• Childhood Kidney Diseases
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• v.9 no.1
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• pp.56-63
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• 2005

Purpose : Development of renal scarring is associated with delayed diagnosis and treatment of urinary tract infection(UTI). This study was performed to clarify how soon treatment should be started to Inhibit renal scarring after onset of UTI and the factors associated with renal scarring in children with a first episode of febrile UTI. Methods : We retrospectively reviewed 163 patients with a first episode of febrile UTI under the age of 2 years from April 2000 to Ap,il 2004. All patients had a DMSA renal scan and voiding cystourethrogram done in the diagnostic period, 6 months after which a follow-up renal scan was done. After patients wet-e divided into 2 groups according to the duration of fever prior to start of treatment, the duration of fever after start of treatment, and total duration of fever, initial and follow-up DMSA scan findings were analyzed among the different groups. We compared the factors associated with renal scars between the groups with and without renal scars. Results : The initial DMSA renal scan identified abnormal finding in 23% of the patients who were treated $\leq$24 hr from the onset of disease and in 43% of those with fever more than 24 hr. Renal scars developed in 33% of patients who were treated $\leq$24 hr and 38% of those with fever >24 hr prior to treatment. Renal scars developed in 34% of patients with remission of fever $\leq$48 hr after treatment and ill 50% of those with fever >48 hr after treatment. The risk for renal scars was significantly higher in children who had total duration of feyer >72 hr(67%) than in those with shorter duration(19%). In children with renal scars, VUR was most highly associated with an increased risk of renal scar formation. Conclusion : Although children with a first episode of febrile UTI are treated within 24 hr after onset of the fever, renal damage cannot be prevented completely and it is mainly associated with VUR. (J Korean Soc Pediatr Nephrol 2005;9:56-63)

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.42-50
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• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.49-56
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• 2013

Purpose: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. Methods: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, ${\bigcirc\bigcirc}$ University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. Results: The mean age of the SUS and Sx groups was $10.8{\pm}2.7$ and $9.5{\pm}3.4$ years (P >0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P <0.001) and edema (P =0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P =0.007) and lower hemoglobin (P =0.007) and albumin (P =0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. Conclusion: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.

• The Korean Journal of Nuclear Medicine
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• v.36 no.6
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• pp.368-380
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• 2002

Purpose: Liver transplantation (LT), one of the therapeutic options of primary liver cancer has been suffering from recurrence caused by metastasis in 8-54% of patients. This study was performed to investigate whether FDG-PET is useful for detecting hidden metastasis in LT candidates. Materials and Methods: Twenty-six patients (male:female=23:3, mean age 55.7 years) underwent FDG-PET. Their previous conventional diagnostic studies (CDS) like abdomen US and CT, chest x-ray and CT, and bone scan were negative (n=22) or equivocal (n=4) for metastasis. Positive FDG-PET findings were confirmed by biopsy or clinical follow-up. Results: Among 4 patients with equivocal metastatic lesions on CDS, 3 had 6 hypermetabolic lesions on FDG-PET, which were confirmed as metastasis and subsequently LTs were cancelled. Of these, 5 lesions were initially negative on CDS. Remained 1 patient underwent LT with a negative FDG-PET result. Among 22 patients without metastasis on CDS, 5 had 7 hypermetabolic lesions on FDG-PET. One of these patients proved to have 2 metastatic lesions, and LT was cancelled. The other 4 patients had S hypermetabolic lesions on FDG-PET, which were confirmed as benign lesions, and 3 patients of them underwent LT. In summary, FDG-PET was useful in avoiding 4 unwarranted LT by detecting unsuspected metastatic lesions on CDS. A total of 17 patients underwent LT. In comparison with pathology, the sensitivity and specificity of FDG-PET for detecting viable primary liver cancer were 55.6% (5/9) and 87.5% (7/8), respectively. Conclusion: FDG-PET can detect additional hidden metastasis and contribute to reducing unwarranted LT in the patients with primary liver cancer.

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.368-380
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• 1999

