3. • The normal TSB level is < 1 mg/dl
• Neonatal clinical jaundice is Dx. If the TSB is
> 5 mg/dL in FT NB.
> 7 mg/dL in preterm NB .
4. Definition :
Is an elevation of TSB level is > 2mg /dl ,
it is a common condition among NB babies ,and most of
the cases are benign problem
However, untreated sever unconjugated
hyperbilirubinemia is potentially neurotoxic,
whereas conjugated hyperbilirubinemia is often
signifies a serious underling illnesses
Definition of Jaundice :
Yellowish discoloration of skin , mucous membranes , and
sclera
6. Source of Bilirubin
75% comes from breakdown of Hb
25% comes from breakdown of free heme , non-
Hb proteins and ineffective erythropoisis
• 1gm of Hb produce about 34-35mg of bilirubin
• 1gm of albumin bind 8.5 mg of bilirubin
8. RBC catabolism •Heme-proteins
•Infective erythropoisis
Biliverdin
Accepter protein (Y or Z)
Glucronyl
transferase
Smooth RES
Bilirubin glucuronideB-
glucuronidase
Fecal bilirubin
Bilirubin
+
Albuimn
25%Heme
75%Heme
Hemeoxidase
RES
Biliverdin reductase
Enterohepatic
Recirculation
Of bilirubin
Intestinal
Bacteria
urobilinoids
9.
10. • unconjugated hyperbilirubinemia
Elevation of serum UCB 2 mg/dl or more
• conjugated hyperbilirubinemia
Is increase level of CB > 20% of the total serum
bilirubin
It is a sign of hepatobiliry dysfunction
11. Lipid-soluble
Can not be excreted by the
kidney
Can enter the CNS
particularly in NB
(neurotoxic)
Skin color lemon yellow or
orange yellow
Physiological & pathological
Water soluble
Can be excreted by
the kidney
Can not enter the
CNS ( non neurotoxic)
Skin color greenish or
muddy yellow
Always pathological
12. 1. Increased bilirubin production
Physiological jaundice
Pathological jaundice
Hemolytic diseases
immune ( RH, ABO incompatibility )
or subgroup
non-immune ( G6PD deficinciecy
H. spherocytosis)
Extravasated blood
(cephalhematoma, extensive bruises )
polycythemia
sepsis
2. Defective transport of bilirubin in the
circulation
Hypoalbuminemia ( prematurity ,
postnatal malnutrition )
Drugs e.g. synthetic vit. K,
sulfonamide , salicylate ,
gentamicin , aminophyline
furosemide , and digoxin .)
by displacement of bilirubin
from its albumin binding sites
3. Defective bilirubin uptake by the liver
Physiological jaundice
Pathological jaundice
prematurity
deficiency of lgandin ( Y and Z proteins )
sepsis
breast milk jaundice
4. Defective conjugation of bilirubin
Physiological jaundice
Pathological jaundice
Hypothyroidism
sepsis
Crigler-Najjar syndrome (Type I and II)
Lucey – Driscoll syndrome
5. Increased enterohepatic circulation
Bowel obstruction ( meconum ileus)
Delayed passage of meconum
( meconum plug , delayed feeding
( breast feeding jaundice ) and
hypothyroidism
13.
14. criteria
• Onset after 36 hr of age ( 2nd – 3rd day )
• Rate of bilirubin rise < 5mg/dl /day
• Peak of bilirubin con. Up to 12mg/dl in FT &
Up to 14mg/dl in preterm
• Duration 8 days in FT NB. & 14 days in preterm
• Serum CB level < 2mg/dl at any time
• The NB. Looks normal not anemic not sick active
with normal color of urine and stool
• It dose not require any treatment
16. PATHOLOGICAL JAUNDICEPHYSIOLOGICAL JAUNDICE
At any time, even 1st hrAfter 36hr ( 2nd – 3rd day )ONSET
>5 mg/dl per 24hr or
>0.5mg/dl/hr
< 5 mg/dl per 24hr
RATE OF
BILIRUBIN
RISE
>12 mg/dl in FT NB
>14 mg/dl in preterm NB
Up to 12mg/dl in FT NB
Up to 14mg/dl in preterm
NB
PEAK
BILIRUBIN
CONC.
