Yoo, Eun Jung;Kim, Eun Young;Kook, Hoon;Woo, Young Jong
Journal of The Korean Society of Inherited Metabolic disease
/
v.5
no.1
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pp.33-43
/
2005
Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.
Lee, Jong Yoon;Lee, Ye Seung;Choi, Joong Wan;Bae, Eun Joo;Park, Won Il;Oh, Phil Soo;Lee, Hong Jin
Journal of The Korean Society of Inherited Metabolic disease
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v.12
no.2
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pp.85-93
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2012
Purpose: Developmental delay is caused by very diverse etiologic diseases. Most chronic disorders has some influence on development. Chronic or acute disorders of CNS are main etiologic diseases of developmental delay. Up to now, over 60 diseases are included in organic acidopathies and most of them causes acute or chronic recurrent CNS damage and developmental delay. We have done this study to find out the importance of organic acidopathies causing developmental delay in Korean childhood and adolescent patients. Method: Retrograde analysis for 738 patients with developmental delay whose clinical informations are available and have done urine organic acid analysis for 5 years period, between Jan. 1st 2007 to Dec. 31th 2011. Statistical analysis was done with Student's t test using SPSS. Result: Out of 738 patients, 340 patients (46.1%) showed abnormalities on urine organic acid analysis. The most frequent disease was mitochondrial respiratory chain disorders (MRCD) (253, 34.3%), followed by ketolytic defects(39, 5.3%), 3-hydroxyisobutyric aciduria (26, 3.5%), glutaric aciduria type II (8, 1.1%), pyruvate dehydrogenase deficiency (3, 0.4%), 3-methylglutaric aciduria (2, 0.3%), glutaric aciduria type I (2, 0.3%), ethylmalonic aciduria (1, 0.15%), methylmalonic aciduria (1, 0.15%), HMG-CoA lyase deficiency (1, 0.15%), 3-methylcrotonylglycinuria (1, 0.15%), fatty acid oxidation disorders(1, 0.15%) and FAOD (1, 0.15%). Conclusion: Mitochondrial disorders are most frequent etiologic disease on all age group, followed by ketolytic defects and various organic acidopathies. The number and diversities of organic acidopathies emphasize meticulous evaluation of basic routine laboratory examinations and organic acid analysis with initial sample on every developmental patient.
Objective : Premenstrual syndrome (PMS) and premenstrual dysphoric disorder (PMDD) is known to reduce the quality of sleep and is associated with various psychiatric disorders. The aim of this study was to investigate the relationship of PMS with depression, sleep qulity and sleep pattern in Korean female adolescents. Methods : Out of 867 high school female students, 400 female subjects were included in this study. All participants completed self-report questionnaires that included demographic variables, shortened premenstrual assessment form (SPAF), patient health questionnaire-9 (PHQ-9), Pittsburgh sleep quality index (PSQI), Epworth sleepiness scale (ESS), insomnia severity index (ISI) and Korean translation of composite scale (CS). A total of 394 individuals who completed the questionnaires were analyzed. Results : As score of SPAF, 143 subjects was defined PMDD group. They had more prevalence of eveningness, more depressive symptom, poorer sleep quality, and more excessive sleepiness than non-PMDD groups. Although the PMDD groups showed more menstrual dysmenorrhea, no difference was found in bleeding duration and cycle. PMS symptom had correlations with depression, poorer sleep quality and excessive sleepiness during the daytime. There was no difference in sleep duration between two groups, but PMS might have a relationship with eveningness of chronotype. Conclusions : Among Korean female high school students, the PMS might have an association with depression and poor sleep quality, and it might lead to daytime impairment due to excessive sleepiness. An appropriate evaluation of PMS could be helpful in determining the mental health condition of adolescents.
Distributions trends were examined in 9159 malocclusion patients who had been examined and diagnosed at Department of Orthodontics, College of Dentistry Yonsei University over a 6 year-period from 1992 to 1997. The results were as follows ; 1. The total number of orthodontic patients in 1997 increased in comparing with that of 1992(86%) and after 1995, the number of annual patients showed an increase. 2. Age distribution had shown over-19 year-old group being the largest(39.9%) but percentages of pre-adolescent patients had been decreasing while that of adult patient had gradually increased. 3. Distributions in the types of malocclusion, the Angle's Classification had shown : 33.3% for Class I, 28.6% for Class II and the largest 38.1% for Class III. 4. Among Class I patients, crowding showed the largest(32.6%) and protrusion group had gradually increased to 33.9% in 1996. Otherwise openbite and crossbite groups tend to decrease, annually. 5. Geographic distribution showed a majority of patients from Seoul(above 70%). Among the patients from Seoul, group within the distance 3-6Km from Yonsei dental hospital was the largest(32.3%) and group within 9Km showed 69% of total patients.
