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Jaundice is the yellow discolouration of the skin due to an elevated level of unconjugated bilirubin.
Approximately 50-60% of term infants will develop physiological jaundice within the first week of life, some of whom will require investigation and treatment.
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The total serum bilirubin level should be used to determine management decisions in cases of predominantly unconjugated hyperbilirubinaemia. The serum albumin level does not need to be measured in addition to the bilirubin to determine management.
A smaller number of infants may develop unconjugated hyperbilirubinaemia secondary to a pathological cause and these infants more frequently require treatment.
Jaundice in the first 48 hours of life should be considered to be from a pathological cause until proven otherwise.
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It is important to recognise infants with pathological jaundice early in order to:
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1. Prevent acute bilirubin encephalopathy and subsequent kernicterus.
2. Prevent severe hyperbilirubinaemia (> 340 micromol/L).
3. Prevent an exchange transfusion.
4. Minimise separation of mother and baby
5. Support breastfeeding.
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Why jaundice occurs
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Neonatal red blood cells have a short lifespan and so are more rapidly broken down. The infants’ haemoglobin level falls in the first few days of life, as a normal adaptation to extrauterine life, and hence produces a greater bilirubin load.
Unconjugated bilirubin is normally taken up by the liver and conjugated by glucuronyl transferase. These processes are less active in the newborn period and are the major contributing factor to jaundice in the newborn. The excretion of bilirubin is affected by method of feeding, intestinal motility and an increase in the enterohepatic circulation.
A number of other pathological conditions can make the bilirubin level higher than normal in the newborn period. These need to be considered and investigated in cases of early (<48 hours), prolonged (>7 days) or conjugated hyperbilirubinaemia.
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Risk Factors
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Risk factors for developing severe hyperbilirubinaemia (> 340 micromol/L)
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Major risk factors include:
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- Jaundice within the first 48 hours
- Blood group incompatibility
- Previous sibling requiring phototherapy for haemolytic disease
- Cephalhaematoma or significant bruising
- Weight loss greater than 10% of birth weight; may be associated with ineffective breast-feeding
- Family history of red cell enzyme defects
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Minor risk factors include:
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- Jaundice occurring before discharge
- Previous sibling requiring phototherapy
- Macrosomic infant of a diabetic mother
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Risks of jaundice
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There is risk of the baby developing acute bilirubin encephalopathy if the level of unconjugated bilirubin rises too rapidly or if it reaches a very high level (340-430 micromol/L) and is not treated adequately.
The clinical features of acute bilirubin encephalopathy include: poor feeding, lethargy, abnormal posture, irritability, opisthotonus and seizures. This is termed kernicterus (bilirubin staining and toxicity to the basal ganglia and thalami).
The long term consequences of acute bilirubin encephalopathy and kernicterus include athetoid cerebral palsy, sensorineural deafness, seizures and cognitive impairment.
Many describe a transient change in infant behaviour, even if the bilirubin level does not reach the level for an exchange transfusion. This correlates with a measurable transient alteration in brainstem evoked potentials.
It is recommended that infants with a bilirubin level >340 micromol/L, or those who received an exchange transfusion have auditory brainstem evoked potentials measured following discharge. All other infants should have routine hearing screening.
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Assessment of the jaundiced infant
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Jaundice should always be assessed in natural light.
Jaundice may only be clinically visible in the newborn infant when the serum level of bilirubin is >100 micromol/L, or even higher in dark-skinned or very plethoric babies.
There is a normal progression of the depth of jaundice from head to toe as the level of bilirubin rises. Kramer described the approximate serum bilirubin level with the level of skin discolouration:
Visual inspection of the infant, including Kramer’s rule, can only be used as a guide to the level of jaundice. There is a wide inter-observer error in the clinical estimation of the depth of jaundice which should therefore not be substituted for a formal bilirubin measurement in equivocal cases.
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Kramer’s Rule
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Transcutaneous bilirubin meters have been used to assess the level of jaundice with some degree of reliability. They are not used at the Royal Women’s Hospital because the results are too inaccurate to base management decisions upon and the results vary considerably depending on the population studied.
