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Congenital central hypoventilation syndrome combined with Hirschsprung disease diagnosed in the neonatal period (신생아에서 진단된 Hirschsprung 병을 동반한 congenital central hypoventilation syndrome 1례)

  • Choi, Jin Hyun;Oh, Jin Hee;Kim, Jong-Hyun;Koh, Dae Kyun;Hong, Seung-Chul
    • Clinical and Experimental Pediatrics
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    • v.49 no.4
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    • pp.446-450
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    • 2006
  • Congenital central hypoventilation syndrome (CCHS) or Ondine's curse is a very rare sleep disorder that is the result of a congenital failure of the autonomic control of ventilation caused by insensitivity of the chemoreceptor to hypercapnea during sleep. Gastrointestinal motility disorders, particularly a congenital megacolon (Hirschsprung disease) is often combined with CCHS. This combination can be explained by a defect in the migration of neuronal cells from the neural crest (neurocristopathy) during the intrauterine period. A diagnosis of CCHS is made by confirming the failure of adequate ventilation in response to hypercapnea and hypoxia during sleep and the exclusion of other diseases. Young infants frequently show atypical clinical courses, and their conditions are frequently complicated with the long-term sequela of hypoxemic episodes. Therefore, a high index of suspicion and active treatment with mechanical ventilation are important for reducing recurrent hypoxemic episodes in the neonatal period. This paper reports the follow up of a case of CCHS in a neonate who showed frequent intractable apnea and cyanosis and was given artificial mechanical ventilation during sleep.

Co-Infection of the Rat Central Nervous System with Genetically Engineered Strains of Pseudorabies Virus (유전자 조작된 Pseudorabies 바이러스에 의한 흰쥐 중추신경계의 이중감염)

  • Kim Jin-Sang;Kwon Young-Shil
    • The Journal of Korean Physical Therapy
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    • v.11 no.2
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    • pp.81-92
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    • 1999
  • 중추신경계의 미주신경동쪽핵(DMV)내 유사핵분열후 신경세포로 외래 유전자를 전달하는 매개체로서 pseudorabies 바이러스(PRV)의 유전자 조작기술은 흰쥐의 결장내로 PRV를 주입시킨 후 복잡한 신경로 추적에 관한 연구에서 하나의 바이러스에 의해 얻어지는 것보다 더욱 유용한 결과산출이 가능하게 하였다. 본 연구에서는 흰쥐의 생체내 실험모델로 하나의 바이러스 또는 이중 바이러스 주입에 PRV의 유전자 조작된 2종 바이러스를 사용하였다. 이 2종의 바이러스는 PRV의 Bartha 종에서 유래되었지만 면역조직화학적으로 검출할 수 있는 동일한 유전산물을 산출할 수 있도록 구성되었다. PRV-BaBlu는 PRV 게놈의 Us 구역 중 gC 자리에 lacZ 유전자를 삽입하여 산출되었는데 $\beta-galactosidase$ 발현은 이 바이러스에 감염된 신경원의 독특한 표시자로 나타났다. PRV-D는 2가지 단계에 의해 조성되었는데 첫째, PRV-Bartha의 Us 구역의 일부 유전자를 제거하고, 야생형인 PRV-Be DNA로 복구시켰는데 이로써 PRV-D는 PRV-Bartha 또는 PRV-Bablu에 존재하고 있지 않는 외피 당단백질인 gE와 gI를 지니게 되었다. 본 연구의 결과는 다음과 같았다. 첫째, PRV-D의 개별적 접종에 의해 얻어진 감염은 PRV-BaBlu에 의한 동일 신경회로의 감염보다 유의하게 빨랐다. 둘째, 유전자 조작된 PRV의 변이종은 변이종 상호간 및 부모 바이러스와 상이하였다. PRV-D는 PRV-Bartha 또는 PRV-BaBlu보다 감염독성이 더 강했고, PRV-BaBlu는 PRV-Bartha보다 감염독성이 약했다. 셋째, 결장을 지배하는 미주신경동쪽핵내 신경원은 변이종 바이러스들을 동시에 접종하였을 경우 이중감염을 나타내었다.

