Crusaders' disease re-emerges in Cyprus, DNA tests urged for early diagnosis

Source: Xinhua| 2018-05-19 02:41:19|Editor: Mu Xuequan
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NICOSIA, May 18 (Xinhua) -- The Crusaders conquered Cyprus just over 800 years ago and their rule over the eastern Mediterranean island ended in the late 15th century, but they left behind a horrific disease that still haunts many Cypriots.

It is appropriately known as the crusaders' disease, which strikes the liver and leads to the degeneration of the nervous system, causing pain and discomfort in the lower gastrointestinal system, fatigue, poor coordination, paralysis of the legs which leads patients to wheel chairs, and finally death.

The issue came to prominence again after the parliamentary health committee was told that the disease reappeared in people who had undergone liver transplants 10 to 15 years ago.

Andreas Savva, community leader of Pachna, a village northwest of Limassol and one of the communities with a high concentration of people with the disease, pleaded with deputies to approve and allocate DNA tests for all inhabitants.

"It is important to diagnose the disease at an early stage so that those who have it start early treatment, which will prevent its progress," Savva told Xinhua in a telephone statement on Friday.

Theodoros Kyriakides, head of the neurology clinic at the Cyprus Institute of Neurology and Genetics, also confirmed the re-emergence of the disease among people who have had a liver transplant.

The re-emergence causes various complications that are treated symptomatically, as there is no definitive cure yet, he said.

It is up to people get a DNA test so that the disease can be diagnosed early, Kyriakides added.

But DNA tests cost around 200 euros (235 U.S. dollars) which many people in the village cannot afford.

"Treatment of the disease to stop its progress is even costlier. The drug taken by patients can cost them up to 18,000 euros a month," said Savva.

DNA tests, he said, are also important, as carriers can avoid marriage with other carriers, which passes the disease to the children.

The disease is treated with the drug Tafamidis. The medicine slows the progression of the disease until a liver transplant can be performed.

Deputies were told, however, that up to 20 percent of people who were carriers and who had a liver transplant, exhibit symptoms of the disease 10 to 15 years post-surgery.

Deputy Andreas Kyprianou said almost all Cypriots diagnosed with Crusaders' disease have undergone a liver transplant abroad, with the state covering the expenses.

But given this latest information regarding the resurgence of the disease, it was important to stay abreast of medical research, he said.

The disease is believed to have been brought to Cyprus by Portuguese crusaders who established themselves in fiefs in the hills northwest of the city port of Limassol, not far from their command center at the Colossi Castle.

Intermarriage with local people helped spread the disease, which became even more concentrated in the area as people used to marry within their communities.

The disease affects two in every 100,000 people worldwide, but it is estimated that in Cyprus its frequency is more than double. Five people in every 100,000 have the disease.

The official name of the disease is Friedreich's ataxia, after the German physician Nikolaus Friedreich, who first described it in the 1860s.

It results from a genetic mutation that leads to reduced expression of the mitochondrial protein frataxin. Over time, this deficiency causes damage to the nervous tissues in the spinal cord, which becomes thinner and nerve cells lose some of their myelin sheath.

Kyriakides said the Institute of Neurology and Genetics was trying to follow international developments in the treatment of the disease.

"We are doing everything possible, in association with foreign medical centers, to be able to offer the best to these patients. When the disease is diagnosed early, treatment can be very effective," he said.

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