Healthy term infants discharged from the Royal Women’s Hospital (the Women's) will be reviewed at home by Hospital in the Home or by the Domiciliary Team. If there are major risk factors for jaundice, minimal assessment will include home review within 24 hours of discharge, and then at 3-5 days of age. Blood collected at home by DOM or HITH should be protected from light by covering the sample with foil during transportation to the Women's. We aim for the sample to reach Pathology within 4 hours of collection.
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Management
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The aim of the treatment of jaundice is to prevent acute bilirubin encephalopathy and the long-term complication of kernicterus.
Parents should be made aware of and provided with the Jaundice and your newborn baby information sheet during preparation for discharge from the Women's.
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The management of the jaundiced infant is determined according to the infant’s age in hours and by the total level of serum bilirubin:
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| | | Exchange
Transfusion if
Intensive
Phototherapy
Fails
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| Exchange
Transfusion and
Intensive
Phototherapy
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| THIS IS A NEONATAL EMERGENCY
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In healthy term infants the bilirubin level does not need adjusting for the serum albumin level.
The serum bilirubin level from a capillary sample is assumed to be the same as that from a venous sample.
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Exchange transfusion
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Refer to the Women's Policy & Procedure Manual: Exchange Transfusion (total & partial) (Intranet only access).
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Very few healthy term infants will require an exchange transfusion in addition to phototherapy. Most are performed for infants with pathological jaundice from ABO incompatibility and less often Rhesus isoimmunisation or other blood group incompatibilities.
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Phototherapy
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Refer to the Women's Policy & Procedure Manual: Phototherapy (Intranet only access).
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Jaundice is treated using a phototherapy light which photo-isomerises bilirubin into a form that is water soluble, and more readily excreted in urine and faeces.
Phototherapy may be delivered by overhead light or by a fibreoptic device (Biliblanket). Older fibreoptic devices may be less effective at reducing the bilirubin level in uncomplicated jaundice in term infants compared with overhead lights which may increase the time needed for phototherapy. The Royal Women’s Hospital currently uses fibreoptic devices in addition to overhead units in severe jaundice. A single overhead light is usually sufficient for most infants with jaundice.
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Consumer fact sheet: Jaundice and your newborn baby
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Complications of phototherapy
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Symptoms that may be of consequence include: overheating, water loss, diarrhoea, rash and potential retinal damage.
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Fluids
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Enteral feeding, particularly breastfeeding, should be maintained and supported during phototherapy. Only in exceptional circumstances, when the need for or an actual exchange transfusion is imminent, would enteral feeds be withheld.
Phototherapy may increase insensible water loss, but there is no evidence to support the administration of additional intravenous fluids to jaundiced infants. A feeding plan that includes effective and more frequent breast feeding and/or expressing with administration of EBM should be considered.
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Investigations and specific management
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All infants, irrespective of the age at presentation, should be assessed for and if necessary investigated and treated for sepsis.
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1. Infants with unconjugated jaundice occurring in the first 24 hours of age
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These infants should be treated as a medical emergency due to the risk of developing acute bilirubin encephalopathy and need to avoid an exchange transfusion. The aetiology is always pathological.
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Infants with jaundice in the first 24 hours require immediate paediatric assessment and admission to the nurseries at the Royal Women’s Hospital for investigation and treatment. An infant in the community should be taken immediately to RWH emergency for assessment by the paediatric HMO.
Blood should be taken immediately for: urgent serum bilirubin, full blood count and film, blood group and direct antiglobulin test. The maternal blood group and antibody titres should be taken if not already known.
‘Triple’ phototherapy – two overhead lights and one fibreoptic unit - should be commenced immediately. The bilirubin should be re-checked four hours after starting phototherapy to re-assess the need for an exchange transfusion.
Infants with pathological causes of jaundice, particularly ABO and Rhesus incompatibility, may require more than a week of phototherapy due to ongoing haemolysis and the parents should be informed of this.
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Link to algorithm: Jaundiced healthy term infant <48 hours of age
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2. Infants with unconjugated jaundice occurring between 24 and 48 hours of age
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These infants need to have prompt paediatric assessment as the aetiology of the jaundice may be pathological.
For infants at home, the domiciliary midwife should refer the baby to RWH emergency for assessment by the paediatric HMO. Infants on the postnatal ward should be referred to the paediatric HMO.
Blood should be requested for: bilirubin, blood group and direct antiglobulin test.
Phototherapy should be commenced depending on the age of the infant, in hours, and the level of bilirubin as per the chart above. Infants who have previously been discharged and require phototherapy should be readmitted to the postnatal ward. They should continue with the method of feeding already initiated and only come out of phototherapy for feeding and changing.
The bilirubin should be rechecked 6-12 hours following starting phototherapy.
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Link to algorithm: Jaundiced healthy term infant <48 hours of age
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3. Infants with unconjugated jaundice occurring between 48 hours and 7 days of age
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The level of bilirubin in physiological jaundice typically peaks at 3-5 days of age as the newborn infant undergoes ex-utero adaptation.
For infants already discharged from hospital, the level of jaundice should be assessed in natural light using Kramer’s rule. A serum bilirubin should be taken if the jaundice extends to zones 4 or 5, if there is a significant change in depth of jaundice from a previous observation, or if there is a significant change in the infant’s behaviour (alertness; frequency, effort and duration of feeding) or condition (weight, urine output, stool frequency and color).
Most infants with uncomplicated physiological jaundice managed with phototherapy may require a few days of treatment.
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Link to algorithm: Jaundiced healthy term infant between 48 hours and 7 days of age
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4. Prolonged unconjugated jaundice occurring beyond 7 days
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5% of breast-fed infants will continue to be jaundiced beyond the first week of life. Breast milk jaundice is a diagnosis of exclusion, and should not lead to the cessation of breastfeeding.
Investigation of prolonged jaundice should be considered if (1) the level of jaundice is not improving or worsening or (2) if the jaundice has become apparent after the first week of life or (3) the conjugated bilirubin level is increasing.
Infants should be investigated for:
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- Adequate feed intake – dehydration, fewer wet nappies, weight loss >10% of birthweight
- Bacterial infection – especially of the urinary tract
- Hypothyroidism
- Haemolytic conditions – red cell enzyme (e.g. G6PD) and membrane (e.g. hereditary spherocytosis) defects, ABO incompatibility
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5. Conjugated hyperbilirubinaemia
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This is defined in relation to the total level of bilirubin:
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- If the total bilirubin is <85 micromol/L and the conjugated bilirubin level is >17 micromol/L
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- Or
- If the total bilirubin level is >85 micromol/L and the conjugated fraction is >20% of the total
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Conjugated jaundice can present at any age but rarely occurs in the first 24 hours of age.
Identifying conjugated hyperbilirubinaemia in a previously healthy term infant is important to exclude conditions in which early treatment improves the infant’s outcome:
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- Biliary atresia
- Metabolic syndromes
- Hepatitis or liver failure
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The following links outline the first-line tests to identify a cause:
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- Royal Children's Hospital Clinical Practice Guideline: Jaundice in Early Pregnancy
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- Newborn Services Clinical Guideline (National Women's, New Zealand): Conjugated Hyperbilirubinaemia
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Monitoring jaundice
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Visual inspection of the infant can only be used as a guide to the level of jaundice. In infants who have been treated with phototherapy, exposed areas of skin cannot be subsequently used to determine the level of jaundice and so repeated bilirubin measurements are required.
The frequency of monitoring the serum bilirubin should be determined by the condition of the infant, the level of bilirubin in relation to the infant’s age and the need for phototherapy or exchange transfusion.
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Algorithms
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References
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References: Jaundice (hyperbilirubinaemia) in the healthy term infant on the postnatal ward or in the community
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Published: 20 December 2007
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Royal Women's Hospital Clinical Practice Guidelines (CPGs) are intended to provide guidance to health care professionals, based on a thorough evaluation of research evidence, on the practical assessment and management of specific clinical issues or situations. The guidelines allow some flexibility on the part of the health care professional based on the needs of the specific patient for whom they are caring.
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