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What Is Hyperbilirubinemia?

Hyperbilirubinemia (also known as jaundice) is anincreased level of bilirubin in the blood

bilirubin : A substance in the blood that is produced bythe breakdown of red blood cells. It is released in the

unconjugated form. Unconjugated bilirubin is fat-soluble. It cannot be

excreted in bile or urine and is absorbed by the SQ fat,causing a yellowish discoloration of the skin

Jaundice is observed during the 1st wk in approximately60% of term infant and 80% of preterm infant.

Hyperbilirubinemia can be toxic, with high levels resultingin an encephalopathy known as kerni-cterus.


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Conjugation of bilirubin

The liver must conjugate the bilirubin (changeit into a water-soluble form) in order for thebody to excrete it through the biliary tree into

the gut.

Conjugated bilirubin is further catabolised byintestinal flora & It forms a major component

of bile in faeces (This gives the characteristicorange colour to faeces).

A small amount is passed in the urine


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When deconjugated in the

intestine:

The bilirubin is absorbed across the intestinal

mucosa and returned to the circulation.

Travels in plasma, bound to albumin. Enters the

liver cells with the aid of Y & Z carrier proteins Becomes conjugated with glucoronic acid by an

enzymeGlucuronyl Transferase

Hypoxia or hypothermia may compromise

bilirubin conjugation Total serum bilirubin is the sum of conjugated

(direct) and unconjugated (indirect) bilirubin.


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BILIRUBIN

UNCONJUGATED

Final product of hemedegeneration

Water-insoluble Tightly complexed to

serum albumin

Cannot be excreted inurine

Free form is toxic Lab test: Total bilirubin

minus direct bilirubin

Normal values 0,2-1.4mg/dl

CONJUGATED

Water-soluble

Loosely bound to

serum albumin Excess amounts are

excreted in urine

Nontoxic

Lab test: measured bydirect bilirubin

Requires O2 andglucose


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666

Risk factors for jaundice

JAUNDICE J - aundice within first 24 hrs of life

A - sibling who was jaundiced as neonate U - nrecognized hemolysis

N on-optimal sucking/nursing

D - eficiency of G6PD

I - nfection

C ephalhematoma /bruising

E - ast Asian/North Indian


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Types of Jaundice

Physiologic Jaundice.

Breast milk Jaundice

Pathologic JaundiceOR

Early Onset

Late Onset


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Physiologic Jaundice

Clinical jaundice appears at 2-3 days.

Total bilirubin rises by less than 5 mg/dl (86

umol/L) per day.

Peak bilirubin occurs at 3-5 days of age.

Peak bilirubin concentration in Full-term infant


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Factors Associated with Physiological

Jaundice

Polycythemia & Hyperbilirubinemia An infant has more RBCs than an adult ( Hb:18-19g/dl),

and the lifespan of an RBC is shorter in neonates ( 80

days). Increased RBCs and a shorter lifespan leads to increased

destruction of RBCs, which leads to more bilirubin in the

blood, which leads to hyperbilirubinemia

Prematurity & Hyperbilirubinemia Premature infants are more susceptible to hyperbilirubinemia due

to: Immature hepatic system

Delayed enteral feedings (Hypoglycaemia )

Decrease in serum albumin levels

Hypoxia /asphyxia, Hypothermia


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Breastfeeding Jaundice

Due to insufficient milk production by

mom or intake by baby;

Supplemental water or dextrose-water

administration may decreases breast

milk production

Decreased volume and frequency of

feedings

Decrease gut motility & delayed stooling


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Breastfeeding Jaundice Management

Increase frequency of feedings to more than 10per day

Formula supplement if infant has decline inweight gain, delayed stooling, and continuedpoor caloric intake

Breastfeeding should be continued to maintainbreast milk production

Neutral thermal environment

Prevent hypoglycaemia & hypoxiaAvoid constipation

Phototherapy for bilirubin 17-22mg/dl


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BreastMILK Jaundice

Occurs later in life (Day 6 to 14)

Bili 12 to 20

May stay high for 1-2 months

Etiology: Unclear; Substances in maternal milk,such as alpha glucuronidases, and nonesterified

fatty acids, may inhibit normal bilirubinmetabolism

Management-Breastfeeding may be temporarily interrupted-With formula substitution, the total serum bilirubin

level should decline rapidly over 48 hours,confirming

the diagnosis


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Pathologic Jaundice

Anything OTHER THAN physiologic orbreastfeeding or breastmilk jaundice!!

