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Surgical Management of Pachyonychia Congenita in a 3-Year-Old

  • Jack D. Sudduth;Christopher Clinker;Matthew Holdaway;Jessica L. Marquez;Jacob Veith;Thomas Wright;W. Bradford Rockwell
    • Archives of Plastic Surgery
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    • v.50 no.6
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    • pp.573-577
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    • 2023
  • Pachyonychia congenita is a rare genetic disorder characterized by hypertrophic nail plates, hyperkeratotic nail beds, and thickened hyponychium of the fingers and toes, impairing manual dexterity and resulting in poor aesthetics. The current body of literature describes various treatment modalities, but no singular approach has been defined as the gold standard. In this case, the authors employed different surgical techniques for treating pachyonychia congenita to evaluate the most effective approach. A 3-year-old boy presented with hypertrophic nail growth involving all digits of both hands and feet. Three surgical procedures were performed on the patient's fingers and toes using germinal matrix excision (GME) alone, GME plus partial sterile matrix excision (pSME), or GME plus complete sterile matrix excision (cSME). The digits treated with GME + cSME exhibited no recurrence of nail growth. Those treated with GME alone exhibited recurrence of hypertrophic nail growth, although their growth slowed. Excision of GME + cSME prevented recurrence of hypertrophic nails, while GME alone or with pSME led to slower-growing hypertrophic nails. Complete excision of the germinal and sterile matrices with skin graft closure may be a definitive treatment for pachyonychia congenita, but further studies are needed to validate these findings.

Application of Occupational Therapy Intervention Process Model: A Case of Child With Sensory Integration Dysfunction (작업치료중재과정모델의 적용: 감각통합기능장애 아동 사례)

  • Kim, Ji-Hyun
    • The Journal of Korean Academy of Sensory Integration
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    • v.9 no.2
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    • pp.1-13
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    • 2011
  • Objective : Purpose of this study is to discuss benefits and implications of the clinical reasoning process and re-evaluation in the OTIPM by introducing a single case that occupational therapy intervention is provided based on the OTIPM. Methods : The case subject is a boy aged 5 years and 10 month who had diagnosed as attachment disorder and anxiety disorder from a pediatric psychiatrist before. The boy is referred to sensory integration therapy clinic and underwent occupational therapy intervention service twice a week for four month. Therapeutic activities for the intervention were consisted of sensory integration activities for restorative model, care-giver education for educational model, and performance skill training for acquisitional model. Measurements used in the initial evaluation are JSI-R, DDST-2, Social Maturity Test, KPPS-R, and observation-based performance task analysis. For the performance task analysis, performance skill items were constructed based on the Occupational Therapy Process Framework (OTPF), and those were assessed by the evaluation system of Assessment of Motor and Process Skill (AMPS) and Evaluation of Social Participation (ESI). Results : The detail process of implementing of the OTIPM in this study is reported by following four phases; 1) establish client-centered performance context; 2) establish baseline and interpret cause (initial evaluation); 3) intervention planning and implementing; and 4) recognize intervention outcome (reevaluation). Conclusion : In this case, occupational therapist could provide the client an occupation-based intervention within comprehensive performance context based on the OTIPM. Therapist could clearly identify the cause of problematic performance skills and behaviors and so provide effective intervention to improve client's occupational performance. Additionally, it was found that client's satisfaction of the intervention can be raised when the concept of 'who is the client' is expanded based on the OTIPM. From this study, it is proposed that OTIPM may be a model educible 'comprehensive' enhancement of 'specific' occupational engagement, as it considers both improvement of occupational performance and satisfaction.

