• The Journal of Korea Assosiation for Disability and Oral Health
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• v.2 no.2
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• pp.147-152
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• 2006

Lesch-Nyhan syndrome is rare X-linked genetic disorder involving absence of the enzyme hypoxanthine guanine phosphoribosyl transferase (HGPRT) related to purine metabolism. The deficiency of HGPRT activity leads to an excesscive uric acid production and consequent hyperuricemia. It occurs almost exclusively in males, and the incidence is estimate to be 1/100,000~380,000. Clinical presentation is characterized by developmental delay, mental retardation, choreoathetosis, spastic cerebral palsy, nephrolithiasis, obstructive nephropathy and acute gouty arthritis. A characteristic feature of Lesch-Nyhan syndrome is the appearance of intractable self-mutilation behavior. Self-mutilation behavior is complicated by secondary infection and tissue loss as well as pain. The dental management of self-mutilation includes presertive methods of using appliances such as lip bumper or soft mouthguard and radical methods such as extraction of all teeth or orthognathic surgery. A case of Lesch-Nyhan syndrome patient with self-mutilation and severe lower lip injury is presented. He was treated successfully with soft mouthguard.

• Journal of Oral Medicine and Pain
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• v.31 no.3
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• pp.199-210
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• 2006

The epidemiological researches on the inpatients hospitalized at the oral medicine ward have been continuously carried out since 1970, and most researches have been performed by centering around the oral medicine wards of college hospitals. Numerous specialists have been produced after the establishment of oral medicine, and they have been active in various fields. As dental clinics have gotten bigger, the function of oral medicine in the secondary clinics is being brought out. As admissive channel, case features, case composition and otherwise have not been researched for a long time, the related researches should be carried out from now on. Hereupon, this study was carried out by targeting the 100 inpatients hospitalized at the oral medicine ward of Sun Hospital located in Daejeon Korea, through questionnaire. As the result, the following results were derived. 1. The ages of the inpatients in Sun Hospital were $29.21{\pm}11.31$ on the average; 71 females' mean average was $29.63{\pm}11.29$ and 29 males' mean average was $28.17{\pm}11.48$. In regard of school career, the patients who finished high-school course or higher accounted for 78%; the patients' school career seemed to be relatively high. The patients who complained of temporomandibular pain accounted for the highest proportion with 65%. In motivation to visit this hospital, internet surfing was 11%, mass media was 10%, acquaintance's introduction was 38%. The patients, who were hospitalized at another hospital due to the same symptom, accounted for 56%. The dental clinics, which made the patients visit this hospital, accounted for 20%. The patients, who were previously aware that the present symptom should be treated by oral medicine, accounted for 38%. The patients, who were not aware of the fact in advance, were 62%. The respondents of 51% answered that they were aware of the fact one month or below before hospitalization. 2. The patients, who complained of craniocervical ache, accounted for 58%; the patients, whose ache aches affect dailylife, were 22%. Continuous ache was 14% and intermittent ache was 68%, and dull pain was 23%. 3. Life variations were compared with each other by using SRRS (Social Readjustment Rating Scale). In consequence, the variation within 3 years indicated a significant difference in the both groups but the variation within 6 months did not indicate any differences. 4. In regard of the questionnaire on the incidents happened for a week, the ache-group was compared with the group free from the ache. As the result, the number of strain arisen for a week, the decrease of favorite works and sudden fear indicated a significant difference. Pleasant feeling and the decrease of interests in looks did not indicate a significant difference, but came close to the significance. 5. In the questionnaire on impatience, the ache-group indicated higher value but there was not a significant difference. 6. In the questionnaire on the symptoms caused by stress, the two groups indicated significant differences in the item of 'the teethridge itches and feels a tooth rising' and 'the occiput or the nape is stiff.' In the item 'the inside of the cheek or the teethridge are widely peeled off, accompanied with ache and hemorrhage', 'the face has acne or pimple' and 'headache frequently attacks', a significant difference was not observed but the two groups came close to the significance.

