Bilirubin - blood

Atualização em bilirrubina

Bilirubin - blood

Images

Blood test

Bilirubin is a yellowish pigment found in bile, a fluid produced by the liver.

This article discusses the laboratory test done to measure bilirubin in the blood. Total and direct bilirubin are usually measured to screen for or to monitor liver or gallbladder problems. Large amounts of bilirubin in the body can lead to jaundice.

A test may also be done to measure bilirubin in a urine sample. For information on that test, see: Bilirubin - urine.

How the Test is Performed

A blood sample is needed. For information on how this is done, see: Venipuncture .

The laboratory specialist spins the blood in a machine called a centrifuge, which separates the liquid part of the blood (serum) from the cells. The bilirubin test is done on the serum.

How to Prepare for the Test

You should not eat or drink for at least 4 hours before the test. Your health care provider may instruct you to stop taking drugs that affect the test.

Drugs that can increase bilirubin measurements include allopurinol, anabolic steroids, some antibiotics, antimalaria medications, azathioprine, chlorpropamide, cholinergics, codeine, diuretics, epinephrine, meperidine, methotrexate, methyldopa, MAO inhibitors, morphine, nicotinic acid, birth control pills, phenothiazines, quinidine, rifampin, steroids, sulfonamides, and theophylline.

Drugs that can decrease bilirubin measurements include barbiturates, caffeine, penicillin, and high-dose salicylates such as aspirin.

Why the Test is Performed

This test is useful in determining if a patient has liver disease or a blocked bile duct.

http://www.nlm.nih.gov/medlineplus/ency/imagepages/10026.htm

http://www.nlm.nih.gov/medlineplus/ency/article/003595.htm






Bilirubin metabolism begins with the breakdown of red blood cells in many parts of the body. Red blood cells contain hemoglobin, which is broken down to heme and globin. Heme is converted to bilirubin, which is then carried by albumin in the blood to the liver.

In the liver, most of the bilirubin is chemically attached to another molecule before it is released in the bile. This "conjugated" (attached) bilirubin is called direct bilirubin; unconjugated bilirubin is called indirect bilirubin. Total serum bilirubin equals direct bilirubin plus indirect bilirubin.

Conjugated bilirubin is released into the bile by the liver and stored in the gallbladder, or transferred directly to the small intestines. Bilirubin is further broken down by bacteria in the intestines, and those breakdown products contribute to the color of the feces. A small percentage of these breakdown compounds are taken in again by the body, and eventually appear in the urine.

Normal Results

Direct bilirubin: 0 to 0.3 mg/dL Total bilirubin: 0.3 to 1.9 mg/dL

Note: mg/dL = milligrams per deciliter

Normal values may vary slightly from laboratory to laboratory.

What Abnormal Results Mean

Jaundice is a yellowing of the skin and the white part of the eye, which occurs when bilirubin builds up in the blood at a level greater than approximately 2.5 mg/dL. Jaundice occurs because red blood cells are being broken down too fast for the liver to process. This might happen due to liver disease or bile duct blockage.

If the bile ducts are blocked, direct bilirubin will build up, escape from the liver, and end up in the blood. If the levels are high enough, some of it will appear in the urine. Only direct bilirubin appears in the urine. Increased direct bilirubin usually means that the biliary (liver secretion) ducts are obstructed.

Increased indirect or total bilirubin may be a sign of:

Crigler-Najjar syndrome Erythroblastosis fetalis Gilbert's disease Healing of a large hematoma (bruise or bleeding under the skin) Hemolytic anemia Hemolytic disease of the newborn Hepatitis Physiological jaundice (normal in newborns) Sickle cell anemia Transfusion reaction











Pernicious anemia

Increased direct bilirubin may indicate:

Bile duct obstruction Cirrhosis Dubin-Johnson syndrome (very rare) Hepatitis Intrahepatic cholestasis (buildup of bile in the liver) due to any cause

Additional conditions under which the test may be performed:

Biliary stricture Cholangiocarcinoma Cholangitis Choledocholithiasis Hemolytic anemia due to G6PD deficiency Hepatic encephalopathy Idiopathic aplastic anemia Idiopathic autoimmune hemolytic anemia Immune hemolytic anemia (including drug-induced immune hemolytic

anemia) Secondary aplastic anemia Thrombotic thrombocytopenic purpura Wilson's disease

Considerations

Factors that interfere with bilirubin testing are:

Hemolysis (breakdown) of blood will falsely increase bilirubin levels Lipids in the blood will falsely decrease bilirubin levels Bilirubin is light-sensitive; it breaks down in light

Alternative Names

Total bilirubin - blood; Unconjugated bilirubin - blood; Indirect bilirubin - blood; Conjugated bilirubin - blood; Direct bilirubin - blood

References

Berk PD, Korenblat KM. Approach to the patient with jaundice or abnormal liver test results. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 150.

