Approach to child with Jaundice.pptttttt
- 1. Approach to Jaundice in Children
- 2. Objectives • To know how to evaluate a child with jaundice. • To familiarize with various causes of jaundice in children. • To recognize Red flag signs suggesting serious liver dysfunction in a child.
- 3. Jaundice is a condition characterized by yellowish discoloration of sclera, skin, mucus membranes due to increased circulating bilirubin usually more than 2.5mg/dl in children
- 4. A child is brought to OPD by parents with complaints of jaundice. • What other questions are important to ask the patient and family? • What tests would be useful to diagnose the child?
- 5. • Is patient vitals stable, resuscitate if needed and maintain hydration status. • Red flag signs suggesting serious liver dysfunction include altered mental status, coagulopathy , hypoglycemia, ascites, gastrointestinal bleeding. If present they should be managed in intensive care.
- 6. Jaundice History—associated symptoms, risk factors, exposures, past medical history, family history Physical examination Findings suggesting specific diagnosis no yes Fractionation of bilirubin Confirmatory diagnostic studies
- 7. Fractionation of bilirubin Conjugated <20% of total <2 mg/dL Conjugated >20% of total >2 mg/dL Diagnosis = UNCONJUGATED HYPERBILIRUBINEMIA Diagnosis = CONJUGATED HYPERBILIRUBINEMIA CBC Reticulocyte count Coombs test PT/PTT Evaluate smear PT Albumin Glucose ALT, AST Alkaline phosphatase, GGT
- 8. Diagnosis = UNCONJUGATED HYPERBILIRUBINEMIA Normal reticulocyte count Elevated reticulocyte count Look for evidence of: 1.Infection 2.Congestive heart failure . None found Familial disorders Gilbert syndrome Crigler-Najjar type II syndrome Consider: 1.Autoimmune hemolytic anemia 2.Drug-induced hemolytic anemia 3.Paroxysmal nocturnal hemoglobinuria 4.Erythrocyte membrane defect 5.Erythrocyte enzyme defect 6.DIC 7.Transfusion reaction 8.Hemolytic uremic syndrome 9.Hemoglobinopathy
- 9. Diagnosis = CONJUGATED HYPERBILIRUBINEMIA Markedly ALT, AST Mildly alkaline phosphatase 1. Infection (viral serology) HAV IgM, HbsAg, anti-HBc, EBV 2. Wilson disease serum ceruloplasmin, urine copper, liver biopsy 3. Drugs or toxins 4. Autoimmune hepatitis autoantibodies (ANA, ASMA, anti-LKM) serum globulin liver biopsy Looking for evidence of: Alkaline phosphatase >3 times normal Mildly ALT, AST Obstructive process Ultrasonography MRCP, ERCP or PTC Definitive diagnosis yes Specific treatment no
- 10. Approach to jaundice • History • Clinical examination • Investigations • Classifiy the type of jaundice • diagnosis
- 11. history Jaundice: • Age of onset • Duration(acute/chronic/acute on chronic) • Colour of urine normal : unconjugated high colour: hepatocellular/cholestatic jaundice • colour of stool pale: cholestatic Normal: hepatocellular • Any bleeding manifestations
- 12. h/o fever • Duration • Fever without chills and rigors - Viral hepatitis, drug induced hepatitis • Fever with chills and rigors Cholangitis, weil’s disease, malarial hepatitis, liver abscess • h/o intake of aspirin. h/o abdominal pain right hypochondrial pain with fever Consider hepatitis, cholangitis, cholecystitis, liver abscess
- 13. • h/o anorexia and fatigue - Consider hepatitis(early feature though nonspecific) possibility of metabolic liver disease • h/o pallor, recurrent blood transfusions. • h/o bleeding tendency • h/o abdominal distension Diffuse /Upper abdomen • h/o rash, arthralgia, arthritis Consider infective/autoimmune (especially in girl child) • h/o recent blood transfusions • h/o recent travel/outbreak in their locality
- 14. H/o poor weight gain • h/o reccurent vomitings, abnormal odour • h/o developmental delay, seizures, poor vision, abnormal behaviour. Past history • h/o jaundice in the past • h/o neonatal jaundice, if phototherapy given • h/o recurrent blood transfusions • h/o needle pricks, tatooing, ear boring • h/o recurrent diarrhea, respiratory tract infections • h/o any abdominal surgeries Perinatal history: • TORCH infections, IUGR, • h/o umbilical cautheterization. metabolic
- 15. Family history • Positive clue to genetic or metabolic disorder • h/o thyroid disease, rheumatoid group of diseases early liver failure suggest - autoimmune • h/o consanguinity: possibility of metabolic disease • h/o viral hepatitis in family member or sibling • h/o sibling death due to jaundice or neuropsychiatric illness • h/o pallor + recurrent blood transfusions • h/o abdominal surgeries • Sociocultural practices: cooking vessels • Familial jaundice
- 16. Drug history Antituberculous drugs Anticonvulsants Antipsychotics Paracetamol Toxins native medicines.
