电邮这篇文章 超声评估外固定装置固定期间患有软骨发育不良的患者胫骨再生体的结构状态
- 作者: Menschikova T.I.1, Aranovich A.M.1
-
隶属关系:
- Ilizarov National Medical Research Centre for Traumatology and Orthopedics
- 期: 卷 12, 编号 4 (2024)
- 页面: 427-436
- 栏目: Clinical studies
- URL: https://journals.rcsi.science/turner/article/view/282512
- DOI: https://doi.org/10.17816/PTORS637440
- ID: 282512
如何引用文章
开放存取
##reader.subscriptionAccessGranted##
订阅存取
详细
背景。在固定期间评估骨再生体的必要性在于，在此治疗期间，进行的临床活动旨在预防可能的并发症：评估肢体轴的正确性，明确延长肢体和对侧肢体的长度（在再次延长时），保持固定的刚度；功能性地控制术后肢体的负荷以及患者的运动活动。所有这些直接影响拉伸再生体的结构状态、 其成熟的时间以及是否准备好拆除外固定装置。由于比远端再生体（5.5 [5.0；6.0] cm）更大的延长量，近端再生体的研究尤为重要，且其成熟决定了外固定装置拆卸的时间。
研究目的。评估患有软骨发育不良的患者在固定期间不同年龄段的胫骨拉伸再生体的结构状态。
材料与方法。本研究使用日本AVISUS Hitachi超声设备，配备7.5 MHz频率的线性探头进行。使用标准程序评估再生体。对6-9岁（I组，n = 15）和10~15岁（II组，n = 15）的软骨发育不良患者进行了研究。研究在固定开始后的第5天、30天、60天和90天进行（对于再次延长肢体的患者）。在I组的单侧小腿骨延长时，延长量为6.5 [6；7] cm；而在I组和II组的双侧小腿延长时，近端再生体的延长量为5.5 [5.0；6.0] cm，远端为2.5 [2.0；3.0] cm。
结果。I组和II组的骨生成过程均表现出良好的进展，并且再生体区域的形成呈现典型周期性。 特征性差异在于II组再生体结构形成较慢，导致固定时间延长。因此，I组儿童的固定时间为55 ± 5天（p ≤ 0.05），而II组则为63 ± 3天（p ≤ 0.05）。唯一例外为1名患者（10名再次延长小腿的患者之一），该患者在拉伸过程中再生体中间区域出现了低回声囊性样病变。皮质板形成期延长至85 ± 5天（p ≤ 0.05）。
结论。在固定期间，超声评估显示，胫骨拉伸再生体的修复骨生成活动与拉伸期的修复骨生成活动相当。尽管由于母骨端的回声密集碎片，固定期间无法完全显示所获得的延长量，但超声检查可以评估再生体填充的动态、血管化特征以及再生体拆除外固定装置的准备情况。
作者简介
Tatyana I. Menschikova
Ilizarov National Medical Research Centre for Traumatology and Orthopedics
编辑信件的主要联系方式.
