Term
|
Definition
|
|
Term
aHemoglobin gets broken down into ___ and then into __ in RES. In circulation, bilirubin is ___ and bound to __. It is transferred to __ and __ by __ __. It is then excreted via the __ __ into the __ __. |
|
Definition
- biliverdin - bilirubin - unconjugated and bound to albumin - hepatocytes and conjugated by glucuronyl transferase - bile duct into the small intestine |
|
|
Term
Gut flora metabolize __ bilirubin into __ that are excreted in the stools. |
|
Definition
- conjugated bilirubin into stercobilins that are excreted in the stools |
|
|
Term
With limited gut flora and slow motility, bilirubin is converted back to the ___ form and reabsorbed. |
|
Definition
|
|
Term
Newborns have higher levels of bilirubin than adults b/c? |
|
Definition
ILIDS: - Immature liver (decreased glucuronyl transferase) - Large RBC load (so higher Hct 60-65) - Increased enterohepatic circulation - Degradation of extra hematopoietic tissue - Shortened RBC lifespan (90 instead of 120) |
|
|
Term
Normal total serum bilirubin: |
|
Definition
|
|
Term
Indirect hyperbilirubinemia: |
|
Definition
total bilirubin > 2.5 with direct < 15% |
|
|
Term
Direct hyperbilirubinemia: |
|
Definition
|
|
Term
__ hyperbilirubinema is lipid soluble and may enter brain. |
|
Definition
|
|
Term
___ hyperbilirubinemia is water soluble. Suspect this if there is bilirubin in the urine. |
|
Definition
|
|
Term
Bilirubin in the urine, suspect ___ hyperbilirubinemia. |
|
Definition
|
|
Term
2 types of indirect hyperbilirubinemia: |
|
Definition
1. Physiologic Jaundice (MC jaundice in newborns) 2. Pathologic Jaundice |
|
|
Term
Indirect Physiologic Jaundice: |
|
Definition
FORP - Full term infant < or equal to 15 - Occurs >24 hours after birth - Resolves by 1 week in full term infants or 2 weeks in preterm infants - Peaks at 3 -5 days |
|
|
Term
|
Definition
BMCVT - Bilirubin increase > 5 mg/dl/day or > 0.5 mg/dl/hr - May appear in first 24 hours - Conjuated bili > or equal to 2.0 - Visible jaundice on DOL 1 or after 2 weeks - Total bilirubin > 5 on DOL 1, or total bilirubin > 15 after DOL 1 |
|
|
Term
2 main causes of pathologic indirect hyperbili: |
|
Definition
- Increased production - Decreased clearance |
|
|
Term
causes of increased production leading to pathologic indirect hyperbilirubinemia: |
|
Definition
- Antibody mediated hemolysis (Coomb's test positive) - Non-antibody mediated hemolysis (Coombs test negative) - Extravascular hemolysis - Increased enterohepatic circulation |
|
|
Term
When is rhogam given to Rh- mom: |
|
Definition
- 28 weeks - delivery - any invasive procedure where blood transfer occurs |
|
|
Term
what is Rh isoimmunization: |
|
Definition
mom is RH-, baby RH+, so mom's blood forms antibodies to baby |
|
|
Term
complications of Rh isoimmunization: |
|
Definition
- hydrops in utero - severe anemia - hyperbilirubinemia |
|
|
Term
Examples of antibody mediated hemolysis (increased production) resulting in pathologic indirect hyperbilirubinemia: |
|
Definition
- ABO mismatch - Rh isoimmunization |
|
|
Term
Examples of non-antibody mediated hemolysis leading to pathologic indirect hyperbilirubinemia: |
|
Definition
- Structural (MC Spherocytosis) - Enzyme deficiencies (MC G6PD deficiency) |
|
|
Term
With spherocytosis RBCs collect in the __ and cause __. These pts tend to have a family history of __, __, and __. |
|
Definition
- spleen - splenomegaly - gallstones - anemia - jaundice |
|
|
Term
G6PD deficiency is __ linked and occurs in __ of __, __, or __ descent. |
|
Definition
- x-linked - males - African - Mediterrranean - Asian |
|
|
Term
Non-hemolytic causes of increased bilirubin production: |
|
Definition
