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Hyperbilirubinemia- Its not easy being yellow
 

Hyperbilirubinemia- Its not easy being yellow

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    Hyperbilirubinemia- Its not easy being yellow Hyperbilirubinemia- Its not easy being yellow Presentation Transcript

    • Hyperbilirubinemia: Update in Newborn Care David Mendez, M.D. Kidz Medical Services Miami Childrens Hospital
    • Hyperbilirubinemia: Update in Newborn Care  All Yellow is Bad  Prevent Yellow at all costs  Watch out for 20  Major inroad in Neonatal Care
    • Hyperbilirubinemia: Update in Newborn Care        Bilirubin Physiology Bilirubin Toxicity Differential Diagnosis Vigintiphobia Work Up Treatment Breast Milk
    • Hyperbilirubinemia: Update in Newborn Care GENERAL BILIRUBIN PHYSIOLOGY     HEME CATABOLISM BILIRUBIN TRANSPORT HEPATIC UPTAKE BILIRUBIN CONJUGATION
    • Hyperbilirubinemia: Update in Newborn Care GENERAL BILIRUBIN PHYSIOLOGY BILIRUBIN IS THE END PRODUCT OF HEME DEGREDATION MAJORITY DERIVED FROM ERYTHROCYTES REMOVED AND DESTROYED BY RES
    • Hyperbilirubinemia: Update in Newborn Care HEME CATABOLISM HEME OXIDASE HEME BILIVERDIN CO
    • Hyperbilirubinemia: Update in Newborn Care NADPH DEPENDENT BILIVERDIN BILIRUBIN BILIRUBIN REDUCTASE
    • Hyperbilirubinemia: Update in Newborn Care HEME CATABOLISM 1 MOLE OF HEME = 1 MOLE OF CO METALLOPORPHRYNS ACT AS A COMPETITIVE INHIBITOR OF HEME OXIDASE
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN TRANSPORT  Bilirubin formed in the RES or hepatic parenchymal cells and is released into the circulation  Bilirubin binds tightly, reversibly to albumin  The free component of bilirubin is toxic
    • Hyperbilirubinemia: Update in Newborn Care COMPOUNDS THAT BIND TO ALBUMIN       SULFA MEDICATIONS RADIOGRAPHIC CONTRAST MEDIA ASPIRIN BENZODIAZOPENES DIURETICS FUSIDIC ACID
    • Hyperbilirubinemia: Update in Newborn Care HEPATIC UPTAKE Uptake is rapid, transport carrier mediated Cytosolic proteins Ligandin (Y) Fatty acid binding protein(Z) * Not the area where bilirubin conjugation is delayed
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN CONJUGATION UDP-GLUCOROSYL TRANSFERASE BILIRUBIN BILIRUBIN MONOGLUCORONIDE -THIS IS THE ENZYME THAT IS RATE LIMITING
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN CONJUGATION GIRLS HAVE LOWER SERUM BILIRUBIN LEVELS THAN BOYS ADULT BILIRUBIN IS IN THE DIGLUCOURONIDE FORM
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN TOXICITY RATE OF PRODUCTION RATE OF ELIMINATION UNCONJUGATED,UNBOUND
    • Hyperbilirubinemia: Update in Newborn Care RATE OF PRODUCTION DESTRUCTION OF FETAL HEMOGLOBIN LIFE SPAN OF HgF ~ 90 DAYS vs HgA ~ 110 DAYS DESTRUCTION BEGINS IN UTERO
