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Pediatric Liver Disease

Pediatric Liver Disease. Ricardo A. Caicedo, MD Pediatric Gastroenterology. Objectives. Review content specification for certification from American Board of Pediatrics in the context of Diagnostic liver tests Jaundice Neonatal Child and Adolescent Hepatitis Hepatomegaly

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Pediatric Liver Disease

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  1. Pediatric Liver Disease Ricardo A. Caicedo, MD Pediatric Gastroenterology

  2. Objectives Review content specification for certification from American Board of Pediatrics in the context of • Diagnostic liver tests • Jaundice • Neonatal • Child and Adolescent • Hepatitis • Hepatomegaly • Chronic liver disease Don’t be afraid of the liver!!!

  3. Terms • Hepatitis: inflammation of the liver • Can be due to multiple causes (infectious, toxic, metabolic) • Can be acute or chronic • Hepatomegaly: enlargement of the liver • Hepatopathy: disease of the liver

  4. Terms • Jaundice: a symptom or sign of hyperbilirubinemia (total bili > 2) • yellow color to skin, mucous membranes, eyes (Conjunctival/scleral icterus) • Can be due to unconjugated or conjugated hyperbilirubinemia • Cholestasis: impairment of bile flow • Conjugated (direct) hyperbilirubinemia • Can be due to cholestatic process or primary liver disease • Defined as direct bili. > 20% of total bili.

  5. Terms • Liver dysfunction: abnormal synthetic function • Defined by low albumin or abnormal PT/INR • Liver failure: all hepatic functions compromised • Encephalopathy • Coagulopathy/bleeding • Ascites • Portal hypertension • Hypoglycemia • Cirrhosis: late stage of progressive hepatic fibrosis (scar tissue formation) • with formation of regenerative nodules; usually irreversible

  6. Hepatic Function Panel Synthetic Excretory Biliary Epithelium Hepatocellular Injury TOTAL PROTEIN TOTAL BILIRUBIN ALKALINE PHOSPHATASE AST (SGOT) ALBUMIN DIRECT BILIRUBIN GGT ALT (SGPT) PT/INR

  7. Imaging Studies • Ultrasonography • identify anatomic problem • GB presence does not exclude biliary atresia • Recommended in cholestasis of unknown etiology • NM bili-scintigraphy • HIDA or DISIDA scan • Tracer taken up by liver should empty into gut • Highly sensitive, not specific

  8. Diagnostic Tests • Liver biopsy • Provides specific diagnostic findings • Recommended in • most infants with cholestasis of unknown etiology • chronic liver disease of unknown cause • HBV or HCV with abnormal liver tests • Cholangiogram • Intraoperative injection of contrast into biliary tree • Excludes biliary atresia • If biliary atresia confirmed, proceed with portoenterostomy (Kasai procedure)

  9. Elevated Transaminases Hepatocellular Injury AST (SGOT) 20-55 • Hx: chronicity, symptoms • Stigmata of chronic liver disease? • Liver panel including PT/INR • Differential dx • Drug/toxin • Review medications including OTC, NSAIDs, herbals • Viral hepatitis • Acute: HAV IgM, HBsAg, HBc IgM, HCV Ab • Chronic: HBsAg, anti-HBs, HBeAg, HCV Ab • Systemic: CMV IgM, EBV serology panel, HIV Ab • Autoimmune hepatitis • Globulin fraction (TP – ALB), ANA, smooth muscle Ab, LKM Ab • Consider overlap with IBD (ESR, CRP) and celiac disease (tTG IgA) • Metabolic • CF: sweat chloride • Alpha-1-antitrypsin deficiency: AAT level and Pi type • Hemochromatosis: serum iron, TIBC, ferritin • Wilson’s disease: ceruloplasmin • Non-alcoholic fatty liver disease (NAFLD) • Ultrasonography • Other • Endocrine: Adrenal insufficiency, thyroid disorders • Muscle disease • AST/ALT > 3 initially • Elevated CPK, aldolase ALT (SGPT) 15-60