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Controversial issues in the legal restriction for prenatal genetic testing in Korea (산전검사 대상 질환에 대한 법적 규제의 문제점에 대한 고찰)

  • Choi, Ji-Young;Jeong, Seon-Yong;Kim, Hyon-J.
    • Journal of Genetic Medicine
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    • v.4 no.2
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    • pp.186-189
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    • 2007
  • More than 6,000 rare disorders including genetic diseases have been reported. Of them, 1,500 diseases (1,211 for clinical diagnosis and 289 for research only) are technically possible for genetic testing. In Korea, since 2005, only 63 genetic diseases is permitted for prenatal genetic testing by the "Bioethics and Biosafety Law". The article 25 in the law prescribes 63 genetic diseases without clear indication for its selection and inclusion criteria. In EU, USA, and other foreign countries, however, there is no provision in the statute on prenatal genetic testing; it is not restricted by a law. Recently, a woman (Mrs. L, 38y) who is a carrier for Menkes disease made an appeal to a government for an amendment of the "Bioethics and Biosafety Law" prohibiting the prenatal diagnosis of her pregnancy at risk for Menkes disease. Menkes disease (MNK) is an X-linked recessive disorder characterized by neurodegeneration, connective tissue defects and hair abnormalities, and no effective treatment is available yet. The prevalence rate of MNK is one in about 250,000 live births. Menkes syndrome patients fail to absorb copper from the gastrointestinal tract in quantities adequate for meeting nutritional needs. These needs seem particularly acute during the initial 12 month of life, when the velocity of brain growth and motor neurodevelopment. Most of pts. die around 3yrs. of age. Mrs. L had a boy with Menkes disease who died at 2y.o. in 2001. Subsequent pregnancy in 2003, she was able to have prenatal genetic testing for mutation of the Menkes (ATP7A) gene and delivered a healthy baby boy. Now, She is pregnant again and wants to have prenatal diagnosis. however, this time, she was not allowed to have any more because Menkes disease is not included in 63 genetic diseases permitted by the law for prenatal genetic testing, in spite of the fact that she is a Menkes disease carrier and her pregnancy is at risk to have an affected baby. This case shows the practical problem of the legal restriction for prenatal genetic testing in Korea. In this study, we report a arguable case and discuss the controversial issues in the legal restriction for prenatal genetic testing in Korea.

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Needs Accessment of Safety Education of High School Students in Seoul (서울시 고등학생의 안전교육 실태 및 요구도 조사)

  • 김민아;이명선
    • Korean Journal of Health Education and Promotion
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    • v.18 no.3
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    • pp.133-162
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    • 2001
  • Unexpected accidents in school has been gradually increased every year, and student's mistaken was the first reason of the accident. To preventing the students from Unexpected accidents in school, safety education is more important than having strong facilities to have much more strong protecting forces for the accidents. Therefore, systematic education of safety is needed most of all, and especially, strengthening safety education in school is needed. Hereby, this study is aimed at investigating and analysing the actual condition and demand of safety education. It also conducted a survey with the target of 1,255 students in the second grade of high school in Seoul from April 20 to May 19, and the result of this study is as follows. 1. In the general features of the subject of the survey, boy students and girl students occupied 50.8% and 49.2% each, and boys' high school (34.3%), girls' high school(32.2%) and co-ed(33.5%) participated in balance. In the location, north from Han river occupied 54.6%, south were 45.2%. 2. According to the status of experiencing an accident, boys were more experienced then girls(p〈0.05). From the section, home accident(56.8%) occupied most followed by school accident, traffic accident, sports accident and poisoning. The accident happening most often in detailed category is sports accident such as basket ball, foot ball and dodge ball. The actual condition of the subject's using a school health facility shows that boys students use it more often than girl students(p〈0.01) 3. In attitude toward safety, the subject showed lower interest in safety issues than other social issues. But attitude in seriality of safety problems were high. Also, they responded ‘individual citizen’(63.1%) as the one who should make efforts for safety. Regarding knowledge of preventing safety accidents in attitude toward individual safety, 42.2% answered ‘they know a little’ and 32.6% of respondents say ‘they do not know’. To a question of the degree of the subject's following safety rules, 36.4% were answered ‘keeping’ and the group using a school health facility shows more ‘keeping’ the others(p〈0.05). 4. To a question of asking if they have experienced safety education, 51.2% answered ‘yes’. Teachers who mainly take care of safety education are answered as training teacher(48.7%). As for education time, training class(51.3%) is said to have safety education most followed by health-related event and PE(Physical Education). Frequency of education shows once or twice a session (62.8%) most often, but in case of co-ed school, 5-7times a session or more(20.1%) are being practiced. Looking at education time, 1-2 hour(s)(22.1%) or for a short time(22.1%) during class are being practiced. As an education method, instruction(43.8%) and video education(32.5%) are being practiced, and when it comes to education evaluation method, ‘not practiced’(70.0%) answered. To the question if they are satisfied with school safety education, they answered more ‘no(43.1%)’ than ‘yes(6.7%)’, and the reason is that safety education class is just for formality's sake, and the fact they already know is being repeated. The contents of safety education is composed of school safety, home safety, and first aid. 5. It is turned out that 56.5% of the total boy students and 61.1% of the total girl students recognize the necessity of safety education. To the question if safety education is needed in an elementary and middle education course, 46.4% of the subject answer answered' it's necessary'. The most reason for their answers are ‘safety education is directly related to life’. 6. Regarding the requested time of safety education is ‘one hour a week’ by 55.9%. For safety education, safety education teachers(38.7%) are answered to be the most proper. As a request for safety education, video education is answered to be the most appropriate(30.6%), followed by practical skills, lecture and discussion(p〈0.05). Demand of educational evaluation, practical skills, interview and observation are answered to be needed. To the question if they want to participate in the way of demanded safety education, 41.9% of respondents answer ‘have a mind to participate in’ (41.9%). To benefits followed by completing safety education, 72.0% of respondents answer ‘agree’, and 24.7% ‘do not agree’, which means lout 4 disagrees with completing safety education. 7. Looking at demand of safety education according to the features of the subject, ‘our position for the person who has handicapped’ was answer to the most.

