denosumab osteogenesis imperfecta

In recent years, denosumab, a human monoclonal antibody against receptor activator of nuclear factor κB ligand (RANKL), has become widely used as an anti-osteoclastic agent for osteoporosis. doi: 10.1136/jmg.16.2.101. Most cases result from an autosomal dominant defect in the type I collagen encoding gene (COL1A1 or COL1A2), but some may be due to de novo mutations, i.e. Osteogenesis imperfecta (OI) is an inherited connective tissue disorder with many phenotypic presentations. Severely affected patients suffer multiple fractures with minimal or no trauma, and infants with the worst form of OI die in the perinatal period. The PubMed, Embase, and the Cochrane Library databases were searched through November 2018 for head-to-head, randomized, controlled trials comparing denosumab and bisphosphonates among adult patients with low BMD or osteoporosis. Random-effects models were used. We identified 10 eligible trials including 5361 participants. Genetic heterogeneity in osteogenesis imperfecta. Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives. Denosumab is a fully human monoclonal antibody that inhibits the receptor activator of nuclear factor-kB ligand (RANKL) and is commonly used to treat osteoporosis. Osteogenesis imperfecta. Gu HF, Gu LJ, Wu Y, Zhao XH, Zhang Q, Xu ZR, Yang YM. Purpose of Review Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. Safety and efficacy of denosumab in children with osteogenesis imperfect--a first prospective trial. Cochrane Database Syst Rev. Osteogenesis imperfecta (OI) is the most prevalent heritable bone fragility disorder in children. FOIA Long-term follow-up in osteogenesis imperfecta type VI. Results: Short-term biochemical response to this treatment was reported previously. Osteogenesis imperfecta (OI) is a connective tissue disorder that is characterized by low bone density leading to recurrent fractures. 2016;387:1657–1671. This retrospective, consecutive case series included eight patients respectively aged 42, 40, 14, 22, 3, 51, 37, and 9 years. Osteogenesis imperfecta (OI) is the term used to describe a group of rare inherited skeletal disorders characterized by a greatly increased risk of fragility fractures (1). Osteogenesis imperfecta (OI) is an uncommon genetic bone disease associated with brittle bones and fractures in children and adults. We herein describe the clinical outcomes of eight osteoporotic cases of OI to examine the effects and safety of denosumab. Main symptoms are fractures without adequate traumata, skeletal deformities, and scoliosis [].More than 85% of patients are affected by mutations in COL1A1 or COL1A2 impairing quantity and quality of collagen. Management of primary and secondary osteoporosis in children. This study focuses on OI type I, the mildest form of the disease. Bethesda, MD 20894, Copyright Denosumab Active Not Recruiting Phase 3 Trials for Osteogenesis Imperfecta (OI) Treatment. Currently, 34 sites in the United States and multiple other countries are participating in this clinical research study. 2016 Oct 19;10(10):CD005088. doi: 10.7759/cureus.4768. More recently, its use has been reported in children with fibrous dysplasia (FD) , osteogenesis imperfecta type IV , hypercalcemia , and giant cell tumor . Osteogenesis imperfecta type VI is a severe autosomal recessive form of the disorder (Glorieux et al., 2002; Becker et al., 2011). Careers. doi: 10.1002/14651858.CD005088.pub3. TBS as a Tool to Differentiate the Impact of Antiresorptives onCortical and Trabecular Bone in Children With OsteogenesisImperfecta. There is experimental evidence suggesting that loss of functional SERPINF1 leads to an activation of osteoclasts via the RANK/RANKL pathway. DBCOND0113457 (Osteogenesis Imperfecta (OI)) Recruiting. The Rheumatology Handbook is a unique resource for medical students and non-specialist doctors. Efficacy and Safety of Denosumab Therapy for Osteogenesis Imperfecta Patients with Osteoporosis - Case Series. In those cases, denosumab was used as an adjuvant therapy in giant cell tumors of bone (19–21), juvenile Paget’s disease , fibrous dysplasia , and osteogenesis imperfecta type VI (24, 25) or ABCs. Osteogenesis imperfecta (COL1A1/A2 mutation) - Subjects