ZINSSER COLE ENGMAN SYNDROME
\zˈɪnsə kˈə͡ʊl ɛnɡmˈan sˈɪndɹə͡ʊm], \zˈɪnsə kˈəʊl ɛnɡmˈan sˈɪndɹəʊm], \z_ˈɪ_n_s_ə k_ˈəʊ_l ɛ_n_ɡ_m_ˈa_n s_ˈɪ_n_d_ɹ_əʊ_m]\
Definitions of ZINSSER COLE ENGMAN SYNDROME
Sort: Oldest first
A predominantly X-linked recessive syndrome characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. Oral and dental abnormalities may also be present. Complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. (from Int J Paediatr Dent 2000 Dec;10 (4):328-34) The X-linked form is also known as Zinsser-Cole-Engman syndrome and involves the gene which encodes a highly conserved protein called dyskerin.
By DataStellar Co., Ltd
Word of the day
Dorsal Root Ganglia
- ganglia located on dorsal roots within vertebral column. ganglion cells are pseudounipolar. single primary bifurcates sending peripheral process to carry sensory information from the periphery and a central branch which relays that spinal cord or brain.