GASTRO 2: BOARDS AND BEYOND Flashcards by Karla Chavez

What demographic is primarily affected by autoimmune hepatitis (AIH)?

Women in their forties and fifties.

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How is AIH commonly identified?

Through incidental findings of elevated aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels on blood work.

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What are potential complications of AIH?

Chronic liver disease and, rarely, cirrhosis.

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What are the diagnostic criteria for AIH?

Presence of antinuclear antibodies (ANA) for sensitivity and anti-smooth muscle antibodies (ASMA) for specificity, especially in type I AIH.

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What is the management strategy for acute episodes of AIH?

Treatment typically involves steroids and immunosuppressants.

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Are highly specific for systemic lupus erythematosus (SLE) and are believed to be implicated in the pathogenesis of lupus nephritis.

Anti-double stranded DNA antibodies

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Are specific for CREST syndrome, a limited cutaneous form of systemic scleroderma. The five main features (as indicated by the acronym) are calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.

Anti-centromere antibodies

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Are associated with drug-induced lupus. Common drugs implicated include procainamide, penicillamine, isoniazid, and methyldopa.

Anti-histone antibodies

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Are found in patients with anti-phospholipid syndrome, a hypercoagulable state that causes thrombosis in arteries and veins. It causes a number of pregnancy-related complications, including miscarriage, stillbirth, preterm delivery, and preeclampsia.

Anti-cardiolipin antibodies

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Are specific for primary biliary cholangitis. Like autoimmune hepatitis, this condition primarily affects middle-aged women. ANA antibodies are also sensitive markers for this disease. Unlike autoimmune hepatitis, patients generally present with signs and symptoms consistent with cholestasis, including itching, fatigue, and jaundice. Primary biliary cholangitis more often progresses to cirrhosis.

Anti-mitochondrial antibodies

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What is a pyogenic liver abscess?

A walled-off infection of the liver.

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What is the most likely cause of a pyogenic liver abscess in a patient with diverticulitis?

Seeding of the portal venous system with bacteria from the diverticulitis episode.

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Which organisms are most commonly responsible for pyogenic liver abscesses?

Enteric flora, particularly gram-negative rods like E. coli and Klebsiella pneumoniae.

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What characterizes acute alcoholic hepatitis?

Hepatitis occurring after acute binge drinking in individuals with a history of alcohol-use disorder.

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What are common symptoms of acute alcoholic hepatitis?

Right upper quadrant abdominal pain, fever, and jaundice.

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What is the main toxic by-product involved in the pathophysiology of acute alcoholic hepatitis?

Acetaldehyde, produced during alcohol metabolism.

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How does acetaldehyde affect hepatocytes?

It damages intermediate filaments within the cells.

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What is a classic histopathological finding in acute alcoholic hepatitis?

Mallory bodies, which are cytoplasmic inclusions representing damaged intermediate filaments in hepatocytes.

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In alcoholic liver disease, nuclei may be pushed aside by fat accumulation with cells, but

Nuclear abnormalities are not a classic feature of alcoholic hepatitis.

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An overabundance of microtubules can be seen in cardiac myocytes in patients with

Hypertrophic cardiomyopathy

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Structural and functional alterations of the Golgi apparatus are seen in several neurodegenerative, diseases including

Amyotrophic lateral sclerosis (ALS), Parkinson’s disease, Alzheimer’s dementia, and Huntington’s disease.

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What is Budd-Chiari syndrome?

Hepatic vein thrombosis leading to abdominal pain, ascites, and hepatomegaly.

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How is Budd-Chiari syndrome diagnosed?

Doppler imaging showing obstruction of the hepatic vein.

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What conditions are associated with Budd-Chiari syndrome?

Hepatocellular carcinoma, polycythemia vera, and hypercoagulable states.

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What comprises the portal triad?

A bile duct, hepatic artery, and portal vein, surrounded by zone I (periportal).

Describe the zones of the liver acinus

Zone I (periportal), Zone II (midzone), Zone III (centrilobular).

What happens in Zone III during hepatic vein thrombosis?

Congestion occurs first, leading to potential hepatic necrosis.

Why is Zone III particularly susceptible to injury?

It is vulnerable to ischemic damage, fatty infiltration, and fibrosis.

What are common etiologies of cirrhosis?

Alcoholic liver disease, viral hepatitis, autoimmune conditions (e.g., autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis).

How does cirrhosis affect liver function?

