• Childhood Kidney Diseases
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• v.13 no.2
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• pp.222-228
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• 2009

Purpose : We checked voiding cystourethrography (VCUG) in patients with congenital hydronephrosis (CHN) and followed up the occurrence of urinary tract infection(UTI) in these patients. And we tried to figure out the relationship between CHN and VUR and UTI. Methods : We performed a retrospective study in 122 CHN patients who received VCUG from 2002 to 2008 at Ajou University Hospital. We executed logistic regression, chi-square test and T-test using version 16.0 SPSS package for the statistical analysis. P<0.05 was regarded as significant. Results : Most patients with CHN were male (73%) and there was a tendency of preferences for unilateral (77%) and left (84%) occurrence of hydronephrosis. The incidence of VUR was 18.9% among 122 CHN patients and no laterality difference was found to the severity of VUR or hydronephrosis. The incidences of VUR and UTI was higher in group of higher grade (III-IV) CHN patients, when compared to the lower group, respectively. When VUR was present in CHN patients, the incidence of UTI during infancy was also higher. Conclusion : The incidence of VUR was higher in patients with higher grades of CHN. The incidence of UTI during infancy was higher when VUR was present in the CHN patients.

• The Korean Journal of Pharmacology
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• v.30 no.1
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• pp.75-86
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• 1994

To assess the role of adrenal medulla and renin-angiotensin system in the regulation of sympathetic neurotransmission, the pressor response to PNS was evaluated in pithed SHR and normotensive WKY or SDR with or without adrenal demedullation and/or enalapril pretreatment. Three weeks after adrenal demedullation, MAP and the heart rate of demedullated rats were similar to their corresponding sham-operated groups. The pressor response to PNS was frequency-dependent, and blocked by prazosin. In contrast to the normotensive rats, in SHR, the pressor response to PNS was attenuated in demedullated rats as compared with sham-operated rats. However, the attenuation of PNS-induced pressor responses in demedullated SHR was not observed in enalapril-treated SHR. The adrenal demedullation in SHR did not affect the plasma and aortic catecholamine contents in spite of the decreased catecholamine contents of adrenal gland, nor ACE activity in aortic strips. But, in WKY rats, the aortic catecholamines, especially epinephrine, contents as well as ACE activity were increased by adrenal demedullation. These results suggest that the facilitatory role of adrenal medulla in sympathetic neurotransmission depends upon the activation of renin-angiotensin system, and that the compensatory regulation of renin-angiotensin system takes place in normotensive rats but not in SHR.

• Journal of Genetic Medicine
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• v.5 no.2
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• pp.111-118
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• 2008

Purpose: Neonatal screening tests are increasingly being used forearly diagnosis of inborn errors of metabolism (IEM) in the hope of avoiding the severe developmental delay, acute illness, and death that may result from these diseases. In this study, a cost-benefit analysis was performed on the neonatal screening of maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia in Korea. Materials and Methods: This study included 1,259,220 Korean newborns born between January 2005 to December 2007, who were screened for maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia. We calculated and compared the total costs in cases where these four screening tests were implemented, and those where they were not. Results: There were no benefits to screening for maple syrup urine disease or homocystinuria due to their low prevalence for these two tests, the costs exceeded the benefits at benefit:cost ratios of 0.5:1 and 0.6:1, respectively. In contrast, benefits far exceed costs at a ratio of 4.1:1 for galactosemia and 2.9:1 for congenital adrenal hyperplasia. The average benefit:cost ratio for all four tests was 2.0:1. Conclusion: Neonatal screening tests for maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia are financially viable.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.2
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• pp.208-215
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• 2000

Patient with congenital heart disease are susceptible to infective endocarditis, and bacteremia following dental procedures may lead to infective endocarditis is these patients. Therefore prophylactic antibiotics are recommended for patients with congenital heart disease who are undergoing dental procedures that are associated with infective endocarditis. In 1997 American Heart Association revised guidelines for a prophylaxis against infective endocarditis. The new American Heart Association recommendations for the prevention of infective endocarditis represent a substantial departure from past guidelines. Major change involve the indications for prophylaxis, antibiotic choice and dosing that may reduce bacteremic risk. Previously, antibiotic prophylaxis was suggested for dental procedures associated with any amount of bleeding. Now only those that are associated with significant bleeding are recommended for prophylaxis as dictated by clinical judgement. Recommended antibiotic prophylaxis regimens now consist of a single preprocedural dose, no second dose is recommended. This report presents three cases of dental treatment of patients with congenital heart disease under the most recent American Heart Association recommendations for antibiotic prophylaxis.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.2
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• pp.210-216
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• 2003

