• Title/Summary/Keyword: No-No Boy

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Cytomegalovirus-induced Childhood Menetrier's Disease with Peripheral Eosinophilia (호산구 증가증을 동반한 거대세포바이러스 감염 소아 Menetrier병 1례)

  • Choi, Won Joung;Lee, Bo Young;Lee, Hee Jung;Oh, Hoon Kyu;Hwang, Jin Bok
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.87-91
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    • 2004
  • Herein, the case of a 5-year-old boy with cytomegalovirus-induced Menetrier's disease, with peripheral eosinophilia, presenting with abdominal pain and vomiting, followed by generalized edema, is reported. The initial laboratory findings, hypoalbuminemia and peripheral eosinophilia were noted, with no evidence of renal, hepatic, cardiac or allergic diseases. Gastrofiberscopy was performed under the suspicion of eosinophilic gastroenteritis with protein losing gastropathy. The gastrofiberscopy showed the characteristic features of giant hypertrophy of the gastric rugae, with large quantities of adherent gelatinous material on the gastric fundus and body. The histological findings revealed foveolar hyperplasia, compatible with Menetrier's disease, with massive eosinophilic infiltrations. The presence of cytomegalovirus infection was identified by serology and confirmed by urine PCR. His symptoms, gastrofiberscopic findings and peripheral eosinophilia resolved spontaneously, and he has remained well for 10 months.

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A Case of DiGeorge Syndrome with Metopic Synostosis (전두봉합유합증(Metopic synostosis)을 동반한 DiGeorge 증후군: 증례보고)

  • Kim, Sue-Min;Park, Sun-Hee;Kang, Nak-Heon;Byeon, Jun-Hee
    • Archives of Plastic Surgery
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    • v.38 no.1
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    • pp.77-80
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    • 2011
  • Purpose: We report a patient with DiGeorge syndrome who was later diagnosed as mild metopic synostosis and received anterior 2/3 calvarial remodeling. Methods: A 16-month-old boy, who underwent palatoplasty for cleft palate at Chungnam National University Hospital when he was 12 months old of age, visited St. Mary's Hospital for known DiGeorge syndrome with craniosynostosis. He had growth retardation and was also diagnosed with hydronephrosis and thymic agenesis. His chromosomal study showed microdeletion of 22q11.2. On physical examination, there were parieto-occipital protrusion and bifrontotemporal narrowing. The facial bone computed tomography showed premature closure of metopic suture, orbital harlequin sign and decreased anterior cranial volume. The interorbital distance was decreased (17 mm) and the cephalic index was 93%. Results: After the correction of metopic synostosis by anterior 2/3 calvarial remodeling, the anterior cranial volume expanded with increased interorbital distance and decreased cephalic index. Fever and pancytopenia were noted at 1 month after the operation, and he was diagnosed as hemophagocytic lymphohistiocytosis by bone marrow study. He however, recovered after pediatric treatment. There was no other complication during the 12 month follow up period. Conclusion: This case presents with a rare combination of DiGeorge syndrome and metopic synostosis. When a child is diagnosed with DiGeorge syndrome soon after the birth, clinicians should keep in mind the possibility of an accompanying craniosynostosis. Other possible comorbidities should also be evaluated before the correction of craniosynostosis in patients as DiGeorge syndrome. In addition, postoperative management requires a thorough follow up by a multidisciplinary team of plastic surgeons, neurosurgeons, ophthalmologists and pediatricians.

