1. Prof. Sushmita N. Bhatnagar MBBS, M.S., M.Ch,M.Phil(Hospital
Management) HEAD, PEDIATRIC SURGERY B.J WADIA CHILDRENS HOSPITAL,
MUMBAI CONSULTANT PEDIATRIC SURGEON BOMBAY HOSPITAL JOINT SECRETARY
ASSOCIATION OF MEDICAL CONSULTANTS JAUNDICE IN CHILDREN
2. JAUNDICE NEWBORNS INFANT CHILD
3. JAUNDICE IN ADULTS
4. JAUNDICE IS IT A DISEASE? Yellowish staining of the skin and
sclerae High levels of bilirubin in blood
5. WHICH DISEASES CAUSE JAUNDICE? Feeding related jaundice
Congenital liver infections Genetic/autoimmune diseases Blood group
incompatibilities Congenital hepatobiliary anomalies Metabolic
Iatrogenic Idiopathic NEWBORNS Idiopathic Metabolic Infections
Biliary atresia Congenital hepatobiliary anomalies INFANTS
Infections Metabolic/Genetic conditions Stones in bile duct
Pancreatitis Acute or chronic Tumors CHILD
6. DIFFERENTIATION Medical Jaundice Surgical Jaundice
7. SURGICAL JAUNDICE Inspissated bile syndrome/Bile sludge
Choledochal cyst Biliary atresia Rare causes NEWBORNS Biliary
atresia Choledochal cyst Bile duct calculi Spontaneous rupture of
bile duct Injury INFANTS Choledochal cyst Biliary calculi
Pancreatitis Injury Tumors CHILD
8. WHICH DISEASES CAUSE JAUNDICE? Increased production of
bilirubin Acute liver inflammation Infiltrative liver diseases Bile
duct inflammation Blockage of bile ducts Drugs Genetic disorders
Developmental abnormalities of bile ducts Jaundice of
pregnancy
9. Skin and sclerae - yellow Stool - light colour, clay
coloured Dark urine Pain in abdomen Itching Trouble with sleeping
Fatigue Swelling Ascites Mental confusion Coma Bleeding WHAT
PROBLEMS DO JAUNDICE CAUSE?