It impairs synthetic functions like albumin and clotting factor production.

What laboratory findings would indicate cirrhosis?

Decreased serum albumin and prolonged prothrombin time (PT), partial thromboplastin time (PTT), and INR.

How are Factor VIII levels affected in chronic liver disease?

Factor VIII levels are usually normal or increased due to synthesis by endothelial cells outside the liver.

Where else is von Willebrand factor (VWF) synthesized?

By endothelial cells outside the liver, similar to Factor VIII.

Evidence of hypervolemia: - Distended abdomen (ascites) - Bilateral pitting edema

Cirrhosis Overview

- Vasodilation - Low systemic vascular resistance (SVR)

Hemodynamic Changes in Cirrhosis

Low SVR activates: - Sympathetic nervous system (SNS) - Renin-angiotensin-aldosterone system (RAAS) Results in: - Sodium and water retention - Hypervolemia

Cirrhosis: Activation of Compensatory Systems

Low albumin → decreased plasma oncotic pressure Fluid leakage from capillaries → Reduces circulating volume Causes ascites and peripheral edema Impact on Renal Blood Flow Low circulating volume → decreased renal blood flow (RBF) Increased serum antidiuretic hormone (ADH) levels

Plasma Oncotic Pressure in Cirrhosis

Total body sodium is increased in hypervolemia (e.g., cirrhosis, heart failure) Serum sodium = sodium in blood Can be elevated, normal, or low in hypervolemia Hyponatremia risk: Due to water retention and dilution of serum sodium

Total Body Sodium vs. Serum Sodium

- Activation of SNS and RAAS → sodium and water retention - Excess fluid increases total body sodium - Retention of water can lead to hyponatremia

Summary of Fluid and Sodium Dynamics

Internal hemorrhoids are a consequence of congestion of the

Superior rectal veins

In cirrhosis, venous collaterals form between the left gastric vein and the esophageal veins. Congestion of this vessel leads to

Esophageal varices

What are Varices?

- Dilated, tortuous veins - Hallmark of end-stage liver disease and cirrhosis Can rupture, leading to life-threatening hemorrhage

Cause of Varices

- Result from portal venous congestion Secondary to obstructed blood flow in cirrhotic liver

Gastric Varices Explained

- Dilated veins due to congestion in short gastric veins - Located along the greater curvature of the stomach - Short gastric veins drain into the splenic vein, then the portal vein Congestion in the splenic vein leads to dilation of short gastric veins Visible during endoscopy

Risks of Bleeding Varices

- High morbidity and mortality in cirrhosis - Screening endoscopy performed to evaluate and treat when bleeding risk is high

The superior rectal veins drain into the inferior mesenteric vein, which then drains to the portal vein. Note that the other rectal veins (middle and inferior rectal veins) bypass the portal-venous system. Blood from these veins eventually drains into the inferior vena cava.

Dilation of these vessels in portal hypertension does not occur.

The umbilical vein is present during fetal development. This structure closes after birth and becomes the

Ligamentum teres (also called the round ligament of the liver).

- Drug of choice for managing ascites in cirrhosis after salt and fluid restriction - Aldosterone receptor antagonist

Spironolactone Overview

- Blocks aldosterone action at the distal tubule Results in: - Decreased sodium and water retention - Increased excretion of salt and water (diuresis) - Improvement of ascites

Mechanism of Action: Spironolactone

- Spironolactone is a potassium-sparing diuretic - Requires monitoring for hyperkalemia

Potassium-Sparing Diuretic: Spironolactone

Common ECG changes: - Peaked T waves (classic finding) Severe hyperkalemia can lead to: - Broadening of QRS - Absence of P-waves - Conversion to sinusoidal wave pattern

Hyperkalemia Monitoring: Spironolactone

Polyuria and volume loss occurs with

Hypercalcemia

Tetany occurs in

Hypocalcemia

Calciphylaxis occurs in

Hyperphosphatemia

Parathyroid inhibition occurs in ?. Severe hypomagnesemia suppresses parathyroid hormone release, leading to hypocalcemia.

