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<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">mimmun</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинская иммунология</journal-title><trans-title-group xml:lang="en"><trans-title>Medical Immunology (Russia)</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1563-0625</issn><issn pub-type="epub">2313-741X</issn><publisher><publisher-name>SPb RAACI</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15789/1563-0625-2013-5-477-492</article-id><article-id custom-type="elpub" pub-id-type="custom">mimmun-665</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>СТАНДАРТЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>STANDARDS</subject></subj-group></article-categories><title-group><article-title>МНОГОСТУПЕНЧАТЫЙ ДИАГНОСТИЧЕСКИЙ ПРОТОКОЛ СКРИНИНГА ПАЦИЕНТОВ НА НАЛИЧИЕ ПЕРВИЧНОГО ИММУНОДЕФИЦИТА, РАЗРАБОТАННЫЙ ДЛЯ ВРАЧЕЙ-НЕИММУНОЛОГОВ (ОБНОВЛЕНИЕ ОТ 2011 Г.)</article-title><trans-title-group xml:lang="en"><trans-title>PATIENT-CENTRED SCREENING FOR PRIMARY IMMUNODEFICIENCY, A MULTI-STAGE DIAGNOSTIC PROTOCOL DESIGNED FOR NONIMMUNOLOGISTS: 2011 UPDATE</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>де Вриз</surname><given-names>Э.</given-names></name><name name-style="western" xml:lang="en"><surname>De Vries</surname><given-names>E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника педиатрии</p></bio><bio xml:lang="en"><p>Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Альварес Кардона</surname><given-names>А.</given-names></name><name name-style="western" xml:lang="en"><surname>Cardona</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>отдел исследования первичного иммунодефицита</p></bio><bio xml:lang="en"><p>Primary Immunodeficiency Investigation Unit</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Абдул-Латиф</surname><given-names>А. Х.</given-names></name><name name-style="western" xml:lang="en"><surname>Abdul Latiff</surname><given-names>A. H.</given-names></name></name-alternatives><bio xml:lang="ru"><p>отделение клинической иммунологии и педиатрии медицинского факультета</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бадолато</surname><given-names>Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Badolato</surname><given-names>R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кафедра педиатрии</p><p>корреспонденцию направлять в больницу Spedali Civili</p></bio><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бродшки</surname><given-names>Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Brodszki</surname><given-names>N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детской иммунологии</p></bio><bio xml:lang="en"><p>Department of Paediatric Immunology</p></bio><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кант</surname><given-names>Э. Дж.</given-names></name><name name-style="western" xml:lang="en"><surname>Cant</surname><given-names>A. J.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-6"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Карбоне</surname><given-names>Х.</given-names></name><name name-style="western" xml:lang="en"><surname>Carbone</surname><given-names>J.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника иммунологии</p></bio><xref ref-type="aff" rid="aff-7"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каспер</surname><given-names>Дж. Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Casper</surname><given-names>J. T.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кафедра педиатрии, иммунологии и трансплантации костного мозга</p></bio><bio xml:lang="en"><p>Department of Paediatrics, Immunology/BMT</p></bio><xref ref-type="aff" rid="aff-8"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чижняр</surname><given-names>П.</given-names></name><name name-style="western" xml:lang="en"><surname>Ciznar</surname><given-names>A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>1-ая кафедра педиатрии, медицинский факультет</p></bio><bio xml:lang="en"><p>1st Paediatric Department</p></bio><xref ref-type="aff" rid="aff-9"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кочино</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Cochino</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кафедра педиатрии</p></bio><bio xml:lang="en"><p>Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-10"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дерфальви</surname><given-names>Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Derfalvi</surname><given-names>B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>2-ая клиника педиатрии, отделение иммунологии-ревматологии-нефрологии</p></bio><bio xml:lang="en"><p>2nd Department of Paediatrics, Immunology–Rheumatology–Nephrology Unit</p></bio><xref ref-type="aff" rid="aff-11"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дриссен</surname><given-names>Г. Й.