APPROACH TO A CHILD WITH JAUNDICE
S.BALASANKAR
2009 MBBS
APPROACH TO A CHILD WITH JAUNDICE
S.BALASANKAR2009 MBBS
JAUNDICE
Jaundice is the visible manifestation of increased level of bilirubin in the body.
It is not a disease rather a symptom of diseases.
In adults sclera appears jaundiced when serum bilirubin exceeds 2 mg/dl.
However it is difficult to see sclera in newborn due to difficulty in opening eye. But in new born it is very easy to see jaundice in skin.
BURDEN
Important problem in the 1st week of life.Almost all neonates (60% Term and 80%
Preterm) will have bilirubin > 5 mg/dl in the 1st week of life and become visibly jaundiced, vast majority being benign
Some of the term babies (8 to 9%) have levels exceeding 15 mg/dl in 1st 7 days of life.
High bilirubin level is toxic to the developing CNS.(KERNICTERUS; Bilirubin≥25mg/dl)
BILIRUBINEnd product of hemoglobin metabolism
that is excreted in bile.In neonates -75% : from catabolism of circulating
RBCs -25% :*from ineffective erythropoiesis (bone marrow) *from turnover of heme
proteins & free heme( liver).
Hyperbilirubinemia -Direct( Conjugated) -Indirect( Un-conjugated)
Conjugated Hyperbilirubinemia is present, * >20% of total bilirubin is conjugated * >2mg/dl is conjugated
If neither criteria is met, hyperbilirubinemia is classified as Un-conjugated.
NEONATAL JAUNDICE
Jaundice after 1 week:
a) Prolonged direct Jaundice› Neonatal hepatitis (common)› Extra-hepatic biliary atresia› Breast milk jaundice› Metabolic disorders› Intra-hepatic biliary atresia› Amino acid toxicity› Inspissated bile syndrome
(uncommon)
b) Prolonged Indirect Jaundice
> Criggler Najjar Syndrome> Breast milk jaundice> Hypothyroidism> Pyloric stenosis> Ongoing hemolysis, malaria
CLINICAL CLASSIFICATION:
PHYSIOLOGICAL JAUNDICE
PATHOLOGICAL JAUNDICE
PHYSIOLOGICAL JAUNDICE:
• In new born babies bilirubin metabolism is immature which results in the occurrence of hyperbilirubinemia in the first few days of life. Also there is increased bilirubin load on the hepatic cell due to physiological polycythemia.
Immaturity could be at various steps of bilirubin metabolism like:
Defective uptake from plasma into liver cell( deficiency of LIGANDIN)
Defective conjugation( UDP-glucoronosyl transferase: <1% of adult activity during the first 10 days of life)
Decreased excretion Increased entero-hepatic circulation
Increased Bilirubin production:
Larger circulating red blood cell volume
Shortened RBC lifespan (70-90 days vs 120 days in adult)
Substantial production from other sources
Characteristics:
First appears between 24-72 hours of age
Maximum intensity seen on 4-5th day in term and 7th day in preterm neonates
Does not exceed 15 mg/dlClinically undetectable after 14 days.No treatment is required but baby
should be observed closely for signs of worsening jaundice.
PATHOLOGICAL JAUNDICEPresence of any of the following signs denotes that the jaundice is pathological.
Clinical jaundice detected before 24 hours of age
Rise in serum total bilirubin by more than 5 mg/dl/ day (>5mg/dl on first day , 10 mg/dl on second day and 12- 13 mg/dl thereafter in term babies)
Serum bilirubin more than 15 mg/dlClinical jaundice persisting beyond 14
days of lifeClay/white colored stool and/or dark
urine staining the nappy yellowDirect bilirubin >2 mg/dl at any time.
Treatment is required in the form of phototherapy or exchange blood transfusion. One should investigate to find the cause of pathological jaundice.
It can be
Overproduction Hyperbilirubinemia
Under-secretion Hyperbilirubinemia
Overproduction Hyperbilirubinemia:
Blood group incompatibilitiesMaternal-fetal or feto-fetal transfusionsNon Immune Hemolytic anemiasStructurally Abnormal Red cellsExtra-vascular Hemolysis
Blood Group Incompatibilities:
Rh negative mother & Rh positive infant
ABO incompatibilities
Strongly considered if there is jaundice in the first 24 hours of life
Non-Immune Hemolytic Anemias:
1. G6PD Deficiency: Deficiency-decreased NADPH-
decreased reduced Glutathione –decreased protection of RBCs from oxidants-hemolysis.