- Hematoma or bruising (cephalohematoma) - Hemorrhage (IVH or abdominal) - Excess RBC production (polycythemia, RBC > 60) |
|
|
Term
Increased enterohepatic circulation can cause pathologic indirect hyperbilirubinemia, causes of this include: |
|
Definition
- decreased motility - decreased stooling (meconium plug, bowel obstruction) |
|
|
Term
Causes of decreased clearance leading to pathologic indirect hyperbilirubinemia: |
|
Definition
- Prematurity: decreased conjugation in liver - Glucuronyl transferase deficiency: Type 1 Criler Najaar Syndrome (recessive, poor prognosis) Type 2 dominant good prognosis - Gilbert's Syndrome- dominant, benign, get ill and become jaundiced in older age, scleral icterus in AA children, decreassed glucuronyl transferase |
|
|
Term
|
Definition
- Normal weight gain - Prolonged unconjugated hyperbilirubinemia, peaks at 10-15 days - Unknown factor in breast milk prevents conjugation of bilirubin - Increased enterohepatic circulation - Interrupt breast feeding for 24 hours, levels drop rapidly, resume breast feeding after 24-48 hours |
|
|
Term
Is breast milk jaundice common? |
|
Definition
|
|
Term
|
Definition
"lack of feeding" - Decreased intake <10min/feeding or less than 8 feedings/day - Decreased stooling < 4/day - Decreased UOP < 6/day - Weight loss > 8-10% - Increased enterohepatic circulation - Failure to establish milk supply - Supplement with formula and/or increase BF to every 2 hours |
|
|
Term
Important hx questions for jaundice newborn? |
|
Definition
BAMMCC - Birth trauma - Antibody titers - Maternal DM or infections - Maternal/infant blood type - Cord blood hemoglobin - Coombs test |
|
|
Term
|
Definition
- bruising/hematoma? - HSM (spleen palpable in 30% of nml newborns) - Plethora |
|
|
Term
Lab studies to order on jaundice newborn: |
|
Definition
- CBC - Peripheral smear (check for hemolysis) - Reticulocyte count (check for hemolysis) - IBT and Coombs test (even if mom is not O or Rh- b/c can find anti-Duffy or anti- Kell antibodies - Total bilirubin - Fractionated bilirubin |
|
|
Term
Kernicterus is staining of the __ __ leading to ___. List the acute sympotoms of this. |
|
Definition
- basal ganglia - encephalopathy Acute symptoms: - lethargy - decreased tone - high pitched cry - poor feeding - vomiting - hypertonic seizures - opisthotonus - scissoring of feet |
|
|
Term
Late sequellae of kernicterus: |
|
Definition
- neuro deficits - mental retardation - deafness - choreoatheltoid cerebral palsy |
|
|
Term
risk of kernicterus in term infants: |
|
Definition
- small if without hemolysis, even at bilirubin of 25 |
|
|
Term
3 main aspects of management of indirect hyperbilirubinemia: |
|
Definition
1. Hydration with supplmental feeding 2. Glycerin suppository to increase stooling 3. Phototherapy |
|
|
Term
when to start phototherapy: |
|
Definition
full term infant bili 15-18 |
|
|
Term
In a hemolytic full term infant, phototherapy should be used to keep bili less than __. |
|
Definition
|
|
Term
5 treatment steps for management of jaundice newborn: |
|
Definition
1. hydration 2. glycerin suppository to increase stooling 3. Phototherapy 4. IV albumin to increase binding 5. Exchange transfusion (only severe cases) |
|
|
Term
Risks with exchange transfusion: |
|
Definition
- Bleeding - Hypocalcemia - Infection - Death |
|
|
Term
Difficulty feeding Excess weight loss Jaundice at DOL 3-5 |
|
Definition
|
|
Term
elevated retic count indicates: |
|
Definition
|
|
Term
|
Definition
AMA - AA - Mediterranean - Asian |
|
|
Term
AA, Meditteranean, Asian, male, sibling affected jaundice; |
|
Definition
|
|
Term
spherocytosis is autosomal __. |
|
Definition
|
|
Term
FHx anemia and splenectomy: |
|
Definition
|
|