- 17. General examination • Level of consciousness and behavior (alert/drowsy/stupor/coma) • odour : fetor hepaticus • vitals pulse, RR, temperature , BP • Pallor, icterus, clubbing, cyanosis, rash lymphadenopathy, pedal edema. • Anthropometry - head circumference (microcephaly) malnutrition, growth retardation
- 18. Ocular signs Subconjunctival hemorrhage: weils disease, acute paracetamol poisoning KF ring, sunflower cataract: Wilsons disease Oil drop cataract: Galactosemia Bitot spots: Cholestatic jaundice Chorioretinitis: autoimmune, TORCH infections
- 19. Oral cavity Oral thrush Fetor hepaticus Nail changes Clubbing Leukonychia Blue lunulae: wilsons disease Shining nails: cholestatic jaundice
- 20. Skin changes Ecchymosis: hepatocellular failure, hypersplenism Hyperpigmentation: Wilsons disease, cirrhosis Vitiligo: consider autoimmune disorder Palmar erythema, spider nevi: Chronic liver disease. Scratch marks, shiny nails: Cholestatic jaundice Dysmorphic features: Alagaile syndrome, cretinism
- 21. Systemic examination •Abd Examination Abdominal distension, Ascites Right hypochondrial tenderness (viral hepatitis, right heart failure, abscess, malignancy, cholangitis) Hepatomegaly: consistency and character of liver edge. splenomegaly, Caput medusae Arterial bruit, venous hums and bowel sounds
- 22. • Respiratory system: Pleural effusion • Emphysematous lungs : alpha 1 antitrypsin deficiency. cystic fibrosis • CVS: to rule out congenital heart disease • CNS: level of consciousness, mental retardation, abnormal behavior. Abnormal neurologic findings, including tremor, fine motor incoordination, clumsy gait and choreiform movements suggest Wilson disease.
- 23. Investigation s Complete hemogram & peripheral smear, hematological tests – leukopenia, smear showing atypical lymphocytes (viriocytes)-viral hepatitis – Leucocytosis with striking neutropilia with very high ESR in weils disease and cholangitis – Target cells- cholestatic jaundice, – Spurr cells in chronic liver disease – If hemolytic jaundice is suspected then reticulocyte count to be done – Thrombocytopenia is typically seen in portal hypertension & hypersplenism – Coagulation profile
- 24. Serum bilirubin: if direct bilirubin more than 20%of total bilirubin – direct hyperbilirubinemia (cholestatic jaundice either intrahepatic or extrahepatic etiology) • Hemolytic jaundice: Total bilirubin usually doesn’t exceed 5mg/dl
- 25. Levels of Serum aminotransferase and their common etiologies • Serum aminotransferases levels : tests to detect liver cell injury Symptomatic children with levels > 1000 IU/L • Acute viral hepatitis • Reyes syndrome • Autoimmune hepatitis • Ischemic injury • Drug induced
- 26. Symptomatic children with levels <1000 IU/L • Chronic heptitis • Infections like leptospirosis, Dengue, Malaria • Storage disorders like Gaucher’s disease • Inborn errors of metabolism • Celiac disease Asymptomatic children with levels <1000 IU/L • Non-alcoholic fatty liver disease(NAFLD) • Wilsons disease • Alpha 1-antitrypsin deficiency • Chronic hepatitis B, C
- 27. • Serum alkaline phosphatase: elevated in choleststic jaundice (intrahepatic or extrahepatic) (Normal range in 5 year old child: 110-460 U/L) • Gamma glutamyl transpeptidase: elevated in choleststic jaundice. (Normal range in 5 year old child:5-55U/L) • Both ALP and GGT cannot differentiate intra- hepatic cholestasis from extrahepatic cholestasis • High ALP, low to normal GGT is seen in PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS
- 28. • Serum albumin, PT, APTT • Blood urea, serum creatinine and electrolytes • Serological tests : HbsAg, HCV • Ultrasound abdomen • CT abdomen detects tumors, cysts, abscess, fatty liver, gall stones • Cholescintigraphy • Liver biopsy: to diagnose inborn errors of metabolism, storage disorders, indian childhood cirrhosis, wilsons disease.