Marked hypomagnesemia

- Impairment of neuropsychiatric function due to impaired hepatic function Often associated with hyperammonemia

Hepatic Encephalopathy Overview

Common in many cases of hepatic encephalopathy Neurologic manifestations include: - Asterixis - Confusion - Coma

Role of Hyperammonemia

Synthetic disaccharide and osmotic laxative - Used to lower elevated serum ammonia in hepatic cirrhosis

Lactulose as Treatment

- Increases excretion of ammonia via the GI tract - Fermented by colonic flora → release of fatty acids

Mechanism of Lactulose

- Increases free fatty acid and hydrogen ion concentration in the colon - Acidifies colonic environment, converting NH3 to NH4+ - NH4+ cannot be reabsorbed and is excreted in stool

Effects of Lactulose Fermentation

- Ammonium excretion via stool lowers serum NH3 levels - Helps improve symptoms of hepatic encephalopathy

Impact on Serum NH3 Levels

Increase in free fluid in the peritoneal cavity - Defined as >25 ml of fluid

Definition of Ascites

Portal hypertension

Primary Cause of Ascites in Cirrhosis

Blood flows from the portal venous system → liver → hepatic veins → inferior vena cava

Normal Blood Flow Pathway

- Normal liver tissue is replaced by fibrous tissue - Disrupted blood flow leads to portal hypertension

Changes in Cirrhosis

- Increased capillary hydrostatic pressure in the abdomen - Fluid shifts from intravascular space to extravascular space

Mechanism of Ascites Formation

The most common cause of liver malignancy is metastasis. Common primary sites include the pancreas, breast, lung, and colon. Metastasis to the liver is more likely to present as multiple lesions, but a single lesion can also occur. Colonic adenocarcinoma is most likely in this case, given the

Coexisting microcytic anemia and heme positive stool.

Hepatocellular carcinoma (HCC) is less common than metastasis to the liver. HCC generally presents as a single lesion; however, this patient has no apparent risk factors. Risk factors include infection with ?.

hepatitis B, hepatitis C, cirrhosis, alcohol abuse, and exposure to aflatoxins

Hepatic adenomas are benign lesions that generally present as solitary nodules in the right lobe of the liver and occur in

Younger women on oral contraceptives.

Hepatic angiosarcomas are rare, highly malignant lesions whose primary risk factors include exposure to

Polyvinyl chloride or arsenic.

Rare, highly malignant primary tumor of the liver - Vascular in nature

Hepatic Angiosarcoma Overview

- Atypical endothelial cells - Disorganized vascular structures

Hepatic Angiosarcoma Histological Features

Death often secondary to rupture of liver nodules

Hepatic Angiosarcoma Cause of Death

Generally poor prognosis - Less than one year from diagnosis

Hepatic Angiosarcoma Prognosis

Common metastasis to the lungs and spleen

Hepatic Angiosarcoma Metastatic Sites

Toxic exposures, notably: - Polyvinyl chloride - Arsenic

Hepatic Angiosarcoma Primary Risk Factors

Most common primary malignancy of the liver

Hepatocellular Carcinoma (HCC) Overview

- Alcohol use - Cirrhosis - Chronic hepatitis B - Exposure to aflatoxins (e.g., from contaminated homegrown vegetables)

Hepatocellular Carcinoma (HCC) Risk Factors

- Commonly associated with polycythemia - Elevated hematocrit due to secretion of erythropoietin (EPO)

Hepatocellular Carcinoma (HCC) Lab Abnormalities

- EPO increases bone marrow production of red blood cells - Normally secreted by the kidney in response to anemia

Erythropoietin (EPO) in HCC

Is a tumor marker elevated in pancreatic cancer.

Ca 19-9

Is a tumor marker that is elevated in multiple malignancies including colonic, gastric, breast, lung, and medullary thyroid cancer.

Carcinoembryonic antigen (CEA)

This man has cirrhosis with no evidence of portal hypertension, such as ascites. Patients with cirrhosis of any cause are at increased risk for hepatocellular carcinoma (HCC). For this reason, abdominal imaging with

Ultrasound, CT scan, or MRI is performed at regular intervals, usually every six months.

A contrast agent, is injected into the rectum via enema to visualize the distal colon and rectum by X-ray. This can be useful in disorders of the distal colon, including colon cancer, inflammatory bowel disease, and Hirschsprung’s disease.

Barium

Occurs in end-stage liver disease when the liver can no longer synthesize sufficient clotting factors.

Coagulopathy with elevated prothrombin time

Alanine aminotransferase (ALT) is a liver enzyme. Its levels are elevated in many forms of liver disease, and may be low in ? when the liver is replaced by fibrous tissue.

Cirrhosis

Occurs in end-stage liver disease with liver failure when the liver can no longer perform gluconeogenesis.