10. JAUNDICE IN NEWBORN BABIES
11. NEONATAL JAUNDICE Is jaundice in newborns normal?
12. NEONATAL JAUNDICE Neonatal jaundice is quite common >50%
of normal newborns and 80% of preterm infants have some degree of
jaundice Two types of neonatal jaundice: Normal / physiological
Abnormal / non-physiological
13. WHY? PRODUCTION : In term newborns, bilirubin production is
2-3 times higher than in adults CLEARANCE decreased in newborns,
mainly due to deficiency of enzyme UGT UGT activity in term infants
at 7 days is ~1% of adult liver and doesnt reach adult levels until
14 weeks CIRCULATION Increase enterohepatic circulation of
bilirubin, further increases bilirubin load
14. PRETERM INFANTS Even more RBC turnover and destruction
Physiologically impaired conjugation and elimination of bilirubin
An even less mature liver Reduced bowel motility due to inadequate
oral intake Delayed elimination of meconium Increased enterohepatic
circulation
15. PHYSIOLOGIC JAUNDICE Jaundice appears around 72 hrs of life
Bilirubin peaks 20%) Blood type of baby and mother, and Coombs test
Syphilis serology (e.g. VDRL) G6PD screen, thyroid function tests,
liver ultrasound
20. JAUNDICE AND ITS EFFECT Deposits in skin and mucous
membranes Unconjugate d bilirubin deposits in the brain Permanent
neuronal damage JAUNDICE ACUTE BILIRUBIN ENCEPHALOPATHY KERNICTERUS
M a y c a u s e I t c h i
21. JAUNDICE IN CHILDREN
22. Hepatitis A is transmitted by contaminated food or water,
or contact with a person who is currently ill with the disease. The
hepatitis A virus is shed in the stools of an infected person
during the incubation period of 15 to 45 days before symptoms occur
and during the first week of illness. Blood and other bodily
secretions may also be infectious. The virus does not remain in the
body after the infection has resolved, and there is no carrier
state (a person or animal that spreads the disease to others but
does not become ill). The symptoms associated with hepatitis A are
fever, poor appetite, nausea & vomiting, abd pain ,jaundice
& yellow urine. This is because the liver is not able to filter
bilirubin from the blood. Risk factors include having a family
member who recently had hepatitis A, HEPATATIS A
23. CONTAMINATING!
24. DRINK ONLY BOILED WATER
25. HEPATATIS B Hepatitis B is transmitted via blood and other
body fluids. Infection can occur through: Contact with blood in
healthcare settings -- this puts physicians, nurses, dentists, and
other healthcare personnel at risk Blood transfusions Sharing
needles during drug use Receiving a tattoo or acupuncture with
contaminated instruments Birth -- an infected mother can transmit
the virus to the baby during delivery or shortly thereafter
26. UNSAFE INJECTIONS
27. OTHER HEPATITIS Hepatitis C Hepatitis E PREVENTION
NECESSARY VACCINATIONS
28. Stones can present with jaundice, especially the stones in
the bile ducts Obstructive jaundice SUSPECT INVESTIGATE TREAT
CALCULUS DISEASE
29. CARE FOR ALL BABIES Risk factors identified? Jaundi ce
Examine for jaundice at every opportunity especially in the first
72 hours An additional inspection within 48 hours YES NO
30. < 24 HOURS OF AGE Visible jaundice MEDICAL EMERGENCY
Measure and record serum bilirubn within 2 hours
Neonatology/Pediatric/Medical review within 6 hours Commence
phototherapy Organise transfer to neonatal referral center YES
31. 4 DAYS 10 DAYS PHYSIOLOGICAL JAUNDICE
32. LATE ONSET OR PROLONGED JAUNDICE Jaundice > 10 days or
prolonged Check stool color Further investigations required Seek
expert advice
33. THANK - YOU
34. PROBLEMS WITH DELAY If significant brain damage occurs
before treatment, a child can develop serious and permanent
problems, such as: cerebral palsy a condition that that affect a
child's movement and co-ordination hearing loss, which can range
from mild to severe learning difficulties involuntary twitching of
different parts of their body problems maintaining normal eye
movements people affected by kernicterus have a tendency to gaze
upwards or from side to side rather than straight ahead the normal
development of the teeth can be disrupted resulting in teeth that
are misshapen, discoloured and vulnerable to tooth decay
35. TWO FORMS OF HYPERBILIRUBINEMIA Unconjugated / indirect
hyperbilirubinemia: Pre-hepatic cause, or impairment in conjugation
VS. Conjugated / direct hyperbilirubinemia: Injury at the level of
the hepatocytes, or post- hepatic obstruction Consider diagnosis of
conjugated hyperbilirubinemia if direct bilirubin is >3mg/dL, or
is >10% of total bilirubin
36. DIFFERENTIAL DIAGNOSIS: UNCONJUGATED HYPERBILIRUBINEMIA
Breastfeeding jaundice Occurs at 1-3 days of age; due to
dehydration and lack of stooling (treat by increasing feeding
frequency) Breast milk jaundice Occurs at 4-10 days of age;
substance in breast milk inhibits glucuronyl transferase (treat by
temporary switch to formula) Hemolysis ABO/Rh incompatibility RBC