Update Date: 2/23/2009

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of



















Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Newborn jaundice

Images

Erythroblastosis fetalis, photomicrograph

Jaundice infant

Exchange transfusion - series

Newborn jaundice is a condition marked by high levels of bilirubin in the blood. The increased bilirubin cause the infant's skin and whites of the eyes (sclera) to look yellow.

Causes

Bilirubin is a yellow pigment that's created in the body during the normal recycling of old red blood cells. The liver processes bilirubin in the blood so that it can be removed from the body in the stool.

Before birth, the placenta -- the organ that nourishes the developing baby -- removes the bilirubin from the infant so that it can be processed by the mother's liver. Immediately after birth, the baby's own liver begins to take over the job, but this can take time. Therefore, bilirubin levels in an infant are normally a little higher after birth.

High levels of bilirubin in the body can cause the skin to look yellow. This is called jaundice. Jaundice is present to some degree in most newborns. Such "physiological jaundice" usually appears between day 2 and 3, peaks between days 2 and 4, and clears by 2 weeks. Physiological jaundice usually causes no problems.

Breast milk jaundice is another common, usually non-harmful form of newborn jaundice. Breast milk may contain a substance that increases reuse of bilirubin in the intestines. Such jaundice appears in some healthy, breastfed babies after day 7 of life, and usually peaks during weeks 2 and 3. It may last at low levels for a month or more.



http://www.nlm.nih.gov/medlineplus/ency/presentations/100018_1.htm

Breastfeeding jaundice is a type of exaggerated physiological jaundice seen in breastfed babies in the first week, especially in those that are not nursing often enough. It is different than breast milk jaundice in that it occurs later and is caused by the milk itself.

Sometimes jaundice can be a sign of a serious underlying problem. Higher levels of bilirubin can be due to:

An event or condition that increases the number of red blood cells that needs to be processed

Anything that interferes with the body’s ability to process and remove bilirubin

The following increase the number of red blood cells that need to be processed:

Abnormal blood cell shapes o Congenital spherocytic anemia o Elliptocytosis

Blood type incompatibilities o ABO incompatibility (Mother has type O blood, baby does not) o Rh incompatibility (Mother is Rh negative, baby is not)

Cephalohematoma or other birth injury Glucose-6-phosphate dehydrogenase deficiency High levels of red blood cells (polycythemia)

o More common in small for gestational age babies o More common in some twins

Infection Prematurity Pyruvate kinase deficiency Transfusions

The following interfere with the body's ability to process and remove bilirubin:

Alpha-1 antitrypsin deficiency Biliary atresia Certain medications Congenital cytomegalovirus (CMV) infection Congenital herpes Congenital hypothyroidism Congenital rubella Congenital syphilis Congenital toxoplasmosis Crigler-Najjar syndrome Cystic fibrosis Gaucher's disease Gilbert syndrome Hypoxia Infections (such as sepsis) Lucey-Driscol syndrome Neonatal hepatitis











Niemann-Pick disease Prematurity

In otherwise healthy babies born at 35 weeks gestation or greater, those most likely to eventually develop signs of newborn jaundice are those who have:

A brother or sister who needed phototherapy for jaundice A high bilirubin level for their age, even if they are not yet jaundiced Been exclusively breastfeed, especially if weight is excessive Blood group incompatibility or other known red blood cell disease Cephalohematoma or significant bruising East Asian ancestry Jaundice in the first 24 hours of life

Symptoms

The main symptom is a yellow color of the skin. The yellow color is best seen right after gently pressing a finger onto the skin. The color sometimes begins on the face and then moves down to the chest, belly area, legs, and soles of the feet.

Sometimes, infants with significant jaundice have extreme tiredness and poor feeding.

Exams and Tests

All newborns should be examined for jaundice at least every 8 to 12 hours for the first day of life.

Any infant who appears jaundiced in the first 24 hours should have bilirubin levels measured immediately. This can be done with a skin or blood test.

Babies should be assigned a risk for later developing jaundice before they leave the hospital. Babies are classified as low risk, low intermediate risk, high intermediate risk, or high risk. Many hospitals do this by routinely checking total bilirubin levels on all babies at about 24 hours of age.

Further testing varies on the infant's specific situation and test results. For example, the possible cause of the jaundice should be sought for babies who require treatment or whose total bilirubin levels are rising more rapidly than expected.