    • Hyperbilirubinemia: Update in Newborn Care RATE OF ELIMINATION UPTAKE INTRACELLULAR BINDING/STORAGE CONJUGATION EXCRETION PLACENTA SERVES AS REMOVER OF UNCONJUGATED BILI
    • DISORDERS OF BILIRUBIN METABOLISM UPTAKE DISORDERS A FAMILY OF ORGANIC ANION TRANSPORT PROTIENS (OATP) HAS BEEN IDENTIFIED. THERE ROLE HAS NOT BEEN DIRECTLY ESTABLISHED AND NO DISORDER HAS BEEN ATTRIBUTED TO THIS PROCESS
    • DISORDERS OF BILIRUBIN METABOLISM BINDING AND STORAGE DISORDERS WITHIN THE HEPATOCYTE, PROTEINS DESIGNATED Y AND Z. Y PROTEIN CALLED LIGANDIN , SMALL % OF HEPACYTE COMPONENT NO KNOWN DISORDER AS A RESULT OF ITS ABSENCE
    • DISORDERS OF BILIRUBIN METABOLISM CONJUGATION DISORDERS CRIGLER –NAJJAR SYNDROME TYPE I ABSENT UDP- GLUCOSYL TRANSFERASE ACTIVITY
    • DISORDERS OF BILIRUBIN METABOLISM CONJUGATION DISORDERS CRIGLER –NAJJAR SYNDROME TYPE II ( aka Arias Syndrome) REDUCED ACTIVITY UDP- GLUCOSYL TRANSFERASE ACTIVITY (makes mostly monglucoronide)
    • DISORDERS OF BILIRUBIN METABOLISM CONJUGATION DISORDERS GILBERTS SYNDROME (aka familial nonhemolytic jaundice) Hepatic UDP transferase acitivty approx. 30%
    • DISORDERS OF BILIRUBIN METABOLISM EXCRETION DISORDERS DUBIN-JOHNSON SYNDROME “black liver disease” Canalicular excretion is defective, affects organic acid secretion from hepatocyte
    • DISORDERS OF BILIRUBIN METABOLISM HEPATIC STORAGE DISORDERS ROTOR SYNDROME Accumulation of Conj. Bili in plasma Liver not pigmented
    • Hyperbilirubinemia: Update in Newborn Care UNCONJUGATED,UNBOUND BILIRUBIN THAT BILIRUBIN THAT IS NOT BOUND TO ALBUMIN AND HAD NOT BEEN CONJUGATED BY THE LIVER IS FREE TO ENTER THE TISSUE
    • Hyperbilirubinemia: Update in Newborn Care KERNICTERUS DEF: BILIRUBIN STAINING OF THE BASAL GANGLIA AND CRANIAL NERVE NUCLEI FOUND AT AUTOPSY
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN ENCEPHALOPATHY EARLY SYMPTOMS (NEONATAL) LETHARGY POOR FEEDING HIGH PITCHED CRY VOMITING HYPOTONIA
    • Hyperbilirubinemia: Update in Newborn Care BILIRUBIN ENCEPHALOPATHY LATE SYMPTOMS IRRITABILITY HYPERTONIA OPISTHOTONOS SEIZURES CEREBRAL PALSY-ATHETOID, HEARING LOSS
    • Hyperbilirubinemia: Update in Newborn Care BLOOD-BRAIN BARRIER DEF: A TIGHT-JUNCTIONED, DENSE PERICAPILLARY SHEATH, COMPOSED OF GLIAL FOOT PROCESSES AND A SERIES OF TRANSPORT SYSTEMS * NORMALLY IMPERMEABLE TO ALBUMIN AND POLAR WATER SOLUBLE BILIRUBIN COMPOUNDS
    • Hyperbilirubinemia: Update in Newborn Care BLOOD-BRAIN BARRIER WAYS TO PENETRATE 1. INCREASE THE VOLUME OF UNCONJUGATED BILIRUBIN 2. INJURE THE BBB 3. DISPLACE BILIRUBIN FROM ALBUMIN