Hypoglycemia

- Autosomal recessive disorder of copper metabolism - Characterized by impaired copper transport and excretion in bile

Wilson’s Disease Overview

- Caused by mutation of the ATP7B gene on chromosome 13 - ATP7B is a copper transport protein

Genetic Cause of Wilson’s Disease

- Impaired function of ATP7B leads to copper accumulation in the liver and other organs - Results in free radical production and tissue damage

Mechanism of Copper Accumulation

- Pathological hallmark of Wilson’s disease - Corneal copper deposits along Descemet's membrane at the limbus (corneoscleral junction)

Kayser-Fleischer Ring

Copper accumulation in the brain leads to symptoms such as: - Ataxia (leading to falls) - Difficulty speaking

Neurologic Impairment in Wilson’s Disease

Total body copper is increased compared to normal Copper is trapped in organs (liver, brain, eye, kidneys)

Copper Metabolism in Wilson’s Disease

- Total serum copper is paradoxically decreased - Ceruloplasmin (copper transport protein) is low due to impaired copper incorporation

Wilson's D: Serum Copper and Ceruloplasmin Levels

- 24-hour urinary copper excretion is increased - Increased free copper in serum is filtered at the glomerulus and excreted

Urinary Copper Excretion in Wilson’s Disease

Increased urinary copper excretion does not reverse total body copper accumulation in tissues

Impact of Urinary Excretion on Copper Accumulation

- Ceruloplasmin is the copper transport protein in serum Levels are low because copper cannot be incorporated and released from the liver

Ceruloplasmin in Wilson’s Disease

- Hepatomegaly - Increased serum aminotransferase levels

Clinical Features of Wilson’s Disease

- Oral chelation therapy with penicillamine - Aids in copper excretion via the kidneys

Treatment for Wilson’s Disease

Condition characterized by excess total body iron

Hemochromatosis Overview

- Can be hereditary due to mutation of the C282Y gene - Gene codes for the HFE protein

Genetic Cause of Hemochromatosis

- Can develop from excessive blood transfusions Associated with disorders like: - Sickle cell anemia - Beta thalassemia major - Myelodysplastic syndromes

Acquired Causes of Hemochromatosis

Skin: Dark bronze discoloration from iron deposition Pancreas: May lead to diabetes ("bronze diabetes") Heart: Common manifestation is dilated cardiomyopathy

Organ Systems Affected by Hemochromatosis

- New complaint of dyspnea on exertion - Pitting edema - Extra heart sound

Symptoms Supporting Dilated Cardiomyopathy

Enlarged cardiac silhouette due to increased size of the left ventricle

Chest X-ray Findings in Dilated Cardiomyopathy

Condition of iron overload with deposition in multiple organs

Hemochromatosis Overview

- Elevated serum iron - Elevated ferritin - Elevated transferrin saturation - Transferrin levels may be decreased or normal

Iron Studies in Hemochromatosis

- Dilated cardiomyopathy (most common) - Rarely, restrictive cardiomyopathy

Cardiac Manifestations of Hemochromatosis

Skin may turn bronze from iron interaction with melanin

Skin Changes in Hemochromatosis

- Iron deposition can lead to diabetes mellitus - Often referred to as “bronze diabetes” due to concurrent skin changes - Symptoms include polyuria and polydipsia

Pancreatic Effects of Hemochromatosis

Iron deposition may lead to: - Hepatomegaly - Abnormal liver function tests - Cirrhosis - Increased risk of hepatocellular carcinoma

Liver Complications in Hemochromatosis

arthralgias

Joint Symptoms in Hemochromatosis

- Erectile dysfunction and small testes indicate testicular atrophy - Leads to decreased serum testosterone

Endocrine Effects in Hemochromatosis

- Increase in ferritin and hemoglobin A1C - Decrease in serum testosterone

Expected Laboratory Changes in Hemochromatosis

Avoid Alcohol: Protects the liver. Avoid Vitamin C: Increases iron absorption.

Dietary Recommendations for Hemochromatosis

Heme Iron: Found in red meat; easily absorbed. Non-Heme Iron: Absorbed as Fe2+; enhanced by Vitamin C (converts Fe3+ to Fe2+).

Iron Absorption Forms

Avoid vitamin C to limit dietary iron absorption.

Implications for Hemochromatosis Patients

GASTRO 2: BOARDS AND BEYOND Flashcards

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