نتایج جستجو برای: hereditary periodic fever syndromes
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Most children with prolonged, recurrent, or periodic fever are healthy and have self-limited, common illnesses, and the primary care practitioner usually can reassure families and continue to reassess the patient as circumstances dictate. For a child with true fever of unknown origin, a pediatric infectious diseases subspecialist should be consulted. This article discusses three objectives for ...
Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease in the world. It shows a high prevalence in countries bordering the eastern Mediterranean. The course of the disease is marked by short and self-limiting bouts of fever, often with a monthly periodicity; other clinical manifestations include serositis and synovitis. Mutations of the cold-induced autoinflam...
Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease in the world. It shows a high prevalence in countries bordering the eastern Mediterranean. The course of the disease is marked by short and self-limiting bouts of fever, often with a monthly periodicity; other clinical manifestations include serositis and synovitis. Mutations of the cold-induced autoinflam...
BACKGROUND Several studies from Western countries have analyzed the profile of pediatric rheumatology practices. Due to differences in demography and health care systems the profile in Israel may differ from those countries. OBJECTIVE To describe the profile of a pediatric rheumatology practice in Israel. METHODS All new patients seen during the course of 2000 as part of my pediatric rheuma...
Introduction PFAPA syndrome is the most common autoinflammatory fever disorder in childhood, characterized by recurrent fever, aphthous stomatitis, pharyngitis and adenitis. Mutations in the MEFV and NLRP3 genes are known to cause syndromes with PFAPA overlapping symptoms (Familial Mediterranean Fever and Cryopyrin-Associated Periodic Syndrome), which are rarely reported in patients from Slovenia.
Methods A web-based registry collecting baseline and crosssectional clinical information on Autoinflammatory diseases is available in the member area of the PRINTO web-site (www.printo.it). The registry is open to all pediatric and adult Centers with a specific interest in Autoinflammatory diseases. The following monogenic autoinflammatory diseases were considered: Familial Mediterranean Fever ...
Inflammasomes are multiprotein complexes that critically control different aspects of innate and adaptive immunity. Among them we could highlight the release of pro-inflammatory cytokines that induce and maintain the inflammatory response. Usually, inflammasomes result from oligomerization of a nucleotide-binding domain-like receptor (NLR) after sensing different pathogenic or endogenous steril...
AIM To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever (UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever score can help predicting the response to treatment and the outcome at follow-up. METHODS Clinical and therapeutic information of patients with recurrent fever who presented at a single pedia...
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