    • Hyperbilirubinemia: Update in Newborn Care BLOOD-BRAIN BARRIER 1. INCREASE THE VOLUME OF UNCONJUGATED BILIRUBIN     HEMOLYSIS (ABO, RH BRUSING) DECREASED ENZYME ACT. (GILBERT SYN) ABSENT ENZYME ACT. (CRIGLER-NAJJAR) LIVER DAMAGE (GALACTOSEMIA)
    • Hyperbilirubinemia: Update in Newborn Care BLOOD-BRAIN BARRIER 2. INJURE THE BBB MORE LIKELY IN: TERM vs PRETERM SICK NEONATES vs ASYMPTOMATIC CONDITIONS: SEIZURES, SEPSIS, MENNIGITIS,ACIDOSIS, HYPOTENSION, DEHYDRATION, BRAIN BLEEDS
    • Hyperbilirubinemia: Update in Newborn Care BLOOD-BRAIN BARRIER 3. DISPLACE BILIRUBIN FROM ALBUMIN “LOW BILI KERNICTERUS IN 1960”S” “SEDATION KERNICTERUS IN 1970’S”
    • Hyperbilirubinemia: Update in Newborn Care DIFFERENTIAL DIAGNOSIS INCREASED PRODUCTION      BLOOD GROUP INCOMPATIBILITY RED CELL MORPHOLOGY HEMORRHAGE POLYCYTHEMIA INCREASED ENTEROHEPATIC CIRCULATION
    • Hyperbilirubinemia: Update in Newborn Care DIFFERENTIAL DIAGNOSIS DECREASED CLEARANCE     INBORN ERRORS OF METABOLISM’ HYPOTHYROIDISM BREAST MILK JAUNDICE PREMATURITY
    • Hyperbilirubinemia: Update in Newborn Care VIGINITIPHOBIA “FEAR OF 20”
    • Hyperbilirubinemia: Update in Newborn Care VIGINITIPHOBIA 1950’S STUDY FROM BOSTON AND LONDON BABY’S WITH ERYTHROBLASTOSIS FETALIS REPEATED EXCHANGE TRANSFUSIONS; KEEP BILI<20 LESS INCIDENCE OF KERNICTERUS THEREFORE ANY BABY WITH BILI RISING TO 20…… EXCHANGE !!
    • Hyperbilirubinemia: Update in Newborn Care RISK FACTORS      JAUNDICE IN THE 1ST 24HRS PREVIOUS SIBLING WITH JAUNDICE/PHOTORX CEPHALOHEMATOMA OR BRUISING AT BIRTH ABO INCOMPATIBILITY PREDISCHARGE BILIRUBIN . 95TH % TILE
    • Hyperbilirubinemia: Update in Newborn Care WORK UP 1. HISTORY - UNDERLYING SIGNS OF ILLNESS (LETHARGY, APNEA ,TACHYPNEA, TEMP. INSTABILITY, BEHAVIOR CHANGES, VOMITING) - 37 OR LESS WEEKS GESTATION
    • Hyperbilirubinemia: Update in Newborn Care WORK UP 1. HISTORY -MOTHER AND INFNAT ABO AND RH STATUS -FAMILY HISTORY OF HEMOLYTIC DISEASE -WHEN DID JAUNDICE PRESENT AND HOW LONG
    • Hyperbilirubinemia: Update in Newborn Care WORK UP -FEEDING ISSUES -STOOL COLOR AND VOLUME, URINE VOLUME
    • Hyperbilirubinemia: Update in Newborn Care WORK UP 2. LABS - BILIRUBIN UC/C - COOMBS (UNLESS BLOOD TYPE KNOWN) - CBC -RETIC -SEPSIS SCREEN(BLOOD C/S, URINE,STOOL, CSF)
    • Hyperbilirubinemia: Update in Newborn Care WORK UP 2. LABS IF EVIDENCE OF HEMOLYSIS G6PD SCREEN SMEAR HGB ELCTROPHORESIS
    • Hyperbilirubinemia: Update in Newborn Care WORK UP 2. LABS IF BABY IS SEVERELY JAUNDICED, EARLY-ONSET, NON-HEMOLYTIC….. THINK METABOLIC
