Marshallov sindrom – izazov u kliničkoj praksi

Marshall syndrome (periodic fever, adenitis, pharyngitis, aphthae, PFAPA syndrome) is characterized by recurrent episodes of fever associated with aphthous stomatitis, cervical adenitis or pharyngitis. Although it is the most common cause of recurrent fever in children, the diagnosis is rarely established. The aim of this study was to describe a group of Romanian children with Marshall syndrome. In seven children with PFAPA, the following methods were used: patient history, clinical examination, and determination of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and tumor necrosis factor α (TNF-α). The treatment consisted in prednisone at a dose of 1 mg/kg. The age at onset was 2.2 years and at diagnosis 4.8 years. The mean interval between episodes was 3.1 weeks and the duration per febrile episode was 3.7 days. The patients presented with pharyngitis (100%), adenitis (100%) and aphthous lesions (57.1%). The mean ESR value was 31 mm/h, CRP 7.8 mg/dL and leukocytes 17700/mm3 . TNF-α remained elevated between febrile episodes. Six patients treated with prednisone had favorable evolution. In conclusion, PFAPA should be suspected in children with periodic fever associated with pharyngitis, cervical adenitis and aphthous stomatitis.Marshallov sindrom (periodična groznica, adenitis, faringitis, afte, sindrom PFAPA) obilježen je opetovanim epizodama groznice udružene s aftoznim stomatitisom, cervikalnim adenitisom ili faringitisom. Iako je to najčešći uzrok opetovane groznice u djece, ova se dijagnoza rijetko postavlja. Cilj ovoga ispitivanja bio je opisati skupinu rumunjske djece s Marshallovim sindromom. U sedmoro djece s PFAPA primijenjene su sljedeće metode: anamneza, klinički pregled te određivanje sedimentacije eritrocita (SE), C-reaktivnog proteina (CRP) i faktora tumorske nekroze alfa (TNF-α). Liječenje je provedeno prednisonom u dozi od 1 mg/kg. Dob pri nastupu PFAPA bila je 2,2 godine, a pri dijagnozi 4,8 godina. Srednja vrijednost intervala među epizodama PFAPA bila je 3,1 tjedan, a trajanja pojedine febrilne epizode 3,7 dana. Bolesnici su imali faringitis (100%), adenitis (100%) i aftozne promjene (57,1%). Srednja vrijednost SE bila je 31 mm/h, CRP 7,8 mg/dL i leukocita 17700/mm3 . TNF-α je ostao povišen između febrilnih epizoda. Povoljan ishod zabilježen je u šestoro bolesnika liječenih prednisonom. U zaključku, na PFAPA treba posumnjati u djece s periodičnim groznicama udruženim s faringitisom, cervikalnim adenitisom i aftoznim stomatitisom