Treatment in Bimaxillary Prognathism with Anterior Open Bite: A Case Report (전치부 개방교합을 지닌 상악골 및 하악골 전돌증의 치료: 증례 보고)

  • Chun, Sang-Deuk;Chin, Byung-Rho
    • Journal of Yeungnam Medical Science
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    • v.21 no.2
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    • pp.242-250
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    • 2004
  • In general, the skeletal class III has the characteristics of mandibular overgrowth with a normal maxillary growth or maxillary undergrowth with a normal mandibular growth And clinical and radiographic evaluations of the patient are needed. However, the treatment plan is not dependent on these evaluations alone, because patient's general condition and hope for aesthetics varies. The aim of this report is to consider the treatment of a medically compromised patient with an anterior open bite and skeletal class III, which showed a severe mandibular overgrowth. In 2003, a 17-year-old boy with epilepsy, mental retardation presented at our clinic complaining of concave profile. A clinical examination showed severe mandibular prognathism with an anterior open bite. The radiographic examination revealed a short cranial base, a moderate maxillary overgrowth, severe mandibular overgrowth and skeletal open bite tendency. In 2004, he was verified to have no potential of growth by hand-and-wrist radiographs and an endocrine examination. He completed the preoperative orthodontic treatment and orthognathic surgery (sagittal split ramus osteotomy, genioplasty). He was evaluated on the first visit, the preoperative period and the postoperative period with a clinical and radiographic examination. At the first visit, the patient showed moderate overgrowth of the maxilla, severe overgrowth of the mandible, and a subsequential skeletal open bite. After the preoperative orthodontic treatment (preoperative period), the patient showed the same skeletal problem as before and a decompensated dentition for orthognathic surgery. After orthognathic surgery, his profile had improved, but he had still a skeletal openbite tendency because the maxillary orthognathic surgery was not performed. Severe mandibular prognathism with a maxillary overgrowth and anterior open bite should be treated by bimaxillary orthognathic surgery. However, one-jaw orthognathic surgery on the remaining the skeletal open bite tendency was performed for his medical problem and facial esthetics. This subsequential open bite should be resolved with a postoperative orthodontic treatment.

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A STUDY OF MIDDLE SCHOOL BOY'S REPORT ON THE FACTORS THAT AFFECT THEIR RELATIONSHIPS WITH FATHERS (남자 중학생들이 보고하는 부자관계에 영향을 주는 요소에 관한 연구)

  • Cho, Sun-Mi;Noh, Kyung-Sun;Lee, Ho-Young;Kim, Hyun-Soo
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.10 no.1
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    • pp.113-120
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    • 1999
  • Our aims is a exploration for relationship between father and male-adolescence. For this purpose, we have employed several self-reported questionnaires, including BDI, Rosenberg self-esteem scale, Attitude for father etc. Our hypothesis is that the perception, the attachment, age and alcohol drinking, accademic career of father influences male-adolescent's self-esteem, mood, sex-role, academic accomplishment, satisfying of school life. We have surveyed questionnaire at one middle school, at Suwon city. We have get 196 samples. We have categorized subjective answers and analyzed relations. The result is that a positive relation is self-esteem, sex-role, and attachment, a neagtive relation is mood and no relation is age, academic career, alcohol drinking. Also, father's overprotection attribute on negative influences. We have many limitations. First limitation is a small number. Second is few previous study. Third is a relative analysis, not cause-effect analysis.

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Cranioplasty in Frontometaphyseal Dysplasia (전두부 골간단 이형성증의 광범위한 수술적 치료)

  • Jung, Eui Seok;Mun, Goo Hyun;Lim, So Young;Hyon, Won Sok;Bang, Sa Ik;Oh, Kap Sung;Pyun, Jae Kyung
    • Archives of Plastic Surgery
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    • v.33 no.3
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    • pp.371-375
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    • 2006
  • Frontometaphyseal dysplasia is a rare genetic syndrome first described by Gorlin and Cohen in 1969. This disease affects the skeletal system and connective tissue, and could be characterized by hyperostosis of the skull, prominence of supraorbital ridges, additional skeletal and extraskeletal abnormalities. It is believed that the condition is an X-linked dominant trait with severe manifextations in males and extreme variability in females.We described a case of 15-year-old boy manifested a pronounced supraorbital ridge associated with exorbitism. He also had bilateral progressive hearing deficit, thoracic spine scoliosis, chest wall deformity, bilateral maxillary sinusitis and both 5th finger arachnodactyly.The patient underwent a fronto-temporo-orbital cranioplasty through a coronal incision. The frontal bone including supraorbital region, orbital lateral rim and temporal bone were extensively contoured with burr. And then, burring of the medial aspect of lateral orbital wall was made to increase both orbital volume for correction of exorbitism. Postoperative results show well corrected prominent supraorbital ridge, hyperostosis of frontotemporal bone and exorbitism. The patient was satisfied with the improved appearance. No recurrence occurred during the 6 months of follow-up period. We report this case as it shows esthetically good result without any complication.

