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Etiologies of pathological jaundice
1
2
3
Kernicterus ( bilirubin encephalopathy)
• Definition: neurological syndrome resulting
from deposition of indirect bilirubin in the
basal ganglia and brain stem
• Multifactorial→ the precise blood level and
the duration of exposure is not known
4
Clinical features of kernicterus
Acute form
• Phase 1 ( 1st-2nd d): poor sucking, stuper,
hypotonia, seizure
• Phase 2(middle of 1st wk): hypertonia of
extensor muscles, opisthonos, retrocollis,fever
• Phase 3(after 1 wk):hypertonia
5
Chronic form
• 1st year: hypotonia, active deep tendon
reflexes, obligatory tonic neck reflex, delayed
motor skill
• After 1st year:movement
disorder(choreoathetosis,ballismus, tremor),
upward gaize, sensorineural hearing loss
6
Prevention of kernicterus
1. for any neonate with jaundice within 24hrs, serum
bilirubin level should be checked
2. Follow up within 2-3d of discharge for all neonate
discharged earlier than 48hr
3. Recognize the presence of possible risk factor
4. Never underestimate the severity of jaundice by
visual assessment
5. Early initiation of phototherapy in those with elevated
bilirubin
6. Recognize parental concern regarding jaundice, poor
feeding, lethargy
7
Diagnosis of pathological jaundice
History and physical examination
• Time of onset, duration, color, and pattern of jaundice
• Symptoms suggestive of anemia,polycythemia
• Family history
• Maternal blood group and rh
• Perinatal hx (sns&sms of infection, drug)
• Term/premature?
• Me conium passed /not?
• Breast feeding/not?
8
• Hematoma/ bruising
• Nutritional status
• Respiratory destress/not
• Hepato-spleenomegally
• Sns &sms suggestive of sepsis
• Vomiting, lethargy, poor feeding, apnea,
bradycardia, excessive weight loss
• Light colored stool, dark urine
9
Laboratory investigation
• TSB and direct bilirubin level
• Blood type(ABO, Rh)
• Direct antibody test(coombs test)
• Serum albumin
• CBC with differential and smear for RBC
morphology
10
• Reticulocyte count
• ETCO(end tidal carbon monoxide)
• G6PD( Glucose-6-phosphate dehydrogenase)
• Urine for reducing agents
11
Treatment of hyperbilirubinemia
Goal: to prevent neurotoxicity while not
causing harm.
• phototherapy
•Exchange transfusion
12
Phototherapy
• Exposure to high intensity of light in the visible
spectrum
• Maximally in the blue range(420-470nm)
• Broad spectrum(white, blue) and narrow-
spectrum(super blue)
• Photochemical reaction
reversible photo-isomerization
lumirubin-irreversible structural isomer
13
• Factors
light enery emitted in the effective range
distance b/n neonate and the lights
surface area of the exposed skin
the rate of production, metabolism and
excretion
• Imtensive phototherapy
special blue fluorescent tubes
lamp 15-20
fiber optic phototherapy blanket
14
• Not substitute for exchange
transfusion(indicated)
• Monitoring: continuous, turned frequently, serum
bilirubin and hematocrit every 4-8 hr should
continue for at least 24 hr after Rx, skin color is
not reliable.
• Role of iv fluid supplementation- dehydrated pts ,
level neat to exchange
15
16
Complications of phototherapy
• Loose stool, erythematous macular rash,
purpuric rash—transient porphyrinemia
• Overheating, dehydration, hypothermia,
bronze baby syndrome ( dark, grayish-brown
skin discoloration), corneal damage
17
Exchange transfusion
• Indications
if intensive phototherapy fails
if the risk of kernicterus exceeds the risk of pr
if signs of kernicterus are evident
if the TSB is in the range for exchange
18
19
• the total amount of blood exchanged is equal to
2x the neonates blood volume
Weight(kg) x 85 ml/Kg x 2
• 5-20ml/cycle are withdrawn and infused, 45-90
min
• Should remove 85% of infant’s RBC, maternal
antibodies, and exchangeable tissue ind.bilirubin
• Catheter
• Continuation of photherapy-reduces subsequent
exchange transfusion.