Purpose: The purpose of this study was to evaluate and compare the scintigraphic findings and diagnostic accuracy of double-phase Tc-99m sestamibi scan in primary and secondary hyperparathyroidism (HPT). Materials and Methods: We retrospectively reviewed 16 cases of primary (18 lesions) and 11 cases of secondary HPT (44 lesions) who underwent Tc-99m-sestamibi scan before the surgical intervention. Scan was performed using LEM camera (Siemens, Germany) after the injection of 740MBq of Tc-99m sestamibi. Routine image consisted of baseline and 3-hour delayed images and each image was obtained using both parallel and pine hole collimator. The study population was 27 patients (male/female=5/22, age: $49.1{\pm}10.8$). Results: Eighteen lesions of primary HPT consisted of 13 adenomas and S hyperplasias, while all lesions of secondary HPT were hyperplasias. Among the case of primary HPT, we could detect all the lesions of 13 adenomas but only 2 lesions of 5 hyperplasias (40%) could be detected by double phase scintigraphy. Three cases of primary lesion showed decreased uptake in delayed images compared with baseline. The sensitivity, specificity, positive predictive value and accuracy of primary and secondary HPT were 58.8% (10/17), 83.3% (10/12), 83.3% (10/12), 75.9% (22/29), and 37.5% (15/40), 50% (2/4), 88.2% (15/17), 38.6% (17/44), respectively. Overall sensitivity, specificity, positive predictive value and accuracy were 43.9% (25/57), 75% (12/16), 86.2% (25/29), and 53.4% (39/73). There were no statistical difference between the weight of primary and secondary HPT lesion (p>0.05). Conclusion: Tc-99m sestamibi scan is fairly good modality to detect parathyroid lesion in patient with primary HPT before the surgical intervention. However, since some of cases may reveal decreased uptake in delayed image, a careful attention to the findings of baseline images may be helpful. Still the low accuracy of sestamibi scan in diagnosis of secondary HPT prohibits routine use of it for this disease.

• Journal of Nutrition and Health
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• v.52 no.6
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• pp.559-568
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• 2019

Purpose: This study examined the prevalence of food allergies and allergenic factors in a selected sample of children living in Seoul, Korea, along with their dietary habits, environmental factors, and diseases as risk factors for food allergy. The results of this study will provide basic data for addressing food allergies. Methods: We selected 3,004 pre-school and school-age children, aged 0 ~ 12, in the 25 districts of Seoul as the study sample. Structured self-report questionnaires were administered over a two-month period in July-August 2018, and the children's parents recorded the answers on their children's behalf. The research tools in this study included the Korean version of the questionnaire from the International Study of Asthma and Allergies in Childhood (ISAAC). Results: The physician-diagnosed prevalence rate of food allergies was 14.2%, while 20.4% of the children experienced allergic symptoms at least once and 17.4% reported symptoms within the previous 12 months. The children's symptoms included skin problems (88.1%), gastrointestinal issues (19.2%), oral issues (16.7%), respiratory issues (12.7%), and systemic issues (1.3%). The causes of allergies included eggs, peaches, milk, peanuts, and shrimps. The factors influencing the experience of food allergies were the consumption of cereal (aOR, 1.52; 95% CI, 1.09 ~ 2.10; p = 0.013), potatoes (aOR, 1.88; 95% CI, 1.33 ~ 2.65; p < 0.001), and fast food (aOR, 1.73; 95% CI, 1.10 ~ 2.72; p = 0.017). Having food allergy symptoms was associated with a higher risk of experiencing asthma (aOR, 4.22 95% CI, 3.10 ~ 5.76; p < 0.001), allergic rhinitis (aOR, 2.53; 95% CI, 2.03 ~ 3.15; p < 0.001), and atopic dermatitis symptoms (aOR, 3.56; 95% CI, 2.88 ~ 4.40; p < 0.001). Conclusion: Episodes of food allergies warrant examining regular food consumption and placing dietary restrictions through early diagnosis as these episodes may imply the presence of other allergies. Our findings offer basic insights into the patterns, prevalence and symptoms of children's food allergies in Seoul, and our findings will contribute to identifying effective interventions for food allergies.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.1
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• pp.77-94
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• 2021

The purpose of this study was to investigate the three-dimensional characteristics of mesiodens using Cone-beam Computed Tomography(CBCT) and analyze the factors affecting complications and anesthetic methods of extraction. This study evaluated 602 mesiodens of 452 patients who underwent extraction of mesiodens at the department of Pediatric Dentistry in Seoul National University Dental Hospital between 2017 and 2019. The ratio of mesiodens patients over total patient per year was gradually increased over the past 20 years. Mesiodens with labio-palatally horizontal direction while root directing labial were the most common among the mesiodens with horizontal direction. Mesiodens were the most common at the cervical side of the adjacent teeth(37.0%) and mesiodens located in the near-palatal side were observed about 3.83 times higher than the far-palatal side. Most of the mesiodens(82.1%) were in contact with adjacent permanent teeth on all three sides of the CBCT and 46.2% of mesiodens had curved roots. The patient's age, vertical position, presence of complications, and proximity showed a significant difference in the selection of general anesthesia among anesthetic methods. The direction and vertical position of mesiodens had a significant effect on complications. These results provide a better understanding of mesiodens for establishing an accurate diagnosis and treatment plan.