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Effect of Dolomite Levels in Various Root Media Containing Micronutrient Mixes on Growth of Marigold Plug Seedlings (미량원소복합제가 혼합된 각종 상토에서 고토석회의 시비수준이 매리골드 플러그 묘의 생육에 미치는 영향)

  • Choi Jong-Myung
    • Journal of Bio-Environment Control
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    • v.15 no.3
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    • pp.217-224
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    • 2006
  • Objective of this research was to determine the effect of application rate of dolomite in root media containing micronutrient mixes on growth of marigold 'Orange boy' in plug trays. To achieve this, three granular micronutrient mixes such as HF 1, MF 2, and MF 3 were produced and incorporated into three root media, peatmoss+composted pine bark (1:1, v/v, PB), peatmoss+composted rice-hull (1:1, v/v, PR), and peatmoss+composted saw-dust (1:1, v/v, PS), with a rate of $0.3g{\cdot}L^{-1}$. Elevated application rate of dolomite in PB medium containing MF 1 decreased crop growth at 35 days after sowing with the highest fresh and dry weight in the control treatment of dolomite. The treatments of 3.0 and $6.0g{\cdot}L^{-1}$ of dolomite in PB medium containing MF 2 produced 0.133g of dry weight and 0.686g of fresh weight, respectively, which were the highest among treatments of dolomite. But the treatments of 3.0 and $6.0g{\cdot}L^{-1}$ of dolomite in PB medium containing MF 3 produced the highest fresh and dry weight, respectively. Elevated application rate of dolomite in PR medium containing MF 1, 2, and 3 resulted in decrease of fresh an dry weight. The relative growth suppression induced by elevated application rate of dolomite was more severe when MF 3 were incorporated than MF 1 and 2. The treatments of 3.0 and $6.0g{\cdot}L^{-1}$ of dolomite in PS medium containing MF 1 and 3 showed good growth, but elevated application rate of dolomite in PS medium containing MF 2 and control resulted in increase of dry and fresh weight. These results indicated that the application rate of dolomite should be adjusted by kinds of root media and micronutrient mixes.

