Clinical Aspect of Hyperbilirubinemia on LBW Infant

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    Clinical Aspect of Hyperbilirubinemia

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    CLINICAL JAUNDICE

    80% of premature baby

    Visible jaundice: serum bilirubin > 5 mg/dL

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    Neonatal Jaundice:

    WHY WE WORRY ?

    Acute Bilirubin EncephalopathyEarly phase

    lethargic, hypotonia, suck poorlyIntermediate phase

    stupor, irritability, hypertonia(retrocollis and opistotonus)

    Fever, high-pitched cry

    Kernicterus

    Chronic form of bilirubin encephalopathyAthetoid CP, auditory dysfunction, paralysis upward gaze

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    Kernicterus - Neuropathology

    Yellow staining and neuronal necrosis Basal ganglia:

    globus pallidus

    subthalamic nucleus

    Cranial nerve nuclei:

    vestibulocochlear

    oculomotor

    facial

    Cerebellar nuclei

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    1990 -..125 CASES OF KERNICTERUSin the United States

    Cases of Kernicterusin Indonesia ?

    A preventable tragedy

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    BILIRUBIN SYNTHESIS, TRANSPORT, AND

    METABOLISM

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    BASIS FOR INCREASED BILIRUBIN LEVELS

    IN THE NEWBORN

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    Serum Bilirubin levels

    in term and preterm infants

    0

    2

    4

    6

    8

    1012

    14

    16

    day 1 day 2 day 3 day 4 day 5 day 6 day 7

    Normal term

    Preterm

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    Jaundice in preterm neonates

    Onset earlier

    Peaks later

    Higher peak

    Takes longer to resolve up to 3 weeks

    What level is physiologic?

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    Physiologic vs Non-physiologic

    hyperbilirubinemia

    0

    24

    6

    8

    10

    12

    1416

    18

    20

    day 1 day 2 day 3 day 4 day 5 day 6 day 7

    physiologic

    non- physiologic

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    Criteria that Rule Out the Diagnosis

    of Physiologic Jaundice

    Clinical jaundice in the first 24 hours of live

    Jaundice lasting longer than 21 days in preterm infants

    STB concentration increasing by more 0.2 mg/dLper hour or 5 mg/dL per day

    Direct serum bilirubin concentration exceeding1.5-2 mg/dL

    Jaundice who need phototherapy

    Sign ofunderlying disease

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    CAUSES OF NEONATAL

    INDIRECT HYPERBILIRUBINEMIA

    BASIS CAUSES

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    Indirect HYPERBILIRUBINEMIA

    OVERPRODUCTION ( HEMOLYSIS) Extravascular blood-hematomas, bruises

    Feto-maternal blood group incompatibilityRh - mom / baby Rh +

    O group mom / baby A or B

    Intrinsic red cell defects

    G-6-PD deficiencyhereditary spherocytosis

    Polycythemia

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    X- Linked disorder (2-6% carrier rate in Indonesia)

    enzyme protects red cell from oxidative damage

    >150 mutations

    Onset of jaundice usually day 2 - 3, peaks day 4 - 5

    Hyperbilirubinemia may be out of proportion to anemia

    Diagnosis- enzyme assay baby and mother DNA analysis

    Indirect HYPERBILIRUBINEMIA

    G6PD DEFICIENCY

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    Prematurity

    Hypothyroidism

    Inherited deficiency of conjugating enzymeuridine diphosphate glucuronyl transferase

    Other metabolic disorders

    Indirect HYPERBILIRUBINEMIA

    UNDERSECRETION

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    ENTEROHEPATIC CIRCULATION

    Decreased enteral intake Pyloric stenosis Intestinal atresia/ stenosis Meconium ileus Meconium plug Hirschsprungs disease

    Indirect HYPERBILIRUBINEMIA

    SECRETED but REABSORBED from gut

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    Cholestasis

    Biliary atresia Choledochal cyst

    # Direct bilirubin > 2 mg/dL

    # Time of appearance# Color of stools# Color of urine

    Direct HYPERBILIRUBINEMIA

    OBSTRUCTIVE DISORDERS

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    Bacterial sepsis Intrauterine infections: TORCH

    HYPERBILIRUBINEMIA

    MIXED

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    History

    Physical exam: gestational age activity/ feeding level of icterus pallor hepatosplenomegaly bruising, cephalhematoma

    HYPERBILIRUBINEMIA

    DIAGNOSIS

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    Laboratory tests

    Bilirubin levels: total and direct Mothers blood group and Rh type

    Babys blood group and Rh type

    Direct Coombs test on baby

    Hemoglobin Blood smear

    Reticulocyte count

    HYPERBILIRUBINEMIA

    DIAGNOSIS

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    Likely

    Rhesus, ABO, or other hemolytic disease Spherocytosis

    Less likely

    Congenital infection G-6-P-D deficiency

    Rapidly developing jaundice

    on Day 1

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    Rapidly onset jaundice

    after 48 hours of ageLikely

    Infection G-6-P-D deficiency

    Less likely

    Congenital Rh, ABO, spherocytosis

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    HYDRATION - FEEDING

    PHOTOTHERAPY

    EXCHANGE TRANSFUSION

    Phenobarbital Tin protoporphyrin

    HYPERBILIRUBINEMIA

    MANAGEMENT

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    Management of Hyperbilirubinemia

    in the Newborn Infant35 or more weeks of gestation

    Promote and support successful breast-feeding

    Perform a systematic assessment before dischargefor the risk of severe hyperbilirubinemia

    Provide early and focussed follow-up based on riskassessment

    When indicated, treat newborns with phototherapyor exchange transfusion to prevent the developmentof severe jaundice and possibly, kernicterus.

