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      • 소아 특발성 중추성 요붕증의 임상경과, 내분비학적 소견 및 뇌자기공명 영상 소견의 경과 관찰

        정윤하,정우영 인제대학교 2008 仁濟醫學 Vol.29 No.-

        Idiopathic central diabetes insipidus (CDI) can be diagnosed when it occurs in the absence of a cause known to be responsible for diabetes insipidus. Some patients initially diagnosed as idiopathic CDI progressed to brain tumors. We reviewed clinical, endocrinological and brain MRI courses in patients with pediatric idiopathic central diabetes insipidus, to assess the predicting factors for progression to brain tumors. We reviewed the medical records of all patients who had documented cases of idiopathic CDI from 1988 to 2008, and studied clinical, endocrinological and brain MRI courses at diagnosis and during follow up. The patients include 6 males and 5 females. Their mean age at diagnosis was 10.0±4.5(4-19) years and mean follow up duration is 4.3±4.7(0.3-14) years. After mean follow up duration of 4.3±4.7(0.3-14) years, follow up Brain MRI showed brain tumor in 3(27%) patients and no interval change in 4(36%) patients with pituitary stalk thickening and in 3(27%) patients with loss of posterior pituitary signal. 4(27%) patients had growth hormone deficiency and 3(27%) patients had multiple pituitary hormone deficiency. Because some patients of idiopathic CDI progressed to brain tumor, we should follow up more carefully brain MRI and pituitary hormone deficiency.

      • KCI등재

        구순구개열환자에서 자기늑골을 이용한 일차성 골이식

        김종렬,정기돈,진성준,조영철,변준호,손우성 대한악안면성형재건외과학회 2000 Maxillofacial Plastic Reconstructive Surgery Vol.22 No.5

        Alveolar bone grafting is an essential component of successful surgical rehabilitation in many common cleft deformities. Primary alveolar bone grafting is defined as alveolar bone grafting which takes place before eruption of the primary dentition or before 1 years of age. The major objective in primary alveolar bone grafting is to prevent significant maxillary segmental collapse and arch disturbances. If severe, this can make secondary rehabilitation difficult, costly, and protracted. Such early stabilization may eliminate or, at least, decrease the amount of orthodontic arch expansion required during the transitional dentition period. Furthermore, early obliteration of alveolus fistulae improves speech and dental hygiene. It is critical that abutting maxillary segmental alignment be achieved(generally when the patient is 8 to 10 months of age) before grafting. Otherwise, the amount of dissection necessary to develop mucosal flaps for bone graft coverage will be more extensive, thus risking increased scar formation with potential adverse effects on growth. We report a preliminary result of 4 cases of primary alveolar bone graft using rib bone, performed by limited dissection and onlay graft technique at 8 to 10 months of age. They showed satisfactory clinical results especially in terms of growth up to now.

      • KCI등재

        아동의 하악골에 발생한 복합 치아종의 외과적 처치에 관한 증례보고

        이긍호,최영철,정우성 大韓小兒齒科學會 1999 大韓小兒齒科學會誌 Vol.26 No.3

        악골에서 비교적 흔한 빈도로 발생하는 치아종은 치배조직의 과성장으로 인하여 발생한다.세계보건기구에서는 치아종을 복잡 치아종과 복합 치아종으로 구분하고 있다.치아종은 악골내 모든 부위에서 발생할 수 있지만 치아와 유사한 복합 치아종은 비교적 상악 전치부에 호발하고,불규칙한 형태를 나타내는 복잡 치아종은 하악 구치부에 호발한다.치아종의 원인은 정확히 알려져 있지 않으며 치배에 대한 구소적인 외상이나 감염이 주된 요인으로 추측되고 있고,최근에는 유전적인 원인에 대해서도 연구되고 있으나 아직 확실히 입증되지는 않고 있다.일반적인 증상이 없기 때문에 일상적인 방사선 검사에서 주로 발견되고 영구치의 맹출지연이나 유치의 만기잔존 등이 증상이 있을 수 있다.치아종은 구강내의 어떤 부위에서도 발생이 가능하고 드물기는 하지만 상악동,하악의 하연,하악지 및 하악과두 하방에서 발견되기도 하며,이공 부위에서 발생되는 경우도 있다. 치아종은 발육중인 치열과 악궁에 미치는 영향을 최소화하기 위하여 발견되는 즉시 낭포 및 주위 연조직을 함께 외과적으로 제거하는 것이 바람직하다.환자의 연령과 치과치료에 대한 협조도,영구치열의 발육상태,치아종의 악골내 위치 및 동시적인 치과치료가 요구되는 가를 고려하여 외래 진료실에서 하는 경우도 있고 전신마취를 통하여 하는 경우도 있다. 본 증례는 치아종의 악골내 위치가 깊지 않고 치과치료에 대한 협조도가 양호하다고 판단되는 환아에서는 외래 진료실에서 국소마취하에 수술하여 치료하였고,연령이 어리고 치아종이 악골내 위치가 깊어 장시간의 시술 시간이 요구되고 동시에 보존적인 치과치료가 필요한 환아에서는 전신마취하에 치료한 것을 보고한 것이다.두 증례에서 수술 4개월 후 외과적 결손 부위에 골이 형성되었고,매복된 영구치의 맹출이 정상적으로 이루어진 것으로 나타났다. The Odontogenic is relatively a common benign odontogenic tumor and caused by overgrowth of odontogenic tissues.The recent classification by World Health Organization divides odontoma into 2 groups such as compound odontoma and complex odontoma.Compound odontoma comprises dental tissues,resembling the morphology of a tooth and has predilection for the anterior maxilla.In contrast,complex odontoma has unorganized mass,not resembling the normal tooth and has predilection for the posterior mandible.The etiology of odontoma is unknown and almost asymptomatic.So,it usually is found in routine radiographic examination,and most common presenting symptom is impacted or unerupted permanent teeth and ratained primary teeth.It can occurs almost anywhere in jaws. It is desirable that odontoma should be removed by surgical enucleation including follicle and surrounding soft tissues.Considering the age and behavioral cooperation of patient,the develoment of parmanent dentition,the location of odontoma in jaw,the need for the concomitant operative dentistry,operation is performed in outpatient department with/without sedation or under general anesthesia with endotracheal intubation. In this case report,2 patients with compound odontoma were treated by surgical enucleation including follicle and surrounding soft tissues.One patient,about 5years old,was treated under general anesthesia and concomitant operative density was performed.The other patient,about 11years old,was treated under local anesthesia in outpatient department.In 2 cases,after 4 months,surgical defects were filled with new bone and normalization of eruption path of impacted permanent teeth was observed.

