aaa pediatric_liver_disease_overview= dr irman.ppt

8/12/2019 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!!!
https://www.abp.org/ABPWebSite/frames/ABPNavigator.jsp?PAGE=DEFAULT


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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
http://images.google.com/imgres?imgurl=http://www.biotrin.com/cmsimages/liver2.jpg&imgrefurl=http://www.biotrin.com/monitioring.liver.transplantation.html&h=303&w=400&sz=22&hl=en&start=6&um=1&tbnid=z2DwzShAwVPVvM:&tbnh=94&tbnw=124&prev=/images%3Fq%3Dliver%26svnum%3D10%26um%3D1%26hl%3Den%26sa%3DX


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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)
http://images.google.com/imgres?imgurl=http://www.medscape.com/content/2000/00/41/06/410632/art-smj9309.16.fig3.jpg&imgrefurl=http://www.medscape.com/viewarticle/410632_2&h=296&w=400&sz=14&hl=en&start=9&um=1&tbnid=fDSoOcUYZVp3TM:&tbnh=92&tbnw=124&prev=/images%3Fq%3Dcholangiogram%26svnum%3D10%26um%3D1%26hl%3Den


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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.
http://images.google.com/imgres?imgurl=http://montysbluff.com/redflag.jpg&imgrefurl=http://www.montysbluff.com/index.php/category/organizational-indicators/&h=274&w=258&sz=9&hl=en&start=3&um=1&tbnid=UGdyeFKpb9ccYM:&tbnh=113&tbnw=106&prev=/images%3Fq%3Dred%2Bflag%26svnum%3D10%26um%3D1%26hl%3Den


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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/

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