Tests that will likely be done include:

Complete blood count Coomb's test Measurement of levels of specific types of bilirubin Reticulocyte count

The level of albumin in the baby's blood may also be checked. Low albumin levels may increase the risk of damage from excessive jaundice.

Treatment

Treatment is usually not necessary. Keep the baby well-hydrated with breast milk or formula. Frequent feedings encourage frequent bowel movements, which helps remove bilirubin through the stools. (Bilirubin is what gives stool a brown color).

Sometimes special blue lights are used on infants whose levels are very high. This is called phototherapy. These lights work by helping to break down bilirubin in the skin. The infant is placed naked under artificial light in a protected isolette to maintain constant temperature. The eyes are protected from the light. The American Academy of Pediatrics recommends that breastfeeding be continued through phototherapy, if possible.

In the most severe cases of jaundice, an exchange transfusion is required. In this procedure, the baby's blood is replaced with fresh blood. Treating severely jaundiced babies with intravenous immunoglobulin may also be very effective at reducing bilirubin levels.

Outlook (Prognosis)

Usually newborn jaundice is not harmful. For most babies, jaundice usually resolves without treatment within 1 to 2 weeks. However, if significant jaundice is untreated, very high levels of bilirubin can damage the brain. For babies who require treatment, the treatment is usually quite effective.

Possible Complications

Rare, but serious, complications from high bilirubin levels include:

Cerebral palsy Deafness Kernicterus -- brain damage from very high bilirubin levels

When to Contact a Medical Professional

All babies should be seen by a health care provider in the first 5 days of life to check for jaundice.

Those who spend less than 24 hours in a hospital should be seen by age 72 hours.

Infants sent home between 24 and 48 hours should be seen again by age 96 hours.

Infants sent home between 48 and 72 hours should be seen again by age 120 hours.

Jaundice is an emergency if the baby has a fever, has become listless, or is not feeding well. Jaundice may be dangerous in high-risk newborns.

Jaundice is generally NOT dangerous in term, otherwise healthy newborns. Call the infant's health care provider if jaundice is severe (the skin is bright yellow), if jaundice continues to increase after the newborn visit, lasts longer than 2 weeks, or if other symptoms develop. Also call the doctor if the feet, particularly the soles, are yellow.

Prevention

In newborns, some degree of jaundice is normal and probably not preventable. The risk of significant jaundice can often be reduced by feeding babies at least 8 to 12 times a day for the first several days and by carefully identifying infants at highest risk.

All pregnant women should be tested for blood type and unusual antibodies. If the mother is Rh negative, follow-up testing on the infant's cord is recommended. This may also be done if the mother blood type is O+, but it not necessarily required if careful monitoring takes place.

Careful monitoring of all babies during the first 5 days of life can prevent most complications of jaundice. Ideally, this includes:

Considering a baby's risk for jaundice Checking bilirubin level in the first day or so Scheduling at least one follow-up visit the first week of life for babies sent

home from the hospital in 72 hours

Alternative Names

Jaundice of the newborn; Neonatal hyperbilirubinemia

References

American Academy of Pediatrics (AAP). Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004 Jul;114(1):297-316.

Mercier CE, Barry SE, Paul K, et al. Improving Newborn Preventive Services at the Birth Hospitalization: A Collaborative, Hospital-Based Quality-Improvement Project. Pediatrics. 2007 Sep;120(3):481-488.

Moerschel SK, Cianciaruso LB, Tracy LR. A practical approach to neonatal jaundice. American Family Physician. 2008 May;77(9).


Updated by: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by

David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. Previously reviewed by Alan Greene, MD, FAAP, Department of Pediatrics, Stanford University School of Medicine, Lucile Packard Children's Hospital; Chief of Future Health, A.D.A.M., Inc., September 2007.

Rh incompatibility

Images

Erythroblastosis fetalis, photomicrograph

Jaundice infant

Antibodies

Exchange transfusion - series

Rh Incompatibility - series

Read More

Antibody Hemoglobin Bilirubin - blood Jaundice - yellow skin Anemia Heart failure Poor feeding in infants Seizures

Rh incompatibility is a condition that develops when a pregnant woman has Rh-negative blood and the baby in her womb has Rh-positive blood.

Causes














During pregnancy, red blood cells from the fetus can get into the mother's bloodstream as she nourishes her child through the placenta. If the mother is Rh-negative, her system cannot tolerate the presence of Rh-positive red blood cells.

In such cases, the mother's immune system treats the Rh-positive fetal cells as if they were a foreign substance and makes antibodies against the fetal blood cells. These anti-Rh antibodies may cross the placenta into the fetus, where they destroy the fetus's circulating red blood cells.

