• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.532-538
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• 2008

Lesch-Nyhan syndrome is a rare X-linked recessively inherited disorder, caused by complete absence or decrease in activity of hypoxanthine guanine phosphoribosyl transferase(HPRT), an enzyme involved in purine metabolism. This enzyme deficiency gives rise to nephropathy symptoms, such as hyperuricosuria and hyperuricemia by excessive uric acid production and neuropathy symptoms, such as mental retardation, choreoathetosis and self mutilation behavior. Patients with Lesch-Nyhan syndrome have tendency to bite their lip, tongue and finger. In severe cases, partial or even total amputation of tongue or finger occur. Self-inflicted bites are often complicated by secondary infection to the injured site as well as pain. Furthermore tissue loss by biting results in esthetic problems. The dental management of self mutilation includes treatment with appliances such as soft mouth guard or lip bumper, extraction of all the teeth, and orthognathic surgery. We report a case of a 13 year-old boy with Lesch-Nyhan syndrome, who severely injured himself on his tongue. At first, conservative treatment using soft mouth guard was considered, but it could not prevent trauma on his tongue. Therefore, extraction of the lower anterior and posterior teeth was carried out.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.306-311
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• 2005

Lesch-Nyhan syndrome is a rare disorder of purine metabolism, first described in 1964. The incidence is estimated to be 1:100,000 birth. It is an X-linked recessive disorder in which affected males have a virtually complete deficiency of the enzyme hypoxanthine guanine phosphorybosyl transferase(HGPT). This enzyme deficiency gives rise to excessive uric acid production and consequent hyperuricemia. Lesch-Nyhan syndrome is clinically characterized by mental retardation, choreoathetosis, spastic cerebral palsy, and severe self-mutilation behavior. Patient with Lesch-Nyhan syndrome mostly bite their lip, tongue and finger. In severe cases, partial or total amputation of the lip and tongue is common. Self-inflicted bites are often further complicated by secondary infection to injuried site as well as pain. And tissue loss by biting results in esthetic problems. This report presents a Lesch-Nyhan syndrome patient with self-mutilation, who have a destruction of perioral tissue, especially the lower lip. He was treated successfully with soft mouthguard, psychological and pharmacological method.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.5
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• pp.480-488
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• 2008

Orthodontists often treat cases which are difficult to treat with conventional orthodontics. In such cases, it could be treated with surgical procedures with the help of an oral surgeon. Especially, transverse deficiency of the mandible can be corrected by widening the transverse width of mandibular symphysis, using distraction osteogenesis. Transverse widening of mandibular sympysis is known as a safe treatment but still complications could occur during the treatment. We are reporting some complications of cases that mandibular symphysis transverse widening were applied. Some cases showed complications because of the inappropriate osteotomy line. Since straight vertical osteotomy line was inclined to the left, only the left bony segment was likely to expand. According to bio-mechanical considerations, it will be better to perform a step osteotomy, cutting the eccentric area of the alveolar crest and the centric area of the basal symphyseal area. Complications could also occur by the failure of the distraction device. The tooth borne distraction device was attached on the lingual side of the tooth with composite resin. During the distraction period, it was impossible to obtain appropriate distraction speed and rhythm because of frequent fall off of the distraction device. Therefore, distraction device should be attached firmly with orthodontic band or bone screw, etc. Tooth mobility increasement could also occur as a complication. 'Walking teeth phenomenon' was observed during the distraction period, showing severe teeth mobility and pain during mastication. These symptoms fade out during the consolidation period. Since the patient could feel insecure and uncomfortable, it should be notified to the patient before the procedure. Finally, alveolar crestal bone loss could occur. Alveolar crestal bone loss occurred because of lack of distraction device firmness and teeth trauma caused by lower lip biting habit. Therefore, adequate firmness of the distraction device and habit control will be needed.

• Journal of Oral Medicine and Pain
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• v.33 no.1
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• pp.85-95
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• 2008

The purpose of this study was to evaluate the effects of oral habits on the muscles of mastication and facial expression by means of two parameters: muscle stiffness and elasticity. 10 healthy, fully-dentate male subjects in their twenties were selected for this study; all had normal Class I occlusal relationships. Muscle stiffness and elasticity were measured with a tactile sensor(Venustron, Axiom Co., JAPAN) while subjects were asked to relax and perform various parafunctional activities such unilateral clenching(biting the bite force recorder with a force of 50kg on each subject's preferred side), jaw thrusting and lip bracing. The following muscles were examined: temporalis anterior(Ta), masseter(Mm), frontalis(Fr), inferior orbicularis oculi(OOci), zygomaticus major(Zm), superior and inferior orbularis oris(OOrs and OOri) and mentalis(Mn). Paired t-test, Correlation Coefficients, ANOVA and Multiple Comparison t-tests were used for statistical analysis. Unilateral clenching was highly correlated with bilateral stiffness and elasticity of all the muscles tested. Mm was affected by all three oral habits; Ta was affected by unilateral clenching(p<0.05); Zm was affected by unilateral clenching and OOrs, OOri and Mn were most affected by lip bracing(p<0.05). This study indicates that not only the masticatory muscles but also the muscles of facial expression, mainly circumoral muscles, can be significantly influenced by parafunctional activities such as unilateral clenching and lip bracing.

• The korean journal of orthodontics
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• v.27 no.4 s.63
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• pp.523-538
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• 1997

In order to investigate TMD prevalence in malocclusion patients and to study its relationship with occlusal factors, 205 malocclusion patients (M67, F138, 6Y1M-46Y8M) were examined. The following examinations were carried out, Questionnaire personal history, TMD symptoms, and the associated factors Clinical examination : TMJ sound and maximum mouth opening Orthopantomogram : condyle abnormalities, length of Co'-Inc' and Co'-Go', ratio Co'- Inc'/ Co'-Go', and depth of antegonial notch Transcranial view limitation of anterior movement of condyle Model Angle classification, overjet, overbite, midline discrepancy, missing of posterior teeth, posterior crossbite, attrition of palatal cusp of maxillary molars, crowding/spacing The results could be summarized as follows, 1. The prevalence of TMD showed that Helkimo Anamestic Inder(Ai) 0 was $46.8\%$, Ai I was $22.0\%$, Ai II was $31.2\%$ and subjective symptoms increased with aging (p<0.001) and were frequent in females (p<0.05). 2. Flattening ($4.4\%$) was the most frequent condyle abnormality on Orthopantomogram, and $8.3\%$ of subjects showed some abnormalities on Orthopantomogram. 3. The cases with neck and shoulder pain (p<0.001), clenching, lip biting (p<0.01), and headache (p<0.05) showed higher scores of Ai. 4. Angle class II showed high frequency of condylar abnormalities on Orthopantomogram, and subjects whose palatal cusp of maxillary molars had been attrided had the tendency to show high hi scores (p<0.05). The other occlusal factors had nothing to do with the symptoms of TMD. 5. In the cases that 1)the value of Co'-Inc', Co'-Go' or Co'-Inc'/Co'-Go' were low or 2)the differences of Co'-Go' or Co'-Inc'/Co'-Go' between the right and the left were large, condylar abnormalities were frequently obserbed on Orthopantomogram.