• Journal of the korean academy of Pediatric Dentistry
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• v.24 no.4
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• pp.751-757
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• 1997

Cherubism is an uncommon, benign fibrosseous lesion of the jaws that causes a progressive, painless, symmetrical expansion of the maxilla and mandible. It's autosomal dominance pattern of inheritance has been confirmed. A characteristic deformity is specific to this disease: hypertrophy of the mandible, swelling of cheeks and sometimes hypertrophy of maxilla with eyes tending to look up ; that looks like the Renaissance cherubs. It usually makes figures during childhood between 2-4 years of age and progresses until puberty, after which it spontaneously regresses 10 most cases. As a result of this case review, the management strategies for cherubismic children in standpoint of pediatic dentistry can be summarized as follows: 1. It can be detected early in childen through its characteristic clinical and radiographic features, which is confirmed by histopathological examination and familial history. 2. The supervision of arch space is required against its frequent sequelae, the early missing of primary teeth and eruption disorders of permanent teeth. 3. It is highly recommended to continue the periodic check-up with clinical and radiographic examination, leading to surgical intervention in cases of aggravation.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.115-119
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• 2020

Distal renal tubular acidosis (dRTA) is a rare renal tubular disorder characterized by normal anion gap metabolic acidosis, hypokalemia, and high urine pH. It can be inherited or acquired. In untreated pediatric patients with dRTA, rickets and growth retardation are common. We report the case of a 12-year-old Lao girl who presented with typical clinical features of dRTA with severe bone deformities that developed after a bed-ridden state due to a bicycle accident at the age of 8 years. Initial laboratory tests revealed metabolic acidosis with a normal anion gap, hypokalemia, and alkali urine. Renal ultrasonography revealed bilateral medullary nephrocalcinosis. Whole exome sequencing revealed no pathogenic mutations. After treatment with oral alkali, potassium, and vitamin D, she could walk and run. Later, she underwent corrective orthopedic surgeries for bony deformities. Thus, in pediatric dRTA patients, despite severe symptoms remaining untreated, accurate diagnosis and proper management can improve quality of life.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.31 no.1
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• pp.45-48
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• 2020

Granular cell tumor is rare tumor origination from Schwann cell. It occurs extremely rarely in pediatric age. Treatment is complete resection, but this may not always be possible because of the risk of airway stenosis or vocal cord paralysis. Six year-old male patient visited otolaryngology clinic due to dyspnea and stridor. Posterior glottis mass was indentified and was partially resected to confirm histology and resolve airway obstruction. One year after operation, the patient was living well without re-growing of tumor. We report a case of granular cell tumor in pediatric larynx with a review of literature.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.6
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• pp.510-518
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• 2022

Purpose: Recently, great interest has been focused on dietary fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs) for the treatment of functional abdominal pain (FAP). Several meta-analyses, including those on the adult population, have been published, potentiating its role. However, pediatric studies are scarce. We aimed to evaluate the effect of a low-FODMAP diet on the severity of FAP in children. Methods: This clinical trial included 50 patients aged 3-18 years with irritable bowel syndrome and FAP that were not otherwise specified. The patients were instructed to receive a low FODMAP diet guided by a dietitian. The primary outcome was the percentage of responders after 2 months of dietary intervention compared with baseline. Other outcomes included changes in stool consistency and quality of life (QoL) scores using the KIDSCREEN-10 questionnaire, and weight-for-age z-scores. Results: After the dietary intervention, 74% of patients showed more than 30% lower pain intensity, as examined using the Wong-Baker Faces pain rating scale. Their QoL significantly improved, and patients have gained weight. Conclusion: A low FODMAP diet can improve pain intensity and QoL among children with functional abdominal pain, with no detrimental effects on body weight.

• Journal of Korean Dental Science
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• v.16 no.2
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• pp.204-210
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• 2023

Lesch-Nyhan syndrome is a rare X-linked recessive disorder characterized by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyl transferase, which is important in the metabolism of purines. Prevalence of Lesch-Nyhan syndrome is 1:100,000 to 1:380,000 live births, and Lesch-Nyhan syndrome mainly affects males. It is characterized by neurologic dysfunction, uric acid overproduction, and cognitive and behavioral disturbances. The self-mutilation is the most distinctive symptom in Lesch-Nyhan syndrome, appearing about 1 year of age. It is expressed as persistent bites in the oral mucosa, tongue, lips, and fingers, which leads to the total or partial destruction of the affected tissues. Several medical and dental management methods might be performed in Lesch-Nyhan syndrome patients. Dental approaches to prevent injuries caused by self-mutilation include treatments with oral appliances such as mouth guards or lip bumpers, extraction, and orthognathic surgery. This report described two brothers with Lesch-Nyhan syndrome, who injured themselves on oral tissues. The methods of tooth reduction and extraction were performed to prevent injuries caused by self-mutilation.

