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Journals / Acta Orthopaedica et Traumatologica Turcica / 2011 / Cilt: 45 - Sayı: 6

Proximal realignment surgery for unilateral chronic patella dislocation in Morquio syndrome: A case report

Morquio sendromunda tek taraflı kronik patella çıkığında proksimal dizilim düzeltici cerrahi tedavi: Olgu sunumu

Ali Bülent BAZ
Serdar AKALIN
Hasan ARIK
Ali ERGÜN
Pages
466–469
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

We report a patient with inherited dwarfism, in which a proximal soft tissue realignment procedure was performed to treat chronic patellar dislocation.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Mucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is a lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase.
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Key Findings & Data:Patients are usually affected by a severe joint degeneration from the 2nd or 3rd decade.
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Conclusion & Impact:We report a patient with inherited dwarfism, in which a proximal soft tissue realignment procedure was performed to treat chronic patellar dislocation.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Mucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is a lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Patients with MPS IVA appear healthy at birth. Morquio-specific radiographic changes can be observed prior to clinical signs and symptoms. Patients are usually affected by a severe joint degeneration from the 2nd or 3rd decade. Hyperlaxity of the joints is prominent due to the excess of intermediate metabolites. We report a patient with inherited dwarfism, in which a proximal soft tissue realignment procedure was performed to treat chronic patellar dislocation.

Özet

Mukopolisakkaridoz IVA (MPS IVA: Morquio sendromu) N-asetilgalaktozamin-6-sülfat sülfa-taz enzim eksikliğinden kaynaklanan bir lizozomal depo hastalığıdır. MPS IVA hastalan doğumda sağlıklı görünürler. Bu sendroma özgün radyolojik değişiklikler klinik belirti ve bulgulardan önce gözlenirler. Hastaların çoğunda, ikinci veya üçüncü on yılda, ilerlemiş eklem dejenerasyonu ortaya çıkar. Ara metabolitlerin artmasına bağlı olarak eklem hiperlaksitesi belirgindir. Bu çalışmada, proksimal yumuşak doku dizilim cerrahisi yapılarak tedavi edilen kronik patella çıkık-lı, kalıtsal cüceliği olan bir hasta sunulmuştur.

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Cite This Article

BAZ, A. B., AKALIN, S., ARIK, H., ERGÜN, A. (2011). Proximal realignment surgery for unilateral chronic patella dislocation in Morquio syndrome: A case report. Acta Orthopaedica et Traumatologica Turcica, Cilt: 45 - Sayı: 6, 466–469. https://ajindex.com/articles/353723

References (8)

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