</given-names></name><name name-style="western" xml:lang="en"><surname>Driessen</surname><given-names>G. J.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детских инфекционных болезней и иммунологии</p></bio><bio xml:lang="en"><p>Department of Paediatric Infectious Disease and Immunology</p></bio><xref ref-type="aff" rid="aff-12"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Эльфеки</surname><given-names>Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Elfeky</surname><given-names>R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кафедра педиатрии</p></bio><bio xml:lang="en"><p>Department of Pediatrics</p></bio><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Эль-Гонейми</surname><given-names>Д.</given-names></name><name name-style="western" xml:lang="en"><surname>El-Ghoneimy</surname><given-names>D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детской аллергии и иммунологии, медицинский факультет</p></bio><bio xml:lang="en"><p>Department of Paediatric Allergy &amp; Immunology, Faculty of Medicine</p></bio><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Эспаньол</surname><given-names>Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Espanol</surname><given-names>T.</given-names></name></name-alternatives><bio xml:lang="ru"><p>отделение иммунологии</p></bio><bio xml:lang="en"><p>Immunology Unit</p></bio><xref ref-type="aff" rid="aff-14"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ациони</surname><given-names>А.</given-names></name><name name-style="western" xml:lang="en"><surname>Etzioni</surname><given-names>A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>медицинский факультет</p></bio><xref ref-type="aff" rid="aff-15"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гамбинери</surname><given-names>Э.</given-names></name><name name-style="western" xml:lang="en"><surname>Gambineri</surname><given-names>E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>кафедра исследования здоровья женщины и ребенка</p></bio><bio xml:lang="en"><p>Department of Sciences for Woman and Child’s Health</p></bio><xref ref-type="aff" rid="aff-16"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гилмор</surname><given-names>К.</given-names></name><name name-style="western" xml:lang="en"><surname>Gilmour</surname><given-names>K.</given-names></name></name-alternatives><bio xml:lang="ru"><p>лаборатории имени Камелии Ботнер</p></bio><bio xml:lang="en"><p>Camelia Botnar Laboratories</p></bio><xref ref-type="aff" rid="aff-17"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гонсалес-Гранадо</surname><given-names>Л. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Gonzalez-Granado</surname><given-names>L. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>отделение иммунодефицитов, клиника педиатрии</p></bio><bio xml:lang="en"><p>Immunodeficiencies Unit, Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-18"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гусева</surname><given-names>М. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Guseva</surname><given-names>M. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Консультативно-диагностический центр Клинической больницы</p></bio><bio xml:lang="en"><p>Consulting Center</p></bio><xref ref-type="aff" rid="aff-19"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Х.</surname><given-names>Хаверкамп М.</given-names></name><name name-style="western" xml:lang="en"><surname>Haverkamp</surname><given-names>M. H.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника инфекционных болезней</p></bio><bio xml:lang="en"><p>Department of Infectious Diseases</p></bio><xref ref-type="aff" rid="aff-20"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хельминен</surname><given-names>М.</given-names></name><name name-style="western" xml:lang="en"><surname>Helminen</surname><given-names>M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детских инфекционных болезней</p></bio><bio xml:lang="en"><p>Department of Paediatric Infectious Diseases</p></bio><xref ref-type="aff" rid="aff-21"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хёниг</surname><given-names>М.</given-names></name><name name-style="western" xml:lang="en"><surname>Honig</surname><given-names>M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника педиатрии,</p></bio><bio xml:lang="en"><p>Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-22"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Канариу</surname><given-names>М. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Kanariou</surname><given-names>M. G.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-23"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Киршфинк</surname><given-names>М.</given-names></name><name name-style="western" xml:lang="en"><surname>Kirschfink</surname><given-names>M.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-24"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кляйн</surname><given-names>К.