• 대한장애인치과학회지
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• 제10권1호
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• pp.26-30
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• 2014

프라더 윌리 증후군과 원인을 알 수 없는 심낭 삼출을 앓고 있는 3세 남환이 치아우식을 주소로 내원하여 임상 구강 검사 결과 다발성 우식증과 구강 전반에 걸친 치아 마모증이 관찰되었다. 약 10개월간 보존적인 수복치료와 구강 위생관리를 시도하였으나, 치아 우식의 빠른 진행과 환아의 협조도 부족으로 전신마취 하 우식 치료를 시행하였다.

• 대한구순구개열학회지
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• 제5권1호
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• pp.43-58
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• 2002

A General survey on the cleft lip and/or palate patients in Pusan National University Hospital was done. The conclusions were as follow. 1. In gender ratio, male was higher than female. 2. In the cleft type, unilateral cleft lip and palate, cleft lip and alveolus, and bilateral cleft lip and palate in higher ratio order. Left unilateral cleft lip and palate was higher than right in ratio. 3. In first hospital visiting age for dental treatment, the group from 6 years old to 12 years old was most and in that the age group for attending elementary school (about 7-8 years old) showed especially higher ratio. The large majority of patient were born in 1980's and lived in Pusan metropolitan city, KyungSangNamDo. Especially, most of them lived in the neighboring Held of Pusan National University Hospital. 4. Class III skeletal pattern and Angle's Class III molar relationship was most prevalent. Angle's Class II molar relationship showed relative higher ratio because tooth loss and malposition. 5. Primary lip closure in 3 months, secondary lip closure in 6-7 years old, palatal closure in 12-16 months and bone graft in 8-10 years old were operated mostly. Rhinoplasty and scar revision were operated in too early age so the need of infantile orthopedics must be considered. 6. Maxillary expansion and protraction were operated mostly in 8-11 years old but operating in primary dentition must be considered. 7. Tooth alignment were started mostly in mixed dentition and consideration about prosthodontic treatment and retention will be need. 8. In tooth anomaly, tooth malformation and missing were most prevalent.

• Clinical and Experimental Pediatrics
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• 제49권12호
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• pp.1358-1362
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• 2006

Niemann-Pick병은 lysosome내에 sphingomyelinase의 결핍으로 sphingomyelin이란 지질이 축적된 세포들이 간, 비장, 골수, 폐, 및 뇌 등에 침착되어 간, 비장 종대 및 신경증상을 나타내며, 상염색체 열성으로 유전되는 대사성 질환이다. 1914년 Niemann에 의해 처음 보고되어 Gaucher병의 한 변형으로 분류되어 있다가, 1927년 Pick에 의해 새로 분류되어 Niemann-Pick 병으로 명명되었다. 세계적으로도 희귀한 질환으로 국내에서는 1962년 정 등이 처음 보고한 이래 현재까지 저자가 조사한 바로 총 7례 정도가 보고되었다.저자들은 18개월 된 남아에서 임상증상 및 검사 소견으로 A 형 Niemann-Pick병으로 생각되는 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권5호
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• pp.423-429
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• 2020

Purpose: Alimentary tract duplication (ATD) is a rare congenital condition that may occur throughout the intestinal tract. Clinical symptoms are generally related to the involved site, size of duplication, or associated ectopic mucosa. This study aimed to identify clinical implications by anatomical locations and age group and then suggest a relevant management according to its distinct features. Methods: We retrospectively reviewed the clinical data of pediatric patients who received a surgical management due to ATD. Furthermore, data including patients' demographics, anatomical distribution of the duplication, clinical features according to anatomical variants, and outcomes were compared. Results: A total of 25 patients were included in this study. ATD developed most commonly in the midgut, especially at the ileocecal region. The most common clinical presentation was abdominal pain, a sign resulting from intestinal obstruction, gastrointestinal bleeding, and intussusception. The non-communicating cystic type was the most common pathological feature in all age groups. Clinically, prenatal detection was relatively low; however, it usually manifested before the infantile period. A laparoscopic procedure was performed in most cases (18/25, 72.0%), significantly in the midgut lesion (p=0.012). Conclusion: ATD occurs most commonly at the ileocecal region, and a symptomatic one may usually be detected before the early childhood period. Surgical management should be considered whether symptom or not regarding its symptomatic progression, and a minimal invasive procedure is the preferred method, especially for the midgut lesion.

