Pembimbing

dr Pulung SpA

Hiperbilirubinemia
UNIVERSITAS YARSI RS SAID SUKANTO
Definition

Increased total bilirubin levels in the first week of

birth. Maximum normal levels are 12-13 mg% (205-
220 mmol/L).
Elevated plasma levels of bilirubin 2 standard
deviation or more than expected lvels based on
infant age or more than presentil 90.
Bilirubin levels in the blood exceed 1 mg/dL (17,1
umol/L)
Etiology

Excessive Production
Disruption in uptake and conjugate hepatic processes
Transportation Interruption
Erection disorder
Clinical Manifestations
Letargi
Seizures
deaf, speech disorders, mental retardation
When the baby lives in old age with muscle spasms,
convulsions, stenosis accompanied by muscle tension
Belly belly
Enlargement of the liver
Feces are colored like putty
Vomiting, anorexia, fatigue
The color of urine is dark
Patofisiologi

Pembentukan
Bilirubin

Transportasi
Bilirubin

Asupan
Blirubin

Konjugasi
Bilirubin

Eksresi
Bilirubin
Patofisiologi
Pathophysiology The formation of bilirubin

Bilirubin is the orange jelly crystalline pigment which

is the final form of heme cation breakdown through
the oxidation-reduction process.
The first oxidation step is biliverdin which is formed
from heme with the help of heme oxygenase
enzyme.
Pathophysiology Bilirubin Transportation

The formation of bilirubin occurring in the

endothelial reticulo system, is then released into the
circulation which will bind to albumin. The bilirubin bound
to serum albumin is a non-polar substance and insoluble in
water and then transported into liver cells. Bilirubin bound
to albumin can not enter the central nervous system and is
non-toxic.
Pathophysiology Bilirubin intake

By the time the bilirubin-albumin complex reaches

the hepatocyte plasma membrane, albumin is bound to the
cell surface receptor. Then bilirubin, transferred via
membrane cells binding to ligandin (y protein), may also
with other cytosilic bond proteins
Pathophysiology Conjugation
Unconjugated bilirubin is converted to a water-
soluble conjugate bilirubin form in the endoplasmic
reticulum with the help of uridine diphospate glucuronosyl
transferase (UDPG-T) enzyme. Catalysis by this enzyme will
convert the formation into bilirubin monoglukoronide
which will then be conjugated into bilirubin diglukoronide.
This bilirubin is then excreted into the gall bladder. While
an unconjugated bilirubin molecule will return to the
endoplasmic reticulum for subsequent reconjugation.
Pathophysiology Eksresi
After the conjugation process, bilirubin will be
excreted into the gallbladder, then enter the
gastrointestinal tract and expressed through the feces.
After being in the small intestine, the conjugated bilirubin
can not be directly reconstituted unless it is converted back
into an unconjugated form by the enzyme beta-
glucoronidase present in the gut. Re-resorption of bilirubin
from the gastrointestinal tract and back to the liver for
conjugation is called enterohepatic circulation.
Pathophysiology Mechanism
There are 4 common mechanisms in which
hyperbilirubinemia and jaundice may occur:
1. Excessive formation of bilirubin
2. Impaired removal of unconjugated bilirubin by the liver
3. Bilirubin conjugate disorder
4. Decreased excretion of bilirubin conjugated in bile due to
intra-hepatic functional or mechanical opbrequency factor.
Classification
Classification of Jaundice in Neonates :
Physiological Jaundice : Occurs after the first 24 hours. In
Term Infants , the peak value 6-8 mg/dl usually reached on 3
through 5 days. In pre-Term infants, the peak value 10-12 mg/dL,
even up to 15 g/dL. Increased serum bilirubin accumulation < 5
mg/dL/day.

Phatological Jaundice : Occurs wthin the first 24 hours.

Increased serum bilirubin accumulation > mg/dl/day. Breastfed
infants, total serum bilirubin levels > 17 mg/dL. Jaundice persists
after 8 days in term infants and after 14 days in pre-term infants.
Bilirubin direct >2 mg/dL.
Classification
Degree of Jaundice Area Estimation of
Jaundice Bilirubin Levels