</given-names></name><name name-style="western" xml:lang="en"><surname>Klein</surname><given-names>C.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-25"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кёйперс</surname><given-names>T. B.</given-names></name><name name-style="western" xml:lang="en"><surname>Kuijpers</surname><given-names>T. W.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детской гематологии, иммунологии и инфекционных болезней</p></bio><bio xml:lang="en"><p>Division of Paediatric Hematology, Immunology and Infectious diseases</p></bio><xref ref-type="aff" rid="aff-26"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кутукджюлер</surname><given-names>Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Kutukculer</surname><given-names>N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника педиатрии, отделение педиатрической иммунологии</p></bio><bio xml:lang="en"><p>Department of Pediatrics, Division of Pediatric Immunology</p></bio><xref ref-type="aff" rid="aff-27"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мартире</surname><given-names>Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Martire</surname><given-names>B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>отделение развития биомедицины</p></bio><bio xml:lang="en"><p>Dipartimento di Biomedicina dell’Eta Evolutiva</p></bio><xref ref-type="aff" rid="aff-28"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мейтс</surname><given-names>И.</given-names></name><name name-style="western" xml:lang="en"><surname>Meyts</surname><given-names>I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника педиатрии</p></bio><bio xml:lang="en"><p>Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-29"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Нихюэс</surname><given-names>Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Niehues</surname><given-names>T.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-30"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пиньята</surname><given-names>Ч.</given-names></name><name name-style="western" xml:lang="en"><surname>Pignata</surname><given-names>C.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника педиатрии</p></bio><bio xml:lang="en"><p>Department of Paediatrics</p></bio><xref ref-type="aff" rid="aff-31"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рида</surname><given-names>С. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Reda</surname><given-names>S. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детской аллергии и иммунологии, медицинский факультет</p></bio><bio xml:lang="en"><p>Department of Paediatric Allergy and Immunology, Faculty of Medicine</p></bio><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Реннер</surname><given-names>Э. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Renner</surname><given-names>E. D.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-32"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Резаеи</surname><given-names>Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Rezaei</surname><given-names>N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>центр молекулярной иммунологии и группа исследования иммунодефицитов</p></bio><bio xml:lang="en"><p>Molecular Immunology Research Centre and Research Group for Immunodeficiencies</p></bio><xref ref-type="aff" rid="aff-33"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рицци</surname><given-names>М.</given-names></name><name name-style="western" xml:lang="en"><surname>Rizzi</surname><given-names>M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Центр хронического иммунодефицита</p></bio><bio xml:lang="en"><p>Center for Chronic Immunodeficiency</p></bio><xref ref-type="aff" rid="aff-34"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сампало Лайнс</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Sampalo Lainz</surname><given-names>M. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника иммунологии</p></bio><bio xml:lang="en"><p>Department of Immunology</p></bio><xref ref-type="aff" rid="aff-35"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Саргур</surname><given-names>Р. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Sargur</surname><given-names>R. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника иммунологии</p></bio><bio xml:lang="en"><p>Department of Immunology</p></bio><xref ref-type="aff" rid="aff-36"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Седива</surname><given-names>А.</given-names></name><name name-style="western" xml:lang="en"><surname>Sediva</surname><given-names>A.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-37"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зайдель</surname><given-names>M. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Seidel</surname><given-names>M. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>иммунологическое поликлиническое отделение</p></bio><bio xml:lang="en"><p>Paediatric Immunology Outpatient Clinic</p></bio><xref ref-type="aff" rid="aff-38"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сеневиратне</surname><given-names>С. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Seneviratne</surname><given-names>S. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника клинической иммунологии</p></bio><bio xml:lang="en"><p>Department of Clinical Immunology</p></bio><xref ref-type="aff" rid="aff-39"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Солер-Паласин</surname><given-names>П.</given-names></name><name name-style="western" xml:lang="en"><surname>Soler-Palacin</surname><given-names>P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клиника детских инфекционных болезней и иммунодефицитов</p></bio><bio xml:lang="en"><p>Pediatric Infectious Diseases and Immunodeficiencies Unit</p></bio><xref ref-type="aff" rid="aff-40"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Томмазини</surname><given-names>А.</given-names></name><name name-style="western" xml:lang="en"><surname>Tommasini</surname><given-names>A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>лаборатория иммунопатологии</p></bio><bio xml:lang="en"><p>Laboratory of Immunopathology</p></bio><xref ref-type="aff" rid="aff-41"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Варнатц</surname><given-names>К.</given-names></name><name name-style="western" xml:lang="en"><surname>Warnatz</surname><given-names>K.</given-names></name></name-alternatives><bio xml:lang="ru"><p>центр хронического иммунодефицита</p></bio><bio xml:lang="en"><p>Centre of Chronic Immunodeficiency</p></bio><xref ref-type="aff" rid="aff-42"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Больница имени Иеронима Босха, Хертогенбос</institution><country>Нидерланды</country></aff><aff xml:lang="en"><institution>Jeroen Bosch Hospital ’s-Hertogenbosch</institution><country>Netherlands</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Национальный институт педиатрии, Мексиканский автономный университет, Мехико</institution><country>Мексика</country></aff><aff xml:lang="en"><institution>Instituto Nacional de Pediatr a, Universidad Aut noma de M xico, Ciudad de Mexico</institution><country>Mexico</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Университет имени Монаша, филиал Санвэй в Бандар-Санвэй</institution><country>Малайзия</country></aff><aff xml:lang="en"><institution>Division of Clinical Immunology and Paediatrics School of Medicine and Health Sciences, Monash University, Sunway Campus</institution><country>Malaysia</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>Университет Брешиа, Брешиа</institution><country>Италия</country></aff><aff xml:lang="en"><institution>Clinica Pediatrica dell’Universit di Brescia c/o Spedali Civili, Brescia, Italy</institution><country>Italy</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>Лундская университетская больница, Лунд</institution><country>Швеция</country></aff><aff xml:lang="en"><institution>Lund University Hospital, Lund</institution><country>Sweden</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>Большая Северная детская больница, Ньюкасл-апон-Тайн</institution><country>Великобритания</country></aff><aff xml:lang="en"><institution>Great North Children’s Hospital, Newcastle upon Tyne</institution><country>United Kingdom</country></aff></aff-alternatives><aff-alternatives id="aff-7"><aff xml:lang="ru"><institution>Больница им. Грегорио Мараньона, Мадрид</institution><country>Испания</country></aff><aff xml:lang="en"><institution>Department of Immunology, Gregorio Mara on Hospital, Madrid</institution><country>Spain</country></aff></aff-alternatives><aff-alternatives id="aff-8"><aff xml:lang="ru"><institution>Медицинский колледж Висконсина; Исследовательский центр Фонда «Спортсмены Среднего Запада против детского рака» (Фонд MACC), Милуоки</institution><country>Соединённые Штаты Америки</country></aff><aff xml:lang="en"><institution>Medical College of Wisconsin, MACC Fund Research Center, Milwaukee</institution><country>United States</country></aff></aff-alternatives><aff-alternatives id="aff-9"><aff xml:lang="ru"><institution>Университет им. Яна Коменского, Детская университетская больница, Братислава</institution><country>Словакия</country></aff><aff xml:lang="en"><institution>Comenius University Medical School, Children’ University Hospital, Bratislava</institution><country>Slovakia</country></aff></aff-alternatives><aff-alternatives id="aff-10"><aff xml:lang="ru"><institution>Медико-фармацевтический университет имени Кароля Давилы, Бухарест</institution><country>Румыния</country></aff><aff xml:lang="en"><institution>University of Medicine and Pharmacy ‘Carol Davila’, Bucharest</institution><country>Romania</country></aff></aff-alternatives><aff-alternatives