IRMA tumbang 2

8/3/2019 IRMA tumbang 2

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Kramers Rule

The easiest (and most common) method a caregiver will use to monitor a

baby for jaundice levels is to observe their physical appearance and

behaviour. This is usually done on a daily basis.

Based on the caregiver gently pressing various areas of the baby's skin

with their fingertip.

The pressure 'blanches' the baby's skin (or momentarily shifts the blood

supply from under the skin) so that the caregiver can see how 'white' or

'yellow' the skin looks. The examination must be carried out in good

lighting conditions and may not be as effective with darker skinned

babies.

In essence the baby's body is categorised into 5 zones with an estimated

bilirubin level for each zone. If the jaundice is believed to be 200 to 250imol / litre or more then a blood test would be used to estimate the exact

bilirubin level.

ZoneBaby's body areaApprox. bilirubin level

1. Head and neck 100

mol/litre

2. Upper body (chest) 150

mol/litre

3. Lower body, below the belly button and upper thighs and arms

200 mol/litre

4. Lower legs and forearms 250

mol/litre

5. Hands and feet < 250

mol/litre


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Jaundice meter

The device has in most cases replaced (or complemented) the 'Kramer's

Rule' method and is aimed at preventing unnecessary blood tests for

jaundiced babies.

The jaundice meter is placed on the baby's forehead by the caregiver. The

caregiver then presses a button so that the meter emits a brief beam of

bright light onto the baby's skin. The reflection of the light beam is

interpreted as a measurement of the degree of 'yellowness' of the skin.

The meter displays a reading called a 'transcutaneous bilirubin index' or(TcBI).


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Jaundice Neonate

Definition

Jaundice is considered pathologic if it presents within the first 24

hours after birth, the total serum bilirubin level rises by more than 5

mg per dL (86 mol per L) per day or is higher than 17 mg per dL

(290 mol per L), or an infant has signs and symptoms suggestive of

serious illness.

Neonatal hyperbilirubinemia, defined as a total serum bilirubin level

above 5 mg per dL (86 mol per L).

Hyperbilirubinemia in the newborn period can be associated with

severe illnesses such as

hemolytic disease,

metabolic and endocrine disorders,

anatomic abnormalities of the liver, and

infections.

Jaundice typically results from the deposition of unconjugated

bilirubin pigment in the skin and mucus membranes.


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Risk Factor of Hyperbilirubinemia

Infants without identified risk factors rarely have total serumbilirubin levels above 12 mg per dL (205 mol per L). As thenumber of risk factors increases, the potential to develop

markedly elevated bilirubin levels also increases.

Common risk factors for hyperbilirubinemia include fetal-maternal blood group incompatibility, prematurity, and apreviously affected sibling. Cephalohematomas, bruising, andtrauma from instrumented delivery may increase the risk forserum bilirubin elevation. Delayed meconium passage alsoincreases the risk. Infants with risk factors should bemonitored closely during the first days to weeks of life.

TABLE 1Risk Factors for Hyperbilirubinemia in Newborns

Maternal factors

Blood type ABO or Rhincompatibility

Breastfeeding

Drugs: diazepam(Valium), oxytocin

(Pitocin)

Neonatal factors

Birth trauma: cephalohematoma, cutaneousbruising, instrumented delivery

Drugs: sulfisoxazole acetyl with erythromycinethylsuccinate (Pediazole), chloramphenicol(Chloromycetin)

Excessive weight loss after birth


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Ethnicity: Asian, NativeAmerican

Maternal illness:

gestational diabetes

Infections: TORCH

Infrequent feedings

Male gender

Polycythemia

Prematurity

Previous sibling with hyperbilirubinemia

TORCH = toxoplasmosis, other viruses, rubella,cytomegalovirus, herpes (simplex) viruses.

Bilirubin Production and Newborns

Bilirubin is the final product of heme degradation. At physiologic pH,bilirubin is insoluble in plasma and requires protein binding withalbumin. After conjugation in the liver, it is excreted in bile.

Newborns produce bilirubin at a rate of approximately 6 to 8 mg perkg per day. This is more than twice the production rate in adults,primarily because of relative polycythemia and increased red bloodcell turnover in neonates. Bilirubin production typically declines tothe adult level within 10 to 14 days after birth.