2. Excess of Vitamin K given IM
Structurally Abnormal RBCs:
Spherocytosis
Pyknocytes ( irregular borders)
Under-secretion Hyperbilirubinemia:
Enzymatic Deficiency( Glucoronyl transferase)
Hormonal suppression (Breast milk jaundice)
Inhibition of conjugationHepatic cell injury due to InfectionsSubstrate deficiency (hypoglycemia)Mechanical obstruction (biliary atresia)
Hormonal Suppression:
Pregnandiol present in maternal breast milk suppresses bilirubin conjugation.
Breast feeding may be stopped and restarted in a period of 48hours.
Thyroxine Deficiency:
Thyroxine increases the activity of Glucoronyl transferase which promotes conjugation of bilirubin.
Inhibition of Conjugation:
Sulfonamides and Vitamin K results in competitive conjugation inhibition of bilirubin.
GALACTOSEMIA: Absent or deficient Galactose 1-
phosphoate uridyl transferase which is needed in glucoronidaton of indirect bilirubin.
APPROACH
HISTORYMaternal and Perinatal History:Delivery at period of gestation,
Postnatal age in hours.Maternal illness during pregnancy which
also includes diabetes; drug use.Previous history of malaria.Traumatic delivery, delayed cord
clamping, oxytocin use.Birth asphyxia, delayed feeding, delay
in meconium passage.
Family history of jaundice, liver disease
Previous sibling with jaundice for blood group incompatibility.
Kernicterus: Lethargy, poor feeding, and hypotonia. Some advanced signs are seizures, retrocollis, paralysis of upward gaze and shrill cry.
Breast feeding.
ON EXAMINATION:Baby lethargic, poor feeding, temperature instability, with apnea: Sepsis
Small for gestation: polycythemia
Cataract, rash: TORCH infections
Extra vascular bleed: Cephalhematoma
Pallor: hemolysis, blood loss
Petechiae: sepsis, TORCH infections.
Hepatosplenomegaly: Rh-isoimmunization, sepsis, TORCH infections.
Dysmorphic features, congenital heart disease (pulmonary stenosis), Intra-hepatic biliary atresia :Alagille syndrome
HOW DO YOU LOOK FOR ICTERUS?
1. Dermal staining :progresses from head to toeExamined in good day light skin of
forehead, chest, abdomen, thigh, legs, palms, and soles.
Blanched with digital pressure and the underlying color of the skin and subcutaneous tissue should be noted.
2. Trans-cutaneous bilirubinometer .
Clinical Jaundice
Measure Bilirubin
> 12 mg/dl and infant < 24 hr old
Fraction of Bilirubin
Indirect
Direct
< 12 mg/dl and infant > 24 hr old
Follow bilirubin level
Direct bilirubin
Evaluate for treatable causes 1.Infections-blood, urine culture, VDRL. 2.Metabolic disorders-urine reducing substances, serum amino acids,T4,TSH.If no abnormality ;screen for Identifiable causes *α-1 antitrypsin deficiency *cystic fibrosis *congenital viral infectionsIf no abnormality; evaluate for anatomical abnormalities *Stool color *USG *Hepato-biliary scintigraphy *Liver biopsy
Indirect
High(>65%)Polycythemia
Hematocrit
Low/Normal
Reticulocyte count
Blood TypeCoomb’s test
PlateletsEvaluate smear
Normal Reticulocyte countLook for evidence of,
*Infections-blood, urine culture*Enclosed hemorrhage
*Congestive cardiac failure*Hypoxia
*Hypothyroidism*Drugs/toxins
None found
Familial Disorders*Criggler Najjar
*Gilbert
Elevated Reticulocyte Count
Consider:1.Iso-immunization
2.Erythrocyte membrane defect
3.Erythrocyte enzyme defect
MANAGEMENT
PHYSIOLOGICAL JAUNDICE
Explain about benign nature of the disease
Encourage to breastfeed frequently & exclusively
Ask Mother to bring baby back if baby looks deep yellow or palms & soles have yellow staining.