> 1 wk in FT NB
> 14 days in preterm NB
8 days in FT NB
14 days in preterm NB
DURATION OF
PERSISTENCE
> 2 mg/dl at any time< 2 mg/dl at any time
SERUM CB
LEVEL
Looks abnormal , anemic
sick & abnormal color of
urine & stool
Looks normal not anemic
not sick normal urine & stool
color
CINICALLY
NB.
17. Two types of jaundice may occur in newborns who are
breast fed. Both types are usually harmless.
Breastfeeding jaundice
• Occurs in babies who do not nurse well or if the
mother's milk is slow to come in.
• Poor weight gain , Delayed stooling
Breast milk jaundice
• appear in some healthy Infant show good weight gain ,
normal LFT no evidence of hemolysis or illness
18. Breast milk jaundiceBreast feeding jaundice
2% of breast-fed term infant
develop UCHB after 1st wk. (10-
30mg/dl) during 2nd – 3rd wk. & if
BF. Continued slow UCB (3-10 wk)
30% of breast-fed infant Have
higher UCB level (>12mg/dl) during
the first week of life
After 7 days ( late onset )(early onset)during the first week
Breast milk has increased B-
glucuronidase enzyme activity ,has
pregnanediol or unknown
substance or FFA that interfere
or inhibit H uptake or conjugation
Milk intake , dehydration ,Occurs
due to delayed stooling or
reduced caloric intake
ttt. stop breast feeding for 1-2
days & give other milk formula
then resume breast feeding
Rapid UCB in 48 hr
ttt. Frequent breast feeding
( >10 feeding/day)
Do not supplement with
glucose water
19. • Major risk factors
– jaundice in first 24 hours (always
pathologic)
– ABO or Rh incompatibility and
positive coombs test
– G6PD deficiency
– Delivery at 35 to 36 weeks
gestation
– Significant Birth trauma
• Cephalhematoma
• Large hematomas
– Infant Breast feeds only (especially
before milk let-down occurs)
– F.H/O sibling who required
phototherapy for Neonatal
jaundice
– Serum bilirubin in high risk range
for age in hours
• Minor risk factors
– Male gender
– Maternal age over 25 years old
– Macrosomic IDM
– Delivery at 37 to 38 weeks
gestation
– Serum bilirubin in intermediate
range for age in hours
• Other risk factors
– Polycythemia
– Medication exposure
• Mother: Diazepam, Oxytcin
• Infant: Pediazole,
Chloramphenicol
20.