Jaundice within 24 hours after birth

Rapidly rising total serum bilirubinconcentration level higher than 17 in a full-term newborn

Other features of concern include prolonged

jaundice Evidence of underlying illness

Elevation of the serum conjugated bilirubinlevel to >2 mg per dL or more than 20% of thetotal serum bilirubin


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Common causes of pathologicalJaundice

a. hemolytic diseases:

ABO, Rh incompatibility

b. Glucose-6-phosphate (G-6-PD)

deficiency


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Rh hemolytic disease

People who are Rh+ have the Rh antigen on theirRBCs. People who are Rh- do not.

If blood that is Rh+ enters the body of a person who isRh-, the body reacts as it would to any foreign

substance, and develops antibodies that destroy theinvading antigen (this is called sensitization).

Rh incompatibility is when the mother lacks the Rh factoron the surface of her red blood cells and her baby isborn with the Rh factor on his or her red blood cells

It does not occur with the first born child

Increased destruction of red blood cells leads toincreased bilirubin in the blood; therefore, leading to

hyperbilirubinemia


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Clinical Presentation

Varies from mild jaundice andanemia to hydrops fetalis

Erythroblastosis fetalis: Thisoccurs as the baby's organs are

unable to handle the anemia. The

heart begins to fail and large

amounts of fluid build up in the

baby's tissues and organs. A fetus

with hydrops is at great risk of beingstillborn. RBC destruction and

anemia cause bone marrow to

release erythroblasts, hence the

name erythroblastosis fetalis)


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Risks during labor and delivery

include:

asphyxia and splenic rupture.

Postnatal problems include:

Pulmonary hypertension

Pallor (due to anemia) Edema (hydrops, due to low serum albumin)

Respiratory distress

Coagulopathies ( platelets & clotting factors)

Jaundice

Kernicterus (from hyperbilirubinemia)

Hypoglycemia (due to hyperinsulinemniafrom islet cell hyperplasia)


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Treatment for Rh Incompatibility

There is an injection called Rh immuneglobulin which is given to pregnantwomen at 28 weeks of pregnancy and

within 72 hours of delivering an infantwho is born Rh positive

This injection prevents the mothers bodyfrom forming antibodiesagainst the Rh

factor found on fetal red blood cells If the mother is already sensitized,

meaning her body has already madeantibodies against the Rh factor, the

injection will be ineffective


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ABO Blood Incompatibility

ABO incompatibility occurs with any blood type;

however, it is more common if the mother has type O

blood and the infant has blood type A or B

Fetal cells cross the placentaand enter the mothers

bloodstream When this occurs the mothers body forms antibodies

against the fetal cells

Those antibodies are then small enough to crossback through the placenta into the babys circulation

and cause destruction of red blood cells Increased destruction of red blood cells leads to

increased bilirubin in the blood; therefore, leading tohyperbilirubinemia


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Clinical Presentation

The first fetus can be affected.

IgG are the only antibodies that can

cross the placental barrier, and usually

most of the antibodies formed are IgM,

so this condition is usually much milder

than the Rh issue.

When delivered, infants may present withmild anemia or normal hemoglobin levels


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Clinical Features Of HemolyticDisease

Clinical Features Rh ABO

Frequency Unusual Common

Anemia Marked MinimalJaundice Marked Minimal to moderate

Hydrops Common Rare

Hepatosplenomegaly Marked Minimal

Kernicterus Common Rare


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Laboratory Features Of HemolyticDisease

Laboratory Features Rh ABO

blood type of Mother Rh negative Oblood type of Infant Rh positive A or B

Direct Commbs test Positive Negative

Indirect Commbs test Positive Usually positive

Hyperbilirubinemia marked Variable


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Coombs test

Positive

-RH

- ABO

-MINOR blood group

incompat

Negative

-Cephalhematoma

-Breast milk jaundice

-Infection-Asphyxia

-RDS

-Galactosemia

-Rare disorders


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Direct Coombs Test

The direct coombs test is a direct measure of

the amount of maternal antibodycoating theinfants red blood cell If the antibody is present,

the test is positive


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Indirect Coombs Test

The indirect coombs test measures the effect of asample of the infants serum (which is thought tocontain maternal antibodies) on unrelated adult RBCs

If the infants serum contains antibodies, they will

interact with and coat these adult RBCs (positive test)


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Glucose-6-Phosphate Dehydrogenase

G6PD

The function of G6PD enzyme is to initiate an

oxidation/reduction reaction. Enzyme to

maintenance of RBC life)

