Sterian A, Balanescu R, Barbilian A, Tevanov I, Carp M, Nahoi C, Barbu M, Ulici A
"Grigore Alexandrescu" Clinical Emergency Hospital for Children, Bucharest, Romania ; "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania ; "Dr. Carol Davila" Central Military University Emergency Hospital, Bucharest, Romania.
J Med Life. 2015 Oct-Dec;8(4):544-7.
Osteogenesis imperfecta is a genetically determined pathology that implies bone variability and osteoporosis with early onset of fractures after low energy trauma. For a better understanding of the clinical problems, Sillence and Danks created a classification. The study group consisted of 12 patients both males and females, with ages ranging from 2 years and 3 months to 12 years. All of them came to the hospital late, after walking, after several fractures occurred and the only treatment they underwent was with prolonged cast immobilization that caused rapid bone demineralization, axial deformations of the affected bones, increased number of fractures and eventually loss of ambulation. Following the discharged patients, we appreciated that the open bone alignment and Fassier Duval osteosynthesis were the best way to treat a patient with Lobstein disease. The results showed that by using these two techniques a lot of time is saved on a long term because all the great complications associated with older techniques are gone and a rapid ambulation is possible due to the soft tissue damage that is kept to a minimum.
成骨不全症是一种由基因决定的病理状况,表现为骨骼变异性和骨质疏松,在低能量创伤后早期即发生骨折。为了更好地理解临床问题,西伦斯和丹克斯创建了一种分类方法。研究组由12名患者组成,男女皆有,年龄从2岁3个月到12岁不等。他们都是在开始行走后、发生多次骨折后才很晚才到医院,并且他们接受的唯一治疗是长时间的石膏固定,这导致了快速的骨质流失、患骨的轴向畸形、骨折数量增加,最终失去行走能力。在对出院患者进行随访后,我们认识到开放的骨骼对线和法西耶 - 杜瓦尔接骨术是治疗洛布斯坦病患者的最佳方法。结果表明,从长远来看,使用这两种技术可节省大量时间,因为与旧技术相关的所有重大并发症都已消除,并且由于软组织损伤保持在最低限度,患者能够迅速恢复行走。