21. Within 1st 24hr of life
• Erythroblastsis fetalis
• TORCH infection
• Extravascular hematoma
• Sepsis
• polycythemia
On the 2nd -3rd day
• Physiological
• Crigler-Najjar syndrome
• Early onset Breast feeding
jaundice
After 3rd day and Within the 1st
wk
• Bacterial sepsis
• TORCH + Enterovirus
• Hematoma
After 1st wk of life
• Breast milk jaundice
• Extrahepatic Biliary atresia
• Hypothyroidism
Persistent jaundice during the 1st mo
of life
• Inpsissated bile syndrome (post-
hemolytic cholestasis )
• Hyperalimenation-associated
cholestasis
• Hypothyroidism
• pyloric stenosis
• Hepatitis (TORCH infection)
• Extrahepatic Biliary atresia
• Galactsemia
• Breast milk jaundice
• Crigler-Najjar syndrome
22. NOTE :
• Jaundice due to hemolytic anemia , in utero
infection (TORCH) and sepsis may present
at any time after birth
23. History
1. Family H/O jaundice , anemia , splenectomy (chronic
hemolytic anemia), liver disease
2. Previous sibling with jaundice (blood group incompatibility)
or Breast milk jaundice
3. Maternal illness during pregnancy : TORCH infection or DM
febrile illness
24. 4. Maternal drugs : sulphonamides , antimalaria or
salicylate (may cause hemolysis in G6PD-def. infant )
5. Maternal blood group and RH ,
6. Hx. Of abortion or SB & use of Anti-D if she was Rh – ve
7. Labor history : use of forceps vacuum asphyxia ,delayed
cord clamping
8. Postnatal H/O day of Onset, general condition, vomiting,
infrequent stooling , light colored stool, delayed Breast
feeding, whether the infant is breast-fed or formula-fed
25. Physical Examination
• Color of Jaundice (lemon yellow or orange yellow UCB type ,
greenish or muddy yellow CB type)
• (face =5mg/dl, mid abdomen =15mg/dl, sole = 20mg/dl )
• Pallor , plethora , petechiae
• Signs of Prematurity
• SGA (polycythemia ,TORCH infection )
• Microcephaly (TORCH infection)
• Cephalhematoma , bruises
• Hepatospleomegaly ( hemolytic anemia , infection )
• Signs of Hypothyroidism
• Signs of neonatal sepsis
• Omphalitis ( septicemia )
• Signs of Kernicterus
26. Laboratory investigation & other paraclinical
• Serum bilirubin total & direct
• blood group and RH of the infant & mother
• Coombs test
• CBC (Hb, Hct ,WBC )& Red cell morphology, S. electrolyte, RBS
• Reticulocyte count , G6PD enzyme assay ,osmotic fragility test
• Screening for TORCH infection
• Screening for sepsis (CRP, Blood cultures & other sites cultures , ESR )
• LFT ( serum albumin )
• Stool & urine analysis
• CXR
• Abdominal U/S
• ERCP
• Thyroid Stimulating Hormone
• Galactosemia Screen
27.
28. Definition ( Bilirubin Encephalopathy )
Neurological syndrome resulting from the
deposition of UCB in the
• Basal ganglia
• Brainstem nuclei
• Various cranial nerve nuclei
• Cerebellar nuclei
29. Incidence
30% in of infant of all gestational age with
untreated hemolytic disease
Total Bilirubin > 25 to 30 mg/dl
30. Risk factors
• UCB level > 30mg/dl but the range is wide (21-
50mg/dl)
• Duration of exposure to hyperbilirubinaemia
• Hypoproteinemia , Drugs , free FA
• Prematurity , Asphyxia , hyperosmlarity ,Seizures ,
hypertension , infection ( increase permeability of
BBB to bilirubin )
33. Phase 3 ( after the 1st wk )Phase 2 ( middle of 1st wk )Phase 1 ( 1st 12 days )
HypotoniaHypertoniaHypotonia
apneaIrritabilityLethargy
opisthotonusRDPoor feeding
SeizuresFeverVomiting
If pt. recover show few
abnormality (hypertonia)
Bulging FontanelHigh – pitched cry
Seizuresloss of Moro reflex
opisthotonusSeizures
DeathDeathDeath
Acute form
34. Chronic form
• Kernicterus (Residual deficits)
– Spasticity
– Severe athetoid Cerebral Palsy
– High frequency Hearing Loss or Deafness
– Mild Mental Retardation