• Sleep Medicine and Psychophysiology
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• v.26 no.1
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• pp.44-48
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• 2019

Objectives: Insomnia is one of the most prevalent sleep disorders. Recent studies suggest that cognitive and physical arousal play an important role in the generation of primary insomnia. Studies have also shown that information processing disorders due to cortical hyperactivity might interfere with normal sleep onset and sleep continuity. Therefore, focusing on central nervous system arousal and normalizing the information process have become current topics of interest. It has been well known that neurofeedback can reduce the brain hyperarousal by modulating patients' brain waves during a sequence of behavior therapy. The purpose of this study was to investigate effects of neurofeedback therapy on electroencephalography (EEG) characteristics in patients with primary insomnia. Methods: Thirteen subjects who met the criteria for an insomnia diagnosis and 14 control subjects who were matched on sex and age were included. Neurofeedback and sham treatments were performed in a random order for 30 minutes, respectively. EEG spectral power analyses were performed to quantify effects of the neurofeedback therapy on brain wave forms. Results: In patients with primary insomnia, relative spectral theta and sigma power during a therapeutic neurofeedback session were significantly lower than during a sham session ($13.9{\pm}2.6$ vs. $12.2{\pm}3.8$ and $3.6{\pm}0.9$ vs. $3.2{\pm}1.0$ in %, respectively; p < 0.05). There were no statistically significant changes in other EEG spectral bands. Conclusion: For the first time in Korea, EEG spectral power in the theta band was found to increase when a neurofeedback session was applied to patients with insomnia. This outcome might provide some insight into new interventions for improving sleep onset. However, the treatment response of insomniacs was not precisely evaluated due to limitations of the current pilot study, which requires follow-up studies with larger samples in the future.

• Journal of Life Science
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• v.29 no.7
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• pp.800-808
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• 2019

Charcot-Marie-Tooth disease (CMT) is a group of rare peripheral neuropathies characterized by progressive muscle weakness and atrophy and areflexia in the upper and lower extremities. The most common subtype of CMT is CMT1A, which is caused by a tandem duplication of the PMP22 gene in the 17p12 region. Patients with CMT1A show a loose genotype-phenotype correlation, which suggests the existence of secondary genetic or association factors. Recently, polymorphisms of rs71428439 (n.83A>G) and rs2292832 (n.86T>C) in the MIR149 have been reported to be associated with late onset and mild phenotypic CMT1A severity. The aim of this study was to examine the intrafamilial heterogeneities of clinical phenotypes according to the genotypes of these two SNPs in MIR149. For this study, we selected 6 large CMT1A families who showed a wide range of phenotypic variation. This study suggested that both SNPs were related to the onset age and severity in the dominant model. In particular, the AG+GG (n.83A>G) and TC+CC genotypes (n.86T>C) were associated to late onset and mild symptoms. Motor nerve conduction velocity (MNCV) was not related to the MIR149 genotypes. These results were consistent with the previous studies. Therefore, we suggest that the rs71428439 and rs2292832 variants in MIR149 may serve as genetic modifiers of CMT1A intrafamilial phenotypic heterogeneity, as they have a role in the unrelated patients. This is the first study to show an association using large families with variable clinical CMT1A phenotypes. The results will be helpful in the molecular diagnosis and treatment of patients with CMT1A.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.78-86
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• 2018

Purpose: We aimed to delineate clinical spectrum and short-term effects after enzyme replacement therapy (ERT) for 5 mucopolysaccharidosis type II (MPS II). Methods: Five patients were diagnosed with MPS II by clinical findings, enzyme activity, and genetic testing. Idursulfase was administered by intravenous infusion at a dose of 0.5 mg/kg every week. Observational chart analysis of patients, who underwent systematic investigations more than 12 months after initiation of ERT was done retrospectively. Results: Three patients were classified as having the attenuated type, and 2 patients were classified as having the severe type. The median age at the diagnosis was 9.6 years (range 3.4-26 years). Four different mutations in 5 Korean patients (4 families) with MPS II were identified, among which two were novel mutations (1 small insertion mutation: p.Thr409Hisfs*22, and 1 missense mutation: p.Gly134Glu). Two severe type sibling patients with the same mutation had different clinical manifestation. Urinary glycosaminoglycan excretion decreased within the twelve months of ERT (P=0.043). Liver and spleen volumes showed reductions that were maintained in all patients (P=0.043 and P=0.043, respectively). Improvements were also noted in left ventricular mass index (P=0.042), shoulder flexion (P=0.043), shoulder abduction (P=0.039), knee flexion (P=0.043), elbow flexion (P=0.042), and respiratory distress index (P=0.041). Conclusion: This study demonstrates that Korean patients with MPS II are clinically heterogeneous and indicates that idursulfase is relatively effective in several clinical parameters including heart size and respiratory distress index without infusion-related reactions in patients with MPS II.