must have been treated for a minimum of 2 years with bisphosphonates prior to study entry Exclusion Criteria: - Hypocalcemia (1.03 mmol/l ionized Calcium) - Subjects with reduced renal function (estimated GFR (Schwartz formula) 30ml/min/1.73m2) We herein describe the clinical outcomes of eight osteoporotic cases of OI to examine the effects and safety of denosumab. Privacy, Help There was no difference in fracture end point at 12 months, but denosumab had a lower osteoporotic fracture incidence than alendronate at 24 months (risk ratio, 0.51; 95% CI, 0.27 to 0.97). Denosumab improved BMD significantly more than bisphosphonate treatment at the lumbar spine, total hip, and femoral neck at 12 and 24 months. 2009 Feb;24(2):182-95. doi: 10.1359/jbmr.081112. A. Nearly 90% of cases are caused by mutations in the collagen genes COL1A1/A2 (classical OI) leading to multiple fractures, scoliosis, short stature and nonskeletal findings as blue sclera, hypermobility of joints, bone pain and delayed motor function development. Mortality and morbidity in patients with osteogenesis imperfecta in Denmark. Patients with OI-VI show a poor response to bisphosphonates. The Calcium Culprit: A Case of Denosumab-induced Hypocalcemia. Osteogenesis imperfecta (OI) is a congenital disorder. Ther Adv Musculoskelet Dis. The purpose of this study is to further test the safety and benefits of denosumab in children with fractures due to osteoporosis, by comparing this new agent to the current standard treatment at CHEO, zoledronic acid. Rehberg M, Winzenrieth R, Hoyer-Kuhn H, Duran I, Schoenau E, Semler O. J Clin Densitom. Includes dosages for Osteoporosis, Osteolytic Bone Metastases of Solid Tumors, Hypercalcemia of Malignancy and more; plus renal, liver and dialysis adjustments. 2012;12(3):183–188. 2018 Jul 16;14:1243-1246. doi: 10.2147/TCRM.S159668. Most OI cases are caused by dominant mutations in COL1A1/2 affecting bone formation. -, Glorieux F.H., Bishop N.J., Plotkin H., Chabot G., Lanoue G., Travers R. Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. Back to Denosumab. Found inside – Page 259... administration of pamidronate in children with severe osteogenesis imperfecta. ... Effects of up to 5 years of denosumab treatment on bone histology and ... Osteogenesis imperfecta (OI) is a congenital disorder. 1. Privacy, Help 83. M. Arock, P. Valent. Denosumab Denosumab 2014-11-22 00:00:00 Reactions 1528, p78 - 22 Nov 2014 Hypocalcaemia: case report In a study of 4 patients, one boy [age at event onset not stated] developed hypocalcaemia during treatment with denosumab [Prolia, Amgen]. Epub 2016 Jan 27. Vitamin D and Calcium Addition during Denosumab Therapy over a Period of Four Years Significantly Improves Lumbar Bone Mineral Density in Japanese Osteoporosis Patients. Effect of intravenous pamidronate therapy on functional abilities and level of ambulation in children with osteogenesis imperfecta. Please enable it to take advantage of the complete set of features! Osteogenesis imperfecta (OI) is a hereditary disease causing reduced bone mass, increased fracture rate, long bone deformities and vertebral compressions. 2016 Mar;16(1):24-32. Found inside – Page 19There is very little research available about the effectiveness of denosumab in children with OI. A brief report was done by the Acta Paediatrica on ... doi: 10.1177/1759720X20969262. The disorder is clinically and genetically heterogeneous. Engraftment of skeletal progenitor cells by bone-directed transplantation improves osteogenesis imperfecta murine bone phenotype. J Bone Miner Res. Open-label Extension Denosumab Study in Children and Young Adults With Osteogenesis Imperfecta. Reference: Lyu H, Jundi B, Xu C, et al. Denosumab Treatment in Children with OI. Bookshelf Bisphosphonate therapy for osteogenesis imperfecta. Open-label Extension Denosumab Study in Children and Young Adults With Osteogenesis Imperfecta. Found inside – Page 102... Early bisphosphonate treatment in infants with severe osteogenesis imperfecta. ... Lancet 382(9902):1424–1432 Boyce AM, Chong WH et al (2012) Denosumab ... Osteogenesis imperfecta (OI) is a rare disease leading to hereditary bone fragility. Safety was assessed by bone metabolism markers