neonatal cholestasis · choledochalcyst bile duct stenosis spontaneous perforation of the bile duct...
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NEONATAL CHOLESTASISNEONATAL CHOLESTASIS
張堯婷張堯婷
CONTENTSCONTENTS
� Definition
� Etiologies
� Clinical Presentation And Diagnosis
� Treatment
� Outcome
� Reference
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DEFINITIONDEFINITION
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NASPGHAN Definition NASPGHAN Definition
� Conjugated bilirubin concentration
>1.0 mg/dL if the total serum bilirubin is <5.0 mg/dL
Or
� Conjugated bilirubin concentration >20 % of the total serum bilirubin if the total serum bilirubin is >5.0 mg/dL
4(Moyer et al J PEDIATR GASTROENTEROL NUTR 2004;39:115)
IAP DefinitionIAP Definition
� Conjugated hyperbilirubinemia > 1.5-2
mg/dL in a newborn/ infant with passage
of high coloured urine with or without
acholic stools.
5(Consensus report on neonatal cholestasis syndrome,INDIAN PEDIATRICS 2000;37:845-851)
ETIOLOGIESETIOLOGIES
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INTRAHEPATIC ETIOLOGIESINTRAHEPATIC ETIOLOGIES
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INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES -- INHINH
� IDIOPATHIC NEONATAL HEPATITIS
▪ Generally normal stools or clay stools with
onset at one month-old
▪ Low birth weight
▪ Male predominance
▪ Familial cases (15-20%)
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INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES -- ToxicToxic
� TPN-associated cholestasis
� Drug-induced cholestasis
▪ Anticonvulsants
▪ Antibiotics
▪ Immunosuppressive Agents
▪ Psychotropic Drugs
▪ Drugs Against HIV
▪ Acetaminophen
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INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES --
InfectiousInfectious� Bacterial sepsis
▪ E. coli
▪ Listeriosis
▪ Staph. aureus
� TORCHES
� Echo virus
� Hepatitis B and C
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INTRAHEPATIC ETIOLOGIESINTRAHEPATIC ETIOLOGIES --
MetabolicMetabolic� Disorders of Carbohydrate
Metabolism
� Disorders of Amino Acid Metabolism
� Disorders of Lipid Metabolism
� Disorders of Bile Acid Metabolism
� Peroxisomal Disorders
� Endocrine Disorders
� Miscellaneous Metabolic Disorders
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INTRAHEPATIC ETIOLOGIESINTRAHEPATIC ETIOLOGIES --
MetabolicMetabolic� Disorders of Carbohydrate
Metabolism▪ Galactosemia
▪ Fructosemia
▪ Glycogen Storage Disease Type IV
� Disorders of Amino Acid Metabolism▪ Tyrosinemia
▪ Hypermethioninemia
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INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES --
MetabolicMetabolic� Disorders of Lipid Metabolism
▪ Niemann-Pick disease
▪ Gaucher disease
� Disorders of Bile Acid Metabolism
▪ 3B-hydroxysteroid dehydrogenase/isomerase
▪ Trihydroxycoprostanic acidemia
� Peroxisomal Disorders
▪ Zellweger syndrome
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INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES --
MetabolicMetabolic
� Endocrine Disorders
▪Hypothyroidism
▪ Idiopathic hypopituitarism
� Miscellaneous Metabolic Disorders
▪Alpha-1-antitrypsin deficiency
▪ Cystic fibrosis
▪Neonatal iron storage disease
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EXTRAHEPATIC ETIOLOGIESEXTRAHEPATIC ETIOLOGIES
� Extrahepatic biliary atresia
� Choledochal cyst
� Bile duct stenosis
� Spontaneous perforation of the bile duct
� Cholelithiasis
� Inspissated bile/mucus plug
� Extrinsic compression of the bile duct
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CLINICAL PRESENTATIONCLINICAL PRESENTATION
AND DIAGNOSISAND DIAGNOSIS
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CLINICAL PRESENTATIONCLINICAL PRESENTATION
� Jaundice
� Scleral icterus
� Hepatomegaly
� Clay stools
� Dark urine
� Other signs and symptoms depend
on specific disease process
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DIAGNOSIS DIAGNOSIS -- IAPIAP
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DIAGNOSIS DIAGNOSIS -- NASPGHANNASPGHAN
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TREATMENTTREATMENT
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TREATMENT TREATMENT -- Medical ManagementMedical Management
� Nutritional support
� Treatment of pruritus
� Choleretics and bile acid-binders
� Management of portal hypertension and
its consequences
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Nutritional supportNutritional support
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IMPAIRMENT MANAGEMENT(NASPGHAN)
MANAGEMENTIAP
Fat soluble vit malabsorption
Vit A deficiency 10,000-15,000 IU/d AQUASOL-A
50,000 IU i.m –diagnosis10,000 IU monthly
Vit E deficiency 50-400 IU/d; oral alfatocopherol
50-200 mg/d orally
Vit D deficiency 5000 -8000IU/d of D23-5 mcg/kg/d of 25 HCC
30,000 IU i.m –diagnosis& monthly
Vit K deficiency 2.5 -5.0 mg alternate day as water soluble derivative of menadione.
