prof. asaad abdullah assiri professor of pediatrics pediatric gastroenterologist department of...
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Prof. Asaad Abdullah AssiriProfessor of Pediatrics
Pediatric GastroenterologistDepartment of Pediatrics
College of MedicineKing 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- POLYSPLENIA- ABSENT INFERIOR VENA CAVA
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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
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BILIARY ATRESIA (continuation)
TREATMENT: SURGERY
BEFORE 6 WEEKS OF AGE
KASAI OPERATION HEPATO PORTOENTEROSTOMY
LIVER TRANSPLANTATION
FAT SOLUBLE VIT. A, D, E, K
MCT7
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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 GIANT CELLIDIOPATHIC
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 CELLS11
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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
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CONJUGATED HYPERBILIRUBINAEMIA
(CH)DIRECT = (DH)
IT IS ALWAYS PATHOLOGICALCLINICALLY
- 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 18
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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 19
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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 NUTRITION20
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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 LEVEL AND PHENOTYPE.
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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
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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 CONTACT24
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VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF SEROLOGICAL MARKERS OF VIRAL HEPATITIS
MARKER SIGNIFICANCE HEPATITIS A: Igm HAV Ab
ACUTE HEPATITIS (MAY BE POSITIVE FOR UP TO ONE YEAR)
IgG HAV Ab IMMUNITY TO HEPATITIS A DUE TO PAST INFECTION, ACTIVE IMMUNIZATION OR 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 MARKERCLINICAL PRESENTATIONMANAGEMENT
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VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF
SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)
MARKER SIGNIFICANCE HEPATITIS B: HBsAg
ACUTE OR CHRONIC HEPATITIS B INFECTION
Igm HBcAb HIGH TITRE: ACUTE HEPATITISLOW TITRE: CHRONIC INFECTION
IgG HBcAb PAST EXPOSURE TO HEPATITIS B OR CONTINUING HEPATITIS B INFECTION (IF HBsAb IS POSITIVE)
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VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF
SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)
MARKER
SIGNIFICANCE
HBsAb IMMUNITY TO HEPATITIS B, POST-INFECTIVE OR WITH ACTIVE OR PASSIVE IMMUNIZATION
HBeAg HIGHLY INFECTIOUS STATE IN ACUTE OR CHRONIC INFECTION
HBeAb LESS INFECTIVE STATE IN THE HBsAb POSITIVE PATIENT
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VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF
SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)
MARKER SIGNIFICANCE HBV-SPECIFIC DNA POLYMERASE
A MORE SENSITIVE INDICATOR OF PERSISTING VIRAL INFECTION
HBV DNA BY DIRECT DNA HYBRIDIZATION
AN EVEN MORE SENSITIVE INDICATOR OF VIRAL REPLICATION
DANE PARTICLE POSITIVE
HIGHLY INFECTIOUS STATE IN ACUTE OR CHRONIC INFECTION
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VIRAL INFECTION OF THE LIVER SIGNIFICANCE OF
SEROLOGICAL MARKERS OF VIRAL HEPATITIS (continuation)
MARKER SIGNIFICANCE DANE ANTIBODY
PAST INFECTION
DELTA AGENT ACUTE OR CHRONIC INFECTION WITH DELTA
IgM DELTA ANTIBODY
CONTINUING DELTA INFECTION
IgG DELTA ANTIBODY
PAST DELTA INFECTION32
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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 GOOD37
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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
HYPOGLYCAEMIASPLENOMEGALYJAUNDICELIVER CIRRHOSISHEPATITIS
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α1-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
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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 TISSUE
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TYPE 1GLUCOSE -6- PHOSPHATASE
DEFICIENCYHYPOGLYCAEMIAHEPATOMEGALYMETABOLIC ACIDOSISHYPERLIPIDAEMIALABORATORY:
- (DEC) RESPONSE OF BLOOD SUGAR TO GLUCAGON- LIVER BIOPSY HISTOLOGY, ENZYME ASSAY
Px HIGH STARCH DIET46
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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 47
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WILSON DISEASEAUTOSOMAL RECESSIVEMANIFESTATIONS OF WILSON’S
DISEASEHEPATIC
- 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 HYPERTENSIONUNCOMMON IN CHILDRENCAUSES:
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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MANAGEMENTPx BLEEDING
I. V. VASOPRESSIN
SCLEROTHERAPY
SURGERY
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HEPATIC FAILUREAETIOLOGY
- 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
COMA
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LABORATORY FINDINGS
(INC) LIVER ENZYME
(INC) BLOOD AMONIN
(DEC) Na+ (DEC) K+ (INC)
UREA
HYPOGLYCAEMIA
METABOLIC ALKALOSIS
RESPIRATORY ALKALOSIS59
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Px
- SUPPORTIVE
- Px CEREBRAL OEDEMA
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