      • 소아에서 성장호르몬 결핍증 진단을 위한 혈중 IGF-1과 IGFBP-3의 절단값

        손미란,안영선,정우영,유병철 고신대학교의과대학 2007 고신대학교 의과대학 학술지 Vol.22 No.2

        Background : It is essential to have reference criteria for the diagnosis of growth hormone deficiency in children. The objective of study was to decide the cut-off value for Insulin-like Growth Factor-Ⅰ(IGF-Ⅰ) and Insulin-like Growth Factor Binding Protein-3(IGFBP-3) for diagnosis of growth hormone deficiency. Methods : We studied the serum level of IGF-Ⅰ and IGFBP-3 in 231 subjects(131 boys and 100 girls) with idiopathic short stature(ISS) and 37 subjects(23 boys and 14 girls) with complete or partial growth hormone deficiency(GHD). IGF-Ⅰ were measured by radioimmunoassay(RIA) method and IGFBP-3 were measured by immnoradiometric assay(IRMA) method. We conducted chi-square test, Fisher's exact test, student t-test, receiver operating characteristic(ROC) curve and discriminant anaylsis using MedCalc and SPSS Program. Results : The ROC curves showed that the best IGF-Ⅰ and IGFBP-3 cut-off value. Cut-off value of serum IGF-Ⅰ concentration was 346ng/mL(sensitivity 86.49%, specificity 85.71%) and of serum IGFBP-3 concentration was 3196ng/mL(sensitivity 81.82%, specificity 81.66%). If discriminant value (Z=0.442+0.661×IGF-Ⅰ+0.469×IGFBP-3) was below -1.47, the subject is discriminated GHD respectively. Conclusion : Our study results support that the measurement of serum IGF-Ⅰ and IGFBP-3 concentration might be an essential supplementary tools in the diagnostic evaluation of children with GHD.

      • KCI등재후보

        8년간(1992~1999) 산업안전보건연구원에 의뢰된 직업병 심의 사례 분석

        강성규,김규상,김양호,최정근,안연순,진영우,최병순,양정선,김은아,채창호,최용휴,김대성,박정선,정호근 대한산업의학회 2000 대한직업환경의학회지 Vol.12 No.2

        Objectives : Pneumoconisis and noise-induced hearing loss (NIHL) have been reported as main occupational diseases by the Special Health Examination. The Industrial Accident Compensation Insurance has reported various work-related diseases, however, these two diseases occupied almost a half of compensated cases. Therefore, it was not well known about the status of occupational diseases other than pneumoconiosis, NIHL, and cardio-cerebrovascular accident (CVA). This study was conducted to analyze claimed cases as an occupational disease, that was requested to the Korea Industrial Safety and Health Agency (KOSHA). Methods : The local office of the Korea Labor Welfare Corporation (KLWC) has asked the KOSHA for confirmation of claimed cases as an occupational disease. We analyzed 379 cases requested from KLWC, the Ministry of Labor, employers, unions and occupational health agencies from 1992 to 1999. Results : Male was 80.7 % of the requested cases. Their mean age was 42 years old and 75.5 % of them were more than 35 years old. The requested cases were increased rapidly from 25 cases in 1992 to 108 cases in 1999 and the accept rate was 50.7 %. The majority of requested cases were respiratory diseases(22.4%), cancers(18.5%), Neuropsychiatric problems(14.5%), and musculoskeletal problems(13.5%). The accept rate was high in reproductive, respiratory, musculoskeletal and digestive disorders and low in neuropsychiatric, renal and otologic problems and occupational cancers. 73.6% of them were caused by chemical agents, especially 28.5 % were by organic solvents. 67% of them were clinically confirmed at university hospitals. A half of the cases were from KyongIn area, even the request came from the whole country. Conclusions : A claim was common in workers whose age was over 35 years old and exposure history was over 10 years. The respiratory diseases and neuropsychiatric disorders were still main problems in occupational health and occupational cancers was increasing even though its accept rate was not high yet.