I Head and Neck 5,0 mg%

II above umbilicus 9,0mg%

under umbilicus to
III upper limb above knee 11,4mg%

IV Up to lower limbs knee 12,4mg%

V Until the palms of the 16,0mg%

hands and feet
Medication
1. Preventive Strategy
a. Primary Preventive
b. Secondary Preventive
2. Pharmacotherapy
a. Intravenous Immunoglobulin
b. Fenobarbital
c. Metalloprotoprophyrin
d. Tin Protoporphyrin ( Sn Pp ) and Tin Mesoporphyrin ( Sn Mp )
e. Inhibitor b - glukuronidasi , example :L aspartic acid and kasein
holdolisat
3. Phototherapy
4. Exchange Transfusion
Medication - Phototherapy
Phototherapy is
indicated at elevated
bilirubin levels in relation
to age in term neonate or
underweight in pre-term
neonates, in accordance
with the recommendation
of the American Academy
of Pediatrics (AAP)
Medication Exchange Transfusion

Exchange transfusion is an act of taking small amounts of blood

followed by the return of blood from donors in the same amount
repeatedly until most of the blood of the patient is exchanged

In hyperbilirubinemia, this action aims to prevent the

occurrence of bilirubin encephalopathy by removing indirect bilirubin
from the circulation. In infants with isoimmunization, exchange
transfusion has additional benefits, as it helps remove maternal
antibodies from the baby's circulation. Thereby preventing further
haemolysis and correcting anemia.
Medication
AAP recommends treatment of hyperbilirubinemia in healthy and moderate
neonates.
Usia ( Considerat Phototherap Exchange Exchange Transfusion
jam ) ion of light y Transfusion and Phototherapy
therapy

25-48 >12mg/dl >15 mg/dl >20 mg/dl >25 mg/dl

(>200 ( >250 (>340 (425 mol/L)
mol/L) mol/L) mol/L)

49-72 >15mg/dl >18 mg/dl >25mg/dl >30 mg/dl

(>250 (>300mol/L (425 (510mol/L)
mol/L) ) mol/L)

>72 >17 mg/dl >20mg/dl >25mg/dl >30mg/dl

(>290 (>340mol/L (>425 (>510 mol/L)
mol/L) mol/L)
Medication
Tatalaksana hiperbilirubinemia pada Neonatus
Kurang Bulan Sehat dan Sakit ( >37 minggu )
Neontaus kurang bulan sehat Neontaus kurang bulan sakit
:Kadar Total Bilirubin Serum :Kadar Total Bilirubin Serum
(mg/dl) (mg/dl)

Berat Terapi sinar Transfusi tukar Terapi sinar Transfusi tukar

Hingga 1000 g 5-7 10 4-6 8-10

1001-1500 g 7-10 10-15 6-8 10-12

1501-2000 g 10 17 8-10 15

>2000 g 10-12 18 10 17
Complications - Phototherapy
1. Increased "insensible water loss" in infants
2. Increased defect frequency
3. The appearance of skin disorders
4. Impaired retina
5. Impaired growth
6. Increase in temperature
7. Some other disorders such as drinking disorders,
lethargy, irritability are sometimes found in patients.
8. Gonadal abnormalities,
Complications Exchange Transfusion
1. Vascular: air embolism or thrombus, thrombosis
2. Cardiac abnormalities: arrhythmias, overload, cardiac
arrest
3. Electrolyte disorders: hypo / hypercalcemia,
hypernatremia, acidosis
4. Coagulation: thrombocytopenia, excess heparinisasi
5. Infection: bacteremia, viral hepatitis, cytomegal,
necrotizing enterocolitis
6. Other: hypothermia, hypoglycemia
Conclusion
Many newborns, especially small infants (babies
with birth weight <2500 g or gestational age <37 weeks)
have jaundice during the first week of life. Existing
epidemiological data show that over 50% of newborns
suffer from clinically detectable jaundice within the first
week of life
Conclusion
Jaundice is a change of color of the skin / eye sclera
(normal white beer) to yellow because of elevated levels of
bilirubin in the blood. Jaundice in newborns can be a
physiological (normal) thing, present in 25% - 50% in well-
born infants. But it can also be pathological (not normal)
for example due to opposite Rhesus blood baby and
mother, sepsis (severe infection), blockage of bile ducts,
and others. Hyperbilirubinemia is a state of bilirubin levels
in the blood> 13 mg / dL.
Conclusion
Accelerate the process of conjugation eg by giving
phenobarbital, giving less substrate for transportation or
conjugation, decomposing bilirubin with phototherapy and
exchange transfusion. Although phototherapy can rapidly
reduce bilirubin levels, it can not replace exchange
transfusions in severe haemolysis processes. Phototherapy
can be used for pre- and post-exchange transfusion.
Conclusion
Factors that influence the determination of the
intensity of this light are the type of light, wavelength of
light used, the distance to the neonate beam and the
surface area of the irradiated neonate and the use of light
reflecting media.
THANKYOU