• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.33-43
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• 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.

• The korean journal of orthodontics
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• v.29 no.2 s.73
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• pp.267-276
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• 1999

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.

• Clinical and Experimental Pediatrics
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• v.61 no.10
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• pp.327-331
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• 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.

• Journal of Trauma and Injury
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• v.19 no.1
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• pp.47-53
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• 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.

• Journal of Oral Medicine and Pain
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• v.33 no.2
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• pp.121-132
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• 2008

To establish the diagnosis and treatment plan for skeletal Class II malocclusion, patient's skeletal morphology, prognosis as well as the treatment effect is one of the important factor to consider. Therefore, the present study classified analyzed the difference between initial(T1) and after use of activator(T2), and after finish of direct multi-bonding system treatment(T3) for Class II malocclusion during growth period according to the treatment result(effective body length) and morphology of vertical skeletal type. The experimental group was classified into two groups(1 group, 2 group) according to the effective body length change between before and after use of activator, showed good treatment effect of activator for patient with small mandible and large differential between maxilla and mandible, and short anterior facial height. And the difference between 1 and 2 group in the experimental group before treatment(T1) disappeared in the finished treatment(T3). But in contrast, the initial difference of T1 stage between a and b group in the control group did not disappear in the finished treatment(T3). In short, experimental group's treatment effect was much better than contrast group and the treatment effect was maintained and got stable results at comparison experimental group with contrast group. Through this study, we can find activator's treatment effect and stable retention of that in growing Class II malocclusion patients. By estimate of activator treatment effect through these results, we can establish the correct diagnosis and treatment plan for adolescent Class II malocclusion estimate of activator treatment effect and lead the ideal facial growth pattern.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.154-162
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• 2011

Purpose: With increasing prevalence of hypertension (HTN) in children and adolescent, pediatricians have become more interested in blood pressure (BP) measurements. The ambulatory blood pressure monitoring (ABPM) is known to be useful to differentiate true HTN and white coat HTN. The object of this study is to assess the clinical usefulness of ABPM in Korean children and adolescents. Methods: A retrospective review of 51 patients in Kyungpook National University Hospital from January 2002 to February 2010 was done. All patients were 6-18 years old and underwent ABPM. We calculated the mean value of ABP, BP load, nocturnal dip and compared the results with the patients' diagnosis and characteristics. Results: The mean age of the 51 patients was $17.8{\pm}1.8$ years and 19 children were obese. 37 patients (72.5%) were truly hypertensive and 1 patient was diagnosed as masked HTN and 7 children (14%) as white coat HTN. The rest of the patients were normotensive. Among patients with white coat HTN, 5 were in a prehypertensive state. Mean systolic and diastolic BP load of patients with true HTN were significantly higher than non-hypertensive children (P<0.001). Although the nocturnal dip of all patients were below 10%, there was no statistical significance. The obese patients showed higher systolic and diastolic BP. Their systolic and diastolic BP load were significantly higher than non-obese patients (P<0.001). Conclusion: ABPM in children and adolescents seems to be a valuable tool in the assessment of white coat HTN and in the confirmation of true HTN. A considerable number of white coat HTN patients are revealed to be in a prehypertensive state and need close follow-up.