• 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.57-64
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• 2013

Purpose: In children, 24-hour urine collections are unreliable for evaluating glomerular filtration rate (GFR) because of the difficulty of regulating voiding and the daily variation of urinary creatinine up to 25%. Additionally, creatinine clearance (Ccr) based on urinary creatinine is considered inaccurate. The purpose of this study was to compare estimated GFR determined using Ccr, formulas with serum cystatin C and creatinine, and $^{99m}Tc$-mercaptoacetyltriglycine (MAG3) dynamic renal scintigraphy. Methods: This retrospective study included 101 patients (age, <18 years) who visited Chung-Ang University Hospital between July 2011 and August 2012. GFR was estimated using 24-hour urinary creatinine, five formulas with serum creatinine and cystatin C, and $^{99m}Tc$-MAG3 renal scan. Results: Of the 101 patients, glomerular renal diseases were present in 60 patients (59.4%) and non-glomerular diseases were present in 41 patients (40.6%). There was a significant correlation between estimated GFR determined using $^{99m}Tc$-MAG3 renal scan and Ccr (r=0.389, P <0.001). The correlation values between estimated GFR determined using $^{99m}Tc$-MAG3 renal scan and each formula of Schwartz, Counahan-Barratt, Cockcroft-Gault, Filler and Lepage, and Bokencamp were 0.265 (P=0.007), 0.128 (P=0.044), 0.230 (P=0.021), 0.356 (P<0.001), and 0.355 (P <0.001), respectively. $^{99m}Tc$-MAG3 renal scan was correlated with estimated-GFR by all formulas in decreased renal function. Conclusion: Estimated GFRs determined using serum creatinine and cystatin C, and $^{99m}Tc$-MAG3 renal scan correlated well with estimated GFR determined using Ccr. $^{99m}Tc$-MAG3 renal scan may be replaced for evaluation of renal function with convenience in patients with renal disease and decreased renal function in childhood.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.92-100
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• 2013

Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Sch$\ddot{o}$nlein purpura nephritis (HSPN). Methods: We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment. Results: The mean protein to creatinine ratio decreased from $3.7{\pm}1.5$ to $0.6{\pm}0.4$(P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA. Conclusion: Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.73-78
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• 2013

Purpose: We aimed to investigate the clinical characteristics and associated diseases in children with a horseshoe kidney and compared these data between children and adults. Method: We performed a retrospective analysis of the medical records and radiological findings of 43 patients diagnosed with a horseshoe kidney in the Busan Paik Hospital. The subjects were divided into the children's group (14 cases, age <18 years) and the adult group (29 cases, age ${\geq}18$ years). Results: The study group consisted of 17 males and 26 females with a median age of 34 years. In the children's group (14 cases), 5 subjects were male and 9 were female, with a mean age of $6.7{\pm}6.2$ years. Most of the subjects were asymptomatic and were incidentally diagnosed with horseshoe kidney during their evaluation for another disease. Among the associated diseases in the children's group, Turner syndrome was the most common (5 cases), whereas ureteropelvic junction (UPJ) stricture was observed in 2 cases (14.2%). None of the children exhibited abnormal renal function during the follow-up period. In the adult group (29 cases), 12 subjects were male and 17 were female, with a mean age of 48 years. Eighteen patients were incidentally diagnosed with horseshoe kidney during their evaluation for another disease, and 11 patients had hematuria or abdominal pain due to renal stones. Among the associated diseases in the adult group, Turner syndrome was the most common (5 cases), and UPJ stricture was observed in 5 cases; the other accompanying diseases included hydronephrosis and overactive bladder. Six patients exhibited decreased renal function (serum creatinine level >1.5) during the follow-up period. Conclusion: Horseshoe kidney is usually diagnosed incidentally in both children and adults. In the present study, we noted that Turner syndrome was the most common associated disease in children. In addition, most children were asymptomatic but had a high risk of urologic complications after the transition to adulthood. Therefore, children with horseshoe kidney require continuous follow-up.