An Analysis on Health Promotion Behavior of Middle and High School Students (중등학교 학생의 건강증진 행태와 관련요인분석)

  • 김귀희;남철현
    • Korean Journal of Health Education and Promotion
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    • v.14 no.1
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    • pp.23-45
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    • 1997
  • This study was conducted from March 1, 1996 through June 30, in order to provide basic data for devising a policy for school health especially students health promotion and for developing of an education program. Middle school students were 1000, high school students were 2000 and a total of 3000 students were selected randomly among the boys/girls/middle/high schools which are in Seoul, Pusan, Taegu, Pohang, Suwon, Kyungsan, Milyang and a town or subcountry. The summary and conclusion are as follows. 1. In general characteristics of respondents, 51.8% were girl students, 33.7% were middle school students, 66.3% were high school students. 37.2% were living in a medium and small city, 89.1% were middle classes, 43.6% were having no religion, 27.3% were buddhists. 2. As a result of analyzing, exercise, nutrition, personal behavior, knowledge of health education and behavior level which are the factors promotion, exercise level were 3.61 of the perfect 9(40.1/100), nutrition level were 3.71(41.1/100), personal hygiene were 6.89(76.6/100), health education level were 5.1(58.9/100), all of the them are low level. 3. Judging from characteristics, in case of exercise behavior level, It was far higher in boy students than in girl students, in middle school students than in high school students. It was respectively higher than other groups in the second graders of middle school, in the first graders of high school, in the residents who live in a big city, in the high classes in the buddhists. 4. The students level against disease was average 9.11 of the perfect score 14(65.1/100). The level of disease consciousness was high in girl students by characteristics, in the second graders of high school by grades, in high school students than middle school students. 5. In health status, 55.4% were healthy, 7.9% were unhealthy. It was respectively higher than the other groups in boy students, in middle school students, in the residents who live in a big city, in high classes of life level, in buddihists, in higher education level of parents. 6. Judging from the factors of health status and health promotion and the degree of significance, there's a significant differences between exercise and dietary life as P〈0.001, in personal hygiene as P〈0.05, in health education an P〈0.01. 7. Knowledge on disease, health promotion behavior level were average 19.42 ± 4.01 of the perfect score 50(38.8/100) this score was too low. As for characteristics, the level between variables was statistically significant in the higher life level, in the higher parents education level, in the happier family. 8. Judging from health status, knowledge on disease, health behavior level, knowledge and health promotion behavior level significantly in the better health status, in the better school record. 9. As a result of the multiplex regression analyzing the factors which were under influence on health status, the variables like exercise, school record level, the degree of family happiness, nutrition, grades, the members of family influenced much and its persuasive power was 10.2%. The factors which are under the influence on the health promotion were exercise, satisfied degree of education, health status, the degree of family happiness, knowledge on disease, the usage of physical training, sex, the number of the family members, mother's education level. It’s explained power was 21.3%. promotion were high We should develop a text book and an education program to study exercise, nutrition(dietary life), personal hygiene, knowledge on disease and health systematically. As far as health education irrespective city and locality without considering the entrance exam for high school and university we should execute it continuously. To do this, it’s important to cultivate and secure qualified men of ability who can teach things related health promotion and the related subject, that is, health or health promotion subject should be established in middle and high school curriculum necessarily.

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Smoking Investigation of the Sixth Year Grade-Students of Elementary School in the Rural and Small Sized Urban Area (농어촌과 중소도시 초등 학교 6년생의 흡연 실태)

  • Kim, Sang-Young;Jung, Moon-Sook
    • Journal of agricultural medicine and community health
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    • v.20 no.1
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    • pp.61-72
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    • 1995
  • To investigate the rate of smoking of 6th year grade students of elementary School by general characteristics in rural and small sized urban area, the survey was conducted during the period of Feb. 1, 1994 to Fed. 28, 1994 to 778 respondents of sixth year students of elementary school in rural area and 649 students in the small sized urban area, and also it was based on the questionnaire method. The sample consisted of 22 questions for general features of the respondents and 19 questions of smoking-ideas, totally 41 questions, which were related to the general features and the smoking oriented-factors, the real smoking fact of students, their ideas on smoking influences, and these questionnaires were statistically analysed by percentage and ${\chi}^2$-test. The results are as follows : The total respondents were 1427 students. 54.5% of respondents was of the farming, fishing area-students and 45.5% was of small, medium-sized city. Talking about the statistics of smoking, the present smoking boy-students in the farming, fishing area was marked to 14.6% ; the present smoking-boy students was marked 5.7% in the small, medium sized-city. From the viewpoint of smoking experience, 13.8% of students in farming, fishing area was shown to be experienced ; 9.7% of students in the small, medium sized-city was shown to be experienced. This indicated that there were significant difference of p<0.01 between two compared areas. From the viewpoint of family scale, in the family with many brothers, the younger brothers have a higher rate of p<0.001 of smoking-oriented than elder brother, and the students with a single parent or without parents have a higher rate of smoking on. In addition, the motivation of their smoking indicated that they had the most curiosity in smoking. From the viewpoint of academic grade, the low-level group has a higher smoking rate. Talking about the smoking knowledge of students, the present smoking students has less ideas about the smoking influences the than non-smoking students. More than 70% of the smoking-student group agreed that the education on the smoking influences was required to the elementary school students.

