Hoppe-Tichy T, Nguyen T H, Hentze B W, Lorke M
Apotheke des Universitätsklinikums, Technische Universität Braunschweig, Germany.
Pharmazie. 2005 Mar;60(3):205-7.
Menkes' Kinky Hair Syndrom is a rare, X-linked recessive multisystemic lethal disorder of copper metabolism. Male infants who are affected usually die at the age of 2-3 years. If the disease is diagnosed early, patients profit from subcutaneously administered copper salts. We describe the preparation and stability of a copper-histidin solution. This solution has some advantages in terms of stability over the solutions described in earlier publications. This is a great advantage for the patients and their parents, because an ambulatory care or a home care of this patients is possible now.
门克斯卷发综合征是一种罕见的、X连锁隐性遗传的铜代谢多系统致死性疾病。患病男婴通常在2至3岁时死亡。如果疾病能早期诊断,患者可从皮下注射铜盐中获益。我们描述了一种铜-组氨酸溶液的制备及稳定性。该溶液在稳定性方面比早期出版物中描述的溶液具有一些优势。这对患者及其父母来说是一个很大的优势,因为现在对这些患者进行门诊护理或家庭护理成为可能。