Oxidation is the loss of electrons and reduction

is the gain of electrons

which leads to hemolysisof red blood cells

Increased hemolysis of red blood cells leads toincreased levels of bilirubin, which then leads to

hyperbilirubinemia


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Clinical Manifestations

Usually no evidence of hemolysis is apparent until 48-

96 hours after the patient has ingested a substance

which has oxidant properties (Antipyretic,Sulfonamide,

Anti malarial producing an acute and severe hemolytic

syndrome called FAVISM, Hb level become very low,and increased reticulocyte count

- Jaundice

- Anemia

- Hydrops- Massive enlargement of the liver & spleen

- Bilirubin encephalopathy (Kernicterus)


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Kernicterus

Kernicterusis a rare, irreversible

complication of hyperbilirubinemia

If bilirubin levels become markedly

elevated, the unconjugated bilirubin may

cross into the blood brain barrierand

stain the brain tissues

If staining of the brain tissues occurs

there is permanent injury sustained to

areas of the brain which leads to

neurological damage


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Complication

Kernicterus:

Phase 1 ( 3-4 day): Hypotonia

Decreased alertness; Poor feeding

Diminished Moro reflex

High pitched cry

Phase 2 ( 1 wk): Hypertonia,

Irritability or Seizures; muscle spasms & back arching

Phase 3 ( by 3 yrs of age): Hypotonia


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G 6 P D deficiency

Diagnosis

Low G6PD activity in red blood cells

(normal range, 4.613.5 U/g Hb).Treatment

When hemolysis has occurred =>

Red blood cell transfusion

Prevention

Avoiding oxidant substances


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Jaundice-Diagnosis

Visual assessment (least reliable)

Check bilirubin level: Total bilirubin at birth is less than

3mg/dL.

Check complete blood count

Check reticulocyte count

Coombs test ( DAT).

Blood groups & types

G6PD level

Albuminlevel: A normal albumin level in a term infant is

between 2.6 - 3.6 g/dL


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Visual assessment (least reliable)

Skin yellowing

Not visible if bili


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Treatments

Intrauterine transfusion Early Delivery If labor is induced, fetal lung maturity must be

determined using the lecithin/sphingomyelon

(L/S) ratio to avoid respiratory distress syndrome Encourage frequent feedings Intravenous hydration

Intravenous immune globulin (IVIG) has been

used to decrease bilirubin levels due to hemolyticanemia; 1 gm/kg inhibits AB that cause RBCdestruction

Phototherapy

Exchange transfusion


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Maisels Chart

Sr Bilirubin

(mg/dl)

Birth

weight

Age in hrs

< 24 2448 4972 >72

2500gInvestigate if bilirubin

> 12mg%

15-19< 2500g

EXCHANGEConsider Exchange

> 2500g Phototherapy

20> AllEXCHANGE


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Phototherapy

The therapy uses a blue light (420-470 nm) that converts bilirubinso that it can be excreted in the urine and feces.

1 -Maximizing energy delivery :

- Distanceshould be no greater than 50 cm and may bereduced down to 10-20 cm if temperature homeostasis ismonitored to reduce the risk of overheating.

- Coverthe inside of the bassinet with reflecting material; whitelinen works well ( aluminum foil).

These simple expedients can multiplyenergy delivery by several fold.2- Maximizing the available surface area.

The infant should be nakedexcept for diapers and the eyes shouldbe covered to reduce risk of retinal damage.


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Phototherapy- Cont.

May be provided using lightweight, fiber-opticblankets ( bili blankets). The baby is wrappedin the blanket. The eyes are not covered.

A combination of a fiber-optic light source inthe mattress under the baby and a standardphototherapy light source above


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Nursing diagnoses:

Body temperature, risk for imbalancedrelated to use of phototherapy.

Risk for Fluid volume deficient related to

phototherapy.

Family processes, interrupted, related to

prolonged hospitalization of infant, or risk

for rehospitalization for therapy


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Jaundice

Nursing Assessment andDiagnosis

Identify the newborn at risk:assess for jaundice.

Monitor bilirubin levels.

Risk for Altered Parentingrelated to parenting anewborn with jaundice.

Risk for Injury related to use

of phototherapy. Fluid Volume Deficit related

to increased insensible waterloss and frequent loosestools.

Nursing Plan andImplementation

Monitor temperature forhyperthermia or

hypothermia. Apply eye patches over

newborns closed eyes.

Turn off lights when drawingblood for repeat bilirubin.

Maintaining a neutral thermalenvironment.

Observe for signs ofdehydration and perinealexcoriation.

