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Transcript of AAA pediatric_liver_disease_overview= dr IRMAN.ppt
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PediatricLiver Disease
Adapted from Ricardo A. Caicedo, MD
Pediatric Gastroenterology of WFUB Medical Centre
Irman Permana,Sp.A,M.Kes
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ObjectivesKnow - Understand overview
content: Diagnostic liver tests Jaundice
Neonatal Child and Adolescent
Hepatitis Hepatomegaly Chronic liver disease
Dont be afraid of the liver!!!
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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
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Terms
Jaundice : a symptom or sign ofhyperbilirubinemia (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 liverdisease
Defined as direct bili. > 20% of total bili.
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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
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Hepatic Function Panel
ALBUMIN
TOTALBILIRUBIN
GGT
ALKALINEPHOSPHATASE
ALT(SGPT)
AST(SGOT)
TOTALPROTEIN
DIRECTBILIRUBIN
PT/INR
Synthetic Excretory BiliaryEpitheliumHepatocellular
Injury
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Imaging Studies Ultrasonography
identify anatomicproblem
GB presence does notexclude biliary atresia
Recommended incholestasis of unknownetiology
NM bili-scintigraphy
HIDA or DISIDA scan Tracer taken up by liver
should empty into gut Highly sensitive, not
specific
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HIDA-PIPIDA-DISIDA A cholescintigraphy scan, also
known as: HepatobiliaryIminodiacetic Acid ( HIDA),Paraisopropyl Iminodiacetic Acid(PIPIDA) , or Diisopropyl
Iminodiacetic Acid ( DISIDA) scanis a nuclear imaging procedure toevaluate the health and function ofthe gallbladder . A radioactivetracer is injected through anyaccessible vein, then allowed to
circulate to the liver , where it isexcreted into the biliary system and stored by the gallbladder andbiliary system .
http://en.wikipedia.org/wiki/Gallbladderhttp://en.wikipedia.org/wiki/Liverhttp://en.wikipedia.org/wiki/Biliary_systemhttp://en.wikipedia.org/wiki/Biliary_systemhttp://en.wikipedia.org/wiki/Biliary_systemhttp://en.wikipedia.org/wiki/Biliary_systemhttp://en.wikipedia.org/wiki/Liverhttp://en.wikipedia.org/wiki/Gallbladder -
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Diagnostic Tests Liver biopsy
Provides specific diagnosticfindings
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 contrastinto biliary tree
Excludes biliary atresia If biliary atresia confirmed, proceed with
portoenterostomy (Kasai procedure)
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Elevated Transaminases 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 Wilsons 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
AST(SGOT)
20-55
HepatocellularInjury
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Jaundice
Know how to evaluate a 2 day old infant with jaundice 1 month old infant with icterus Child with conjugated hyperbilirubinemia
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UnconjugatedHyperbilirubinemia
Know the appropriate diagnostic tests toestablish the cause of unconjugated hyperbili.
Diagnostic studies to detect hemolytic disease
Breast feeding does not cause conjugatedhyperbili, but it is the most frequent cause ofexaggerated unconj. hyperbili. in neonates
Know mgmt. of infant with breast-milk jaundice
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Features of Pathologic Jaundice
Red flags jaundice in the first 24 hours of life
jaundice that appears excessive forage
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.
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Early Diagnosis is Important
Unconjugated (I) Hyperbilirubinemia
Conjugated (D) Hyperbilirubinemia
HemolysisMinimize potentialfor kernicterus
Breast-milkAvoid excessinvestigations
IntrahepaticIdentify treatable
conditionsExtrahepatic
Time limit for BiliaryAtresia (2 Mo)
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No 2 -week o ld yel lowbaby shou ld gowi thou t a to ta l anddirect bi l irubin !
