neonatology neonatal jaundice l.
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Neonatology Neonatal Jaundice. Contents. Billirubin metabolism in normal neonates Special problems in neonates The diseases in relation with Neonatal Jaundice Dangerous of the Hyperbillirubinemia. Normal Billirubin Metabolism in neonates. ineffective erythropoiesis. liver.

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contents
Contents
  • Billirubin metabolism in normal neonates
  • Special problems in neonates
  • The diseases in relation with

Neonatal Jaundice

  • Dangerous of the Hyperbillirubinemia
slide3

Normal Billirubin Metabolism in neonates

ineffective erythropoiesis

liver

Reticulo-

endothelial

system

breakdown of hemoglobin80%

Intrahepatic hemachrome

Tissue hemachrome

breakdown of

hemoproteins

Billirubin +plasm albumin---in blood

Bilirubin+ProteinY、Z

Hepato-

enteral

circulation

Smooth

endoplasmic

reticulum

conjugative

—Hepatic

cells

Reabsoption:90%

conjugated Bi

Capillary bile duct

Kidney 10%

intestine lumen

Glucuronic acid90%

Glucose conjugates

Xylose conjugates

Urine :urobilinogen

(0-4mg/d)

Bacteria

conjugated Bi

Portal vein

urobilinogen

80%excrete

Fecal:urobilinogen(40-280mg/d)

specificity of billirubin metabolism in newborns
Specificity of Billirubin Metabolism in newborns
  • Increased bilirubin formation
    • Excesive RBCs after birth
    • Shorter half life of fetal hemoglobin
    • Heme oxygenase
    • Increased bilirubin originated from bypass
specificity of billirubin metabolism in newborns5
Specificity of Billirubin Metabolism in newborns
  • Insufficient in transiting bilirubin
    • albumin linking/transporting bilirubin 
specificity of billirubin metabolism in newborns6
Specificity of Billirubin Metabolism in newborns
  • Insufficient in treatingbilirubin by liver
    • Y. Z protein 
    • Hepatic Enzyme under developed
    • Activity of hepatic enzymeeasily being undermined
    • Serums glucose rapidly 
specificity of billirubin metabolism in newborns7
Specificity of Billirubin Metabolism in newborns
  • Load of hepatoenteral circulation
    • Intestine flora
    • Activity ofβ-Glucuronidase within mesocaval 
    • High quantity of bilirubin in meconium
pathological jaundice
Pathological Jaundice
  • Presenting earlier, <24-48 h after birth
  • Higher Serum Billirubin level
    • TBI >12mg/dl
    • increasing rapidly, >5mg/dl/d
  • Longer duration:
    • Term:>2 w preterm:>4 w
  • Persistently progressing, or re-presence after disappearing
etiology of pathological jaundice
Etiology of Pathological Jaundice
  • Unconjugated
    • Hemolytic Disease of the Newborn
      • alloimmune hemolytic (ABO and Rh types)
      • deformity of RBC
      • RBC Enzyme deficiency
      • Extravascular Hemolysis
    • BreastfeedingJaundice
    • Erythrocytosis
etiology of pathological jaundice11
Etiology of Pathological Jaundice
  • Conjugated
    • Neonatal Hepatic diseases
    • Congenital bile duct disorders
    • Congenital metabolic disorders
etiology of pathological jaundice12
Etiology of Pathological Jaundice
  • Mixed by Unconjugated with Conjugated
    • Infectious
hemolytic disease of newborn
Hemolytic disease of newborn
  • ABO type incompatability alloimmune hemolytic disease
    • 50% happened in first pregnancy
    • 20% ABO type incompatability,incidence 10%
    • majority maternal type O and infant type A or B
  • RH type incompatability alloimmune hemolytic disease
    • No Occurrence in first pregnancy, except the one had history of abortion and transfusion
    • Antigenic: D>E>C>c>e
    • RH D most commonly seen
hemolytic disease of newborn14
Hemolytic disease of newborn

Clinical manifestation

  • Jaundice
    • Present at different time: ABO 2-3d. RH<24H
    • Level of TBi:

ABO: light elevated

RH: severely elevated and progressing

  • Anemia: severe in RH and may complicated with heat failure
  • Hepatosplenomegaly
  • Hydrops fetalis (universal edema of the fetus)
hemolytic disease of newborn15
Hemolytic disease of newborn

Laboratory investigation

  • Prompt hemolysis indicators
    • RBC Hb
    • Reticulocyte
    • Nuclear RBC> 10%
  • blood type incompatibility
    • Maternal: O, newborn: A,B
    • Maternal: Rh-, newborn: Rh+
hemolytic disease of newborn16
Hemolytic disease of newborn