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    Feeding to Prevent and Treat

    Neonatal Jaundice

    Mothers should breast feed their babies

    caloric intake / dehydrationJaundice

    Supplementation with water or dextrosewater will not prevent or treathyperbilirubinemia

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    Systematic Assessment for

    Neonatal Jaundice

    Pregnant women:Blood group and Rh type

    If mom is Rh negative or O group:Babys cord blood group/ Rh type/ DAT

    Monitor infant for jaundice at least every 8-12 hours

    If level of jaundice appears excessive for age,perform transcutaneous bilirubin or total serumbilirubin measurement

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    Clinical

    assessmentof severity of

    jaundice

    Cephalocaudal progression

    face 5 mg/dL (approximately)

    upper chest 10 mg/dL (approximately)

    abdomen and upper thighs 15 mg/dL (approximately) soles of feet 20 mg/dL (approximately)

    Visual inspection may be misleading

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    Transcutaneous Bilirubinometers

    Useful as screening device TcB measurement fairly accuratein most infants with TSB < 15 mg/dL Independent of age, race and weight Not accurate after phototherapy

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    Complications of phototherapy

    Significant complications very rare

    separation of mother and baby increased insensible water loss and

    dehydration in premature baby

    PDA

    ROP

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    What decline in serum bilirubin can

    you expect with phototherapy?

    Rate of decline depends on effectiveness ofphototherapy and underlying cause of jaundice

    Intensive phototherapy should produce a decline inSTB of 1-2 mg/dL within 4-6 hours, and the STB levelshould continue to decline and remain below thethreshold level for exchange transfusion

    With standard phototherapy, expect decrease of 6%to 20% of the initial bilirubin level in the first 24 hours

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    Exchange Transfusion

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    Exchange Transfusion

    waste

    Red Blood Cells

    Double volumeExchange Transfusion2 X 85 mL/kg

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    EXCHANGE TRANSFUSION

    COMPLICATIONS cardiac failure

    metabolic- hypoglycemia, hyperkalemia, hypocalcemia

    air embolism

    bacterial sepsis

    transfusion transmitted viral disease

    necrotizing enterocolitis portal vein thrombosis

    Mortality / permanent sequelae 1-12%

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    Total Bilirubin Level (mg/dL)*Birth Weight (g)

    Phototherapy Exchange Transfusion

    < 1.500 5-8 13-16

    1.500-1.999 8-12 16-18

    2.000-2.499 11-14 18-20

    Guidelines for the use of phototherapy andexchange transfusion in low birth weight infants

    based on birth weight

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    Total bilirubin level (mg/dL)

    Exchange transfusionGestational age

    (weeks) Phototherapy

    Sick* Well36 14.6 17.5 20.5

    32 8.8 14.6 17.5

    28 5.8 11.7 14.6

    24 4.7 8.8 11.7

    Guidelines for use of phototherapy and exchange

    transfusion in preterm infants based on gestational age

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    < 1.250 g 1.250-1.499 g 1.500-1.999 g 2.000-2.499 g

    Standard risk

    Total bilirubin 13 15 17 18

    B/A ratio 5.2 6.0 6.8 7.2

    High risk*

    Total bilirubin 10 13 15 17

    B/A ratio 4.0 5.2 6.0 6.8

    Guidelines according to birth weight forexchange transfusion in low birth weight infants

    based on total serum bilirubin (mg/dL) andbilirubin/albumin ratio (mg/g) (whichever comes first)

    Guidelines for the Management of Hyperbilirubinemia Based on

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    g yp

    Birth Weight and Relative Health of the Newborn

    Serum Total Bilirubin Level (mg/dL)

    Healthy SickBirth Weight

    Phototherapy ExchangeTransfusion

    Phototherapy ExchangeTransfusion

    Premature

    < 1000 g

    1001 1500 g

    1501 2000 g

    2001 2500 g

    Term> 2500 g

    5 7

    7 10

    10 12

    12 15

    15 18

    Variable

    Variable

    Variable

    Variable

    20 25

    4 6

    6 8

    8 10

    10 12

    12 15

    Variable

    Variable

    Variable

    Variable

    18 20

    Averys Diseases of the Newborn. 2005

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    Tatalaksana IkterusBilirubin Serum Total (mg/dL)

    Terapi sinar Transfusi tukarUSIA

    Tanpa

    Faktor Risiko

    Prematur atau

    Dengan Faktor Risiko

    Tanpa

    Faktor Risiko

    Prematur atau

    Dengan Faktor Risiko

    Hari 1 Setiap ikterus yang terlihat 15 13

    Hari 2 15 13 25 15

    Hari 3 18 16 30 20

    Hari 4 dst 20 17 30 20

    Pocket Book WHO, 2005