• Advances in pediatric surgery
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• 제11권1호
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• pp.27-33
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• 2005

Infantile hypertrophic pyloric stenosis (HPS) is a relatively common entity. A number of studies for the postoperative feeding schedule has been studied to allow for earlier hospital discharge and improve cost-effectiveness in the treatment of HPS. The purpose of this study was to compare 3 feeding-methods and to evaluate the usefulness of ad lib feeding for HPS. The authors retrospectively reviewed the records of 116 patients who underwent pyloromyotomy for HPS from 1995 to 2004. Three cases were excluded because of the duodenal perforation during pyloromyotomy. Three feeding-methods were defined as: Conventional feeding (>10 hours nothing by mouth and incremental feeding every 2 hours, C), Early feeding(for 4 to 8 hours nothing by mouth and incremental feeding every 2 hours, E), and Ad lib feeding (for 4 hours nothing by mouth and ad lib feeding, A). Time to normal feeing in C, E and A were $51{\pm}24$, $34{\pm}12$ and $24{\pm}6$ hours, respectively. Hospital-stay in C, E and A were $72{\pm}17$, $55{\pm}13$ and $43{\pm}12$ hours, respectively. There were statistically significant differences according to the method of feeding. Frequency of postoperative emesis in C, E and A were 38 %, 47 % and 53 %, but was not significant statistically. Ad lib feeding decreased time to normal feeding and hospital stay, and did not increase postoperative emesis. We conclude that ad lib feeding is recommended for patient with pyloromyotomy in HPS.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.254-258
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• 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.

• 영어영문학
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• 제56권2호
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• pp.301-328
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• 2010

Many recent British novels engage with the construction and deconstruction of history and identity; and in dealing with these historical, or historicised novels it seems to be an untouchable ground that truth is beyond grasp. Even when approached, its authenticity should be examined under the post-modern "incredulity toward metanarrative" discourses. Julian Barnes's 1998 novel England, England may be one of these. Yet, unlike others it achieves a complicated and controversial status as a new kind of historiographic metafiction by providing selfconscious reflections on the invention of innocence and the questionable notion of historical authenticity against the background of current postmodern historical, cultural, and literary explorations. The book, set in a near-future, namely post-post-modern England, starts with a story of a young girl, Martha Cochrane, whose first memory goes back to her early infantile years. Yet, the narrator comments that it is a lie, "her first artfully, innocently arranged lie," since memory, or history, is a product of identity, and vice versa. Her memory of the jigsaw puzzle is both a reminiscent and a significant component of who she is now, both a simulacrum and the original of herself. The correlation between her individual memory and identity parallels that of a region, England, in formation of its history and nationality. "England, England" is the replicated miniature of the former glorious Kingdom as well as a becoming der Ding an sich (the thing itself). In search of the English history and identity, the author satirizes the modern mind's perception of the unreliability and arbitrariness of memory and history, and further explores the alternative to the postmodern discourses by suggesting the probability of inventing innocence glimpsed in children's face "believing while disbelieving." In doing so, the author reconstructs not only the history of Englishness on the ground where nothing seems to be solid, but more importantly also the postmodern theme of relativity in relation to memory, history and identity.

• Journal of Genetic Medicine
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• 제21권1호
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• pp.31-35
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• 2024

Allan-Herndon-Dudley syndrome (AHDS) is a rare X-linked neurodevelopmental disorder with abnormal thyroid function caused by mutation in the solute carrier family 16 member 2 (SLC16A2) gene. Clinical manifestations of AHDS are global or axial hypotonia, a variety of movement disorders, severe intellectual disability, quadriplegia or spastic diplegia, growth failure, and seizures. A 10-year-old boy visited our hospital with the chief complaint of newly onset generalized tonic seizures with vocalization of weekly to daily frequency. He showed early infantile hypotonia, severe intellectual disability, and frequent respiratory infections. He could not walk independently and was non-verbal. Electroencephalogram revealed generalized slow spike and waves with multifocal spikes and slow background rhythms. His tonic seizures were controlled with more than two anti-seizure medications (ASMs). At 11 years of age, he was evaluated for thyroid function as part of regular screening for ASM maintenance and was found to have abnormal thyroid function. We performed whole exome sequencing for severe global developmental delay, drug-resistant epilepsy, and abnormal thyroid function. The hemizygous c.940C>T (p.Arg314Ter) variant in the SLC16A2 gene (NM_006517.5) was identified and confirmed based on Sanger sequencing. Herein, we describe a case of an AHDS patient with late-onset drug-resistant epilepsy combined with congenital hypotonia, global developmental delay, and abnormal thyroid function results. To the best of our knowledge, this is the oldest adolescent among AHDS cases reported in Korea. In this report, clinical characteristics of a mid-adolescence patient with AHDS were presented.