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Effect of Interactive Metronome® Training on Timing, Attention and Motor Function of Children With ADHD : Case Report (상호작용식 메트로놈(Interactive Metronome: IM)이 타이밍과 주의력, 운동기능에 미치는 영향: 사례보고)

  • Namgung, Young;Son, Da-In;Kim, Kyeong-Mi
    • The Journal of Korean Academy of Sensory Integration
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    • v.13 no.2
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    • pp.63-73
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    • 2015
  • Objective : To report the effects of a specific intervention, the Interactive Metronome$^{(R)}$ (IM), on timing, attention and motor function of a children with ADHD. Methods : The study is case reports about two boys with ADHD. One boy who is born 2008 is attending general elementary school as a first year student (case 1), and another boy who is born 2001 is attending general elementary school as a second year student (case 2). For each case subject, IM training was provided during 3 weeks, from January 2015 to Febrary 2015. Evaluations were performed pre- and post-intervention in order to exam timing, attention and motor skills. The measurements uses in this study are Long Form Assessment (LFA) for the timing, RehaCom screening module for the attention, and Bruininks-Oseretsky Test of Morot Proficiency, second version (BOT-2) for the motor function. Results : The timing function was improved in both cases since both showed reduced response time for all motor tasks of LFA. In terms of attention, case 1 showed improvement of visual attention division, neglect and response Inhibition, and case 2 showed improvement of sustained attention. Lastly, in the BOT-2, case 1 showed improved the percentile rank of short (from 42%ile to 96%ile), and case 2 also showed similar improvement (from 21%ile to 66%ile). Conclusion : This study provides positive evidence that the Interactive Metronome$^{(R)}$ training has positive power to facilitate several body functions such as timing, attention and motor control of children with ADHD, through two case studies.

The Study on the State of Health and dietary Habits of boy′s and Girl′s High School Students in Seoul (서울시내 남.녀 고등학생들의 식습관과 건강상태에 관한 연구)

  • 박성효;정낙원;이효지
    • Korean journal of food and cookery science
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    • v.7 no.1
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    • pp.67-80
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    • 1991
  • This study researched the dietary habit of boy's and girl's high school students, and into low it was connected with the state of health between the group of having the right dietary habit and the group of having the wrong habit. Results were as follows: 1. The age about the object person of research was the most numerous in 79% at 16~17 years old, the average weight was $55.67\pm9.08$kg, the average height was $165.47\pm7.56$cm. 2. In the parent's school career of the object person of research, persons who graduated high school were many. The mother's educational level was lower than the father's school career. The parent's school career in the district south of a river was higher than the parent's school career in the district north of a river. And the parent's school career of cultural students was high. 3. The frequency of food intake in the district north of a river and in the district south of a river was a meaningful difference in the vegetables blended in green and yellow things (p<.01), fruit (p<.05), rice, flour, potatoes (p<.05), and so the district north of a river took less than the district south of a river. 4. The most regular diet in a day was lunch. The appetite of the students was generally good. 5. The general environment and state of health were a meaningful difference about melancholia (p<.05) in the district of north and south of river, and so melancholia in students of the north of a river was high. 6. The state of health according to regular diet was a meaningful difference, so regular student was better than irregular student in state of health, and was the same in study. 7. Eating habit correlated much to each state of health, Especially in physical health, the muscular frame correlated to fruit (p<.001), seaweeds (p<.05), fried food, jun, panbroiled food (p<.05), salty taste (p<.05), sour taste(p<.001).