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Intestinal Neuronal Dysplasia in Twins (쌍생아에서 발생한 Intestinal Neuronal Dysplasia)

  • Lim, In-Suk;Chung, Ju-Young;Choi, Myung-Jai;Kim, Sang-Woo;Kim, Hong-Ju;Kim, Jeong-Yeon
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.6 no.2
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    • pp.202-207
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    • 2003
  • Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

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Paragonimiasis with Simultaneous Multifocal Lesions (동시 다발성 폐흡충증의 임상적 치험)

  • Shin, Dong-Il;Chang, Woon-Ha;Kim, Jung-Tae;Sohn, Jin-Hee;Kim, Kyoung-Min;Oh, Tae-Yoon
    • Journal of Chest Surgery
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    • v.40 no.5 s.274
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    • pp.380-383
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    • 2007
  • A 42-years-old man was referred to our department due to his hemoptysis. Chest CT showed a cavitary lung lesion in the apical segment of the RUL and an anterior mediastinal mass. The patient underwent wedge resection for the cavitary lesion and complete resection for the mediastinal mass. The pathologic finding was Paragonimus Westermani infestation in both the lung and thymus. The abdomen CT taken postoperatively showed an inflammatory mass involving the transverse colon and a small nodular lesion around the descending colon, which strongly suggested paragonimiasis. Postoperatively, the patient took Praziquantel for 2 days and he was discharged without any complications. There was no evidence of recurrence for the last 2 years.

A Study on the Social Support System of Colostomy Patients and Their Quality of Life (결장루보유자의 사회적지지정도와 생활 적응정도에 관한 조사연구 -한국 ostomy 협회 회원 중심으로-)

  • 김채숙;강규숙
    • Journal of Korean Academy of Nursing
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    • v.16 no.3
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    • pp.22-30
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    • 1986
  • The objective of the study was to determine the degree of social support and the quality of life of the 200 colostomy patients who are members of the Korea Ostomy Association. Data was collected by means of a questionnaire which was distributed Foully six Percent of the 200 colostomy patients returned the questionnaire during the period from Mar. 3-Mar. 31. 1986. The results of the study were as follows: 1. The score of the social support was 93.4598 (S.D-20.1787) and the highest score for respect Iron other people was 102.00. 2. The mean score of the quality of life was 3.11 (S.D. 0.58) The highest score for Pull attention received from the family was 3. 85 and the lowest score for insufficient sexual activity was 2.34. 3. The correlation between social support and the quality of life was (r=.1787, p<.05) when the quality of life score was high the social support score was also high. 4. The variables that were found to have a significant correlation to the social support were a9e (r=.2039, p<.05) and number of people who could help them (r=.2847 p<.01) 5. The variables that were found to have a significant correlation to the quality of life were the level of the living standard (F=4.534, p< .05) and the individuals perception of their health. status(F=18.943, p<.001) =Recommendations= The results of this study show that there is a significant correlation between the social support of the individual and the quality of life. It is important for nurses to support self-help groups of colostomy patients as this will contribute highly to the betterment of the quality of the life of patients who have colostomies.

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A Case of Mucinous Adenocarcinoma of the Sigmoid Colon in a Child (소아의 결장에서 발생한 원발성 선암종 1예)

  • 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
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.10 no.1
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    • pp.81-85
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    • 2007
  • 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.

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A case of extragastrointestinal anisakiasis involving a mesocolic Iymph node (횡행결장간막의 림프절을 침범한 고래회충유충증의 장외감염 1례)

  • Kim, Hui-Jeong;Park, Chan-Il;Jo, Seung-Yeol
    • Parasites, Hosts and Diseases
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    • v.35 no.1
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    • pp.63-66
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    • 1997
  • In a 43-year-old Korean man who underwent radical gastrectomy due to a malignant stromal tumor, was found to have an enlarged Iymph node at transverse mesocolon. The Iymph node exhibited histologically necrotizing eosinophilic granuloma formed around a track containing sections of a nematode larva. The well preserved nematode sections revealed polymyarian muscle cells, Y-shaped lateral cord, a large excretory gland cell, intestine and eosinophilic cuticle. The nematode sections were identified as a larva of Anisnkis species In Korea, thIns is the first case of elrtragas trio into stinal anisakiasis.