Evaluation of the Yellow Baby
Jaundiced infant 2-4 wks
T/D Bili
Direct < 15% total Direct > 15% total
UnconjugatedBreast milk
Hemolytic(CBC, Coombs)
Hx/physical, growth and feedingStool color
Liver panel, UA/UCx
Review neonatal screenRED FLAGS
UltrasoundPT/INR
Pediatric GI Consult
Follow q 1-2 weeks
Resolves before 2 mPersists
HIDA scan, metabolic studies,liver bx, cholangiogram
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LABORATORY TESTS
- Hepatic function panel LFTs
- PT/INR
- CBC/diff, Blood culture
- UA and Urine culture
- TORCH serology
- Alpha-1-antitrypsin level and Piphenotype
- Neonatal screen
- TSH
- Sweat chloride
- Urine succinylacetone
- Urine organic and serum aminoacids
Know the differential dx of jaundice in a 2month old infant
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
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Recognize that sepsis, galactosemia andendocrine disorders can be readilydiagnosed in the neonate with conjugatedhyperbilirubinemia
Know the metabolic diseases that can leadto conjugated hyperbilirubinemia in theneonatal period
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Recognize the signs/sx of BILIARY ATRESIA
Cholestatic jaundice Acholic/pale stools Dark urine
Growth failure
Abdominal distention Ascites Hepatomegaly
Signs of chronic liverdisease Bruising/bleeding Variceal bleeding Splenomegaly Caput medusa
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Atresia Biliaris(kasus Bilqis 2 tahun)
Kondisi pada awal tahun 2010
Lahir Februari 2008
10 April 2010
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CholecystitisRecognize 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
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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 conjugatingenzyme (UDPGT) activity
Severe deficiency: Crigler-Najjar (neonatal)
Recognize the clinical presentation of achild with Gilbert syndrome
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Know how to evaluate
- A child with conjugated hyperbilirubinemia
- A 12 year old child with icterus
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Pashankar D, Schreiber RA
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Hepatitis
Recognize the signs and sx of infectioushepatitis
Know the laboratory evaluation of hepatitis Know the immediate and long term
complications of hepatitis
Hochman J, Balistreri WF.
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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
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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
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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 (Wilsons, CF, alpha -1 antitrypsin deficiency), drug/toxin-mediated, idiopathic
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Chronic Viral HepatitisRisk Factors
Hochman J, Balistreri WF. Pediatr Rev. 2003; 24:399-410.
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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
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Hepatitis B Virus (HBV)
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Sequelae of HBV vs. HCV
B = Bad for Babies C = Chronic
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HepatomegalyINFANT Anatomic
Biliary obstruction Biliary atresia
CHF
Congenital hepatic fibrosis Metabolic disorder
Lysosomal storage Glycogen storage
Infection Sepsis TORCH
Wolf A, Lavine J. Hepatomegaly in Neonates and Children. Pediatrics in Review, Vol. 21 (9), Sept. 2000.
OLDER CHILD Anatomic
Biliary obstruction CHF Hemangiomas
Malignancy
Leukemia/lymphoma Hepatoblastoma Metastases
Infection Sepsis
Viral hepatitis (esp. HAV) Liver abscess
Chronic hepatitis Autoimmune Wilsons Non alcoholic fatty liver disease
Know the significance of hepatomegaly in a1 month old infant and 12 y old child
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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)
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Portal Hypertension
Hepaticdisease
Portal veinthrombosis
IVCthrombosis
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Know the signs, symptoms and lab findings associated withportal hypertension
Ryckman FC, Alonso MH.Causes and management of portal hypertension in the pediatric population.
Clin Liver Dis. 2001 Aug;5(3):789-818.
Ascites
Caput medusa
Rectal varices
Hemorrhoids(LGI bleeding)
Esophagealvarices (UGIbleeding)
Hypersplenism
SplenomegalyThrombocytopenia
https://www.abp.org/ABPWebSite/frames/ABPNavigator.jsp?PAGE=DEFAULThttp://www.gulf-news.com/images/2004/02/01_spleen.jpghttp://www.cptc.ctc.edu/library/Endoscopyphotos_files/image002.jpg -
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North American Society for Pediatric Gastroenterology, Hepatology andNutrition (NASPGHAN) www.naspghan.org
American Association for the Study of Liver Diseases (AASLD)www.aasld.org
http://www.naspghan.org/http://www.aasld.org/http://www.aasld.org/http://www.naspghan.org/