Acute and Chronic liver

disease
Dr. Prima Paul
Dept. of Child Health

Introduction

Blood supply to the liver:

oxygenated blood flows in from the hepatic
artery
nutrient-rich blood flows in from the portal vein
(13 percent) of the body's blood supply at any
given moment.
two main lobes > lobules > small ducts >
larger ducts >> hepatic duct >>>transports
the bile produced by the liver cells to the
gallbladder and duodenum (the first part of the
small intestine).

All the blood leaving the stomach

and intestines passes through the
liver.
liver processes this blood and
breaks down the nutrients and
drugs into forms that are easier to
use for the rest of the body.
More than 500 vital functions have
been identified with the liver.

primary functions of the

liver:

Bile production and excretion

Excretion of bilirubin, cholesterol, hormones,
and drugs
Metabolism of fats, proteins, and carbohydrates
Enzyme activation
Storage of glycogen, vitamins, and minerals
Synthesis of plasma proteins, such as albumin,
and clotting factors
Blood detoxification and purification

Did you know ...

The liver can lose three-quarters of
its cells before it stops functioning?
In addition, the liver is the only
organ in the body that can
regenerate itself.

LIVER FUNCTION TESTS

Total bilurubin ,congugated bilirubin

Serum amino transferases :ALT,AST
Serum albumin
Alkaline phosphatase,
5nucleotidase,gamma glutamyl
transpeptidase
Prothrombin time

Typical AST or ALT

Values in Disease

imaging

Plain x ray:calcifications ,gallstones,gas

with in abcess,biliary tract or portal
circulation
Ultrasonography :size,composition and
blood flow,spenic size ,ascites
Doppler US :patency of portal vein and
collaterals
Radionucleotide scan:Tc 99m
imidodiacetic acid agents

LIVER BIOPSY

Histological diagnosis
Enzyme analysis
Analysis of stored material

Topics

Acute liver disease

Fulminant hepatic failure

Hepatic encephalopathy

Chronic liver disease

Case 1

5 YR OLD BOY
H/O Fever low grade for 5 days
anorexia+
vomiting+

O/E alert,no pallor /icterus

liver 4cm below RCM,soft ,tender

INV LFT 2.2/1.4/6.3/3.5/934/1345/335

PT
PTT

12/10
35/34

Etiology

Infections

Viral hepatitis

Hep A,B,D,E ( B+D )

EBV, HSV, Adenovirus, enterovirus, CMV,
varicella

Non viral causes

Bacteria: leptospira,salmonella,brucella
Protozoa: toxoplasma
Helminths:Echinococcus

Etiology

Drugs

Hepatotoxic drugs acetaminophen ( overdose)

Idiosyncratic damage sod. Val, prophthiouracil, halothane, INH,

tetracycline

Poisons

Amanita Phalloids

Carbon tetra chloride

Metabolic disorders

(Wilsons, Galactosemia, hereditary tyrosinemia, NIS, organic

acidemias,)

Ischemia and hypoxia

Circulatory shock, hepatic VOD, CCF, CHD

Clinical features

Prodrome of malaise
Anorexia
Nausea,vomiting
Fever
Tender hepatomegaly
Jaundice
Pale stools
Splenomegaly / lymphadenopathy

Hepatitis A

Asymptomatic infection(<5% have

identifiable illness)
Feco oral spread
Incubation period:2-6weeks
Clinical features
Anti HAV IgM
Good prognosis
=0.1% develop fulminant hepatic failure
Immunization :HAV vaccine

Hepatitis B

Perinatal exposure to HBsAg

positive mother
Majority if infections are
asymptomatic
Risk of chronic infection is
inversely related to age
Infected needles ,sexual contact

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

HBsAg: first serological marker

HBeAg:acute phase,highly infectious
IgManti HBcAg:acute HBV infection
antiHBsAg & anti HBcAg:resolving
infection
antiHBsAg(only):immunized with
Hep B vaccine

