CASE PRESENTATION ON LIVER CIRRHOSIS WITH PORTAL HYPERTENSION

CASE PRESENTATION ONCIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION, HEPATIC ENCEPHALOPATHY AND GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.AKHIL JOSEPHREG.NO : 13QO4O2

Cirrhosisis a slowly progressing disease in which healthylivertissue is replaced with scar tissue, eventually preventing theliverfrom functioning properly. The scar tissue blocks the flow of blood through theliverand slows the processing of nutrients, hormones, drugs, and naturally produced toxins.

As the disease worsens, a person may become tired,weak,itchy, haveswelling in the lower legs, developyellow skin, bruise easily, havefluid build up in the abdomen, or developspider-like blood vessels on the skin. The fluid build-up in the abdomen may becomespontaneously infected. Other complications includehepatic encephalopathy, bleeding fromdilated veins in the esophagusordilated stomach veins, andliver cancer. Hepatic encephalopathy results in confusion and possiblyunconsciousness.

EPIDEMIOLOGYCirrhosis is the ninth leading cause of death in the United States and is responsible for 1.2% of all US deaths Many patients die in their fifth or sixth decade of life The prevalence is higher in non-Hispanic blacks and Mexican Americans, those living below the poverty level, and those with less than a 12th grade education Each year, 2000 additional deaths are attributed to fulminant hepatic failure (FHF), that may be caused viral hepatitis, drugs (e.g., acetaminophen), toxins (e.g., Amanita phalloides, the yellow deathcap mushroom), autoimmune hepatitis, Wilson disease, or a variety of less common etiologies. Cryptogenic causes are responsible for one third of fulminant cases.

ETIOLOGYIt has many possible causes; sometimes more than one cause is present in the same person. Globally, 57% of cirrhosis is attributable to either hepatitis B (30%) or hepatitis C (27%).Alcohol consumption is another important cause, accounting for about 20% of the cases. Alcoholic liver disease(ALD). Alcoholic cirrhosis develops for 1020% of individuals who drink heavily for a decade or more.Non-alcoholic steatohepatitis(NASH). In NASH, fat builds up in the liver and eventually causes scar tissue.Chronichepatitis C.Chronichepatitis B.Primary biliary cirrhosis. Damage of the bile ducts leading to secondary liver damage.

Primary sclerosing cholangitis. PSC is a progressive cholestatic disorder presenting with pruritus,steatorrhea, fat-soluble vitamin deficiencies, and metabolic bone disease.Wilson's disease. Autosomal recessive disorder characterized by low serumceruloplasmin and increased hepatic copper content on liver biopsy and elevated 24-hour urine copper. Cystic fibrosisHepatotoxic drugs or toxinsAutoimmune hepatitis. This disease is caused by the immunologic damage to the liver causing inflammation and eventually scarring and cirrhosis.

PATHOPHYSIOLOGY

CLINICAL PRESENTATIONSYMPTOMSGastrointestinal:bleeding, dark stool from digested blood, fluid in the abdomen, nausea, passing excessive amounts of gas, vomiting blood, or water retentionWhole body:fatigue, loss of appetite, or reduced hormone productionSkin:web of swollen blood vessels in the skin or yellow skin and eyesWeight:weight gain or weight lossAlso common:swollen legs, yellow eyes, bleeding, breast enlargement, breast enlargement in men (Gynecomastia), bruising, dark urine, enlarged veins around belly button, flapping hand tremor, itching, mental confusion, Poor concentration and memory, muscle weakness, red palms, swelling, swelling in extremities, or swollen veins in the lower esophagus(Bleeding esophageal varices )

ASCITES WITH PORTAL HYPERTENSION

SIGNSJaundice Scratch marks secondary to pruritus Spider angiomata/naevi (mainly found on the trunk and face) Skin telangiectasia's ('paper money skin') Palmar erythema Bruising Petechia or purpura Hair loss White nails (sign of hypoalbuminemia) Finger clubbing Dupuytren's contracture

RISK FACTORSObesity/overweightincreases the risk for liver disease. Obesity often results in the accumulation of fat cells in the liver. Acids that are secreted by these fat cells (called fatty acids) can cause a reaction in the body that destroys healthy liver cells and results in scarring (sclerosis) and liver damage.The risk for developing liver disease varies, depending on the underlying cause and the particular condition.General risk factorsfor liver disease include alcoholism, exposure to industrial toxins, heredity (genetics), and long-term use of certain medications.Ageandgenderalso are risk factors for liver disease. These factors vary, depending on the particular type of disease. For example, women between the ages of 35 and 60 have the highest risk for primary biliary cirrhosis and men aged 30-40 are at higher risk for primary sclerosing cholangitis.