  10. Jaundice • Know how to evaluate a • 2 day old infant with jaundice • 1 month old infant with icterus • Child with conjugated hyperbilirubinemia

  11. Unconjugated Hyperbilirubinemia • Know the appropriate diagnostic tests to establish the cause of unconjugated hyperbili. • Diagnostic studies to detect hemolytic disease • Breast feeding does not cause conjugated hyperbili, but it is the most frequent cause of exaggerated unconj. hyperbili. in neonates • Know mgmt. of infant with breast-milk jaundice

  12. Features of Pathologic Jaundice Red flags • jaundice in the first 24 hours of life • jaundice that appears excessive for age • bilirubin rise > 5 mg/dl/24 hours • bilirubin total > 12 mg/dl • jaundice persists beyond 2 weeks AAP Clinical Practice Guideline. Pediatrics 2004;114:297-316.

  13. Early Diagnosis is Important Unconjugated (I) Hyperbilirubinemia Conjugated (D) Hyperbilirubinemia Hemolysis Minimize potential for kernicterus Breast-milk Avoid excess investigations Intrahepatic Identify treatable conditions Extrahepatic Time limit for Biliary Atresia

  14. Evaluation of the Yellow Baby No 2 -week old yellow baby should go without a total and direct bilirubin! Jaundiced infant 2-4 wks T/D Bili Direct < 15% total Direct > 15% total Unconjugated Breast milk Hemolytic (CBC, Coombs) Hx/physical, growth and feeding Stool color Liver panel, UA/UCx Review neonatal screen RED FLAGS Follow q 1-2 weeks Ultrasound PT/INR Pediatric GI Consult Persists Resolves before 2 m HIDA scan, metabolic studies, liver bx, cholangiogram

  15. Anatomic Biliary atresia Choledochal cyst GB stones/sludge Inspissated bile Alagille syndrome (syndromatic BD paucity) Cong. Hepatic Fibrosis Infectious Sepsis UTI “TORCH” incl. CMV, HIV Enterovirus, adenovirus Metabolic Panhypopit. Hypothyroidism Galactosemia Alpha-1-antitrypsin deficiency CF Tyrosinemia Other TPN-associated Progressive familial intrahepatic cholestasis Idiopathic neonatal hepatitis Know the differential dx of jaundice in a 2 month old infant • LABORATORY TESTS • Hepatic function panel “LFTs” • PT/INR • CBC/diff, Blood culture • UA and Urine culture • “TORCH” serology • Alpha-1-antitrypsin level and Pi phenotype • Neonatal screen • TSH • Sweat chloride • Urine succinylacetone • Urine organic and serum amino acids

  16. Recognize that sepsis, galactosemia and endocrine disorders can be readily diagnosed in the neonate with conjugated hyperbilirubinemia Know the metabolic diseases that can lead to conjugated hyperbilirubinemia in the neonatal period

  17. Recognize the signs/sx of BILIARY ATRESIA • Cholestatic jaundice • Acholic/pale stools • Dark urine • Growth failure • Abdominal distention • Ascites • Hepatomegaly • Signs of chronic liver disease • Bruising/bleeding • Variceal bleeding • Splenomegaly • Caput medusa

  18. Recognize cholecystitis in children Jaundice Pale stools/dark urine Cholestasis RUQ pain Nausea/vomiting Fatigue/malaise /+ Fever TESTS LFT incl. GGT Ultrasound Stones, sludge, dilated BD NM Biliary Scan CAUSES Idiopathic Obesity Hemolytic disorder Cystic fibrosis Systemic disease TPN Cholecystitis

  19. Gilbert Syndrome Recognize the clinical presentation of a child with Gilbert syndrome • Mild unconjugated hyperbilirubinemia • Typically in healthy adolescents • Triggered by stress, fasting, or infection • No hemolysis • Benign: no long-term sequelae • Caused by mild deficiency in conjugating enzyme (UDPGT) activity • Severe deficiency: Crigler-Najjar (neonatal)

  20. Know how to evaluate • A child with conjugated hyperbilirubinemia • A 12 year old child with icterus

  21. Pashankar D, Schreiber RA

  22. Hepatitis • Recognize the signs and sx of infectious hepatitis • Know the laboratory evaluation of hepatitis • Know the immediate and long term complications of hepatitis Hochman J, Balistreri WF.