Bilirubin Toxicity

Kernicterus refers to the neurologic consequences of thedeposition of unconjugated bilirubin in brain tissue. Subsequentdamage and scarring of the basal ganglia and brainstem nuclei mayoccur.

If the serum unconjugated bilirubin level exceeds the bindingcapacity of albumin, unbound lipid-soluble bilirubin crosses theblood-brain barrier. Albumin-bound bilirubin may also cross theblood-brain barrier if damage has occurred because of asphyxia,acidosis, hypoxia, hypoperfusion, hyperosmolality, or sepsis in thenewborn.

Although the risk of bilirubin toxicity is probably negligible in ahealthy term newborn without hemolysis, the physician shouldbecome concerned if the bilirubin level is above 25 mg per dL (428 mol per L). In the term newborn with hemolysis, a bilirubin levelabove 20 mg per dL (342 mol per L) is a concern.

The effects of bilirubin toxicity are often devastating and

irreversible. Early signs of kernicterus are subtle and nonspecific,typically appearing three to four days after birth. However,


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hyperbilirubinemia may lead to kernicterus at any time during theneonatal period. After the first week of life, the affected newbornbegins to demonstrate late effects of bilirubin toxicity. If the infantsurvives the initial severe neurologic insult, chronic bilirubinencephalopathy (evident by three years of age) leads todevelopmental and motor delays, sensorineural deafness, and mildmental retardation.

TABLE 2

Effects of Bilirubin Toxicity in Newborns

Early Late Chronic

Lethargy Irritability Athetoid cerebral palsy

Poor feeding Opisthotonos High-frequency hearing loss

High-pitched cry Seizures Paralysis of upward gazeHypotonia Apnea Dental dysplasia

Oculogyric crisis Mild mental retardation

Hypertonia

Fever

Opisthotonos is a condition in which the body is held in an abnormalposition. The person is usually rigid and arches the back, with thehead thrown backward.

Oculogyric refers to rotating of the eye balls into afixed position, usually upward. Oculogyric crisis is adystonic reaction to neuroleptic drugs and/or medicalconditions.


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Classification of Neonatal Hyperbilirubinemia

The causes of neonatal hyperbilirubinemia can be classified intothree groups based on mechanism of accumulation: bilirubinoverproduction, decreased bilirubin conjugation, and impairedbilirubin excretion.

TABLE 3

Classification of Neonatal Hyperbilirubinemia Based on Mechanism of

Accumulation


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Increased bilirubin load

Decreased bilirubin

conjugation

Impaired bilirubin

excretion

Hemolytic causes

Characteristics: increasedunconjugated bilirubin level,

normal percentage ofreticulocytes

Physiologic jaundice

Crigler-Najjar syndrome types1 and 2

Gilbert syndrome

Hypothyroidism

Breast milk jaundice

Characteristics:increased unconjugatedand conjugated bilirubinlevel, negative Coombs'test, conjugated bilirubin

level of >2 mg per dL(34 mol per L) or >20%of total serum bilirubin

level, conjugatedbilirubin in urine

Biliary obstruction:biliary atresia,

choledochal cyst,primary sclerosing

cholangitis, gallstones,neoplasm, Dubin-

Johnson syndrome,Rotor's syndrome

Infection: sepsis, urinarytract infection, syphilis,

toxoplasmosis,tuberculosis, hepatitis,

rubella, herpes

Metabolic disorder:alpha1 antitrypsindeficiency, cystic

fibrosis, galactosemia,glycogen storage

disease, Gaucher'sdisease, hypothyroidism,

Wilson's disease,Niemann-Pick disease

Chromosomalabnormality: Turner'ssyndrome, trisomy 18

and 21 syndromes

Drugs: aspirin,acetaminophen, sulfa,

alcohol, rifampin(Rifadin), erythromycin,

corticosteroids,tetracycline

Characteristics:increased unconjugated

bilirubin level, >6percent reticulocytes,

hemoglobin

concentration of


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Physiologic Bilirubin

The average total serum bilirubin level usually peaks at 5 to 6 mgper dL (86 to 103 mol per L) on the third to fourth day of life andthen declines over the first week after birth.

Infants with multiple risk factors may develop an exaggerated formof physiologic jaundice in which the total serum bilirubin level mayrise as high as 17 mg per dL (291 mol per L).

