Author/Editor     Tedesco, Francesco
Title     Complement deficiencies and infectious diseases
Translated title     Pomanjkljivosti sistema komlementa in nalezljive bolezni
Type     članek
Source     Med Razgl
Vol. and No.     Letnik 39, št. Suppl 4
Publication year     2000
Volume     str. 101-7
Language     eng
Abstract     In the last three decades several hundred individuals with inherited deficiencies of components of the complement system have been recognized mostly in Western Countries and in Japan and, more recently, in other areas as a result of a more widespread use of screening assays for this biological system. A retrospective analysis of a substantial number of these individuals has led Densen and associates in the States to critically evaluate the type of diseases that are more often associated with the deficiencies. A clear picture has emerged showing that patients with inherited defects of components of the classical pathway suffer more often from lupus-like syndromes whereas individuals with deficiencies of all other components, including those of the alternative and terminal pathways, experience bacterial infections often presenting as recurrent episodes. This type of infections also occur in patients with deficiencies of early components, particularly of C2, though with a lower frequency. The bacterial strains that are more often responsible for infections in patients with deficiencies of all but the terminal complement components include Streptococcus pneumoneae, Haemophil s influenzae and Neisseria meningitidis. Conversely, the infections occurring in patients with deficiencies of the terminal components and properdin are caused almost exclusively by Neisseria meningitidis. However, the latter two groups of patients differ in the outcome of the meningoccal disease, which may be fatal in properdin deficient patients while tends to have a mild course and is recurrent in patients with terminal components deficiencies. Although the infecting meningocci often belong to rare serogroups (X, Y, W-135), common serogroups (B and C) may also cause the disease, particularly in patients living in Mediterranean areas. (Abstract truncated at 2000 characters).
Summary     V zadnjih treh desetletjih so odkrili več sto bolnikov s podedovanimi pomankljivostmi sestavin komplementnega sistema. Bolniki so predvsem iz dežel zapadne hemisfere in Japonske, v zadnjem času, ko so postale dosegljive presejalne metode za ugotavljanje dejavnosti komplementnega sistema, pa tudi v drugih deželah. Densen s sodelavci se je pred kratkim lotil retrospektivne študije s katero je raziskoval, katere bolezni so pogosto povezane z okvarami komplementnega sistema. Tako se pri bolnikih, ki imajo okvarjeno klasično pot aktivacije komplementnega sistema pogosteje pojavljajo lupusu eritematozusu podobni sindromi, pri bolnikih z okvarami drugih sestavin, alernativne poti in tvorbe litičnega kompleksa pa ponavljajoče se bakterijske okužbe. Izjema je okvara sestavine C2, ki je tudi pretežno povezana z bakterijskimi okužbami. Bakterije, ki povzročajo okužbe pri bolnikih z okvarjeno klasično, alternativno in manansko potjo, ne pa z okvarami v terminalni fazi aktivacije komplementa, so: Strepococcus pneumoniae, Haemophilus influenzae in Neisseria meningitidis. Pri bolnikih z okvarami tvorbe terminalnega kompleksa in properdina pa pride praktično vedno do okužbe z Neuseria meningitidis. Pri bolnikih z okvaro properdina so okužbe težke in se pogosto končajo s smrtjo, medtem ko so pri bolnikih z okvarami končne faze komplementne aktivacije okužbe lažje, vendar se pa ponavljajo. Neiserije, ki povzročajo navedene okužbe, sodijo v nenavadne serološke skupine, kot so X, Y, W-135, v Sredozemlju pa sta pogosta tudi serotipa B in C. Nadzor nad navedenimi okužbami opravljajo s profilaktičnim cepljenjem. Pri okvarah properdina in terminalne faze uporabljajo meningokokno cepivo, ki ji pri drugih okvarah komplementa dodajo še pnevmokokno in hemofilusno cepivo. Kdaj cepiti, katera cepiva in načini s katerimi bi lahko učinkovito izvedli in merili zaščitno moč cepljenja, pa so pomembni dejavniki, ki še niso povsem dognani.
Descriptors     IMMUNOLOGIC DEFICIENCY SYNDROMES
INFECTION
COMPLEMENT
COMPLEMENT PATHWAY, ALTERNATIVE
COMPLEMENT PATHWAY, CLASSICAL
COMPLEMENT 3C