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Effect of filtrates of Serpulina(Treponema) hyodysenteriae on the epithelium in colonic loops in swine (Serpulina(Treponema)hyodysenteriae 배양여과액의 돼지 공장 계제 점막에 미치는 영향)

  • Kang, Boo-hyon;Olander, Harvey J
    • Korean Journal of Veterinary Research
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    • v.33 no.3
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    • pp.429-442
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    • 1993
  • The cytotoxic effects of S hyodysenteriae strain B 204 on the mucosal surface were studied in surgically prepared ligated colonic loops in two male convenitonal mixed-breed pigs. In each one of four loops was inoculated with either sterile trypticase soy broth(TSB) of Serpulina, filtrate of Serpulina TSB culture. washed Serpulina cells or whole culture of Serpulina. Mucosal tissues were examined by transmission and scanning electron microscopy 24 and 48 hours after inoculation(p.i.). The filtrate did not induce any significant effect on the mucosal surface. The washed cells produced early lesions similar to those caused by the whole culture. These observations suggest that cytotoxins of the culture do not play a significant role in invasion of the epithelium in this experimental infection. The possible role of toxins associated with the organism at the site of interaction with the epithelial cells was not elucidated.

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The Relationship Between Hardiness and Self-Care Agency of Persons with a Colostomy (결장루보유자의 강인성과 자가간호역량과의 관계)

  • Bae, Eun-Sook
    • The Korean Journal of Rehabilitation Nursing
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    • v.3 no.1
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    • pp.15-26
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    • 2000
  • This study examined the relationship between the level of hardiness and self-care agency of persons with a colostomy. The purpose of the study was to explore the effect of hardiness on self-care agency of persons with a colostomy and to identity factors influencing on. The ultimate goal was to help nurses improve the person's self-care agency and to contribute theoretical base for the planning of supportive nursing interventions to increase the level of self care. The subjects for the study consisted of 43 persons aged above eighteen below seventy had had Miles operation and permanent colostomy. The data were collected from July, 1998 to September 15, 1998. The instruments used for this study were the Health-Related Hardiness (Pollock, 1984) and the Self-carer As Inventory Scale(Genden & Taylor, 1988). The Self-Carer As Inventory translated by So Hyang-sook and was modified by author of this study. The data were analysed by using SAS computerlized program and included number, percentage, t-test, ANOVA and Pearson Correlation Coefficient. The results of the study are summarized as follows. 1. Hardiness was significantly related to Self-care agency(r=-0.531. p=0.0002). That is, the higher the hardiness, the higher the level of Self-care agency. 2. In the relationship between general characteristics and traits of persons with a colostomy and hardiness were significant difference in monthly income (F=2.79, p=0.03), problem in stoma management (F=3.29, p=0.01). 3. In the relationship between general characteristics and traits of persons with a colostomy and Self-care agency were significant difference in monthly income (F=2.93, p=0.03), problem in stoma management(F=2.93, p=0.01). Therefore, in order to increase the level of Self-care agency of ostomate Nurses should be helpful to provide them with hardiness training to strengthen their control, commitment and challenge(subconcepts of hardiness).

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Hypospadias and Megacolon in a Persian Cat (페르시안 고양이에서 발생한 요도밑 열림증과 거대결증증)

  • Kim, Sung-Eon;Choi, Ran;Park, Jasil;Yang, Hye-Mi;Hyun, Changbaig
    • Journal of Veterinary Clinics
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    • v.31 no.5
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    • pp.454-456
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    • 2014
  • A 9-month-old intact male Persian cat (3.2 kg of body weight) was referred with primary complaint of constipation. Diagnostic studies found severe constipation and megacolon. After immediate medical treatment (e.g. enema), the physical examination was performed and revealed that the unusual urethral opening was found approximately 0.5 cm ventral to the tip of the penis where a 3.5 fr tomcat catheter was easily advanced into the bladder. The case was diagnosed as hypospadias. The cat was treated with castration and medical therapy for constipation. To our best knowledge, this case is the first case report for feline hypospadias complicated with chronic constipation in Korea.