• Journal of Oral Medicine and Pain
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• v.36 no.3
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• pp.161-167
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• 2011

Sialolith is one of the most common pathologic conditions found in the salivary glands. The mechanisms responsible for the formation of sialoliths have not been elucidated so far. In this article, the chemical composition and micromorphology of a sialolith of a 58-year old female patient suffering from chronic sialoadenitis of the submandibular gland was analyzed using scanning electron microscopy (SEM) and energy-dispersive X-ray spectroscopy (EDX). In a SEM evaluation, the highly mineralized amorphous core surrounded by lamellar and concentric structures was revealed, however no foreign body, organic material, or signs of microorganism were observed in the core of the sialolith. EDX analysis showed the central core was composed of only Ca, O and P, and that a high level of C was detected near the central area as well. These results indicated that the inorganic composition of the sialolith was hydroxyapatite crystals, and that inorganic and organic substances existed around the central cores. This study suggests that the sialolith was composed mainly of hydroxyapatite crystals and the formation of the nucleus of the sialolith in the submandibular gland duct was secondary to sialadenitis, which favors the growth of an inorganic crystalline nucleus.

• 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.39 no.2
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• pp.186-191
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• 2012

The sodium hypochlorite is widely used in endodontic treatment. While it is effective solution for disinfection of root canal system, complications during canal irrigation are rarely reported, especially in primary teeth. This report demonstrates that sodium hypochlorite extruding through the root apex might cause severe complications. A 4-year-old female patient was referred from local dental clinic to the emergency room for the management of sudden facial swelling and pain during re-endodontic treatment of the maxillary primary central incisor using sodium hypochlorite. The patient was given systemic steroids, antihistamines, antibiotics and analgesics and the maxillary primary central incisor was extracted to prevent secondary infection. Swelling began to subside after 2 days. A negative result was obtained from skin patch test with sodium hypochlorite. Thorough care must be taken in primary teeth to prevent the inadvertent injection of sodium hypochlorite to periapical tissues during root canal irrigation. When adverse reaction occurs, proper management such as analgesia, cold compression and adequate medication should be done.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.97-101
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• 2018

Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.

• Restorative Dentistry and Endodontics
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• v.24 no.1
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• pp.100-115
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• 1999

The purpose of this study was to investigate the distribution of calcitonin gene-related peptide containing nerve fibers in rat pulp after dentinl injury by means of immunohistochemistry and confocal laser scanning microscope. The Spague-Dawley rats weighing about 250-300gm were used. The animals were devided into normal control and experimental groups. Experimental animals were sacrified 1, 2, 4, 7, 10, 21days after dentinal injury (dentin cutting, and then acid etching with 35% phosphoric acid) on the maxillary molar teeth. The maxillary teeth and alveolar bone were removed and immersed in the 4% paraformaldehyde in 0.1M phosphate buffer (pH 7.4), then were decalcified with 15% formic acid for 10 days. Serial frozen $50{\mu}m$ thick sections were cut on a cryostat. The rabbit CGRP antibody was used as a primary antibody with a dilution of 1:2000 in 0.01M PB. The sections were incubated for 48 hours at $4^{\circ}C$, and placed into biotinylated antirabbit Ig G as a secondary anti body with dilution of 1:200 in 0.01M PB and incubated in ABC(avidin-biotin complex). The peroxidase reaction was visualized by incubating the sections in 0.05% 3,3 diaminobenzidine tetrahydrochloride containing 0.02% $H_2O_2$. For the confocal laser scanning microscopic examination, Primary antibody reaction was same as immunoperoxidase stainning, but fluorescein isothiocyanate(FITC)-conjugate antirabbit IgG as a secondary antibody was used. The confocal laser scanning microscope was used for the examination. A series of images of optical sections was collected with a 20x objective at $3{\mu}m$ intervals throughout the depth of specimen. FITC fluerescence was registrated through a 488nm and 568nm excitation filter, and images were saved on optical disk. The stereoscopic images and three dimentionnal images were reconstructed by computer software, and then were analyzed. The results were as follows : 1. In normal control group, CGRP containing nerve fibers were coursed through the root with very little branching, and then formed a dense network of terminals in coronal pulp. 2. A slight increase in CGRP containing nerve fibers at 1 and 2day postinjury was noted subjacent to the injury site. In the 4day group, there were an extensive increase in the number of reactive fibers, followed by a partial return toward normal levels at 7~10 day postinjury, and return by 21days. 3. The sprouting of the CGRP containing nerve fibers was evident within 2day after dentinal injury, and by 4days there was a maximal increased, but was decreased at 7days and returned to normal 10~21 day postinjury. 4. In confocal laser scanning microscopic exammination, the distinct distribution pattern and sprouting reaction of CGRP containing nerve fibers were observed in stereoscopic images and three dimentional images. These results suggest that CGRP containing nerve fiber can be important role in the response to dental injury and pain regulation.