HUNTER SYNDROME GARGOYLISMS
\hˈʌntə sˈɪndɹə͡ʊm ɡˈɑːɡɔ͡ɪlˌɪzəmz], \hˈʌntə sˈɪndɹəʊm ɡˈɑːɡɔɪlˌɪzəmz], \h_ˈʌ_n_t_ə s_ˈɪ_n_d_ɹ_əʊ_m ɡ_ˈɑː_ɡ_ɔɪ_l_ˌɪ_z_ə_m_z]\
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Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15.
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Platidiam
- An inorganic water-soluble platinum complex. After undergoing hydrolysis, it reacts DNA produce both intra interstrand crosslinks. These crosslinks appear to impair replication and transcription of DNA. The cytotoxicity cisplatin correlates with cellular arrest in G2 phase cell cycle.