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多中心骨质溶解、结节病和关节病(MONA):病例系列及文献综述

Multicentric Osteolysis Nodulosis and Arthropathy (MONA): A Case Series and Review of the Literature.

作者信息

Mamadapur Mahabaleshwar, Mahadevan Sabarinath, ArulRajamurugan Ponniah Subramanian, Gandham Srilakshmi, Singh Swati

机构信息

Department of Clinical Immunology and Rheumatology, JSS Medical College and Hospital, JSS Academy of Higher Research, Mysore, Karnataka, India.

O.M. HEALTH CARE, Tamilnadu, India.

出版信息

Mediterr J Rheumatol. 2024 Jul 24;35(3):486-489. doi: 10.31138/mjr.311203.mon. eCollection 2024 Sep.

Abstract

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.

摘要

多中心性骨质溶解、结节病和关节病(MONA)是一种由MMP2基因突变驱动的罕见骨骼疾病,会导致骨骼和关节退化。本病例系列展示了三例独特的MONA病例,突出了临床、放射学和遗传学方面的情况。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岁男性,父母为近亲结婚,有5年进行性关节畸形、疼痛和行走困难病史。最初被诊断为幼年特发性关节炎(JIA),尽管接受了治疗,但其症状仍持续存在。检查发现了多项临床体征,包括关节挛缩和结节。基因分析在MMP2基因中发现了一个致病变异,确诊为MONA。病例2和病例3是两名分别为12岁和17岁的兄弟姐妹,自幼年起双手和双脚出现进行性关节挛缩。临床检查发现挛缩和皮下结节。基因分析通过共享的纯合致病MMP2变异确诊为MONA,强调了这种罕见疾病的遗传基础。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e26/11500111/1609e477ecf1/MJR-35-3-486-g001.jpg

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