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8/11/2019 approachtoachildwithjaundice2-131209103904-phpapp02

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APPROACH TO A CHILD

WITH JAUNDICE

S.BALASANKAR

2009 MBBS

PPRO CH TO CHILD

WITH J UNDICE

S.BALASANKAR

2009 MBBS


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JAUNDICE

Jaundice is the visible manifestation of increasedlevel 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 newborndue to difficulty in opening eye. But in new bornit is very easy to see jaundice in skin.


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BURDEN

Important problem in the 1 st week of life.Almost all neonates (60% Term and 80% Preterm)will have bilirubin > 5 mg/dl in the 1 st week oflife and become visibly jaundiced, vast majority being benignSome of the term babies (8 to 9%) have levelsexceeding 15 mg/dl in 1 st 7 days of life.High bilirubin level is toxic to the developingCNS.( KERNICTERUS; Bilirubin 25 mg/dl )


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BILIRUBIN

End product of hemoglobin metabolism that isexcreted in bile.In neonates

-75% : from catabolism of circulating RBCs-25% :*from ineffective erythropoiesis

(bone marrow)*from turnover of heme proteins &free heme( liver).


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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 isclassified as Un-conjugated.


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NEONATAL JAUNDICE


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b) Prolonged Indirect Jaundice

> Criggler Najjar Syndrome> Breast milk jaundice

> Hypothyroidism> Pyloric stenosis> Ongoing hemolysis, malaria


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CLINICAL CLASSIFICATION:

PHYSIOLOGICAL JAUNDICE

PATHOLOGICAL JAUNDICE


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PHYSIOLOGICAL JAUNDICE:

In new born babies bilirubinmetabolism is immature which results in theoccurrence of hyperbilirubinemia in the first fewdays of life. Also there is increased bilirubin loadon the hepatic cell due to physiological

polycythemia.


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Immaturity could be at various steps of bilirubinmetabolism like:

Defective uptake from plasma into liver cell(deficiency of LIGANDIN)Defective conjugation( UDP-glucoronosyltransferase:


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Characteristics:

First appears between hours of ageMaximum intensity seen on 4-5th day in term

and 7th day in preterm neonatesDoes not exceed 15 mg/dlClinically undetectable after 14 days.

No treatment is required but baby should beobserved closely for signs of worsening jaundice.


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Presence of any of the following signs denotes thatthe jaundice is pathological.

Clinical jaundice detected before 24 hours ofageRise in serum total bilirubin by more than 5

mg/dl/ day (>5mg/dl on first day , 10 mg/dl onsecond day and 12- 13 mg/dl thereafter in term babies)


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Serum bilirubin more than 15 mg/dlClinical jaundice persisting beyond 14 days oflifeClay/white colored stool and/or dark urinestaining the nappy yellowDirect bilirubin >2 mg/dl at any time.

Treatment is required in the form of phototherapy or exchange blood transfusion. Oneshould investigate to find the cause of pathological jaundice.


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It can be

Overproduction Hyperbilirubinemia

Under-secretion Hyperbilirubinemia


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Overproduction Hyperbilirubinemia :

Blood group incompatibilitiesMaternal-fetal or feto-fetal transfusions Non Immune Hemolytic anemiasStructurally Abnormal Red cells

Extra-vascular Hemolysis


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Blood Group Incompatibilities:

Rh negative mother & Rh positive infant

ABO incompatibilities

Strongly considered if there is jaundice in thefirst 24 hours of life


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Non-Immune Hemolytic Anemias:

1. G6PD Deficiency:Deficiency-decreased NADPH- decreased

reduced Glutathione decreased protection ofRBCs from oxidants-hemolysis.

2. Excess of Vitamin K given IM


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Structurally Abnormal RBCs:

Spherocytosis

Pyknocytes ( irregular borders)


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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)


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Hormonal Suppression:

Pregnandiol present in maternal breast milksuppresses bilirubin conjugation.Breast feeding may be stopped and restarted in a period of 48hours.


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Thyroxine Deficiency:

Thyroxine increases the activity of Glucoronyl

transferase which promotes conjugation of bilirubin.


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Inhibition of Conjugation:

Sulfonamides and Vitamin K results incompetitive conjugation inhibition of bilirubin.

GALACTOSEMIA:Absent or deficient Galactose 1-phosphoate

uridyl transferase which is needed inglucoronidaton of indirect bilirubin.


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APPROACH


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HISTORY

Maternal and Perinatal History:Delivery at period of gestation, Postnatal age inhours.

Maternal illness during pregnancy which alsoincludes diabetes; drug use.Previous history of malaria.Traumatic delivery, delayed cord clamping,oxytocin use.Birth asphyxia, delayed feeding, delay inmeconium passage.