• Allergy, Asthma & Immunology Research
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• v.10 no.6
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• pp.648-661
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• 2018

Purpose: Pollen-food allergy syndrome (PFAS) is an immunoglobulin E (IgE)-mediated allergy in pollinosis patients caused by raw fruits and vegetables and is the most common food allergy in adults. However, there has been no nationwide study on PFAS in Korea. In this study, we investigated the prevalence and clinical characteristics of PFAS in Korea. Methods: Twenty-two investigators participated in this study, in which patients with allergic rhinoconjunctivitis and/or bronchial asthma with pollen allergy were enrolled. The questionnaires included demographic characteristics, a list of fruits and vegetables, and clinical manifestations of food allergy. Pollen allergy was diagnosed by skin prick test and/or measurement of the serum level of specific IgE. Results: A total of 648 pollinosis patients were enrolled. The prevalence of PFAS was 41.7% (n = 270). PFAS patients exhibited cutaneous (43.0%), respiratory (20.0%), cardiovascular (3.7%) or neurologic symptoms (4.8%) in addition to oropharyngeal symptoms. Anaphylaxis was noted in 8.9% of the PFAS patients. Seventy types of foods were linked to PFAS; e.g., peach (48.5%), apple (46.7%), kiwi (30.4%), peanut (17.4%), plum (16.3%), chestnut (14.8%), pineapple (13.7%), walnut (14.1%), Korean melon (12.6%), tomato (11.9%), melon (11.5%) and apricot (10.7%). Korean foods such as taro/taro stem (8.9%), ginseong (8.2%), perilla leaf (4.4%), bellflower root (4.4%), crown daisy (3.0%), deodeok (3.3%), kudzu root (3.0%) and lotus root (2.6%) were also linked to PFAS. Conclusions: This was the first nationwide study of PFAS in Korea. The prevalence of PFAS was 41.7%, and 8.9% of the PFAS patients had anaphylaxis. These results will provide clinically useful information to physicians.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.251-262
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• 2018

Purpose: This study aimed to identify changes in smart device usage trends of young children using two studies conducted in 2015-2016 and 2017 respectively. Methods: We compared the data of the previous study of 130 children (Group A) and the new study of 162 children (Group B). The children and parents were recruited from kindergartens in Seoul and Guri/Namyangju cities. We used the "Parental questionnaire for smart device usage status." Results: There were some changes in the smart device usage in young children and parental perception. In the 2017 study, smart device usage time increased during weekends (P<0.05) and the usage with siblings decreased (P<0.05). In 2017, the smart device was mostly used when children had to be quiet without disturbing others (36.8%). No significant difference existed in the main purpose of use: watching video clips (79.3% vs 76.6%). Overall control of the usage was still largely exercised by mothers; however, when using applications, mothers still only helped the children on request (51.8% vs 49.7%). Regarding the effect of smart device on children, responses of "not knowing" decreased and "will be negative" and "will be positive" increased (P<0.05). Additionally, most mothers thought that "Although the smart device is currently unnecessary, it will be needed in future" in 2017 (46.3%). Conclusion: Limiting the smart device usage time during the weekends and increasing parental involvements are recommended. Guidelines for smart devices usage in young children are also necessary considering the changes in parental attitudes in recognizing the smart device usage as unavoidable.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.205-209
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• 2018

Purpose: Several studies have shown that magnesium plays an important role in modulating N-methyl-D-aspartate (NMDA)-related seizures by blocking NMDA ion channel receptors. Clinicians usually measure total serum magnesium levels instead of biologically active ionized magnesium levels. We compared the serum ionized magnesium ($iMg^{2+}$) level between epileptic children with and without a history of fever-triggered seizure (FTS). Methods: All epileptic children who visited the outpatient clinic or pediatric emergency department at Korea University Guro Hospital between January 2015 and July 2017 were included. Only epileptic children aged 1-8 years who were newly diagnosed within 2 years were included. Results: There were 12 children with FTS and 16 without FTS. Median serum $iMg^{2+}$ level was 0.93 (0.85-1.14, quartile) mEq/L. Serum $iMg^{2+}$ level was significantly lower in epileptic children with FTS (0.86 mEq/L) compared to those without FTS (1.10 mEq/L) (P=0.005). No difference was noted in clinical variables between the two groups. Lower serum $iMg^{2+}$ level significantly increased the risk of having FTS in epileptic children based on multivariable logistic regression analysis (odds ratio [OR]=0.028). Conclusion: Serum $iMg^{2+}$ level was significantly lower in epileptic children with FTS than in those without FTS. Measurement of biologically active serum $iMg^{2+}$ level could be considered in epileptic children with recurrent FTS. A large-scale prospective study is warranted.

