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Introduction
Yellowish discolouration of: Skin mucous membranes Sclera Due to deposition of excess plasma bilirubin Jaundice is quite common (5mg/dl).
Full term infants: at least 50% Preterm infants: over 80% Elevated blood bilirubin levels: 97%
Introduction continued
When? in the first week of life Where? skin , mucosa and white of eye How many? blood bilirubin concentrations is 80mol/L (5-7mg/dl)
Introduction continued
Excreting
Bilirubin Metabolism:
1. RBC: Heme bilirubin (UCB) 2. Blood: carried by bound to albumin 3. Liver: uptaken : Y protein, Z protein conjugated: UDPGT excreted to the biliary system 4. Intestine: stercobilins -glucuronidase enterohepatic circulation
8.8mg/Kg/d
3.8mg/Kg/d a. preterm infant; b. acidosis
in newborns
in adults
glucoronyl transferase)
c. Defective bilirubin excretion ability to bile system 4. Enterohepatic circulation
Bilirubin toxicity
1. Conjugated bilirubin
water-soluble 2. Unconjugated bilirubin lipid-soluble bilirubin-encephalopathy kernicterus
Clinical Manifestations
Jaundice appears
When: at any time during the neonatal period Where: from face chest feet abdomen
Manifestations continue
Evaluation of jaundice :
1. By eyes: face, 5mg/dl ( 85mol/L ); abdomen, 10-15mg/dl; feet, 15-20mg/dl ; 2. By transcutaneous measurement : used for screening 3. By serum levels : standard
Manifestations continue
Classification: Physiological Jaundice
Pathological Jaundice
Manifestations continue
Physiological jaundice :
1. General state is well 2. Appears 2-3days (>24h of age) peaks < 12.9mg/dl (full term infants) <15mg/dl (preterm infants) fades <2 week (term infants) <4 weeks (preterm infants) 3. Accumulates <5mg/dl/d 4. Direct bilirubin <2mg/dl
Manifestations continue
1. Appears earlier (first 24 hours of life) 2. Peaks >12.9mg/dl (full term infants) >15mg/dl (preterm infants) Fades >2 weeks (term infants) >4 weeks (preterm infants) 3. Accumulates >5mg/dl/d 4. Direct bilirubin >2mg/dl 5.Jaundice recurrent
Pathological Jaundice
ABO incompatibility
the mother: type O the infant: type A or B
Rh incompatibility
the mother Rh- the infant: Rh+D,E,C,d,e,c
Pathogenesis
Pathophysiology
Red blood cell breakdown
Hyperbilirubinemia Jaundice Anemia 1. Liver 2. Spleen 3. Heart, other organs Seizures etc. 4. Hydrops
Kernicterus
Clinical Manifestations:
ABO 1.Jaundice : 2.Anemia: 3.Hepatomild 1-2 day mild (3-6 weeks) rare Rh severe 24 h severe heart failure common
splenomegaly
Complication
Kernicterus:
Phase 1: decreased alertness
Hypotonia
Poor feeding Phase 2: Hypertonia, Retrocollis, opisthotonus Phase 3: Hypotonia
Laboratory tests:
1. Blood type incompatibility 2. Hyperbilirubinemia : Unconjugated bilirubin level 3. Hemolytic tests
2). Antibody release test (+) 3). Free antibody test (+)
Treatments
1). Phototherapy
2). Exchange transfusion 3). Internal Medicine
Treatments
Phototherapy Principle : photon of light Three photochemical reactions: 1). Structure isomer 2). Geometric isomer 3). Photo-oxidation Photoproducts excretion: w/o conjugation
Treatments continued
Indications of phototherapy :
Unconjugated bilirubinemia Bilirubin level >12mg/dl
Light source:
Spectral outputs 420 to 500nm
Treatments continued
Side effects of phototherapy :
a. diarrhea b. fever c. skin rash d. bronze baby syndrome (conjugated bilirubin>4mg/dl)
Treatments continued
2. Exchange Transfusions:
a. Severe hemolytic disease
b. Refractory to phototherapy
Treatments continued
Aims of transfusions:
a. Remove antibodies b. Remove bilirubin c. Correct anemia
Treatments continued
Indication of transfusions:
one of the follows
a. 20mg/dl (340 mol/L)
b. >4mg/dl,Hgb<120g/L, edema c. 0.7mg/dl/h d. Kernicterus
newborns
ABO
Rh
O cells
Treatments continued
3. Pharmacological agents:
a. Phenobarbital Effects: Uptake, Conjugation Excretion b. Albumin c. IVIG
Preventions
For ABO incompatibility: No
For Rh incompatibility 300 g of human anti-D globulin within 72 h of delivery.
1.Unconjugated bilirubinemia:
a. Hemolytic diseases: ABO, Rh incompatibility b. G-6-PD deficiency; c. Breast milk jaundice
1.Unconjugated bilirubinemia:
b. G-6-PD deficiency; male, jaundice, enzyme activity c. Breast milk jaundice causes: unclear, -glucuronidase follows physiologic jaundice: 4-7 d breast feeding persist for several weeks.
Conjugated bilirubinemia:
2.Conjugated bilirubinemia: a. neonatal hepatitis b. biliary obstruction (cholestatic jaundice)
biliary atresia,
common bile duct stenosis c. congenital metabolic diseases -1 antitrypsin deficiency
Case analysis :
24 old male infant, gravida1,para 1. Apgar scores: 8 at 1 min Mother: blood type O PE: icterus appeared on
Department of Pediatrics