PATHOLOGICAL JAUNDICE
Mainly 2 modalities of Treatment:
PHOTOTHERAPY
EXCHANGE TRANSFUSION
PHOTOTHERAPY
Mainstay of treatmentUnder blue-green light(460-490nm),
insoluble bilirubin is converted into soluble isomers that can be excreted in urine & feces.
To be effective, bilirubin must be present in skin; hence nor role for prophylactic phototherapy
Term & near term neonates:
The American Academy of Pediatrics (AAP) has laid down criteria for managing babies with elevated serum bilirubin based on gestational age and other risk factors( hemolysis , asphyxia, low albumin level, hypothermia)
MECHANISMS:
CONFIGURATIONAL ISOMERIZATION
STRUCTURAL ISOMERIZATION
PHOTO OXIDATION
Configurational Isomerization
Z-isomer converted to E-isomerReaction is instantaneous but
reversible.After exposure of 8-12 hrs, this
constitutes about 25% of the TSB which is non-toxic.
Excreted slowly from the body; hence not a major mechanism for decrease in TSB.
Structural Isomerization
Irreversible reactionBilirubin is converted into Lumirubin.This product forms 2-6% of TSB which
is rapidly excreted from body thus is mainly responsible for phototherapy induced decline in TSB.
Photo oxidation
Minor reaction
Administering Phototherapy:
Optimum ambient room temperature( 25-28celcius) to prevent hypothermia.
Remove all clothes of baby except diaper
Cover baby’s eyes with an eye patch(to prevent retinal degeneration) ensuring that it does not block the baby’s nostrils.
Place the baby under the lights in a cot if weight is more than 2kg or in an incubator if baby is small(<2kg)
Keep the distance between the baby & the light 30-45cm.
Ensure optimum breastfeeding as intermittent feeding sessions.
Monitor temperature of the baby every 2-4hrs
Measure TSB every 12-24hrs.Discontinue, once 2 TSB values 12hr
apart fall below current age-specific cut-offs.
Monitor for rebound bilirubin rise within 24hrs.
Complications of Phototherapy:Loose stoolsErythematous macular rashPurpuric rash associated with transient
porphyrinemiaOver-heatingDehydrationBronze baby syndrome
Bronze Baby Syndrome
Intense grey-brown discoloration of the skin, serum, and urine, especially in premature infants; when phototherapy was used to reduce hyperbilirubinemia. Pre-existing hepatic disease is suspected as a cause of the jaundice and may have prevented the biliary excretion of the photo oxidation products of bilirubin; their retention resulted in the bronze discoloration.
EXCHANGE TRANSFUSION
Double Volume Exchange Transfusion (DVET) : 160-180ml/kg; is to be performed if TSB levels reach age-specific cut-offs or if the infant shows signs of bilirubin encephalopathy, irrespective of TSB levels.
If baby shows signs of cardiac decompensation at birth, partial exchange transfusion with 50ml/kg of packed red cells should be done to quickly restore oxygen carrying capacity of blood.
Umbilical venous route.
FOLLOWUP
Babies with serum bilirubin>20 mg/dl & those who required ET should be kept under follow-up in high-risk clinic for neuro-developmental outcome.
Hearing assessment should be done at 3months of age.
PREVENTIONAnte-natal screening to detect Rh iso-
immunization & prompt administration of Anti D after first obstetric event.
Ensure adequate breast feeding.Educate parent about danger signs to
ensure immediate checkup.Follow-up high risk babies( large
cephalo-hematoma, family history of jaundice) for 2-3 days of discharge.
JAUNDICE IN THE CHILD & ADOLESCENT
HISTORY:Age at onset of symptoms. E.g.:
Wilson’s disease commonly manifests in pre-adolescents & adolescents.
Past/present use of any drugsH/o of blood transfusion/ dialysisExposure to viral hepatitisAny h/o of chronic illness;
hemoglobinopathies.Family h/o of inheritable disorders;
Wilson’s.
DDs of Un-conjugated Hyperbilirubinemia:Auto-immune hemolytic anemiasDrug induced hemolytic anemiasErythrocyte membrane defectsErythrocyte enzyme defectsHemoglobinopathiesCongestive cardiac failureSepsisGilbert syndrome.
DDs of Conjugated Hyperbilirubinemia:1. Obstructive Gall stones Primary sclerosing cholangitis Choledochal cyst Tumors2. Infections Hepatitis A, B, C, D, E EBV,CMV Varicella zoster HIV Leptospirosis
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