• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.1
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• pp.139-145
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• 1999

Congenital Insensivity to Pain with Anhidrosis(CIPA) is rare autosomal recessive disorder which is known to be hereditary sensory and autonomic neuropathies(HSAN) type IV. It is predilection for male and observed in early infant. Its clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mild to moderate mental retardation. Its dental features include self-mutilation by nail peeling, which leads to gingival ulcer, tongue ulcer due to tongue biting, and enamel hypoplasia. Partial anodontia is often observed as well. Seventeen-months-old boy with CIPA was reported. This is the case in which the dental characteristics are described and the dental treatment of patient is discussed.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.1
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• pp.93-98
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• 2020

Lesch-Nyhan syndrome is a rare X-linked, recessively inherited disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. This syndrome is characterized by 3 major features: neurological dysfunction, hyperuricemia, and cognitive and behavioral disturbances (e.g., self-mutilation, which begins at 2 to 3 years of age). Uncontrollable self-mutilation begins with biting of the perioral tissues and extends into patterns such as finger biting and head hitting. This report describes the case of a 31-month-old boy who was diagnosed with Lesch-Nyhan syndrome with severe lip injuries caused by self-mutilative behaviors. The behaviors were blocked with a semi-fixed lip bumper for a short period. The device was applied to the patient on the day of the visit without the requirement for an oral impression. It was easy to manage oral hygiene and adjust the device because it was detachable by clinicians and guardians. Therefore, a semi-fixed lip bumper may be useful as an interim appliance to block selfmutilative behaviors in children with Lesch-Nyhan syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.46 no.4
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• pp.416-421
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• 2019

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disorder; affected patients are characterized by inability to feel pain and to sweat over the entire body, as well as by mental retardation. Because, in the oral examination, no specific findings on soft or hard tissue may be found except possible lesions due to self-mutilation, early recognition and diagnosis are essential for these patients. Pediatric dentists must be aware of the clinical manifestations and treatment considerations related to uncontrolled body temperature, tactile hyperesthesia and lack of pain reflex. In this case report, dental management of CIPA was suggested by presenting a 6-year follow-up of young patient.

• Journal of the Korean Society for Library and Information Science
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• v.28
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• pp.83-107
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• 1995

The purpose of this research is to investigate the attitudes of students, who are used to study at the Library of Duksung Women's University, concerning about the loss and mutilation of books, articles and other research materials m a university library, and to take measures for preventing the library materials from being lost and mutilated. This study made the surveys of student's recognition about the immoral behaviors like larcenous and mutilating acts, the causes of such destructive works and effective preventive measures to keep library materials in good condition, including student's opinion about library services. The investigations were conducted in two times with the same questionnaire on randomly selected 480 students and 540 students of 1989 and 1994 respectively. The sample size is estimated to reach each $10\%$ of the total number of students of 1989 and 1994 in Duksung Women's University. And then, the students were divided into two groups. The one is a group that has had experiences stealing and mutilating the materials from the Library of the University. The other is another group which has done none of them. Thereafter the responses of the two groups are analyzed to compare the differences of the students' behaviors between 1989 and 1994, and thereby finding out important factors inviting the loss and mutilation and accordingly improving effective checks to deter the students from stealing and mutilating the library materials. Some of the research findings suggested in this paper are pointed out as follows: 1) The students who has experienced neither stealing nor mutilating the library materials visited the library more frequently and are more serious about such destructive behavior as stealing and mutilating. 2) The attitudes of the students about services and equipments supplied by the library are slightly different among the students who experienced such immoral activities or not. For example, the experienced students had more preference about an application of self-help duplicating machine being able to use card. 3) To prevent the students from stealing and mutilating the library materials, the non-experienced students demanded an education for them to duly recognize the public interest of the library and also suggested to keep more duplicate materials ready in the library, meanwhile, the experienced students indicated strongly such proposals as strict regulations against stealing and mutilating behaviors, having a correct understanding of costing a lot of time and money to restore the damaged materials to their original state and keeping many duplicates ready in the library. 4) It appears to be that there were different between the experienced and non- experienced students concerning causes invited stealing and mutilated behaviors. 5) Over all, the number of the non-experienced students is more increased in 1994 than that of 1989.

• The Research Journal of the Costume Culture
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• v.23 no.3
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• pp.412-424
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• 2015

The purpose of this study was to analyze the meaning of soul fashion in American pop culture. This study was conducted using a literature research method based on the prior theses, journals and relevant books. Soul as a concept, originated in African-American communities and evolved from the ideology of Black Power, which prompted Black Nationalism. Soul fashion, which took on two styles in African American culture began to embody black resistance and community pride in the late 20th century. One of these, hip-hop style represented the message of resistance and a sense of beauty outside the mainstream. The other, African-inspired fashion, which utilized a look inspired by African tradition, rejected white supremacy by expressing a proud dignity. As a result, the meaning of "soul" in soul fashion represented by American pop culture resulted in contrasting appearance due to different elements. First, one of its meanings is ironic and sarcastic, and it expressed historical trauma, cultural stereotypes, self-hatred, and self-degradation and, the self-mutilation of African-American by cynically distorting their silhouettes and, using modified materials and patterns, fantastic colors, and extraordinary accessories. Second, the other meanings is the pride and dignity of Black Power, which visualized the concentration of ideas implied by the tradition of African-American, through soul fashion by using fierce traditional of African costumes, unique patterns and accessories.

• Research in Community and Public Health Nursing
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• v.32 no.4
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• pp.457-466
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• 2021

Purpose: The purpose of this study was to examine the correlations among emotional perception clarity, emotion regulation, family relationship, non-suicidal self-injury, and depression, and to determine associated factors of non-suicidal self-injury and depression for senior elementary school students. Methods: Data were collected from 150 early adolescences in K region, Korea. A self-report questionnaire consisted of Trait Meta-Mood Scale, Cognitive Emotion Regulation Questionnaire, Family Relationship Assessment Scale, Functional Assessment of Self-Mutilation, and Children's Depression Inventory. The data were analyzed using t-test, Pearson's correlation coefficient, logistic regression, and multiple regression analysis. Results: Non-suicidal self-injury and depression were positively associated with maladaptive emotion regulation strategy and family conflict, but negatively related to emotional perception clarity and family support. Adaptive emotion regulation strategy and family togetherness were only significantly correlated with depression. In logistic regression analysis, significant predictors of non-suicidal self-injury were emotional perception clarity, maladaptive emotion regulation strategy, and family support. Multiple regression analysis found that significant factors of depression were adaptive and maladaptive emotion regulation strategies, which explained 38.0% of the variance. Conclusion: Our study findings suggest that targeted intervention to reinforce the adaptive emotion regulation strategy and family relationship may prevent non-suicidal self-injury, and depression for senior elementary school students.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.392-399
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• 2019

Wilson disease a rare autosomal recessive inherited disorder of copper metabolism, is characterized by excessive deposition of copper in the liver, brain, and other tissues. Wilson disease is often fatal if it is not recognized early and treated when it is symptomatic. Gitelman syndrome is also an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. Hereditary sensory autonomic neuropathy type IV (HSAN-IV), a very rare condition that presents in infancy, is characterized by anhidrosis, absence of pain sensation, and self-mutilation. It is usually accompanied by developmental delay and mental retardation. We report a case of Wilson disease manifested as fulminant hepatitis, acute pancreatitis, and acute kidney injury in a 15-year-old boy comorbid with HSAN-IV and Gitelman syndrome. Such concurrence of three genetic diseases is an extremely rare case.