First-born infants are often not affected -- unless the mother has had previous miscarriages or abortions, which could have sensitized her system -- as it takes time for the mother to develop antibodies against the fetal blood. However, second children who are also Rh-positive may be harmed.

Hemoglobin changes into bilirubin, which causes an infant to become yellow (jaundiced). The jaundice of Rh incompatibility, measured by the level of bilirubin in the infant's bloodstream, may range from mild to dangerously high levels of bilirubin.

Rh incompatibility develops only when the mother is Rh-negative and the infant is Rh-positive. Special immune globulins, called RhoGAM, are now used to prevent this sensitization. In developed countries such as the US, hydrops fetalis and kernicterus have decreased markedly in frequency as a result of these preventive measures.

Symptoms

Rh incompatibility can cause symptoms ranging from very mild to fatal. In its mildest form, Rh incompatibility causes destruction of red blood cells.

Symptoms may include:

Jaundice Hypotonia Motormental retardation Polyhydramnios (before birth)

Exams and Tests

There may be:

A positive direct Coombs test result Higher-than-normal levels of bilirubin in the baby's cord blood Signs of red blood cell destruction in the infant's blood

Treatment







Since Rh incompatibility is almost completely preventable with the use of RhoGAM, prevention remains the best treatment. Treatment of the already affected infant depends on the severity of the condition.

Mild Rh incompatibility may be treated with:

Aggressive hydration Phototherapy using bilirubin lights

Outlook (Prognosis)

Full recovery is expected for mild Rh incompatibility.


Possible complications include:

Hydrops fetalis Kernicterus Neurological syndrome with mental deficiency, movement disorder,

hearing loss, speech disorder, and seizures


Call your health care provider if you think or know you are pregnant and have not yet seen a doctor.

Prevention

Rh incompatibility is almost completely preventable. Rh-negative mothers should be followed closely by their obstetricians during pregnancy.

If the father of the infant is Rh-positive, the mother is given a mid-term injection of RhoGAM and a second injection within a few days of delivery.

These injections prevent the development of antibodies against Rh-positive blood. This effectively prevents the condition.

Alternative Names

Rh-induced hemolytic disease of the newborn


Updated by: Deirdre O’Reilly, MD, MPH, Neonatologist, Division of Newborn Medicine, Children’s Hospital Boston and Instructor in Pediatrics, Harvard Medical School, Boston, Massachusetts. Review Provided by VeriMed Healthcare Network.


ABO incompatibility

Images

Jaundice infant

Antibodies

ABO incompatibility is a reaction of the immune system that occurs if two different and not compatible blood types are mixed together.

Causes

A, B, and O are the three major blood types. The types are based on small substances (molecules) on the surface of the blood cells. In people who have different blood types, these molecules act as immune system triggers (antigens).

Each person has a combination of two of these surface molecules. Type O lacks any molecule. The different blood types are:

Type A (AA or AO molecules) Type B (BB or BO molecules) Type AB Type O

People who have one blood type form proteins (antibodies) that cause their immune system to react against other blood types. Being exposed to another type of blood can cause a reaction. This is important when a patient needs to receive blood (transfusion) or have an organ transplant. The blood types must be matched to avoid an ABO incompatibility reaction.

For example:

A patient with type A blood will react against type B or type AB blood A patient with type B blood will react against type A or type AB blood A patient with type O blood will react against type A, type B, or type AB

blood

Because type O lacks any surface molecules, type O blood does not cause an immune response. This is why type O blood cells can be given to patients of any blood type. People with type O blood are called "universal donors." However, people with type O can only receive type O blood.



Since antibodies are in the liquid part of blood (plasma), both blood and plasma transfusions must be matched to avoid an immune reaction.

Symptoms

The following are symptoms of transfusion reactions:

Back pain Blood in urine Feeling of "impending doom" Fever Yellow skin (jaundice)

Exams and Tests

Bilirubin level is high Complete blood count (CBC) shows damaged red blood cells, may also

show mild anemia Lab testing of patient's and donor's blood shows that they are not

compatible

Treatment

Treatment may include:

Drugs used to treat allergic reactions (antihistamines) Drugs used to treat swelling and allergies (steroids) Fluids given through a vein (intravenous) Medicines to raise blood pressure if it drops too low

Outlook (Prognosis)

This can be a very serious problem which can even result in death. With the right treatment, a full recovery is likely.


Kidney failure Low blood pressure needing intensive care Death


Call your health care provider if you have recently had a blood transfusion or transplant and you have the symptoms listed above.

Prevention

Careful testing of donor and patient blood types before transfusion or transplant can prevent this problem.





