Download Liver Diseases

Prof. Asaad Abdullah Assiri
Professor of Pediatrics
Pediatric Gastroenterologist
Department of Pediatrics
College of Medicine
King Saud University
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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
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BILIARY ATRESIA
(continuation)
 INCIDENCE 1:15,000 LIVE BIRTHS
 CLINICALLY
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- 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
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BILIARY ATRESIA
(continuation)
TREATMENT:
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SURGERY
BEFORE 6 WEEKS OF AGE
KASAI OPERATION HEPATO PORTOENTEROSTOMY
LIVER TRANSPLANTATION
FAT SOLUBLE VIT. A, D, E, K
MCT
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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
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NEONATAL HEPATITIS
INFECTIOUS
IDIOPATHIC
GIANT CELL
 PRENATAL TORCHS
 POSTNATAL CMV, ECHOVIRUS
 TYPE II, GRAM NEGATIVE
 SEPTICAEMIA
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CLINICAL
PRESENTATION
 SGA
 PURPURA
 HEPATOSPLENOMEGALY
 CATARACT
 THROMBOCYTOPENIA
 LIVER BIOPSY MARKED INFILTRATE OF
INFLAMMATORY CELLS FOCAL
HEPATOCELLULAR NECROSIS, GIANT CELLS 11
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CONJUGATED NONCHOLESTATIC
HYPERBILIRUBINAEMIA
 DUBIN – JOHNSON SYNDROME
 ROTOR’S SYNDROME
 DEFECTIVE EXCRETION OF CONJUGATED
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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
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CONJUGATED
HYPERBILIRUBINAEMIA
(CH)
DIRECT
=
(DH)
 IT IS ALWAYS PATHOLOGICAL
 CLINICALLY
- PALE
- DARK URINE
- PRURITIS
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D.D. DIAGNOSIS OF
CONJUGATED
HYPERBILIRUBINAEMIA
 EXTRA HEPATIC BILE DUCT
OBSTRUCTION
- BILIARY ATRESIA
- CHOLEDOCHAL CYST
- SPONTANEOUS RUPTURE OF BILE
DUCT
- INSPISSATED BILE SYNDROME
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D.D. DIAGNOSIS OF
CONJUGATED
HYPERBILIRUBINAEMIA
 INTRA-HEPATIC CHOLESTASIS
WITH PAUCITY OF BILE DUCT
- ALAGILE SYNDROME
- NON-SYNDROMATIC PAUCITY OF
INTRA-HEPATIC
DUCTS
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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
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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
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CONJUGATED HYPERBILIRUBINAEMIA
EVALUATION (continuation)
 METABOLIC SCREEN-URINE / SERUM AMINO ACIDS; URINE FOR REDUCING
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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
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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
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VIRAL INFECTION OF THE LIVER
SIGNIFICANCE OF SEROLOGICAL
MARKERS OF VIRAL HEPATITIS
MARKER
SIGNIFICANCE
 HEPATITIS A: ACUTE HEPATITIS
Igm HAV Ab
(MAY BE POSITIVE FOR UP
 IgG HAV Ab
IMMUNITY TO HEPATITIS A
INFECTION, ACTIVE
PASSIVE IMUNIZATION
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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
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VIRAL INFECTION OF THE LIVER
SIGNIFICANCE OF SEROLOGICAL
MARKERS OF VIRAL HEPATITIS
(continuation)
MARKER
 HEPATITIS B:
 Igm HBcAb
 IgG HBcAb
SIGNIFICANCE
ACUTE OR CHRONIC
INFECTION
HIGH TITRE: ACUTE
LOW TITRE: CHRONIC
PAST EXPOSURE TO
CONTINUING HEPATITIS B
(IF HBsAb IS POSITIVE)
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VIRAL INFECTION OF THE LIVER
SIGNIFICANCE OF SEROLOGICAL
MARKERS OF VIRAL HEPATITIS
(continuation)
SIGNIFICANCE
 HBsAb
 HBeAg
 HBeAb
IMMUNITY TO HEPATITIS B,
OR WITH ACTIVE OR
IMMUNIZATION
HIGHLY INFECTIOUS STATE
CHRONIC INFECTION
LESS INFECTIVE STATE IN
POSITIVE PATIENT
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VIRAL INFECTION OF THE LIVER
SIGNIFICANCE OF SEROLOGICAL
MARKERS OF VIRAL HEPATITIS
(continuation)
MARKER
 HBVPOLYMERASE
 HBV DNA BY
DNA
 DANE
POSITIVE
SIGNIFICANCE
A MORE SENSITIVE
PERSISTING VIRAL
AN EVEN MORE
OF VIRAL REPLICATION
HIGHLY INFECTIOUS
OR CHRONIC INFECTION
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VIRAL INFECTION OF THE LIVER
SIGNIFICANCE OF SEROLOGICAL
MARKERS OF VIRAL HEPATITIS
(continuation)
MARKER
SIGNIFICANCE
 DANE
 DELTA AGENT
PAST INFECTION
 IgM DELTA
 IgG DELTA
CONTINUING DELTA
ACUTE OR CHRONIC
DELTA
PAST DELTA INFECTION
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ACUTE VIRAL
HEPATITIS
 HEPATITIS D: (DELTA VIRUS)
- HEPATITIS
- FULMINANT HEPATIC FAILURE
- LIVER CIRRHOSIS
- HEPATO-CELLULAR
CARCINOMA
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ACUTE VIRAL
HEPATITIS
HEPATITIS C:
-
CHRONIC LIVER DISEASES
LIVER CIRRHOSIS
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ACUTE VIRAL
HEPATITIS
HEPATITIS E:
- WATER
BORNE EPIDEMIC OF
HEPATITIS
- MILD ILLNESS
- NO CHRONOCITY ?
