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HYPERBILIRUBINEMIA

HYPERBILIRUBINEMIA. Fatima C. Dela Cruz. Jaundice. Yellowish discoloration of the skin, sclera and other mucous membranes of the body. Jaundice.

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HYPERBILIRUBINEMIA

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  1. HYPERBILIRUBINEMIA Fatima C. Dela Cruz

  2. Jaundice • Yellowish discoloration of the skin, sclera and other mucous membranes of the body

  3. Jaundice • Accumulation in skin of unconjugated, non-polar, lipid-soluble bilirubin pigment formed from Hgb by the action of hemeoxygenase, biliverdinreductase, & non-enzymatic reducing agents in the reticuloendothelial cells • Deposition of the pigment after it has been converted in the liver cell microsome by the enzyme uridinediphosphoglucuronic acid (UDP)-glucuronyltransferase to the polar, water-soluble ester glucuronide of bilirubin (direct reacting)

  4. Jaundice • Face (total serum bilirubin ~ 5mg/dl) • Midabdomen (TSB ~15 mg/dl) • Feet (TSB ~20mg/dl)

  5. Jaundice Dermal Zones of Jaundice (Kramer, 1969) ZoneRange of TSB (mg/dL) 1 4.3 – 7.9 2 5.4 – 12.2 3 8.1 – 16.5 4 11.1 – 18.3 5 >15

  6. Bilirubin Macrophages Bloodstream Liver Gut RBCs  Heme  Unconjugated  Unconjugatedbilirubin-  Conjugated  Urobilinogen bilirubin albumin complex bilirubin ----------------------- ----------------------- ----------------------- Albumin Uridine Glucuronyl transferase 80% -feces 20% 10%-urine 90%-liver Indirect Bilirubin Direct bilirubin

  7. Jaundice • Unconjugatedhyperbilirubinemia is bright yellow or orange • Direct hyperbilirunemia is greenish or muddy yellow

  8. Differential Diagnoses Jaundice appearing at birth <24 hours • Sepsis • Erythroblastosisfetalis • Concealed hemorrhage • Cytomegalic inclusion disease • Rubella • Congenital toxoplasmosis

  9. Differential Diagnoses Jaundice appearing on the 2nd or 3rd day • Physiologic hyperbilirubinemia of the newborn • Criggler-Najjar syndrome (familial nonhemolyticicterus)

  10. Differential Diagnoses Jaundice appearing after the 3rd day, within the 1st week • Septicemia • Syphilis • Toxoplasmosis • Cytomegalic inclusion disease • Other causes of early jaundice (Intrauterine transfusions, Extensive ecchymosis or hematomas, Polycythemia)

  11. Differential Diagnoses Jaundice appearing after the 1st week • Breast milk jaundice • Septicemia • Congenital atresia of the bile ducts • Hepatitis • Rubella • Galactosemia, hypothyroidism • Spherocytosis (congenital hemolytic anemia) • Other hemolytic anemias(G6PD deficiency, Glutathione synthetase deficiency, Peroxidase deficiency, Pyruvatekinase deficiency)

  12. Physiologic Jaundice • Result of increased bilirubin production following breakdown of fetal red blood cells and limitation of liver bilirubin conjugation • Indirect bilirubin: 1-3 mg/dL; rises at a rate <5mg/dL/24h • Usually visible by the 2nd-3rd day and disappears by the 5th-7th day

  13. Pathologic Jaundice • Jaundice appears in the first 24-36 h of life • Total serum bilirubin (TSB) rises by > 5 mg/dL/day • Serum bilirubin >12 mg/dL term and 10-14 mg/dL in preterm infants • Jaundice persists after 10-14 days of life • Direct-reacting bilirubin >2 mg/dL at any time

  14. Pathologic Jaundice 1. Hemolytic disease • Rh incompatibility • ABO incompatibility • Drugs (vitamin K) • Congenital hypothyroidism • Increased hemolysis • Cephal hematoma 2. Hepatocellular injury • Biliaryatresia • Cholestasis • Hepatitis • Infection 3. Mixed hemolytic and hepatotoxic factors • Infection (bacterial and viral) 4. Hyperbilirubinemia secondary to metabolic factors • Hypoxia • Respiratory distress • Hypoglycemia • Hypothyroidism

  15. Breastfeeding Jaundice • bilirubin during the first week of life in breastfed infants due to both caloric and fluid deprivation • Resolves with increased breast feeding frequency and amount of milk intake

  16. Breastmilk Jaundice • Jaundice among breastfed infants probably secondary to hormones (pregnanediol) in milk acting on infant’s hepatic metabolism and an enzyme (glucuronidase) facilitating intestinal reabsorption of bilirubin

  17. Treatment • Phototherapy • Exchange transfusion • Intravenous immunoglobulin • Metalloporphyrins

  18. Treatment Phototherapy • Exposure to a high intensity of light in the visible spectrum • Bilirubin absorbs light maximally in the blue range • Photoisomerization • converts unconjugatedbilirubin (4Z, 15Z) into unconjugated isomer (4Z, 15E) which is excreted in the bile • Structural change • converts unconjugatedbilirubin to lumirubin, which is excreted in the urine in an unconjugated state

  19. Treatment Exchange transfusion • Partial removal of the infant’s circulating antibody coated RBCs as well as unattached antibodies and replace

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