20
complications
• Blood( transfusion rxn, metabolic instability,
infection), catheter(vessel perforation),
procedure( hypotension or necrotizing
enterocolitis)
• Late-anemia
21

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patholo jaundice.pptx

  • 2. 2
  • 3. 3
  • 4. Kernicterus ( bilirubin encephalopathy) • Definition: neurological syndrome resulting from deposition of indirect bilirubin in the basal ganglia and brain stem • Multifactorial→ the precise blood level and the duration of exposure is not known 4
  • 5. Clinical features of kernicterus Acute form • Phase 1 ( 1st-2nd d): poor sucking, stuper, hypotonia, seizure • Phase 2(middle of 1st wk): hypertonia of extensor muscles, opisthonos, retrocollis,fever • Phase 3(after 1 wk):hypertonia 5
  • 6. Chronic form • 1st year: hypotonia, active deep tendon reflexes, obligatory tonic neck reflex, delayed motor skill • After 1st year:movement disorder(choreoathetosis,ballismus, tremor), upward gaize, sensorineural hearing loss 6
  • 7. Prevention of kernicterus 1. for any neonate with jaundice within 24hrs, serum bilirubin level should be checked 2. Follow up within 2-3d of discharge for all neonate discharged earlier than 48hr 3. Recognize the presence of possible risk factor 4. Never underestimate the severity of jaundice by visual assessment 5. Early initiation of phototherapy in those with elevated bilirubin 6. Recognize parental concern regarding jaundice, poor feeding, lethargy 7
  • 8. Diagnosis of pathological jaundice History and physical examination • Time of onset, duration, color, and pattern of jaundice • Symptoms suggestive of anemia,polycythemia • Family history • Maternal blood group and rh • Perinatal hx (sns&sms of infection, drug) • Term/premature? • Me conium passed /not? • Breast feeding/not? 8
  • 9. • Hematoma/ bruising • Nutritional status • Respiratory destress/not • Hepato-spleenomegally • Sns &sms suggestive of sepsis • Vomiting, lethargy, poor feeding, apnea, bradycardia, excessive weight loss • Light colored stool, dark urine 9
  • 10. Laboratory investigation • TSB and direct bilirubin level • Blood type(ABO, Rh) • Direct antibody test(coombs test) • Serum albumin • CBC with differential and smear for RBC morphology 10
  • 11. • Reticulocyte count • ETCO(end tidal carbon monoxide) • G6PD( Glucose-6-phosphate dehydrogenase) • Urine for reducing agents 11
  • 12. Treatment of hyperbilirubinemia Goal: to prevent neurotoxicity while not causing harm. • phototherapy •Exchange transfusion 12
  • 13. Phototherapy • Exposure to high intensity of light in the visible spectrum • Maximally in the blue range(420-470nm) • Broad spectrum(white, blue) and narrow- spectrum(super blue) • Photochemical reaction reversible photo-isomerization lumirubin-irreversible structural isomer 13
  • 14. • Factors light enery emitted in the effective range distance b/n neonate and the lights surface area of the exposed skin the rate of production, metabolism and excretion • Imtensive phototherapy special blue fluorescent tubes lamp 15-20 fiber optic phototherapy blanket 14
  • 15. • Not substitute for exchange transfusion(indicated) • Monitoring: continuous, turned frequently, serum bilirubin and hematocrit every 4-8 hr should continue for at least 24 hr after Rx, skin color is not reliable. • Role of iv fluid supplementation- dehydrated pts , level neat to exchange 15
  • 16. 16
  • 17. Complications of phototherapy • Loose stool, erythematous macular rash, purpuric rash—transient porphyrinemia • Overheating, dehydration, hypothermia, bronze baby syndrome ( dark, grayish-brown skin discoloration), corneal damage 17
  • 18. Exchange transfusion • Indications if intensive phototherapy fails if the risk of kernicterus exceeds the risk of pr if signs of kernicterus are evident if the TSB is in the range for exchange 18
  • 19. 19
  • 20. • the total amount of blood exchanged is equal to 2x the neonates blood volume Weight(kg) x 85 ml/Kg x 2 • 5-20ml/cycle are withdrawn and infused, 45-90 min • Should remove 85% of infant’s RBC, maternal antibodies, and exchangeable tissue ind.bilirubin • Catheter • Continuation of photherapy-reduces subsequent exchange transfusion. 20
  • 21. complications • Blood( transfusion rxn, metabolic instability, infection), catheter(vessel perforation), procedure( hypotension or necrotizing enterocolitis) • Late-anemia 21