Docstoc

Cirrhosis

Document Sample
Cirrhosis Powered By Docstoc
					                    CIRRHOSIS
• Slow, progressive
• Fibrous bands replace
  normal liver structure
• Causes abnormal blood and
  lymph flow
  Disruption of the entire
  liver architecture by
• Fibrous septae
•   Liver nodules




                                1
        Incidence of Cirrhosis

• Tenth leading cause of death in US
• At least 25,000 deaths annually
• Higher death rates for men than women
        Causes of cirrhosis
      (Etiologic classification)
•   Alcoholic liver disease: 60-70%
•   Viral hepatitis: 10%
•   Biliary diseases: 5-10%
•   Hemochromatosis: 5%
•   Wilson’s disease: rare
•   α-1 antitrypsin deficiency:rare
•   Cryptogenic: 10-15%
• Cardiac cirrhosis
• Indian childhood cirrhosis          3
Morphological classification




  MICRONODULAR     MACRONODULAR

  Nodules < 3mm    Nodules > 3mm   4
Normal Liver Histology

                         CV




PT
      Cirrhosis


Fibrosis




Nodule
            Clinical features
• Clinically silent
• Nonspecific manifestations
  – Anorexia
  – Weight loss
  – Weakness
• Advanced disease
  – Debilitation
  – GI hemorrhage
  – Systemic infections
                    Effects
• Hepatocellular failure.
  - low albumin & clotting factor: ascites,
  bleeding
• Portal hypertension: due to increased
  resistance to portal blood flow
  – Ascites
  – Porta systemic shunts           TRIAD of Portal
                                     hypertension
  – splenomegaly.

                                                      8
Anatomy of the portal venous system
The Effect of The Liver Nodule
11
Collaterals
     Portosystemic shunts


• GE junction : Esophagogastric
  varices
• Rectum : Hemorrhoids
• Falciform ligament : Caput
  medusae

                                  13
       ESOPHAGEAL VARICES




- Cause of death due to rupture
   & massive hematemesis          14
CAPUT MEDUSAE




                15
HAEMORRHOIDS




               16
17
Cirrhosis : Clinical manifestations




                                      18
              Pathogenesis of ascites in Cirrhosis

- Portal   Hypertension

- Hypoalbuminemia

- Secondary hyperaldosteronism.




                                                     19
Ascites in Cirrhosis
  Cause: Hyperestrenism
as detoxification of estrogen
        is impaired




            SPIDER ANGIOMA




                                21
Cause: Hyperestrenism




PALMAR ERYTHEMA




                        22
GYNAECOMASTIA




                Cause: Hyperestrenism




                                        23
       Causes of death

• Massive haemetemesis
• Progressive liver failure :
  life threatening complications:
bleeding,encephalopathy,
hepatorenal syndrome
• Hepatocellular carcinoma
                                    24
                                          Porta systemic
                                              shunts




   Toxic metabolites
Toxic N2 N2 metabolites   Toxic N2 metabolites
From Intestines
   From Intestines        From Intestines
    Flapping Tremor




                   Asterexis
Stupor,coma
Foetor hepaticus
             Wilsons disease

 hepatolenticular degeneration
 Rare genetic disorder affecting copper
  metabolism ((1:30,000-1:300,000)
- mutation in copper transporting ATP7B gene-
  that codes for copper transporting ATP ase
  enzyme
- Autosomal recessive
- Accumulation of copper in tissues
- Predominantly in brain and liver
                                                27
ATP7B
Cu Metabolism
Liver, CNS, Eye and kidney affected:
Liver:
Acute/Chronic hepatitis
Cirrhosis




                                       30
Neuropsychiatric
manifestations            Spasticity

Extrapyramidal            Tremor
symptoms:

Ataxia,tremors,rigidity
personality changes




                          Contracture
                               31
Basal
ganglia




      32
Kayser-Fleischer ring
Deposition of copper in the Descemet’ s
membrane of cornea at limbus
                                          33
Sunflower cataract
                  Diagnosis
Clinical symptoms        Blood tests
                         Ceruloplasmin : decreased
K-F ring                 Urinary copper : increased
                         Liver biopsy copper levels:
                            increased




