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Liver Diseases

Liver Diseases. Prof. Asaad Abdullah Assiri Professor of Pediatrics Pediatric Gastroenterologist Department of Pediatrics College of Medicine King Saud University. BILIARY ATRESIA. Etiology / Pathology

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Liver Diseases

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  1. Liver Diseases Prof. Asaad Abdullah Assiri Professor of Pediatrics Pediatric Gastroenterologist Department of Pediatrics College of Medicine King Saud University

  2. BILIARY ATRESIA • Etiology / Pathology • Progressive panductularsclerotic process that may continue in the intrahepatic duct even after surgical relief of biliary obstruction • Intra-uterine reovirus Type III infection • 10%-15% incidence of associated anomalies - pre-duodenal portal view - intestinal malrotation - polysplenia - absent inferior vena cava

  3. BILIARY ATRESIA (continuation) • Incidence 1:15,000 live births • Clinically - well - jaundice 2 weeks • Laboratory Investigation: • 99m TC iminodiacetic acid (IDA scan) - slow uptake with no or delayed excretion (parenchymal dysfunction) - rapid hepatocyte uptake with no intestinal EXCRETION (EXTRA HEPATIC OBSTRUCTION) • ABDOMINAL ULTRASOUND • LIVER BIOPSY • OPERATIVE CHOLANGIOGRAPHY

  4. BILIARY ATRESIA (continuation) TREATMENT: • SURGERY • BEFORE 6 WEEKS OF AGE • KASAI OPERATION HEPATO PORTOENTEROSTOMY • LIVER TRANSPLANTATION • FAT SOLUBLE VIT. A, D, E, K • MCT

  5. BILIARY ATRESIA (continuation) PROGNOSIS OF KASAI OPERATION • 10% NO BILE DRAINAGE • 90% BILE DRAINAGE - 1/3 FAIL SEVERE LIVER DAMAGE - 1/3 INDETERMINATE – MODERATE LIVER DISEASE - 1/3 CURED – MINIMAL LIVER DISEASE

  6. NEONATAL HEPATITIS INFECTIOUS GIANT CELL IDIOPATHIC • PRENATAL TORCHS • POSTNATAL CMV, ECHOVIRUS • TYPE II, GRAM NEGATIVE • SEPTICAEMIA

  7. CLINICAL PRESENTATION • SGA • PURPURA • HEPATOSPLENOMEGALY • CATARACT • THROMBOCYTOPENIA • LIVER BIOPSY MARKED INFILTRATE OF INFLAMMATORY CELLS FOCAL HEPATOCELLULAR NECROSIS, GIANT CELLS

  8. CONJUGATED NON-CHOLESTATIC HYPERBILIRUBINAEMIA • DUBIN – JOHNSON SYNDROME • ROTOR’S SYNDROME • DEFECTIVE EXCRETION OF CONJUGATED BILIRUBIN FROM HEPATOCYTE • NORMAL HANDLING OF BILE ACID • NORMAL LFTA • MILD CONJUGATED HYPERBILIRUBINAEMIA • LIVER BIOPSY - NORMAL IN ROTORS - PIGMENTED GRANULE IN DUBBIN-JOHNSON • PROGNOSIS EXCELLENT

  9. CONJUGATED HYPERBILIRUBINAEMIA (CH) DIRECT = (DH) • IT IS ALWAYS PATHOLOGICAL • CLINICALLY - PALE - DARK URINE - PRURITIS

  10. D.D. DIAGNOSIS OF CONJUGATED HYPERBILIRUBINAEMIA • EXTRA HEPATIC BILE DUCT OBSTRUCTION - BILIARY ATRESIA - CHOLEDOCHAL CYST - SPONTANEOUS RUPTURE OF BILE DUCT - INSPISSATED BILE SYNDROME

  11. D.D. DIAGNOSIS OF CONJUGATED HYPERBILIRUBINAEMIA • INTRA-HEPATIC CHOLESTASIS WITH PAUCITY OF BILE DUCT - ALAGILE SYNDROME - NON-SYNDROMATIC PAUCITY OF INTRA-HEPATIC DUCTS

  12. D.D. DIAGNOSIS OF CONJUGATED HYPERBILIRUBINAEMIA • INTRA-HEPATIC CHOLESTASIS WITH NORMAL BILE DUCT - GIANT CELL HEPATITIS - INFECTIOUS AGENTS - CMV, RUBELLA, HERPES - METABOLIC: * GALACTOSEMIA * a1 ANTITRYPSIN DEFICIENCY * CEREBRO HEPATORENAL SYNDROME (ZELLWEGER SYNDROME) * RECURRENT FAMILIAL CHOLESTASIS (BYLER DISEASE) * TOTAL PARENTAL NUTRITION

  13. CONJUGATED HYPERBILIRUBINAEMIA EVALUATION • FRACTIONATE SERUM BILIRUBIN • SERUM TRANSMINASES, ALKALINE PHOSPHATASE (OR 5’ – NUCLEOTIDASE), ALBUMIN CHOLESTEROL PROTHROMBIN TIME • STOOL COLOR • CULTURES (BLOOD, URINE, ETC.) • HEPATITIS B SURFACES ANTIGEN, TORCH TITERS, VDRL SERUM a1-ANTITRYPSIN LEVEL AND PHENOTYPE.

  14. CONJUGATED HYPERBILIRUBINAEMIA EVALUATION (continuation) • METABOLIC SCREEN-URINE / SERUM AMINO ACIDS; URINE FOR REDUCING SUBSTANCE • THYROID SCREEN • OPHTHALMOLOGIC EXAMINATION • SWEAT CHLORIDE • SKULL, LONG BONES, ABDOMINAL AND CHEST X-RAY FILMS ABDOMINAL ULTRASOUND • DUODENAL INTUBATION (STRING TEST FOR COLOR, BILIRUBIN, BILE ACIDS) • HEPATOBILIARY SCINTIGRAPHY • PERCUTANEOUS LIVER BIOPSY

  15. ACUTE VIRAL HEPATITIS HEPATITIS A: • I. P. 6 WEEKS • TRANSMISSION FOETAL – ORAL ROUT • NO CHRONIC CARRIER STATE • LAB: Igm SPECIFIC ANTI HAV MANAGEMENT: • ISOLATION • BED REST • PERSONAL HYGIENE • HUMAN IMMUNOGLOBULIN 0.02 ml/kg FOR CONTACT

  16. VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS

  17. ACUTE VIRAL HEPATITIS • HEPATITIS B: - I. P. 150-180 DAYS • SOURCES OF INFECTION: - BLOOD TRANSFUSION - DIRECT CONTACT WITH CASES • VIRUS AND VIRUS MARKER • CLINICAL PRESENTATION • MANAGEMENT

  18. VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)

  19. VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)

  20. VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)

  21. VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)

  22. ACUTE VIRAL HEPATITIS • HEPATITIS D: (DELTA VIRUS) - HEPATITIS - FULMINANT HEPATIC FAILURE - LIVER CIRRHOSIS - HEPATO-CELLULAR CARCINOMA

  23. ACUTE VIRAL HEPATITIS • HEPATITIS C: - CHRONIC LIVER DISEASES - LIVER CIRRHOSIS

  24. ACUTE VIRAL HEPATITIS • HEPATITIS E: - WATER BORNE EPIDEMIC OF HEPATITIS - MILD ILLNESS - NO CHRONOCITY ? - MAYBE FULMINANT HEPATITIS

  25. CHRONIC PERSISTANT HEPATITIS • HISTOLOGY: - MONONUCLEAR CELL INFILTRATION - NO ENCROACHMENT INTO THE PERIPORTAL AREA • CLINICAL: - ASYMPTOMATIC - (INC.) LIVER ENZYME • PROGNOSIS GOOD 37

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  27. CHRONIC ACTIVE HEPATITIS (CAH) • PATHOLOGY: MONONUCLEAR AND PLASMA CELL INFILTRATION OF THE PORTAL AND PERIPORTAL AREAS OF THE LIVER AND DESTRUCTION OF THE HEPATOCYTES.

