• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.1-12
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• 2018

Purpose: Disorders of organic acid metabolism have various clinical manifestations and it may be life-threatening. The prognoses of affected children are dependent on early diagnosis and treatment. We report this study to find out detection rate of referred samples, clinical manifestations and age distribution after introduction of neonatal screening test using tandem mass spectrometry in Hallym University Chuncheon Sacred Heart Hospital during 8 years and 9 months. Methods: The 2,794 patients referred from Jan. 2007 to Sep. 2015 were divided into four groups according to age. We conducted organic acid analysis of urine samples of patients and analyzed clinical manifestations and distributions of age at the diagnosis. For patients with ambiguous results, reanalysis of urine organic acid after diet restriction, protein loading and restriction, has been done. Results: A total of 626 patients with 20 disorders were diagnosed. Mitochondrial disorders (482 patients) were the most common diagnosis, followed by ketolytic defects (67), 3-hydroxyisobutyric aciduria (32), EPEMA syndrome (8), 3-methylcrotonyl glycinuria (7), glutaric aciduria type II (6) and type I (4), methylmalonic aciduria (3), isovaleric aciduria (3) and medium chain acyl-CoA dehydrogenase deficiency (3). Conclusion: As neonatal screening test using tandem mass spectrometry is increasingly common and medical environment is changed, detection rate of disorders of organic acid metabolism in this study has decreased compared to previous report. Because the deterioration can be prevented by early diagnosis and treatment, many pediatricians have to pay special attention to possibility of the disorders and make an effort for early diagnosis in clinical setting.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.226-232
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• 2006

Purpose: The aim of this study was to evaluate the influence of leptin on biochemical markers of bone metabolism in childhood obesity. Methods: A total of 50 male children (25 obese and 25 controls) were recruited from the pediatric outpatient clinic at the Chosun University Hospital from November 1st 2005 to May 30th 2006. BMI, body fat percentage, serum leptin, bone-specific alkaline phosphatase (B-ALP), C-terminal propeptide of type 1 collagen (CICP), total deoxypyridinoline crosslinks (total DPD) were measured. The correlations of leptin with BMI, body fat percentage, B-ALP, CICP, total DPD were analyzed by Pearson's correlation. In a multiple stepwise regression analysis, leptin after correction for body weight was evaluated if there was a correlation with biochemical markers of bone formation and resorption respectively. Results: The leptin levels of the obese group were significantly higher than those of the control group (p=0.012). In the obese group, the leptin level was significantly positively correlated with the BMI (r=0.551, p=0.01) and the percentage of body fat (r=0.584, p=0.018). In the obese group, of bone markers, B-ALP (r=-0.613, p=0.026) and CICP (r=-0.583, p=0.037) were negatively correlated with leptin. B-ALP (r=-0.728, p=0.007) and CICP (r=-0.684, p=0.014) were negatively correlated with leptin when corrected for body weight. In the control group, bone markers were not correlated with leptin. In the multiple stepwise regression analyses, there was a negative correlation between the leptin and B-ALP (Y=-39.653X+356.341, p=0.026), CICP (Y=-13.437X+ 116.013, p=0.037) respectively in the obese group. Conclusion: Leptin was a significant factor in the bone formation but not in bone resorption in childhood obesity.

• Advances in pediatric surgery
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• v.19 no.1
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• pp.1-13
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• 2013

The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was $66.2{\pm}28.7$ days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was $1.1{\pm}1.3$ years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was $8.6{\pm}2.9$ months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1219-1226
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• 2002

