Biliary atresia

What is biliary atresia?

Bile is an important fluid produced by your child's liver and stored in the gallbladder. In a healthy system, it flows through a tube called the common bile duct to the small intestine, where it helps to digest food.

If your child has biliary atresia, the common bile duct is blocked or damaged, so that it's impossible for bile to flow through it, just as it's hard for water to pass through a clogged pipe. This is called cholestasis, or poor bile flow, and quickly leads to malnutrition and liver damage. Biliary atresia symptoms, such jaundice and dark urine, typically occur within the first few weeks of a child's life.

It is fatal if left untreated. This condition:

• is the most common cause of chronic liver disease in newborns
• occurs once in every 30,000 births
• is the most common reason for liver transplants in children
• affects children of all races and ethnicities, but may be more common in Asians

There are two main reasons why biliary atresia is a problem. First, it makes it harder for bile to get to your child’s small intestines. Without enough bile in the small intestines, it's more difficult for your child's body to absorb fat and harder for the body to get rid of certain toxins that need bile to help them exit the body.

Second, bile accumulation in the liver causes liver damage. As this condition progresses, inflammation and scarring damage the liver more and more, eventually resulting in cirrhosis. If bile can’t leave the liver, it accumulates. This can result in a buildup of toxins that causes even more liver damage.

Biliary Atresia |Symptoms & Causes

What are the symptoms of biliary atresia?

Infants with biliary atresia may be born with jaundice, but usually appear healthy at birth. Most often, you or your child's pediatrician will notice signs within the first few weeks of your child's life. These may include:

• jaundice
• dark urine
• light-colored stools

Signs that may appear later include:

• distended (swollen) abdomen
• weight loss

These are later signs, not usually in the first months of life.

What causes biliary atresia?

We know that biliary atresia is caused by inflammation and scarring of the bile ducts, but what causes the inflammation and scarring is still unknown.

There are three types of biliary atresia:

• biliary atresia without any other malformations: this is the most common type seen in about 85 percent of the babies
• biliary atresia with at least one major malformation but without laterality, about 5 percent
• biliary atresia with laterality defects is about 10 percent; this is what was previously called biliary atresia splenic malformation syndrome.

Biliary Atresia |How is biliary atresia diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. If your child has biliary atresia, it’s important that your child be diagnosed quickly, since surgery must be performed early in order to be effective.

Usually, the diagnostic process begins when you or your child’s pediatrician notices that your child appears jaundiced. The doctor will do one or more blood tests to determine whether it may be because of a liver problem.

These blood tests may include:

• bilirubin level test
• liver enzymes and bile duct enzymes tests
• coagulation tests
• viral tests — some viruses can cause liver damage
• tests for some metabolic diseases (such as enzyme deficiencies) that can cause early jaundice and liver test abnormalities
• tests for cystic fibrosis, such as a sweat test

The doctor may also want to check your child’s urine and stool.

He or she may also order diagnostic tests to view your child’s biliary system, including:

• abdominal ultrasound to see if there is a normal gallbladder or abnormalities that may explain the jaundice
• biliary excretion or “HIDA” scan — a special chemical is injected into your baby’s vein, then pictures are taken to see if it is excreted normally by the liver, just as bile would be
• liver biopsy — a tissue sample is taken from your child's liver and examined for abnormalities

We may also need to perform a diagnostic surgical procedure to directly see the biliary tree — this is considered the gold standard to rule out biliary atresia. This is called:

• intraoperative cholangiogram — a small incision is made in your child’s abdomen, and a contrast agent is injected into her bile duct. This agent can be seen on a special monitor, helping surgeons to see any blockages in the bile ducts.

After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we'll meet with you and your family to discuss the results and outline the best treatment options.

What are the treatment options for biliary atresia?

While there's no medical cure for biliary atresia, your child will typically undergo an operation to restore the drainage of bile and connect the liver back with your intestine; this is called Kasai portoenterostomy. If the biliary atresia is a diagnosed late a primary liver transplant might be necessary.

