1. 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. 2. JAUNDICE NEWBORNS INFANT CHILD
3. 3. JAUNDICE IN ADULTS
4. 4. JAUNDICE IS IT A DISEASE? Yellowish staining of the skin and sclerae High levels of bilirubin in blood
5. 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. 6. DIFFERENTIATION Medical Jaundice Surgical Jaundice
7. 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. 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. 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. 10. JAUNDICE IN NEWBORN BABIES
11. 11. NEONATAL JAUNDICE Is jaundice in newborns normal?
12. 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. 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. 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. 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
16. 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
17. 21. JAUNDICE IN CHILDREN
18. 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
19. 23. CONTAMINATING!
20. 24. DRINK ONLY BOILED WATER
21. 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
22. 26. UNSAFE INJECTIONS
23. 27. OTHER HEPATITIS Hepatitis C Hepatitis E PREVENTION NECESSARY VACCINATIONS
24. 28. Stones can present with jaundice, especially the stones in the bile ducts Obstructive jaundice SUSPECT INVESTIGATE TREAT CALCULUS DISEASE
25. 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
26. 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
27. 31. 4 DAYS 10 DAYS PHYSIOLOGICAL JAUNDICE
28. 32. LATE ONSET OR PROLONGED JAUNDICE Jaundice > 10 days or prolonged Check stool color Further investigations required Seek expert advice
29. 33. THANK - YOU
30. 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
31. 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
32. 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
33. 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)
34. 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
35. 39. SIGNS OF KERNICTERUS Acute sequelae: Poor suck, lethargy, hypotonia, seizure Then hypertonia (opisthotonus, retrocollis), fever, high-pitched cry Chronic sequelae: Choreoathetoid CP, gaze paresis, sensorineural hearing loss, mental retardation
36. 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
37. 45. IN UNCONJUGATED BILIRUBIN WWW.DRSARMA.IN 46 Hemolytic Jaundice - Uncommon 1. Hemolytic Disorders + Anemia Inherited Sphero, SS, G6PD, PK Acquired MAHA, PNH 2. Ineffective Erythropoesis B12, Fe, F 3. Drugs Rifampicin, Probenecid 4. Inherited Crigler Najjar, Gilberts
38. 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
39. 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
40. 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
41. 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
42. 50. ANATOMY
43. 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
44. 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
45. 53. WHAT TO DO FOR PATIENT WITH SCD PROTECT AGAINST DIARRHOEA GIVE PENICILLIN TILL AGE 5 HYDROXYUREA GENETIC ADVISE
46. 54. DISEASES FROM CONTAMINATED WATER HEPATIC JAUNDICE Dysentery, Amebic dysentery, Cholera, Giardia Entamoeba histolytica Hepatitis A, Leptospirosis Typhoid fever, Cercarial dermatitis, Cryptosporiosis, Cyclosporiasis, Cysticercosis, Dracunculiasis, Hookworm,, Melioidosis, Neurocysticercosis, Polio, Relapsing fever, Toxocariasis, Trachoma, Traveler's diarrhea,
47. 55. JAUNDICE IN NEWBORN
48. 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
49. 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
50. 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
51. 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
52. 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
53. 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