TY - CHAP
T1 - Special Aspects of Familial Mediterranean Fever in Childhood
AU - Kallinich, Tilmann
AU - Aktay, Nuray
AU - Ozen, Seza
N1 - Publisher Copyright:
© 2015, Springer International Publishing Switzerland.
PY - 2015
Y1 - 2015
N2 - FMF is a disease, which usually manifests in early childhood: in 90 % of cases, first symptoms are present before the 20th year of life and in 75 % before the 10th year of life [1] (Table 3.1). Immediate postnatal manifestations are reported [2]. A late onset of disease, defined as manifestation after the 40th birthday, is rare and only be present in less the 1 % of patients [3]. In the large Turkish registry, the mean age of manifestation was 9.6 years, the mean age of diagnosis 16.4 years [4]. But the general awareness for the diseases as well as the better diagnostical tools improved the early diagnosis dramatically in the last decades [5].
AB - FMF is a disease, which usually manifests in early childhood: in 90 % of cases, first symptoms are present before the 20th year of life and in 75 % before the 10th year of life [1] (Table 3.1). Immediate postnatal manifestations are reported [2]. A late onset of disease, defined as manifestation after the 40th birthday, is rare and only be present in less the 1 % of patients [3]. In the large Turkish registry, the mean age of manifestation was 9.6 years, the mean age of diagnosis 16.4 years [4]. But the general awareness for the diseases as well as the better diagnostical tools improved the early diagnosis dramatically in the last decades [5].
KW - Autoinflammatory Disease
KW - Colchicine Treatment
KW - Familial Mediterranean Fever
KW - MEFV Gene
KW - Posterior Reversible Leukoencephalopathy Syndrome
UR - https://www.scopus.com/pages/publications/85060491312
U2 - 10.1007/978-3-319-14615-7_3
DO - 10.1007/978-3-319-14615-7_3
M3 - Chapter
AN - SCOPUS:85060491312
T3 - Rare Diseases of the Immune System
SP - 31
EP - 45
BT - Rare Diseases of the Immune System
PB - Springer Nature
ER -