Kasai portoenterostomy

During a Kasai portoenterostomy, your child's surgeon removes the damaged bile ducts and connects the liver to the intestine. This allows bile to drain directly from the small bile ducts at the edge of your child's liver straight into her intestine. Following the surgery, your child may stay in the hospital between seven and 10 days. If done within the first two to three months of your child's life, a Kasai portoenterostomy can allow her to grow and remain in fairly good health for many years.

In about 40 to 50 percent of cases, even after a successful portoenterostomy, jaundice may not go away as the inflammatory process of biliary atresia involves the bile ducts in and outside the liver. Bile won't flow, and these children will need a liver transplant early on in life.

Along with the Kasai portoenterostomy, your child's doctors will do a liver biopsy if not already done to determine the extent of your child's liver damage. It may also help predict how her condition will progress in the coming months.

After surgery, your child will need ongoing medical care and monitoring, including tests. Your child's doctor can explain to you what the tests mean, and what the signs are that your baby's liver is doing well or getting worse. The doctor will also talk to you about the complications of biliary atresia that may require a liver transplant.

Liver transplant

A liver transplant is an operation in which the transplant surgeon removes your child's damaged liver and replaces it with a new liver.

The new liver can be the whole liver from a deceased donor, or a segment from a deceased donor or a living family member (if he or she is a suitable candidate). In the case of a living donor, the donor's liver will grow back to more or less its full size just a few weeks after the operation.

After surgery, the new liver begins functioning and the child's health often improves quickly, although transplants carry complications of their own.

Nutrition and biliary atresia

Children with biliary atresia who do not clear their jaundice have poor to no bile flow into their intestine. Since bile is important for absorption of fat this will result in poor special nutritional needs. Our registered pediatric dietitians can make recommendations including:

• feeding with special formulas that contain a kind of fat that's more easily absorbable
• adding specific vitamins to your child's diet, as directed by your child's physician
• adding MCT (medium-chain triglyceride) oil for extra calories to the diet. MCTs are absorbed as compared to LCT (long-chain triglycerides), which is the most common fat that we eat. MCT oil can be added to foods and liquids that your child eats.

If your baby can't take in the amount of calories needed to maintain a normal growth rate, we may recommend extra milk/formula feedings. These are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus, and into her stomach.

What is the long-term outlook for biliary atresia?

While there's no cure for the condition, there are ways for your child to have a longer and healthier life. The first step is surgery, called the Kasai portoenterostomy, in which the blocked bile duct is bypassed and your child's intestine is brought up/sewn up to the liver. Afterwards, special formulas and vitamin preparations, as well as other medications, can help your child grow and help to preserve her liver function.

Nonetheless, many children who have Kasai portoenterostomies early in life will need a liver transplant as the inflammatory process of biliary atresia involves the bile ducts in and outside the liver.

Current national statistics confirm that about 85 percent of children who have liver transplants survive the first year, and most children who survive the first year go on to live into adulthood.

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Prof.Dr.E.Mahir Gülcan 23:29  No comment

Pediatric liver diseases

What are some common liver disease symptoms?

When diagnosing liver disease, the healthcare provider looks at the patient's symptoms and conducts a physical exam. In addition, the healthcare provider may ask for a liver biopsy, liver function tests, an ultrasound, a computed tomography (CT) scan, and/or a magnetic resonance imaging (MRI) scan.

Some common liver disease symptoms include the following, each of which are described briefly below:

• Jaundice. A yellowing of the skin and eyes.

• Cholestasis

• Liver enlargement

• Portal hypertension

• Esophageal varices

• Ascites. A fluid buildup in the abdominal cavity.

• Hepatic encephalopathy

• Liver failure

• Abnormal bleeding

• Severe itching

• Fatigue

What is jaundice?

Jaundice is a yellow discoloration of the skin and whites of the eyes due to abnormally high levels of bilirubin (bile pigment) in the bloodstream. Urine is usually dark because of the bilirubin 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. Sometimes, jaundice is caused by the breakdown of a large number of red blood cells. This can happen in newborns. Jaundice is usually the first sign, and sometimes the only sign, of liver disease.

What is cholestasis?