• 대한소아치과학회지
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• 제36권3호
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• pp.337-347
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• 2009

영유아기 치아우식증은 복잡하고 다인자적 질환으로 숙주 및 미생물, 식이, 시간 등의 위험요소에 의해 질환이 진행된다. 이 중 미생물로 대변되는 Streptococcus mutans(S. mutans)는 우식 개시에 있어 중요한 역할을 하며, 어머니를 통한 수직 감염이 잘 알려져 있다. 영유아기의 어머니를 통한 조기 감염은 향후 유치열기 우식 발생과 높은 연관성을 나타내므로 우식위험요소에 대한 조기 파악은 미래의 치아우식증 예방에 있어 매우 중요하다. 최근 치아우식증의 원인 및 이에 미치는 요소들에 대한 연구가 활발하게 이루어지면서 우식 진행에 대한 정확한 예측을 위한 여러 가지 우식위험도평가(caries risk assessment) 도구가 개발되었는데, 이 중 대표적인 한 가지가 Cariogram이다. Cariogram은 개인의 우식활성도 및 우식위험요소(caries risk factor)의 상호작용을 그래프로 나타낸다. 이번 연구의 목적은 3${\sim}$5세의 중증 유아기우식증(Severe Early Childhood Caries; S-ECC)에 해당되는 어린이들과 그들의 어머니 사이에서 우식관련타액검사 및 최근 개발된 Cariogram을 이용하여 S. mutans의 수준 및 우식활성도에 대한 상관관계가 존재함과 우식위험요소 간의 연관성을 파악해 보았는데, 이를 통해 다음과 같은 결론을 얻을 수 있었다. 1. 중증 유아기우식증을 보이는 어린이에서 구강 내 존재하는 Streptococcus mutans의 수준은 비우식군보다 높았다(p<0.05). 2. 모자간 구강 내 존재하는 Streptococcus mutans 수준은 상관관계를 보였다(p<0.05). 3. 모자간 우식활성도를 Cariogram을 통해 측정해 본 결과 상관관계를 보였다(p<0.05). 4. 우식활성도에 미치는 우식위험요소는 어린이에서 숙주요소가 가장 큰 영향을 주는 것으로 나타났다. 이상의 결과로 S. mutans가 향후 치아우식증 발생 가능성을 예측하는데 있어 유용한 우식위험요소가 될 수 있으며, Cariogram을 통한 향후의 우식진행과 관련된 여러 가지 우식위험요소가 예측 가능하므로 이에 대한 조기 대처 및 예방적 처치가 가능하리라 사료된다.

• Clinical and Experimental Pediatrics
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• 제51권8호
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• pp.804-811
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• 2008

목 적 : 최근 소아비만이 증가 추세에 있으며, 소아비만은 성인비만으로 이행할 가능성이 높다. 그러므로 소아기 부터 비만의 예방이 중요하다. 이에 본 연구는 학령전기 소아비만에 영향을 주는 영유아기 인자를 알아보고자 하였다. 방 법 : 2007년 10월 부산에 위치한 어린이집과 왈레스기념 침례병원 외래를 방문한 만 3세 이상 5세 미만 아동 350명을 대상으로 하여 그들의 부모에게 설문조사를 하였고, 그중 223명이 최종 분석에 사용되었다. 아동들의 현재 신장과 체중에 대한 신체계측은 어린이집과 병원에서 전자체중계와 신장계를 이용하여 측정하였으며, 체질량지수를 산출하여 85백분위수 이상인 경우 과체중위험 군, 85백분위수 미만인 경우 정상체중군으로 분류하였다. 설문조사를 통해 부모의 일반적인 특성(어머니 학력, 직업유무, 부모의 비만 여부), 출생체중, 수유 관련 내용(수유방식, 밤중 수유 지속 기간, 이유식 시작 시기), 양육자에 대한 정보를 얻었다. 결 과 : 과체중위험 군에 속하는 소아는 68명(30.5%)이었고, 정상체중군에 속하는 소아는 155명(69.5%)이었다. 분유를 수유한 경우가 모유를 수유한 경우에 비해 과체중이상인 경우가 11.3배 높게 나타났고(P<0.05), 출생 후 첫 6개월간 빠른 체중 증가가 학령전기 소아 과체중위험 군과 연관이 있는 것으로 나타났다(P<0.05). 과체중위험 군의 어머니의 체질량지수($22.2{\pm}2.4kg/m^2$)가 정상체중군($20.6{\pm}2.3kg/m^2$)에 비해서 유의하게 높았다(P<0.05). 아버지의 체질량지수(P=0.658), 어머니 학력(P=0.134), 직업유무(P=0.396), 밤중 수유 지속 기간(P=0.530), 이유식 시작 시기(P=0.078), 양육자(P=0.881), 6-12개월간 빠른 체중 증가(P=0.628), 출생체중(P=0.598)은 비만과 유의한 관련이 없었다. 결 론 : 소아비만이 될 영유아기 인자를 가지고 있는 자녀를 둔 가족들에게 영유아기에 초점을 맞춘 비만 예방 프로그램을 적극 제시해 주어, 영유아기 부터 비만의 위험을 예방하는 것이 중요할 것으로 사료된다.