membrane defects Alpha thalassemia G6PD deficiency Cephalohematoma
Polycythemia Infection Hypothyroidism Gilberts impaired
conjugation, associated with stress, no overt hemolysis
Crigler-Najjars absent (type 1) or diminished (type 2)
UDP-glucoronyl transferase
37. DIFFERENTIAL DIAGNOSIS: CONJUGATED HYPERBILIRUBINEMIA
Biliary atresia ~60% of cases; an obliterative process of bile
ducts; diagnosed by U/S or biopsy Infection Hepatitis B, TORCH
Metabolic Galactosemia Alpha-1-antitrypsin deficiency: most common
genetic cause Dubin Johnson or Rotors syndrome: defective liver
secretion of bilirubin Iatrogenic Drug-mediated TPN-related: occurs
in ~2/3 of infants given TPN over 2 weeks of duration; unknown
mechanism, possibly mediated by bacterial endotoxins, oxidative
stress, glutathione depletion Idiopathic neonatal non-infectious
hepatitis (diagnosis of exclusion)
38. THE CONCERN: KERNICTERUS Bilirubin exceeds albumin- binding
capacity, crosses BBB, and deposits on basal ganglia and brainstem
nuclei Risks increase with levels >20 mg/dl Or lower levels in
setting of sepsis, meningitis, hemolysis, hypothermia,
hypoglycemia, or prematurity
40. CAUSE ANALYSIS OF KERNICTERUS Early discharge 1.0 MG
WWW.DRSARMA.IN 45 Is it unconjugated bilirubin ? Haemolytic
Jaundice Is it Conjugated Bilirubin ? (> 20%) Hepatocellular
jaundice Obstructive jaundice
46. THIRD STEP : IF CSB IS INCREASED WWW.DRSARMA.IN 47 Do - AST
and ALT (SGOT and SGPT) Elevated AST and ALT Hepatocellular
jaundice AKP, 5N, GGT will be normal Do - Alkaline Phosphatase and
GGT AKP, GGT in Obstructive Jaundice AST and ALT will be
normal
47. FOURTH STEP : HEPATOCELLULAR WWW.DRSARMA.IN 48
Hepatocellular Features and D.D Conjugated SB is increased AST and
ALT are increased AKP, 5NS, GGT are normal Hepititis A,B,C,D,E,
CMV,EBV Toxic Hepatitis Drugs, Alcohol Malignancy Primary Ca
Cirrhosis ALD, NAFLD
48. LABORATORY TESTS Bilirubin level in serum (total and
direct) Aminotransferase Alkaline phosphatase U/A for bilirubin and
urobilogen Complete blood count Prothrombin time Other laboratory
tests pertinent to history Coombs test Electrophoresis of
hemoglobin Viral hepatitis panel
49. LITERATURE
http://en.wikipedia.org/wiki/Jaundice#Neonatal_jaundice
http://www.medicinenet.com/jaundice/article.htm
http://www.nlm.nih.gov/medlineplus/ency/article/003243.htm
50. ANATOMY
51. SICKLE CELL DISEASE . Sickle cell anemia is an inherited
blood disease in which the red blood cells produce abnormal pigment
(hemoglobin). The abnormal hemoglobin causes deformity of the red
blood cells into crescent or sickle- shapes, as seen in this
photomicrograph
52. JAUNDICE BODY PAIN/JOINT PAIN PAINFUL SWELLINGS TIRED/NOT
WORKING POOR GROWTH RECURRENT RESP INFECTION STROKE BLOOD TEST AT
GAH WHEN DO YOU SUSPECT SCD AND WHAT SHOULD YOU DO
53. WHAT TO DO FOR PATIENT WITH SCD PROTECT AGAINST DIARRHOEA
GIVE PENICILLIN TILL AGE 5 HYDROXYUREA GENETIC ADVISE
56. WORK UP: LABORATORY STUDIES Where possible, confirm
clinical jaundice with bilirubin levels Possible additional
investigations, depending on likely diagnoses and lab availability:
Hemoglobin/hematocrit (PCV) to look for hemolysis Blood smear
Reticulocyte count WBC to look for signs of infection (WBC 20, or
I:T ratio >20%) Blood type of baby and mother, and Coombs test
Syphilis serology (e.g. VDRL) G6PD screen, thyroid function tests,
liver ultrasound
57. CLINICAL SYMPTOMS: Jaundice/Icterus: Newborn icterus
notable once total bilirubin > 5-6 mg/dL (versus older
children/adults once > 2 mg/dL) Progresses cranially to caudally
CAUTION: Visual assessment is subjective, inaccurate, and dependent
on observer experience! Keren et al Visual assessment of jaundice
in term and late-preterm infants (2009) Nurses at HUP used 5
point-scale to rate cephalocaudal extent of jaundice Showed weak
correlation between predicted and actual levels
58. PRE-TERM VS. FULL-TERM HYPERBILIRUBINEMIA: Pre-term infants
at higher risk due to further reduced activity of liver conjugating
enzymes Pre-term infants can develop encephalopathy or kernicterus
at lower total bilirubin levels
59. DIRECT HYPERBILIRUBINEMIA: Considered elevated when: Level
> 2.0 mg/dL (severe > 5.0 mg/dL) Level > 15% of total
serum bilirubin Risk factors: Low gestational age Early and/or
prolonged exposure to TPN Lack of enteral feeding Sepsis Clinical
hallmarks: icterus, acholic stools, dark urine
60. DIFFERENTIAL DX OF DIRECT HYPERBILIRUBINEMIA: More common
causes: TPN-associated Hepatitis: Idiopathic, Infectious, Toxic
Infection: Sepsis, TORCH, UTI Biliary atresia Inspissated bile plug
Choledochal cyst Alpha-1-antitrypsin deficiency Galactosemia
61. DIFFERENTIAL DX OF DIRECT HYPERBILIRUBINEMIA: Less common
causes: Cholelithiasis Cystic fibrosis Hypothyroidism Rotors
Syndrome Dubin-Johnson Syndrome Storage diseases (Niemann-Pick,
Guachers) Metabolic disorders (tyrosinemia, fructosemia) Trisomy 21
or 18 Drug-induced Shock Alagille Syndrome Zellweger Syndrome