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CINCA Syndrome
(Chronic, Infantile, Neurologic, Cutaneous and Articular syndrome)
aka/or
NOMID

Excerpted from article available at:
http://pw1.netcom.com/~jkbarton/nomid/Html/FAQs.htm


What is it?
A very rare disorder (54 cases reported in literature to 1997) involving a constellation of inflammatory diseases including which may include some of the following:

  • Rash that comes and goes, throughout the body (first obvious sign, from birth.   Distinguishes NOMID from JRA where the rash starts later)
  • Swelling of the optic nerves
  • Rheumatoid arthritis type complaints, typically starting in the knees 
  • meningitis  
  • intermittent spiking fever
  • hearing and sight loss
  • mental retardation
  • stunted growth
  • large head, saddle-back nose and general angelic appearance
  • and others
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Symptoms vary from child to child

How is it caused?
Not clear.  Current ideas include infection in 3rd trimester of pregnancy, or some genetic cause.   Many parents here report nothing exceptional during pregnancy and there are a few families with more than one children with NOMID

Cures? How is it treated?
No cures.  Treatment typically includes steroids which effectively reduce some of the inflammatory symptoms.  However, see personal stories for other drugs used which we have found helpful for our children.  Click here to learn more about a US government sponsored study to '.....examine and test patients with neonatal onset multi-system inflammatory disease (NOMID) to learn more about the cause and course of the disease. It will study the disease signs and symptoms and the possible role of a gene called CIAS1, and it will develop a database to gather information on patients with NOMID in the United States and around the world. It will also serve as a screening protocol to offer eligible patients participation in a treatment protocol, if an appropriate one is available'

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Not to be confused with...
Not to be confused with Juvenile Rheumatoid Arthritis, which has many of the same symptoms.  However, 'systemic-onset JRA is rarely observed in the frist year of life and orbital inflammation is exceptional.  Central nervous system involvement in systemic-onset JRA is uncommon.....The character of IOMID arthopathy differs significatnly from that of JRA'. (Hashkes, J in J. Pedriatrics 1997, 130:513-5)




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NOMID/CINCA References:

Hashkes PJ, Lovell DJ. Recognition of infantile-onset multisystem inflammatory disease as a unique entity. J Pediatr. 1997 Apr;130(4):513-5.  PMID: 9108844

Sadiq SA, Gregson RM, Downes RN. The CINCA syndrome: a rare cause of uveitis in childhood. J Pediatr Ophthalmol Strabismus. 1996 Jan-Feb;33(1):59-63. PMID: 8965228

Miura M, Okabe T, Tsubata S, Takizawa N, Sawaguchi T. Chronic infantile neurological cutaneous articular syndrome in a patient from Japan. Eur J Pediatr. 1997 Aug;156(8):624-6. PMID: 9266194


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