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BACKGROUND Ornithine transcarbamylase deficiency (OTCD) is an X-linked semi-dominant disorder, causing possible fatal hyperammonemia. Late-onset OTCD can develop at any time from 2 months after birth to adulthood, accounting for 70% of all OTCDs. CASE REPORT A 35-year-old man with chronic headaches stated that since childhood he felt sick after eating meat. Fourteen days before hospital admission, he began receiving 60 mg/day of intravenous prednisolone for sudden deafness. The prednisolone was stopped 5 days before hospital admission. Four days later, he was transferred to our hospital because of confusion. On admission, he had hyperammonemia of 393 µmol/L. Because he became comatose 7 hours after admission, and his serum ammonia increased to 1071 µmol/L, we promptly started hemodialysis. Because his family history included 2 deceased infant boys, we suspected late-onset OTCD. On day 2 of hospitalization, we began administering ammonia-scavenging medications. Because he gradually regained consciousness, we stopped his hemodialysis on day 6. After his general condition improved, he was transferred to the previous hospital for rehabilitation on day 32. We definitively diagnosed him with late-onset OTCD due to the low plasma citrulline and high urinary orotic acid levels found during his hospitalization. CONCLUSIONS Clinicians should suspect urea cycle disorders, such as OTCD, when adult patients present with marked hyperammonemia without liver cirrhosis. Adult patients with marked hyperammonemia should immediately undergo hemodialysis to remove ammonia, regardless of causative diseases.

症例の概要：患者は61歳の男性．歯周炎の進行による臼歯喪失に伴い，咬頭嵌合位も喪失していた．まず，治療用の部分床義歯で適切な咬合高径を与え，歯周初期治療を行った．引き続き，歯周外科治療を行い歯周組織の安定が確認できた後に最終補綴を行った．考察：本症例は初診時のエックス線所見で保存困難に思えた歯も喪失することなく最終補綴装置装着後7年経過している．義歯で適切な咬合高径を与え，残存歯の補綴装置は必要に応じて連結したことと，治療後の適切なメインテナンスにより，良好な結果が得られたと考える．結論：本症例は歯周治療後に適切な補綴処置を行うことで重度歯周炎に罹患した歯も長期にわたり保存できることを示した．

Purpose The maxillary unilateral or bilateral molars of rats were extracted, and the influences of the partial loss of occlusal support were evaluated using an 8-arm radial maze and a step-through type passive-avoidance apparatus. Methods Rats were randomly allocated to three groups not undergoing molar extraction or undergoing extraction of the maxillary unilateral or bilateral molars. Each group was further divided into two groups for maze or passive-avoidance experiments. Thus, a total of six groups were established. The maze experiment was conducted once daily for 10 days. The number of correct choices, number of errors, and the trial time were recorded. The passive-avoidance experiment consisted of an acquisition trial and retention trial. In the acquisition trial, rats were placed in a light room, and the response latency until their entry into a dark room was measured. After 24h, a similar procedure was performed as a retention trial. Results In the maze experiment, there was no significant difference by all the groups except on the day 1 in the number of correct choices. But bilateral molar loss group, the number of errors were significantly lower than no extraction group on days 1, 2, 3, 4, and 7. In the passive-avoidance experiment, though the response latency in the retention trial was longer than that in the acquisition trial in all three groups, according to the increase in the number of tooth extraction, it became significantly shorter between P1 and P3. Conclusion These results suggested that molar loss may be a cause of learning/memory impairment.

Purpose : To clarify the relationship between the height of occlusal interference and stress responses by measuring the amount of dopamine released in the prefrontal cortex of rats.Materials and Methods : Aluminum laminate (0.5, 1.0 or 2.0 mm thick) was fixed to the maxillary left molar to interfere with the occlusion of the rats. No laminates were fixed in the control rats. The amount of dopamine released in the prefrontal cortex was measured by microdialysis before and after fixing the laminate. After fixing the laminate, the dopamine was measured for 360 minutes in experiment 1. In experiment 2, it was measured for 180 minutes and later measured for an additional 180 minutes after the rats were given 20 minutes to eat a food pellet.Results : In experiment 1, the amount of dopamine released in the prefrontal cortex was not significantly different among the groups during the 360 minutes following the operation to insert the laminate. The amount of dopamine released in experiment 2 increased only in the rats with an occlusal interference of 0.5 mm (p=0.04). Furthermore, a comparison of the amount of dopamine released before and after feeding within each group indicated that the only significant change was an increase during the 60 to 100-minute period after the 20-minute feeding session in the 0.5 mm group.Conclusion : The amount of dopamine released in the prefrontal cortex of rats in this study was influenced by the height of occlusal interference. Low (0.5 mm) occlusal interference was more stressful than higher (1.0 and 2.0 mm) occlusal interference.

目的: 咬合障害とストレス反応の関連を明らかにするために咬合障害を与えたラットの前頭皮質ドーパミン放出量を測定した. 咬合障害を付与した状態での摂食時ドーパミン放出量の増加は不安を含めた精神医学的なストレス反応に関与しているという仮説を検証するために, 抗不安作用のあるベンゾジアゼピン系薬物ジアゼパムの影響を調べた.方法: 前頭皮質中のドーパミン放出量の測定はマイクロダイアリシス法を用いて行った. 各実験において対照群と咬合障害群の2群に分け, おのおのをn=6とした. ドーパミン放出量の測定は摂食180分後まで行った. 実験1固形飼料を摂食させ, ドーパミン放出量の経時的変化を測定した. 実験2摂食前にジアゼパムを投与し, その後のドーパミン放出量の経時的変化を測定した. 実験3摂食前に生理食塩水を投与し, その後のドーパミン放出量の経時的変化を測定した.結果: 実験1咬合障害群は摂食後ドーパミン放出量に有意な増加を認めた.実験2咬合障害群と対照群でドーパミン放出量に有意差は認められなかった. 実験3咬合障害群は摂食後, ドーパミン放出量に有意な増加を認めた.結論: 咬合障害を付与したラットの摂食時前頭皮質ドーパミン放出は増大し, その反応はジアゼパムにより抑制されることが統計学的に証明された. 本研究により, 咬合障害は不安を含めた精神医学的ストレスを惹起することが明らかにされた.

A 30-year-old woman presented with multiple nodular shadows which enclosed a cavity on a chest radiograph. Chest computed tomographic (CT) images showed mediastinal lymphadenopathy, and multiple nodular opacities enclosing a cavity. Histopathological findings of biopsy specimens from the lung and mediastinal lymph nodes revealed noncaseating epithelioid cell granulomas without any evidence of Mycobacterium or fungal growth. The lesion in the lung included granulomatous vasculitis. Even without corticosteroid or any other therapy, the lung lesions resolved and the cavity disappeared. We report a case of sarcoidosis with primary acute cavitation.