1. Heme

2. Structure

8. کم خونی سیدروبلاستی
پورفیریای حاد متناوب
پورفیریای اریتروپوئیتیک مادرزادی
پورفیری تاخیری پوستی
کوپروپورفیری ارثی
پورفیری دگرگون
پروتوپورفیریای اریتروپوئیتیک

9. Porphyrias

10. hemoglobin
heme
globin
Fe (reutilized)
degraded (bilirubin)
free amino acids

13. Unconjugated bilirubin Reticuloendothelial system
Heme
Biliverdin
Unconjugated bilirubin
Reticuloendothelial system
Unconj.bilirubin/albumin complex
Systemic circulation
Hepatocytes
Unconj. bilirubin
Bilirubin diglucuronide
Small intestine
Large intestine
Bilirubin
Urobilinogen
Stercobilins
Kidney
urine

14. HYPERBILIRUBINEMIA
-- elevated bilirubin in serum (above 1 mg/dL)
-- can be conjugated or unconjugated or both depending on the situation
-- elevated bilirubin can diffuse into tissues, making them appear yellow (jaundice)

15. Clinical Consequences:
HYPERBILIRUBINEMIA
Clinical Consequences:
-- Conjugated hyperbilirubinemia: benign
-- Unconjugated hyperbilirubinemia: benign at concentrations < 25 mg/dL (albumin capacity)
-- At concentrations >25 mg/dL, unconjugated bilirubin is free (uncomplexed) and can enter the brain.
bilirubin encephalopathy (kernicterus)

16. Causes of JAUNDICE
Hemolytic anemia
--  destruction of erythrocytes
Hepatitis or cirrhosis
--  conjugation and excretion of bilirubin
Bile duct obstruction
-- conjugated bilirubin not delivered to intestine;
it backs up, spills over into the blood
Neonatal “physiological jaundice”
-- immature hepatic system of the newborn:
 uptake, conjugation, excretion of bilirubin