Liver Cirrhosis and Wilson disease.. Types causes clinical manifestations complications..
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LIVER CIRRHOSIS SUBMITED BY :- DIVYA DORA SUBMITTED TO :- Dr . Suruchi
CIRRHOSIS It represent the Irreversible end-stage of several diffuse diseases causing hepatocellular injury. It is Characterized by:- It involves the entire liver. Normal lobular architecture of hepatic parenchyma is disorganized. Formation of nodules separated from one another by irregular bands of fibrosis. It occurs following hepatocellular necrosis of varying etiology so that there are alternate areas of necrosis and regenerative nodules.
PATHOPHYSIOLOGY OF LIVER CIRRHOSIS Continued destruction of hepatocytes . Collapse of normal lobular hepatic parenchyma. Proliferation of fat storing Ito cells which are transformed into myofibroblasts and fibrocytes. Increased synthesis of type I and III collagen in the space of Disse. Fibrosis in the liver lobules portal-central, portal, or both.
CLASSIFICATION OF LIVER CIRRHOSIS MORPHOLOGIC Micronodular (nodules less than 3mm). Macronodular (nodules more than 3mm). Mixed. ETIOLOGIC Alcoholic cirrhosis (the most common, 60-70%). Post-necrotic cirrhosis (10%). Biliary cirrhosis (5-10%). Pigment cirrhosis in haemochromatosis (5%). Cirrhosis in alpha-1-antirypsin deficiency. Cirrhosis in Wilson's diseases. Cardiac cirrhosis. Indian childhood cirrhosis (ICC). Cirrhosis in autoimmune hepatitis. Cirrhosis in non-alcoholic forms of cirrhosis (metabolic, infectious, GI, infiltrative) diseases. Cryptogenic cirrhosis.
ALCOHOLIC LIVER DISEASE AND CIRRHOSIS Alcoholic liver disease is the term used to described the spectrum of liver injury associated with acute and chronic alcoholism. Acetaldehyde is toxic and may cause membrane damage and cell necrosis.
ALCOHOLIC STEATOSIS (FATTY LIVER) Initial micro vesicular droplets of fat in the hepatocyte cytoplasm. Followed by macro vesicular large droplets of fat displacing the nucleus to the periphery. Fat cysts may develop due to the coalescence and rupture of fat-containing hepatocytes.
ALCOHOLIC HEPATITIS Alcoholic hepatitis acutely, usually following a bout of heavy drinking. Ballooning degeneration and necrosis. Inflammatory response: Polymorphs and scattered.
ALCOHOLIC CIRRHOSIS Alcohol cirrhosis is the most common form of lesion, constituting 60-70% of all cases of cirrhosis. Normal lobular architecture is effect is replaced with nodule formation . Fibrous septa. Inflammation and bile duct proliferation.
WILSON'S DISEASE Copper accumulates in the liver, kidneys, brain, and eyes Autosomal recessive disease of copper metabolism. Young patients: Symptoms of liver disease, Older patients: neuropsychic symptoms. KAISER-FLEISCHER RING Deposition of copper in cornea. HEPATIC : Most common organ. Fatty change. Acute and chronic hepatitis. Cirrhosis. CNS : Basal ganglia is involved primarily. There is dystonia, dysarthria, tremor and chorea. OCULAR INVOLVEMENT : Kayser-Fleischer ring (brown/green deposit in Descemet's membrane of cornea)
Wilson disease. Mutation in ATP7B gene present on chromosome 13. So copper accumulation in liver (primarily) and there is decrease in circulating ceruloplasmin. The accumulated copper causes toxic liver injury. Spillage of non-ceruloplasmin-bond copper from the liver into the circulation. Deposited in extrahepatic tissue such as the brain, eyes.
DIAGNOSIS Liver biopsy with quantitative copper assay The gold standard for diagnosis is liver biopsy with quantitative copper assay connected of copper in a liver biopsy sample> 200m/g dry weight. Serum ceruloplasmin level low. Urine copper execration increased KF rings. DNA Haplotype analysis.
CLINICAL MANIFESTATIONS OF LIVER CIRRHOSIS Pedal oedema and anasarca (decrease albumin decrease oncotic pressure increase interstitial fluid oedema) Jaundice Increase infections Indigestion Anorexia/Nausea/Vomiting Some patient are asymptomatic and diagnosis is made incidentally at ultrasound or at surgery.
