the value for neonatal jaundice?
Greater than 5mg/dl bilirubin
What is physiological jaundice?
Increased unconjugated bilirubin during 1st week of life
What is phase I and phase II physiological jaundice?
Phase I: lasts 5 days in term infants and 7 days in preterm infants (Serum bilirubin = 12-15mg/dl)
Phase II: decreased serum bilirubin lasts for 2 weeks (normal adult values are reached)
What is pathological jaundice?
Clinical jaundice appearing in 1st 24 hours, total bilirubin greater than 15mg/dl and conjugated bilirubin greater than 2mg/dl
There are two types of pathological jaundice, Unconjugated hyperbilirubinemia and Conjugated hyperbilirubinemia. First we will discuss Unconjugated hyperbilirubinemia, now there are three different types. The first is fetomaternal blood group incompatibility, what are some features?
Also called hemolytic disease of the newborn
- –erythroblastosis fetalis, most common antigens: Rh and ABO blood antigens
- -Extent of hemolytic disease varies: death in utero, hydrops fetalis (hepatosplenomegaly and erythroid hyperplasia in the bone marrow) and Kernicterus (increased unconjugated bilirubin due to immature neurons of CNS)
How is the dx made and what is the treatment and prevention of fetomaternal blood group incompatibility?
Dx: high level of bilirubin on aminocentesis and Coombs test positive on fetal cord blood
Tx: exchange transfusion and phototherapy
Prevention: human anti D globulin within 72 hours of delivery
The second type of unconjugated hyperbilirubinemia is crigler-najjar syndrome type I, what are the features?
Complete absence of UDP-glucuronyltransferase activity — unremitting unconjugated bilirubinemia — bilirubin encephalopathy
The third type of unconjugated hyperbilirubinemia is crigler-najjar syndrome type II, what are some features?
Partial decreased in UDP-glucuronyltransferase activity so treat with phenobarbital
(enzyme that converts unconjuated to conjugated bilirubin)
Now the next type of pathological jaundice is conjugated hyperbilirubinemia, caused by two different pathologies. each card will go through one. The first is biliary atresia, what are some features?
Complete obstruction of the lumen of the extrahepatic biliary tree within first 3 months of life — chronic cholestasis, marked bile ductular proliferation and portal tract edema and fibrosis
- -most common indication of liver transplant in this age group
- -embryonic/fetal type: due to aberrant intrauterine development of extrahepatic biliary tree
- -perinatal type: normally developed biliary tree is destroyed by virus after birth (Viral hepatitis)
The second cause of conjugated hyperbilirubinemia in kids is idiopathic neonatal hepatitis, what are some features?
Excluded known associated factors (alpha 1 antitrypsin, extrahepatic biliary atresia and infectious agents)
Next topic is SIDS, sudden infant death syndrome. What is it?
Sudden death of an infant under 1 year which is unexplained after a thorough case investigation which includes a complete autopsy, examination of death scene and thorough review of clinical history
What are the associated risk factors of SIDS?
In 75% of SIDS cases there is NO associated risk factors
What is the pathogenesis for SIDS?
Prolonged spell of apnea, followed by cardiac arrhythmia, in susceptible infant
- multiple petechiae in thymus, pleura and epicardium
- -congestion of lungs
- -hypoplasia of arcuate nucleus in the brainstem and decrease in brainstem neurons (gliosis of the brainstem)
What are differential diagnosis for SIDS?
Infection: viral myocarditis
Unsuspected congenital anomaly: anomalous origin of coronary artery from pulmonary artery
Child Abuse
Genetic/Metabolic defects: long QT syndrome, fatty acid oxidation disorders, histiocytoid cardiomyopathy
What are parental risk factors for SIDS??
Young maternal age Smoking Drugs Late or no prenatal care Low income Black or American Indian