Banjar Hanaa, Angyalosi Gerhild
Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Novartis Pharma AG, Basel, Switzerland.
Int J Pediatr Adolesc Med. 2015 Jun;2(2):47-58. doi: 10.1016/j.ijpam.2015.05.006. Epub 2015 Jun 19.
Cystic fibrosis (CF) is a lethal, monogenic disorder that affects multiple organ systems of the body. The incidence has been described before in the Middle East to be 1 in 2000 to 1 in 5800 live births, and the median survival was estimated to be from 10 to 20 years of age. The present article attempts to revisit various facets of this disease and specifically highlights the most important lacunae that exist in treating CF. In addition, it also tries to emphasize the steps in improving the median survival of patients with CF, in these countries.
囊性纤维化(CF)是一种致命的单基因疾病,会影响身体的多个器官系统。此前在中东地区,其发病率据描述为每2000至5800例活产中有1例,预计中位生存期为10至20岁。本文试图重新审视该疾病的各个方面,并特别强调在治疗CF方面存在的最重要的缺陷。此外,它还试图强调在这些国家提高CF患者中位生存期的步骤。