5 mg/d im x3 days,5 mg wkly.Perform PT monthly.
Microutrientdeficiency
Ca, P, Zn supplementation Ca, P, Zn supplementation
Water soluble Vit def. 2 times RDA supplementation 2-5 times RDA supplementation
Treatment of Treatment of prurituspruritus
� Bile acid-binders: cholestyramine (4-8
g/day)
� Ursodeoxycholic acid (15-20 mg/kg/day)
� Phenobarbital (5mg/kg/day)
� Diphenhydramine (1-3 mg/kg/day)
� Photothearpy with UV/ Infrared rays x 3-
10 min/day
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Management of portal Management of portal
hypertension and its consequenceshypertension and its consequences� Variceal bleeding▪ Blood products
▪ Sclerotherapy
▪ Balloon tamponade
▪ Propranolol
� Ascites▪ Sodium restriction
▪ Diuretics (spironolactone, furosemide)
▪ Albumin
▪ Paracentesis
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KASAI PROCEDUREKASAI PROCEDURE
� Roux-en-Y
portoenterostomy
� Bile flow re-established in 80-90% if performed < 8 weeks-old.
� Bile flow re-established in less than 20% if performed > 12 weeks-old
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LIVER TRANSPLANTATIONLIVER TRANSPLANTATION
Indications:
�Decompensated liver disease(ascites
and/or encephalopathy) .
�Failed portoenterostomy.
▪ 1-year survival rate- 85-90%
▪ 5-8 year survival rate- 75-80%
▪ 1/3 to 1/2 patients are of Biliary Atresia
�Cost: In excess of 100,000 $
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LONG TERM OUTCOMELONG TERM OUTCOME
� Biliary Atresia: ▪ Mean survival in untreated pts : 19 months
▪ 3-year survival : <10%
� Neonatal Hepatitis:▪ > 60% of pts with idiopathis NH recover
completely without any specific therapy.
▪ > 10% die acutely of bleeding manifetstationsor fulminant hepatic failure.
▪ 30 % progress to liver cirrhosis and death.
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REFERENCEREFERENCE
� Indian Journal of Pediatrics, Volume 74—July, 2007
� NeoReviews Vol.14 No.2 February 2013
� Lien TH, Chang MH, Wu JF, et al; Taiwan Infant Stool Color Card Study Group.
Effects of the infant stool color card screening program on 5-year outcome of biliary
atresia in Taiwan. Hepatology.2011;53(1):202–208
� Canadian Family Physician • Le Médecin de famille canadien Vol 55: december •
décembre 2009
� Schreiber RA, Barker CC, Roberts EA, et al. Biliary atresia: the Canadian experience. J
Pediatr. 2007;151(6):659665, 665.e120. Nio M, Ohi R, Miyano T, Saeki M, Shiraki K,
Tanaka K;Japanese Biliary Atresia Registry. Five- and 10-year survival rates after
surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J
Pediatr Surg. 2003;38(7):997–1000
� Serinet MO, Wildhaber BE, Broué P, et al. Impact of age at Kasai operation on its
results in late childhood and adolescence: a rational basis for biliary atresia
screening. Pediatrics. 2009;123(5):1280–1286
� Prevention and Management of Gastroesophageal Varices and Variceal
Hemorrhage in Cirrhosis.HEPATOLOGY Vol. 46, No. 3, 2007.
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