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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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Making Christmas relevant to a suffering people - NCRonline.org: a sampling of some of the regular features on NCR Web site - Excerpt
From National Catholic Reporter, 1/9/04 by Joe Komakoma

Global Perspective by Joe Komakoma Excerpt from Dec. 24

LUSAKA, Zambia -- We face the dilemma of preaching the Good News of Christmas to more than 8 million people (out of 10 million total population) who live on $1 a day.

Only half a million people work in the formal job sector. An estimated 2 million people live with HIV; a quarter of a million with full-blown AIDS. Government hospitals lack basic drugs to give to poor people who cannot afford the readily available but expensive drugs in private hospitals, clinics and drugstores.

To make it worse, a privileged few flaunt their wealth in the faces of the suffering majority. These are the ones who, when Christmastime comes, want to shop until they drop.

What message do you preach in this context?

An entry point for trying to make Christmas relevant to a suffering people is the family. As Africans, the family is the place for solidarity and warmth in human relationships.

Sadly, the family is under siege. Poverty and disease are stretching to the limits the natural tendency we have to care for others. When there is so little, survival becomes paramount.

The clarion call, then, at Christmas, is that we must save the community values that make the African family unique in the world. It is time to identify with the struggling families as they try to make ends meet, so that they do not feel abandoned.

It was not by accident that the African Synod chose the "church-as-family" as the model of the African church. This was meant to capture what Pope John Paul II called "an acute sense of solidarity and community life" that we Africans have.

Christmas is, therefore, a time for us to rediscover the deeply rooted values that are being tested severely by market-based values that tell people to maximize individual satisfaction, often to the detriment of others. We have to counter the logic of the market, which has been given too much leeway in shaping social norms, even for impoverished countries like Zambia.

Fr. Joe Komakoma is a priest from Lusaka, Zambia, where he directs the Catholic Center for Justice, Development and Peace.

NCRonline.org draws on writers throughout the world to offer insights from a global perspective.

COPYRIGHT 2004 National Catholic Reporter
COPYRIGHT 2004 Gale Group

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