- MAYBE FULMINANT HEPATITIS
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CHRONIC PERSISTANT HEPATITIS
 HISTOLOGY:
- MONONUCLEAR CELL INFILTRATION
- NO ENCROACHMENT INTO THE PERIPORTAL AREA
 CLINICAL:
- ASYMPTOMATIC
- (INC.) LIVER ENZYME
 PROGNOSIS GOOD
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CHRONIC ACTIVE
HEPATITIS (CAH)
PATHOLOGY:
MONONUCLEAR AND PLASMA CELL
INFILTRATION OF THE PORTAL AND
PERIPORTAL AREAS OF THE LIVER
AND
DESTRUCTION
OF
THE
HEPATOCYTES.
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AUTOIMMUNE CAH
 CLINICAL
- FEMALE
- 10-25 YEARS OLD
- JAUNDICE
- CHRONIC LIVER DISEASE
- AUTO-IMMUNE HAEMOLYTIC
ANAEMIA
- AUTO-IMMUNE THYROIDITIS
- LEUCOPENIA, ANAEMIA
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INVESTIGATION
 AUTO ANTIBODIES
 (INC) LIVER ENZYME
 HLA – B8, HLADW3
 PX:
- STEROID
- AZATHIOPRINE
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HBV-INDUCED CHRONIC
ACTIVE HEPATITIS
 CHRONIC LIVER DISEASE
 HEPATO-CELLULAR
CARCINOMA
 PX:
- INTERFERONE
- VIDARABINE
- IMMUNO-SUPPRESSION
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METABOLIC LIVER
DISEASE
HYPOGLYCAEMIA
SPLENOMEGALY
JAUNDICE
LIVER CIRRHOSIS
HEPATITIS
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α1-ANTITRYPSIN
DEFICIENCY
 a ANTITRYPSIN
 A GYCO PROTEIN
 A POTENT INHIBITOR OF MANY PROTEOLYTIC
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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
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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
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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
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GALACTOSEMIA
 INH: AUTOSOMAL RECESSIVE GALACTOSE -1PHOSPHATE URIDYL TRANSFERAN
DEFICIENCY
 CLINICAL
- VOMITING
- DIARRHOEA
- HYPERBILIRUBINAEMIA
- CATARACT
- URINE POSITIVE FOR REDUCING SUBSTANCE
- RBCS GALACTOS 1 PHOSPHATE URIDYL
TRANSF (DEC)
 Px GALACTOSE FREE DIET
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WILSON DISEASE
 AUTOSOMAL RECESSIVE
 MANIFESTATIONS OF WILSON’S
DISEASE
 HEPATIC
- HEPATOMEGALY
- HEPATOSPLENOMEGALY
- JAUNDICE
- CHRONIC AGGRESSIVE HEPATITIS
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 EYE
- KAYSER – FLEISCHER RINGS
 HAEMATOLOGY
- HAEMOLYTIC ANAEMIA
 CNS
- BEHAVIOURAL DISTURBANCES,
 LABORATORY
- SERUM CAERULO PLASMIN (DEC)
- URINARY COPPER (INC)
- LIVER COPPER (INC)
 Px PENICILLAMINE
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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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 INVESTIGATION:
- ABDOMINAL U/S
- ABDOMINAL CT SCAN
- LIVER BIOPSY
 Px THE CAUSE
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PORTAL
HYPERTENSION
 UNCOMMON IN CHILDREN
 CAUSES:
 PRESINUSOIDAL
- IDIOPATHIC
- NEONATAL SEPSIS
- UMBILICAL VEIN CATHERIZATION
 CLINICAL:
- HEMATEMESIS
- SPLENOMEGALY
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 INTRAHEPATIC CIRRHOSIS
 SUPRAHEPATIC
- BUDD CHIARI SYNDROME (HEPATIC
VEIN THROMBOSIS)
- JAMAICAN VENO-OCCLUSIVE DISEASE
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MANAGEMENT
 Px BLEEDING
 I. V. VASOPRESSIN
 SCLEROTHERAPY
 SURGERY
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HEPATIC FAILURE
 AETIOLOGY
- VIRAL HEPATITIS A, B, C, E
- PARACETAMOL
INH, COTRIMOXAZOLE
Na VALPORATE, PHENYTOIN
- REYE’S SYNDROME
- WILSON DISEASE
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HEPATIC FAILURE
(continuation)
 CLINICAL
- DROWSINESS
- CONFUSION
- FLAPPING TREMOR
- COMA
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 Px
- CIMITIDINE
- RESPIRATORY SUPPORT
- HEMODIALYSIS
- Px CEREBRAL OEDEMA
- Px ENCEPHALOPATHY
- ORAL LACTULOSE
NEOMYCIN,
METRONIDAZOLE
- PROTEIN RESTRICTION
- FFP, VIT K
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REYE’S SYNDROME
 ENCEPHALOPATHY
 FATTY DEGENERATION OF THE
LIVER
 CLINICAL
- FLU LIKE ILLNESS
- APPARENT IMPROVEMENT VOMITING
- DETERIORATING CONSCIOUSNESS
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LABORATORY
FINDINGS
 (INC) LIVER ENZYME
 (INC) BLOOD AMONIN
 (DEC) Na+ (DEC) K+ (INC)
UREA
 HYPOGLYCAEMIA
 METABOLIC ALKALOSIS
 RESPIRATORY ALKALOSIS
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 Px
- SUPPORTIVE
- Px CEREBRAL OEDEMA
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