NEONATAL CHOLESTASIS Choledochalcyst Bile duct stenosis Spontaneous perforation of the bile duct...

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Transcript of NEONATAL CHOLESTASIS Choledochalcyst Bile duct stenosis Spontaneous perforation of the bile duct...

  • NEONATAL CHOLESTASISNEONATAL CHOLESTASIS

    張堯婷張堯婷

  • CONTENTSCONTENTS

    � Definition

    � Etiologies

    � Clinical Presentation And Diagnosis

    � Treatment

    � Outcome

    � Reference

    2

  • DEFINITIONDEFINITION

    3

  • NASPGHAN Definition NASPGHAN Definition

    � Conjugated bilirubin concentration

    >1.0 mg/dL if the total serum bilirubin is 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

    6

  • INTRAHEPATIC ETIOLOGIESINTRAHEPATIC ETIOLOGIES

    7

  • 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

    9

  • INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES --

    InfectiousInfectious � Bacterial sepsis

    ▪ E. coli

    ▪ Listeriosis

    ▪ Staph. aureus

    � TORCHES

    � Echo virus

    � Hepatitis B and C

    10

  • 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

    11

  • INTRAHEPATIC ETIOLOGIESINTRAHEPATIC ETIOLOGIES --

    MetabolicMetabolic � Disorders of Carbohydrate

    Metabolism ▪ Galactosemia

    ▪ Fructosemia

    ▪ Glycogen Storage Disease Type IV

    � Disorders of Amino Acid Metabolism ▪ Tyrosinemia

    ▪ Hypermethioninemia

    12

  • 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

    13

  • INTRAHEPATIC ETIOLOGIES INTRAHEPATIC ETIOLOGIES --

    MetabolicMetabolic

    � Endocrine Disorders

    ▪Hypothyroidism

    ▪ Idiopathic hypopituitarism

    � Miscellaneous Metabolic Disorders

    ▪Alpha-1-antitrypsin deficiency

    ▪ Cystic fibrosis

    ▪Neonatal iron storage disease

    14

  • 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

    15

  • CLINICAL PRESENTATIONCLINICAL PRESENTATION

    AND DIAGNOSISAND DIAGNOSIS

    16

  • 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

    18

  • DIAGNOSIS DIAGNOSIS -- NASPGHANNASPGHAN

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  • 20

  • 21

  • 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)

    MANAGEMENT IAP

    Fat soluble vit malabsorption

    Vit A deficiency 10,000-15,000 IU/d AQUASOL-A

    50,000 IU i.m –diagnosis 10,000 IU monthly

    Vit E deficiency 50-400 IU/d; oral alfa tocopherol

    50-200 mg/d orally

    Vit D deficiency 5000 -8000IU/d of D2 3-5 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.

    Microutrient deficiency

    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

    25

  • 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

    26

  • 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 : 60% of pts with idiopathis NH recover

    completely without any specific therapy.

    ▪ > 10% die acutely of bleeding manifetstations or fulminant hepatic failure.

    ▪ 30 % progress to liver cirrhosis and death.

    29

  • 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.

    30

  • THANKS FOR LISTENINGTHANKS FOR LISTENING

    Special thanks for kido1256. 2015/07/13 produced by Sakurai Haruka

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