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Liver Disorders

Non-Infectious:Fatty Liver,Wilson's, hemochromatosis

Michelle Jose

on 13 September 2012

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Transcript of Liver Disorders

Disorders of the Liver It's time to look after your liver. Liver Facts: The liver is the largest solid organ in the body, weighing on average about 3.5 pounds. The liver forms and secretes bile that contains bile acids to aid in the digestion and intestinal absorption (taking in) of fats and the fat-soluble vitamins A, D, E, and K. The liver carries out a large number of critical functions, including manufacture of essential proteins, and metabolism of fats and carbohydrates.
The liver also serves to eliminate harmful biochemical waste products and detoxify alcohol, certain drugs, and environmental toxins. DIsorders of the Liver Fatty Liver Disease
Wilson's Disease FATTY LIVER DISEASE Fatty liver, also known as fatty liver disease (FLD), is a REVERSIBLE CONDITION where large vacuoles of triglyceride fat accumulate in liver cells via the process of steatosis (i.e. abnormal retention of lipids within a cell). ALCOHOLIC Alcoholic Fatty Liver Disease OBESE Non-Alcoholic Fatty Liver Disease causes Metabolic Abetalipoproteinemia
glycogen storage diseases
Weber-Christian disease
acute fatty liver of pregnancy
lipodystrophy Nutritional Malnutrition
total parenteral nutrition
severe weight loss
refeeding syndrome
jejunoileal bypass
gastric bypass
Jejuna diverticulosis with bacterial overgrowth Drugs and Toxins Amiodarone
expired tetracycline
highly active antiretroviral therapy
environmental hepatotoxins (e.g., phosphorus, mushroom poisoning) Others Inflammatory bowel disease
hepatitis C (especially genotype 3)
alpha 1-antitrypsin deficiency Pathophysiology Fatty change represents the intracytoplasmic accumulation of triglycerides (neutral fats) microvesicular fatty change hepatocytes present small fat vacuoles (liposomes) around the nucleus macrovesicular fatty change size of the vacuoles increases, pushing the nucleus to the periphery of the cell, giving characteristic signet ring appearance Acute fatty liver
of pregnancy Reye's syndrome alcohol diabetes obesity corticosteroids Defects in fatty acid metabolism imbalance in energy consumption and its combustionimbalance in energy consumption and its combustion a consequence of peripheral resistance to insulin inhibition of receptor molecules (PPAR-, PPAR- and SREBP1) that control the enzymes responsible for the oxidation and synthesis of fatty acids alcoholism is known to damage mitochondria and other cellular structures Symptoms Diagnosis Fatigue
Weight loss or loss of appetite
Confusion, impaired judgment, or trouble concentrating

These symptoms may also be present:

