A recently recognised chronic inflammatory disease of early onset characterised by the triad of rash, central nervous system involvement and arthropathy.

نویسنده

  • A M Prieur
چکیده

Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome, also called Neonatal Onset Multisystemic Inflammatory Disease (NOMID) is characterised by the triad of cutaneous rash, chronic meningitis and arthropathy. It is a chronic inflammatory illness that starts most often at birth and persists for the whole lifespan of the patient. Attempts at therapy have been disappointing. The long-term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations. Some cases of death have been reported secondary to infection, vasculitis and amyloidosis. Usually observed as sporadic cases, some familial association is recognised.

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

گزارش یک مورد اسپاسم شیرخوارگی در زمینه تو بروس اسکلروزیس

ABSTRACT Tuberous Sclerosis(T.S) is an inherited disease (with autosomal dominant trait). It is characterised by different skin manifestations involvement of central nervous system and hamartomas in different organs of the body .Of Course ,about 10% of cases are due to new mutations Presentation of this patient, a 4-month-old male infant with recurrent convulsions cflexor type infantile spasm ...

متن کامل

P 153: Neuroinflammation in Multiple Sclerosis

Multiple sclerosis (MS) is a complex disease which is correlated with increasing inflammatory factors, demyelination and axonal loss. In this auto-immune disease, Neuroinflammation is mediated by different types of T cells with macrophage/microglial activation and B cells involvement that interact in a collaborative manner. Focal inflammation is the main cause for the onset of relapses and coul...

متن کامل

O 23: Anti-Inflammatory Treatment in Children with Refractory Seizure

Epilepsy is a neurological disease of the central nervous system. It is estimated that about 50-70 million people worldwide suffer from this chronic disorder and 20 to 30% are resistant to conventional anti-epileptic drugs. In the epilepsy therapeutic arena, there is real need for developing novel antiepileptogenesis treatments that offer a way to prevent the onset or the progression of the dis...

متن کامل

Electrodiagnostic study of peripheral nervous system in Behcet’s disease

Introduction: Bechcet’s disease (BD) is a chronic disease with multiple organ involvement such as central nervous system (CNS) and peripheral nervous system (PNS). Involvement of PNS has been reported controversially and uncommon in textbooks and literatures. The aim of this study in to evaluate the auxiliary role of electrodiagnosis in early detection of PNS involvement to prevent neuropathy...

متن کامل

Fabry dissase from the dentist view

                 Fabry disease is a rare, inherited disease with lack of the enzyme alpha-galactosidase A (α-Gal)  in the cells of the body that participates in the breakdown of fat. The disease begins in early childhood, progresses slowly throughout life and results in severe damage of the kidneys, heart and central nervous system. The disease is life-threatening and if left untreated, death ...

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:
  • Clinical and experimental rheumatology

دوره 19 1  شماره 

صفحات  -

تاریخ انتشار 2001