Mukopolisakkaridozlu Hastalarda Karpal Tünel Sendromu ile Kardiyak Tutulumun İlişkisi

Yıl 2024, Cilt: 34 Sayı: 5, 733 - 739, 30.10.2024

Banu Kadıoğlu Yılmaz , Melih Timuçin Doğan , Onur Bulut , Ahmet Sert , Hüseyin Yılmaz

https://doi.org/10.54005/geneltip.1519915

Öz

Giriş / Amaç: Mukopolisakkaridozlar (MPS) progresif, multisistemik tutulumla giden bir hastalık grubudur ve bu sistem tutulumları arasında bir ilişki olup olmadığı bilinmemektedir. Bu çalışmada, MPS hastalarında kardiyak tutulum ile karpal tünel sendromu (KTS) arasında ilişki olup olmadığını araştırmayı amaçladık.
Yöntem: Çalışma, 12 Mayıs 2023 ile 30 Haziran 2024 tarihleri arasında, retrospektif başlangıçlı prospektif bir çalışma olarak gerçekleştirildi. MPS Tip I, II, IV A ve VI tanısı enzimatik ve genetik olarak konulmuş olan, KTS taraması için elektromiyografi ve kardiyak tutulum açısından transtorasik ekokardiyografi tetkiki yapılan 2-20 yaş arası hastalar çalışmaya alındı.
Bulgular: Çalışmaya 26 MPS hastası dahil edildi. Hastaların yaş ortalamaları 128±58 ay idi. Hastaların 21 (% 80.8)’inde kardiyak tutulum mevcuttu. Kalp kapak tutulumu hastaların 20 (%76.9)’sinde mevcuttu. Çalışmamızda MPS hastalarımızın 14 (%53.8)’ünde KTS olduğunu tespit ettik. KTS, MPS tip I, II ve VI hastalarının tamamında vardı, MPS tip IV A hastalarının ise hiçbirinde yoktu. Yapılan istatistiksel analizler sonucunda, MPS Tip IV A olma durumu KTS olmayan grupta, KTS olan gruba göre istatistiksel olarak anlamlı derecede yüksekti (p<0.001). Kardiyak tutulum varlığı, aort ve mitral kapakların birlikte tutulumu ve kalp kapak tutulum şiddeti, KTS olan grupta KTS olmayan gruba göre istatistiksel olarak anlamlı derecede yüksek saptandı (sırasıyla, p=0.007, p=0.005, p=0.009).
Sonuç: Çalışmamızda, kalp kapak tutulumu başta olmak üzere kardiyak tutulumu olan hastalarda KTS’nin daha sık olduğunu bulduk. Özellikle kardiyak tutulumu olan MPS hastalarında KTS taramasının yapılması gerektiğini öneriyoruz.

Anahtar Kelimeler

Mukopolisakkaridoz, karpal tünel sendromu, kalp kapak tutulumu, kardiyak tutulum

The Relationship Between Carpal Tunnel Syndrome and Cardiac Involvement in Patients with Mucopolysaccharidosis

Öz

Background / Aims: Mucopolysaccharidoses (MPSs) are a group of progressive multisystemic diseases, and it is unknown whether there is a relationship between these system involvements. In this study, we aimed to investigate whether there is a relationship between cardiac involvement and carpal tunnel syndrome (CTS) in MPS patients.
Methods: The study was conducted between May 12, 2023, and June 30, 2024, as a prospective study with a retrospective onset. Patients between the ages of 2 and 20 years who were diagnosed with MPS Types I, II, IV A, and VI enzymatically and genetically and who underwent electromyography for CTS screening and transthoracic echocardiography for cardiac involvement were included in the study.
Results: Twenty-six MPS patients were included in the study. The mean age of the patients was 128±58 months. There was cardiac involvement in 21 (80.8%) of the patients. Heart valve involvement was present in 20 (76.9%) patients. Our study found that 14 (53.8%) of our MPS patients had CTS. CTS was present in all patients in the MPS type I, II, and VI groups but not in any MPS type IV A patients. As a result of the statistical analysis, the presence of MPS Type IV A was statistically significantly higher in the group without CTS than in the group with CTS (p<0.001). The presence of cardiac involvement, combined involvement of the aortic and mitral valves, and the severity of heart valve involvement were found to be statistically significantly higher in the patient group with CTS than in the patient group without CTS (p=0.007, p=0.005, p=0.009, respectively).
Conclusions: Our study found that CTS was more common in patients with cardiac involvement, especially heart valve involvement. We recommend that CTS screening be performed, especially in MPS patients with cardiac involvement.

Anahtar Kelimeler

Mucopolysaccharidosis, carpal tunnel syndrome, heart valve involvement, cardiac involvement

Etik Beyan

The study was conducted according to the guidelines of the Declaration of Helsinki and approved by Selçuk University Faculty of Medicine Local Ethics Committee (Decision No: 2023/302, Date: 06/06/2023).

Destekleyen Kurum

none

Teşekkür

none

Kaynakça

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