References

McPherson RA, Pincus MR. Henry's Clinical Diagnosis and Management by Laboratory Methods. 21st ed. Philadelphia, Pa: Saunders; 2006.

Hoffman R, Benz E, Shattil S, Furie B, Cohen H. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingstone; 2004.


Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Stuart I. Henochowicz, MD, FACP, Associate Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

Glucose-6-phosphate dehydrogenase deficiency

Images

Blood cells

Read More

Enzyme Anemia Acute Chronic Hemolytic anemia Congenital spherocytic anemia Newborn screening tests

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection.

Causes

javascript:hwsopen();

javascript:edpolopen();

javascript:edpolopen();

javascript:ppopen();









G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase, which helps red blood cells work properly.

Too little G6PD leads to the destruction of red blood cells. This process is called hemolysis. When this process is actively occurring, it is called a hemolytic episode. The episodes are usually brief, because the body continues to produce new red blood cells, which have normal activity.

Red blood cell destruction can be triggered by infections, severe stress, certain foods (such as fava beans), and certain drugs, including:

Antimalarial drugs Aspirin Nitrofurantoin Nonsteroidal anti-inflammatory drugs (NSAIDs) Quinidine Quinine Sulfonamides

Other chemicals, such as those in mothballs, can also trigger an episode.

In the United States, G6PD deficiency is more common among blacks than whites. Men are more likely to have this disorder than women.

You are more likely to develop this condition if you:

Are African American Are of Middle Eastern decent, particularly Kurdish or Sephardic Jewish Are Male Have a family history of the deficiency

A form of this disorder is common in whites of Mediterranean descent. This form is also associated with acute episodes of hemolysis. Episodes are longer and more severe than in the other types of the disorder.

Symptoms

Persons with this condition do not display any signs of the disease until their red blood cells are exposed to certain chemicals in food or medicine, or to stress.

Symptoms are more common in men and may include:

Dark urine Enlarged spleen Fatigue Paleness Rapid heart rate Shortness of breath Yellow skin color (jaundice)







Exams and Tests

A blood test can be done to check the level of G6PD. See: G6PD screen

Other tests that may be done include:

Bilirubin level Complete blood count, including red blood cell count Hemoglobin - blood Hemoglobin - urine Haptoglobin level LDH test Methemoglobin reduction test Reticulocyte count

Treatment

Treatment may involve:

Medicines to treat an infection, if present Stopping any drugs that are causing red blood cell destruction Transfusions, in some cases

Outlook (Prognosis)

Spontaneous recovery from hemolytic crises is the usual outcome.


Rarely, kidney failure or death may occur following a severe hemolytic event.


Call for an appointment with your health care provider if you have symptoms of this condition.

Call your health care provider if you have been diagnosed with G6PD deficiency and symptoms do not disappear after treatment.

Prevention

Persons with G6PD deficiency must strictly avoid things that can trigger an episode. Talk to your health care provider about your medications.

Genetic counseling or testing may be available to those who have a family history of the condition.

Alternative Names








G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency

References

Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingston; 2005:658-60.

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004:1027-28.


Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Hemolytic anemia

Images

Red blood cells, sickle cell

Red blood cells, multiple sickle cells

Red blood cells, sickle cells

Red blood cells, sickle and pappenheimer

Blood cells

Read More






Anemia Intrinsic factor Autoimmune disorders Sickle cell anemia Glucose-6-phosphate dehydrogenase deficiency Congenital spherocytic anemia Idiopathic autoimmune hemolytic anemia Immune hemolytic anemia Cardiovascular Stroke

Hemolytic anemia is a condition in which there are not enough red blood cells in the blood, due to the premature destruction of red blood cells. There are a number of specific types of hemolytic anemia, which are described individually.

Causes

Hemolytic anemia occurs when the bone marrow is unable to increase production to make up for the premature destruction of red blood cells. If the bone marrow is able to keep up with the early destruction, anemia does not occur (sometimes called compensated hemolysis).

There are many types of hemolytic anemia, which are classified by the reason for the premature destruction of red blood cells. The defect may be in the red blood cell itself (intrinsic factor), or outside the red blood cell (extrinsic factors).

Intrinsic factors are often present at birth (hereditary). They include:

Abnormalities in the proteins that build normal red blood cells Differences in the protein inside a red blood cell that carries oxygen

(hemoglobin)

Extrinsic factors include:

Abnormal immune system responses Blood clots in small blood vessels Certain infections Side effects from medications

Types of hemolytic anemia include:

Hemoglobin SC disease (similar in symptoms to sickle-cell anemia) Hemolytic anemia due to G6PD deficiency Hereditary elliptocytosis Hereditary ovalocytosis Hereditary spherocytosis Idiopathic autoimmune hemolytic anemia Malaria Microangiopathic hemolytic anemia (MAHA) Non-immune hemolytic anemia caused by chemicals or toxins



















Paroxysmal nocturnal hemoglobinuria (PNH) Secondary immune hemolytic anemia Sickle-cell anemia Thalassemia Transfusion of blood from a donor with a different blood type

Symptoms

Chills Dark urine Enlarged spleen Fatigue Pale skin color (pallor) Rapid heart rate Shortness of breath Yellow skin color (jaundice)

Exams and Tests

These are tests for red blood cell destruction (hemolysis). Specific tests can identify the types of hemolytic anemia. They are usually performed when hemolysis is suspected or has been determined.

Absolute reticulocyte count Free hemoglobin in the serum or urine Hemosiderin in the urine Red blood cell count (RBC) and hemoglobin Serum haptoglobin levels Serum indirect bilirubin levels Serum LDH Urine and fecal urobilinogen

Directly measuring the red cell life span with radioactive tagging techniques shows a shortened life span.

This disease may also affect the following test results, depending on the specific cause:

AST Coombs' test, direct Coombs' test, indirect Donath-Landsteiner test Febrile or cold agglutinins Hematocrit Leukocyte alkaline phosphatase Peripheral blood smear Platelet count Protein electrophoresis - serum RBC indices Serum creatinine





























Serum ferritin Serum iron Serum potassium level Serum uric acid TIBC White blood count differential

Treatment

Treatment depends on the type and cause of the hemolytic anemia. Folic acid, iron replacement, and corticosteroids may be used. In emergencies, a blood transfusion or removal of the spleen (splenectomy) may be necessary.

Outlook (Prognosis)

The outcome depends on the type and cause of hemolytic anemia.


The complications depend on the specific type of hemolytic anemia. Severe anemia can cause cardiovascular collapse. Severe anemias can aggravate heart disease, lung disease, or cerebrovascular disease.


Call for an appointment with your health care provider if you develop symptoms of hemolytic anemia.

Prevention

There is no known prevention for hemolytic anemia.

Alternative Names

Anemia - hemolytic

References

Schwartz RS. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 164.


Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.










Transfusion reaction - hemolytic

Images

Surface proteins causing rejection

Read More

Immune response Antigen Antibody Protein in diet Acute kidney failure Shock

A hemolytic transfusion reaction is a serious problem that occurs after a patient receives a transfusion of blood. The red blood cells that were given to the patient are destroyed by the patient's own immune system.

Causes

Blood is classified into different blood types called A, B, AB, and O.

The immune system normally can tell its own blood cells from blood cells from another person. If other blood cells enter your body, your immune system may make antibodies again them. These antibodies will work to destroy the blood cells that the body does not recognize. For example, a person with type A blood makes antibodies against type B blood cells.

Another way blood cells may be classified is by Rh factors. People who have Rh factors in their blood are called "Rh positive." People without these factors are called "Rh negative." Rh negative people form antibodies against Rh factor if they receive Rh positive blood.

There are also other factors to identify blood cells, in addition to ABO and Rh.

Blood that you receive in a transfusion must be compatible. Being compatible means that your body will not form antibodies against the blood you receive. Blood transfusion between compatible groups (such as O+ to O+) usually causes no problem. Blood transfusion between incompatible groups (such as A+ to O-) causes an immune response. This can lead to a very serious transfusion reaction. The immune system attacks the donated blood cells, causing them to burst.








Today, all blood is carefully screened. Modern lab methods and many checks have helped make these transfusion reactions very rare.

Symptoms

Bloody urine Chills Fainting or dizziness Fever Flank pain or back pain Rash

Symptoms of transfusion reaction usually appear during or right after the transfusion. Sometimes, they may develop after several days (delayed reaction).

Exams and Tests

This disease may change the results of these tests:

Bilirubin CBC Coombs' test, direct Coombs' test, indirect Fibrin degradation products Haptoglobin Hematocrit Hemoglobin RBC count Serum creatinine Serum hemoglobin Urinalysis

Treatment

Therapy can prevent or treat the severe effects of a hemolytic transfusion reaction. If symptoms occur during the transfusion, the transfusion is stopped immediately. Blood samples from the person getting the transfusion and from the donor may be tested to tell whether symptoms are being caused by a transfusion reaction.

Mild symptoms may be treated with the following:

Antihistamine drugs (such as diphenhydramine) can treat itching and rash.

