Lecture 5 - Pediatric GI Flashcards
Which type of bilirubin is toxic to the CNS? (Be specific!)
FREE (not bound to albumin); UNCONJUGATED bilirubin
The large amounts of unconjugated bilirubin in the blood of a new baby is due to what factors?
- Due to hemolysis
- Due to inadequate conjugation and clearance
What is the most common cause of unconjugated hyperbilirubinemia in an infant?
Hemolysis of RBC’s
Differentiate breast feeding jaundice vs. breast milk jaundice?
- Breast feeding jaundice is more of a function of dehydration and decreased excretion of bilirubin in the stool (related to the supply of the breast milk, which is sometimes low in first few days)
- Breast milk jaundice is due to presence of deconjugating enzymes in milk
Which type of hyperbilirubinemia is never non-pathologic?
Conjugated
What is Rh and why should Rh testing be done on all pregnant women?
- Rh is inherited and found on surface of RBC’s
- If mom is Rh (-) and baby is Rh (+) –> some of babies RBC’s get into Mom’s circulation during pregnancy and Mom develops Rh antibodies
- No big deal in 1st pregnancy, but w/ subsequent pregnancies the Rh antibodies can cross placenta and wreak havoc on Rh (+) baby (hydrops fetalis or erythroblastosis fetalis)
If Mom is blood type O or if Mom is Rh negative, the infant’s cord blood should be evaluated for what 3 things/how?
- A direct antibody (Coomb’s) test = DAT
- Blood type
- Rh determination
If a newborn has conjugated hyperbilirubinemia in the first few post-natal weeks what is the first thing you should think?
Biliary atresia/cholestasis
What is seen in Phase 1 (the first 1-2 days) of acute bilirubin toxicity in an infant (w/ high unconjugated bilirubinemia)?
- Poor suck
- High pitched cry
- Stupor
- Hypotonia
- Seizures
What is seen in Phase 2 (middle of first week of life) of acute bilirubin toxicity in an infant (w/ high unconjugated bilirubinemia)?
- Hypertonia of extensor ms.
- Opisthotonus
- Retrocollis
- Fever
What is seen in Phase 3 (after first week of life) of acute bilirubin toxicity in an infant (w/ high unconjugated bilirubinemia)?
Hypertonia
What is the chronic and permanent sequelae of Bilirubin Induced Neurologic Dysfunction (BIND)?
Kernicterus
What are some of the main signs of Kernicterus in a newborn?
- Choreoathetotic movements
- Ballismus
- Upward gaze
- Dental dysplasia
If there is jaundice in the first 24 hrs or the jaundice is excessive for infants age; which tests should be run?
When should Total Serum Bilirubin (TSB) be rechecked?
1) Bloody type and Direct Antibody (Coomb’s) test
2) CBC and peripheral blood smear
3) Conjugated bili level
4) Reticulocyte count
- Repeat TSB in 4 hours
If there is prolonged jaundice in an infant and you are approaching 2 months, what should you start thinking about?
Gilbert’s
What are 3 signs/symptoms of Biliary Atresia in an infant?
- Cholestatic jaundice (conjugated hyperbilirubinemia)
- Hepatomegaly
- Acholic stools
Extrahepatic biliary atresia occurs where in the hepatobilirary system?
Common bile duct
Crigler-Najjar Type 1 and Type 2 differ in their response to what drug?
- Type 1 has NO bilirubin decrease w/ phenobarbital
- Type 2 the bilirubin levels will decrease with phenobarbital
Which clinical test is the most definitive way of determining if someone is having clinically significant reflux?
- Esophageal pH monitoring w/ multichannel intraluminal impedance testing (MII)
- Measures frequency of GER and association of low esophageal pH w/ sx’s
Surgery as a treatment for GERD in a child should only be considered after what has been done first?
More commonly done in which children?
Usually only done if the GERD is severe enough to cause?
- pH /MII esophageal study has been completed
- More common in developmentally delayed childre
- Only if GERD is putting the child’s nutrition or respiratory status at risk
What is the most common location for intussusception in infancy?
- ileocolic
- Ileum invaginates into colon at the ileocecal valve/junction
What is the consistency of the stools associated with intussusception?
- Bright red blood and mucous
- “Currant jelly stools”
Which other symptom is often times seen intermittently in children with intussusception?
Striking lethargy is present intermittently
2 types of treatment for Intussuception?
- Hydrostatic reduction w/ a contrast enema (less common)
- Pneumatic reduction with an AIR enema
What are the 3 classic metabolic findings associated with Pyloric Stenosis?
- Hypochloremic
- Hypokalemic
- Metabolic alkalosis
*Due to all the vomiting!
What are 4 pathologic abnormalities resulting in increased production unconjugated bilirubin in a new born?
- Erythrocyte-enzyme deficiencies
- Blood group incompatibility (ABO)
- Structural defects in RBC’s
- G6PD deficiency (enzyme deficiency)