- 29. Classification of jaundice jaundice Hemolytic jaundice Hepatocellular jaundice Cholestatic jaundice
- 30. Hemolytic jaundice • Anemia /pallor • Mild jaundice • Abdominal pain is uncommon feature • Splenomegaly with or without hepatomegaly • Stools yellow, urine variable colour • Hemolytic facies +/- • Positive family history +/- • Reticulocytosis +/- • Smear evidence of hemolysis • Indirect hyperbilirubinemia
- 31. Hepatocellular jaundice • Jaundice • High coloured urine • Pale stools +/- • GI symptoms: anorexia, vomiting, abdominal pain • Hepatomegaly with or without splenomegaly • Bleeding tendency
- 33. Cholestatic jaundice • Usually deep jaundice • Very pale stools • High coloured urine • Intense pruritis(scratch marks) • Xanthomas (chronic cholestasis) • Fat malabsorption • bleeding tendency Cholestatic jaundice Hepatocellular cholestasis Obstructive
- 34. Stepwise approach • Step 1: To assess if the jaundice is hemolytic in origin or hepatic in origin. • Step2: To decide if the hepatic cause of jaundice is secondary to hepatocellular dysfunction or defect in bile flow • Step 3: Is it a chronic liver disease? • Step 4: Is it compensated, decompensated or acute fulminant?
- 35. Viral hepatitis-Hepatocellular Hepatotropic viruses Non hepatotropic viruses Acute: HAV, HEV Chronic: HBV, HCV HSV, EBV, CMV, Enterovirus, Adenovirus Cause more isolated liver injury than non-hepatotropic virus Cause hepatitis as a part of multisystem disease • Signs and symptoms of hepatitis are mild to severe. • Extrahepatic manifestations unusal. • Transaminases can be as high as thousands during acute stage • Signs and symptoms of hepatitis is mild. • Extrahepatic manifestations like rash, lymphadenopathy, neurological disease more common • Transminases increase moderately
- 36. Non viral heptitis – when to consider • Significant fever • Extrahepatic manifestations- Nephritis, Anemia, Diarrhea, Rash are prominent. • AST/ALT – Elevation in hundreds. • Causes: Malaria, leptospirosis, Typhoid fever, liver abscess (jaundice uncommon)
- 37. Drug induced or toxin induced heptatitis • Clinical manifestations can be mild & nonspecific or severe in the form of fever, rash, malaise • Mild elevations of transaminases are very common, usually resolve spontaneously even on continuation of the drug . • A 2-3 fold increase of transaminases or presence of jaundice indicates significant liver injury, which needs immediate discontinuation of the drug. • So children on hepatotoxic drug: need close follow up
- 38. Metabolic disorder – when to cosider • No clear identifiable cause • Positive family history • Child with abnormal phenotype like dysmorphism or short stature • Growth retardation • Recurrent vomiting • Associated neurological abnormality like- developmental delay, hypotonia, seizures, neurodegenaration • Associated cardiac disease • Abnormal odour • Presence of cataract , rickets • Presence of organomegally • Presence of hypoglycemia, metabolic acidosis, hyperamonemia
- 39. Autoimmune hepatitis • Considered in any child with non-viral or non-drug induced hepatitis • Girls more commonly affected with auto immune diseases • Older children (though young children can be affected) • Extrahepatic manifestations- common • Features of chronic liver disease where viral, toxic, metabolic causes have been ruled out • Prominent elevation of transaminases and not alkaline phosphatase. • Elevated gamma globulin level • Presence of autoantibodies: ANA, anti LKM antibody, anti smooth muscle antibody, anti mitochondrial antibody. • Liver biopsy needed in these children
