• Childhood Kidney Diseases
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• v.21 no.2
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• pp.94-100
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• 2017

Purpose: The American Academy of Pediatrics provides guidelines for managing febrile urinary tract infection (UTI) in infants and children 2-24 months old, but little guidance is offered regarding UTIs in those younger than 8 weeks of age. The definition of UTI is unclear and whether to proceed with micturating cystourethrography (MCUG) or $^{99m}$technetium-dimercaptosuccinic acid (DMSA) scintigraphy scan in this age group is controversial. Methods: We retrospectively analyzed 29 neonates and infants younger than 2 months of age who underwent late DMSA scans 9 months following the first episode of febrile or symptomatic UTI between July 2009 and June 2016. Results: In total, 192 children aged 0-24 months underwent ultrasound and DMSA scans (MCUG in 174/192). Neonates and infants younger than 2 months of age were significantly less likely to develop fever, and had a lower fever peak, shorter duration of fever before admission and after starting antibiotics, longer hospitalization period, lower C-reactive protein, and greater incidence of nonEscherichia coli infection. There was no difference in pyuria response at diagnosis. The prevalence rates of an ultrasound abnormality (28%), vesicoureteral reflux (28%), UTI recurrence (38%), and renal scarring (10%) in infants younger than 8 weeks of age were similar to those in children 2-24 months old. Conclusion: Neonates and infants younger than 2 months of age with UTI warrant special consideration because the fever response used for diagnosis in older children may be absent or blunted. Clinical guideline is needed for the diagnosis and management of UTI in this age group.

• Development and Reproduction
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• v.13 no.2
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• pp.63-77
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• 2009

The neuronal ceroid-lipofuscinoses (NCLs) are a kind of neurodegenerative storage disorders. The NCLs are charecterizated by accumulation of autofluorescent lipofuscin or lipopigment in the brain. All NCL group belongs to in lysosomal storage disorders (LSDs), except Northern epilepsy. NCLs are the most common group of progressive neurodegenerative disorders in childhood, with an incidence as high as I in 12,500 live births. Four main clinical types have been described based on the onset age : infantile, late infantile, juvenile and adult types. Clinical symptoms of NCLs include loss of vision, seizures, epilepsy, progressive mental retardation and a premature death. Although mutation causes neurodegeneration in NCLs, the molecular mechanism by which mutation leads to neurodegeneration remains unclear. In this paper, we review the characteristics of these NCLs.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.11 no.1
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• pp.5-8
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• 2015

Neuroblastoma is a common malignant tumor of the sympathetic nervous system in childhood, arises from embryonic neural crest cells. The period of tooth development is matched with peak times of diagnosis and treatment of neuroblastoma. The intensive multimodality treatment including radiotherapy and chemotherapy is used in patients with neuroblastoma has been shown to have late adverse effects and disturbances in dental development like tooth agenesis, microdontia, enamel hypoplasia and short roots. A 8-year old girl had been on medication and radiotherapy for neuroblastoma since she was 15 months old at Department Pediatrics, Chonnam National University Hospital. Oligodontia, microdontia, and short root formation was notable in clinical and radiological examination. Mobility of lower permanent incisor was detected and measured at about degree 2. Resin wire splint using mini-screw implantation on buccal alveolar bone was conducted for maintenance of mandibular incisors and alveolar bone. Excessive mobility has been eliminated and maintained well so far. Further treatment is planned for re-evaluation of mobility, preventing dental caries and regular oral hygiene management. Although we need further evaluation, this treatment could be one of alternative therapy for those who have similar dental anomalies.

• Journal of the Korea Convergence Society
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• v.11 no.2
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• pp.261-269
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• 2020

The purpose of the study was to investigate the relationship among children's perception of parenting style, their social anxiety, aggression, and school adjustment. A total of 267 children(male 117, female 150) and 10 homeroom teachers participated. Each child completed questionnaires on perception of parenting style, and social anxiety. Children's aggression and school adjustment was measured by their homeroom teachers' reports. The results of the structural equation model showed that children's perception of parenting style high in rejection and control significantly influenced aggression as well as school adjustment, but didn't influence social anxiety. Both parenting style high in rejection and control and aggression had significant effect on school adjustment. Our results showed that parenting style high in rejection and control directly influenced school adjustment, and mediated by their aggression.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.329-331
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• 2006

Alkaptonuria is a rare metabolic disease in which homogentisic acid cannot be metabolized due to a lack of the enzyme homogentisic acid oxidase. The disease often manifests itself in childhood by darkening of the urine upon standing. The disease leads to such serious consequences as ochronosis of cartilage and connective tissues with arthritis. It is expected that treatment with ascorbic acid and a dietary restriction of protein may decrease the late and serious consequences by diminishing the serum concentration of the metabolite benzoquinone acetic acid. A thirteen month-old girl was recently diagnosed with alkaptonuria by urine organic acid analysis. She excreted pinkish urine on a diaper and as time went by the urine color changed to a light brown. In laboratory findings, urine examination and culture results were normal. But urine organic acid analysis detected abnormal findings a prominent and massive elevation of homogentisic acid. The other physical findings were normal. This is the first case diagnosed in Korea.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.1-5
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• 2013

