Behçet Disease (BD) in two siblings affected with Familial Mediterranean Fever (FMF)

نویسندگان

  • G Calcagno
  • A Vitale
  • F La Torre
  • N Decembrino
  • C Fede
  • F Falcini
چکیده

Their medical history of recurrent fever attacks started at the age of 14 and 6 months respectively. Fever was accompanied by cervical adenopathy, severe exudative pharyngitis, oral aphtosis, abdominal pain and diarrhoea, sometimes with bloody stools. At the age of 10 and 9 years, screening for autoinflammatory disorders was performed revealing a single mutated FMF gene (E148Q). Since they fulfilled Tel Hashomer criteria, colchicine was started.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

FAMILIAL MEDITERRANEAN FEVER: A STUDY OF 32 CASES

From April 1983 to September 1990, 32 patients with familial mediterranean fever (FMF) were studied. FMF is characterized by short, self-limited, febrile episodes that occur with inflammation of serosal surfaces. Major symptoms include fever and abdominal pain, presenting as acute surgical abdomen. These attacks are associated with considerable morbidity and in some patients lead to unnece...

متن کامل

PReS-FINAL-2223: The relationship between FMF, BD and epilepsy

Introduction Familial Mediterranean Fever(FMF) is an autoinflammatory disease particularly frequent around the Mediterranean basin. Behçet disease (BD) is a autoinflammatory disease which distribution is mainly around the Mediterranean Sea, Middle East and Far East.Epilepsy is a chronic disorder of the brain that affects people in every country of the world.It is characterized by recurrent seiz...

متن کامل

تب فامیلی مدیترانه دریک دوقلو و بیماری بهجت در مادر آنها

Familial Mediterranean fever (FMF) is a hereditary condition which is characterized by recurrent episodes of fever and abdominal pain. On the other hand, Behcet`s disease (BD) is an immune mediated condition typified by recurrent oral aphthous lesions, inflammatory eye disease and multiple organ involvement. Association of these two conditions is rare. We present a pair of twins with FMF and B...

متن کامل

'Silent' carriage of two familial Mediterranean fever gene mutations in large families with only a single identified patient.

The presence of two mutations in the familial Mediterranean fever gene, without overt familial Mediterranean fever (FMF), designated as phenotype III, predisposes to developing 'silent' AA amyloidosis, recognized as phenotype II, due to the absence of medical supervision and colchicine prophylaxis. We sought to determine the prevalence of phenotype III in large families with only one subject af...

متن کامل

The Risk of Familial Mediterranean Fever in MEFV Heterozygotes: A Statistical Approach

BACKGROUND Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder due to MEFV mutations and one of the most frequent Mediterranean genetic diseases. The observation of many heterozygous patients in whom a second mutated allele was excluded led to the proposal that heterozygosity could be causal. However, heterozygosity might be coincidental in many patients due t...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 6  شماره 

صفحات  -

تاریخ انتشار 2008