Email: tat-mench@mail.ru
ORCID iD: 0000-0002-5244-7539
SPIN 代码: 2820-9120
PhD, Dr. Sci. (Biology)
俄罗斯联邦, KurganAnna M. Aranovich
Ilizarov National Medical Research Centre for Traumatology and Orthopedics
Email: aranovich_anna@mail.ru
ORCID iD: 0000-0002-7806-7083
SPIN 代码: 7277-6339
MD, PhD, Dr. Sci. (Medicine), Professor
俄罗斯联邦, Kurgan参考
- Richette P, Bardin T, Stheneur C. Achondroplasia: from genotype to phenotype. Joint Bone Spine. 2008;75(2):125–130. doi: 10.1016/j.jbspin.2007.06.007
- Legare JM. Achondroplasia. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2023. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1152/
- Pauli RM. Achondroplasia: a comprehensive clinical review. Orphanet J Rare Dis. 2019;14(1):1. doi: 10.1186/s13023-018-0972-6
- Foreman PK, van Kessel F, van Hoorn R, et al. Birth prevalence of achondroplasia: a systematic literature review and meta-analysis. Am J Med Genet A. 2020;182(10):2297–2316. doi: 10.1002/ajmg.a.61787
- Matsushita M, Esaki R, Mishima K, et al. Clinical dosage of meclozine promotes longitudinal bone growth, bone volume, and trabecular bone quality in transgenic mice with achondroplasia. Sci Rep. 2017;7(1):7371. doi: 10.1038/s41598-017-07044-8
- Bonafe L, Cormier-Daire V, Hall C, et al. Nosology and classification of genetic skeletal disorders: 2015 revision. Am J Med Genet A. 2015;167A(12):2869–2892. doi: 10.1002/ajmg.a.37365
- Coi A, Santoro M, Garne E, et al. Epidemiology of achondroplasia: a population-based study in Europe. Am J Med Genet A. 2019;179(9):1791–1798. doi: 10.1002/ajmg.a.61289
- Wang Y, Liu Z, Liu Z, et al. Advances in research on and diagnosis and treatment of achondroplasia in China. Intractable Rare Dis Res. 2013;2(2):45–50. doi: 10.5582/irdr.2013.v2.2.45
- Couser NL, Pande CK, Turcott CM, et al. Mild achondroplasia/hypochondroplasia with acanthosis nigricans, normal development, and a p.Ser348Cys FGFR3 mutation. Am J Med Genet A. 2017;173(4):1097–1101. doi: 10.1002/ajmg.a.38141
- Wrobel W, Pach E, Ben-Skowronek I. Advantages and disadvantages of different treatment methods in achondroplasia: a review. Int J Mol Sci. 2021;22(11):5573. doi: 10.3390/ijms22115573.
- Duggan S. Vosoritide: first approval. Drugs. 2021;81(17):2057–2062. doi: 10.1007/s40265-021-01623-w
- Wendt DJ, Dvorak-Ewell M, Bullens S, et al. Neutral endopeptidase-resistant C-type natriuretic peptide variant represents a new therapeutic approach for treatment of fibroblast growth factor receptor 3-related dwarfism. J Pharmacol Exp Ther. 2015;353(1):132–149. doi: 10.1124/jpet.114.218560
- Paton DM. Efficacy of vosoritide in the treatment of achondroplasia. Drugs Today (Barc). 2022;58(9):451–456. doi: 10.1358/dot.2022.58.9.3422313
- Savarirayan R, Tofts L, Irving M, et al. Once-daily, subcutaneous vosoritide therapy in children with achondroplasia: a randomised, double-blind, phase 3, placebo-controlled, multicentre trial. Lancet. 2020;396(10252):684–692. doi: 10.1016/S0140-6736(20)31541-5
- Savarirayan R, Irving M, Maixner W, et al. Rationale, design, and methods of a randomized, controlled, open-label clinical trial with open-label extension to investigate the safety of vosoritide in infants, and young children with achondroplasia at risk of requiring cervicomedullary decompression surgery. Sci Prog. 2021;104(1):368504211003782. doi: 10.1177/00368504211003782
- Foreman PK, van Kessel F, van Hoorn R, et al. Birth prevalence of achondroplasia: a systematic literature review and meta-analysis. Am J Med Genet A. 2020;182(10):2297–2316. doi: 10.1002/ajmg.a.61787
- Popkov AV, Shevtsov VI, Dzhanbakhishov GSO. Achondroplasia: a guide for doctors. Moscow: Medicine; 2001. 352 p. (In Russ.) EDN: UHZIDG
- Zheng X, Qin S, Shi L, et al. Preliminary study of Ilizarov technique in treatment of lower limb deformity caused by achondroplasia. Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2023;37(2):157–161. (In Chin.) doi: 10.7507/1002-1892.202210072