  23. Acute Viral Hepatitis • Acute hepatocellular injury/inflammation • Reflected by elevated transaminases • Clinical manifestations often include fever, malaise, jaundice, RUQ pain, nausea/vomiting • Typically self-limited and of short duration • Contrast with: chronic, fulminant • Causative agents • HAV (50% of cases in U.S.), HEV • CMV, EBV, VZV

  24. Fulminant Hepatitis • Acute, massive hepatocellular necrosis • Impaired synthetic, excretory, and detoxifying functions of the liver • Cholestasis, ascites, coagulopathy, encephalopathy, multi-system failure • Initially very elevated transaminases • Falling transaminases and rising bilirubin ominous • Hyperammonemia, hypoalbuminemia, prolonged PT, hypoglycemia • Viral agents (50% of cases) • Most cases of fulminant hepatic failure are caused by unidentified agent, presumably viral • HAV, HBV+/-HDV, HCV, HEV • HSV, enteroviruses, EBV, CMV, HHV-6, VZV

  25. Chronic Hepatitis • Prolonged necroinflammatory process • Elevated transaminases for > 4-6 months • Insidious clinical manifestations • Can include cholestasis (jaundice, pruritus), ascites, hypoalbuminemia, coagulopathy, encephalopathy • Can progress to fibrosis and then cirrhosis • Viral agents: HBV (+/- HDV), HCV • Other causes include autoimmune, metabolic disorders (Wilson’s, CF, alpha-1 antitrypsin deficiency), drug/toxin-mediated, idiopathic

  26. Chronic Viral HepatitisRisk Factors Hochman J, Balistreri WF. Pediatr Rev. 2003; 24:399-410.

  27. Hepatitis A Virus • Causes 33% of acute viral hepatitis in U.S. • NOT a cause of chronic hepatitis • rarely causes fulminant hepatitis (< 1% cases) • Can trigger autoimmune hepatitis in predisposed individuals • Epidemiologic factors • Fecal-oral transmission • Poor hygiene • High population density • Daycare centers and minor epidemics • Universal HAV vaccination recommended • Treatment is supportive A = Acute

  28. Hepatitis B Virus (HBV)

  29. Sequelae of HBV vs. HCV B = Bad for Babies C = Chronic

  30. INFANT Anatomic Biliary obstruction Biliary atresia CHF Congenital hepatic fibrosis Metabolic disorder Lysosomal storage Glycogen storage Infection Sepsis TORCH Hepatomegaly • OLDER CHILD • Anatomic • Biliary obstruction • CHF • Hemangiomas • Malignancy • Leukemia/lymphoma • Hepatoblastoma • Metastases • Infection • Sepsis • Viral hepatitis (esp. HAV) • Liver abscess • Chronic hepatitis • Autoimmune • Wilson’s • Non alcoholic fatty liver disease Know the significance of hepatomegaly in a 1 month old infant and 12 y old child Wolf A, Lavine J. Hepatomegaly in Neonates and Children. Pediatrics in Review, Vol. 21 (9), Sept. 2000.

  31. Evaluation of Hepatomegaly • Hx and exam • Concomitant splenomegaly? • Liver panel • CBC/diff • Ultrasound • Doppler flow study of hepatic vessels • Liver biopsy • Additional imaging (CT, MR)

  32. Portal Hypertension Hepatic disease Portal vein thrombosis IVC thrombosis

  33. Know the signs, symptoms and lab findings associated with portal hypertension Hypersplenism Splenomegaly Thrombocytopenia Rectal varices Hemorrhoids (LGI bleeding) Esophageal varices (UGI bleeding) Ascites Caput medusa Ryckman FC, Alonso MH. Causes and management of portal hypertension in the pediatric population.Clin Liver Dis. 2001 Aug;5(3):789-818.

  34. North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) www.naspghan.org American Association for the Study of Liver Diseases (AASLD) www.aasld.org

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