Definition:
ICF Syndrome is a condition characterized by a weak immune system and anomalies in the face. ICF Syndrome is an inherited condition. The main feature of ICF Syndrome is variable immunodeficiency combined with centrometric instability of chromosome 1, 9, 16 and even 2. ICF syndrome is a rare condition that affects less than 200,000 people in the United States.
Diagnosis:
ICF Syndrome is diagnosed through a decrease in the level of serum IgM, IgA and IgG along with normal circulating B-cell count as detected by a processes called immunoelectrophoresis. The condition is confirmed through antigenic stimulation. Chronic lung disease or sinusitis as detected by x ray is also a sign.
Treatment:
The main treatment for ICF Syndrome is repeated infusions of immunoglobulin. If there is vitamin A deficiency then vitamin A supplements are given to the patient. Vitamin A is also effective in preventing ICF diseases in cases where vitamin A is deficient.
Symptoms and Signs:
The main symptoms of ICF Syndrome include retarded growth, unusual facial appearance, retardation of psychomotor facilities, failure to thrive and low IgG serum level. Less common symptoms include umbilical hernia, wide set eyes, low set ears, protruding tongue and a flat face.
Causes:
ICF Syndrome can be caused by Bloom's syndrome, Non-Hodgkin's lymphoma, Chronic granulomatous disease, Efalizumab and Autoimmune polyendocrine syndrome type 1.
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