первичный иммунодефицит (ПИД) -> πρωτοπαθής ανοσοανεπάρκεια (ΠΑΑ)


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первичный иммунодефицит (ПИД) -> συγγενής ανοσοανεπάρκεια, πρωτοπαθής ανοσοανεπάρκεια (ΠΑΑ), πρωτογενής ανοσοανεπάρκεια

Первичный иммунодефицит (ПИД) – гетерогенная (неоднородная) группа наследственных заболеваний, приводящих к нарушению в работе иммунной системы. Причиной ПИД является мутация в генах, которая передалась по наследству от родителей или произошла спонтанно. В настоящее время идентифицировано около 300 генов первичных иммунодефицитов.

Primary immunodeficiencies are disorders in which part of the body's immune system is missing or does not function normally. To be considered a primary immunodeficiency (PID), the cause of the immune deficiency must not be secondary in nature (i.e., caused by other disease, drug treatment, or environmental exposure to toxins). Most primary immunodeficiencies are genetic disorders; the majority are diagnosed in children under the age of one, although milder forms may not be recognized until adulthood. While there are over 300 recognized PIDs, most are very rare. About 1 in 500 people in the United States are born with a primary immunodeficiency. Immune deficiencies can result in persistent or recurring infections, autoinflammatory disorders, tumors, and disorders of various organs. There are currently limited treatments available for these conditions; most are specific to a particular type of PID. Research is currently evaluating the use of stem cell transplants (HSCT) and experimental gene therapies as avenues for treatment in limited subsets of PIDs.

ar: نقص المناعة الأولي; be: спадчынны імунадэфіцыт; ca: immunodeficiència primària; en: primary immunodeficiency; eo: primara imuna deficito; fi: primaari immuunipuutos; fr: déficit immunitaire primitif; ja: 原発性免疫不全症候群; ko: 원발성면역결핍증후군; nl: primaire immuundeficiëntie; pl: pierwotne niedobory odporności; pt: imunodeficiência congênita; sh: primarna imunodeficijencija; sr: primarna imunodeficijencija; uk: спадковий імунодефіцит


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