[The metaraminol provocation test in the diagnosis of familial Mediterranean fever].
Familial Mediterranean Fever (FMF) is a cyclic inflammatory disease of unknown pathogenesis and autosomal recessive inheritance. Diagnosis is notoriously difficult by the lack of specific signs or laboratory tests although early diagnosis is mandatory to avoid developmental delay or possibly fatal amyloidosis by treatment with colchicine. In adults, Metaraminol Provocative Test (MPT) has been described as specific and highly sensitive in the diagnoses of FMF. We tested 18 children, 9 of whom suffered from FMF. They were ill for 5 years and had been treated as in-patients for 3 months without improvement (median values). 5 of the children with FMF had a positive test results. 4 children with FMF were negative. All 9 children with other disorders were negative, too. During the course of diagnosis, they had been suspected of suffering from FMF. So, in childhood, MPT is specific for FMF but does not identify all children with FMF. However, a positive MPT may be a great help in diagnosing FMF.
Huppertz
H I
HI
Universitäts-Kinderklinik, Würzburg.
Michels
H
H
D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D008680:Metaraminol
D008680:Metaraminol; D003078:Colchicine
Der Metaraminol-Provokationstest in der Diagnostik des Familiären Mittelmeerfiebers.