Laboratory investigation

  • Coomb’s test
    • Rh:
      • Direct: positive
      • Indirect: positive (Ab. present and the type)
    • ABO:
      • Direct: positive
      • Free antibody present
      • Ab. Release test: positive
hemolytic disease of newborn17
Hemolytic disease of newborn
  • Diagnosis
    • History:
      • Past production history of the mother
      • Newborn with hyperbilirubinemia, anemia
      • Stillbirth
      • Hydrops fetalis
    • Clinical manifestation
    • Laboratory evidences
hemolytic disease of newborn18
Hemolytic disease of newborn
  • Prenatal diagnosis
    • Mother with Rh-negative: measure Rh Ab in GA 28,32,36 w
    • Examining amniotic fluid: bilirubine level
hemolytic disease of newborn19
Hemolytic disease of newborn
  • Prevention
    • Mother with Rh-negative delivering the first fetus given anti-D Ab.
neonatal hepatitis
Neonatal Hepatitis
  • Intrauterine, intrapartum and during delivering infected
    • Virus: common: CROTCHS/CMV
  • Pathogenesis
    • Cholestasis: Capillary bile duct and hepatic duct obstruction
    • Liver cells and mesenchymal inflammatory change
    • portal area hyperplasia
neonatal hepatitis21
Neonatal hepatitis

Clinical manifestation

  • Obstructive jaundice
    • Jaundice represent following the fading of physiological jaundice
    • Dark yellow urine and grey-white stool
  • Gastrointestinal symptoms:
    • anorexia, hyperphagia, vomiting, diarrhea
  • Anemia
  • hepatosplenomegaly
neonatal hepatitis22
Neonatal hepatitis

Laboratory investigation

  • TBi , VDB: direct and indirect positive
  • Hepatic function:
    • ALT , serum proteins , albumin 
  • Serous virology
    • Positive: CMV IgG/M, TOXO IgG/M
    • HBV: antigens and antibodies
biliary atresia cholestasis
Biliary atresia cholestasis
  • Severe obstructive jaundice with high Conjugated Bi
  • Persistent grey-white stool
  • Severe hepatosplenomegaly
  • Progressive worsening in hepatic function
  • Diagnosis based on ultrasound, CT and MRI

Isotope ECT may helpful

jaundice with infection
Jaundice with Infection
  • When there are no find in particular
  • General manifestation of the infection
  • Blood stream infection or organ infection
  • Both conjugated and unconjugated Bi
  • Jaundice subsided when infection controled
breastfeeding jaundice
BreastfeedingJaundice
  • Babies with exclusive breast feeding
  • Jaundice persists
  • Unconjugated Bi predominant
  • Without hepatosplenomegaly and injury of hepatic function
  • Jaundice fading or disappearing after suspending breast feeding
  • Good outcome
kernicterus
Kernicterus
  • Pathology
    • staining and necrosis of neurons in the basal ganglia, hippocampus, and subthalamic nucleus of the brain
kernicterus27
Kernicterus
  • For a healthy term infant with a STB concentration less than 30mg/dl (513μmol/L) will usually not suffer neurologic damage
  • No certainty that lower STB levels under some circumstances eliminates the possibility of permanent injury, in particular with respect to auditory function.
  • The neurologic consequences of prolonged exposure to moderate hyperbilirubinemia are uncertain.
kernicterus28
Kernicterus
  • Major risk factor
    • Albumin binding of bilirubin.
    • 1 gram of albumin can bind 8.2mg of bilirubin
    • A serum albumin concentration of 3g/dl could theoretically bind approximately 25mg/dl of bilirubin
  • Not cross the blood-brain barrier
    • Albumin-bound bilirubin
    • Conjugated bilirubin
  • Premature
    • low serum albumin concentrations
    • frequency of acidosis
kernicterus29
Kernicterus
  • Clinical manifestation
    • First 1 to 2 d: poor sucking, stupor, hypotonia, seizures
    • In the middle of the first week: hypertonia of extensor muscles, opisthotonus,retrocollis, and fever
    • After the first week: hypertonia
    • Survivors: appeared with hypotonia, active deep tendon reflexes, obligatory tonic neck reflexes, delayed motor skills and after the first year, movement disorder
clinical management
Clinical management
  • Phototherapy
  • Plasma or albumin
  • Exchange transfusion
  • Enzyme inducer: phenobarbital
  • Original diseases treatment
  • The other symptomatic treatment
summery
Summery
  • Almost all newborns have jaundice and the majority are transient or physiological
  • Certain diseases causing neonatal jaundice have prolong and serious presentation
  • Kernicterus is the most severe complication and has very poor outcome and sequelae