Recurrent Acute Pancreatitis Associated with Sphincter of Oddi Dysfunction in a Child (오디 괄약근 기능 이상으로 인한 소아의 재발 급성 췌장염 1예)

  • Choi, Byung-Ho;Park, Sun-Min;Kim, Ho-Gak;Kim, Jung-Mi;Hong, Suk-Jin;Kim, Jung-Ok;Cho, Min-Hyun;Choe, Byung-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.193-197
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    • 2008
  • Recent studies suggest that sphincter of Oddi dysfunction (SOD) is one of the possible causes of unexplained recurrent acute pancreatitis in children. A 14-year-old boy who had suffered from idiopathic recurrent acute pancreatitis was diagnosed with SOD. Abdominal ultrasonography, computerized tomography, and magnetic resonance cholangiopancreatography revealed no evidence of stone, tumor, or pancreatic ductal anomaly. Endoscopic retrograde cholangiopancreatography (ERCP) and sphincter of Oddi manometry (SOM) revealed elevated basal pressure and tachyoddia consistent with SOD. Hence, an endoscopic pancreatic sphincterotomy was performed. We report a case of recurrent acute pancreatitis associated with SOD in a child. ERCP and SOM may be considered in patients with multiple unexplained attacks of pancreatic pain and negative abdominal imaging.

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A Case of Crigler-Najjar Syndrome Type 2 Diagnosed Using Genetic Mutation Analysis (유전자 검사로 진단된 제2형 Crigler-Najjar 증후군 1예)

  • Kim, Sang-Yee;Lee, Soo-Hyun;Koh, Hong;Lee, Seung-Tae;Ki, Chang-Seok;Kim, Jong-Won;Chung, Ki-Sup
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.219-222
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    • 2008
  • Crigler-Najjar syndrome is a rare inherited disease associated with unconjugated hyperbilirubinemia. It is inherited via an autosomal recessive pattern and is caused by mutation in one of the five exons of the bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (UGT1A1) gene. The synthesis of inactive isoforms of bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (B-UGT) results in unconjugated hyperbilirubinemia. A 13-year-old boy with jaundice for 4 months was admitted to our hospital. He had unconjugated hyperbilirubinemia with no evidence of infection, hemolysis, or structural abnormalities on abdominal ultrasonography or 99mTc-DISIDA scan. The authors identified a missense mutation of Tyr486Asp in the fifth exon of the UGT1A1 gene and diagnosed the patient with Crigler-Najjar syndrome type II. This is the first reported case of Crigler-Najjar syndrome in a Korean child, and it is also the first reported case of a genetic mutation leading to Crigler-Najjar syndrome in Korea.

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Congenital Esophageal Atresia and Tracheoesophageal Fistula: Report of Two Cases (선천성 식도폐쇄 및 기관식도루: 2례 보고)

