고체온으로 경련이 유발된 경우 경련의 양상과 쥐 해마부의 Neuronal Nitric Oxide Synthase 발현

김두권,정동근,Kim, Doo-Kwun,Jung, Dong-Keun 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.5

Purpose : Our study was aimed to investigate the characteristics of seizures as well as to determine whether the expression of neuronal nitric oxide synthase expression(nNOS) of hippocampus has an affect in the hyperthermic seizure in developing rat. Methods : Hyperthermic seizures were repeatedly induced twice a week for four weeks in 20-day old Spraque-Dowley rats. Fifty two rats were used as a hyperthermic group and 30 rats used as a normothermic control group. Hyperthermic seizures were induced in a water bath at $45^{\circ}C{\pm}1$ for 4 min. The characteristics of seizures were recorded. Using western blot, hippocampal nNOS expression was measured in normothermic control, hyperthermic non-seizure, and hyperthermic seizure groups, respectively. Results : Eighty seven percent of hyperthermia exposed rats showed generalized tonic-clonic seizure most frequently. The duration of seizure was ranged from 12 to 145 sec(mean 55 sec) and the latency to seizure ranged from 158 to 240 sec(mean 204 sec). The duration of seizure was prolonged but there was no significant difference in the seizure latency as the rat exposed more number of hyperthermia. Interestingly, the expression level of hippocampal nNOS in hyperthermic seizure and hyperthermic non-seizure groups was not different from each other, however, the expression in these groups was lower than that of the control group. Conclusion : Our results indicate that nNOS do not have an affect in this repeated hyperthermic seizures. Further studies are required to clarify a role of nNOS in hyperthermic seizure.

짠 음식으로 유발된 가족성 저칼륨성 주기성 마비 2례

김현지(Hyun Ji Kim),이소영(So Young Lee),김미영(Mi Young Kim),김나연(Na Yun Kim),이선주(Sun Ju Lee),김원덕(Won Duck Kim),조성민(Sung Min Jo),이동석(Dong Seok Lee),김두권(Doo Kwun Kim),최성민(Sung Min Choi) 대한소아신경학회 2002 대한소아신경학회지 Vol.10 No.2

저칼륨성 가족성 주기성 마비증은 의식, 언어, 지각의 장애 없이 골격근의 마비를 초래하는 질환으로 상염색체 우성 유전으로 남아에게 좀더 심하게 나타나며 대개 소아 후기에 발생하는 비교적 희귀한 질환이다. 본 증례에서는 주로 운동 후의 휴식 시에 증상이 발현되지만 그 외 특이하게도 다른 보고에서 찾아볼 수 없는 라면이나 피자, 김치 같은 짠 음식을 먹었을 때 사지 마비 증상이 나타났다. 이에 저자들은 짠 음식으로 유발된 저칼륨성 가족성 주기성 마비등 2례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Hypokalemic periodic paralysis is an autosomal dominant disorder characterized by episodic weakness of skeletal muscle associated with hypokalemia. It may be presented as familial or sporadic. Familial hypokalemic periodic paralysis is caused by mutations in the muscle membrane dihydropyridine sensitive calcium channel alpha-1 subunit. The abnormal genes in most cases are located in the chromosome 1q31-32. We experienced 2 cases(a 14-years-old male, a 12-year old male) of familial hypokalemic periodic paralysis. Two cases presented with quadriplegia after eating salty foods. The diagnosis was made by demonstrating decreased plasma level of potassium. The patient responded dramat-ically to potassium replacement therapy. We report two cases of familial hypokalemic periodic paralysis caused by salty foods with a brief review of related literatures.

Crohn병 1례

김지은,김준호,이동석,김두권,최성민,김우택,Kim, Ji-Eun,Kim, Jun-Ho,Lee, Dong-Seok,Kim, Doo-Kwun,Choi, Sung-Min,Kim, Woo-Taek 대한소아소화기영양학회 1999 Pediatric gastroenterology, hepatology & nutrition Vol.2 No.2

Crohn disease is a chronic transmural inflammatory disease that may involve any portion of the gastrointestinal tract. An increased incidence of Crohn disease in the general population has been reported, along with a greater than threefold increase of Crohn disease in children under the age of 16 years noted in a recent study. Crohn disease may be seen as early as infancy, but the most common pediatric age of onset is during the teenage period. We experienced a case of Crohn disease in 6 year old male child complained abdominal pain, oral aphthous ulcers, arthralgia, anorexia, and growth failure. A brief review of related literature is also presented.