Background : Since 2001 the Korean Association of Health Promotion has attempted to establish her own reference valves that can be used in her all fifteen branch laboratories instead of using those adapted from the published data or there commended data by the reagent companies supplied as inserts. However, the previous two reference values derived from the statistics(year 2001 and 2002) were need to adjust to apply to actual practice. Besides there was an unavoidable situation that the reagent has to be changed to other companies in 2002 that creates another statistical problem. Subsequently, the third attempt to derive the reference ranges of tests in KAHP to solve those problems and define common)v acceptable reference ranges was done and and reported here. Methods : Al1 the results performed during January 2, 2003 through September 30, 2003 were collected in Excel tile format. All the data include dthe necessary information such as age and sex. The age was grouped in six; baby(0-3y), children(4-l2y), adolescent(13-l8y), adult(19-S4y), younger elderly (65-79y),old elderly(oyer 80y), with references of statistics in medical informatics and WHO classification. The data were statistically analyzed with SAS 6.04 for-Gaussian distribution as the previous two occasions. None of the tests showed Gaussian distribution. These procedures had been repeated twice or three times after trimming out the results lying outside three standard deviations. Though, all the tests showed non-Gaussian distribution. Subsequently, the reference ranges were defined in the range from the point of lower 2.5% to the point of higher 97.5 %. And in case the lower range could be "0", the reference ranges were defined in the range of 0 to 95%.Results : The reference ranges of most of 56 test items were newly assigned. Also with adaptation of the recommendation of WHO etc. on fasting blood sugar, hemoglobin, cholesterol. Among these there were eight tests that needed reference ranges by the age groups and nine tests by the sex. Conclusions : The third attempt will credit more the reference range of all15 laboratories of Korean Association of Health Promotion, which will be essential part of the better service to the patient and clients to visit KAHP.
Kim, Ki-Baek;Kim, Seon-Mi;Choi, Nam-Ki;Yang, Kyu-Ho
Journal of the korean academy of Pediatric Dentistry
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v.34
no.3
/
pp.454-460
/
2007
Traumatic dental injuries in childhood and adolescent occurred more frequently than in adult. The time between the accident and the treatment is one of the most critical factors to prognosis, and because of the limited time available to examine and treat patients with traumatic dental injuries, if not treat appropriately, the result would be critical for the patient. In the previous studies, the prevalence and incidence of traumatic injuries were the most frequent at the age of 8 to 10 years, the majority of dental injuries involve the anterior teeth, especially the maxillary incisors, and males were more prevalent than females in an approximated proportion of 2:1. As the mean age of complete root formation is 10 years old, the maxillary permanent incisor involved in the most affected age group is usually immature, and the possibility of pulpal healing through excellent revascularization exists, more positive prognosis for pulp vitality would be expected. These are treatment cases of the immature maxillary permanent central incisor involved in the traumatic injury, and reports for progress and results of preserving the pulp vitality through the conservative treatment instead of the conventional endodontic root therapy.
Journal of Dental Rehabilitation and Applied Science
/
v.36
no.1
/
pp.12-20
/
2020
Purpose: This study was designed to analyze the relationship between the presence and severity of depression and chewing problems (CPs) in a representative sample of the general population. Materials and Methods: Health surveys and examinations were conducted on a nationally representative sample (n = 8150) of Korean was conducted. CPs was determined by a simple survey response concerning "Do you feel uncomfortable about chewing your food because of problems with your mouth such as teeth, dentures and gums?" Depression was defined as individuals with a total score ≥ 10 on the Patient Health Questionnaire (PHQ)-9 survey. Data regarding demographics, socioeconomic history and comorbid health conditions were used to analyze adjusted logistic regression models. Results: In the Korean population, the prevalence of depression was significantly greater in individuals with CP (17.2%) than in those without CP (10.2%). On multivariate logistic regression analysis, the presence of depression was significantly associated with CPs (adjusted odd ratio [aOR]: 1.90, P < 0.001). The risk of CPs increased with increasing severity of depression as follows: severe depression (OR: 2.62, P < 0.001), moderately severe depression (OR: 2.19, P < 0.001). Conclusion: The presence of depression was significantly associated with CPs, especially in severely depressed individuals. Depression screening should be considered in treating CP patients.