The double teeth include gemination and fusion. The congenital missing tooth is the absence of the tooth. The purpose of this study was to investigate the prevalence of double teeth and congenital missing teeth in preschool children of Iksan city. The study population consisted of 1,031 children, 569 boys and 462 girls, aged from 17 to 84 months. The double teeth possess a variety of diagnostic and treatment problems. Gemination is the partial splitting of a single bud into two distinct entities that remain joined in a Siamese twin fashion. Fusion is the joining of two buds. Both may be normal, or one may be a supernumerary tooth. Congenital missing is the absence of one or a few teeth. Twenty three(11 boys and 12 girls) of the 1,031 children had double teeth and 17(8 boys and 9 girls) exhibited congenital missing of teeth. The prevalence of double primary teeth was 2.2%. The prevalence of congenital missing teeth was 1.6%. One subject had double teeth and congenital missing tooth at the same time. Of the 17 cases of congenital missing teeth, one girl showed it in the maxilla and aye boys and six girls had them in the unilateral side of the mandible. There were no significant differences in the prevalence of double teeth and congenital missing teeth between the boys and the girls.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.190-194
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• 2010

Purpose : To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. Methods : Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter and shimmer) were analyzed using a multi-dimensional voice program. Results : The average fundamental frequency showed a significant decrease in patent ductus arteriosus, ventricular septal defect, and tetralogy of Fallot, except in atrial septal defect and pulmonary stenosis. The length of the analyzed sample (duration) did not show a significant difference when compared with the control group. There was a significant increase in jitter percent in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. There was an increase in shimmer in ventricular septal defect, patent ductus arteriosus, and atrial septal defect. The noise-to-harmonic ratio increased in ventricular septal defect, patent ductus arteriosus, and atrial septal defect but there was no significant difference in pulmonary stenosis and tetralogy of Fallot. While analyzing acoustic variables, the voice change was significantly higher, especially in patent ductus arteriosus followed by ventricular septal defect and atrial septal defect. Most of these acoustic variables were deviant in left-to-right shunt lesions in congenital heart disease, especially in patent ductus artriosus. Conclusion : The results of the voice change analysis of preoperative children with congenital heart disease revealed that the acoustic variables differed by each congenital heart disease. Moreover, the acoustic variables were prominently deviant in congenital heart disease with left-to-right shunts.

• Korean Journal of Cleft Lip And Palate
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• v.13 no.1
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• pp.1-10
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• 2010

구순구개열은 선천성 악안면 기형중에서 발생률이 가장 높은 질환이며 특히 동양인에게 높은 빈도로 발생된다. 그리고 순구개열에서는 파열부 조직의 선천적 상실과 파열부 봉합 수술 후 형성된 반흔에 의한 상악골의 열성장과 상악궁의 협착, 코의 형태 이상, 등과 함께 치아의 수, 크기와 형태 및 맹출 이상도 높은 빈도로 동반된다. 선천성 치아 발육 이상이 구순열 또는 구개열 가진 환자에게서 종종 보고되고 있다. 이런 치아 이상은 과잉치, 결손치, 크기, 형태, 맹출시기, 법랑질 광화 등의 많은 특징을 포함한다. 이번 연구의 목적은 다음과 같다. 1. 구순열 또는 구개열을 가진 환자의 선천성 치아 결손 발병률을 결정하여 정상인과 비교하는 것이다. 2. 상하악에서 파열이 있는 부위와 없는 부위의 제2소구치 결손가능성을 비교 하는 것이다. 구강악안면외과에서 구순구개열로 진단한 환자 중 2005년 1월~2009년 8월까지 전북대학교 구강악안면외과에서 치조열부위 자가골이식수술을 받은 32명의 환자로 초진시의 교정 chart, 구강악안면외과 chart, x-ray(파노라마, 치근단사진, 교합사진(상악), 석고 모델, 구강내 외 임상사진을 사용하여 순구개열군 별로 매복치, 과잉치, 결손치, 왜소치의 유무와 위치를 조사하여 다음과 같은 결론을 얻었다. 1. 결손치는 비교적 높은 발생빈도(53.1%)를 보였으며, 치아별 발생빈도는 상악 측절치와 상악 제2소구치, 하악 제2소구치 순이었다. 구순구개열군이 구순치조열군에 비해 발생률이 높게 나타났다. 그리고 구순구개열군 내에서 양측성이 편측성에 비해 결손치의 발생률이 높게 나타났다. 2. 왜소치는 71.6%에서 발견되었으며, 치아별 발생빈도는 상악측절치에서 가장 많았다. 구순치조열군이 구순구개열군에 비해 발생률이 높게 나타났다. 3. 치조열을 가진 환자에 있어 상/하악간 제2소구치의 선천적 결손에 유의한 차이가 있었다. 4. 구순구개열이 인접한 상악측절치의 발생중인 미성숙 조직에 영향을 미치며 파열부위와 좀 더 떨어져 있는 상악 제2소구치에도 일정한 영향을 미침을 이번 연구를 통해 다시 확인할 수 있었다.