id="aff-11"><aff xml:lang="ru"><institution>Семелвейский медицинский университет Будапешта, Будапешт</institution><country>Венгрия</country></aff><aff xml:lang="en"><institution>Semmelweis University Budapest, Budapest</institution><country>Hungary</country></aff></aff-alternatives><aff-alternatives id="aff-12"><aff xml:lang="ru"><institution>медицинский центр «Эразмус», Университетский медицинский центр Роттердама, Роттердам</institution><country>Нидерланды</country></aff><aff xml:lang="en"><institution>Erasmus MC, University Medical Center Rotterdam, Rotterdam</institution><country>Netherlands</country></aff></aff-alternatives><aff-alternatives id="aff-13"><aff xml:lang="ru"><institution>Университет Айн-Шамс, Каир</institution><country>Египет</country></aff><aff xml:lang="en"><institution>Ain Shams University, Cairo</institution><country>Egypt</country></aff></aff-alternatives><aff-alternatives id="aff-14"><aff xml:lang="ru"><institution>Университетская больница Долины Хеврона, Барселона</institution><country>Испания</country></aff><aff xml:lang="en"><institution>University Hospital Vall d’Hebron, Barcelona</institution><country>Spain</country></aff></aff-alternatives><aff-alternatives id="aff-15"><aff xml:lang="ru"><institution>Детская больница «Майер», Технион, Хайфа</institution><country>Израиль</country></aff><aff xml:lang="en"><institution>Meyer’s Children Hospital, Faculty of Medicine, Technion, Haifa</institution><country>Israel</country></aff></aff-alternatives><aff-alternatives id="aff-16"><aff xml:lang="ru"><institution>Университет Флоренции, Детская больница им. 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Лёвен</institution><country>Бельгия</country></aff><aff xml:lang="en"><institution>University Hospitals Leuven, Leuven</institution><country>Belgium</country></aff></aff-alternatives><aff-alternatives id="aff-30"><aff xml:lang="ru"><institution>клиника «Гелиос» Крефельда; Крефельдский центр иммунодефицита (KIDZ), Крефельд</institution><country>Германия</country></aff><aff xml:lang="en"><institution>Helios Klinikum Krefeld; Krefeld Immunodeficiency Centre KIDZ, Krefeld</institution><country>Germany</country></aff></aff-alternatives><aff-alternatives id="aff-31"><aff xml:lang="ru"><institution>Университет имени Фридриха II, Неаполь</institution><country>Италия</country></aff><aff xml:lang="en"><institution>‘Federico II’ University, Naples</institution><country>Italy</country></aff></aff-alternatives><aff-alternatives id="aff-32"><aff xml:lang="ru"><institution>Университетская детская больница, Университет Людвига Максимилиана, Мюнхен</institution><country>Германия</country></aff><aff xml:lang="en"><institution>University Children’s Hospital, Ludwig Maximilians Universit t, Munchen</institution><country>Germany</country></aff></aff-alternatives><aff-alternatives id="aff-33"><aff xml:lang="ru"><institution>Детский медицинский центр, Тегеранский университет медицинских наук, Тегеран</institution><country>Иран</country></aff><aff xml:lang="en"><institution>Children’s Medical Center, Tehran University of Medical Sciences, Tehran</institution><country>Islamic Republic of Iran</country></aff></aff-alternatives><aff-alternatives id="aff-34"><aff xml:lang="ru"><institution>Университетский медицинский центр Фрайбурга, Фрайбург</institution><country>Германия</country></aff><aff xml:lang="en"><institution>University Medical Center Freiburg, Freiburg</institution><country>Germany</country></aff></aff-alternatives><aff-alternatives id="aff-35"><aff xml:lang="ru"><institution>Университетская клиника Пуэрта-дель-Мар, Кадис</institution><country>Испания</country></aff><aff xml:lang="en"><institution>Puerta del Mar Universitary Hospital, Cadiz</institution><country>Spain</country></aff></aff-alternatives><aff-alternatives id="aff-36"><aff xml:lang="ru"><institution>Северная многопрофильная больница, Шеффилд</institution><country>Великобритания</country></aff><aff xml:lang="en"><institution>Northern General Hospital, Sheffield</institution><country>United Kingdom</country></aff></aff-alternatives><aff-alternatives id="aff-37"><aff xml:lang="ru"><institution>Институт иммунологии, Университетская больница Мотол, Прага</institution><country>Чехия</country></aff><aff xml:lang="en"><institution>Institute of Immunology, University Hospital Motol, Prague</institution><country>Czech Republic</country></aff></aff-alternatives><aff-alternatives id="aff-38"><aff xml:lang="ru"><institution>Детская больница Cв. Анны, Вена</institution><country>Австрия</country></aff><aff xml:lang="en"><institution>St Anna Children’s Hospital, Vienna</institution><country>Austria</country></aff></aff-alternatives><aff-alternatives id="aff-39"><aff xml:lang="ru"><institution>Больница Cв. Марии и Имперский колледж, Лондон</institution><country>Великобритания</country></aff><aff xml:lang="en"><institution>St