  28. AUTOIMMUNE CAH • CLINICAL - FEMALE - 10-25 YEARS OLD - JAUNDICE - CHRONIC LIVER DISEASE - AUTO-IMMUNE HAEMOLYTIC ANAEMIA - AUTO-IMMUNE THYROIDITIS - LEUCOPENIA, ANAEMIA

  29. INVESTIGATION • AUTO ANTIBODIES • (INC) LIVER ENZYME • HLA – B8, HLADW3 • PX: - STEROID - AZATHIOPRINE

  30. HBV-INDUCED CHRONIC ACTIVE HEPATITIS • CHRONIC LIVER DISEASE • HEPATO-CELLULAR CARCINOMA • PX: - INTERFERONE - VIDARABINE - IMMUNO-SUPPRESSION

  31. METABOLIC LIVER DISEASE • HYPOGLYCAEMIA • SPLENOMEGALY • JAUNDICE • LIVER CIRRHOSIS • HEPATITIS

  32. 1-ANTITRYPSIN DEFICIENCY • a ANTITRYPSIN • A GYCO PROTEIN • A POTENT INHIBITOR OF MANY PROTEOLYTIC ENZYMES 20 PHENOTYPES • Pi ZZ > LIVER DISEASE • CLINICAL • CONJUG. HYPERBILIRUBINAEMIA • HEPATOMEGALY • HEPATIC FAILURE • LIVER CIRRHOSIS, PORTAL HYPERTENSION - LIVER BIOPSY PERIODIC ACID – SCHIFF – POSITIVE DIASTASE RESISTANT GRANULES IN PERIPORTAL HEPATOCYTE - BIOCH 1 ANTITRYPSIN PHENOTYPE

  33. GLYCOGEN STORAGE DISEASE • ACCUMULATION OF GLYCOGEN IN THE LIVER, MUSCLES AND KIDNEY • 10 VARIANTS ARE RECOGNIZED • THE DIAGNOSIS IS CONFIRMED BY ENZYME ASSAY IN LIVER TISSUE

  34. TYPE 1 • GLUCOSE -6- PHOSPHATASE DEFICIENCY • HYPOGLYCAEMIA • HEPATOMEGALY • METABOLIC ACIDOSIS • HYPERLIPIDAEMIA • LABORATORY: - (DEC) RESPONSE OF BLOOD SUGAR TO GLUCAGON - LIVER BIOPSY HISTOLOGY, ENZYME ASSAY • Px HIGH STARCH DIET

  35. GALACTOSEMIA • INH: AUTOSOMAL RECESSIVE GALACTOSE -1- PHOSPHATE URIDYL TRANSFERAN DEFICIENCY • CLINICAL - VOMITING - DIARRHOEA - HYPERBILIRUBINAEMIA - CATARACT - URINE POSITIVE FOR REDUCING SUBSTANCE - RBCS GALACTOS 1 PHOSPHATE URIDYL TRANSF (DEC) • Px GALACTOSE FREE DIET

  36. WILSON DISEASE • AUTOSOMAL RECESSIVE • MANIFESTATIONS OF WILSON’S DISEASE • HEPATIC - HEPATOMEGALY - HEPATOSPLENOMEGALY - JAUNDICE - CHRONIC AGGRESSIVE HEPATITIS

  37. EYE - KAYSER – FLEISCHER RINGS • HAEMATOLOGY - HAEMOLYTIC ANAEMIA • CNS - BEHAVIOURAL DISTURBANCES, • LABORATORY - SERUM CAERULO PLASMIN (DEC) - URINARY COPPER (INC) - LIVER COPPER (INC) • Px PENICILLAMINE

  38. LIVER CIRRHOSIS • WIDESPREAD HEPATIC FIBROSIS • CLINICAL: - FAILURE TO THRIVE - HEPATOSPLENOMEGALY - SPLENOMEGALY - HEPATIC ENCEPHALOPATHY - SIGNS OF CHRONIC LIVER DISEASE - MAYBE ONLY SPLENOMEGALY - MAYBE NORMAL LABORATORY FINDING

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