偽性副甲状腺機能低下症患者の歯列異常と矯正特性

Dental anomalies and orthodontic characteristics in patients with pseudohypoparathyroidism.

• Jane Hejlesen
• Line Underbjerg
• Hans Gjørup
• Tanja Sikjaer
• Lars Rejnmark
• Dorte Haubek

抄録

背景:

偽性副甲状腺機能低下症（PHP）は、GNAS遺伝子またはGNAS複合体遺伝子座の上流での突然変異によって引き起こされるまれな遺伝性疾患です。PTHに対する末梢臓器の抵抗性を特徴とし、低カルシウム血症と高リン血症を引き起こす。本研究では、PHP患者の歯型別の歯列異常と矯正歯科的特徴を調べることを目的とした。

BACKGROUND: Pseudohypoparathyroidism (PHP) is a rare and inherited disease caused by mutations in the GNAS-gene or upstream of the GNAS complex locus. It is characterized by end-organ resistance to PTH, resulting in hypocalcemia and hyperphosphatemia. We aimed to investigate the dental anomalies according to tooth types and the orthodontic characteristics of patients with PHP.

方法:

デンマーク在住のPHP患者29人（女性23人）を対象に、横断的デザインを用いて、口腔内写真とレントゲン写真を調べた。

METHODS: Using a cross-sectional design, 29 patients (23 females) with PHP, living in Denmark, were included, and their clinical intraoral photos and radiographs were examined.

結果:

歯髄石灰化は76％の患者に認められた。根尖の鈍化は55%に認められ、根の短縮は48%に認められました。歯根の鈍化と短縮は、他の歯種に比べて小臼歯に多く見られた（p<0.01）。また、下前歯の混み合い（36％）、上顎弓部の歯牙浮腫（25％）、正中線部の歯牙浮腫（18％）、クラスIII不正咬合（11％）が頻繁に観察された。

RESULTS: Pulp calcification was found in 76% of the patients. Blunting of root apex was present in 55% and shortening of root in 48% of the examined patients. Blunting and shortening of roots were seen more often in premolars than in other tooth types (p < 0.01). Crowding of lower anterior teeth was frequently observed (36%) as well as diastema in the upper arch (25%), midline diastema (18%), and Class III malocclusion (11%).

結論:

今回の研究では、歯髄石灰化や歯根形態の逸脱の影響を受けていることが多かった。歯根の鈍化と短縮は、他の歯種に比べて小臼歯に多く見られました。クラスIIIの不正咬合が比較的多く見られました。PHP患者に適切な治療を行うためには、歯の異常や咬合に注意を払うことが重要である。

CONCLUSION: In the present study population, the teeth were frequently affected by pulp calcification and/or deviation of the root morphology. Blunting and shortening of root(s) were more often seen in premolars than in other tooth types. Class III malocclusion was relatively prevalent. It is important to pay attention to dental anomalies and occlusion in order to provide adequate care for patients with PHP.