- 40. Cholestatic jaundice • Direct hyperbilirubinemia, elevated ALP and GGT, intense pruritis, hypercholesterolemia and xanthomas with evidence of fat malabsorption. • Presence of significant pain with or without mass points towards obstructive cause of cholestasis • High ALP, low to normal GGT consider PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS • Child presenting with cholestasis needs to be subjected to imaging studies like ultrasound and CT of abdomen, cholangiography. • Radionuclear scans like HIDA are useful to differentiate hepatocellular cholestasis from obstructive cholestasis.
- 41. Is it chronic liver disease Features of chronic liver diease: • Firm/hard hepatomegaly • Splenomegaly/varices (portal hypertension) • Ascites • Clubbing • Malnutrition • Spider angioma • Palmer erythema • White nails
- 42. Is it decompensated liver disese Signs of decompensation include: • Worsening jaundice in the background of shrinking liver • Bleeding tendency • Encephalopathy- Altered sleep pattern, behavioral abnormality, coma • Worsening edema and ascites
- 43. Causes of jaundice • Hepatocellular jaundice Infectious hepatitis Viral • Hepatotropic : A ,B ,C ,D,E • Non hepatotropic: HSV, EBV, CMV, enterovirus, adenovirus Bacterial • Typhoid, brucellosis, disseminated TB, E. coli septicemia Spirochaetal • Weils disease Protozoal • Malaria, kala azar, schistosomiasis
- 44. Hepatocellular jaundice- causes Drugs Antitubercular, Antipsychotics, Paracetamol, anticonvulsants, Toxins • Aflatoxins • Mushroom poisoning • Vinyl chloride, carbon tetrachloride • Benzene derivatives • 4,4-diamino diphenyl methane
- 45. Heptocellular jaundice causes Metabolic causes • Wilsons disease • Galactosemia • Glycogen storage disorders • Tyrosinemia • Urea cycle disorders • Gauchers and Niemann pick disease • Indian childhood cirrhosis • Hemochromatosis • Crigler najjar, Gilbert, Dubin Johnson and Rotor syndrome • Alpha 1 antitrypsin defiency
- 46. Cholestatic jaundice causes Obstructive cholestasis • Choledochal cyst • Gall stones • cholangitis • Alagille syndrome. Hepatocellular cholestasis • HAV, HEV hepatitis • Drug induced hepatitis • Metabolic – Galactosemia • Progressive familial intrahepatic cholestasis
- 47. Management • Focus on treating underlying cause, optimisation of hydration, nutrition and controlling pruritis • Diet high in medium chain triglycerides should be used to promote growth in children with chronic cholestasis. • Supplementation of fat soluble vitamins is essential • Agents shown to reduce pruritis : Cholestyramine (bile acid binding agent), Ursodeoxycholic acid (cholerectic agent), Phenobarbitone,
- 48. Pearls • Acute viral hepatitis due to HAV or HEV continues to be most common cause of acute hepatitis in childhood. • Drug induced hepatitis should always be considered in a child with exposure to drugs. • Metabolic disorders and autoimmune disorders should be considered when the diagnosis of heptitis is unclear. • Transaminases can be in thousands in acute viral hepatitis & drug/toxic hepatitis, • Children presenting with features of cholestasis need imaging studies to rule out obstructive causes.