Pain in the center or right upper part of the abdomen
An enlarged liver
Patchy, dark skin discoloration, usually on the neck or underarm area With alcoholic liver disease, symptoms may worsen after periods of heavy drinking. With NAFLD, the disease process can stop or reverse, or it may worsen. If cirrhosis develops, the liver loses its ability to function. This can cause signs and symptoms such as: Fluid retention
Muscle wasting
Internal bleeding
Jaundice (yellowing of skin and eyes)
Liver failure The diagnosis of steatosis is made when fat in the liver exceeds 5–10% by weight Blood tests. During routine blood tests, elevations in certain liver enzymes may show up. These might include alanine aminotransferase (ALT) or aspartate aminotransferase (AST). Imaging studies. A fatty liver may also show up on imaging tests, such as an abdominal ultrasound. fatty liver normal liver Liver biopsy. This is usually done once other causes have been ruled out. After application of local anesthesia, the doctor inserts a needle through the skin to remove a tiny piece of liver. This is then examined under a microscope for signs of fat, inflammation, and damaged liver cells. If inflammation or damage is not present, the diagnosis is simply a fatty liver. There is no specific treatment at this time for fatty liver disease. However, getting treatment for any underlying disease, such as diabetes, is essential Clinical trials are also looking at the effectiveness of certain antioxidants and newer diabetes medications in treating fatty liver disease (because of disease's link to oxidative stress and diabetes). These include: Vitamin E
Betaine Metformin
Pioglitazone Find the support you need to be successful. QUIT drinking Do what you can to gradually lose weight no more than 1 or 2 pounds a week limit calories avoid diets rich in refined, rapidly digested carbohydrates. This includes limiting foods such as: bread
concentrated sugar that is found in sports drinks and juice. counteracting unbalanced diets with probiotics Avoid unnecessary medications Acquire necessary vitamins in minerals naturally. Hemochromatosis Hemochromatosis is an inherited condition that causes your body to absorb too much iron from the food you eat. The excess iron is stored in your organs, especially your liver, heart and pancreas. If you have hereditary hemochromatosis, the stored iron damages these organs, leading to life-threatening conditions such as cancer, heart problems and liver disease. How hemochromatosis affects your organs Normal: 10%
Hemochromatosis: 30% Absorbed Iron Iron an essential component of proteins involved in oxygen transport.
It is also essential for the regulation of cell growth and differentiation.
A deficiency of iron limits oxygen delivery to cells, resulting in fatigue, poor work performance, and decreased immunity.
On the other hand, excess amounts of iron can result in toxicity and even death Reduced cellular ATP levels, impaired cellular calcium homeostasis, and damage to DNA may all contribute to hepatocellular injury in iron overload. Long-term dietary iron overload can lead to increased collagen gene expression and hepatic fibrosis, perhaps due to activation of hepatic lipocytes. Symptoms Early signs and symptoms of hereditary hemochromatosis mimic those of many other common conditions, making it difficult to diagnose. Signs and symptoms include: Joint pain
Loss of sex drive (libido) or impotence
Lack of normal menstruation (amenorrhea)
Pain on the upper right portion of the abdomen Cause: Gene Mutation inherit two abnormal genes inherit one abnormal gene you may develop hemochromatosis you won't develop hemochromatosis Other types of hemochromatosis Juvenile hemochromatosis Neonatal hemochromatosis Risk factors Having two copies of a mutated HFE gene
Family history
Being a man Complications Liver cirrhosis
Liver failure
Liver cancer Arthritis
Heart problems
Increased risk for certain bacterial infections
Testicular atrophy
Skin coloring changes Diagnosis Physical exam. This may show liver and spleen swelling, and skin color changes. Blood tests Ferritin level
Iron level
Percentage of transferrin saturation (high) Other tests may include: Blood sugar (glucose) level
Alpha fetoprotein
Echocardiogram to examine the heart's function
Electrocardiogram (ECG) to look at the electrical activity of the heart Imaging tests
Liver function tests
liver biopsy or phlebotomy
If a genetic defect is confirmed, other blood tests can be used to determine if other family members are at risk for iron overload. Treatment Procedure called
PHLEBOTOMY One-half liter of blood is removed from the body each week How often you need this procedure depends on your symptoms and your levels of hemoglobin and serum ferritin, and how much iron you take in your diet Medication Chelation therapy is a mainstream treatment used to treat heavy metal poisoning: Deferoxamine must be administered intravenously 1.0 gram, three to four times per week with monitoring of iron excretion in a cumulative 24-hour urine sample. Side effects of IV iron chelation: The urine can become orange colored visual disturbances,
blurred vision,
rash or hives,
stomach or leg cramps,
rapid heart beat,
hypotension dizziness,
anaphylactic shock,
pain or swelling at site of intravenous entry. Long term problems might include
kidney or liver damage,
loss of hearing or
cataracts. Some physicians will begin iron chelation therapy when serum ferritin is between 1,000-1,500ng/mL. Serum ferritin that is above 2,500 ng/mL before beginning iron chelation therapy might result in organ damage. Patient Counseling Do not use iron cookware
Do not eat raw seafood (cooked is fine)
Do not eat foods fortified with iron, such as 100% iron breakfast cereals
Extra iron may also build up in other areas of the body, including the thyroid gland, testicles, pancreas, pituitary gland, heart, or joints.
Avoid iron supplements and multivitamins containing iron
Avoid vitamin C supplements, especially with food
Avoid alcohol
Avoid eating raw shellfish Wilson's Disease (cc) image by nuonsolarteam on Flickr Wilson's disease is an inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Another term for Wilson's disease is hepatolenticular degeneration. Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin Symptoms Clumsiness
Difficulty speaking
Difficulty swallowing
Difficulty walking
Easy bruising
Fatigue Involuntary shaking
Joint pain
Loss of appetite
Skin rash
Swelling of arms and legs
Yellowing of the skin and eyes (jaundice) Causes genetic mutation protein copper liver Complications Scarring of the liver (cirrhosis) Liver failure Liver cancer Persistent neurological problems Kidney problems Tests and diagnosis Blood and urine tests. Your doctor may recommend blood tests to monitor your liver function and look for copper in your blood. Brain scans. If you have signs and symptoms that indicate Wilson's disease is affecting your brain, Eye exam. Using a microscope with a high-intensity light source (slit lamp), an ophthalmologist checks your eyes for golden-brown discoloring (Kayser-Fleischer rings). Removing a sample of liver tissue for testing. In a procedure called a liver biopsy, your doctor inserts a thin needle through your skin and into your liver. Genetic testing. A blood test called DNA mutation analysis can identify the genetic mutations that cause Wilson's disease. Treatments
and drugs chelating agents Penicillamine (Cuprimine, Depen). Penicillamine can cause serious side effects, including skin problems, bone marrow suppression, worsening of neurological symptoms and birth defects. Trientine (Syprine). Trientine works much like penicillamine but tends to cause fewer side effects. Still, there is a risk that neurological symptoms can worsen when taking trientine, though it's thought to be a lower risk than is penicillamine Zinc acetate prevents your body from absorbing copper from the food you eat. Zinc acetate causes few side effects but can cause upset stomach. Liver transplant During a liver transplant, a surgeon removes your diseased liver and replaces it with a healthy liver from a donor.
Most transplanted livers come from donors who have died. But in some cases a liver can come from a living donor, such as a family member.
In that case, the surgeon removes your diseased liver and replaces it with a portion of your family member's liver. Lifestyle and
home remedies Copper-containing foods oCopper-containing vitamin and mineral supplements
oNuts oChocolate
oDried fruit
oDried peas, beans and lentils
oBran products •Copper in tap water Have your tap water's copper levels tested if you have copper pipes in your home or if your water comes from a well.
Most municipal water systems don't contain high levels of copper.
If you have copper pipes, run the tap for several seconds before collecting water for drinking or cooking.
Water that sits in the copper pipes can pick up copper particles. Running the water flushes that contaminated water out of the pipes.
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