Familial Mediterranean fever in children

Familial Mediterranean fever (periodic disease) is the most common disease from the group of hereditary periodic fevers, which is caused by a mutation in the MEFV gene and is inherited in an autosomal recessive manner. Familial Mediterranean fever is most common among representatives of certain ethnic groups — Armenians, Turks, Sephardic Jews — in these populations, the incidence of Familial Mediterranean fever reaches 1:500. The clinical picture is characterized by periodic attacks of fever combined with abdominalgia, relieved within 12–72 hours. Aseptic inflammation of the serous membranes causes peritonitis, synovitis, pleurisy. Surgery for periodic disease can lead to the development of adhesive disease, and as a consequence — intestinal obstruction. The article presents clinical cases of periodic disease in children who received and or not receive colchicine therapy, including those with colchicine resistance.