• 대한장애인치과학회지
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• 제12권2호
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• pp.92-95
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• 2016

Beckwith-Wiedemann syndrome은 다양한 임상적 특징을 보이는 질환이다. 거대설은 BWS 환아에서 가장 흔히 관찰되는 특징으로 그로 인한 섭식 문제와 호흡곤란이 야기될 수 있어 치과 치료 시, 주의를 요하게 된다. 또한 거대설로 야기될 수 있는 골격적인 문제에 대한 장기적인 평가가 필요하다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제25권1호
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• pp.61-69
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• 2022

Purpose: Studies in adults have shown an increasing incidence of Clostridioides difficile infection (CDI) in patients hospitalized with acute pancreatitis (AP). There is lack of epidemiological data on CDI and its impact on hospitalized pediatric patients with AP. Methods: We analyzed the National Inpatient Sample and Kids' Inpatient Database between the years 2003 and 2016 and included all patients (age <21 years) with a primary diagnosis of AP using specific International Classification of Diseases codes. We compared clinical outcomes between children with CDI and those without CDI. Our primary outcome was severe AP and secondary outcomes included length of stay and hospital charges. Results: A total of 123,240 hospitalizations related to AP were analyzed and CDI was noted in 0.6% of the hospital. The prevalence rate of CDI doubled from 0.4% (2003) to 0.8% (2016), p=0.03. AP patients with CDI had increased comorbidities, and also underwent more invasive surgical procedures, p<0.05. AP patients with CDI had a higher in-hospital mortality rate and increased prevalence of severe AP, p<0.001. Multivariate regression models showed that CDI was associated with 2.4 times (confidence interval [CI]: 1.91 to 3.01, p<0.001) increased odds of severe AP. CDI patients had 7.24 (CI: 6.81 to 7.67, p<0.001) additional hospital days while incurring $59,032 (CI: 54,050 to 64,014, p<0.001) additional hospitalization charges. Conclusion: CDI in pediatric patients with AP is associated with adverse clinical outcomes and increased healthcare resource utilization. Further studies are needed to elucidate this association to prevent the development of CDI and to improve outcomes.

• Advances in pediatric surgery
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• 제15권1호
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• pp.64-67
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• 2009

Splenic cystic lesion is uncommon in children, and cystic lymphangioma of the spleen has not been reported in Korean pediatric patients. Here we report a case of cystic lymphangioma arising from the spleen in a 16 year-old male. The patient presented with left flank pain for 5 days after blunt trauma to the same site. On physical examination, left abdominal tenderness and a palpable spleen were noted. Abdominal ultrasound and MRI revealed multiple septated macro-cystic mass abutting to the spleen medially, suggestive of cystic lymphangioma of the spleen. Laparotomy revealed a 20 cm sized cyst in the spleen, and 2,000mL of dark-brownish fluid was aspirated from the cyst. Splenectomy was performed. Pathological examination revealed the cystic lymphangioma. Post-operative recovery was uneventful, and the patient was discharged at 7 days after surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권3호
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• pp.196-200
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• 2014

Co-existing pyloric submucosal masses with hypertrophic pyloric stenosis (HPS) are very rare and treating these lesions is always a problem. A 20-day-old boy presented with recurrent episodes of projectile non-bilious vomiting lasting for 5 days. HPS was suspected due to the presenting age and the symptoms. The sonography demonstrated not only circumferential wall thickening of the pylorus, but also a pyloric submucosal mass. At laparotomy, a 0.8 cm sized pyloric submucosal mass was identified along with a hypertrophied pylorus. Pyloric excision was performed due to the possibility of sustaining the symptoms and malignancy. The pathological report of the submucosal mass was ectopic pancreas. Coexisting pyloric lesions can be diagnosed along with HPS, and surgical excision, not just pyloromyotomy, should be considered in these circumstances. To the best of our knowledge, this is the first case report of pyloric ectopic pancreas and HPS to be diagnosed concurrently.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.487-492
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• 2019

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

• Advances in pediatric surgery
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• 제6권2호
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• pp.106-123
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• 2000

The purpose of the survey was to clarify the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7 : 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(< 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were high(H) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed.

• 대한소아치과학회지
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• 제24권3호
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• pp.597-601
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• 1997

This paper presents improper use of latex-elastic ring in movement of teeth and its consequence. Simple orthodontic movement of teeth including closure of diastema may be achieved by the use of simple method with fixed or removable appliance associated with latex-elastic ring. But, if it is used alone, it acts as a local irritant and then produces localized periodontitis. In case of localized periodontitis resulting from latex-elastic ring, diagnosis is difficult because the patient is usually unaware of the presence of the rings on the roots of the involved teeth and because the ring is not probed and is radiolucent and therefore not discernible radiographically and clinically. Occasionally, surgery is requred for the detection. This case emphasizes the necessity that the use of latex-elastic ring in closing diastema should be accompanied with removable or fixed appliance as well as close professional supervision. Its use without adequate provision for stabilization or retention on the crowns of teeth results in destruction of periodontal support and the teeth may be lost.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권3호
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• pp.207-209
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• 2016

Benzalkonium chloride (BAC) is a caustic agent which is used in farms, homes and hospitals for cleaning skin and wounds as an antiseptic solution. It may lead to digestive system injuries in case of ingestion. We present a two-days-old newborn case which was carried to the emergency unit with complaints of poor breastfeeding, uneasiness and crying for 4-6 hours. Her mom confessed that she had given a spoon of 10% BAC solution for her cough. Initial laboratory tests were in normal ranges. A gastroscopy performed in the second hour of her admission revealed an hyperemic and edematous mucosa in the middle third of esophagus and a circumferential ulceration followed in the distal portion. Hereupon, a conservative treatment for 10 days was administered and the control gastroscopy demonstrated that the damage was almost totally improved. She was the youngest case with this etiology and successfully treated with conservative approach.

• 대한소아치과학회지
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• 제24권4호
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• pp.776-780
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• 1997

Patient with alveolar abscess due to dental caries with severe alveolar bone loss, severe tooth mobility, root resorption need extraction of tooth because it is impossible to carry out pulp treatment and restoration by using conventional method. Early loss of primary molar might cause masticatory interference, extrusion of opposing tooth, problem in maintaining space and interference on eruption of permanent tooth. Especially, early loss of primary second molar before the eruption of permanent first molar might cause space closure by mesially erupted permanent first molar and impaction of second premolar. In such a case, distal shoe space maintainer and removable space regaining appliance was the first choice of treatment. But, distal shoe space maintainer need precise adaptation and might cause chronic inflammation if the oral hygiene is poor. In a case using removable space regaining appliance, patient's cooperation is most important. If the distal root of primary second molar is comparably sound and alveolar abscess with alveolar bone loss is localized at mesial root, hemisection should be carried out for precise guide to eruption of the permanent first molar, restoration of masticatory fuction and solution to the discomfort of the patient

• 대한소아치과학회지
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• 제24권4호
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• pp.735-742
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• 1997

Class II malocclusion can be treated via early orthopedic, orthodontic treatment or orthognathic surgery with orthodontic treatment. In the mixed dentition, early orthopedic treatment can be used. Especially, in the case of mandibular retrognathism, the functional appliances can be used, and in the case of maxillary protrusion is combined, they can be used together with headgear. After using activator and activator combined with headgear to the class II malocclusion paitent in the mixed dentition, the results were as follows: 1. Lateral profile was improved, and lower face height was increaed. 2. Overjet was decreased, and molar relationship was changed to class I molar relationship. 3. Growth can be undisturbed, and the aggravation of malocclusion can be prevented to make the 2nd phase orthodontic treatment be much easier.