Dysregulace imunitního systému u pacientů s běžným variabilním imunodeficitem

• Název práce v češtině: Dysregulace imunitního systému u pacientů s běžným variabilním imunodeficitem
• Název v anglickém jazyce: Dysregulation of immune system in the patients with Common Variable Immunodeficiency
• Klíčová slova: běžný variabilní imunodeficit, asthma bronchiale, genetické vyšetření, nádorová onemocnění, imunoglobulinová substituční terapie
• Klíčová slova anglicky: Common variable immunodeficiency , bronchial asthma, genetics, malignancies, immunoglobulin substitution therapy
• Akademický rok vypsání: 2015/2016
• Typ práce: disertační práce
• Jazyk práce: čeština
• Ústav: Ústav imunologie (13-722)
• Vedoucí / školitel: doc. MUDr. Rudolf Horváth, Ph.D.
• Řešitel: skrytý - zadáno a potvrzeno stud. odd.
• Datum přihlášení: 28.12.2015
• Datum zadání: 28.12.2015
• Datum potvrzení stud. oddělením: 28.12.2015
• Datum a čas obhajoby: 14.09.2020 13:00
• Datum odevzdání elektronické podoby: 17.03.2020
• Datum odevzdání tištěné podoby: 23.03.2020
• Datum proběhlé obhajoby: 14.09.2020
• Oponenti: prof. MUDr. Ilja Stříž, CSc.

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Předběžná náplň práce

Tato disertační práce je koncipována jako soubor experimentálních prací publikovaných v rámci projektů řešených na Ústavu imunologie 2. lékařské fakulty Univerzity Karlovy. Společným jmenovatelem zahrnutých prací je bazální a aplikovaný výzkum primárních imunodeficitů (PID) se zaměřením na běžný variabilní imunodeficit (CVID).
První, teoretická část je rozdělena do dvou sekcí. První sekce je věnována obecné charakteristice PID protilátkového typu. Druhá sekce se zaměřuje na CVID se zaměřením na epidemiologické, etiopatogenetické, klasifikační, klinické a terapeutické aspekty onemocnění. Pozornost je věnována zejména neinfekčním komplikacím, které se významně podílejí na celkové mortalitě a morbiditě pacientů.
Druhou část disertační práce tvoří soubor vlastních publikovaných prací, které se věnujíidentifikaci konkrétních mechanismů dysregulace imunitního systému a jejich klinickýmprojevům u CVID a jsou opatřeny stručným komentářem. Spektrum publikovaných prací se dotýká čtyř základních studovaných aspektů CVID: 1) charakteru plicních komplikací, především z pohledu bronchiálního astmatu, 2) charakteru asociovaných malignit, 3) významu genetického pozadí a jeho vztahu ke specifické terapii, 4) terapii se zaměřením na imunoglobulinovou substituci.
Experimentální práce, které do koncepce disertační práce nezapadají, a jiné typy publikací, např. přehledné články a články z českých neimpaktovaných, recenzovaných časopisů, jsou uvedeny na konci v seznamu publikované literatury.

Předběžná náplň práce v anglickém jazyce

This thesis includes set of published experimental results, which were obtained at the Department of Immunology, Second Faculty of Medicine Charles University within the project focused on the basal and applied research of the Primary immunedeficiencies (PID), particularly Common variable immunodeficiency (CVID).
The first, theoretical part, is divided into two sections. The first section is dedicated to the general aspects of Primary antibody deficiencies (PAD). The second section is focused on epidemiology, ethiopathogenesis, classification, clinical and therapeutical aspects of CVID. The main consideration is devoted particularly to non-infectious complications, which significantly contribute to morbidity and mortality of CVID patients.
The second part consists of the set of publications describing specific mechanisms of immune system dysregulation and their clinical manifestation, which are briefly commented. The spectrum of issues resolved within the project covers following basic aspects: 1) characteristics of lung complications in CVID from the point of view of bronchial asthma, 2) characteristics of associated malignancies, 3) significance of genetic background for the specific treatment, 4) therapy of CVID focused on aspects of immunoglobulin substitution.
The results of the other experimental projects, which are not consistent with this thesis, and other publications including review articles or articles published in the Czech peer reviewed journals (without impact factor) are mentioned in the end in the list of publications.