Featured Research

from universities, journals, and other organizations

Potential Therapy Reported For Children, Adults With End-Stage Liver Disease

Date:
May 16, 2002
Source:
Johns Hopkins Medical Institutions
Summary:
A Johns Hopkins Children's Center scientist reports success in animal studies in preventing a cascade of brain pathology that appears to both cause and signal the final and fatal stages of acute and chronic liver disease in children and adults. The findings ("Hyperammonemic Encepalopathy") appear in the May issue of Medicine.

A Johns Hopkins Children's Center scientist reports success in animal studies in preventing a cascade of brain pathology that appears to both cause and signal the final and fatal stages of acute and chronic liver disease in children and adults. The findings ("Hyperammonemic Encepalopathy") appear in the May issue of Medicine.

In the review article, pediatric biochemical geneticist Saul Brusilow, M.D., writes that the chemical methionine sulfoximine (MSO) appears to prevent the brain swelling (encephalopathy) in patients with acute liver failure. It is the swelling, he believes, that causes pressure on the patient's brain, which leads to coma and ultimately death.

MSO is known to prevent the formation of glutamine, a by-product of high ammonia levels in the blood (hyperammonemia). Patients with advanced liver disease develop hyperammonemia as the failing liver can no longer process ammonia, a natural by-product in humans. "Interrupt glutamine formation and the swelling that follows and we might be able to prevent brain damage and death," says Brusilow.

Experimental evidence suggests that glutamine forms in one type of brain cell, the astrocyte. As glutamine accumulates, water also accumulates, causing the astrocyte to swell. It is this swelling, Brusilow theorizes, that puts pressure on the brain in acute liver failure, and interrupts the normal functions of the astrocyte; patients may then lapse into unconsciousness and death. Patients with chronic liver failure demonstrate the consequences of astrocyte swelling by exhibiting behavioral changes rather than brain swelling.

In their animal studies, Brusilow, emeritus professor of Pediatrics at Johns Hopkins, and his colleagues Richard Traystman, Ph.D., and Raymond Koehler, Ph.D., both in the Department of Anesthesiology and Critical Care Medicine, pretreated rats with MSO, then induced hyperammonemia. Compared to rats not pretreated, the test animals showed no swelling in either their astrocytes or brains, and none showed signs of brain damage.

It's well known that patients with end-stage liver disease are hyperammonemic and have swollen astrocytes and increased levels of glutamine, Brusilow notes. He plans to begin clinical trials of MSO in such patients within a year.

Brusilow is widely regarded for his research on and the treatment of patients with a rare biochemical defect known as urea cycle disorder, which has some of the earmarks of the ammonia toxicity and brain pathology that characterize end-stage acute and chronic liver disease. In 1992, he developed the first and only successful treatment for urea cycle disorder, which uses the drug Buphenyl to prevent the buildup of excess ammonia, and thus prevents glutamine formation in the brain.

The study was funded by the National Institute of Child Health and the National Institute of Neurologic Diseases.


Story Source:

The above story is based on materials provided by Johns Hopkins Medical Institutions. Note: Materials may be edited for content and length.


Cite This Page:

Johns Hopkins Medical Institutions. "Potential Therapy Reported For Children, Adults With End-Stage Liver Disease." ScienceDaily. ScienceDaily, 16 May 2002. <www.sciencedaily.com/releases/2002/05/020514072126.htm>.
Johns Hopkins Medical Institutions. (2002, May 16). Potential Therapy Reported For Children, Adults With End-Stage Liver Disease. ScienceDaily. Retrieved September 30, 2014 from www.sciencedaily.com/releases/2002/05/020514072126.htm
Johns Hopkins Medical Institutions. "Potential Therapy Reported For Children, Adults With End-Stage Liver Disease." ScienceDaily. www.sciencedaily.com/releases/2002/05/020514072126.htm (accessed September 30, 2014).

Share This



More Health & Medicine News

Tuesday, September 30, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

How 'Yes Means Yes' Defines Sexual Assault

How 'Yes Means Yes' Defines Sexual Assault

Newsy (Sep. 29, 2014) Aimed at reducing sexual assaults on college campuses, California has adopted a new law changing the standard of consent for sexual activity. Video provided by Newsy
Powered by NewsLook.com
Scientists May Have Found An Early Sign Of Pancreatic Cancer

Scientists May Have Found An Early Sign Of Pancreatic Cancer

Newsy (Sep. 29, 2014) Researchers looked at 1,500 blood samples and determined people who developed pancreatic cancer had more branched chain amino acids. Video provided by Newsy
Powered by NewsLook.com
Colo. Doctors See Cluster of Enterovirus Cases

Colo. Doctors See Cluster of Enterovirus Cases

AP (Sep. 29, 2014) Doctors at the Children's Hospital of Colorado say they have treated over 4,000 children with serious respiratory illnesses since August. Nine of the patients have shown distinct neurological symptoms, including limb weakness. (Sept. 29) Video provided by AP
Powered by NewsLook.com
Dr.'s Unsure of Cause of Fast-Spreading Virus

Dr.'s Unsure of Cause of Fast-Spreading Virus

AP (Sep. 29, 2014) Doctors at the Children's Hospital of Colorado say they have treated over 4,000 children with serious respiratory illnesses since August. Nine of the patients have shown distinct neurological symptoms, including limb weakness. (Sept. 29) Video provided by AP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins