• Childhood Kidney Diseases
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• 제18권1호
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• pp.51-55
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• 2014

Primary renal artery aneurysm has been estimated to account for an incidence of 0.015-1% with associated morbidities including renovascular hypertension and rupture. Renovascular hypertension associated renal artery aneurysms in children is not a common disease. In patients with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be hazardous in these situations. We report a case of a renal artery aneurysm in a 13-year-old Korean child presenting hypertension detected during school health examination. Preoperative workup demonstrated a $2.8{\times}2.1{\times}1.9$ cm saccular aneurysm in the right renal hilum that was not amendable to endovascular repair. A surgical strategy including extracorporeal renal artery reconstruction with autotransplantation was applied in order to restore renal artery anatomy and to treat renovascular hypertension. Immediately he complained of severe right flank pain and postoperative doppler sonography revealed lack of perfusion. On the 5th day after autotransplantation, the patient underwent a transplant nephrectomy. He was well postoperatively and was found to have a normal kidney function and stable blood pressure control without antihypertensive medication. This is the first pediatric case of renal artery aneurysm in Korea who underwent extracorporeal repair followed by autotransplantation failure. More pediatric cases with renal artery aneurysm should be reported to identify therapeutic outcome and long term prognosis.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.88-92
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• 2008

Infection of Epstein-Barr virus(EBV) gives rise to a broad spectrum of clinical manifestations in children. Although renal involvement is rare, diverse renal manifestations are known from hematuria to acute renal failure. Secondary membranous nephropathy(MN) associated with systemic EBV infection is an uncommon renal pathology and only two cases have been reported. We are adding another case of MN associated with EBV infection in a child. An 8-year-old girl was admitted for renal biopsy. She had been followed up for microscopic hematuria and intermittent proteinuria for 5 months. There had been no specific findings in serology and radiology. Tonsil biopsy had been done due to exudative tonsillar hypertrophy and enlarged multiple cervical lymph nodes. And it showed EBV-associated lymphoproliferative findings. Serologic tests for EBV showed positive evidence of recent infection; viral capsid antigen(VCA) IgM was borderline positive, VCA IgG and early antigen IgG were positive, and EB nuclear antigen IgG was negative. In Situ Hybridization of tonsil for EBV mRNA was positive. Because her proteinuria and hematuria were aggravated at that time(protein 3 +, RBC >60/HPF), renal biopsy was done. Renal biopsy showed the findings of MN, characterized by thickened capillary walls with epimembranous spikes on light microscopy and subepithelial, mesangial and subendothelial electron dense deposits on electron microscopy. On immunofluorescence microscopy, IgG, C1q, kappa and lambda chains were positive. After steroid administration, proteinuria and hematuria resolved gradually within 6 months.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제16권3호
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• Childhood Kidney Diseases
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• 제14권2호
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• pp.223-229
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• 2010

Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.

• Journal of the korean academy of Pediatric Dentistry
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• 제32권2호
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• pp.256-261
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• 2005

Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.

• Journal of the korean academy of Pediatric Dentistry
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• 제38권1호
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• pp.95-100
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• 2011

Fibrodysplasia ossificans progressiva(FOP) is characterized by episodes of permanent heterotopic ossifications of soft tissues throughout the body. FOP is inherited in an autosomal dominant pattern, but most cases result from new mutations in the ACVR1 gene. Even minimal trauma can cause permanent ossifications of soft tissues and give rise to complications following routine dental care. Dental block anesthesia, severe stretching of the jaw and biopsies are all contraindicated in children with FOP. There is no effective treatment. Since the prevalence of FOP is very low and most patients with FOP are misdiagnosed during childhood, they undergo dangerous and unnecessary treatment that can lead to permanent harm. For patients with FOP, early diagnosis and prevention of complications are most important. This study aims at contemplating the characteristic features and consideration factors for the dental treatment of FOP patients in relation to the case of an eight years-and-one month old boy who was referred to Pediatric Dentistry due to prolonged retention of mandibular right and left deciduous central incisors after being diagnosed with sporadic FOP at Pediatric Orthopaedics, Seoul National University Hospital, and received dental treatment without the exacerbation of the FOP symptoms.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.341-348
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• 2010

Purpose : Body composition is important to define and manage obesity and undernutrition. Obesity is a significant health problem with medical and psychological consequences for children and adolescents. Body composition analysis (BIA) is a simple, rapid, noninvasive, and reproducible technique. However, comparative analysis of body composition has not been done in young children below 7 years old. The aim of this study is to estimate antropometric data and body composition by BIA, and to investigate the correlation between anthropometric data and fat mass or fat percent in young children. Methods : We measured height, weight, body mass index (BMI), fat mass, and fat percent by BIA in 1,376 children aged 3-6 years of whom 688 were males and 688 were females. Results : Fat mass, fat percent, intracellular fluid, extracellular fluid, protein, and minerals were significantly higher in the obese group. A significant positive correlation exists between fat mass and BMI or weight. A significant positive linear correlation was also noted between fat percent and BMI. Protein, weight, fat mass, and fat percent were significantly different among groups. Conclusion : BIA is an objective, accurate method to estimate body fat in childhood obesity cases. Fat mass and fat percent data acquired by using BIA highly correlated with BMI. However, a large-scale study is needed to diagnose obesity in young children.

