Oral and Dental Health of Children with Mucopolysaccharidoses: A review

Abstract

Mucopolysaccharidosis (MPS) is a group of rare genetic disorders caused by deficiencies in enzymes responsible for breaking down glycosaminoglycans (GAGs), leading to their accumulation in various tissues and organs. This accumulation disrupts normal cellular processes, causing progressive damage to multiple systems including the eyes, central nervous system, bones, respiratory, cardiac, and gastrointestinal systems. There are seven main types of MPS, each associated with a specific enzyme deficiency and GAG accumulation. The clinical manifestations of MPS vary widely, from mild to severe forms, and often include physical, neurological and skeletal abnormalities. Oral health issues are common in MPS patients, with dental problems such as caries, gingival inflammation, jaw deformities and delayed dental eruption frequently observed. Given that general anaesthesia poses significant challenges in these patients due to airway limitations and skeletal abnormalities, maintaining optimal oral health becomes even more critical to reduce the need for invasive procedures. The complexity of MPS requires a multidisciplinary approach to treatment, including careful planning for dental procedures. This review highlights the importance of addressing oral health in the broader management of MPS, emphasizing the need for tailored care to mitigate complications and improve overall patient outcomes. Dental professionals must be vigilant in recognizing and managing oral health concerns in each type of MPS patients to ensure comprehensive and safe treatment.

Keywords

• mucopolysaccharidosis
• genetic disorders
• oral health

Mukopolisakkaridozisli Çocukların Ağız ve Diş Sağlığı: Bir Derleme

Abstract

Mukopolisakkaridoz (MPS), glikozaminoglikanların (GAG'lar) yıkımından sorumlu enzimlerin eksikliği nedeniyle oluşan nadir bir genetik hastalıktır. Bu eksiklikler, çeşitli doku ve organlarda GAG birikimine yol açar. Birikim, normal hücresel süreçleri bozarak gözler, merkezi sinir sistemi, kemikler, solunum, kardiyovasküler ve gastrointestinal sistemler dahil olmak üzere birçok sistemde ilerleyici hasara neden olur. MPS'nin her biri belirli bir enzim eksikliği ve GAG birikimi ile ilişkili yedi ana tipi vardır. MPS’nin klinik belirtileri hafif ile şiddetli arasında değişiklik gösterebilir ve genellikle fiziksel, nörolojik ve iskeletsel bozuklukları içerir. MPS hastalarında ağız ve diş sağlığı sorunları yaygındır; çürükler, diş eti iltihabı, çene deformiteleri ve gecikmiş diş sürmesi gibi diş problemleri sıkça gözlenir. Bu hastalarda hava yolu kısıtlılıkları ve iskelet anormallikler nedeniyle genel anestezi önemli zorluklar oluşturduğundan, invaziv işlemlere duyulan ihtiyacı azaltmak için ağız sağlığının en iyi şekilde korunması çok daha büyük önem taşımaktadır. MPS’nin karmaşık yapısı, dental işlemler için dikkatli planlamayı içeren multidisipliner bir yaklaşım gerektirir. Bu derleme, MPS’nin genel yönetimi içinde ağız sağlığının ele alınmasının önemini vurgulayarak, komplikasyonları azaltmak ve prognozu iyileştirmek için kişiselleştirilmiş bakımın gerekliliğine dikkat çekmektedir. Diş hekimleri, her tip MPS hastasında dental sorunları tanıma ve yönetme konusunda dikkatli olmalı, kapsamlı ve güvenli bir tedavi sağlamalıdır.

Keywords

• mukopolisakkaridoz
• genetik hastalıklar
• ağız sağlığı

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