Liver failure &portal hypertension

Transcription

1 Liver failure &portal hypertension Objectives: by the end of this lecture each student should be able to : Diagnose liver failure (acute or chronic) List the causes of acute liver failure Diagnose and treat hepatic encephalopathy Diagnose and treat portal hypertension List the investigation needed for assessment of liver failure

2 What are the functions of the liver?

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4 Clinical Approach-Suspected liver disease Acute liver disease Chronic liver disease Acute on chronic liver disease End stage liver disease compensated liver disease Decompensated liver disease What are the signs of acute & chronic liver disease?

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6 Diagnostic consideration #confirm the presence and type of liver disease a) compensated may be asymptomatic b) Decompensated presenc of liver failure and complications c)end stage * persistent rise in bilirubin * INR more than 1.3, * persistent fall in serum albumin * Faltering growth, * severe hepatic complications such as chronic hepatic encephalopathy, refractory ascites, intractable pruritis or recurrent esophageal varices despite medical treatment.

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11 Chronic liver disease &and End stage liver failure Chronic liver diseases of childhood leads to cirrhosis and/or cholestasis. The resulting fibrosis and regenerative nodular formation distorts the liver architecture and compresses hepatic vascular and biliary structures, resulting in portal hypertension and a vicious cycle of events that worsen the hepatic injury.

12 Fulminant Hepatic Failure It is a clinical syndrome resulting from massive necrosis of hepatocytes or from severe functional impairment of hepatocytes. The currently accepted definition in children include: 1. Biochemical evidence of acute liver injury<8 weeks 2. No evidence of chronic liver disease 3. Hepatic based coagulopathy PT>15 sec or INR>1.5 not corrected by vitamin k in the presence of hepatic encephalopathy

13 ACUTE LIVER FAILURE ---CAUSES Infections (viral, bacterial, malaria) Drugs NSAIDS, INH, carbamezapine, sodium valporoate, ketokanazole,..) Toxins Metabolic Wilson disease Autoimmune Vascular/ischemic (acute circulatory failure, cardiomyopathy, acute cardiac failure) Infiltrative(lymphoma, leukemia) Herbal supplements idiopathic

14 Fulminant liver failure-pathology Massive necrosis of hepatocytes Pathogenesis: 1. Increased serum level of ammonia, false neurotransmitters, increased circulating levels of endogenous benzodiazepinelike compounds. 2. Decreased hepatic clearance lead to CNS dysfunction

15 LIVER FAILURE----CAUSES NEONATES Metabolic causes Disseminated herpes simplex infection Neonatal haemochromatosis OLDER CHILD Viral hepatitis Metabolic causes acetaminophen toxicity Autoimmune hepatitis Wilson disease idiopathic

16 Clinical Presentation and Complications of acute liver failure Previously healthy change of sleep rhythm Encephalopathy Progressive jaundice Fever, anorexia & abdominal pain Rapid decrease in liver size Fetor hepaticas Metabolic(hypoglycemia, electrolyte imbalance,acid base imbalance) Coagulopathy-bleeding Infections Disturbed level of consciousness,, asterrixis Renal insufficiency Rapid respiration

17 Biochemical abnormalities in Acute liver failure Prolonged Prothrombin time Increased direct and indirect bilirubin Increased serum ammonia Increase aminotransferases activity Hypoglycemia, hypokalemia, hyponatremia Metabolic acidosis

18 Clinical presentation -- hepatic encephalopathy May be absent or difficult to recognize in children. Stage1-1.mild confusion /anxiety, disturbed or reversal of sleep rhythm, short attention span. stage 2- Drowsiness, confusion, intermittent disorientation of time and place, gross deficit in ability to perform mental task. stage 3- Delirious but arousable, persistent disorientation of person and place, hypereflexia. stage4- Comatose with or without decerebrate or decorticate posturing response to pain(stage4a) or no response to pain(stage4b)

19 Management of Acute liver failure Refer to a liver centre Emergency liver transplantation is the only curative treatment Supportive treatment Involves treatment of the cause and complications Enteral feeding

20 Management of acute liver failure Ventilate for respiratory failure fluid balance Fresh frozen plasma if there is bleeding No sedation Lactulose (laxative) Intravenous broad spectrum antibiotics as prophylaxis Specific therapy in cases of poisoning Continuous monitoring(saturation, neurological observation, vital signs electrolytes,acid base balance,blood glucose,urine output,pt,ptt,inr,hb &platelets)

21 Portal hypertension Portal pressure>10-12mmhg normal 7 1.Extra hepatic obstruction Important cause in childhood.(umbilical infection/umbilical catheter) Portal vein thrombosis in neonatal dehydration & systemic infections Hypercoagulable state

22 Etiology portal hypertension 2.Intrahepatic obstruction acute and chronic hepatitis, congenital hepatic fibrosis& schistosomiasis, infiltration by malignancy and granuloma, idiopathic Leads to splenomegally &port systemic collaterals Esophageal varices Cirrhosis is the predominant cause of portal hypertension 3.Postsinusoidal causes Budd- Chiari syndrome

23 Clinical presentation--portal Hypertension Bleeding from esophageal varices is the most common presentation hemorrhage Stigmata of chronic liver disease ascites + others Splenomegally,sometimes with hypersplenism

24 Diagnosis Portal hypertension Doppler flow ultrasonography-ct-mri Endoscopy

25 Treatment Portal hypertension Emergency treatment Prophylactic treatment

26 Ascitis --Treatment 50 percent of patients will die within 2 years of developing ascitis Treatment ---step 1 sodium restriction Step 2---spironolactone Step 3---chlorthiazide/ frusemide and fluid restriction *spontaneous bacterial peritonitis can occur with high mortality (pneumococci)

27 Prevention and treatment of oesopheal variceal bleeding 1.sclerotherapy 2.variceal ligation 3.surgical (porto-systemic shunt) 4.oesophageal transection and devascularization 5.drugs e.g. Propanolol

28 THANKS