Cholestasis means any condition in which bile flow is reduced or stopped. "Chole" refers to bile and "stasis" means "not moving." Bile flow may be blocked inside the liver, outside the liver, or in both places. Symptoms may include:

• Jaundice

• Dark urine

• Pale stool

• Easy bleeding

• Itching

• Ascites 

• Chills

• Pain from the biliary tract (the bile ducts and gallbladder) or pancreas

• Enlarged gallbladder

Some causes of cholestasis include the following:

• Hepatitis

• Alcoholic liver disease

• Primary biliary cirrhosis

• Drug effects

• Hormonal changes during pregnancy

• A stone in the bile duct

• Bile duct narrowing

• Bile duct cancer

• Pancreatic cancer

• Inflammation of the pancreas

What is liver enlargement?

Liver enlargement is usually an indicator of liver disease, although there are usually no symptoms associated with a slightly enlarged liver (hepatomegaly). Symptoms of a grossly enlarged liver include abdominal discomfort or "feeling full."

What is portal hypertension?

Portal hypertension is high blood pressure in the portal vein, which supplies the liver with blood from the intestine and spleen. Portal hypertension may be due to increased blood pressure in the portal blood vessels, or resistance to blood flow through the liver. Portal hypertension can lead to the growth of new blood vessels (called collaterals) that connect blood flow from the intestine to the general circulation, bypassing the liver. When this happens, substances that are normally removed by the liver pass into the general circulation. Symptoms of portal hypertension may include:

• Ascites

• Development of varicose veins. Varicose veins (esophageal varices) develop most commonly at the lower end of the esophagus and in the stomach lining, although they can develop anywhere from the mouth to the anus.

What are esophageal varices?

Esophageal varices are dilated blood vessels within the walls of the lower part of the esophagus that are prone to bleeding. They can appear in people with severe liver disease. A diseased liver can cause portal hypertension. This is high blood pressure in the portal vein. The portal vein supplies the liver with blood. Over time, this pressure causes blood vessels to grow, called collateral blood vessels. These vessels act as channels to divert the blood under high pressure. The extra pressure in these vessels causes them to dilate and become tortuous. These vessels can eventually reach the lower esophagus and stomach. They are prone to rupture. The rupture can lead to significant blood loss from vomiting or from lost blood passing through the gastrointestinal tract. Symptoms of esophageal varices may include:

• Painless vomiting of blood

• Black, tarry or bloody stools

• Decreased urine output

• Excessive thirst

• Light-headedness

• Paleness

• Anemia. A condition that indicates a low red blood cell count.

What is ascites?

Ascites is fluid buildup in the abdominal cavity caused by fluid leaks from the surface of the liver and intestine. Ascites due to liver disease usually accompanies other liver disease characteristics, such as portal hypertension. Symptoms of ascites may include a distended abdominal cavity. This causes discomfort and shortness of breath. Causes of ascites may include the following:

• Liver cirrhosis (especially cirrhosis caused by alcoholism)

• Alcoholic hepatitis

• Obstruction of the hepatic vein

Ascites can also be caused by nonliver disorders.

What is hepatic encephalopathy?

Hepatic encephalopathy is the deterioration of brain function and damage to the nervous system. This is because of toxic substances building up in the blood, which are normally removed by the liver. Liver encephalopathy is also called portal-systemic encephalopathy, hepatic encephalopathy, or hepatic coma. Symptoms may include:

• Impaired consciousness

• Changes in logical thinking, personality, and behavior

• Mood changes

• Impaired judgment

• Drowsiness

• Confusion

• Sluggish speech and movement

• Disorientation

• Loss of consciousness

• Coma

• Seizures (rare)

• Muscle stiffness or tremors

• Uncontrollable movement

What is liver failure?

Liver failure is severe deterioration of liver function. Liver failure happens when a large portion of the liver is damaged due to any type of liver disorder. Symptoms may include:

• Jaundice

• Tendency to bruise or bleed easily

• Ascites

• Impaired brain function

• General failing health

• Fatigue

• Weakness

• Nausea

• Loss of appetite

• Diarrhea

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Prof.Dr.E.Mahir Gülcan 20:46  No comment

Liver enzyme elevation

One of the important functions of the liver is to manufacture enzymes, which are proteins that help the body to carry out certain chemical reactions at a faster rate. Usually these enzymes reside in the liver but are also found in small amounts in the bloodstream. When the liver gets damaged, its ability to hold these liver enzymes reduces.

