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Christmas disease

Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. It is the second most common form of haemophilia, rarer than haemophilia A. It is sometimes called Christmas disease after Stephen Christmas, the first patient described with this disease. In addition, the first report of its identification was published in the Christmas edition of the British Medical Journal. Treatment (bleeding prophylaxis) is by intravenous infusion of factor IX. more...

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Genetics and pathophysiology

The factor IX gene is located on the X chromosome (Xq27.1-q27.2). It is inherited X-linked recessive, which explains why - as in haemophilia A - only males are generally affected.

Factor IX deficiency leads to an increased propensity for haemorrhage. This is in response to mild trauma or even spontaneously, such as in joints (haemarthrosis) or muscles.

Reference

  • Biggs RA, Douglas AS, MacFarlane RG, Dacie JV, Pittney WR, Merskey C, O'Brien JR. Christmas disease: a condition previously mistaken for haemophilia. Br Med J 1952;2:1378-1382. PMID 12997790.

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Chemical Christmas: immigrant tree farm workers face pesticide dangers
From E: The Environmental Magazine, 11/1/05 by Jesse James DeConto

Every Christmas, little Katie Alvarez asks her daddy for two trees, one for her formal living room, and one for the family room. Katie has no idea what those trees might cost her father. Treating Christmas tree fields each spring, farm-worker Felix Alvarez, 43, soaks his skin with Roundup, a weedkiller linked to cancer among applicators. "Later on, when I get older, I'll probably have some consequence about it because I've been getting wet all over," Alvarez says.

Second only to Oregon, the state of North Carolina produces roughly one out of five Christmas trees sold in the U.S.--about five million a year, worth more than $100 million. Each spring and summer, Hispanic workers like Alvarez handle some of the deadliest pesticides allowed by law, potentially risking their health to help Americans celebrate life.

Fifty million Fraser firs grow on 25,000 acres in the mountains of North Carolina, and almost every acre is treated with Roundup. A salt compound, Roundup can irritate the eyes and is toxic when inhaled. In 1999, the American Cancer Society published the results of a survey of non-Hodgkin's lymphoma patients showing the disease was 2.7 times more likely among those who had applied glyphosate, the herbicide sold under the "Roundup" trade name.

The most hazardous and second-most common pesticide in North Carolina Christmas tree farming is Di-Syston 15-G, a powder traditionally applied with a bucket and measuring spoon. "If one grain gets in your boot, and your foot sweats, by the end of the day, you could be dead" says Richard Boylan, an alternative agriculture agent with the North Carolina State University Cooperative Extension.

Last year, Bayer CropSciences voluntarily discontinued Di-Syston 15-G for all crops other than Fraser firs in North Carolina and coffee in Puerto Rico. U.S. farms had previously used it for food crops such as Brussels sprouts, cabbage, cauliflower, peanuts and peppers. The EPA had threatened to ban it completely, but North Carolina's Christmas tree growers worked to develop a new closed-system applicator that enables workers to pour the granules from a stainless-steel spout rather than scooping them from an open bucket. "We believe that the granular risks can be adequately mitigated by closed systems," says EPA spokesperson Enesta Jones.

Christmas tree farmers use Di-Syston to control balsam twig aphids in the spring, and its residues prevent spruce spider mites throughout the year. The aphids cuff needles and stunt growth in firs, sometimes enough to make them worthless; the spider mites discolor evergreen needles by sucking their sap.

"If they lost [Di-Syston] and didn't come up with something that would take its place, you can put the Christmas tree business out of business," one grower told Wake Forest University researcher Pamela Rao in a paper published in Human Organization.

Ever since a public outcry over childhood leukemia in the early 1990s, growers have been cutting pesticide use through a technique called Integrated Pest Management, which targets chemicals only where they're really needed. Between 1994 and 2000, growers reduced Di-Syston use from 65 percent of total acreage to 50 percent, according to North Carolina State Christmas tree specialist Jill Sidebottom.

"The industry has grown a lot," says Debbie Fishel, whose Grouse Ridge Farms employ 30 to 60 Hispanic workers each year. "I live in the same area they do and drink the same water. I am exposed to the same environment they are, and we have become aware of the risks."

As they have reduced reliance on Di-Syston, growers have increased the spread of other insecticides, including Dimethoate, Lindane and Asana, all slightly or moderately toxic chemicals. Though Lindane is the least immediately harmful of these, the EPA banned it in 2002 for its chronic effects and persistence in the environment.

Like Di-Syston, Dimethoate is an organophosphate that can cause numbness, tingling sensations, headaches, dizziness, tremors, nausea, abdominal cramps, sweating, blurred vision, difficulty breathing and slow heartbeat, experts say.

Asana is another spray that controls the balsam woolly adelgid, a European insect that has devastated Fraser firs in forest stands, flattening their tops, swelling their joints and hardening their wood. A worker poisoned with Asana could experience dizziness, burning, itching, blurred vision, tightness in the chest, convulsions, nausea, vomiting, headache, weakness or tremors, according to EXTOXNET, a network of university cooperative extension offices from across the United States. As with Dimethoate, swallowing less than two tablespoons of Asana can kill a grown man.

"Asana can make your skin burn for about six hours" said one Christmas tree worker in 2001. "Sometimes I can't sleep at night because my face burns so much. I use a mask and glasses, but there are still parts of my face that aren't covered, and the spray gets on your face anyway."

A Wake Forest public health professor, Thomas Arcury, has led a series of studies on pesticide exposure among Christmas tree workers, finding chemical traces in their homes, on their children's hands and toys, and in family urine samples. One study concluded that growers do not take the threat seriously enough. They sometimes neglect their own personal protective equipment, setting a poor example for the workers. Assessing the level of risk is a point of contention among growers, the public and even the experts. Though workers share anecdotes of pesticide poisonings and neighbors worry about groundwater contamination, not one case of long-term illness has been linked to Christmas tree farming in North Carolina.

"I can't prove that you're going to get sick 20 years from now," says Arcury. "We don't have any direct evidence like that. However, if you look at the preponderance of all the scientific evidence ... then we would have to argue that there is, in fact, an increased risk." CONTACT: Pesticide Action Network, (415)981-1771, www.panna.org.

COPYRIGHT 2005 Earth Action Network, Inc.
COPYRIGHT 2005 Gale Group

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