Breakthrough in understanding life-threatening childhood liver disease

Public release date: 26-Oct-2010

Contact: Dan Meyers
dan.meyers@ucdenver.edu
303-724-5377
University of Colorado Denver

CU School of Medicine researchers find link to infection

Researchers at the University of Colorado School of Medicine and The Children's Hospital have taken a big step toward understanding what causes one of the most serious liver diseases in infants.

The disease is called biliary atresia, It blocks the bile ducts in young infants, through which bile, crucial for digestion, flows to the small intestine. The disease is rare – it strikes in about one in 10,000 births. But it's life-threatening.

"It is fatal if not treated quickly," says Cara Mack, MD, who led the CU research.

Surgical removal of the blocked main bile duct can buy time but ultimately the treatment in the majority of cases is a liver transplant during infancy or childhood, a procedure that is both complicated and expensive.

Until now, doctors weren't sure what caused biliary atresia, which is important to know in order to develop better treatments. The CU researchers propose that an infection late in the third trimester of pregnancy or soon after birth initiates the bile duct injury.

The body fights off the infection and infants initially show no signs of a problem. But then, Mack says, the body continues to battle as if the infection still was active. The body, however, is attacking itself -- the bile ducts specifically -- not the infection. This is called an autoimmune process.

Why? Mack's research in a mouse model of the disease suggests that it may be that the bile ducts have been changed and the body's protective system senses that. Or it may be that the bile ducts give off a protein that is similar to proteins produced by the infection, launching the body's defenses into action. In these investigations, Mack and colleagues identified an immune system compound, anti-enolase antibody, that reacts to both virus and bile duct proteins. This antibody may contribute to the bile duct injury in biliary atresia.

"After a viral infection has resolved," Mack says, "the body's immune defenses turn on the bile ducts and cause continued damage."

That leads to scarring of the bile ducts, eventually blocking them so that "bile is not able to flow from the bile ducts into the intestines," says Mack, an associate professor in pediatrics with CU, who practices at The Children's Hospital in Aurora.

The discovery, published recently in the journal Gastroenterology, isn't a cure. But it is a big step, "pointing the way to new diagnostic tests and, eventually, to improved treatment options for this devastating disease," Mack says.

What is Kasai Procedure??Very interesting Video...

My Baby was diagnosed with Billiary Atresia and had Kasai procedure done at Hospital Kuala Lumpur On 18th February 2010 when she's only 8 weeks old .Feel free to see how its done...
http://www.youtube.com/watch?v=4D39nphvRYs

What is Billiary Atresia??

Biliary atresia is a rare condition in newborn infants in which the common bile duct between the liver and the small intestine is blocked or absent. If unrecognized, the condition leads to liver failure -- but not kernicterus, as the liver is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood-brain barrier. The cause of the condition is unknown. The only effective treatments are certain surgeries such as the kasai procedure, or liver transplantation.

If wanna see how Kasai is perfomed feel free to go to http://www.youtube.com/watch?v=4D39nphvRYs

Science Project-Preparation of Crystals

PROJECT TITLE:

Preparation of Crystals

Aim:

To prepare small and large crystals of different salts by cooling hot saturated solutions of the salts.

Materials Required:

1. Copper sulphate 2. Potash alum 3. Epsom salt (magnesium sulphate) 4. Sodium sulphate 5. Beakers 6. Test tubes 7. Watch glasses 8. Hand lens 9. Bunsen burner

Procedure:

Prepare a saturated solution of each specimen of salt in four different beakers (250 ml.) such that solid particles of the salt settle at the bottom. Now heat the mixture to dissolve the solid particles, and obtain a hot saturated solution. Pour a portion of the hot solution from each beaker into separate test tubes, another portion into separate watch glasses, and leave the rest in the beaker. Cool the solutions in the test tubes rapidly under tap water, but leave the rest on the table to cool down slowly.

Observation:

Observe the particles obtained from each of A, B and C with a hand lens. You will find that crystals are deposited in all four cases; while those that separate from the test tubes are tiny crystals, those from the beakers and the watch glasses are larger crystals. When observed through the hand lens, each crystal is found to have a definite geometrical shape, plane surfaces and a shiny appearance. Crystals of common salt are cubes; alum forms eight-sided or octahedral crystals, Epsom salt crystals are prismatic and blue copper sulphate crystals triclinic.

Inference:

Crystals can be obtained by cooling a hot saturated solution of the substance. Rapid cooling, e.g. under the tap, produces small crystals, while slow cooling produces larger crystals.

Science Project-Boiling Point of water

PROJECT TITLE:

Boiling Point of Water

Aim:

To find the boiling point of water and to study the effect on it of adding sugar.

Materials Required:

1. Round-bottomed flask 2. Thermometer 3. Sugar 4. Bunsen burner

Procedure:

Take about 50cc. of water in the round-bottomed flask and add a few fragments of broken porcelain. Heat the flask and note the temperature when boiling begins. Take readings every thirty seconds for five minutes, i.e. ten readings in all, and take the average as the boiling point. Now add about 15 gm. of sugar to the water and repeat the experiment, taking the average often readings as before as the boiling point.

Observation:

The solution boils at a higher temperature than water. You can repeat the experiment with several substances.

Inference:

The addition of a solute to water raises the boiling point of the water. By changing the solvent, the same conclusion may be extended to all solvents as well. It may be useful to mention that the addition of a solute to a solvent also has an effect upon the freezing point of the solvent, it lowers the freezing point. For instance, water freezes at 0?C, but if sugar is dissolved in it, it will freeze at a lower temperature. Thus in "freezing mixtures" salts are generally added to ice to give a temperature below the freezing point of water.