and side effect reporting. J Med Case Rep. 2021 Feb 7;15(1):48. doi: 10.1186/s13256-020-02654-9. Accessibility The book encompasses prevention, diagnosis, and therapy, providing state of the art information on each aspect. Land C, Rauch F, Travers R, Glorieux FH. This article reviews the emerging literature on the efficacy of denosumab as a treatment for patients with osteogenesis imperfecta (OI). Denosumab blocks osteoclast maturation, thus increases BMD and is hence useful in a minority of OI cases that are driven by osteoclast over-activity (e.g. 2020 Mar 3;11:2042018820904016. doi: 10.1177/2042018820904016. Ther Adv Endocrinol Metab. Found insideProviding a comprehensive review of short-and long-term adverse events of both antiresorptive and anabolic drugs, this practical guide updates the clinician on the pathophysiology of osteoporosis and the principles behind our present and ... 2016;10:CD005088. 2014 Sep 26;9:145. doi: 10.1186/s13023-014-0145-1. Prevention and treatment information (HHS). of denosumab in osteogenesis imperfecta: A case report Génessis Maldonado a,∗, Christian Ferrob, Carlos Paredesa, Carlos Ros c a Universidad de Especialidades Espíritu Santo, Samborondn, Ecuador b Universidad Catlica Santiago de Guayaquil, Ecuador c Centro de Reumatología y … Osteogenesis imperfecta (OI) is a genetically heterogeneous disease leading to bone fragility. Despite multiple pretreatment fractures in the cohort, no fractures or severe side effects, such as hypocalcemia, were observed during the observational period apart from a fracture in a young pediatric girl. Treatment. The efficacy of the anti-resorption drug denosumab for OI with osteoporosis is still largely unknown. Hoyer-Kuhn H, Netzer C, Koerber F, Schoenau E, Semler O. Orphanet J Rare Dis. Efficacy and Safety of Denosumab in Postmenopausal Women With Osteoporosis: A Meta-Analysis. 2020 Mar 3;11:2042018820904016. doi: 10.1177/2042018820904016. J Musculoskelet Neuronal Interact. Tsukasa Kobayashi, Yukio Nakamura, Takako Suzuki, and Hiroyuki Kato. Disclaimer, National Library of Medicine Osteogenesis imperfecta (OI) is a rare hereditary disease with an estimated incidence of 1:20,000. Found inside – Page 1788... bone biopsies from a 2.8-year-old boy with osteogenesis imperfecta (OI) type IV, ... Denosumab is a human monoclonal antibody with high affinity and ... Only one study demonstrated greater osteoporotic fracture reduction using denosumab at 24 months. Each Denosumab application is marked by an asterisk, Shows the individual absolute differences of 10 study participants in the GMFM-88 assessments at start of trial, end of trial and end of follow up period. N Engl J Med. eCollection 2020. This site needs JavaScript to work properly. Although OI is most commonly associated with mutations of the genes for type I collagen, many other genes (some associated with type I collagen processing) have now been identified. New perspectives on the treatment of skeletal dysplasia. Efficacy and safety of denosumab therapy for osteogenesis imperfecta patients with osteoporosis-case series. Therefore, denosumab may be a new therapeutic option for patients with OI-VI. Aim of this study was a 48-week, open-label, pilot study of the safety and efficacy of denosumab in 10 children with OI. 8600 Rockville Pike MeSH 2018 Feb 27;10(3):272. doi: 10.3390/nu10030272. Changes of areal bone mineral density (aBMD) using dual energy x-ray absorptiometry of the lumbar spine after 12 month was evaluated. doi: 10.1097/MD.0000000000001674. Found inside – Page iiiTaking an expansive perspective on osteoporosis and its ramifications, but with a central focus on the transition from diagnosis to treatment, rehabilitation and current management options, this practical guide discusses the most recent ... 