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【bio-news】基因缺失引发新型免疫性疾病
ScienceDaily (Aug. 2, 2008) — A new study, published in the journal Science, describes 9 cases of children severely infected with common bacteria due to a deficiency in the MyD88 gene -- key to innate protection against infections.
From the moment we are born, we all come into continuous contact with microbes that can cause disease. To deal with them, we have a highly effective immune system that allows our bodies to identify and eliminate agents that cause infections. Part of this mechanism is innate (already present at the time of birth) and the remaining part improves as we come into contact with new pathogens.
From birth, the immune system reacts to infection with an inflammatory response that cause fever, pain, and an increased number of white blood cells in the blood, together with dilation of the blood vessels in the affected area. This reaction serves to isolate and destroy the pathogen and is a warning that something is wrong.
An international study, which involved the participation of Hospital Germans Trias (pediatrics and immunology), Hospital Clínic (immunology) and Hospital Sant Joan de Déu (pediatrics) in Barcelona and Hospital Dr. Negrín in Las Palmas de Gran Canaria, has identified a strange disease in which the innate immune system works in an irregular fashion.
The study describes 9 cases of children severely infected by common bacteria, specifically pneumococci and staphylococci, who do not react to the infection with an inflammatory response; that is, they have no fever and there is no detected increase in the number of white blood cells in the blood. By the time they see a doctor, the infection is widespread. In fact, 3 of the children, aged between 1 and 11 months, died.
The most curious aspect is that the affected children were able to see off other infections and responded to vaccines and antibiotics. This means that their immune system was able to detect other microbes and stimulate the production of antibodies.
A genetic analysis of the children finally revealed a deficiency in a gene, known as myD88, which is essential for correctly identifying the presence of an infection caused by pathogens and responding to it.
In order to make sure that this was the true cause of the disease, the researchers used genetically modified animals with this gene altered.
Unlike the affected children, the animals were susceptible to a large number of pathogens, not just pneumococci and staphylococci. This suggests that the human immune system has alternative mechanisms to compensate for the MyD88 deficiency. Another suspicion of the study's authors is that the affected children will improve with age. New research is needed to confirm these hypotheses.
The 6 surviving patients are now aged between 3 and 16 years. They are being treated with antibiotics effective against pneumococcus and staphylococcus infections and lead a normal life.
The study was coordinated by researchers from the Necker hospital in Paris (attached to the INSERM) and, as well as the 4 Spanish centres, the study included participation by centres from the US, Thailand, Turkey, Portugal, France, UK, Hungary, Israel and Canada. Three of the children were diagnosed at Hospital Germans Trias, 2 at Hospital Sant Joan de Déu, 2 in Portugal, 1 in France, and 1 in Turkey.
http://www.sciencedaily.com/releases/2008/07/080731143331.htm 本人已认领该篇文章，48小时候如果未交出请其他战友自动认领。 New Immune Disease Identified
新型免疫疾病
ScienceDaily (Aug. 2, 2008) — A new study, published in the journal Science, describes 9 cases of children severely infected with common bacteria due to a deficiency in the MyD88 gene -- key to innate protection against infections.
每日科学（2008年8月2日）发表于科学杂志的一项研究描述9例受普通细菌严重感染的儿童，他们所以受到感染是因为缺乏MyD88基因，一种先天抗感染的保护基因。
From the moment we are born, we all come into continuous contact with microbes that can cause disease. To deal with them, we have a highly effective immune system that allows our bodies to identify and eliminate agents that cause infections. Part of this mechanism is innate (already present at the time of birth) and the remaining part improves as we come into contact with new pathogens.
人们在出生后都会不断的接触那些可能导致疾病的微生物。为了避免被感染，人体具有高效的免疫系统使机体能够识别和清除导致感染的病原。抗感染机制的一部分系先天因素（在出生时就已经存在），其余部分在机体接触新病原体过程中不断产生。
From birth, the immune system reacts to infection with an inflammatory response that cause fever, pain, and an increased number of white blood cells in the blood, together with dilation of the blood vessels in the affected area. This reaction serves to isolate and destroy the pathogen and is a warning that something is wrong.
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作者:admin@医学,生命科学 2011-05-05 17:01
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