Steatorrhea (Indigested fat present in stool). Bleeding tendencies (Due to deficiency of vitamin K synthesis of clothing factors in liver). Detoxification Impairment. Hepatic encephalopathy (Due to increase ammonia crossed BBB and develops CNS symptoms). Thrombocytopenia. Anemia. Hepatosplenomegaly. Parotid enlargement. Circulatory changes. Spider telangiectasia, palmar erythema, cyanosis. Endocrine changes. Loss of libido, hair loss Men: Gynecomastia, testicular atrophy, impotence. Women: breast atrophy, irregular menses, amenorrhea. Hemorrhagic tendency. Bruises, purpura, epistaxis Portal hypertension. Splenomegaly, collateral vessel, variceal bleeding
DIAGNOSIS Liver function test: increase alkaline phosphate, ALT,AST. Blood test: decrease total protein, decrease albumin, increase serum bilirubin and globulin, increase ammonia. Prothrombin time is prolonged (normal:10-14sec). Liver cell biopsy to identify liver cell changes. Ascites fluid test. Liver ultrasound. CT Scan: enlarged or atrophied, characteristics. Stool for occult blood. ERCP.
INVESTIGATIONS FOR THE ETIOLOGY Viral markers Serum autoantibodies. Iron indices and ferritin. Copper, ceruloplasmin. Alpha1 antitrypsin. (Serum copper and alpha 1 antitrypsin should always be measured in young cirrhotic)
MANAGEMENT OF LIVER CIRRHOSIS CONSERVATIVE MANAGEMENT Treat underlying cause: if cirrhosis is form heavy alcohol use, the treatment is to completely stop drinking alcohol. If cirrhosis is caused by hepatitis C, then treatment of hepatitis C. Avoidance of hepatotoxic substances. Dietary modification: table salt, salted butter, margarine, ordinary can froze foods should be avoided. The diet should be be adequate calories and protein (75-100gm/day) unless hepatic encephalopathy is present, in which case protein is limited. Restrict fluid.
Lactulose: to reduce serum ammonia level. Low-protein diet: 1.0 and 1.5 g/kg or up to 0.5 g/kg. Intravenous administration of glucose to minimize protein breakdown. Administration of vitamins to correct deficiencies. Correction of electrolyte imbalances (potassium with proctor). Neurologic status is assessed frequently.
SURGICAL MANAGEMENT Liver transplantation. Removing the liver and replacing it with a healthy donor organ is another way to treat liver cancer or liver cirrhosis. About 80-90% of people who undergo liver transplantation, survive. Esophageal varices.
HOMEOPATHIC MANAGEMENT OF LIVER CIRRHOSIS NUX VOMICA In liver affections occurring in those who have indulged to excess in alcoholic liquors , highly seasoned food , quinine or in those who have abused themselves with purgatives , Nux is the first remedy to be thought of. Bruised soreness of abdominal walls. Liver engorged, with stitches and soreness . Colic, with upward pressure, causing short breath and desire for stool. Constipation , with frequent ineffectual urging incomplete and unsatisfactory ; feeling as if part remained unexpelled. Alternate constipation and diarrhea-after abuse of purgatives.
PHOSPHORUS Phosphorus is the remedy for fatty degeneration of the liver , with well-marked Soreness and jaundice . The stools are grayish white . Jaundice accompanying pneumonia may also call for phosphorus . Suddenness of symptoms, sudden prostration, faints, sweats, shooting pains, etc. Fatty degenerations, cirrhosis, caries, are pathological states often calling for phosphorus
BRYONIA When there are stitching pains in the right hypochondriac region , Bryonia is the first remedy to be thought of. Under Bryonia is the liver is swollen, congested and inflamed . The pains in the hypochondriac region are worse from any lying on the right side , which lessens the motion of the parts when breathing. Nausea and faintness when rising up. Abnormal hunger loss of taste. Thirst for large quantity . Vomiting of bile and water immediately after eating , as of a stone. There is a bitter in the mouth and the stools hard, dry, as if burnt ; seem too large. Stools brown, thick and bloody. Bryonia pre-eminently a gastro-hepatic remedy, and has pain in right shoulder, giddiness, skin and eyes slightly yellow.
ARSENIC ALBUM For liver cirrhosis with fatigue and weight loss . Natural medicine Arsenic Album is of great help for patients of liver cirrhosis who experienced fatigue as the main symptom. The patients feels totally exhausted from doing a little labor. He or she goes on losing weight due to debilitating effects of cirrhosis of liver . Arsenic Album is also the remedy for patients who experienced nausea and vomiting of blood . Along with vomiting, burning in abdomen is a characteristic feature pointing towards the use of Arsenic album. Vomiting follows drinking and eating. The liver region feels painful. Ascites , or accumulation of fluid in abdomen , can also be dealt with by remedy Arsenic Album, though the general symptoms of fatigue and burning in abdomen should match too. A general relief from taking warm drinks may be narrated by patients in addition to the above symptoms. If the mental picture is to be considered, the symptoms that assume top position are anxiety, restlessness and fear of death .