                        Rx
          COPPER CHELATORS: D -pencillamine

                                                       35
      Summary: Wilsons disease
• Rare genetic disorder
• Usually onset before age 25, but consider up
  to age 40
• Suspect if psych/neuro problems
• Screen with ceruloplasmin, reduced in 85%
• Also diagnosed by Kayser-Fleischer rings
• 24 hour urine for copper excretion – excretion
  of >100 g suggests Wilson’s
• Confirm by liver biopsy
    Alpha 1 antitrypsin deficiency
 produced in the liver
 most important function
   is to degrade the
   proteases (eg elastase)
   produced by the
   neutrophils at sites of
   inflammation
PiMM - normal genotype
Pi ZZ –severe deficiency

                                     37
Emphysema
Cirrhosis of liver


Investigations:

Decreased serum alpha
1 anti-trypsin




                        PAS positive globules in the liver
                                                             38
   HEREDITARY
HEMOCHROMATOSIS
       HEREDITARY HEMOCHROMATOSIS
• Autosomal recessive disorder
• Mutations in the HFE gene
• One of these mutations results in a cysteine-to-tyrosine
  substitution at amino acid 282 (C282Y)
• The most common genetic disorder in the US white
  population:
   – heterozygote carriers is about 10%
   – homozygous state is approx 1 in 250 to 300
• Increased intestinal iron absorption
• Deposition of excessive amount of iron into the liver,
  pancreas, and other organs

                                                             40
Haemochromatoses




H&E         Prussian Blue

                            41
           CLINICAL MANIFESTATIONS

• Classic bronze diabetes:
• hyperpigmentation, diabetes mellitus, liver
  cirrhosis
• Other Clinical features:
  – fatigue
  – arthropathy
  – hypogonadism (impotence in males)
  – cardiomyopathy

                                                42
43
       DIFFERENTIAL DIAGNOSIS


  HHC should be distinguished from
  secondary iron overload:
• multiple blood transfusions
• parenteral or oral iron supplements




                                        44
        DIAGNOSIS OF IRON OVERLOAD

  A diagnosis of HHC is suggested by:

• Elevated transferrin saturation

• Elevated ferritin


• Diagnosis of iron overload is liver biopsy

• Genetic screening
                                               45
Rx




     Iron chelators - Dysferrioxime   46
          Biliary Cirrhosis

Cirrhosis occuring due to bile duct
    obstruction
- Primary
- Secondary
                Conditions
• Primary biliary Cirrhosis : auto –immune
 condition
• Secondary biliary cirrhosis
 Extrahepatic biliary obstruction – by gall
 stone, Ca head of pancreas etc
         Primary Biliary Cirrhosis
• Auto-immune-mediated
  attack on bile ducts that
  leads to their gradual
  destruction and
  eventual disappearance.
• 95 % - women
• onset is usually
  between 30 to 65 yrs
• Presence of
  antimitochondrial
  antibodies
                   GROSS




   Gross: Liver is green in colour (due to bile
stasis).
    Dense portal tract
    inflammation –
    composed of
    lymphocytes,
    macrophages, plasma
    cells
    Epithelioid
    Granulomas in the
    portal tracts may be
    seen
.
• Clinical features:
• Insidious onset - Pruritus, Jaundice, Hepatomegaly,
  Xanthoma and Xanthelasma (due to cholesterol
  retention) Laboratory findings:
• 1. Alkaline phosphatase - increased
• 2. Cholesterol - increased
• 3. Bilirubin - increased
• 4. Antimitochondrial antibody –seen in more than
  90% -95% of patients.
             Causes:
SECONDARY
             In Adults:
  BILIARY
             • Gall stones
 CIRRHOSIS
             • Carcinoma of Common bile
                  duct, ampulla of Vater and
                  head of pancreas
             • Stricture due to previous
                  surgery
             In Children:
             • Biliary atresia
Cardiac Cirrhosis

•   Seen with right sided heart failure
•   Venous congestion results in anoxia
•   Cell necrosis results
•   Cirrhosis

				
DOCUMENT INFO
Shared By:
Tags:
Stats:
views:282
posted:12/1/2011
language:English
pages:55