- Donaldson J, Aftab S, Bradish C. Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature. J Orthop. 2015;12(1):31–34. doi: 10.1016/j.jor.2015.01.001
- Aranovich AM, Gofman FF, Korkin AY, et al. Surgical rehabilitation of patients with systemic skeletal diseases. In: XII All-Russian congress of traumatologists and orthopedists. Collection of abstracts. Saint Petersburg: Saint Petersburg Public Organization “Man and His Health”, 2022. P. 37–38. (In Russ.) EDN: MZZFEG
- Luneva SN, Menshchikova TI, Aranovich AM. Features of reparative osteogenesis of distraction regenerate of the tibia and the content of some osteotropic growth factors in patients with achondroplasia aged 9–12 years. Pediatric Orthopedics, Traumatology and Reconstructive Surgery. 2022;10(3):223–234. EDN: WHEOFF doi: 10.17816/PTORS108618
- Menshchikova TI, Aranovich AM. Lengthening of the shins in patients with achondroplasia 6–9 years old as the first stage of growth correction. Genius of Orthopedics. 2021;27(3):366–371. EDN: RQVSDS doi: 10.18019/1028-4427-2021-27-3-366-371
- Chirkova AM, Silantyeva TA, Erofeev SA, et al. Histomorphometric features of distraction regenerates formed after disruption of the integrity of the tibia in various ways. In: Danilov RK, editor. Fundamental and applied problems of histology. Histogenesis and tissue regeneration. Proceedings of a scientific conference. Saint Petersburg: Kirov Military Medical Academy; 2004. P. 137–139. (In Russ.)
- Onoprienko GA, Voloshin VP. Microcirculation and regeneration of bone tissue: theoretical and clinical aspects. Moscow: Binom. Laboratory of knowledge; 2017. 184 p. (In Russ.)
补充文件
附件文件
动作
1.
JATS XML
2.
Fig. 3. Sonograms of the proximal tibial regenerate after 30 days of fixation in colour Doppler mapping and 3D reconstruction mode. The vessels in the zone of active osteogenesis are visualised (asterisks show the cluster of bone trabeculae with an acoustic density of 168 units)
下载 (79KB)
3.
Fig. 4. Sonogram of the tibial regenerate of patient with achondroplasia K., 16 years old. Repeated tibia lengthening was performed - 5.5 cm. Structural state of the regenerate after 60 days of fixation. The length of the echopositive area of the regenerate - 33 mm, ultrasound penetration depth - 34 mm; acoustic density of the structure in the zone of active osteogenesis - 198.6 specific units (asterisks), hypoechogenic zone of the regenerate - 19 specific units (1), connective tissue layer (2) - 125.6 specific units
下载 (60KB)
4.
Fig. 1. Sonograms of the proximal tibial regenerate at various fixation periods in patient B., 7 years old (group I). Achondroplasia, short stature; stage I of treatment; tibial elongation of 8.5 cm. a, Fixation period, 2 days (echogenic fragments in different zones of the regenerate are marked with asterisks). b, Fixation period, 31 days (asterisks indicate echogenic fragments with actively ongoing osteogenesis, showing an acoustic density (AD) of 200 ± 22.0 arbitrary units. The connective tissue areas between these fragments had an AD of 122 ± 21.0 arbitrary units; regenerate length, 17 mm; ultrasound penetration depth, 10 mm). c, Fixation period, 60 days (asterisks mark the formed cortical plate contour with an AD of 191 ± 12 arbitrary units)
下载 (254KB)
5.
Fig. 2. Sonograms of the proximal regenerate of the tibia in patient I., aged 14 years (group II, stage I treatment). Achondroplasia, short stature. a, Fixation period, 5 days; elongation, 7 cm; echopositive regenerate length, 38.6 mm; ultrasound penetration depth, 30.7 mm; acoustic density of the structure in the active osteogenesis zone, 186 ± 11.0 arbitrary units (echo-dense fragments marked with asterisks). b, Fixation period, 30 days; echopositive regenerate length, 30.6 mm; ultrasound penetration depth, 18.9 mm; acoustic density of the structure in the active osteogenesis zone, 189 ± 11 arbitrary units. c, Fixation period, 65 days; echopositive regenerate length, 24.2 mm; ultrasound penetration depth, 11.8 mm; acoustic density of the structure in the active osteogenesis zone, 198 ± 6 arbitrary units
下载 (278KB)
