多中心骨溶解结节病(MONA):病例系列和文献综述。

Q4 Medicine Mediterranean Journal of Rheumatology Pub Date : 2024-07-24 eCollection Date: 2024-09-01 DOI:10.31138/mjr.311203.mon
Mahabaleshwar Mamadapur, Sabarinath Mahadevan, Ponniah Subramanian ArulRajamurugan, Srilakshmi Gandham, Swati Singh
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摘要

多中心骨溶解结节病和关节病(MONA)是一种罕见的骨骼疾病,由 MMP2 基因突变引起,导致骨和关节退化。本系列病例介绍了三个独特的MONA病例,突出了临床、放射学和遗传学方面的问题。这些见解揭示了MONA的复杂性,有助于早期诊断和多学科管理。病例1是一名13岁的男性,父母为近亲结婚,5年来出现进行性关节畸形、疼痛和行走困难。最初被诊断为幼年特发性关节炎(JIA),尽管接受了治疗,但症状依然存在。检查发现了多种临床表现,包括关节挛缩和结节。基因分析发现,MMP2基因存在致病变异,从而确诊为MONA。病例2和病例3是两兄妹,年龄分别为12岁和17岁,自幼出现进行性手足关节挛缩。临床检查发现他们有挛缩和皮下结节。遗传学分析证实,MONA与MMP2变异基因同为致病基因,强调了这种罕见疾病的遗传学基础。
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Multicentric Osteolysis Nodulosis and Arthropathy (MONA): A Case Series and Review of the Literature.

Multicentric Osteolysis Nodulosis and Arthropathy (MONA) is a rare skeletal disorder driven by mutations in the MMP2 gene, leading to bone and joint degradation. This case series presents three unique MONA cases, highlighting clinical, radiological, and genetic aspects. These insights shed light on the complexities of MONA, aiding early diagnosis and multidisciplinary management. Case 1 is a 13-year-old male, born to consanguineous parents, presented with a 5-year history of progressive joint deformities, pain, and difficulty walking. Initially diagnosed as juvenile idiopathic arthritis (JIA), despite treatment, his symptoms persisted. Examination revealed multiple clinical findings, including joint contractures and nodules. Genetic analysis identified a pathogenic variant in the MMP2 gene, confirming MONA. Case 2 and Case 3 were two siblings, aged 12 and 17 years respectively, who presented progressive joint contractures in their hands and feet since early childhood. Clinical examinations revealed contractures and subcutaneous nodules. Genetic analysis confirmed MONA with a shared homozygous pathogenic MMP2 variant, emphasising the genetic basis of this rare disorder.

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来源期刊
CiteScore
2.00
自引率
0.00%
发文量
42
审稿时长
8 weeks
期刊最新文献
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