• Clinical and Experimental Pediatrics
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• v.58 no.5
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• pp.194-198
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• 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.2
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• pp.173-180
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• 2006

Knowledge of growth and development is essential for pediatric dentistry treating growing patients. The data from the pediatric growth curve being used today does not reflect the growth transition of modern times, nor does it match the age range required for dental purposes. The present study, therefore, aims to introduce the process of producing a growth curve and growth rate curve based on data which represents a more accurate description of the present situation. The original data used in this study were from the 5th nationwide survey, SIZE KOREA 2004 study carried out by the Technology and Standards Policy Division, Department of Technology and Standards Planning, Ministry of Commerce, Industry. and Energy. Processing and rearranging the produced data with variations divided into the three quartiles and the 1st, 5th, 95th and 99th percentiles were included to produce a growth observation chart according to sex, height and weight differences. In the same way, a growth rate curve based on the geometric mean value was produced. The resulting growth charts can be used as an index for growth and development, and used for better communication between the pediatric dentist, patients or their parents.

• Journal of the korean academy of Pediatric Dentistry
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• v.45 no.4
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• pp.464-473
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• 2018

The purpose of this study was to investigate the distribution of eruption disturbance and to analyze its causes, treatment methods, and duration of orthodontic traction, based on 703 patients with eruption disturbance who were treated in the pediatric dental clinic of Seoul National University Dental Hospital between July 2011 and June 2016. Eruption disturbance in pediatric patients was most prevalent in the maxillary canine, followed by the maxillary central incisor and maxillary first molar. Eruption disorder of the maxillary canine was more common in females (p < 0.001), whereas the maxillary central incisor (p = 0.009), maxillary first molar (p < 0.001) and mandibular first molar (p = 0.028) were more common in males than females. The most common causes of eruption disturbance were abnormality of the eruption pathway and the presence of obstacles in the pathway. Orthodontic traction was the most prevalent treatment choice for eruption disorder, mostly done for the maxillary central incisors. The duration of orthodontic traction was shorter with younger age (p < 0.001) and lower crown position (p < 0.001). It is important for pediatric patients to detect eruption disorders early through regular checkup, and it is necessary to initiate treatment at an appropriate time with an accurate diagnosis and treatment plan.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.2
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• pp.140-150
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• 2021

A total of 580 patients, who visited and received an orthodontic diagnosis in the Department of Pediatric Dentistry, Seoul National University Dental Hospital from 2017 to 2019, were investigated in this study. The aim of this study was to evaluate skeletal patterns of pediatric orthodontic patients determined with lateral cephalometric analysis and to analyze the relationship between skeletal pattern and probable associated clinical features. Also, the modality of orthodontic treatment for each skeletal classification was investigated to aid in therapeutic decisions. Patients aged 7 year accounted for the largest age group; 54.2% of patients showed a skeletal class I pattern, 22.2% showed a skeletal class II pattern, and 23.6% showed a skeletal class III pattern. Bi-maxillary retrusion for skeletal class I, retruded mandible with normal positioning of the maxilla for skeletal class II, and retrusion of the maxilla with protrusion of the mandible for skeletal class III were the largest subgroups by skeletal pattern. Brachyfacial type accounted for 55.0% of patients, followed by 31.9% of mesofacial type and 13.1% of dolichofacial type. The prevalence of anterior crossbite in the study was 43.3%, higher than that in previous studies.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.203-211
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• 2007

A nationwide survey on acute appendicitis (AAP) was undertaken among members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the 6 months period from the $1^{st}$ October, 2005 to $31^{st}$ March, 2006. Questionnaires were collected from 23 members. Four hundred seventy six patients were registered from 21 hospitals where the members were working. The male to female ratio was 1.67:1. Average age was 9 years and 5 months (range 21 months-20 years). Operations were performed on average 10.4 hours (range 1-230 hours) from arrival at hospital. The average operation time was 59.1 minutes. The average admission days were 5.8 days (range 2-45 days). The most frequent symptoms were abdominal pain (95.1 %), vomiting (50.6 %) and fever (43.7%). The average duration of symptoms was 42.2 hours (range 1 hour-22 days). Leukocytosis (WBC count > 10,000) was found in 85.9%. The most popular diagnostic tools were ultrasonography and CT. Open surgery was performed in 72.1 % and laparoscopic surgery in 27.5 %. Two laparoscopic surgeries were converted to open surgery (0.4 %). Simple appendicitis was found in 54.5 % and complicated appendicitis such as abscess, gangrenous change and perforation in 45.5%. According to the questionnaires 12 hospitals were performing open surgery only in all patients. Four hospitals were performing laparoscopic surgery in all patients. Seven hospitals are performing both surgical methods according to surgeon or occasion. Regarding the use of antibiotics in acute appendicitis, three kinds of antibiotics were used in 40 % of total simple appendicitis patients. The results showed the trend of management in acute appendicitis in Korea. Especially it is necessary for the members to discuss the use of antibiotics for prophylaxis in the simple appendicitis.