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Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases.
Caption: Figure 1 This child suffers from congenital ichthyosis and presented with exposure keratopathy.
Harlequin ichthyosis is a rare form of congenital ichthyosis with an overall incidence of 1 in 300,000 births (3).
It is due to mutation of certain genes and is usually an autosomal recessive, congenital ichthyosis ( scaly skin condition).
The pathogenesis of severe congenital ichthyosis of the neonate.
Chanarin-Dorfman syndrome (CDS) is a very rare autosomal recessive inherited neutral lipid metabolism disorder associated with congenital ichthyosis and multi-system involvement.
Fischer J: Autosomal recessive congenital ichthyosis.
Congenital ichthyosis is a phenotypic expression of several different genotypes, and presents with varying degrees of severity.
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