Fulminant hepatic failure

Definition

Clinical syndrome resulting from

massive necrosis of hepatocytes

or from severe functional impairment

of hepatocytes

in a patient who does not have preexisting liver disease

Definition

Evolves over a period of 8 weeks

Synthetic, excretory and detoxyfying

function of the liver are usually affected

Pathology

Liver biopsy

Patchy / confluent massive necrosis of

hepatocytes

Etiology

CAUSES OF ACUTE HEPATITIS

Clinical features

FHF may complicate previously known acute liver

disease

or may be the presenting feature of liver disease in a

previously well child

Fever, anorexia, vomiting, abd. pain

Progressive jaundice

Fetor hepaticus

Rapidly decreasing liver size

Ascites, hemorrhagic manifestations

Complications of FHF

Hepatic encephalopathy

Cerebral edema

Respiratory failure

Hypotension, Hypothermia

Infection

Bleeding

Renal failure

Metabolic

Diagnosis of FHF

Consists of

Evidence of acute onset of liver disease and

no evidence of CLD

Duration 8wks

No physical evidence of CLD

Biochemical evidence of hepatic dysfunction

Hepatic Encephalopathy

Hepatic encephalopathy

Hepatic encephalopathy

Neuropsychiatric syndrome

Occurs most often on pts. with cirrhosis

Can also occur in FHF

Hepatic encephalopathy

Factors ppt. hepatic encephalopathy

GI bleed

Excess dietary protein

Constipation

Paracentasis

Infections

Trauma

Uremia spont / diuretic induced

Electrolyte disturbances- hypokalemia

Portosystmeic shunts

Hepatic encephalopathy

Stages

Stage 1

Symptoms

Signs

Lethargy, euphoria, reversal of day and night

sleeping
Trouble drawing figures, performing mental
tasks

EEG

Normal

Hepatic encephalopathy

Stages

Stage 2

Symptoms

Signs

Drowsiness, inapp. Behaviour, agitation, wide

mood swings
Asterixis, fetor hepaticus, incontinence

EEG

Generalized slowing of waves

Hepatic encephalopathy

Stages

Stage 3

Symptoms

Signs

Stupor but arousable, confused, incoherent

speech
Asterixis, hyperreflexia, extensor reflexes,
rigidity

EEG

Markedly abnormal , triphasic waves

Hepatic encephalopathy

Stages

Stage 4

Symptoms

Signs

4a ( respond to noxious stimuli), 4b ( no

response)
Areflexia, no astereixs, flacidity

EEG

Markedly abnormal , b/l slowing of waves

Hepatic encephalopathy

Clinical signs

Fetor hepaticus

fecal smell in breath

Due to volatile substances normally
found in stool by bacteria

Asterixis

Impaired inflow of joint and afferent

information to the brainstem reticular
system, resulting in lapses in posture

Hepatic encephalopathy

Clinical signs

Constructional apraxia

Inability to reproduce simple designs

Reitan test

Multifactorial mechanism of
HE
Ammonia
Direct neural toxin

Encephalopathy
Ammonia
( glutamine)

False NTM

Tryptophan
( serotonin)

Endogenous BZD

Lab investigations

Etiology

IgM anti HBs

IgM anti HAV

Anti HEV, CMV, HS, EBV

Se.Ceruloplasmin , Copper

Auto Ab ANF, ANA, ASMA, LKM

USG liver + Doppler of hepatic vein

Toxicology screen of blood & urine

Lab investigations

LFT

TB / DB

AST / ALT/ALP

TP/ALB

PT/APTT

RBS. Electrolytes, ABG

Serum ammonia

Lab investigations

General tests

CBC, platelets

Creat, Ca, Mg

Infection surveillance

Cultures of blood and urine

Chest radiographs

Liver biopsy

Treatment

PICU

Cont. monitoring of vital signs/GRBS

Mechanical vent if required

IVF glucose ( 10 % D) 2/3 maint

Vit K / FFP to correct coagulopathy

Packed cells/platelets

Prophylactic antacids

Treatment

Measures to decrease ammonia

(Enteral neomycin)
Oral Ampiciliin )
Lactulose Q6h ) NG tube
Bowel wash

Mannitol to reduce cerebral edema

Cover for infections
Parentral suppl. Of Ca, Mg, Ph
Flumazenil

Management of FHF

Double volume exchange

Plasmapherasis

Extra corporeal blood cleansing

MARS

Activated charcoal

Cultured hepatocytes

Liver transplantation

Prognosis

No reliable criteria in children

Poor

NANB Hepatitis 10 % recovery

Higher stage of HE

Complications ( cerebral edema, renal failure)