• The Journal of Korean Society for Radiation Therapy
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• v.30 no.1_2
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• pp.35-40
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• 2018

Purpose : The range of force differs from the size of proton energy used in our hospital. The compensator enables to change energy size based on distal thickness which also makes changes in dose rate. Therefore, the purpose of this study is to evaluate the effect of changing the thickness of compensator distal on dose range and beam on time. Subject and Methodology : Five low energy patients who have received proton therapy were selected as subjects for this study. Beam on was checked for the selected patients during the existing therapy. After then, the thickness of distal of compensator was increased by 2 cm up to 14 cm through proton therapy plan system(TPS) for comparative analysis. For the evaluation of dose range, the value of the target's conformity index(CI) and the maximum dose of rear side target's organ at risk(OAR) were compared. Furthermore, to evaluate the effect of therapy time, beam on time was compared by making compensator distal in each thickness. Result : The result of homogeneity index and conformity index of the increased compensator distal showed the same level in all patients. The comparison results of OAR of target rear side showed 7 cGy at spine cord of abdomen at maximum, 88 cGy at eyeball's RT lens, 391 cGy at RT lens of nasal cavity 51 cGy at trachea of the mediastinum, and 661 cGy at a small bowl of the pelvis. The comparison results of the beam on time showed a reduction from 126 seconds to 62 seconds for the abdomen, from 105 seconds to 37 seconds for the eyeball, from 187 seconds to 134 seconds for nasal cavity, from 100 seconds to 40 seconds for mediastinum, from 440 seconds to 118 seconds for the pelvis. Conclusion : The research result showed that as the distal thickness of compensator increased, the size of energy increased. In addition, beam on decreased due to the increase of dose rate. It is expected that the result would help reduce the treatment time and increase the convenience of patients if it is applied to liver patients who need respiratorygated therapy and pediatric patients. However, distal penumbra increased as the size energy increased. Therefore, in treating cases where OAR is in the vicinity of the target rear side, the influence of penumbra should be taken into account in adjusting thickness level of the compensator in proton therapy plan.

• Korean Journal of Psychosomatic Medicine
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• v.24 no.2
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• pp.165-173
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• 2016

Objectives : Since the awareness of autism spectrum disorders(ASD) is growing, as a result, it is increasing numbers of infants and toddlers being referred to specialized clinics for a differential diagnosis and the importance of early autism spectrum disorders detection is emphasized. This study is to know the difference between ASD and intellectual disability(ID) from comparison of the demographics, clinical characters and obstetric complications. Methods : The participants are 816 toddlers who visited the developmental delay clinic(DDC) in National Health Insurance Ilsan hospital. The number of toddlers diagnosed as ASD and ID was 324 and 492. 75 toddlers out of 114 who returned to DDC were diagnosed as ID at the first visit but 7 of them had changed diagnosis to ASD at the second visit. After compared ASD with ID from the first visit, we analyzed characters of toddlers who had the changed diagnosis to ASD at the second visit. Results : As a result, the comparison between ASD and ID at the first visit shows that the boys have higher ratio, lower obstetric complication and lower language assessment score in ASD. The toddlers who had the changed diagnosis at the second visit were all boys and they had more cases of family history of developmental delay and had lower score of receptive language developmental quotient. Conclusions : These findings suggest that sex, language characteristics and obstetric complication could be useful in the early detection of ASD.

• 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.