  • 김형묵
    • Journal of Chest Surgery
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    • v.6 no.1
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    • pp.89-94
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    • 1973
  • Esophagel atresia and tracheoesophageal fistula may occur as separate entities but usually occur in combination. First described by Durston in 1670, esophageal atresia was not successfully treated until 1939 when Ladd in Boston and Leven in St. Paul obtained the first survivors utilizing the methods of gastrostomy, esophagostomy and extrapleural ligation of the tracheoesophageal fistula as multiple operations which required months of hospitalization. Two years later Cameron Haight performed the first successful primary repair and afterward about 2000 cases of esophageal atresia with distal tracheoesophageal fistula reported in the world. In Korea, there appeared about 27 cases in the literature and 8 successful repaired cases noted in these year. Anther report two cases of esophageal atresia, of which one case was successfully treated with Haight`s method. Case 1.: Normal full term delivered boy with chief complaints of respiratory difficulty and persistent drooling with chocking, 3. lkg, was admitted with emergency 5 hours after delivery. Physical findings revealed no specific abnormal signs except distended abdomen and grunting respiration. Esophagograrn and bronchogram revealed proximal esophageal atresia and distal tracheoesophageal fistula proximal to the carina. Parent refused operative therapy and patient died 24 hours after discharge. Case 2. :3. lkg. normal full term delivered girl was admitted 4 days after delivery with chief complaints of regurgitation after feeding, chocking, cyanotic spell and fever since the day after delivery. Physical examination revealed persistent drooling, grunting respiration, and fever with moderate dehydration. Tracheoesophageal suction and fluid therapy with antibiotics improved her condition and subsided ]pneumonic condition. Esophagogram revealed markedly dilated proximal esophagus as blind loop and stomach distended with gas, and repairing operation as Haight`s method was performed on the 7th day after delivery. Patient tolerated all the operative procedure well and recovered uneventfully. Esophagogram on the 7th postoperative day showed passage of the lipiodol through the anastomotic side with moderate stricture,and feeding permitted. Patient tolerated all the feeding amount well and discharged on the 11th postoperative day. Followup revealed intermittent regurgitation after feeding and corrected with bougination.

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Young Children's Basic Habits Development by Parents' Recognition about the Importance of Basic Habits and Parenting Styles (기본생활습관의 중요성에 대한 부모의 인식 및 양육태도에 따른 유아의 기본생활습관 형성)

  • Kim, Song Yee
    • Korean Journal of Childcare and Education
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    • v.7 no.3
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    • pp.101-124
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    • 2011
  • The purpose of this study was to examine the differences of children's basic habits formation by their mother's and father's parenting style and their recognition about the importance of basic habits. The participants of this study were 160 three and four-year-old children's parents. The results of this study are as follows. The parental features on children's basic habits formation were significant different by children's sex. Girls' basic habits were significant different by their mother's and father's recognition about the importance of basic habits. On the other hand, boy's basic habits were significant different by mother's and father's affectionate parenting and their recognition about the importance of basic habits. Especially, boys whose parents did not consider important their children's basic habits formed higher level of basic habits in case that their mothers showed affectionate parenting. Also, there are no significant differences in children's basic habits by autonomous parenting.

DENTAL MANAGEMENT OF ECTODERMAL DYSPLASIA : A CASE REPORT (외배엽 이형성증 환자의 치험례)

  • Jang, Hyang-Gil;Lee, Sang-Ho;Lee, Nan-Young
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.4
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    • pp.631-639
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    • 2009
  • Ectodermal dysplasia is a genetic disease which shows various congenital dysplasias in tissues differentiated from the ectoderm. As the most common type of the ectodermal dysplasia, hypohidrotic ectodermal dysplasia(HED) shows dysplasia mainly in the hair, fingernails, teeth and the skin. Symptoms are more severe in males than in females and heterozygous females are usually normal showing no symptom. The treatment for these patients differ according to individuals, but since patients can easily become depressed socially and emotionally due to a decrease in mastication and speech function caused by multiple loss of teeth as well as some aesthetic problems, an early treatment is required. In a case, with a 10 years-old boy diagnosed with HED which shows partial edentia of the maxilla, and the edentia of the mandible in the pediatric dentistry department of the Chosun University Dental Hospital, a fabrication of denture resulted in the recovery of mastication and speech function and aesthetic improvement due to an increase of the face height.

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