    • Hyperbilirubinemia: Update in Newborn Care DIAGNOSIS  IT IS IMPORTANT TO INTERPRET BILIRUBIN LEVELS IN TERMS OF THE BABY’S AGE IN HOURS- NOT DAYS  THAT BABY’S ARE ALLOWED TO GO HOME AS SOON AS 36-72 HOURS AFTER BIRTH COMPROMISES THAT INTERPRETATION
    • Hyperbilirubinemia: Update in Newborn Care MAJOR RISK FACTORS        PREDISCHARGE BILI IN HIGH-RISK ZONE(95%) JAUNDICE 1ST 24 HRS KNOWN BLOOD GROUP INCOMPATIBILITY GESTATIONAL AGE < 36 WKS CEPHALOHEMATOMA OR BRUISING EXCLUSIVELY BREASTFEEDING EAST ASIAN RACE
    • Hyperbilirubinemia: Update in Newborn Care MINOR RISK FACTORS        PREDISCHARGE BILI IN INTERMIEDIATE ZONE GESTATIONAL AGE 37-38 WEEKS JAUNDICED OBSERVED BEFORE DISCHARGE PREVIOUS SIBLING WITH JAUNDICED MACROSOMIC INFANT OF DIABETIC MOTHER MATERNAL AGE > 25 YRS MALE
    • Hyperbilirubinemia: Update in Newborn Care DIAGNOSIS     JAUNDICED IN 1ST 24 HRS JAUNDICE APPEARS EXCESSIVE FOR AGE LESS THAN 38 WEEKS EXCLUSIVELY BREAST FED “DON’T JUST LOOK….TEST”
    • Algorithm for the management of jaundice in the newborn nursery Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316 Copyright ©2004 American Academy of Pediatrics
    • Hyperbilirubinemia: Update in Newborn Care
    • Hyperbilirubinemia: Update in Newborn Care PHOTOTHERAPY  RESULTS IN A PHOTOISOMER OF BILIRUBIN WITH POLAR PROPERTIES THAT ALLOWS FOR BILE EXCRETION  DESCRIBED BY RJ CREMER IN ENGLAND(1958) WITH 1ST PHOTOTHERAPY PAPER IN LANCET BLUE LIGHT (450NM) 1ST U.S. PAPER BY LUCEY IN 1968.  
    • Hyperbilirubinemia: Update in Newborn Care      PHOTOTHERAPY Can’t overdose on Phototherapy Halogen lights effective but “hot” Uncover the baby, “bathe in light” Special Blue light (F20T12/BB) (TL 52/20W phillips) Irradiance level- 40-45
    • Hyperbilirubinemia: Update in Newborn Care PHOTOTHERAPY  INDICATIONS FOR BABIES > 35 WEEKS - SICK OR HEALTHY - HEMOLYTIC OR NOT - MAJOR RISK FACTORS - MINOR RISK FACTORS
    • Nomogram for designation of risk in 2840 well newborns at 36 or more weeks' gestational age with birth weight of 2000 g or more or 35 or more weeks' gestational age and birth weight of 2500 g or more based on the hour-specific serum bilirubin values Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316 Copyright ©2004 American Academy of Pediatrics
    • Guidelines for phototherapy in hospitalized infants of 35 or more weeks' gestation Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316 Copyright ©2004 American Academy of Pediatrics
    • Hyperbilirubinemia: Update in Newborn Care EXCHANGE TRANSFUSION A PROCEDURE WHERE THE TOTAL BLOOD VOLUME IS ESTIMATED BASED ON NEONTAL WEIGHT AND TRANSFUSED INTO THE INFANT WHILE DRAWING OUT AN EQUAL AMOUNT OF BLOOD