– Upward gaze paralysis
• Dental dysplasia
35. American academy of pediatrics has identified potentially preventable
causes of Kernicterus :
• Early discharge (<48hr ) with no early follow-up within 48hr
• Failure to check the bilirubin level in infant noted to be jaundiced in
the 1st 24hr
• Failure to recognize the presence of risk factors for hyperbilirubinaemia
• Underestimation of the severity of jaundice by clinical ( visual )
assessment
• Lack of concern regarding the presence of jaundice
• Delay in measuring S.Bilirubin level despite marked jaundice or delay
in initiating phototherapy in presence of elevated Bilirubin level
• Failure to respond to parental concern regarding jaundice , poor
feeding or lethargy
36. Recommendation
• Any infant who is jaundiced before 24hr requires measurement of TSB
level & if it is elevated then should evaluated for possible hemolytic
disease
• Follow-up within 2-3 days of discharge to all NB discharged earlier
than 48hr after birth esp. < 38 wk gestation
• Avoid routine supplementation with water or glucose water
• Provide parents with written &verbal information about NB jaundice
37. • The goal of therapy is to prevent kernicterus
• Interpret all bilirubin levels according to the
infants age in hours
• Treat newborn ,when indicated with
phototherapy or exchange
38. 1. Phototherapy
2. Exchange Transfusion
3. Treatment of the Etiological factor :
Hypothyroidism
Stop drugs
Correction any factor that increase the
permeability of UCB eg. Acidosis
Increase frequency & volume of milk feeding
39. A . Conventional Phototherapy
A blue light and day light with wave 425-475nm
and 550-600 consequently is best
B . Intensive phototherapy
Fluorescent blue , fiberoptic blanket phototherapy
NOTE : Phototherapy decrease the need for exchange
transfusion & decrease No. of exchange transfusion, but it
is not substitute for exchange transfusion
40. The effectiveness of phototherapy in reducing
UCB depends on :
A. The light emitted in the effective range of
wavelengths (240- 470nm)
B. The distance between light source and infant
(most effective if 45cm)
C. The surface area of skin exposed
D. The rate of hemolysis and excretion
41. In premature NB without of significant of
hemolysis, UCB level usually declines 1-3
mg/dl after 12- 24hr of exposure
43. • Phototherapy is usually started at 50 – 70 % of
the maximal indirect level
• If the values exceed this level ,if Phototherapy
is unsuccessful in reducing the maximal
indirect level ,or if signs of kernicterus are
evident EXCHANGE TRANSFUION is indicated
44.
45.
46. Indication of Phototherapy
1. Abnormal rise of UCB level
2. While waiting for & in between exchange
transfusion
3. Prophylactic in some cases :
a. VLBW infant
b. Hemolytic disease of NB
c. Severely bruised premature infant
47. Procedure
• It is applied continuously except during feeding
• Infant kept naked except for eyes bandage and a diaper
• The infants are turned every 2 hr
• Monitoring of Temp. /2hr , UCB level & Hct. /4-8hr in
case of hemolysis and 12-24hr for other cases , and
body Wt. daily
• The Lamps should be changed after 2,000 hr of use
48. When discontinue phototherapy ?
When the level of UCB is low to eliminate
concern about toxic effects of UCB
• TSB level should be followed for at least 24hr
after discontinue phototherapy
• Risk factors for toxic level of UCB are absent
• The baby is old enough to handle the bilirubin load
• Usually at the level < 13-14 mg/dl in term NB
49. Guidelines for phototherapy in hospitalized infants of 35 or more weeks’ gestation. Note:
These guidelines are based on limited evidence and the levels shown are approximations.