Jang, Hea Min;Baek, Hee Sun;Kim, Jung-Eun;Kim, Ju Young;Lee, Yeon Hee;Cho, Hee Yeon;Choe, Yon Ho;Kang, Ben;Choe, Byung-Ho;Choi, Bong Seok;Cho, Min Hyun
Clinical and Experimental Pediatrics
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v.61
no.10
/
pp.327-331
/
2018
Purpose: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. Methods: We retrospectively reviewed the medical records of 456 children and adolescents aged <20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. Results: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. Conclusion: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.
Kim, Suk Hwan;Choi, Kyung Ho;Choi, Se Min;Oh, Young Min;Seo, Jin Sook;Lee, Mi Jin;Park, Kyu Nam;Lee, Won Jae
Journal of Trauma and Injury
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v.19
no.1
/
pp.47-53
/
2006
Purpose: Recently, the American Heart Association recommended that routine cervical spine protection in submerged patients was not necessary, except in high-energy injury situations. However, until now, this recommendation has few supportive studies and literatures. This retrospective study was performed to demonstrate the risk of cervical spine injury in patients who had been submerged in a river. Methods: Seventy-nine submerged patients who visited St. Mary's Hospital between January 2000 and December 2005 were included in this retrospective study. We investigated and analyzed the victim's age, sex, activity on submersion, mental status and level of severity at admission, prognosis at discharge, associated injuries, and risk group by using the medical records and cervical spine lateral images. According to the activity on submersion, victims were classified into three groups: high risk, low risk, and unknown risk. The reports of radiologic studies were classified into unstable fracture, stable fracture, sprain, degenerative change, and normal. Results: The patients' mean age was 36.8 yrs, and 54% were males. Of the 79 patients, adult and adolescent populations (80%) were dominant. Jumping from a high bridge (48%) was the most common activity on submersion and accounted for 52% of the high-risk group. The Glasgow coma scale at admission and the cerebral performance scale at discharge showed bimodal patterns. The results of the radiologic studies showed one stable fracture, one suspicious stable fracture, and 18 sprains. The incidence of cervical spine fracture in submerged patients was 2.5% in our study. The incidence of cervical spine injury was higher in the high-risk group than it was in the low-risk group, especially in the jumping-from-high-bridge subgroup; however this observation was not statistically significant. No other factors had any significant effect on the incidence of cervical spine injury. Conclusion: Our study showed that even submerged patients in the high risk group had a low incidence of cervical spine fracture and that the prognosis of a patient did not seem to be influenced by the cervical spine fracture itself.
Background: Smartphone addiction has recently been highlighted as a major health issue among adolescents. In this study, we assessed the degree of agreement between adolescents' and parents' ratings of adolescents' smartphone addiction. Additionally, we evaluated the psychosocial factors associated with adolescents' and parents' ratings of adolescents' smartphone addiction. Methods: In total, 158 adolescents aged 12-19 years and their parents participated in this study. The adolescents completed the Smartphone Addiction Scale (SAS) and the Isolated Peer Relationship Inventory (IPRI). Their parents also completed the SAS (about their adolescents), SAS-Short Version (SAS-SV; about themselves), Generalized Anxiety Disorder-7 (GAD-7), and Patient Health Questionnaire-9 (PHQ-9). We used the paired t-test, McNemar test, and Pearson's correlation analyses. Results: Percentage of risk users was higher in parents' ratings of adolescents' smartphone addiction than ratings of adolescents themselves. There was disagreement between the SAS and SAS-parent report total scores and subscale scores on positive anticipation, withdrawal, and cyberspace-oriented relationship. SAS scores were positively associated with average minutes of weekday/holiday smartphone use and scores on the IPRI and father's GAD-7 and PHQ-9 scores. Additionally, SAS-parent report scores showed positive associations with average minutes of weekday/holiday smartphone use and each parent's SAS-SV, GAD-7, and PHQ-9 scores. Conclusion: The results suggest that clinicians need to consider both adolescents' and parents' reports when assessing adolescents' smartphone addiction, and be aware of the possibility of under- or overestimation. Our results cannot only be a reference in assessing adolescents' smartphone addiction, but also provide inspiration for future studies.
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