Weigh daily

Nursing care of infant receiving


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Nursing care of infant receiving

phototherapy

Perform hand wash Obtain V/S including axillary temperature

Place baby naked in cradle or incubator except the diaper

Apply eye coverings

Monitor temp q 4hr

Fluids q2 hr to avoid dehydration

Change position q 2 hr

Weigh q12 hr, I &O, assess hydration, Weight diapers before discarding

Observe stools & urine for darkening, skin

Monitor bili levels Q 12 hr or daily, turn the phototherapy lights offwhile the blood is drawn.

Cluster care activities

Discontinue phototherapy & remove eye patches at least once per

shift. Also discontinue phototherapy and remove patches when

feeding the infant & when the parents visit.


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Nursing consideration

Accurate charting is important nursing responsibility andincludes:

1. Times that phototherapy is started and stopped.

2. Proper shielding of the eyes.

3. Type of fluorescent lamp.

4. Number of lamps.

5. Distance between surface of lamps and infant

6. Use of phototherapy in combination with an incubator oropen bassinet.

Occurrence of side effects .as: loose, greenish stools, transient

skin rashes, hyperthermia, increase metabolic rate,

dehydration, electrolyte disturbances as hypocalcemia,

Oxidize essential fatty acids, decreases vitamins and

calcium in premature infants lead to adverse effect on cell

growthTannin /Bronze Bab S ndrome


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Phototherapy Precautions

Ensure patent airway

Maintain constant body temperature by using incubator

Maintain fluid balance by increasing intake and minimizing loss

Assure skin integrity

frequent diaper changes ( perianal excoriation due to irritating

effect of diarrhea stools).

water baths

no lotions or oils on skin position to avoid skin irritation

Careful technique when repeatedly drawing labs

Warm foot before procedure

Avoid areas of previous puncture


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NEONATAL EXCHANGE

TRANSFUSIONS

Exchange transfusion is a critical therapeuticprocedure in babies with, or who are likely to develop,neurotoxic levels of bilirubin. Historically it was one ofthe most common procedures performed in neonatalservices. Three factors have led to exchangetransfusions becoming less commonly performed,namely:

1. The effective prevention of rhesus iso-immunisationwith prophylactic Anti D for rhesus negative mothers;

2. Foetal intrauterine transfusions; and,3. The recognition that well babies born at term withphysiological jaundice can tolerate higher levels ofbilirubin than previously thought


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Exchange Transfusion

An exchange transfusion is used only in extremecases when phototherapy has failed

The process for an exchange transfusion involves

small amounts of blood being removed from theinfant and then replaced with the same amount ofdonor RBCs and plasma

The exchange replaces ~ 87% of the circulatingblood volume and decreases the bilirubin level by

~ 55% Mechanism: removes bilirubin and antibodies

from circulation and correct anemia

M f E h


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Management of ExchangeTransfusion

NPO, aspirate stomach contents,

suction airway prior

Informed consent signed by parents

Check blood typing

Restrain infant & Place under radiant

warmer

Exchange transfusions are performed

using a one catheter or two catheter

push-pull method under a septic

technique

N.B: Blood Volume = 70-90 ml/kg forterm and 85-110 ml/kg for preterm

infants


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Two Catheter Push-pull Technique

Blood is removed from the artery while infusing fresh

blood through a vein at the same rate.

In Out

Umbilical vein Peripheral artery

OR Umbilical vein Umbilical artery

OR Peripheral vein Peripheral artery

OR Peripheral vein Umbilical artery


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One Catheter Push-pull Technique

This can be done through an umbilicalvenous catheter. Exceptionally, an umbilicalartery catheter can be used.

Ideally, the tip of the UVC should be in theIVC/right atrium (at or just above thediaphragm), position should be checked byan X-ray. Catheter is usually removed aftereach exchange.

Withdraw blood over 2 minutes, infuse slightlyfaster.

What type of blood to give


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CMV negative

Irradiated

Fresh Whole Blood(to avoid Ca++

),less than 7 days old

Group O, -negative (Maternal blood if

possible)

What type of blood to give

fetus:


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Exchange Therapy Complications

Air embolism

Vasospasm

Infarction or arrythmias Infection

Death

Thrombocytopenia


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Transfusion Reactions

Rare in neonates

Signs & symptoms in neonates

Temperature instability

Reaction at infusion site, flushing of skin, urticaria,rash

Vomiting or diarrhea

Wheezing or acute respiratory distress

Hypotension, shock, tachycardia

Sudden rise in bilirubin

Acute bleeding from venipunctures or surgical sites


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Transfusion Reactions

What to do if you suspect a transfusion

reaction

Stop the transfusion immediately Begin treatment for any new, emergent

problems

Notify the physician

Notify the blood bank

Blood bank may request a fresh blood

sample

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