The hepatic function panel evaluates:

• Alanine aminotransferase (ALT). This enzyme, found in the liver, plays a role in metabolism, the process that converts food into energy. If the liver is injured, ALT is released into the bloodstream. Its levels are especially high with acute hepatitis.
• Alkaline phosphatase (ALP). This enzyme is found in the liver, bones, intestines, kidneys, and other organs. Kids and teens normally have higher levels of ALP than adults, even when they're healthy, due to bone growth. But ALP levels can also increase when kids have viral infections, liver diseases, or blocked bile ducts.
• Aspartate aminotransferase (AST). This enzyme, which plays a role in processing proteins, is found in the liver, heart, muscles, and kidneys. When the liver is injured or inflamed, levels of AST in the blood usually rise.
• Total bilirubin and direct bilirubin. Bilirubin is a byproduct of the normal breakdown of red blood cells. It usually passes through the liver and is excreted from the body. But if that doesn't happen due to a liver disease, bilirubin levels in the blood can rise and the skin can take on the yellow discoloration known as jaundice. Tests for bilirubin may be total (measuring the level of all of the bilirubin in the blood) or direct (measuring only bilirubin that has been processed by the liver and attached to other chemicals).
• Albumin and total protein. Protein is needed to build and maintain muscles, bones, blood, and organ tissue. Sometimes when there's a problem with the liver, it can't make proteins as well, so protein levels decrease. Liver function tests measure albumin specifically (the major blood protein produced by the liver), as well as the total amount of all proteins in the blood.

Following are the causes that can damage the liver, eventually causing abnormally high levels of liver enzymes to flow into the bloodstream:

Liver Infections
The hepatitis virus is the one that attacks the liver and causes abnormal buildup of enzymes in the blood. Having unhygienic food or water is the primary reason behind catching this viral infection.

Liver Diseases
Liver disorder and enlarged liver in children is yet another factor that causes liver enzymes to accumulate in the blood. Children suffering from diseases such as jaundice, liver cancer, or liver cirrhosis have been diagnosed with this condition.

Unhealthy Diet
An increasing number of children worldwide have developed a liking for fast foods like hamburgers, pizzas, and French fries. All these food items are loaded in unhealthy fats and so too much consumption cannot be tolerated by the liver. The liver has to work harder to promote digestion of these fatty foods. This workload can damage the liver, thus forcing it to expel enzymes in abnormally high amounts into the bloodstream.

Pain Relievers
Although painkillers help to alleviate the discomfort, they can become problematic if they are given in higher doses to children. Studies have proved that children taking high strength tablets of acetaminophen or ibuprofen show elevated liver enzymes.

Vitamins
An unhealthy diet can lead to vitamin deficiency in children. In such cases, to overcome this problem, doctor often prescribe multivitamin supplements for children. However, these supplements have to be given in the right dosage as instructed by the doctor. If this is not followed, it can affect the liver and eventually cause too much of enzymes to circulate in the blood.

Household Chemicals
Many of the household chemicals and products that we use to clean our home contain harmful ingredients. So, if children accidentally inhale or consume these ingredients, the liver may get damaged. In such cases, the damaged liver is likely to send more enzymes into the bloodstream.

If the enzymes are slightly higher than normal, symptoms are usually not observed in children. The liver function test that measures the amount of liver enzymes present in blood, is used to diagnose this condition. As per the test results and the underlying cause, the doctor will suggest a treatment approach to resolve the issue.

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Prof.Dr.E.Mahir Gülcan 22:16  1 comment

What is liver biopsy in children? How is liver biopsy performed in children? ​

Fast Facts About Liver Biopsy

• A liver biopsy is done when a patient has an abnormal liver test to help the doctor figure out the cause of liver problems and the extent of the liver injury.
• A liver biopsy uses a needle that is passed quickly through the skin and into the liver to take out a very small sample of liver tissue so that it can be looked at under a microscope.
• Your child will be asleep for the test.
• There are special rules for eating and drinking in the hours before the test.
• The test itself takes only 20 to 45 minutes, but the entire appointment will take several hours. 
• If your child is young, he or she may need to stay in the hospital overnight after the test.

What Is a Liver Biopsy?

A liver biopsy allows the doctor to look at the liver tissue under the microscope. Not every child with an abnormal liver test will need a liver biopsy, but it is very useful when there are questions about the diagnosis or if the doctor needs to check for cirrhosis (seer-OH-siss) or scarring. The liver biopsy also allows the doctor to see the degree of inflammation (in-flah-MAY-shun) or swelling in the liver and which liver cells are most affected. The most common reasons for a liver biopsy are to look for causes of jaundice (JON-duss), which makes the skin and eyes look yellow; hepatitis (hep-uh-TITE-us) or liver cell inflammation; an enlarged liver; or abnormal liver function tests.

• Before the liver biopsy can be done, your child will have an ultrasound of the upper right side of his or her belly where the liver is located to determine the best place to do the liver biopsy. A soft-tipped marker will be used to place an "X" on your child’s skin at the spot right over the liver where the liver biopsy will be done.
• The pediatric GI doctor doing the biopsy will also look at and examine your child’s belly to confirm that the mark is in the right spot over the liver.
• The doctor will speak to you before the biopsy is performed to answer any questions and your child will then be taken to the room where the biopsy will be performed.
• After your child is asleep, the doctor will give an injection (shot) of pain-killing medication called an anesthetic (ann-ess-THET-ick) into the skin to numb the area.
• When the anesthetic has taken effect, a needle will be inserted quickly through the skin and into the liver.
• The doctor will draw out a small piece of your child’s liver tissue through the needle.
• This procedure may be repeated once or twice in order to get enough liver tissue for testing and examination.
• The liver tissue will be placed in a special preservative and taken to the pathologist (a specialist in the study of tissues). 
• The final result of the liver biopsy will not be available for several days, depending upon the number and types of tests that need to be performed on the liver tissue.

Fast Facts About General Anesthesia

To keep your child comfortable, the liver biopsy will be done while your child is under general anesthesia (an-ess-THEEZ-ya), meaning that he or she will be sound asleep during the test.

• A pediatric anesthesiologist — a doctor who specializes in anesthesia for children — will give the medications that will make your child sleep during the test. 
• Under general anesthesia, your child will not feel any pain or have any memory of the test.

Home Preparation

When general anesthesia is needed, there are important rules for eating and drinking that must be followed in the hours before the test. One business day before your child’s test, you will receive a phone call from a nurse between the hours of 1 and 9 p.m. (Nurses do not make these calls on weekends or holidays.) Please have paper and a pen ready to write down these important instructions.

• The nurse will give you specific eating and drinking instructions for your child based on your child’s age. Following are the usual instructions given for eating and drinking. No matter what age your child is, you should follow the specific instructions given to you on the phone by the nurse.

For children older than 12 months:

• After midnight the night before the test, do not give any solid food or non-clear liquids. That includes milk, formula, juices with pulp, chewing gum or candy.

For infants under 12 months:

• Up to 6 hours before the scheduled arrival time, formula-fed babies may be given formula. 
• Up to 4 hours before the scheduled arrival time, breastfed babies may nurse.

For all children:

• Up to 2 hours before the scheduled arrival time, give only clear liquids. Clear liquids include water, PedialiteTM, Kool-Aid® and juices you can see through, such as apple or white grape juice.
• In the 2 hours before scheduled arrival time, give nothing to eat or drink.

Your child’s procedure will be cancelled if these instructions are not followed.

If Your Child Is Having General Anesthesia

Before your child’s liver biopsy, a member of the anesthesia staff will meet with you to take your child’s vital signs, weight and medical history. As the parent or legal guardian, you will be asked to sign a consent form before the anesthesia is given.