수유 및 분만방법에 따른 신생아실 로타바이러스 원내감염의 증상 차이

이소영,김현지,김미영,김원덕,이동석,김두권,최성민,Lee, So Young,Kim, Hyun Ji,Kim, Mi Young,Kim, Won Duck,Lee, Dong Seok,Kim, Doo Kwun,Choi, Sung Min 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.5

Purpose : Rotavirus is the main cause of infantile diarrheal disease worldwide. The purpose of this study is to assess the difference between clinical manifestations and feeding or delivery methods in healthy full-term neonates and those with nosocomially-acquired rotaviral infection. Methods : The study was conducted on 348 babies who were delivered in Dongguk University Kyongju Hospital from Jan 1 to June 30 in 2001. Stool specimens were collected from clinically symptomatic neonates. We studied the rate of positive stool rotazyme ELISA tests and positive symptoms. We compared the frequency of clinical manifestations according to the feeding methods [breast feeding(BF) or formula feeding(FF)] and the delivery methods[normal vaginal delivery(NVD) or Cesarean section(C/sec)]. Results : Rotavirus was detected in 152 of 348 babies. The rate of positive rotazyme ELISA test was 43.7% in healthy full-term symptomatic neonates. The most common symptom of rotaviral enteritis was mild fever; the others were watery diarrhea, abdominal distension, vomiting, delayed weight gain, irritability, poor oral intake and dehydration. Compared with FF neonates, BF neonates have lower frequency of symptoms, especially watery diarrhea, delayed weight gain and poor oral intake. The symptomatic frequency was higher in FF and NVD neonates than BF and C/sec. Conclusion : The symptoms of rotaviral enteritis were less frequent in BF or C/sec delivered neonates. BF appeared to alleviate the rotaviral enteritis but further studies are needed. The cause of the lower frequency of symptoms in C/sec delivered neonates was unknown.

유전자 검사를 통해 진단한 선천성 신성 요붕증 1례

김지현,이선주,김애숙,조성민,이동석,김두권,최성민,기창석,김종원,Kim Ji Hyun,Lee Sun Ju,Kim Ae Suk,Cho Sung Min,Lee Dong Seok,Kim Doo Kwun,Choi Sung Min,Ki Chang Seok,Kim Jong Won 대한소아신장학회 2005 Childhood kidney diseases Vol.9 No.2

Nephrogenic diabetes insipidus(NBI) is characterized by an inability to concentrate urine despite normal or elevated plasma concentrations of the antidiuretic hormone; arginine vasopressin(AVP). Polyuria with hyposthenuria, and polydipsia are the cardinal clinical manifestations of the disease. Ninety percent of congenital nephrogenic diabetes insipidus patients are males with the X-linked recessive form of the disease; the mutation is in the AVP receptor 2 gene(AVPR2), which is located in chromosomal region Xq28. We report a case of NDI who suffered from unexplained fever and failure to thrive, which has been recognized since about ,3 months after birth. His genomic DNA analysis identified a novel AVPR2 gene mutation as W200C. (J Korean Soc Pediatr Nephrol 2005;9:269-274)

소아의 결장에서 발생한 원발성 선암종 1예

정재열,서유경,김애숙,이선주,조성민,이동석,김두권,최성민,김동훈,Jung, Jae-Youl,Seo, Yu-Koyng,Kim, Ae-Suk,Lee, Sun-Ju,Cho, Sung-Min,Lee, Dong-Seok,Kim, Doo-Kwun,Choi, Sung-Min,Kim, Dong-Hoon 대한소아소화기영양학회 2007 Pediatric gastroenterology, hepatology & nutrition Vol.10 No.1

Colorectal carcinomas occur primarily in elderly people and are rare in children. Unlike adult colorectal carcinomas, the overall prognosis is very poor because of the usual delay in diagnosis and advanced stages at presentation or initial diagnosis, and a high incidence of aggressive tumor pathology such as mucinous adenocarcinoma. Colon cancer should not be excluded in children only based on age or barium enema results. Therefore, colonoscopy should be performed in pediatric patients with unexplained rectal bleeding and abdominal pain. We report a rare case of a child with a mucinous adenocarcinoma of the sigmoid colon in a 12-year-old boy, who presented with an abdominal mass and abdominal pain and review the medical literature.

염색체 7번 장완의 부분적 역위 중복 1례

김현지,이소영,김미영,백청순,김원덕,이동석,김두권,최성민,Kim, Hyun Ji,Lee, So Young,Kim, Mi Young,Baek, Chung Sun,Kim, Won Duck,Lee, Dong Seok,Kim, Doo Kwun,Choi, Sung Min 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.5

Duplication of chromosome 7q has been reported as either partial or complete. Partial 7q duplication was first described by Carpentier in 1972. Pure partial duplication of the long arm of chromosome 7 is extremely rare and only 16 cases with a pure partial duplication of different 7q segment have been described in the literature. Pure partial duplication of the long arm of chromosome 7 is characterized by growth and developmental retardation, muscular hypotonia, distinct craniofacial dysmorphic features, a short neck and skeletal abnormalities. A 3 month-old male was referred to our department of Pediatrics because of dyspnea, hypotonia and delayed development. He shows growth and developmental delay, hypertelorism, a depressed nasal bridge, low set ears, a short neck and muscular hypotonia. Karyotype revealed 46, XY, dup(7)(q36q33) by GTC-banding. We report a case of a partial inverted duplication of chromosome 7q.

