Dental Management and Consideration of Patients with Isovaleric Acidemia in Clinic - A Case Report

Isovaleric acidemia is a metabolic disorder which disrupts normal metabolism of leucine due to the elimination of activity of isovaleryl-CoA dehydrogenase. Therefore, isovaleric acid and related metabolites accumulate in the body of patients. Because the treatment consists of dietary protein restriction, the percentage of carbohydrates in nutrition intake increases to maintain adequate calories to avoid metabolic imbalance. Thus, we need to pay more attention to the management and prevention of dental caries. In addition, dehydration and metabolic acidosis would happen when the patients are challenged with pressure or when they are sick, behavior management is a major concern when treating the patients with this disease. This case report presents a treatment of a 5-year-3-month-old boy who suffered from various dental caries in his mouth, diagnosed with early childhood caries, and under multiple dental visits, we have efficiently controlled the infection of dental caries and reestablished the occlusion of the patient.

關鍵字

isovaleric academia ； leucine ； metabolic acidosis ； early childhood caries

並列摘要

異戊酸血症是由於粒腺體中異戊醯輔A去氫酶缺乏導致白胺酸代謝異常，造成體內異戊酸與其相關代謝物的堆積。由於患者採低蛋白飲食治療來嚴格控制白胺酸的攝取，為了給予適當的熱量避免代謝性失調，醣類在飲食比例中的攝取勢必增加，對於齲齒預防與處理是一大挑戰。除此之外，患者在生病或受到壓力時，體內會脫水並產生代謝性酸中毒，如何在牙科治療中善用行為管理來降低患者的焦慮感也是治療時需考量的重點。本病例報告是一個五歲三個月大的男孩，全口因有多處嚴重齲齒而求診，為早發性兒童齲齒，經多次治療後已達到有效控制感染並重建咬合的目的。

並列關鍵字

異戊酸血症；白胺酸；代謝性酸中毒；早發性兒童齲齒

國際替代計量

Dental Management and Consideration of Patients with Isovaleric Acidemia in Clinic - A Case Report

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