Posts Tagged bilirubin

What is Biliary Atresia?

Posted by on Thursday, 31 March, 2011

Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes inside and outside the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia, bile ducts that are located inside or outside the liver are blocked. When the bile is unable to leave the liver through the bile ducts, the liver becomes damaged and many vital body functions are affected.

The cause of biliary atresia is not known. Some researchers and physicians believe that babies are born with biliary atresia, implying the problem with the bile ducts occurred during pregnancy while the liver was developing. Others believe that the disease begins after birth, and may be caused by exposure to infections or exposures to toxic substances.

Biliary atresia does not seem to be linked to medications the mother took, illnesses the mother had, or anything else the mother did during her pregnancy. Currently, there is not a genetic link known for biliary atresia.

The disease is unlikely to occur more than once in a family.

  • Biliary atresia is the most common cause of chronic liver disease in neonates.
  • Biliary atresia occurs once in every 15,000 births.
  • Asian populations are most frequently affected. African Americans are affected approximately twice as much as Caucasians. 

Biliary Atresia

Biliary atresia causes liver damage and affects numerous important processes that allow the body to function normally. It is a life-threatening disease and is fatal without treatment.

Infants with biliary atresia usually appear healthy at birth. Most often, symptoms develop between two weeks to two months of life, and may include:

  • Jaundice
  • Dark urine
  • Light colored stools
  • Distended abdomen
  • Weight loss

Jaundice is a yellow discoloration of the skin and whites of the eyes due to an abnormally high level of bilirubin (bile pigment) in the bloodstream, which is then excreted through the kidneys. High levels of bilirubin may be attributed to inflammation or other abnormalities of the liver cells, or blockage of the bile ducts. Jaundice is usually the first sign, and sometimes the only sign, of liver disease.

Symptoms of biliary atresia may resemble other liver conditions or medical problems. Please consult your child’s physician for a diagnosis.

A physician or healthcare provider will examine your child and obtain a medical history. Several diagnostic procedures are done to help evaluate the problem and may include the following:

  • Blood tests
  • Liver enzymes 
- elevated levels of liver enzymes can alert physicians to liver damage or injury, since the enzymes leak from the liver into the bloodstream under these circumstances.
  • Bilirubin – bilirubin is produced by the liver and is excreted in the bile. Elevated levels of bilirubin often indicate an obstruction of bile flow or a defect in the processing of bile by the liver.
  • Albumin and total protein
 Below-normal levels of proteins made by the liver are associated with many chronic liver disorders.
  • Clotting studies, such as prothrombin time (PT) and partial thromboplastin time (PTT)
Tests that measure the time it takes for blood to clot. Blood clotting requires vitamin K and proteins made by the liver.
  • Liver cell damage and bile flow obstruction can both interfere with proper blood clotting.
  • Viral studies – including hepatitis and HIV
Checking for viruses in the bloodstream can help determine the cause of the liver problems.

Blood culture
 – Checking for bacterial infection in the bloodstream that can affect the liver may be used to diagnose biliary atresia. Also imaging tests:

  • Abdominal ultrasound – a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view the liver, gallbladder, and bile ducts.
  • Hepatobiliary (HIDA) scan – a low radioactive isotope (technetium) is injected into the child’s vein. The liver and intestine are scanned by a nuclear medicine machine. If the isotope passes through the liver into the intestine, the bile ducts are open and the child does not have biliary atresia.

The test that gives the most definitive diagnosis is a liver biopsy. A tissue sample is taken from your child’s liver and examined for abnormalities, allowing biliary atresia to be distinguished from other liver problems.

Specific treatment for biliary atresia will be determined by your child’s physician based on the following:

  • The extent of the problem
  • Your child’s age, overall health, and medical history
  • Your child’s tolerance for specific medications, procedures, or therapies
  • Expectations for the course of the problem
  • The opinion of the physicians involved in the child’s care
    your opinion and preference.

Biliary atresia is an irreversible problem. There are no medications that can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Until that happens, biliary atresia will not be curable. However, two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life. Your child’s physician can help determine whether either of these operations are an option.

Kasai portoenterostomy
, this operation connects the bile drainage from the liver directly to the intestinal tract. It is most successful when done before an infant is 8 weeks old. The Kasai procedure is helpful because it can allow a child to grow and remain in fairly good health for several years. Eventually, cholestasis (backup of bile in the liver) will occur, causing liver damage. Up to 80 percent of children who undergo the Kasai portoenterostomy will eventually need to have a liver transplant.

Liver transplant
- a liver transplant operation removes the damaged liver and replaces it with a new liver from a donor. The new liver can be either:

  • A whole liver, received from a child who has died.
  • Part of a liver, received from a child who has died or
    part of a liver, received from a relative or other person whose tissue types match the child’s tissue type.

After surgery, the new liver begins functioning and the child’s health often improves quickly. After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body’s normal protective mechanisms that helps fight against invasion of viruses, tumors, and other foreign substances. Anti-rejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ. Frequent contact with the physicians and other members of the transplant team is crucial after a liver transplant.

Before your child has either one of these operations, nutrition may be a problem. With biliary atresia, not enough bile reaches the intestine to assist with the digestion of fats in the diet. Protein deficiencies may occur due to liver damage. Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because of a faster metabolism.

Your physician may recommend that a pediatric nutritionist make recommendations regarding your child’s diet. Nutritional guidelines may include the following:

  • Provide your child with a good, well-balanced diet.
  • Supplement your child’s diet with vitamins, as directed by your child’s physician.

