Professional Documents
Culture Documents
2009
Definition
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Differential diagnosis
Alagille’s syndrome.
Nonsyndromic ductal paucity.
Ductopenic allograft rejection.
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3-Hepatocellular cholestasis:
- Hepatitis.
_ alpha-1 antitrypsin deficiency.
- Inborn errors of bile acid synthesis.
- Drug-induced cholestasis.
- TPN associated cholestasis.
-Progressive familial intrahepatic cholestasis.
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Pathology:
1- Parenchyma:
- Disarray of lobular architecture.
- Focal necrosis.
- Ballooning of the cells.
- Giant cell.
2- Portal tracts:
- Expanded.
- Inflammatory cells lymphocytes and PMNs.
- Fibrosis.
- Extramedullary hematoposesis.
- Few bile ducts/ductules.
3- Bile stasis:
- Present in hepatocytes and canaliculi.
- Absent in portal tract.
Prognosis
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Etiology
Reovirus type 3 has been suggested with no
confirmation.
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Clinical presentation
Stool: acholic.
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Spleen: firm enlarged.
Laboratory findings
B- Hepatobiliary scintigraphy
C- Liver biopsy.
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1- History and physical examination:
1- Irritability, poor feeding and vomiting:
- Generalized infection.
- Metabolic disorders:
- Galactosemia. - Tyrosinemia.
2- Vertebral arch anomalies, post embryotoxon and
the murmur of peripheral pulmonic stenosis:
arteriohepatic dysplasia (alagille’s syndrome).
3- Stool examination:
- Acholic stool persistent suggest BA for 10 days.
- Pigmeneted stool exclude BA.
Laboratory evaluation:
1- Urine – reducing substances (+ve in galactosemia).
2-↓ RBC galactose -1- phosphate uridyl transferase
activity = galactosemia.
3-↑serum succinylacetone, succinylacetoactic
“(tyrosinemia).
4-Alpha- antirypsin phenotyping for alpha-1
antitrypsin deficiency.
5- TORCH:
- Low diagnostic yield.
- Culture is more specific.
6- Duodenal intubation with analysis of fluid for
bilirubin content: green or pigmental fliud exclude
BA.
3- Radiological evaluation:
1- Ultrasonography:
- Choledochal cyst.
- Absence of a gallbladder → BA.
2- Radionuclide imaging: TC labeled iminodiacetic
acid:
BA:
- Radioisotope uptake by hepatocytes is good.
- Absent intestinal excretion.
Neonatal hepatitis:
- Radioisotope uptake by hepatocytes is delayed.
- Intestinal excretion is +ve.
Pretreatment with oral phenobarbital (5mg/kg/day) for 5 days
enhances biliary excretion of the isotope and increases
sensitivity 94%.
4- PTC & ERCP:
Per cuteneous transhepatic cholangiography.
Endoscopic retrograde cholongio pancreatography.
5- Liver biopsy:
Most reliable and definitive procedure.
Correct diagnosis in 90-95% of cases by
experienced pathologist.
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Diminished bile flow will lead to:
1- Retention of substances dependent on bile
secretion bile acids, bilirubin and
cholesterol.
2- Decreased bile acid delivery to the intestine
fat and fat-soluble vitamin malabsorption.
3-Progressive hepatocellular damage → portal
hypertension → liver failure.
Management:
No specific therapy either reverse existing
cholestasis or prevent ongoing damage.
Therapy is empirical and aim at improving
nutritional status and maximizing growth
potential and minimizing discomfort.
The success of this therapeutic intervention is
limited by:
A- Residual capcity of the liver.
B- Rate of progression of the underlying disorder.
Malabsorption and malnutrition:
Decreased intraluminal bile acids.
Long-chain triglyceride → not absorbed.
Medium-chain triglyceride → readily
absorbed.
Fat-soluble vitamins (A,D,E and K): 2-4 times
the daily requirement.
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Recommended oral vitamin supplementation: