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Neonatal Hemolytic Jaundice
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Hyperbilirubinemia ( severe jaundice ) is the most common condition requiring re-hospitalization during the first week of life. Total Serum Bil (TSB) > 17 mg/dl ≈ 10% Total Serum Bil (TSB) > 20 mg/dl ≈ 1.5%
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Most neonates with extreme hyperbilirubinemia have no underlying etiology.
55% to 66% of neonates with a TSB >30 mg/dl have no etiology (idiopathic jaundice), according to 2 studies.
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Hyperbilirubinemia = bilirubin production - bilirubin elimination
Low activity of UGT1A1 ≈ 1% in newborns (diminished conjugation in all newborns) Prodominant factor in severe jaundice is increased bilirubin production (hemolysis)
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Increased risk of neurotoxicity
Why hemolytic jaundice is important? Increased risk of neurotoxicity
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Hemolytic jaundice is likely when:
jaundice observed in first 24 hours pre-discharge Bil in high risk zone rising Bil more than 0.5 mg/dl/h cord Bil more than 4 mg/dl prolonged hyperbilirubinemia low hemoglobin ( < 13 g/dl) positive direct anti-globulin test (DAT) Elevated reticulocyte count
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Hemolytic jaundice is likely when:
hepatosplenomegaly finding hemoglobin without erythrocytes in urine pink serum on a visual examination of a blood sample absence of haptoglobin in the serum
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ABO hemolytic disease Indirect hyperbilirubinemia during the first 24h of life Mother with blood group O, infant with blood group A or B Spherocytosis on blood smear Increased reticulocyte count Evidence of hemolysis based on increased endogenous production of CO Direct or indirect coombs positive
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Incompatibility to other RBC antigens
Anti -c Anti -E Anti-Kell
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Five diagnostic tests or approaches useful in hemolytic jaundice
Erythrocyte morphology End-tidal carbon monoxide (ETCO) measurement EMA flow cytometry for HS and other membrane defects NGS (Next Generation Sequencing) of relevant genes An algorythm in judicious use of standard tests
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Prediction of Hemolytic Jaundice
Cord bilirubin Clincal risk factors Hour-specific bilirubin nomogram
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Hour-specific Bilirubin Nomogram
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Treatment Phototherapy Exchange transfusion IVIG
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Guidelines for Exchange Transfusion
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