Mary’s Hospital and Imperial College, London</institution><country>United Kingdom</country></aff></aff-alternatives><aff-alternatives id="aff-40"><aff xml:lang="ru"><institution>Университетская больница Долины Хеврона (Vall d’Hebron), Барселона</institution><country>Испания</country></aff><aff xml:lang="en"><institution>Vall d’Hebron University Hospital, Barcelona</institution><country>Spain</country></aff></aff-alternatives><aff-alternatives id="aff-41"><aff xml:lang="ru"><institution>Институт материнского и детского здоровья, IRCCS Burlo Garofolo, Триест</institution><country>Италия</country></aff><aff xml:lang="en"><institution>Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste</institution><country>Italy</country></aff></aff-alternatives><aff-alternatives id="aff-42"><aff xml:lang="ru"><institution>Университетская больница Фрайбурга, Фрайбург</institution><country>Германия</country></aff><aff xml:lang="en"><institution>University Hospital of Freiburg, Freiburg</institution><country>Germany</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2013</year></pub-date><pub-date pub-type="epub"><day>25</day><month>07</month><year>2014</year></pub-date><volume>15</volume><issue>5</issue><fpage>477</fpage><lpage>492</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; де Вриз Э., Альварес Кардона А.A., Абдул-Латиф А.Х., Бадолато Р., Бродшки Н., Кант Э.Д., Карбоне Х., Каспер Д.Т., Чижняр П., Кочино А.В., Дерфальви Б., Дриссен Г.Й., Эльфеки Р., Эль-Гонейми Д., Эспаньол Т., Ациони А., Гамбинери Э., Гилмор К., Гонсалес-Гранадо Л.И., Гусева М.Н., Х. Х.М., Хельминен М., Хёниг М., Канариу М.Г., Киршфинк М., Кляйн К., Кёйперс T.B., Кутукджюлер Н., Мартире Б., Мейтс И., Нихюэс Т., Пиньята Ч., Рида С.М., Реннер Э.Д., Резаеи Н., Рицци М., Сампало Лайнс М.А., Саргур Р.Б., Седива А., Зайдель M.Г., Сеневиратне С.Л., Солер-Паласин П., Томмазини А., Варнатц К., 2014</copyright-statement><copyright-year>2014</copyright-year><copyright-holder xml:lang="ru">де Вриз Э., Альварес Кардона А., Абдул-Латиф А.Х., Бадолато Р., Бродшки Н., Кант Э.Д., Карбоне Х., Каспер Д.Т., Чижняр П., Кочино А.В., Дерфальви Б., Дриссен Г.Й., Эльфеки Р., Эль-Гонейми Д., Эспаньол Т., Ациони А., Гамбинери Э., Гилмор К., Гонсалес-Гранадо Л.И., Гусева М.Н., Х. Х.М., Хельминен М., Хёниг М., Канариу М.Г., Киршфинк М., Кляйн К., Кёйперс T.B., Кутукджюлер Н., Мартире Б., Мейтс И., Нихюэс Т., Пиньята Ч., Рида С.М., Реннер Э.Д., Резаеи Н., Рицци М., Сампало Лайнс М.А., Саргур Р.Б., Седива А., Зайдель M.Г., Сеневиратне С.Л., Солер-Паласин П., Томмазини А., Варнатц К.</copyright-holder><copyright-holder xml:lang="en">de Vries E., Cardona A.A., Abdul Latiff A.H., Badolato R., Brodszki N., Cant A.J., Carbone J., Casper J.T., Ciznar A., Cochino A.V., Derfalvi B., Driessen G.J., Elfeky R., El-Ghoneimy D., Espanol T., Etzioni A., Gambineri E., Gilmour K., Gonzalez-Granado L.I., Guseva M.N., Haverkamp M.H., Helminen M., Honig M., Kanariou M.G., Kirschfink M., Klein C., Kuijpers T.W., Kutukculer N., Martire B., Meyts I., Niehues T., Pignata C., Reda S.M., Renner E.D., Rezaei N., Rizzi M., Sampalo Lainz M.A., Sargur R.B., Sediva A., Seidel M.G., Seneviratne S.L., Soler-Palacin P., Tommasini A., Warnatz K.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://www.mimmun.ru/mimmun/article/view/665">https://www.mimmun.ru/mimmun/article/view/665</self-uri><abstract><p>Резюме. Члены Европейского общества иммунодефицитов (ESID) и другие коллеги обновили многоступенчатый экспертный диагностический протокол для неиммунологов, включающий недавно описанные первичные иммунодефициты (ПИД). Протокол, представленный здесь, стремится привлечь внимание к ПИД врачей, работающих в различных областях. Своевременная диагностика ПИД важна для прогноза течения заболевания, хотя она может оказаться нелегкой задачей. Протокол начинается с клинической картины, наблюдаемой у пациента. Поскольку ПИД могут присутствовать во всех возрастных группах, данный протокол предназначен как для терапевтов, так и для детских врачей. Многоступенчатый характер протокола позволяет провести малозатратный скрининг по выявлению ПИД у большого числа подозреваемых на наличие ПИД на ранних стадиях, оставляя более дорогие исследования для дифференциальной диагностики таких состояний у пациентов в сотрудничестве со специалистом-иммунологом в области иммунной недостаточности на следующих стадиях.</p></abstract><trans-abstract xml:lang="en"/><kwd-group xml:lang="ru"><kwd>диагностический протокол</kwd><kwd>иммунологическое обследование</kwd><kwd>первичные иммунодефициты</kwd></kwd-group><kwd-group xml:lang="en"><kwd>diagnostic protocol</kwd><kwd>immunological evaluation</kwd><kwd>primary immunodeficiency</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">De Vries E., Clinical Working Party of the European Society for Immunodeficiencies (ESID). 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