      • KCI등재

        A 10-year-old Boy with Microscopic Hematuria and Renal Biopsy Findings Mimicking Fabry Disease

        Woo Yeong Chung,Mi Seon Kang 대한소아신장학회 2016 Childhood kidney diseases Vol.20 No.2

        Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A, resulting in the accumulation of glycosphingolipids within the lysosomes of various cell types. It has a wide spectrum of clinical pheno types, and renal failure is a serious complication. Fabry disease is confirmed either by measurement of α-galactosidase A activity or by genetic testing for GLA muta tions. Renal biopsy findings on light microscopy, specifically enlarged podo­cytes with foamy cytoplasm, and osmiophilic inclusion bodies in the cytoplasm in all types of renal cells on electron microscopy, are characteristic of this disease. The predominant differential diagnosis is iatrogenic phospholipidosis in association with certain drugs that can cause cellular injuries indistinguishable from Fabry disease. Here, we report the case of a 10-year-old boy with microscopic hematuria who underwent a renal biopsy that showed morphological findings consistent with Fabry disease, although the patient had neither a GLA mutation nor a history of drug consumption. Six years later, spontaneous regression of this renal patho logy was observed in a second renal biopsy examination.

      • SCOPUSKCI등재

        Impact of acute kidney injury in deceased donors with high Kidney Donor Profile Index on posttransplant clinical outcomes: a multicenter cohort study

        ( Woo Yeong Park ),( Yoon Kyung Chang ),( Young Soo Kim ),( Kyubok Jin ),( Chul Woo Yang ),( Seungyeup Han ),( Byung Ha Chung ) 대한신장학회 2021 Kidney Research and Clinical Practice Vol.40 No.1

        Background: This study evaluated the impact of acute kidney injury (AKI) on posttransplant clinical outcomes for deceased donor (DD) kidney transplantation (KT) using the Kidney Donor Profile Index (KDPI) system. Methods: Overall, 657 kidney transplant recipients (KTRs) receiving kidneys from 526 DDs from four transplant centers were included. We divided them into the high and low KDPI donor groups by 65%, the KDPI score, and both groups were subdivided into the AKI-DDKT and non-AKI-DDKT subgroups according to AKI in DDs. Results: There was no significant difference in the incidence of delayed graft function (DGF) between the high and low KDPI-KTR groups; however, the AKI-DDKT subgroup showed significantly higher incidence of DGF than the non-AKI-DDKT subgroup in both groups (p = 0.001, p < 0.001, respectively). The death-censored graft survival rate was significantly lower in the high KDPI-KTR group than in the low KDPI-KTR group (p = 0.005). Only in the high KDPI-KTR group, the death-censored graft survival rate was significantly lower in the KT from DDs with AKI stage 3 than KT from DDs with non-AKI or AKI stage 1 or 2 (p = 0.040). The interaction between AKI stage 3 in DDs and high KDPI on the allograft outcome was significant (p = 0.002). Conclusion: KTs from DDs with AKI stage 3 showed an adverse impact on the allograft outcome in the high KDPI-KTR group. Therefore, DDs with a high KDPI score should be managed carefully so that severe AKI does not occur prior to KT.

      • KCI등재후보

        A 10-year-old Boy with Microscopic Hematuria and Renal Biopsy Findings Mimicking Fabry Disease

        Chung, Woo Yeong,Kang, Mi Seon Korean Society of Pediatric Nephrology 2016 Childhood kidney diseases Vol.20 No.2

        Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme ${\alpha}-galactosidase$ A, resulting in the accumulation of glycosphingolipids within the lysosomes of various cell types. It has a wide spectrum of clinical phenotypes, and renal failure is a serious complication. Fabry disease is confirmed either by measurement of ${\alpha}-galactosidase$ A activity or by genetic testing for GLA mutations. Renal biopsy findings on light microscopy, specifically enlarged podocytes with foamy cytoplasm, and osmiophilic inclusion bodies in the cytoplasm in all types of renal cells on electron microscopy, are characteristic of this disease. The predominant differential diagnosis is iatrogenic phospholipidosis in association with certain drugs that can cause cellular injuries indistinguishable from Fabry disease. Here, we report the case of a 10-year-old boy with microscopic hematuria who underwent a renal biopsy that showed morphological findings consistent with Fabry disease, although the patient had neither a GLA mutation nor a history of drug consumption. Six years later, spontaneous regression of this renal pathology was observed in a second renal biopsy examination.

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