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disease
Dr. Prima Paul
Dept. of Child Health
Introduction
imaging
LIVER BIOPSY
Histological diagnosis
Enzyme analysis
Analysis of stored material
Topics
Hepatic encephalopathy
Case 1
5 YR OLD BOY
H/O Fever low grade for 5 days
anorexia+
vomiting+
12/10
35/34
Etiology
Infections
Viral hepatitis
Etiology
Drugs
Poisons
Amanita Phalloids
Metabolic disorders
Clinical features
Prodrome of malaise
Anorexia
Nausea,vomiting
Fever
Tender hepatomegaly
Jaundice
Pale stools
Splenomegaly / lymphadenopathy
Hepatitis A
Hepatitis B
Pathogenesis
Hepatocyte injury is by immune
mediated response
Severity of hepatocyte injury
reflects degree of immune
response
Extrahepatic manifestation
Clinical features
asymptiomatic,acute hepatitis
,skin and joint manifestations,68weeeks course
Fulminant hepatitis,chronic
hepatitis,cirrhosis and
hepatocellular ca
Diagnosis
Definition
Definition
Pathology
Liver biopsy
Etiology
Clinical features
Progressive jaundice
Fetor hepaticus
Complications of FHF
Hepatic encephalopathy
Cerebral edema
Respiratory failure
Hypotension, Hypothermia
Infection
Bleeding
Renal failure
Metabolic
Diagnosis of FHF
Consists of
Duration 8wks
Hepatic Encephalopathy
Hepatic encephalopathy
Hepatic encephalopathy
Neuropsychiatric syndrome
Hepatic encephalopathy
GI bleed
Constipation
Paracentasis
Infections
Trauma
Portosystmeic shunts
Hepatic encephalopathy
Stages
Stage 1
Symptoms
Signs
EEG
Normal
Hepatic encephalopathy
Stages
Stage 2
Symptoms
Signs
EEG
Hepatic encephalopathy
Stages
Stage 3
Symptoms
Signs
EEG
Hepatic encephalopathy
Stages
Stage 4
Symptoms
Signs
EEG
Hepatic encephalopathy
Clinical signs
Fetor hepaticus
Asterixis
Hepatic encephalopathy
Clinical signs
Constructional apraxia
Reitan test
Multifactorial mechanism of
HE
Ammonia
Direct neural toxin
Encephalopathy
Ammonia
( glutamine)
False NTM
Tryptophan
( serotonin)
Endogenous BZD
Lab investigations
Etiology
Se.Ceruloplasmin , Copper
Lab investigations
LFT
TB / DB
AST / ALT/ALP
TP/ALB
PT/APTT
Serum ammonia
Lab investigations
General tests
CBC, platelets
Creat, Ca, Mg
Infection surveillance
Chest radiographs
Liver biopsy
Treatment
PICU
Packed cells/platelets
Prophylactic antacids
Treatment
(Enteral neomycin)
Oral Ampiciliin )
Lactulose Q6h ) NG tube
Bowel wash
Management of FHF
Plasmapherasis
MARS
Activated charcoal
Cultured hepatocytes
Liver transplantation
Prognosis
Poor
Higher stage of HE
PT > 50 secs
Prognosis
Good
HAV
ETIOLOGY
Metabolic disorders
Wilsons disease, alpha 1 anti trypsin def,
tyrosinemia ,NP disease, GSD type IV, cystic
fibrosis, galactosemia
History taking
History taking
appetite,nausea,vomiting,wt loss
Night blindness
Renal symptoms
Endocrine symptoms
Involuntary movements
Fever and abd pain
Effect of disease in school and family
HISTORY
H/o transfusions
Recurrent LRI
Malabsorption
Drug history
ATT
Sodium valporate
Chemotherapy
COOKING UTENSILS
O/E examination
Mental state
Pallor,Icterus ,clubbing
Signs of liver cell failure ascites ,FTT
Vit def
xanthomata
Asterixis
Leuconychia
Examination
Cataract
KF ring
Facies
Veins
Liver span
Free fluid
spleen
complications of cirrhosis
ascites
spontaneous bacterial peritonitis
hepatic encephalopathy
portal hypertension
variceal bleeding
hepatorenal syndrome
KF Ring
Palmar erythema
Diagnosis
Investigations
GRBS , LFT
HB TC DC PLAT BP MCV
PT PTT RBS NA K HCO3 Creat BLOOD C/S
VIRAL MARKERS
CERULOPASMIN,24hr urine Cu
Alpha 1 antitrypsin levels
Sweat electrolytes
OPTHAL
Investigations
treatment
Wilson's disease
hepatolenticular degeneration
autossomal recessive
acumulation of copper in tissues all
over the body, mainly in the liver,
brain, kidneys and cornea.
Clinical Manifestations
Diagnosis
treatment
THANK YOU