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.1
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• pp.122-130
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• 2006

Congenital Missing Teeth(hypodontia, oligodontia) is the developmental absence of one or more teeth. It has been reported as being the most common anomaly of dental development in human, relatively common in the permanent dentition. In a recent review, Vastardis has quoted incidence ranges of $1.6%{\sim}9.6%$ in the permanent dentition. Brook has quoted a prevalence of $3.5%{\sim}6.5%$ in most populations, with severe hypodontia, defined as the absence of six or more teeth, having a prevalence of $0.3{\sim}0.4%$. The most commonly affected teeth are third molars, followed by maxillary lateral incisor, and second premolars. The etiology is unknown, several hypotheses include trauma, nutritional deficiency, infection, metabolic abnormalities, systemic disease and genetic influence. The multiple congenital missing is commonly associated with specific syndrome or severe systemic abnormalities such as cleft lip & palate and Down's syndrome. These cases present that children have multiple congenital missing teeth in the permanent dentition, without any systemic disease. Management of this condition must be considered orthodontic and prosthodontic treatment comprehensively. In these cases, children were treated by space maintainer or orthodontic appliance and follow-up checked.

• Journal of the Korean Orthopaedic Association
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• v.53 no.6
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• pp.530-539
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• 2018

Purpose: We aimed to evaluate the magnetic resonance imaging (MRI) findings of congenital postaxial polydactyly of the foot. Materials and Methods: Three-hundred and forty-seven feet of 288 patients who underwent congenital postaxial polydactyly or polysyndactyly correction were divided into five subtypes according to the radiographic shapes of deformity origins (widened metatarsal head, bifid, fused duplicated, incompletely duplicated, or completely duplicated). MRIs were assessed to determine whether they unrevealed areas were fused or separated. MRI was also used to assess cases with radiographic phalangeal aplasia. Results: Huge variations were noted in MRIs. Fusion or separation at the base or head between original and extra digits were observed, and MRI effectively depicted phalangeal aplastic areas. Conclusion: MRI evaluations of congenital postaxial polydactyly of the foot are useful for determining the anatomical statuses which were not visualized by plain radiography (level of evidence: 3).

• Proceedings of the KSCN Conference
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• 2005.04a
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• pp.14-24
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• 2005

선천성(유전성) 대사이상은 유전적인 원인으로 인해 단백질, 탄수화물, 지방 등의 대사에 이상이 생겨 정상적인 영양소 대사가 이루어지지 않는 질병이다. 대부분의 대사이상은 심각한 임상적 문제를 초래하며 출생직후 발견되므로 빠른 진단과 치료가 필수적이다(Alluri and Mulvihill 2002). 대사이상 질환은 매우 드물게 발생하고 자세한 주의 관찰을 요하므로 환자마다 영양치료로서 개별적인 관리가 필요하다(Enns and Packman 2002). 대사이상 환자의 생존율이 증가하면서 임상적으로 성장기 이후의 문제와 임신, 출산까지 관리에 대한 필요성이 점차 증가하고 있다(Radomyska 2003).