MCT (medium-chain triglyceride) oil or infant formulas with MCT (Portagen® or Pregestimil®) may be recommended to add extra calories to help your child grow. Medium-chain triglycerides are more easily digested without bile than other types of fats. MCT oil can be added to foods and liquids that your child eats.
Provide your child with high-calorie liquid feedings, as directed by your child’s physician. Some children with liver disease become too sick to eat normally. In this case, your physician may recommend that your child have liquid feedings given to help meet his/her body’s requirements. These feedings are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus, and into the stomach. A high-calorie liquid can be given through the tube to supplement your child’s diet if he/she is able to eat only small amounts of food, or to replace meals if your child is too sick to eat.

After surgery, your child’s digestion may return to normal, or you may still need to give extra vitamins and/or work with your child’s diet. Please consult your child’s physician for recommendations.
Many factors affect the long-term outlook for these children.

Some of them include:

  • The extent of bile duct damage.
  • The age at which either a Kasai portoenterostomy or liver transplant is done.
  • The extent of liver damage that has occurred.
  • The overall health of your child.

Over 65 percent of children who have the Kasai portoenterostomy will eventually require a liver transplant. After liver transplant, the child’s health will usually improve; however, a rigorous medical regimen must be followed.


Jaundice in newborns

Posted by on Friday, 11 March, 2011

Over half of all newborns develop some amount of jaundice, a yellow coloring in their skin, during the first week. This is usually a temporary condition, but may be a more serious sign of another illness. Jaundice is caused by the breakdown of red blood cells. As the old cells are broken down, hemoglobin is changed into bilirubin and removed by the liver. The build-up of bilirubin in the blood is called hyperbilirubinemia. Because bilirubin has a pigment, or coloring, it causes a yellowing of the baby’s skin and tissues. As liver function matures, the jaundice goes away. A premature infant is more likely to develop jaundice. The yellow tint to the skin can often be seen by gently pressing on the baby’s forehead or chest and watching the color return.
There are several types of jaundice:

  • Physiologic jaundice
 – Physiologic jaundice occurs as a “normal” response to the baby’s limited ability to excrete bilirubin in the first days of life.
  • Breast Milk Jaundice-
About 2 percent of breastfed babies develop jaundice after the first week. Some develop breast milk jaundice in the first week due to low calorie intake or dehydration.
  • Hemolysis
 Jaundice– May occur with the breakdown of red blood cells due to hemolytic disease of the newborn (Rh disease), having too many red blood cells, or bleeding.

Jaundice related to inadequate liver function
Jaundice may be related to inadequate liver function due to infection or other factors.

Although low levels of bilirubin are not usually a concern, large amounts can circulate to tissues in the brain and may cause seizures and brain damage. This is a condition called kernicterus.

The following are the most common symptoms of jaundice. However, each baby may experience symptoms differently.

Symptoms may include:

  • Yellow coloring of the baby’s skin – usually beginning on the face and moving down the body.
  • Poor feeding or lethargy

baby with jaundice

Symptoms of jaundice may resemble other conditions or medical problems. Always consult your baby’s physician for a diagnosis.

The timing of the appearance of jaundice helps with the diagnosis. Jaundice appearing in the first 24 hours is quite serious and usually requires immediate treatment. When jaundice appears on the second or third day, it is usually “physiologic.” However, it can be a more serious type of jaundice. When jaundice appears on the third day to the first week, it may be due to an infection. Later appearance of jaundice, in the second week, is often related to breast milk feedings, but may have other causes.

Laboratory testing for hyperbilirubinemia may include:

  • Direct and indirect bilirubin levels – These levels reflect whether the bilirubin is bound with other substances by the liver so that it can be excreted (direct), or is circulating in the blood circulation (indirect).
  • Red blood cell counts
  • Blood type and testing for Rh incompatibility (Coomb’s test)

Specific treatment for jaundice will be determined by your baby’s physician based on:

  • Your baby’s gestational age, overall health, and medical history.
  • Extent of the disease
  • Your baby’s tolerance for specific medications, procedures, or therapies
  • Expectations for the course of the disease
  • Your opinion or preference- This depends on many factors, including the cause of the jaundice and the level of bilirubin. The goal is to keep the level of bilirubin from increasing to dangerous levels.

Treatment may include:

  1. Phototherapy– 
Because bilirubin absorbs light, jaundice and increased bilirubin levels usually decrease when the baby is exposed to special blue spectrum lights. Phototherapy may take several hours to begin working and it is used throughout the day and night. The baby’s position is changed to allow all of the skin to be exposed to the light. The baby’s eyes must be protected and the temperature monitored during phototherapy. Blood levels of bilirubin are checked to monitor if the phototherapy is working. Use of a fiberoptic blanket
 Another form of phototherapy is a fiberoptic blanket placed under the baby. This may be used alone or in combination with regular phototherapy.
  2. Exchange transfusion
– May be used to replace the baby’s damaged blood with fresh blood. This helps increase the red blood cell count and lower the levels of bilirubin. An exchange transfusion is done by alternating giving and withdrawing blood in small amounts through a vein or artery. Exchange transfusions may need to be repeated if the bilirubin levels remain high.
  3. Discontinued breast feeding
- Treatment of breast milk jaundice often requires stopping the breastfeeding for one to two days. Giving the baby formula often helps lower the bilirubin levels. Breastfeeding can then be resumed.
  4. Treatment of underlying conditions
- Treating any underlying cause of hyperbilirubinemia, such as infection.

While jaundice cannot be totally prevented, early recognition and treatment are important in preventing bilirubin levels from rising to dangerous levels. If your baby’s color is turning more yellow, promptly call your baby’s physician.