Current approach to diagnosis and treatment of children with osteogenesis imperfecta

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Abstract

Osteogenesis imperfecta (OI) is a heritable bone dysplasia characterized by bone fragility and long bone deformities. Approximately 85% of OI cases are caused by dominant autosomal mutations in the type I collagen coding genes (COL1A1 and COL1A2), which affect the quantity or structure of collagen. The remaining percentage of cases is caused by mutation in the proteins responsible for posttranslational modification, processing and crosslinking of collagen, bone mineralization, and osteoblast differentiation. In the past decade, new recessive, dominant, and X-linked inheritance. As a result, new types of OI were added to the Sillence classification, and a new genetic classification consisting of XVIII types is formed. Treatment of patients with OI is a complex task which requires a multidisciplinary care. Pharmacological treatment is based on bisphosphonate treatment, which increases the bone mineral density. In this article, we will describe other approaches in which the effectiveness is studied. Surgical treatment of the fractures and deformities of the extremities showed a positive effect on the patients’ quality of life, despite existing complications. There are a lot of debates about the choice between telescopic and non-telescopic fixators. Rehabilitation plays huge role in the recovery process after fracture and surgeries.

Osteogenesis imperfecta (OI) is a heritable bone dysplasia characterized by bone fragility and long bone deformities. Approximately 85% of OI cases are caused by dominant autosomal mutations in the type I collagen coding genes (COL1A1 and COL1A2), which affect the quantity or structure of collagen. The remaining percentage of cases is caused by mutation in the proteins responsible for posttranslational modification, processing and crosslinking of collagen, bone mineralization, and osteoblast differentiation. In the past decade, new recessive, dominant, and X-linked inheritance. As a result, new types of OI were added to the Sillence classification, and a new genetic classification consisting of XVIII types is formed. Treatment of patients with OI is a complex task which requires a multidisciplinary care. Pharmacological treatment is based on bisphosphonate treatment, which increases the bone mineral density. In this article, we will describe other approaches in which the effectiveness is studied. Surgical treatment of the fractures and deformities of the extremities showed a positive effect on the patients’ quality of life, despite existing complications. There are a lot of debates about the choice between telescopic and non-telescopic fixators. Rehabilitation plays huge role in the recovery process after fracture and surgeries.

About the authors

Mikhail E. Burtsev

European Clinic of Sports Traumatology and Orthopaedics (ECSTO); Peoples’ Friendship University of Russia

Author for correspondence.
Email: drburtsev91@gmail.com
ORCID iD: 0000-0003-1614-1695
SPIN-code: 6268-0522

Orthopaedic Trauma Surgeon; MD, PhD Student in Department of Traumatology and Orthopaedics

Russian Federation, 7, Orlovsky per., Moscow, 129110; 6, Miklukho-Maklaya street, Moscow, 117198  

Aleksandr V. Frolov

European Clinic of Sports Traumatology and Orthopaedics (ECSTO); Peoples’ Friendship University of Russia

Email: drburtsev91@gmail.com

Orthopaedic Trauma Surgeon, Chief of Trauma Department; MD, PhD, Assistant Professor in Department of Traumatology, Orthopaedics

Russian Federation, 7, Orlovsky per., Moscow, 129110; 6, Miklukho-Maklaya street, Moscow, 117198  

Aleksei N. Logvinov

European Clinic of Sports Traumatology and Orthopaedics (ECSTO); Peoples’ Friendship University of Russia

Email: drburtsev91@gmail.com

MD, Orthopaedic Trauma Surgeon; PhD Student in Department of Traumatology and Orthopaedics

Russian Federation, 7, Orlovsky per., Moscow, 129110; 6, Miklukho-Maklaya street, Moscow, 117198  

Dmitry O. Ilyin

European Clinic of Sports Traumatology and Orthopaedics (ECSTO)

Email: drburtsev91@gmail.com

MD, PhD, Orthopaedic Trauma Surgeon

Russian Federation, 7, Orlovsky per., Moscow, 129110

Andrey V. Korolev

European Clinic of Sports Traumatology and Orthopaedics (ECSTO); Peoples’ Friendship University of Russia

Email: drburtsev91@gmail.com

Orthopaedic Trauma Surgeon, Chief Doctor and Medical Director; MD, PhD, D.Sc., Professor in Department of Traumatology and Orthopaedics

Russian Federation, 7, Orlovsky per., Moscow, 129110; 6, Miklukho-Maklaya street, Moscow, 117198  

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Supplementary files

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2. Fig. 1. Hypertrophic callus after surgical treatment of a patient with type V osteogenesis imperfecta

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3. Fig. 2. Lines of bone sclerosis after treatment with bisphosphonates

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4. Fig. 3. Deformations of the lower extremities with osteogenesis imperfecta

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5. Fig. 4. Correction of multiplaned deformities of the lower extremities, intramedullary fixation with a Fassier–Duval rod [57]

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6. Fig. 5. Intramedullary fixation with a Sheffield rod, deformation of the solid part of the rod (a) [51]; telescopic rod disconnection (b); migration of the distal end of the telescopic rod (c); migration of T-shaped tip Bailey–Dubow pin (d) [56]

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7. Fig. 6. The eruption of the rod through the anterior cortical bone layer (a); reimplant fracture (b); bone growth outside the splint area (c); reimplant fracture after osteosynthesis with plate (d)

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Copyright (c) 2019 Burtsev M.E., Frolov A.V., Logvinov A.N., Ilyin D.O., Korolev A.V.

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