OBJECTIVES To establish a normal range for the frontomaxillary facial (FMF) angle by three-dimensional (3D) ultrasound imaging and to examine the FMF angle in trisomy 21 fetuses at 16-24 weeks of gestation. METHODS We measured the FMF angle using 3D volumes of the fetal profile obtained with the transducer parallel to the long axis of the nose and at 45 degrees to the palate, which had been a...

BACKGROUND Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder, for which systemic AA amyloidosis is the major complication revealed most of the time by renal abnormalities. Current treatment is daily colchicine that prevents both recurrent inflammatory attacks and amyloidosis deposition in most patients. However, some patients still develop amyloidosis and re...

Familial Mediaterranian Fever (FMF) is an ethnically related genetic disease, characterized by spontaneously resolving episodes of fever and pain. Amyloidosis of the amyloid A type is the most important manifestation of FMF, affecting many organs, including the kidneys, adrenal glands, intestines, spleen, thyroid, heart, lungs and liver. There is an increasing concern about the association of n...

Methods We observed 2300 patients with FMF (1408 boys and 892 girls; mean age: 8.86±0.29) in the National Pediatric Centre for FMF. Diagnosis of FMF was based on Tel-Hashomer criteria and MEFV genetic analysis. The epilepsy was diagnosed based on clinical manifestations (>2 unprovoked epileptic seizures),neurological history, exam, EEG and MRI. The statistical analysis was performed using Epi-I...

Introduction A patient with familial Mediterranean fever (FMF) in addition to undiagnosed Anklosing Spondylitis (AS) and also having cutaneous leukocytoclastic vasculitis (LV) is presented. Coexistence of FMF with inflammatory disorders including spondyloarthritis and various systemic vasculitides has been reported before. Meanwhile this is the first reported case of FMF, AS, and LV present tog...

Background. Psoralen plus ultraviolet A (PUVA) is the preferred phototherapeutic modality for early-stage folliculotropic mycosis fungoides (FMF), and non-FMF refractory to narrow-band B (NBUVB). However, PUVA has a problematic safety profile. Literature on treatment with combination of UVA NBUVB MF sparse. Objective. To evaluate effectiveness combined MF, specifically FMF NBUVB-refractory non-...

familialmediterranean fever (fmf) is a hereditary syndrome characterized by recurrent attacks of fever and serositis. liver involvement in fmf has been reported in association with amyloidosis or rarely with vasculitis.in this report, a 32 year-old man with recurrent attacks of fever, abdominal pain and arthralgia is described who had moderate increase in liver transaminases only during disease...

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by episodic fever and inflammatory polyserositis, which could lead to a variety of manifestations, including recurrent abdominal pain. Herein, a 12-year-old boy who has suffered from fever and bloody diarrhea since early childhood is described. All structural and underlying disorders leading to bleeding we...

Background and question Colchicine is the gold standard treatment for prevention of inflammatory attacks and prevention of reactive amyloidosis in FMF. However, noncompliance to colchicine treatment is common among FMF patients. On the other hand, the disease may not be controlled in some patients despite use of full dose colchicine. In this study, we aimed to investigate the rates of disease c...

Background FMF is a systemic auto-inflammatory disorder, characterized by recurrent episodes of fever and serosal inflammation. The MEF gene, which is associated with FMF, encodes for the protein pyrin. FMF associated mutations, interrupt with pyrin normal function, leading to activation of the innate immune system and overexpression of IL-1b, and consequently to a systemic inflammatory respons...