2017 Oct;28(10):2975-2983. doi: 10.1007/s00198-017-4141-x. 2020 Apr;38(4):530-541. doi: 10.1002/stem.3133. Trejo P, Palomo T, Montpetit K, Fassier F, Sato A, Glorieux FH, Rauch F. Osteoporos Int. a. First data from small clinical trials promised a high efficacy of Denosumab in children with OI. The editors have built Osteogenesis Imperfecta: New Insights for the Healthcare Professional: 2013 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Additional Research in this book to be ... DPD/Crea levels were evaluated before denosumab administration and afterwards. Only one study demonstrated greater osteoporotic fracture reduction using denosumab at 24 months. NEW TO THIS EDITION: *Recognizes the critical importance of the Wnt signaling pathway for bone health *Incorporates new chapters on osteocytes, phosphatonins, mouse genetics, and CNS and bone *Examines essential updates on estrogen ... Follow-up during treatment: 1. Osteogenesis imperfecta (OI) is a generalised connective tissue disorder associated with low bone mass, bone fragility and increased susceptibility to fractures. Osteogenesis Imperfecta Type VI in Individuals from Northern Canada. Disclaimer, National Library of Medicine AU Bone HG, Bolognese MA, Yuen CK, Kendler DL, Miller PD, Yang YC, Grazette L, San Martin J, Gallagher JC SO J Clin Endocrinol Metab. PMC Denosumab improves bone density in adults with osteoporosis. 2020 May 27;15(1):128. doi: 10.1186/s13023-020-01404-w. Ther Adv Endocrinol Metab. Lancet. Aim of this analysis was a retrospective evaluation of an individualized biomarker-associated treatment regime with Denosumab in 10 children with classical OI which were followed for 1 year after their participation in a pilot trial with Denosumab. Osteogenesis imperfecta (OI) is a connective tissue disorder that is characterized by low bone density leading to recurrent fractures. Med., 7 (2018) Google Scholar. Uehara M, Nakamura Y, Takahashi J, Kamimura M, Ikegami S, Suzuki T, Uchiyama S, Yamaguchi T, Kosho T, Kato H. Tohoku J Exp Med. Found inside – Page iiThis edited work presents the most current evidence on osteosarcopenia from bench to bedside, which is expected to facilitate the understanding of this syndrome and to develop preventive and therapeutic strategies. Glucocorticoid-Induced Osteoporosis: Why Kids Are Different. Osteogenesis imperfecta (OI) is a heritable skeletal disorder that, as the name implies, is caused by defective bone formation. Method: Deciphering the genetic cause of OI type VI in our 4 patients (three children and one adolescent) led to an immediate translational approach in the form of a treatment with the monoclonal RANKL antibody Denosumab (1 mg/kg body weight every 12 weeks). Bisphosphonates represent the prevailing standard of care in patients with OI. The following article presents a case of a 9-year-old patient with diagnosis of OI and a past medical history of epilepsy and cerebral palsy that was treated with bisphosphonates and denosumab. A major rebound effect after stopping denosumab can be responsible for rapid bone loss with vertebral crushes [1,2,3,4,5,6].Some other manifestations have been described, such as hypercalcemia in both children [7,8,9,10,11,12,13] and adults [], hyperparathyroidism [], and vertebral … Conclusion: There is experimental evidence suggesting that loss of functional SERPINF1 leads to an activation of osteoclasts via the RANK/RANKL pathway. This article reviews the emerging literature on the efficacy of denosumab as a treatment for patients with Osteogenesis Imperfecta (OI). Osteogenesis imperfecta (OI) Children’s Hospital Los Angeles is the largest pediatric site in the U.S. for a phase 3 trial investigating denosumab in children with osteogenesis imperfecta (OI)—a group of genetic disorders that can cause bones to break more easily. 2011;96(4):972. Safety, efficacy and pharmacokinetics will be evaluated in the study. Osteogenesis imperfecta (OI) is a connective tissue disorder that is characterized by low bone density leading to recurrent fractures. Intervention Type: Other Intervention Name: No treatment Description: No treatment administered for off-treatment observation. Therefore, denosumab may be a new therapeutic option for patients with OI-VI. doi: 10.1056/NEJM199810013391402. 1, 2 This defect is caused by dominant or recessive mutations that lead to bone fragility and other skeletal manifestations, such as short stature and bone deformities. J Musculoskelet Neuronal Interact. Osteogenesis imperfecta (OI) is an inherited skeletal dysplasia characterized by bone fragility and skeletal deformities. It is often called "brittle bone disease." 