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Family history of jaundice, liver disease

Previous sibling with jaundice for blood groupincompatibility.Kernicterus: Lethargy, poor feeding, andhypotonia. Some advanced signs are seizures,retrocollis, paralysis of upward gaze and shrillcry.Breast feeding.


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ON EXAMINATION:

Baby lethargic, poor feeding, temperatureinstability, with apnea: Sepsis

Small for gestation: polycythemia

Cataract, rash: TORCH infections

Extra vascular bleed: Cephalhematoma

Pallor: hemolysis, blood loss


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Petechiae: sepsis, TORCH infections.

Hepatosplenomegaly: Rh-isoimmunization,sepsis, TORCH infections.

Dysmorphic features, congenital heart disease(pulmonary stenosis), Intra-hepatic biliary atresia: Alagille syndrome


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HOW DO YOU LOOK FORICTERUS?

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 theunderlying color of the skin and subcutaneous

tissue should be noted.

2. Trans-cutaneous bilirubinometer .


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Clinical Jaundice

> 12 mg/dl and infant < 24 hrold

Fraction of Bilirubin

Indirect Direct

< 12 mg/dl and infant > 24 hrold

Follow bilirubin level

Measure Bilirubin


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Indirect

High(>65%)Polycythemia

Hematocrit

Low/Normal

Reticulocyte countBlood Type

Coombs test Platelets

Evaluate smear


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Normal Reticulocyte count

Look 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 defect3.Erythrocyte enzyme defect


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MANAGEMENT


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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 yellowstaining.


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Mainly 2 modalities of Treatment:

PHOTOTHERAPY

EXCHANGE TRANSFUSION


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Term near term neonates:

The American Academy of Pediatrics (AAP) has

laid down criteria for managing babies withelevated serum bilirubin based on gestationalage and other risk factors( hemolysis ,asphyxia, low albumin level, hypothermia)


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MECHANISMS:

CONFIGURATIONAL ISOMERIZATION

STRUCTURAL ISOMERIZATION

PHOTO OXIDATION


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Configurational Isomerization

Z-isomer converted to E-isomerReaction is instantaneous but reversible.

After exposure of 8-12 hrs, this constitutes about25% of the TSB which is non-toxic.Excreted slowly from the body; hence not a

major mechanism for decrease in TSB.


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Photo oxidation

Minor reaction


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Administering Phototherapy:

Optimum ambient room temperature( 25-28celcius) to prevent hypothermia.Remove all clothes of baby except diaperCover babys eyes with an eye patch(to preventretinal degeneration) ensuring that it does not block the babys nostrils.

Place the baby under the lights in a cot if weightis more than 2kg or in an incubator if baby issmall(


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Bronze Baby Syndrome

Intense grey-brown discoloration ofthe skin, serum, and urine, especiallyin premature infants; when phototherapy was used to reduce

hyperbilirubinemia. Pre-existinghepatic disease is suspected as acause of the jaundice and may have prevented the biliary excretion of the photo oxidation products of bilirubin; their retention resulted inthe bronze discoloration.


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FOLLOWUP

Babies with serum bilirubin>20 mg/dl & thosewho required ET should be kept under follow-upin high-risk clinic for neuro-developmentaloutcome.Hearing assessment should be done at 3monthsof age.


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PREVENTION

Ante-natal screening to detect Rh iso-immunization & prompt administration of Anti Dafter first obstetric event.

Ensure adequate breast feeding.Educate parent about danger signs to ensureimmediate checkup.

Follow-up high risk babies( large cephalo-hematoma, family history of jaundice) for 2-3days of discharge.

JAUNDICE IN THE CHILD


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JAUNDICE IN THE CHILDADOLESCENT

HISTORY:Age at onset of symptoms. E.g.: Wilsons diseasecommonly manifests in pre-adolescents &

adolescents.Past/present use of any drugsH/o of blood transfusion/ dialysis

Exposure to viral hepatitisAny h/o of chronic illness; hemoglobinopathies.Family h/o of inheritable disorders; Wilsons.

DDs of Un conjugated


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DDs of Un-conjugatedHyperbilirubinemia:

Auto-immune hemolytic anemiasDrug induced hemolytic anemias

Erythrocyte membrane defects

Erythrocyte enzyme defects

Hemoglobinopathies

Congestive cardiac failureSepsis

Gilbert syndrome.

DDs of Conjugated


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DDs of ConjugatedHyperbilirubinemia:1. Obstructive

Gall stonesPrimary sclerosing cholangitisCholedochal cystTumors

2. InfectionsHepatitis A, B, C, D, E

EBV,CMVVaricella zosterHIVLeptospirosis


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