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German patient is immune to deadly poison ricin
Ricin is one of the most toxic substances in the world. The plant poison was also used for biological warfare. No effective antidote is known to date. However, there are people who are immune to ricin. Three of these people are known worldwide, one of them comes from Germany.
Ricin is considered one of the deadliest poison
Ricin is one of the deadliest poisons in the world. In the German War Weapons Control Act, the plant poison is listed as a weapon of war. Throughout history, the material has been used again and again for biological warfare and in recent years also in the context of bio-terrorist attacks. In April 2013, the poison hit the headlines internationally because the FBI intercepted a letter with ricin to then-President Obama. But some people are immune to the dangerous poison. One of them comes from Germany.
Umbrella attack with plant poison
Ricin became famous worldwide when the Bulgarian dissident Georgi Markow was murdered in London in 1978. The University Hospital Münster (UKM) wrote in a message:
"A reporter is waiting for the bus in city traffic when a passing passer-by apparently accidentally touches his leg with the tip of his umbrella - four days later the man is dead."
And further: "The autopsy shows that the plant poison ricin was injected into the leg with a millimeter-sized platinum ball - the legend of the umbrella attack was born."
Although no effective antidote has been found to date, according to the UKM, there are three people in the world who would have survived the attack: They have a genetic metabolic defect and are unable to convert the sugar fucose.
One of them is 20-year-old Jakob (name changed), who has been treated at the UKM since his birth.
The mechanism by which the poison is absorbed is now better understood
Jakob, who was born prematurely in 1997 at 770 grams, had to be operated on for the first time the day after birth. In his first two years of life he often had a high fever.
"For a long time we could not explain why he always has a fever," explains Prof. Dr. Thorsten Marquardt, Head of Congenital Metabolic Diseases at the UKM, in a message from the dpa news agency.
According to the information, a young doctor on the ward finally got on the right track: "He bit into the fact that Jakob has far too many white blood cells," explains the doctor.
"Jakob is a stroke of luck for research," Marquardt told the German Press Agency. Thanks to him, the mechanism of absorption of the poison is better understood today. "Where you know the mechanisms, you can develop antidotes," says the expert.
Ricin is a toxic protein from the seeds of the miracle tree. The dose of just a few seeds can sometimes be fatal.
"Symptoms of poisoning include vomiting, diarrhea, circulatory and kidney failure," explains toxicologist Prof. Markus Christmann from the University Medical Center Mainz in the dpa report.
Only three patients worldwide
A few months ago, scientists from the UKM and researchers from the Institute for Molecular Biotechnology at the Austrian Academy of Sciences (IMBA) reported that they had deciphered the mode of action of the natural toxin ricin.
One patient helped: “Almost 20 years ago we met Jakob here, who had this rare metabolic disorder. We provided our colleagues from Vienna with skin samples that they could use to decipher the mechanism of action of ricin, ”explains Marquardt in the message.
According to the experts, there are two genes that make ricin fatal. One of them is defective in patients, so their cells are immune to the poison.
In addition to Jakob, there are only two other people worldwide who are known to have the same defect. Both live in Israel.
Since it is now known that those affected cannot produce fucose due to the genetic defect, a therapy was developed: Fucose is artificially administered to the patient in Münster.
According to the UKM, the febrile episodes that forced the boy to spend a whole year in the hospital were quickly over thanks to the medication.
Step on the way to the antidote
"The newly gained knowledge about deciphering the mechanism of action of one of the deadliest poisons at all is a prime example of how dealing with rare diseases can not only help the affected patients, but also provide fundamental scientific results that benefit medicine as a whole," said Marquardt in the UKM Communication.
Her study, which was published in the specialist magazine "Cell Research", was another step on the long road to the antidote: "It is still a dream of the future, but we have made some progress."
Rare diseases are not that rare
Jakob was able to help the doctors, but many other patients often have to wait long or in vain for help.
According to the National Action Alliance for People with Rare Diseases (NAMSE), a disease is considered rare if fewer than five out of 10,000 people are affected.
In Germany alone, around four million people suffer from one of the approximately 6,000 rare diseases that have so far had a name. Across Europe, 30 million patients are affected. Rare diseases are often not that rare.
Because specialists and often also effective therapies are lacking, the diagnosis is often difficult and lengthy. An innovation that was introduced a few years ago at the “Frankfurt Reference Center for Rare Diseases” (FRZSE) is promising.
There young medical students are looking for the diagnosis of rare diseases. They often recognize them better than older, more experienced doctors.
Since 2010, the NAMSE has been working on coordinated action in the field of such ailments - this includes the establishment of specialist centers.
As Christine Mundlos of the Alliance for Chronic Rare Diseases (Axis) explains in the agency report, there are currently 28 centers nationwide.
So far, however, there has been no certification procedure - the clinics can call themselves a specialist center. Work is underway to change that. (ad)