The pain reliever, acetaminophen can reduce fever and discomfort. Corticosteroids (such as prednisone or dexamethasone) can reduce the

immune response. Fluids given through a vein (intravenous) and other medications may be

used to treat or prevent kidney failure and shock.



















Outlook (Prognosis)

The outcome depends on the severity of the reaction. The disorder may disappear without problems. Or, it may be severe and life threatening.


Acute kidney failure Anemia Discomfort Lung dysfunction Shock


Tell your health care provider if you are having a blood transfusion and you have had a reaction before.

Prevention

Donated blood is put into ABO and Rh groups to reduce the risk of transfusion reaction.

Before a transfusion, patient and donor blood is tested (crossmatched) to see if it is compatible. A small amount of donor blood is mixed with a small amount of patient blood. The mixture is checked under a microscope for signs of antibody reaction.

Before the transfusion is given, the health care provider will usually check again to make sure you are receiving the right unit of blood.

Alternative Names

Blood transfusion reaction

References

Goodnough L. Transfusion medicine. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 183.


Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.




Crigler-Najjar syndrome

Images

Liver anatomy

Read More

Jaundice - yellow skin Enzyme Bilirubin - blood Rh incompatibility

Crigler-Najjar syndrome is a very rare inherited disorder in which bilirubin (a substance made by the liver) cannot be broken down.

Causes

Crigler-Najjar syndrome is caused by an abnormal gene. The gene fails to make the enzyme that normally converts bilirubin into a form that can easily be removed from the body. Without this enzyme, bilirubin can build up in the body and lead to jaundice (yellow discoloration of skin and eyes) and damage to the brain, muscles, and nerves.

Crigler-Najjar (type 1) is the early-onset form of the disease. Arias syndrome (type 2) is a later-onset condition.

The syndrome runs in families (inherited). A child must get the defective gene from both parents to develop the severe form of the condition. Parents who are carriers (with just one defective gene) have about half the enzyme activity of a normal adult.

Symptoms

Confusion and changes in thinking Yellow skin (jaundice) and yellow in the whites of the eyes (icterus),

which begin a few days after birth and get worse over time

Exams and Tests

Tests used to evaluate liver function include:

Conjugated (bound) bilirubin Liver biopsy, enzyme assay Total bilirubin level Unconjugated (unbound) bilirubin in blood












Treatment

Light treatment (phototherapy) is needed on a regular basis throughout life. In infants this is done using bilirubin lights (bili or 'blue' lights). Phototherapy becomes less successful after age 4, because thickened skin blocks the light.

Liver transplantation has been used successfully in some people with type 1 disease.

Blood transfusions may help control the amount of bilirubin in blood plasma. Calcium compounds are sometimes used to bind with and remove bilirubin in the gut.

The drug phenobarbitol is sometimes used to treat Arias syndrome (type 2).

Outlook (Prognosis)

Milder forms of the disease (type 2) do not cause severe toxicity, liver damage, or changes in thinking during childhood. People affected still have jaundice, but they have fewer symptoms and less organ damage.

Infants with the severe form of the disease (type 1) may continue to have jaundice into adulthood, and may need daily treatment. If left untreated, this severe form of the disease will lead to death in childhood.

People with this condition who reach adulthood will develop brain damage due to jaundice (kernicterus), even with regular treatment. The life expectancy for type 1 disease is 30 years.


Possible complications include:

A form of brain damage caused by jaundice (kernicterus) Chronic yellow skin/eyes


Seek genetic counseling if you are planning to have children and have a family history of Crigler-Najjar.

Call your health care provider if you or your newborn infant has jaundice that does not go away.

Prevention

Genetic counseling is recommended for prospective parents with a family history of Crigler-Najjar syndrome. Blood testing can identify people who carry the gene.


Alternative Names

Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar)

References

Carey RG, Balistreri WF. Metabolic Diseases of the Liver. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 354.


Updated by: Diana Chambers, MS, EdD, Certified Genetics Counselor (ABMG), Charter Member of the ABGC, University of Tennessee, Memphis, TN. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Thalassemia

Images

Thalassemia major

Thalassemia minor

Read More

Hemoglobin Anemia Endocrine glands

Thalassemia is an blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen. The disorder results in excessive destruction of red blood cells and anemia.

See also:

Hemolytic anemia Sickle cell disease

Causes









Hemoglobin is made of two proteins: Alpha globin and beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of these proteins.

There are two main types of thalassemia:

Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated).

Beta thalassemia occurs when similar gene defects affect production of the beta globin protein.

Alpha thalassemias occur most commonly in persons from southeast Asia, the Middle East, China, and in those of African descent.

