hyperbilirbiunimia Flashcards
Jaundice and Hyperbilirubinemia in the Newborn in general
Hyperbilirubinemia is a common and in most cases benign problem in neonates. Nonetheless, untreated severe, indirect hyperbilirubinemia is potentially neurotoxic, and conjugated-direct hyperbilirubinemia often signifies a serious illness. Jaundice is observed during the 1st wk of life in approximately 60% of term infants and 80% of preterm infants
Production of bilirubin
Bilirubin is produced mainly from old RBCs
o Old RBCs give rise to globin and haem
o Globin enter the amino acid pool of the body o Haem spilt into iron and biliverdin which change into unconjugated bilirubin
o Unconjugated (indirect) bilirubin has 3 criteria:
- Fat solublep can cross Blood Brain Barrier (BBB)
- Water insoluble p can not be excreted in urine
- Detected by indirect Van Den Berg reaction
Conjugation of bilirubin
Conjugation of bilirubin stimulated by . glucoronyl transferase enzyme give rise to conjugated or cholebilirubin which is Cholebilirubin water soluble (excrectable in urine) and lipid insoluble (cannot cross BBB)
Defective conjugation: Glucoronyle transferase enzyme may be:
o Absent➡️ Criggler – Najjar syndrome type I
o Deficient➡️ Criggler – Najjar syndrome type II p Gilbert syndrome
o Immature➡️ Physiologic jaundice
o Under stimulated➡️ Hypothyroidism, hypoglycemia, hypoxia
o Inhibited➡️ Breast milk jaundice, Lucy- Driscoll syndrome
Non hemolytic causes (normal reticulocyte count.) of over production
😱 Extra vascular hemorrhage : Cephalhematoma & Internal hemorrhage
😱 Elevated RBCs load (Polycythemia) ➡️⬆️ RBCs turnover
😱 Enhanced enterohepatic circulation of bilirubin 2 ry to gastro intestinal stasis e.g. congenital pyloric stenosis and breast feeding jaundice
Clinical features of indirect hyperbilirubinemia
🍊 Skin and sclera: bright yellow / orange
🍊 Color of urine: usually normal.
🍊 Color of stool : may be dark
🍊 Possible Concurrent problems:(Absent in physiologic jaundice)
* Risk of kernicterus if indirect bilirubin exceeds the binding sites on
albumin or with leaky blood brain barrier
* Risk of anemia: if hemolysis exists
toxic effects⬆️ of indirect bilirubin
1- factors that reduce the retention of bilirubin in the circulation (hypoproteinemia, displacement of bilirubin from its binding sites on albumin by competitive binding of drugs such as sulfisoxazole and moxalactam, herbal tea, acidosis, increased free fatty acid concentration secondary to hypoglycemia, starvation, or hypothermia),
2 -factors that increase the
permeability of the blood-brain barrier or nerve cell membranes to bilirubin or the susceptibility of brain cells to its toxicity such as asphyxia, prematurity, hyperosmolality, and infection
Timing of Clinical jaundice indirect
- 🍊In the 1 stday of life➡️ Hemolytic disease of newborn (Rh or ABO incompatibility (until prove otherwise).
- 🍊In the 2 nd - 3 rd day of life
- Physiologic jaundice
- Criggler Najjar syndrome
- Hemolytic anemia
- 🍊By the 4 th –7 th days
- Physiologic jaundice in premature
- Hemolytic anemia
- 🍊After the 1 st week
- Breast milk jaundice
- Hemolytic anemia
- 🍊Persistent > 3 rd week
- Criggler-Najjar syndrome
- Physiologic jaundice in hypothyroid infant
Timing of direct hyperbilirubinemia
- 🍐In 1 st day of life
- TORCH infection
- 🍐In the rest of 1 st week of life
- Neonatal sepsis
- TORCH infection
- 🍐Persistent during 1 st month
- Neonatal hepatitis (metabolic or infections )
- Congenital biliary atresia.
- Inspissated bile syndrome
Physiologic Jaundice
Icterus Neonatorum
⬆️ indirect bilirubin production from breakdown of fetal RBC combined with transient limitationin conjugation of bilirubin by the immature liver. It is a diagnosis of exclusion.
🍊In Term infant, it usually started in the 2nd-3rd day of life, peaking in 2nd-4th day & gradually it resolve after 5th-7th day. It rarely reachs > 12 mg/dl.
🍊In Preterm infant, it started in the 3rd-4th day of life, peaking in 4th7th day & gradually it resolve after 7th-9th day. It rarely reachs > 15 mg/dl. Thus it tends to be slower & longer duration with higher level.
Factors that suggest non-physiologic jaundice include:-😱😱😱
- Jaundice appears in the 1st day of life.
- TSB > 12 mg/dl in term or > 15 mg/dl in preterm infant.
- TSB that rapidly increasing > 5 mg/dl/day.
- Direct bilirubin > 2 mg/dl at any time.
- Jaundice that persist > 10-14 days.
- Other factors include: family hx of hemolytic disease, pallor, hepatomegaly, splenomegaly, vomiting, lethargy, poor feeding, excessive weight loss, abnormal vital signs, light-colored stools, dark urine positive for bilirubin, failure of phototherapy to lower bilirubin, and signs of kernicterus.
7-• failure of phototherapy to lower bilirubin, vomiting, lethargy, poor feeding, excessive weight loss,
- apnea, bradycardia, abnormal vital signs including hypothermia,
- light-colored stools, dark urine positive for bilirubin, and signs of kernicterus
Physiologic Jaundice
Icterus Neonatorum
Physiologic jaundice is benign conditions that usually require no Rx except if other risk factors are presented e.g. prematurity; therefore it may need phototherapy.
Breast Milk Jaundice. Incidence
- Affects 2-4 % of adequately breast fed, healthy full term.
- Recurrence rate 70% in subsequent pregnancies
Breast Milk Jaundice clinical picture
-Instead of the usual fall of serum bilirubin by the end of first week it continues to rise
_ Peaking at 10-15 days of age With a maximal level of 10-30 mg/dl
_ Decline slowly over weeks
_ If breast feeding is interrupted for 24-48 hours, bilirubin level drops quickly
Breast milk jaundice etiology
Unknown ; Breast milk may contain:
- Pregnandiole Ļ inhibit glucoronyle transferase enzyme.
- F glucoronidase Ļenhance entero hepatic circulation of bilirubin
Breast Milk Jaundice dx
By Exclusion (Normal liver functions &CBC) + Therapeutic trial