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Urea-cycle disorder treatment reviewed
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BALTIMORE (Map, News) - Nitrogen is a big part of the air we breathe, but too much can kill.
Researchers at Johns Hopkins University reviewed 25 years of studies on using sodium phenylacetate and sodium benzoate to help eliminate excess nitrogen, which can generate ammonia in the blood of children with urea-cycle disorders. This causes brain damage, retardation, coma and even death.
“In all my years I never came across another disease where patients come in near-comatose and you stick a needle in them and lo and behold, they wake up — just like that. It was just astonishing,” said Dr. Saul Brusilow, professor emeritus of pediatrics at Hopkins and the first to use the treatment in 1980.
The body produces sodium phenylacetate and sodium benzoate in small amounts to help regulate nitrogen, but in some cases can’t produce enough, according to Brusilow’s report in this week’s New England Journal of Medicine. Most patients hospitalized for ammonia poisoning are children.
“Sodium phenylacetate and sodium benzoate already know how to eliminate nitrogen in urine, so having more in the body carries more nitrogen out and reduces the toxic effects of excess nitrogen accumulation,” Dr. Ada Hamosh, clinical director of the McKusick-Nathans Institute of Genetic Medicine, said in a statement.
Nitrogen is generated when the body breaks down proteins into amino acids, Hamosh said. It binds to these sodium compounds to form urea, which is a component of urine.
Despite the immediate clinical success of the treatment, the U.S. Food and Drug Administration finally approved the drug combination in 2005.
Hamosh estimated one in 40,000 children is born with a urea-cycle disorder.
“We’re teaching all medical students at Hopkins to consider hyperammonemia and immediately do blood tests when they see a combative, lethargic or comatose newborn or child,” she said. “The longer the hyperammonemia lasts, the higher the risk for brain damage.”
Treatment consists of high-doses of intravenous sodium phenylacetate and sodium benzoate for two hours followed by maintenance infusions until blood ammonia levels are normal, according to the article. Overall, 84 percent of patients survived, and 96 percent survived individual episodes of severe ammonia poisoning.
For older children and adults, a protein-restricted diet and maintenance medications usually control the disorder, Hamosh said.
khille@baltimoreexaminer.com
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2:20 PM MST on Sun., Feb. 10, 2008 re: "Inmate gets drunk on hand sanitizer"
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6:48 PM MST on Wed., Aug. 8, 2007
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8:24 AM MST on Sun., Jun. 10, 2007
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Examiner Reader said:
Your alcohol facts are not quite straight. You mentioned Avant Hand Sanitizer- it has denatured alcohol. The denaturing process adds a bitter agent- it make sit taste horrible- definitely not a vodka type drink. That is why alcohol is denatured- to avoid abuse like this. It will likely make you vomit.
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Examiner Reader said:
Former Minneapolis Fire Chief: A First Responder in I-35W Bridge Collapse; Coordinates Helping Hand Contribution of Soapopular Hand Sanitizers For EMS Workers For Immediate Release Minneapolis, MN, Aug 8, 2007-- Former Minneapolis Fire Department Chief Bonnie Bleskachek, an embattled hero to many in the Minneapolis community, hasn't allowed recent personal controversy to stand in the way of helping Minnesota citizens in times of crisis. Since the August 1 catastrophe first occurred, Bleskachek has been working tirelessly by coordinating volunteer and emergency supply logistics, and she was the first to respond to an unsolicited call from a Connecticut company offering to contribute a shipment of Soapopular, a new, alcohol-free hand sanitizer, for emergency workers at the disaster scene.
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Examiner Reader said:
Hand-Sanitizer=Alcohol Poisoning.. As inane as the subject might seam, the exponential growth in the use of hand sanitizer products over the past few years has lead to an ever-increasing number of alcohol-poisoning instances--and too many within school/educational settings. Most recent report was two weeks ago in Hartford CT, where second grader, overloaded her hands from a Purell bottle on her teachers desk ,then licked it off--and was soon rushed to Yale University Hospital and diagnosed with alchohol poisoning. Thank goodness that some new manufacturers, including Soapopular--which offers a full line of Alcohol-FREE hand sanitizing products, are now getting retailers to put their products on their shelves. Soapopular, which is Canada's leading brand in the alcohol-free segment, made its debut last week here in the US.
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