What is BILIARY ATRESIA - Causes & Symptoms, Diagnosis and its Treatment

Hello Steemians, for now not a drawing, nor a contest, even a tutorial or photography entry I will share. I want to understand and open every reader's mind about the disease BILIARY ATRESIA. To know the causes, symptoms and it's proper treatment for this dangerous conditions.

It is extremely difficult for a parent to see your child suffered from a disease. Especially if the victims are newborn babies. Parents will make every effort to secure or save their child and extend their life.

This article will also explain the importance of our Liver. You'll know how the liver works and its functions throughout our body. Additional facts and information about the liver.

Article Summary as follows:

1. WHO are the majority victims of biliary atresia?
2. A brief explanation and definition of BILIARY ATRESIA
3. Types of BILIARY ATRESIA
4. Symptoms and Causes of Biliary Atresia disease
5. Complications of BILIARY ATRESIA and Liver Failure
6. Diagnosis and Treatment (Kasai Procedure & Liver Transplant)
7. Bonus: Facts and all about the Liver

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~ WHO are the majority victims of Biliary Atresia?

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Babies

Biliary Atresia is a rare disorder who about one of 15,000 to 20,000 infants do not have complete bile ducts. This disorder seems to affect a majority of girls more than boys. In this case, in the same family, only one child will be positive or in a pair of twin one child only will be affected.

Experts say that babies with biliary atresia do not have the chance to release the liquid bile and it is in the liver alone. Since the liquid is inside only it destroys the liver cells causing cirrhosis. The Liquid will damage the whole liver and will result in having scars.

Frequently affected by this disorder are more Asian and African- American than Caucasian race.

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~WHAT IS BILIARY ATRESIA ?~

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Biliary Atresia

BILIARY ATRESIA is one of the liver diseases that lead to transplantation. It is a gastrointestinal disorder in which the biliary system is absent or clogged. Biliary system is consists of tiny particles or tubular structures that connect to the intestine from the liver and drains the bile.

Bile is the nasty greenish-brown liquid which helps for digestive process and excreted by the liver cells and preserved in the gallbladder. This discharged liquid is composed of cholesterol, bile salts, and other waste or bad compounds.

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~ 2 TYPES OF BILIARY ATRESIA ~

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Based on the research, doctors have identified two different types of Biliary Atresia as follows:

1. Biliary Atresia without birth defects

A common type of Biliary Atresia which infants have no major birth defects. They called it Prenatal or Isolated Biliary Atresia. According to a North American study found that 84 percent of infants with Biliary Artesia have this cases.

2. Biliary Artesia with birth defects

Doctors called this condition Fetal or Embryonic Biliary Artesia for having major defects including problems with heart, spleen or intestines. North American study found that 16 percent of an infant have this Biliary Atresia with major defects.

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~SYMPTOMS AND CAUSES ~

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Baby Kendra

~ SYMPTOMS~

Frequently, the first sign of Biliary Atresia is having a yellowing of skin and white part of the eyes which called jaundice. Jaundice is a situation where excessive bilirubin in your blood that results in becoming yellow of skins color and white part of the eyes. Many possible types of disorder jaundice can be and not only biliary atresia disease.

Often babies develop jaundice in the first two weeks after birth. In this case, Biliary atresia is very difficult to identify with newborn babies. And since jaundice is a sign not only for Biliary Atresia disorder.

Infants having Biliary Atresia may also have pale yellow, grey or white excrement. Stool's color is changing because of the bilirubin is not reaching the intestine and passing out of the body in the stool.
For the diagnose of this disease, you can check the diagnose section of this article.

~ CAUSES ~

According to the experts/doctors, there's no yet explanation on how completely Biliary Atresia occurred. Some of the statements of doctors, Biliary Atresia happens when during pregnancy where their bile ducts are underdeveloped properly. In other interpretation, children having these diseases are there are a damage bile ducts because of a viral infection acquired after birth and their immune systems can't contend with this infections.

This disease is not an inherited means it does not transferable from parent to child.

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~ COMPLICATIONS and Liver Failure~

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In every disease there's a complication, Biliary Atresia complication includes failure to thrive and malnutrition, cirrhosis and other liver failures.

Without any treatment infants with Biliary Atresia would develop cirrhosis within 6 months and liver failure within 1 year. By age of 2 years, the untreated infant having this disease need a transplant to survive.

Cirrhosis is a condition which the liver breaks down and will result in malfunction. In these cases, where healthy liver tissues are replaced to scar, where partially obstructs the blood flow through the liver.

This is the sad part when the cirrhosis gets worse, the liver begins to malfunction.

Another complication will arise from cirrhosis which is Portal Hypertension. Portal hypertension is high blood pressure in the portal vien, a blood vessel that carries blood from the intestines to the liver. This complication may lead to produced fluid in the abdomen which is Ascites.

Ascites is a liquid an extremely dangerous liquid produced by liver failure. As you can see in the photo there's an abnormal enlargement of the abdomen, increasing its weight, briefness of breath, and experiencing some abdominal discomforts.

Lastly, you will be noticed the infant for being malnutrition and failure to thrive. These are another two complications. Infants having Biliary Atresia are inefficient to get proper vitamins, mineral, and nutrients needed to stay them healthy. In this case, they will be faced a problem with growth such as failure to thrive.

~ Liver Failure ~

When all the healthy cells removed and replaced with scars, the liver cannot perform properly to its functions and stop from working turns to a Liver failure. Since all the liver tissues are replaced with damage cells liver no longer to work as regenerating gland. Infants, Children or any victim of this liver failure needs a liver transplant to survive.

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~DIAGNOSIS AND TREATMENT ~

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~ DIAGNOSIS ~

To examine this disease, doctors will require your infant's family and medical history, consummate a physical test and will provide other series examinations to get the exact condition of the patient. Biliary Atresia and other diseases, experts recommended several tests for the infants who still have jaundice 3 weeks after birth. After all the test and confirmed the real status where the infant is likely to have Biliary Atresia, next step is surgery to confirm the diagnosis.

Before the physical examinations, doctors will ask first regarding the family and medical history. They will demand also for the jaundice and the stools changing colors informations.

In the Physical test, they will examine again the signs of jaundice, to test and discover some birth defects other than biliary atresia. Doctors will check the abdomen, check the liver or spleen. And they will check the stool color and urine.

After all the Physical Test and gathering information, doctors will recommend to having a Blood test, Ultrasound, Hepatobiliary scan and Liver Biopsy.

~ TREATMENT ~

Treatment for Biliary Atresia

Biliary Atresia has two kinds of Treatment which are Kasai Procedure and Liver Transplant.

KASAI TREATMENT

KASAI Procedure is usually the first treatment for biliary atresia. This procedure does not cure the biliary atresia disease but it helps gradually liver damage, interrupt or prevent the complications once the surgery is successful before the liver transplant.

This is the surgery where doctors removed the damage or clogged outside bile ducts and connect the small intestine directly to the liver. By this surgery, it will help the bile to flow from the liver to intestine, this will result in no longer jaundice and reduce the developing complications of advancing liver diseases.

After the surgery doctors need to prescribe antibiotics to prevent the so-called cholangitis. Cholangitis is a complication of the Kasai procedure after the surgery and cause of bacteria. If cholangitis happens, after the surgery doctors treat it with antibiotics, usually intravenous (IV) antibiotics.

If the procedure is not successful, the flow of the bile will remain clogged. An unsuccessful operation, infants will develop some complication of Biliary Atresia and needed a liver transplant by the age of 2. Somehow successful Kasai procedure, infants still will develop slowly complications of biliary atresia, over years or decades, and eventually necessary to have a LIVER TRANSPLANT.

LIVER TRANSPLANT

After a successful or unsuccessful procedure of Kasai, Liver Transplant still needed, especially Biliary Atresia leads to serious complications.

Both the donor and the recipient should be healthy before the operation. Doctors will gather some test for the donor. These tests are to determine if the donor's organ (liver) is compatible and healthy for the operations. Donors should eat and make a healthy lifestyle, stop smoking, stop drinking alcoholic drinks and need an exercise.

REFERENCE 1
REFERENCE 2

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~ALL ABOUT LIVER ~

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THE LIVER

The liver is classed as part of the digestive systems. This is the largest organ and gland in the human body where carries out over 500 essential task. The main function of the liver is detoxification, protein synthesis and production of chemicals that helps for digestion of food.

Liver is the only visceral organ can regenerate. Evolution has ensured that it can grow rapidly as long as it is kept healthy and fine. It can regenerate at least 25 percent of the tissue remains.

Major Function of the Liver

Production of Bile

Bile liquid helps the small intestine break down and absorb fats, cholesterol, and some vitamins. Only a liver can produce a bile liquid which the only one used for digestion.

Fascinating & Metabolizing Bilirubin

When haemoglobin breakdown bilirubin will be formed. The iron released from haemoglobin will be stored in the liver or bone marrow after this new generation of blood cells will make.

Liver uses for carrying blood clots, filters the blood and other uses.

Liver is the factory of vitamin K that gets from bile. Bile liquid is imperative for vitamin K absorption. If the liver does not produce /make adequate bile, clotting factors cannot be produced.

Liver filters and removes compounds consists of hormones, as such as estrogen and aldosterone and other compounds outside the body including alcohol and other drugs.

Albumin also produced in the liver. This is the most common protein in the blood serum. It helps to transport the fatty acid and steroid hormones to maintain the normal and right pressure and restrain the leaking or breakage of the blood vessels.

Angiotensinogen is hormones formed in the blood pressure shrinking the blood vessel when alerted by a production of an enzyme called renin in the kidney and liver help to develop these hormones.

Metabolization of fat, Carbohydrates, and proteins

By the use of bile, fats are easier to digest. Ou body needs to maintain normal glucose level. Liver helps to store and broke down the carbohydrates into glucose and syphoned into the bloodstream to have balance and normal glucose level. From glucose, it will be stored as glycogen and will be released whenever a quick burst of energy is needed.

LIVER as Vitamins and Minerals storage.

Liver is like a warehouse. Where all the vitamins and minerals are stored. Several years worth of these vitamins it will be like a backup.

Liver as Immunological Function

Mononuclear Phagocytic System (MPS) is part of the immune system that consists of phagocytic cells. Phagocytic cells are cells destroying any kind of disease-causing agents that might enter the liver through the gut.

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Thanks for the time !!!

This article is an explanation and widens the knowledge of every reader regarding the Biliary Atresia diseases which majority of the victims are infants and family who don't have enough fund for the liver transplant. On my future post, I will discuss and share the story of a baby girl Kendra who caught my attention and give me the strength to have this research and article.

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