The etiopathogenesis of SIDS is not well know so far and it is assumed that it is a heterogeneous clinical syndrome. Several risk factors are know which may lead to the development of SIDS, however, none of them alone causes sudden death. Impaired fatty acid oxidation (in particular medium chain fatty acids - MCAD) can have a similar clinical picture and fatal outcome as SIDS. According to data in the literature the ratio of MCAD in SIDS is not higher than 3-4%. In a group of 24 hitherto comprehensively examined families (incl. polysomnography and PCR) with the incidence of SIDS the ratio of MCAD was 12.5%. The authors draw attention to the clinical picture of impaired beta-oxidation of fatty acids, possible diagnosis and prevention of these conditions. Comprehensive care of children from families with a positive case-history can prevent not only a fatal outcome but also long-term negative sequelae in these children.

        Key words: SIDS - MCAD - PCR - ALTE.