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

• What are the functions of the liver?

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?

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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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.

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

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

Fulminant liver failure-Pathology

• Massive necrosis of hepatocytes

• Pathogenesis:

1. Increased serum level of ammonia, false neurotransmitters, increased circulating levels of endogenous benzodiazepine-like compounds.

2. Decreased hepatic clearance lead to CNS dysfunction

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

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

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

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)

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

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)

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

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

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

Diagnosis—Portal hypertension

• Doppler flow ultrasonography-CT-MRI

• Endoscopy

Treatment—Portal hypertension

• Emergency treatment

• Prophylactic treatment

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)

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

THANKS