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.155-158
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• 2015

Lymphangioma is a congenital abnormality of the lymphatic system detected primarily in early childhood. There are rare reports of mediastinal lymphangioma in older adults. We hereby report on a 66-year-old female patient who underwent kidney transplantation 20 years previously and who developed pathologically confirmed solitary mediastinal lymphangioma 1 year ago. Chest radiography showed a mediastinal nodule, which was not observed 2 year previously, therefore she was referred to the pulmonary division. She had no symptoms, and chest computed tomography demonstrated a 25-mm, well-defined, low-density nodule located at the anterior mediastinum. The size of the nodule had increased from 25 mm to 34 mm 1 year later, and it was completely resected via video-assisted thoracic surgery. The histological diagnosis was cystic lymphangioma. Therefore, we recommend that clinicians consider cystic lymphangioma as a possible diagnosis even in older patients with a mediastinal cystic mass that shows progressive enlargement.

• Journal of Korean Neurosurgical Society
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• v.57 no.3
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• pp.185-191
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• 2015

Objective : In order to provide normal values of the pediatric sub-axial cervical spinal canal and vertebral body growth pattern using computed tomographic scans, a total of 318 patients less than 10 years old were included. Methods : The growth of the vertebral body and canal space was investigated using four different age groups. The Torg ratio (TR) was calculated and all patients were classified into a low TR group and a high TR group according to a cutoff value of 1.0. To account for spinal curvature, the C3-7 angle was measured. Results : Very little axial expansion and growth in height were observed (2.9 mm and 3.4 mm, respectively), and the spinal canal increments (1.8 mm) were much smaller than the dimensions of the vertebral body. The mean TR values were $1.03{\pm}0.14$ at the C3 vertebral level, $1.02{\pm}0.13$ at C4, $1.05{\pm}0.13$ at C5, $1.04{\pm}0.13$ at C6, and $1.02{\pm}0.12$ at C7 in all patients. The mean sub-axial angle (C3-7) was $7.9{\pm}10.6^{\circ}$ (range: $-17-47^{\circ}$). Conclusion : The upper sub-axial spinal canal continuously increased in size compared to the lower sub-axial spine after 8 years of age. Considerable decrements in the TR was found after late childhood compared to younger ages. Generally, there were no significant differences between boys and girls in vertical length of the cervical vertebrae. However, the axial dimension of the vertebral body and the spinal canal space varied according to gender.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1175-1183
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• 1997

Background. Infective endocarditis s an uncommon but serious disease in children. Optimal treatment strategy, especially surgical indications, continues to evolve. Method. In this report, we retrospectively reviewed 19 patients who underwent operations in Seoul National University Children's Hospital for infective endocarditis between September 1986 and February 1996. There were 8 male and 11 female patients(mean age 70.6 months) and their main symptoms were fever and dyspnea. Preoperative echocardiography detected vegetation in 14 cases out of 19. Congenital anomalies were in 15 cases and acquired forms were in 4 cases. Causative organisms were identified in 12 cases and Gram-positive cocci were in 7 cases. Adequate preoperative antibiotic management was done. Result. With cardiopulmonary bypass in all cases, surgical corrections including vegetation removal were performed. 4 patients died after operations(21.1%). All the mortality cases were having underlying complex cardiac anomalies. There were no late deaths. Act arial survival rate was 79.0% at 1 year and 79.0% at 5 year and reoperationfree survival rate was 73.7% at 1 year and 73.7% at 5 year. Collclusion. Despite advances in antibiotic therapy, early surgical treatment is imperative in a significant subset, and concurrent intracardiac repair should be appropriate.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.26 no.1
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• pp.1-11
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• 2015

Phenomenology has been developed by philosophers like Kant and Husserl since the late 18th century. Jaspers, a German psychiatrist, adopted it into psychopathology studies and accumulated data by closely observing and recording the patients' symptoms and signs. Among descriptions done even before the psychopathology or diagnostic criteria of disorders in the field of child psychiatry was established, we can find exact and valuable descriptions matching the autism spectrum disorder or attention deficit/hyperactivity disorder. The diagnostic criteria of modern childhood psychiatric disorders were established based on these grounds. Phenomenological/descriptive methods in various psychiatric fields lead to medical study methods for social phenomenon such as oiettolie, hikikomori, and internet game addiction. Since Romanian orphans were adopted to the western world, descriptive studies along with neurobiological studies on the influence of stimulus deprivation on emotional and physical development are being conducted. While phenomenology, which was adopted by Jaspers to verify psychopathology, was developed mainly by observation and description, recent studies are explaining such descriptive phenomena even at the synapse level due to advances in neurobiology. Although phenomenological/descriptive psychiatry, describing precise and detailed experiences of patients, is less applied nowadays among modern study methods, we must remember that such descriptions may lead to biological studies and provide evidence to improve the accuracy of choosing and applying treatment methods.