Factors that contribute to the development of physiologichyperbilirubinemia in the neonate include an increased bilirubinload because of relative polycythemia, a shortened erythrocyte lifespan (80 days compared with the adult 120 days), immaturehepatic uptake and conjugation processes, and increasedenterohepatic circulation.

Jaundice And Breastfeeding

Early-Onset Breastfeeding Jaundice

Breast-fed newborns may be at increased risk for early-onsetexaggerated physiologic jaundice because of relative caloric

deprivation in the first few days of life. Decreased volume andfrequency of feedings may result in mild dehydration and thedelayed passage of meconium. Compared with formula-fednewborns, breastfed infants are three to six times more likely toexperience moderate jaundice (total serum bilirubin level above 12mg per dL) or severe jaundice (total serum bilirubin level above 15mg per dL).

In a breastfed newborn with early-onset hyperbilirubinemia, thefrequency of feedings needs to be increased to more than 10 perday. If the infant has a decline in weight gain, delayed stooling, and

continued poor caloric intake, formula supplementation may benecessary, but breastfeeding should be continued to maintainbreast milk production. Supplemental water or dextrose-wateradministration should be avoided, as it decreases breast milkproduction and places the newborn at risk for iatrogenichyponatremia.

Late-Onset Breast Milk Jaundice

Breast milk jaundice occurs later in the newborn period, with thebilirubin level usually peaking in the sixth to 14th days of life. Thislate-onset jaundice may develop in up to one third of healthy

breastfed infants. Total serum bilirubin levels vary from 12 to 20 mgper dL (340 mol per L) and are nonpathologic.


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Substances in maternal milk, such as -glucuronidases, andnonesterified fatty acids, may inhibit normal bilirubin metabolism.

The bilirubin level usually falls continually after the infant is twoweeks old, but it may remain persistently elevated for one to threemonths.

If the diagnosis of breast milk jaundice is in doubt or the total serumbilirubin level becomes markedly elevated, breastfeeding may betemporarily interrupted, although the mother should continue toexpress breast milk to maintain production. With formulasubstitution, the total serum bilirubin level should decline rapidlyover 48 hours (at a rate of 3 mg per dL [51 mol per L] per day),confirming the diagnosis. Breastfeeding may then be resumed.

Pathologic Jaundice

Features of pathologic jaundice include the appearance of jaundicewithin 24 hours after birth, a rapidly rising total serum bilirubinconcentration (increase of more than 5 mg per dL per day), and atotal serum bilirubin level higher than 17 mg per dL in a full-termnewborn. Other features of concern include prolonged jaundice,evidence of underlying illness, and elevation of the serumconjugated bilirubin level to greater than 2 mg per dL or more than20 percent of the total serum bilirubin concentration. Pathologiccauses include disorders such as sepsis, rubella, toxoplasmosis,occult hemorrhage, and erythroblastosis fetalis.

Diagnosis

PHYSICAL EXAMINATION

The presence of jaundice can be determined by examining theinfant in a well-lit room and blanching the skin with digital pressureto reveal the color of the skin and subcutaneous tissue. Neonatal

dermal icterus is not noticeable at total serum bilirubin levels below4 mg per dL (68 mol per L).

Increasing total serum bilirubin levels are accompanied by thecephalocaudal progression of dermal icterus, predictably from theface to the trunk and extremities, and finally to the palms and soles.

The total serum bilirubin level can be estimated clinically by thedegree of caudal extension: face, 5 mg per dL; upper chest, 10 mgper dL (171 mol per L); abdomen, 12 mg per dL; palms and soles,greater than 15 mg per dL.

The physical examination should focus on identifying one of the

known causes of pathologic jaundice. The infant should be assessed


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for pallor, petechiae, extravasated blood, excessive bruising,hepatosplenomegaly, weight loss, and evidence of dehydration.

Laboratory Evaluation

Management


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The management of the jaundiced infant is determined according to the infants age in hours

and by the total level of serum bilirubin:

Age (hrs)

Consider

Phototherap

y

Phototherap

y

Exchange

Transfusion if

Intensive

Phototherapy

Fails

Exchange

Transfusion and

Intensive

Phototherapy

SBR

(micromol/L)

SBR

(micromol/L)

SBR

(micromol/L)

SBR

(micromol/L)

200 > 260 > 340 > 430

49 - 72 hours > 260 > 310 > 430 > 510

> 72 hours > 290 > 340 > 430 > 510


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