Purpose : To assess the clinical characteristics of the 2000-2001 measles outbreak in the Seoul metropolitan area, Korea, the clinical data of measles inpatients were analyzed. Methods : Three hundred and five children diagnosed with measles by clinical manifestation from July, 2000 to February, 2001, in Seoul, Ilsan and Ansan City were grouped according to their age and investigated for clinical manifestations, vaccination history and measles-specific IgM/IgG antibody positivity. Results : Thirty eight point seven percent of the subjects were less than 12 months of age, 27.5 % were 12-47 months of age and 33.8% were 48 months of age or older. There was no significant sexual difference(male : female=1.2 : 1). This epidemic started in June, 2000 and the number of patients increased abruptly in October, peaked in December and finally decreased after February, 2001. It started from the older age group and moved to the younger. Sixty five point two percent had a history of more than 1-dose vaccination and 13.6% of the patients equal or more than 48 months of age had a history of 2-dose vaccination. Primary vaccine failure rate was 59.4%(107/180) and secondary vaccine failure rate was 3.9%(7/180) in 1 dose vaccinees. Sixty one point six percent showed more than one complication and 38.4% had no complication. The most common complication was pneumonia(31.8 %), followed by bronchitis(11.5%) and acute otitis media(4.6%). Vaccination and dose were not related significantly with the occurrence of complications. Conclusion : Compared with previous outbreaks in Korea, clinical features showed no specific change in the 2000-2001 measles epidemic. However, primary vaccine failure rate was so high that the second vaccination at four to six years of age must be emphasized in Korea.

• The korean journal of orthodontics
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• v.32 no.1 s.90
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• pp.19-31
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• 2002

To investigate the association between the hypertrophy of adenoid and jaw morphology during growth, this paper was based on children patients with experimental adenoids (male-15 subjects at each bone age group, female-15 subjects at each bone group) and comparing them to data taken from a control group (male-15 subjects at each bone age group, female-15 subjects at each bone group) with normal respiratory function. The comparisons between the groups were done at each growth stage using cervical vertebrae maturation index(CVMI) of Hassel. The obtained results were as follows : 1. The differences in craniofacial morphology between experimental group and control group were appeared from CVMI 3 and CVMI 4 (aroud adolescent period) in males, and from CVMI 1 in females. 2. The mandibular position of experimental group was more inferior than control group. The difference appeared at adolescent period(male : at CVMI 4, female : at CVMI 5). 3. Experimental group had greater anterior facial height than control group. This difference seemed a relation with lower anterior facial height. The difference appears at CVMI 3(11.94 ${\pm}$ 1.38 years old, at adolescent period) in male and at CVMI 1 in female. 4. The adenoid size of control male group was increased until CVMI 2(10.58 ${\pm}$ 1.07 years old, just before adolescent growth peak) and then decreased, but in female the adenoid size was decreased from CVMI 1(6.92 ${\pm}$ 0.53 years old).

• Neonatal Medicine
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• v.15 no.1
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• pp.80-83
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• 2008

Purpose : Inguinal hernias are common in children and sometimes are associated with dangerous complications, such as incarceration. There are no established management guidelines for ovarian hernias. We have reviewed the clinical course of ovarian hernias in infants. Methods : We reviewed the medical records of female infants diagnosed with ovarian hernias by ultrasonogram at Kwandong University College of Medicine, Cheil General Hospital, and the Women's Healthcare Center between March 2001 and August 2007. We analyzed the patients gestational age, birth weight, age at the time of detection of the inguinal mass, the patients chief complaints, operative time, post-operative complications, and ultrasonographic findings. Results : Eight female infants had ovarian hernias, four of whom were born prematurely. Seven infants had left-sided ovarian hernias, and one infant had a right-sided ovarian hernia. Five infants underwent surgery and there were no postoperative complications or recurrences. Three girls did not have surgery, and the ovarian hernias regressed spontaneously, with no recurrences or complications. The regression time of inguinal masses ranged from 70-161 days after birth. Conclusion : Physical examination to detect movable masses within the labium major in premature female infants is important because the incidence of premature inguinal hernias is much higher than in term infants. No rational medical treatment plans for female ovarian hernias have been published to date. We cared for three girls with spontaneous regression of ovarian hernias. Pediatricians should be aware whether emergent surgery for ovarian hernias is indicated.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.27-32
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• 2006

Purpose : Conservative management of multicystic dysplastic kidney(MCDK) without nephrectomy has recently been advocated. The purpose of this study was to determine the clinical course of conservatively managed MCDK and to find out possible predictive factors for involution of MCDK by ultrasonography(US). Methods : A retrospective analysis was made on 45 patients(26 boys and 20 girls) in whom MCDK was detected and had been traced by US between Dec. 1993 and Aug. 2005 at Severance Hospital. Results : Median follow-up time was 30 months(range 2-102 months). All patients under-went radionuclide scans and voiding eystourethrograms. The serial follow-up US showed complete involution in 11(24%), partial involution in 19(41%), and no interval change or increased in cyst size in 13(28%) patients. Nephrectomy was done in 3 patients(7%) due to relapsing urinary tract infection(UTI) and severe abdominal distension. The mean age of complete involution of MCDK was 37 months(range 12-84 months). Episodes of UTI were present in 17 patients(37%) and additional genitourinary(GU) abnormalities were found in 22 patients(44%). Hypertension and renal insufficiency was complicated in one patient. No child developed malignant tumor. Univariate analysis showed that five variables were associated with complete involution of the MCDK; gender, site, UTI episode, additional GU abnormalities, and renal length on initial US. After adjusting using the Pearson model, the presence of additional GU abnormalities was exclusively associated with complete involution among the 5 variables(P=0.034). Conclusion : In our review of 46 cases of MCDK, non-surgical approach for patients with MCDK was advisable and we could predict poor prognosis when MCDK is associated with other GU anomalies.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.65-72
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• 2013

Purpose: Urinary tract infection (UTI) caused by gram-positive uropathogens is usually hospital-acquired and associated with predisposing conditions. However, the incidence of gram-positive bacteria in community-acquired UTIs has recently increased worldwide. We aimed to investigate the clinical significance of UTI and associated genitourinary malformations in young children with febrile UTIs caused by gram-positive bacteria. Methods: We retrospectively reviewed the medical records of 566 patients (age, <1 year) who visited the Korea University Medical Center for febrile UTIs between January 2008 and May 2013. We classified the patients into the following two groups: gram-positive (P group) and gram-negative (N group), according to the results of urine culture. The fever duration; white blood cell (WBC) counts and C-reactive protein (CRP) levels in peripheral blood; and the presence of hydronephrosis, cortical defects, vesicoureteral reflux (VUR), and renal scarring were compared between the two groups. Results: The number of patients with gram-positive bacteria was 23 (4.1%) and with gram-negative bacteria was 543 (95.9%). The most common pathogen was Escherichia coli, and Enterococcus faecalis showed the highest incidence among gram-positive uropathogens. Patients with gram-positive bacteria showed longer fever duration compared to that in patients with gram-negative bacteria (P vs. N, $3.4{\pm}1.2$ vs. $2.9{\pm}1.6$ days, P <0.05). The incidence of VUR was increased in the gram-positive group compared to that in the gram-negative group (P vs. N, 55.6 vs. 17.8%, P<0.05). However, there were no significant differences in other laboratory and radiologic findings. Conclusion: The findings of our study show that community-acquired UTIs in patients younger than 1 year of age, caused by gram-positive uropathogens, can be associated with prolonged fever duration and the presence of VUR.

• Journal of the Korean Society of Child Welfare
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• no.36
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• pp.289-322
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• 2011

The purpose of this study is to examine the relationship between job characteristics such as role conflict, workload, and self-efficacy, secondary traumatic stress(STS), and burnout of social-workers who are working with survivors of child abuse, spouse abuse and sexual assault. While burnout has been studied over two decades all over the world, secondary traumatic stress(STS) is a less familiar term in the field of social work. Studies explaining the relationship between job-demands, STS, and burnout for social workers working with violence victims lack integrative insights across agencies serving children and adults. Thus, we examine whether the relationships between change based on wheter the survivor is a child or an adult. Two hundred thirty three social workers in Korea who work with violence victims participated in the survey. The data were analyzed with Amos 7.0. Results indicate that STS plays a key role in explaining social worker burnout across the agencies, and Child Protective Service workers were found to have the highest STS symptoms. The pathways to social worker burnout proved to be different based on who you served. Theoretical and practical implications of these findings were discussed.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.15 no.2
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• pp.169-182
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• 2002

Background: Allergic rhinitis(AR) is a heterogeneous disorder that despite its high prevalence is often undiagnosed. It is characterized by one or more symptoms including sneezing, itching, nasal congestion, and rhinorrhea. And it is frequently accompanied by symptoms involving the eyes, ears, and throat, including postnasal drainage. There are many different causes of rhinitis in children and adults. Approximately 50$\%$ of all cases of rhinitis are caused by allergy. In the case of rhinitis caused by allergens, symptoms arise as a result of inflammation induced by a gamma globulin E-mediated immune response to specific allergens such as pollens, molds, animal dander, and dust mites. The immune response involves the release of inflammatory mediators and the activation and recruitment of cells to the nasal mucosa. AR is similar to 鼻？, hypersensitive rhinitis in Oriental Medicine. I think hypersensitive rhinitis is including of AR, vasomotor rhinitis and non-allergic rhinitis related with eosinophil increased and so on. Purpose: To perform a clinical analysis of hypersensitive rhinitis including allergic rhinitis and estimate the efficacy of Oriental Medical treatment. Objective: We studied 96 patients who had visited our hospital with complaints of nasal symptoms from March 2000 to February 2002; they had the signs more than 2 - nasal obstruction, watery discharge, sneezing and eye or nasal itching. Parameters Observed & Methods: We treated them with acupuncture & herb-medication. Sometime they used aroma oil or external medicine. 1) the distribution of sex & age groups 2) the clinical type based on duration & the severity of symptom 3) the breakdown of complication & pasl history of Otolaryngologic or allergic disease 4) the clinical assessment and classification of rhinitis(sneezers and runners & blockers) 5) the associated symptoms and signs 6) the classification of Byeonjeung 7) the classification of prescriptions and 8) the efficacy of treatment. Result: 1. In the clinical type of based on duration, the intermittent type was 42.7$\%$ and the persistent was 57.3$\%$. 2. We observed the severity of symptoms based on the quality of life. The mild type was 24.0$\%$ and the moderate-severe was 76.0$\%$. 3. In the clinical assessment and classification of rhinitis, the sneezers and runners type was 69.8$\%$ and the blockers was 30.2$\%$. 4. The most common family history with otolaryngologic or allergic disease were allergic rhinitis(17.7$\%$), urticaria, paranasal sinusitis and T.B.(3.1$\%$). 5. The most common past history with otolaryngologic or allergic disease were paranasal sinusitis(14.6$\%$), atopic dermatitis and asthma(8.3$\%$). It was 31.3$\%$ they had a family history and 44.8$\%$, past history. 6. The most common complication was paranasal sinusitis(15.6$\%$). In decreasing order the others were otitis media with effusion(9.4$\%$), GERD and headache(6.3$\%$), asthma, bronchitis, nasal bleeding and allergic dermatitis(5.2$\%$). 7. Classification through Byeonjeung : ⅰ) 39 cases(34.9$\%$) were classified as showing Deficiency syndrome. The insuffficiency of Qi was 17.7$\%$, deficiency of Kidney-Yang, 12.5$\%$ and Lung-Cold, 10.4$\%$. ⅱ) 57 cases(59.4$\%$) were classified as showing Excess syndrome. The Fever of YangMing-meridian was 35.4$\%$, Lung-Fever, 24.0$\%$. 8. The efficacy of treatments showed: an improvement in 22cases(22.9$\%$); an improvement partly in 24 cases(25.0$\%$); no real improvement or changes in 16 cases(16.7$\%$); and couldn't check the results 18cases(18.6$\%$). Conclusion: We suggest that this study could be utilized as a standard of clinical Oriental Medical treatment when we treat hypersensitive rhinitis including allergic rhinitis.