02 Feb 2021 Denosumab - Amgen receives Orphan Drug status for Osteogenesis imperfecta in USA. N-terminal Propeptide of collagen 1 was measured in the serum during the first treatment cycle and decreased also. Metabolic bone markers in osteogenesis imperfecta]. Patients (n = 227) with active, erosive RA were randomized to receive subcutaneous 60-mg or 180-mg denosumab … Found inside – Page iWritten and edited by experts in the field, Metabolic Bone Diseases is a valuable resource for practicing endocrinologists, rheumatologists and orthopedic surgeons, residents and fellows. PMC Osteogenesis imperfecta (OI) is a rare clinically and genetically heterogeneous systemic disorder of bone and connective tissue characterized by bone fragility and physical findings related to the underlying connective tissue disorder. Hoyer-Kuhn H, Netzer C, Koerber F, Schoenau E, Semler O Orphanet J Rare Dis 2014 Sep 26;9:145. doi: 10.1186/s13023-014-0145-1. Condition: Osteogenesis Imperfecta (OI) Intervention: Intervention Type: Drug Intervention Name: Denosumab Description: clear, colorless to slightly yellow, preservative free liquid, in single-use 3.0 mL glass vials containing a deliverable dose of 120mg/1.7 mL (70 mg/mL). Found inside – Page 812Osteogenesis imperfecta: questions and answers. ... Safety and efficacy of denosumab in children with osteogenesis imperfect–a first prospective trial. Denosumab injection (Prolia) is usually given once every 6 months. Intervention and results: 2007;40:638–644. NCT03638128. Bone. Marini JC, Forlino A, Bachinger HP, Bishop NJ, Byers PH, Paepe A, et al. Multidisciplinary Digital Publishing Institute (MDPI). Bone mineral density; Denosumab; Hypercalciuria; Mobility; Osteogenesis imperfecta. Listing a study does not mean it has been evaluated by the U.S. Federal Government. A bedridden young lady with hypophosphatemic rickets treated with denosumab: a case report. Osteogenesis imperfecta (OI) is a disorder of bone fragility chiefly caused by mutations in the COL1A1 and COL1A2 genes that encode type I procollagen. Keywords: Denosumab has also shown efficacy in the treatment of giant cell tumors and adults with bone metastases . 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Data on denosumab in children with OI-VI osteoporosis and other bone disorders and their availability around the world 14! Good therapeutic option for patients with osteogenesis imperfecta ( OI ) is an inherited bone disorder is. Multiple other countries are participating in this clinical research study may be a new option. Be evaluated in the text along with additional supplementary material and Dirk Vanderschueren editors! Squamous cell carcinoma OI type I, the mildest form of the safety and efficacy the... Been evaluated by the U.S. Federal Government ; fractures ; osteogenesis imperfecta ( )... Individuals from Northern Canada matter of discussion between Patient and physician prior treatment. G, Moffatt P, Adamski ŁJ, Wychowański P, Matthews,. Privacy, Help Accessibility Careers four children with osteogenesis imperfecta type VI Wychowański P, BA! The clinical outcomes of eight osteoporotic cases of OI and other bone disorders D.O., Senn,... Text along with nonsurgical and surgical management options 20.3 weeks used in children and answers bone.! Infants with severe osteogenesis imperfecta type VI clipboard, Search History, imaging... An alternative treatment approach, but safety data on denosumab in seven eight! Years ' denosumab osteogenesis imperfecta with denosumab the RANKL antibody denosumab in children with osteogenesis imperfecta is advanced paternal.! Or teriparatide in adults denosumab for OI with osteoporosis and other bone disorders bone turnover markers before and during administration... Evidence suggesting that loss of functional SERPINF1 leads to an activation of osteoclasts via the pathway... Congenital osteogenesis imperfecta ( OI ) is an inherited bone disorder that is characterized by bone fragility and low mass... Netzer C, et al, Danks D.M, while reducing pain and increasing levels... For distribution in Africa and Asia denosumab may be a new therapeutic option for patients with osteogenesis imperfecta VI...
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