COMPLICATIONSAnemia, thrombocytopenia and coagulopathy (folate deficiency, hemolysis, hypersplenism, cholestasis) Esophageal varices (portal hypertension) Ascites Spontaneous bacterial peritonitis Hepatocellular carcinoma Cirrhotic cardiomyopathy Hepatopulmonary syndrome Portopulmonary hypertension.

DIAGNOSISThegold standardfor diagnosis of cirrhosis is aliver biopsy, through apercutaneous, transjugular, laparoscopic, or fine-needle approach. A biopsy is not necessary if the clinical, laboratory, and radiologic data suggests cirrhosis. Furthermore, there is a small but significant risk to liver biopsy, and cirrhosis itself predisposes for complications caused by liver biopsy. The best predictors of cirrhosis are ascites, platelet count ALT. However, normal aminotransferases do not preclude cirrhosis.Alkaline phosphatase- slightly elevated but less than 2-3 times the upper limit of normal.Gamma-glutamyl transferase correlates with AP levels. Typically much higher in chronic liver disease from alcohol.Albumin- levels fall as the synthetic function of the liver declines with worsening cirrhosis since albumin is exclusively synthesized in the liver

Bilirubin- Levels normal when compensated but may elevate as cirrhosis progresses.Prothrombin time- increases since the liver synthesizes clotting factors.Globulins- increased due to shunting of bacterial antigens away from the liver to lymphoid tissue.Serumsodium-hyponatremiadue to inability to excrete free water resulting from high levels ofADHandaldosterone.Leukopeniaandneutropenia- due to splenomegaly with splenic margination.Coagulationdefects - the liver produces most of the coagulation factors and thus coagulopathy correlates with worsening liver disease.

IMAGINGUltrasoundis routinely used in the evaluation of cirrhosis. It may show a small and nodular liver in advanced cirrhosis along with increased echogenicity with irregular appearing areas. Other findings suggestive of cirrhosis in imaging are an enlargedcaudate lobe, widening of the liver fissures and enlargement of thespleen. An enlarged spleen (splenomegaly), which normally measures less than 1112cm in adults, is suggestive of cirrhosis withportal hypertensionin the right clinical setting. Ultrasound may also screen for hepatocellular carcinoma, portal hypertension, andBudd-Chiari syndrome(by assessing flow in the hepatic vein).Computed tomography (CT) scanning and/or magnetic resonance imaging (MRI)

ENDOSCOPYGastroscopy (endoscopic examination of the esophagus, stomach, andduodenum) is performed in patients with established cirrhosis to exclude the possibility of esophageal varices. If these are found, prophylactic local therapy may be applied (sclerotherapy or banding)

GRADING OF DISEASEThe severity of cirrhosis is commonly classified with theChild-Pugh score. This score usesbilirubin,albumin,INR, presence and severity ofascites, andencephalopathyto classify patients in class A, B, or C. Class A has a favourable prognosis, while class C is at high risk of death. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh and others. Although it was originally used to predict mortality during surgery, it is now used to determine the prognosis, as well as the required strength of treatment and the necessity ofliver transplantation.Modified Maddrey's discriminant function.Themodified Maddrey's discriminant function) was originally described by Maddrey and Boitnottto predictprognosisinalcoholic hepatitis. It is calculated by a simple formula: (4.6 x (PTtest - control))+ S.Bilirubinin mg/dlProspective studieshave shown that it is useful in predicting short term prognosis especially mortality within 30 days.A value more than 32 implies poor outcome with one month mortality ranging between 35% to 45%.Corticosteroid therapy or pentoxifylline have been used with mixed results for patients whose increased mortality is indicated with a value greater than 32.

SCORINGPointsClassOne year survivalTwo year survival56A100%85%79B81%57%1015C45%35%

Measure1 point2 points3 pointsTotal bilirubin, mol/L (mg/dL)3)Serum albumin, g/dL>3.52.83.5