    • Hyperbilirubinemia: Update in Newborn Care EXCHANGE TRANSFUSION  INDICATIONS FOR BABIES > 35 WEEKS -IMMEDIATELY IF S/SX OF ENCEPHALOPATHY (HYPERTONIA, ARCHING, RETROCOLIS OPISTHOTONOS, FEVER, HIGH PITCHED CRY) OR IF TSB IS 5 MG/DL OVER LINE  PRESENCE OF MAJOR RISK FACTORS+TSB
    • Guidelines for exchange transfusion in infants 35 or more weeks' gestation Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316 Copyright ©2004 American Academy of Pediatrics
    • Hyperbilirubinemia: Update in Newborn Care
    • Hyperbilirubinemia: Update in Newborn Care BREAST MILK JAUNDICE COMPOUND IN BREAST MILK EITHER INTERFERES WITH CONJUGATION OR PROMOTES ENTEROHEPATIC CIRCULATION
    • Hyperbilirubinemia: Update in Newborn Care BREAST MILK JAUNDICE IN A PROPERLY BREAST-FED, HEALTHY WELL-HYRDATED NEWBORN, BILI LEVELS NOTE A PHYSIOLOGIC DISTRIBUTION AMONG A STUDY OF BABY’S WITH BMJ* *ALONSO, GARTNER ET.AL
    • Hyperbilirubinemia: Update in Newborn Care BREAST MILK JAUNDICE MOTHERS SHOULD NURSE THEIR INFANTS 8-12 TIMES/DAY DO NOT SUPPLEMENT NON-DEHYDRATED BREAST FED INFANTS WITH WATER OR DETROSE WATER
    • Hyperbilirubinemia: Update in Newborn Care BREAST MILK JAUNDICE ADEQUACY OF FEEDS - BABY’S LOSE MAXIMUM WEIGHT LOSS DAY 3 - % LOSS ON AVERAGE 6.1% + 2.5% (SD) - 4 TO 6 WET DIAPERS EVERY 24 HRS - 3 TO 4 STOOLS PER DAY BY DAY 4 - MUSTARD YELLOW STOOLS BY DAY 3-4 IF WEIGHT LOSS >10%, EVALUATE INTAKE
    • Hyperbilirubinemia: Update in Newborn Care FOLLOWING BILIS FOR INFANTS ON INTENSIVE PHOTORX -IF TSB > 25, REPEAT EVERY 2-3 HRS -IF TSB 20-25 REPEAT EVERY 3-4 HRS -IF TSB < 20 REPEAT EVERY 4-6 HRS - IF TSB < 13-14 MAY DISCONTINUE PHOTO MAY CHECK FOR REBOUND 24 HRS D/C PHOTO WHENEVER POSSIBLE CONTINUE TO BREAST FEED
    • Relationship between average spectral irradiance and decrease in serum bilirubin concentration Subcommittee on Hyperbilirubinemia, Pediatrics 2004;114:297-316 Copyright ©2004 American Academy of Pediatrics
    • Hyperbilirubinemia: Update in Newborn Care FOLLOW UP    BABY D/C’D BEFORE 24 HRS BABY D/C’D 24 TO 47.9 HRS BABY D/C’D 48 TO 72 HRS 72 HR F/U 96 HR F/U 120 HR F/U
    • Hyperbilirubinemia: Update in Newborn Care AAP JAUNDICE GUIDELINES 1. 2. 3. 4. 5. PROMOTE AND SUPPORT SUCCESSFUL BREAST FEEDING ESTABLISH NURSERY PROTOCOLS GET TSB IF JAUNDICED IN 1ST 24 HOURS DON’T RELY ON VISUAL ASSESSMENT INTERPRET BILI LEVELS BASED ON INFANT AGE IN HOURS
    • Hyperbilirubinemia: Update in Newborn Care AAP JAUNDICE GUIDELINES 6. 7. 8. 9. 10. INFANTS LESS THAN 38 WEEKS, PARTICLUARLY IF BREAST FED ARE AT HIGHER RISK PERFORM RISK ASSESSMENT PRIOR TO D/C GIVE PARENTS WRITTEN AND ORAL INFORMATION PROVIDE TIME-APPROPRIATE FOLLOW UP TREAT NEWBORNS WHEN INDICATED WITH PHOTOTHERAPY OR EXCHANGE TRANSFUSION