두 종합 병원을 방문한 영유아 양육자의 이유식에 대한 인지도 조사

위현우,서유경,김애숙,이선주,조성민,이동석,김두권,최성민,We, Hyun Woo,Seo, Yu Kyung,Kim, Ae Suk,Lee, Sun Ju,Cho, Sung Min,Lee, Dong Seok,Kim, Doo Kwun,Choi, Sung Min 대한소아소화기영양학회 2006 Pediatric gastroenterology, hepatology & nutrition Vol.9 No.2

Purpose: This study was carried out to investigate the awareness of the weaning of food using questionnaires, and the relationship with the weight gain in young infants. Methods: From September 2005 to December 2005, we performed a survey on 141 guardians of young infants aged from 6 to 18 months, who visited the pediatric out-patient department at Dongguk University Medical Center. We calculated the total score for each responder from ten questions on the weaning of food and assessed the body weight percentile of each of the young infants. Results: The most commonly reported information source for weaning food was 'the friends around' by 62 respondents (44.0%); 54 (38.3%) responded that the definition of weaning food was the preparatory step before starting a solid diet. Most used a spoon (90.8%) to feed when weaning food with a thin gruel of rice (78.7%). The time for weaning of food was before breast or infant formula feeding (55.3%). Addition of cow's milk was around 12 months (77.3%). The mean score was 6.21; however this did not show a statistically significant correlation with weight gain in young infants. Conclusion: The overall awareness of weaning of food has improved; however, information from doctors has decreased. Although the relationship between the awareness of weaning of food and the growth of young infants was not statistically significant, further studies on weaning of food, with larger and controlled sample sizes may provide important information.

Klippel-Trenaunay 증후군과 Sturge-Weber 증후군이 동반된 1례

김미영,이소영,김나연,이선주,김원덕,조성민,이동석,김두권,최성민,Kim, Mi Young,Lee, So Young,Kim, Na Yeon,Lee, Sun Ju,Kim, Won Duck,Cho, Sung Min,Lee, Dong Seok,Kim, Doo Kwun,Choi, Sung Min 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.9

저자들은 26개월된 여아에서 양측 안면과 우측 상, 하지에 화염상모반증, 골조직 및 연부조직의 이상 비대 소견을 보이면서 우안의 녹내장 및 맥락막 혈관종, 연수막 혈관종, 전신성 경련, 발달지연을 동반한 Klippel-Trenaunay 증후군과 Sturge-Weber 증후군의 특성이 동시에 관찰된 드문 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

다기관 공동으로 개최한 제 1회 대구·경북 소아청소년 간질 캠프의 경험과 참가한 부모들의 간질에 대한 인식

조성민(Sung Min Cho),권순학(Soon Hak Kwon),김두권(Doo Kwun Kim),김준식(Jun Sik Kim),김진경(Jin Kyung Kim),문한구(Han Koo Moon),박우생(Woo Saeng Park),여지현(Ji Hyun Yeo),오현희(Hyun Hee Oh),이은주(Eun Ju Lee),이준화(Jun Hwa Lee),김보영( 대한소아신경학회 2006 대한소아신경학회지 Vol.14 No.2

목 적: 본 연구는 다기관 공동으로 개최한 제 1회 대구·경북 소아청소년 간질 캠프의 경험을 알리고, 동시에 캠프에 참가한 부모들의 간질에 대한 인식을 연구하기 위하여 시행되었다. 방 법: 2006년 2월 25-26일 경북 경주시에서 캠프가 열렸다. 33가족이 참가하였고 진행요원까지 포함하여 총 160여명이 참가하였다. 캠프 끝에 부모들의 간질에 대한 이해와 캠프에 대한 만족도를 알아보기 위하여 37문항으로 구성된 설문을 하였다. 결 과 : 부모들은 간질에 대하여 어느 정도 정보와 지식을 가지고 있었으나 좀더 많은 정보를 얻기 원하였다. 부모들은 간질 캠프 프로그램 중 교육 프로그램에 대하여 대체적으로 만족하였으나 레크리에이션 프로그램에 대해서는 만족하지 않는 경우가 많았다. 결 론 : 간질 환자와 가족들에게 간질에 대한 더 많은 정보와 지식을 제공하려면 간질 캠프와 같은 사회적 접근이 더욱 필요하다. Purpose : This study was undertaken to announce our experience of the 1st Daegu-Gyeongbuk Multicenter Epilepsy Camp for children and their parents to improve the understanding of epilepsy. Methods : We held the camp on February 25th to 26th, 2006 at Gyeongju. 33 families with epileptic childlend joined the camp. At the end of the camp, we performed a survey with 37 questionnaires to obtain data concerning the parents' understanding of epilepsy and the satisfaction with the camp. Data was analyzed with SPSS 10.0 program using frequency analysis. Results : Parents knew about epilepsy but for some degrel still wanted to get more information about epilepsy. Parents were satisfied with the educational programs but not with the recreation programs. Concl usi on : This study suggests that more social approaches such as an epilepsy camp is needed to provide the families with more information fot the understanding about epilepsy.