Shrinking liver mass

PT > 50 secs

Prognosis

No reliable criteria in children

Good

HAV

Drug induced ( PCT)

Chronic liver disease

5 yrs old girl

Jaundice noticed since 3 yrs of age
Abd distension for 2yrs
2 episodes of hemetemesis
Admitted 2 times in hospital for altered sensorium
Transfused multiple plasma so far
O/E marasmic child,pale&icteric
bitots spot+
Liver not palpable span 4cms, spleen 8cm,
ascites+
Diagnosis
approach

ETIOLOGY

Metabolic disorders
Wilsons disease, alpha 1 anti trypsin def,
tyrosinemia ,NP disease, GSD type IV, cystic
fibrosis, galactosemia

Chronic viral hepatitis:B,C,D

Autoimmune hepatitis
Drug induced hepatitis
Bile acid biosynthetic abnormalities

History taking

Jaundice- onset, duration

Urine colour
Stool colour
Itching
Swelling site
Bleeding tendencies
Encephalopathy symptoms
History s/o cystic fibrosis

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

Family history :Similar history

(inherited metobolic disorder of
liver eg. Wilsons ICC, GSD, galact.)

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

Early and well-compensated cirrhosis

anorexia and weight loss, weakness,
fatigue, and even osteoporosis as a
result of vitamin D malabsorption and
subsequent calcium deficiency.
Decompensated disease
complications such as ascites,
spontaneous bacterial peritonitis,
hepatic encephalopathy, and variceal
bleeding from portal hypertension

complications of cirrhosis

ascites
spontaneous bacterial peritonitis
hepatic encephalopathy
portal hypertension
variceal bleeding
hepatorenal syndrome

KF Ring

Palmar erythema

Diagnosis

Chronic liver disease - etiology

Cirrohsis
Compensated vs decompensated
Liver cell failure
Portal hypertension
SBP
ENCEPALOPHATHY

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

USG liver size ,composition ,blood flowpatency of portal vein/collateral circulation

, stones in gb/ biliary tree, ascites
LIVER BX
Precise histological dsis neonatal
cholestasis,CAH,metabolic liver
disease,intrahepatic cholestasis,cong
hepatic fibrosis
Enzyme analysis to detect IEM
Stored material cu,iron,specific
metabolites

treatment

Nutrition low protien/MCT

Fat soluble vitamins-A 10-15,000IU/day
D2
5- 8000iu/day
E
50 400IU/day
K
2.5-5mg/Alt day
Micronutrient deficiency-Ca,Zn
Water soluble vit twice RDA
Ascitis-salt restriction+spironolactone
Pruritis- UDCA 15mg/kg/day
Portal hypertension - OGD

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

median age 5 - 20 years.

Liver disease: acute hepatitis, chronic
active hepative hepatitis, cirrhosis and
fulminant hepatitis
Recurrent episodes of liver disease may
occur in a variable interval (months to
years) until neurologic symptoms develop
Hemolytic anemia

Kayser-Fleischer ring denotes

neurologic impairment and
consists of copper deposition in the
cornea. It presents as a greenish or
golden brown ring around the
cornea and is pathognomonic of
Wilson's disease

Diagnosis

(1) Kayser-Fleischer rings,

(2) low serum ceruloplasmin (< 20
mg/dl)
(3) increased amounts of amounts
liver and urinary copper.

treatment

Should start as soon as possible,

either in symptomatic or
asymptomatics patient.
Total symptomatic recovery can be
acheived if intervention is made
early.
Penicillamine is the drug of choice.

THANK YOU