51. Side effects of phtoyherapy
1. Dehydration
2. GIT ( loose or watery Diarrhea )
3. Damage of Corneal
4. Dermatitis (erythemtous macular rash &
purpuric rash )
5. DNA damage
6. Decrease maternal-infant interaction
7. Dark grayish-brown discoloration of skin (bronze
baby syndrome)
52. 2- Exchange transfusion
The goals of exchange transfusion in immune
Hemolytic disease :
o To correct anemia
o To remove the sensitized RBCs
o To remove the antibodies
o To reduce the UCB level
53. Indications
A. Severe hyperbilirubinaemia refractory to phototherapy
B. Hemolytic disease of NB
C. Others :
Septicemia
DIC
Polycythemia
IEM
Hypomagnesaemia
Respiratory depression
54. Procedure
Start phototherapy until Exchange transfusion
• Estimate the BWt , GA, vital signs and CVP
• Pre-exchange blood sample labs
Serum bilirubin total & direct
blood group and RH of the infant
Coombs test
CBC (Hb, Hct ,WBC )& Red cell morphology
Reticulocyte count
S,Ca ,RBS
LFT
ABG
55. • Aspirate 20 cc of infant's blood
• Infuse 20 cc of donors blood
• Exchange transfusion should carried out over (45-60 min )
• Monitoring during Exchange transfusion:
Body temp., CVP , Bp , PaO2 , pH,BS, S.Ca ,
• Blood Volume is double the blood volume of the infant
= 2 × 85 ml /kg = 170 ml /kg
• Umbilical Venous Catheter placed at 7 cm or less
• Alternate aspiration and infusion
56. Blood used in exchange transfusion :
• Fresh , warm , washed and irradiated
• Grouping :
in Rh incompatibility use infant’s ABO group, Rh – ve
in ABO incompatibility use group O, infant’s Rh type
For others infant’s ABO, Rh group.
57.
58.
59. Guidelines for exchange transfusion in infants 35 or more weeks’ gestation. Note that these
suggested levels represent a consensus of most of the committee but are based on limited
evidence, and the levels shown are approximations.
62. Other therapies
• Phenobarbital:
as adjunct management It is recommended only in Crigler-Najjar
type II
• Metalloporphyrins :
by inhibits conversion of heme to biliverdin, so reduce UCB level
in (ABO incompatibility or G6PD deficiency)
• Intravenous immunoglobulin
0.5 – 1 g/kg/dose over 4 hr repeated in 12 hr it is
recommended in persist isoimmune hemolytic disease (both ABO
& Rh ).
63. Crigler-Najjar Disease :
• Congenital deficiency or absence of glucuronyl
transferase enzyme
• Autosomal recessive
Type I : Congenital absence of the enzyme
kernicterus develops early leading to death
Type II : partial deficiency of the enzyme
the condition improves on administration of
phenobarbitone & kernicterus is unusual
64. Gilbert’s syndrome
• Benign disorder
• Can result from mutations to genes coding for
UDP-glucuronyl transferase
65.
66.
67. Definition
An increased level of direct bilirubin >20% of the
total serum bilirubin
It is a sing of hepatobiliry dysfunction
68. Hepatic
Idiopathic neonatal hepatitis
intrahepatic cholestasis with paucity
of bile duct (Alagille syndrome )
Infections
Sepsis
Viral Hepatitis
TORCH infections
Metabolic
Galactosemia
Alpha-1-antitrypsin deficiency
Cystic fibrosis
Drugs
Total parenteral nutrition
Post-hepatic
Biliary atresia
Bile duct obstruction
Choledochal cyst
causes
Others
Dubin-Johnson syndrome
Rotor s syndrome
69. Clinical manifestations
• Greenish jaundice due to CB
• The stool are clay in color while urine is dark
• Hepatospleomegaly
• Other clinical manifestations of the etiology may
be present e.g. refuse feeding purpura ... etc.
Guidelines for exchange transfusion in infants 35 or more weeks’ gestation.Note that these suggested levels represent a consensus of most of the committee but are based on limited evidence, and the levels shown are approximations. See ref. 3 for risks and complications of exchange transfusion. During birth hospitalization, exchange transfusion is recommended if the TSB rises to these levels despite intensive phototherapy. For readmitted infants, if the TSB level is above the exchange level, repeat TSB measurement every 2 to 3 hours and consider exchange if the TSB remains above the levels indicated after intensive phototherapy for 6 hours.The following B/A ratios can be used together with but in not in lieu of the TSB level as an additional factor in determining the need for exchange transfusion52: If the TSB is at or approaching the exchange level, send blood for immediate type and crossmatch. Blood for exchange transfusion is modified whole blood (red cells and plasma) crossmatched against the mother and compatible with the infant.53