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Effect of Toothbrushing Facilities on PHP index and Oral Health-related Behaviors in Elementary schools (양치시설 여부에 따른 초등학생의 구강환경관리능력 및 구강건강관리 행태 변화)

  • Hwang, Yoon-Suk;Kim, Kwang-Soo;Jung, Jae-Yeon;Yoo, Young-Jae;Kim, Soo-Hwa;Lim, Mi-Hee
    • The Journal of Korean Society for School & Community Health Education
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    • v.14 no.3
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    • pp.27-40
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    • 2013
  • Objectives: This study was conducted at a request for cooperation through an analysis of the effect of toothbrushing facilities at a public health center in Seongdong-gu. Also, with the aim of furnishing basic data to the proposal of a program for improving the ability of school aged children in managing oral cavities and developing a correct toothbrushing habit, the study conducted an investigation of how toothbrushing facilities affect change in the oral environment management ability and behavior of oral health care. Methods: From among elementary schools located in Seongdong-gu, Seoul the study selected A Elementary School where toothbrushing facilities were established and have been operated since 2008, B Elementary School in an adjacent region within the jurisdiction of Seongdong-gu where the demographic environment was similar among schools where toothbrushing facilities were newly established in 2012, and C Elementary School without toothbrushing facilities. Then the study was aimed at first grade students of the schools and an investigation was carried out from April to December 2012. Through a dental checkup, the study evaluated the teeth and periodontal health conditions, and a test of the oral environment management ability was undertaken three times. Regarding change of oral health care behavior, the study carried out a self-recording survey. Results: 1. Concerning decayed and filled tooth(dft) and decayed and filled surface (dfs), A Elementary School where toothbrushing facilities have been established and operated from the past showed a relatively lower decayed, missing, and filled teeth index than B Elementary School where toothbrushing facilities were established in 2012 or C Elementary School without toothbrushing facilities; however, there was no significant difference (p>0.05). For CPI, there was no significant difference by school; however, in looking into the difference between boy students and girl students, Code (0) was discovered higher in boy students whereas Code (1) was shown higher in girl students. 2. In the PHP index test in accordance with the existence of toothbrushing facilities before the installation of toothbrushing facilities, for A Elementary School where toothbrushing facilities have been established and operated from the past, the school recorded 4.28 points whereas B Elementary School where the facilities were established in 2012 recorded 3.51 points. Meanwhile C Elementary School without the facilities posted 4.30 points. Therefore there was a statistically significant difference according to the existence of toothbrushing facilities (p<0.05). 3. In a comparison of teeth health care behavior according to the existence of toothbrushing facilities, the number of answers that the respondent did not brush their teeth after lunch over the past one week was higher in B Elementary School and C Elementary School where there were no toothbrushing facilities. Regarding the average number of brushing after lunch for one week, it was discovered higher in A Elementary School (p<0.01). 4. In change of teeth health care behavior before and after the establishment of toothbrushing facilities, the case of answering that the respondents did not brush their teeth after lunch for one week increased more after establishment than before establishment. Also the average number of teeth brushings after lunch for one week decreased further after the establishment of toothbrushing facilities; however, it did not show a significant difference (p>0.05). One of the reasons that they do not brush their teeth, "the lack of a place", decreased significantly after establishment than before establishment (p<0.05), whereas the answer, "because their friends do not brush their tooth" increased greatly after establishment than before establishment; however, there was no significant difference (p>0.05). 5. In the comparison of the degree of knowledge about dental health according to the existence of toothbrushing facilities, the degree of knowledge about dental health was shown significantly higher in A Elementary School with toothbrushing facilities than in B Elementary School and C Elementary School where there were no toothbrushing facilities (p<0.01). Conclusions: Given the above results, it is difficult to attract change in behavior only with an environmental improvement; therefore, it is deemed necessary to develop an educational program that will help children to make a habit of oral health care not only through a school but also through a related policy and financial support of government organizations as well as the construction of the basis of a systematic and consistent cooperative system with relevant organizations.

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