ANTIFOSFOLIPIDNI SINDROM KOD DECE

  • Gordana Petrovic pedijatar
  • Srđan Pašić profesor, pedijatar, imunolog
Ključne reči: antifosfolipid syndrome, antiphospholipid syndrome, systemic lupus erythematosus, children

Sažetak


Antifosfolipidni sindrom je multisistemska autoimunska bolest koju karakteriše pojava arterijskih i/ili venskih tromboza, često multiple lokalizacije, trombocitopenija i ponavljani spontani abortusi obolelih trudnica, usled permanentnog prisustva antifosfolipidnih antitela. Može se javiti i kod dece i ispoljiti u bilo kom periodu detinjstva, od novorođenačkog doba, usled transplacentarnog prelaska majčinih, ili de novo produkcije autoantitela, do adolescencije. Kliničke manifestacije opisane kod odraslih mogu se javiti i kod dece ali su modifikovane karakteristikama uzrasta, kao što su nezrelost imunskog ili drugih sistema organa, veća izloženost virusnim i bakterijskim infekcijama, sprovođenje rutinske imunizacije, odsustvo za odrasle uobičajenih trombogenih faktora rizika (hipertenzija, arterioskleroza, konzumiranje cigareta, alkohola i/ili oralnih kontraceptiva), kao i odsustvo trudnoće. Antifosfolipidna antitela najčešće korišćena u svakodnevnom kliničkom radu su antikardiolipinska antitela, anti-b2 glikoprotein I antitela i lupus antikoagulant. Patogeni mehanizmi nisu detaljno istraženi, pretpostavlja se da su slični adultnom obliku bolesti. Međutim, postoje retki dokazi o postojanju i specifičnih obrazaca imunološkog odgovora na pojedine antigenske komponente, što  može dovesti do specifične proizvodnje antifosfolipidnih antitela tokom detinjstva. Važan je i podatak da se ova antitela mogu naći i kod oko 25% zdrave dece. Za dijagnostikovanje antifosfolipidnog sindroma kod odraslih primenjuju se Sapporo kriterijumi. Obuhvataju kliničke (vaskularne tromboze, ponavljani gubici ploda) i laboratorijske parametre (perzistentno prisustvo jednog od antifosfolipidnih antitela) u IgG i /ili IgM klasi, u srednjem ili visokom titru. Ažurirani Sapporo kriterijumi primenjuju se i kod dece. Podrazumevaju iste laboratorijske i kliničke kriterijume, osim patologije trudnoće. Diferencijalna dijagnoza je veoma široka i zahteva opsežnu dijagnostiku. S obzirom specifičnosti bolesti u pedijatrijskom uzrastu, preporuke koje važe za odrasle, u određenom stepenu modifikovane, primenjuju  za lečenje bolesti kod dece.

Biografija autora

Srđan Pašić, profesor, pedijatar, imunolog

Načelnik odeljenja za kliničku imunologiju i alergologiju

Reference

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2023/08/23
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