Beta thalassemias occur in persons of Mediterranean origin, and to a lesser extent, Chinese, other Asians, and African Americans.

There are many forms of thalassemia. Each type has many different subtypes. Both alpha and beta thalassemia include the following two forms:

Thalassemia major Thalassemia minor

You must inherit the defective gene from both parents to develop thalassemia major.

Thalassemia minor occurs if you receive the defective gene from only one parent. Persons with this form of the disorder are carriers of the disease and do not have symptoms.

Beta thalassemia major is also called Cooley's anemia.

Risk factors for thalassemia include:

Asian, Chinese, Mediterranean, or African American ethnicity Family history of the disorder

Symptoms

The most severe form of alpha thalassemia major causes stillbirth (death of the unborn baby during birth of the late stages of pregnancy).

Children born with thalessemia major (Cooley's anemia)are normal at birth, but develop severe anemia during the first year of life.

Other symptoms can include:

Bone deformities in the face Fatigue Growth failure




Liver and spleen swelling Shortness of breath Yellow skin (jaundice)

Persons with the minors form of alpha and beta thalassemia have small red blood cells (that can be seen under a microscope), but no symptoms.

Exams and Tests

A physical exam may reveal a swollen (enlarged) spleen.

A blood sample will be taken and sent to a laboratory for examination.

Red blood cells will appear small and abnormally shaped when looked at under a microscope.

A complete blood count (CBC) reveals anemia. A test called hemoglobin electrophoresis shows abnormal hemoglobin.

A test called mutational analysis can help detect alpha thalassemia that cannot be detected with hemoglobin electrophoresis.

Treatment

Treatment for thalassemia major often involves regular blood transfusions and folate supplements.

If you receive blood transfusions, you should not take iron supplements. Doing so can cause a high amount of iron to build up in the body, which can be harmful.

Persons who receive significant numbers of blood transfusions need a treatment called chelation therapy to remove iron from the body.

Bone marrow transplant may help treat the disease in some patients, especially children.

Outlook (Prognosis)

Severe thalassemia can cause early death due to heart failure a, usually between ages 20 and 30. Frequent blood transfusions with therapy to remove iron from the body helps improve the outcome.

Less severe forms of thalassemia usually do not result in a shorter life span.


Untreated, thalassemia major leads to heart failure and liver problems, and makes a person more likely to develop infections.








Blood transfusions can help control some symptoms, but may result in too much iron which can damage the heart, liver, and endocrine system.


Call for an appointment with your health care provider if:

You or your child have symptoms of thalassemia You are being treated for the disorder and new symptoms develop

Prevention

Genetic counseling and prenatal screening may be available to those with a family history of this condition who are planning to have children.

Alternative Names

Mediterranean anemia; Cooley's anemia; Beta thalassemia; Alpha thalassemia

References

Forget BG, Cohen AR. Thalassemia syndromes. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2005:chap 35.

Debaun MR, Vichinsky E. Hemoglobinopathies. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap.462.


Updated by: Todd Gersten, M.D., Hematology/Oncology, Palm Beach Cancer Institute,West Palm Beach, FL. Review provided by VeriMed Healthcare Network. Also reviewed by Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital; and David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Gilbert's disease

Images

Digestive system



Digestive system organs

Read More

Bilirubin - blood Jaundice - yellow skin Benign

Gilbert's disease is a common disorder passed down through families.It affects the way bilirubin is processed by the liver, and causes jaundice.

Causes

Gilbert's disease affects up to 10% of people in some Caucasian populations. The condition is usually noncancerous (benign).

Symptoms

Fatigue Yellowing of the skin and whites of the eyes (mild jaundice)

Note: Jaundice typically appears during times of exertion, stress, not eating, and infection.

Exams and Tests

An indirect bilirubin blood test shows changes that occur with Gilbert's disease.

Treatment

No treatment is necessary for Gilbert's disease.

Outlook (Prognosis)

Jaundice may come and go throughout your life, especially during illnesses such as colds. However, it usually does not cause health problems.


There are no known complications.


Call your health care provider if you have jaundice or persistent abdominal pain.

Prevention









There is no proven prevention.

Alternative Names

Icterus intermittens juvenilis; Low-grade chronic hyperbilirubinemia; Familial non-hemolytic-non-obstructive jaundice; Constitutional liver dysfunction; Unconjugated benign bilirubinemia

References

Berk PD, Korenblat KM. Approach to the patient with jaundice or abnormal liver test results. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 150.


Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias: http://www.nlm.nih.gov/medlineplus/ency/article/000301.htm

Bilirubin - blood

Documents

Transcript of Bilirubin - blood