• Journal of Korean Academy of Nursing
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• 제29권1호
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• pp.117-126
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• 1999

Accidents involving children are an important cause of death and disability. They also have enomorous financial implications. In order to prevent childhood accidents, research and education for safety should be strengthened. The purpose of this study was to determine how often young children have accidents and what factors affect the accident rate. The sample consisted of 771 children who were in the second, third and forth grades of two elementary schools located in Kyung-gi Province. One school had students from middle class families living in apartment complexes and the other, students from lower income families mainly living in single houses. The questionnaires included items on the occurance of accidents and the parents' attitudes regarding accidents during the academic year from March 1997 to February 1998. The Questionnaires were distributed to conventiently selected students to be compeleted by their parents and collected during the period of May 28, to June 6, 1998. The data were analyzed using SAS PC statistical package. The results of the study are as follows ; 1. Of 771 student subjects, 393 had 887 accidental injuries during the study period. 2. The month, the day and the time with the highest accident rate were May, Sunday, and between 1 and 4 p. m. each. 3. In the analysis of the location where the injury took place, the most frequent place was on around their homes followed by school and, then, inside the home 4. Most of the accidents were caused by carelessness on the part of the children and the most frequent type of injury was an abrasion. 5. Children most injured their legs 6. They were treated at home most often and usually emergency treatment was performed by family members with, disinfection being the main type of first aid. Cost of the treatment ranged from 8,000 to 20,000 won in most cases. 7. House type and parents' education level were statistically significant in chi-square analysis. 8. Parents educate their children about traffic safety most frequently followed by fire safety and, then, prevention of violence. 9. Parents think that prevention of violence should be the most important part of injury prevention education both at school and home. 10. To identify factors related to accident occurrence, multiple logistic regression was performed and the main factors were birth order and house type.

• Child Health Nursing Research
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• 제8권4호
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• pp.435-448
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• 2002

Accidents are important causes of death and disability in children. They also have enormous financial implications. Young children become an victim of accidents easily because of their physical fragileness and their coping behavior being vulnerable to any actions taken by accidents. Once they have a accident, the children whose not fully developed, suffer from devastating long-term after-effects. Lee, Lee, Kang and Han(1995) reported that ninety percent of accidents can be prevented. But there is no national system to manage, evaluate and analyse the information about child accidents, even though it is necessary for accident prevention policies and health promotion of the general public. The purpose of the study was to determine how often children have accidents and define the accident prevention strategies in children. The investigator conducted a descriptive study by performing the surveys, interviews, and workshops for the 2,458 young children, 10 teachers, and 1,494 parents. The data collection for the study began on September 2000 and completed on April 20, 2001. The analysis of the data was done with Window SPSS 10.0 for descriptive statistics. Among those children, 1,298 children(52.8％) injured from accidents. The children who had accident answered that they injured from traffic accident(27.3％), inside the home(26.3％), on the playground(17.0％), during playtime(13.6％), in the school(5.9％) and food poisoning(7.1％). To define accident prevention strategies for the school children, the parents and the teachers who had a special interest in this topic formed a special task force under the guidance of the investigator. The team was charged to prepare the basis of content materials by identifying the problems, setting standards for the program, prioritizing the process, and selecting the methods of implementation and evaluation. Eight issues and concerns identified by the team were: 1)allowing young children to learn undesirable habits and behaviors that would bother others without knowing; 2) not guarding young children from car accident; 3)unattended accident at playground; 4) considering home places safe; 5)unattended accident at school. These issues were found to be coinciding with the actual child accident cases occurred recent years in Korea. Greater efforts are required to reduce unnecessary deaths and disability from childhood accidents. This study gave a very useful and important data to prepare accident prevention educational program and to prepare accident prevention strategies.

• Tuberculosis and Respiratory Diseases
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• 제40권2호
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• pp.171-176
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• 1993

Background : Mycoplasma pneumonia (M. pneumonia) is a major cause of atypical pneumonia, and its incidence is predominantly at childhood and early adulthood. In contrast, the incidence of adult patients with M. pneumonia has been known to be low. Furthermore the clinical aspects of M. pneumonia are different from those of community acquired pneumonia. Thus, we evaluated the clinical aspects of M. pneumonia in the adult patients. Method : Mycoplasma antibody and cold agglutination tests were performed in patients with clinically suspected pneumonia who had abnormal infiltrations on chest P-A. The 12 patients with pneumonia, who fulfilled entry criteria of more than 1:64 of cold agglutination titer and 1:40 of mycoplasma antibody titer or four-fold increase of mycoplasma antibody titer during one week, were analyzed in terms of clinical aspects. Results : 1) Twelve patients, male 3 and female 9, were included in this study. The peak incidence was teenager. 2) M. pneumonia occured perennially, but predominantly between June to October in eight patients. 3) The main symptoms were fever, coughing, sputum. 4) The main patterns of chest P-A were bronchopneumoina in 8 cases, and involved lesion were nearly both lower lobe. Conclusion : The clinical aspects with Mycoplasma pneumonia in adult patients were different from those of community acquired pneumonia.