• The anesthesiologist will meet with you and your child to review your child’s medical information and decide which kind of sleep medication your child should get. 
• If your child is very scared or upset, the doctor may give a special medication by mouth to help him or her relax. This medication is flavored and takes effect in 10 to 15 minutes. 
• Your child will be moved to the operating room to receive the sleep medication and you will be taken to the family waiting room.
• Younger children will get their sleep medication through a mask that will carry air mixed with medication. Your child may choose a favorite scent to flavor the air flowing through the mask. There are no shots or needles used while your child is still awake. 
• Older children may choose between getting their medication through the mask or directly into a vein through an intravenous (IV) line. 
• If it has not already been done, an IV will be started so that medication can be given to keep your child sleeping throughout the test. While your child is asleep, his or her heart rate, blood pressure, temperature and blood oxygen level will be checked continuously.
• Your child may have a breathing tube placed while he or she is asleep. If a breathing tube is used, your child may have a sore throat after the test.
• To keep your child asleep during the test, he or she may be given anesthetic medication by mask, through the IV tube or both. When the test is over, the medications will be stopped and your child will begin to wake up.

A Parent’s/Guardian’s Role During the Test

We welcome your help and support during this test. One of the most important roles of a parent or guardian is to help your child stay calm and relaxed before and after the test. The easiest way to help your child stay calm is for you to stay calm.

The Test

• Your child’s liver biopsy will be scheduled through the Same Day Surgery Center at Children’s Hospital. The Same Day Surgery Center will call you the day before your child’s liver biopsy to give you your arrival time and go over the eating and drinking instructions. 
• You and your child should come to the Same-Day Surgery Center to check in at your scheduled time. After checking in, your child will be sent to the Department of Pediatric Radiology at Children’s Hospital to have an ultrasound. 
• Your child will be awake for the ultrasound. An ultrasound is a safe test done on the outside of the body and does not hurt. 
• The radiologist will use a gel and a wand on your child’s belly to locate the position of your child’s liver. Using the pictures from the ultrasound machine, the radiologist will make a mark on your child’s skin to let the G.I. doctor know where the liver is located under the skin.
• After the ultrasound, your child will be taken to an exam room, where you will meet the anesthesiologist to discuss the sleep medication your child will receive. 
• When it is time for the test to begin, your child will be moved to the operating room. 
• In the operating room, the anesthesiologist will give your child the anesthesia medicine to make him or her sleep throughout the entire liver biopsy.
• Once your child is asleep, the doctor will find the mark made by the radiologist, and also will examine your child’s belly to be sure the liver is directly under the mark.
• The doctor will clean the skin on the belly and inject a "local anesthetic," which is a type of medication that numbs the area right around the injection site.
• When the local anesthetic has taken effect, the doctor will use a needle to take out a small piece of your child’s liver tissue. This part of the test is over quickly.
• After the tissue sample has been taken, the anesthesia ­medication will be stopped and your child will be taken to the recovery room. 
• A bandage will be placed over the site of the liver biopsy. When the bandage is removed the following day, you will see only a small "dot" where the needle entered the skin. 
• The doctor will come to the surgical family waiting room to speak with you as soon as your child is moved to the recovery room.

Waking Up/ Going Home

• When the biopsy is done, you will be called to the room to be with your child as the sleep medications wear off. The length of time it will take the medications to wear off will vary, as some children take longer than others to become alert.
• Your child will have no memory of the biopsy. 
• Your child will be resting on the side where the liver biopsy was performed. This position allows for a little extra pressure to be placed on the biopsy site. 
• Children coming out of anesthesia may react in different ways. Your child may cry, be fussy or confused, feel sick to his or her stomach, or vomit. These reactions are normal and will go away as the anesthesia wears off. 
• It is more important to help your child relax and feel calm than to worry about him or her lying on the side where the liver biopsy was done.
• Every child who has a liver biopsy will have a blood count done about 4 hours after the liver biopsy. When the results of the blood count come back, we will determine whether your child can be discharged from the hospital.
• When your child is discharged, he or she may still be groggy and should take it easy for the day. 
• Young children are often scheduled to remain in the hospital overnight for more extended monitoring after a liver biopsy. The decision for an overnight stay is made prior to the liver biopsy on a case-by-case basis.

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Prof.Dr.E.Mahir Gülcan 14:06  No comment