• Title/Summary/Keyword: Rare

Search Result 11,800, Processing Time 0.051 seconds

Prenatal diagnosis and clinical course of restrictive foramen ovale in otherwise normal heart (단순 조기 난원공 협착의 산전 진단과 임상경과)

  • Lee, Ji Joung;Lee, Min A;Rhee, Yun ee;Chang, Mea Young;Kil, Hong Ryang
    • Clinical and Experimental Pediatrics
    • /
    • v.50 no.3
    • /
    • pp.268-271
    • /
    • 2007
  • Purpose : Premature narrowing of the foramen ovale is rare but serious clinical entity. Prenatal narrowing or obstruction of the foramen ovale shows symptoms such as right heart failure, fetal hydrops, triscupid regurgitation, left heart obstructive disease, and supraventricular tachycardia. This study aimed to assess the prenatal diagnosis and postnatal clinical course of restrictive foramen ovale in utero in otherwise normal heart. Methods : The subjects were five patients diagnosed with restrictive foramen ovale in utero from January 2001 to June 2005 at Chungnam National University Hospital. The diagnostic criteria was defined when the maximum diameter in a 4-chamber view is less than 2.5 mm and there is a continuous doppler velocity at the foramen ovale of more than 0.6m/s. Results : At the time of diagnosis of restrictive foramen ovale, gestation age was 34~37 wks, and chief complaints were fetal arrhythmia(2 cases), pericardial effusion, Ebstein anomaly and subaortic stenosis. Two cases which were diagnosed fetal hydrops and supraventricular tachycardia delivered by emergent cesarian section. Five cases were found to have right heart dilatation on echocardiogram after birth, but right heart dilatation became normalized at day 7 after birth and the clinical courses were not eventful. Conclusion : Identifying an obstructed foramen ovale in the fetus warrants the further search for additional cardiac and extracardiac anomalies, which may alter the prognosis. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.

Analysis of disease mechanism of subacute necrotizing lymphadenitis in children (소아 아급성 괴사성 림프절염의 임상적, 방사선학적, 면역조직화학적 소견)

  • Kim, Hyun Jung;Yeom, Jung Suk;Park, Ji Suk;Park, Eun Sil;Seo, Ji Hyun;Lim, Jae Young;Park, Chan Hoo;Woo, Hyang Ok;Cho, Jae Min;Lee, Jeong Hee;Youn, Hee Shang
    • Clinical and Experimental Pediatrics
    • /
    • v.51 no.11
    • /
    • pp.1198-1204
    • /
    • 2008
  • Purpose : The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. Methods : We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. Results : The patient's age ranged from 5 to 19 years (average age :11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. Conclusion : We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis.

Renal manifestations in tuberous sclerosis complex (결절성 경화증 환자에서의 신장 발현)

  • Jeong, Il Cheon;Kim, Ji Tae;Hwang, You Sik;Kim, Jung A;Lee, Jae Seung
    • Clinical and Experimental Pediatrics
    • /
    • v.50 no.2
    • /
    • pp.178-181
    • /
    • 2007
  • Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

Change of Flora of Damaged Land in Juwangsan National Park for Five Years (2010~2014) (주왕산국립공원 내 훼손지의 5년간(2010~2014년) 식물상 변화)

  • You, Juhan;Kim, Mijin
    • Journal of Environmental Impact Assessment
    • /
    • v.25 no.4
    • /
    • pp.233-247
    • /
    • 2016
  • The purpose of this study is to offer the basic data for management of National Park by surveying and analysing the change of flora of damaged lands in Juwangsan National Park, Korea. In cause of occurring the damaged lands, site-1 was the vegetation damage by stamping, the removing Gwangamsa of site-2, 3, 4 and the removing Naewon village of site-5, 6, 7. Whole sites are caused by the artificial disturbance and interference. The numbers of flora were summarized as 135 taxa including 52 families, 109 genera, 116 species, 2 subspecies and 17 varieties in whole sites. The status of flora by sites, site-6 is the largest number of 52 taxa, site-1 is the lowest of 23 taxa. The rare plant is 1 taxa, 4 taxa of endemic plants, 10 taxa of specific plants by floristic region, 9 taxa of naturalized plants and 1 taxa of invasive alien plant. In the results of analysis about the change of coverage ratio, for this shrub layer, site-7 was changed to the most $0%{\rightarrow}50%$, and the herb layer, site-5 was changed to the most $75%{\rightarrow}95%$. In case of the change of species numbers, most sites tended to increase in the sites introduced different species or generated by the growth. It is contemplated that is similar to the restoration with the surrounding natural vegetation that is in progress is a transition occurs with the passage of time. In the dominant species, the shrub layer is Fraxinus sieboldiana, Lespedeza bicolor, Rhus javanica, Lespedezamaximowiczii, Salix koreensis and Zelkova serrata, and Miscanthus sinensis var. purpurascens, Oplismenus undulatifolius, Artemisia princeps, Bothriochloa ischaemum and Erigeron annuus of herb layer. In the results of analysing Naturalized Index(NI) and Urbanized Index(UI), NI was increased or decreased this phenomenon with the lapse of time, UI was the most increase. In particular, the increase in UI is due to the transition process, the pioneering plant, naturalized plant was imported to continue.

The Types, Roles and Socio-semiotic Features of Visual Materials in Elementary Science Textbooks (초등 과학 교과서에 실린 시각 자료의 종류, 역할 그리고 사회-기호학적 특징 분석)

  • Kim, Hyoungjin;Shin, Myeong-Kyeong;Lee, Gyuho;Kwon, Gyeong-Pil
    • Journal of Science Education
    • /
    • v.38 no.3
    • /
    • pp.641-656
    • /
    • 2014
  • This study aimed at analyzing visual materials included in school science textbooks, specifically the textbooks for first semester courses of 3rd to 6th graders. The purpose was to provide directions for future textbooks by understanding the functions of the images in both pedagogical and social perspectives as well. The study was conducted by investigating the types, roles and socio-semiotic features of the images in science textbooks. The results were as follows. Firstly, the most used types of images in 2007 curriculum textbooks were photographs and drawn pictures. Uses of other visual aids than above were extremely rare. It was also found that as the educational level rises, the use of images for decorative functions drastically declined. The majority of the images were used in providing supplementary explanations or examples. This implies that the images effectively play the role of helping science education. In addition, more use of worksheets images was found, indicating that as educational level increases, students participate more actively in research sessions or data analysis. In socio-semiotic perspective, visual images showed high accessibility to students in 'Type of visual image', 'Function of visual image', 'Distance of shot', 'Horizontal angle of shot', 'Color moduation'. It was implied that there will a close correlation between the type, role and the socio-semiotic characteristics of visual images in textbooks. For example, photograph-type visuals were mostly used as supplementary references. And when applying the socio-semiotic analysis to photograph-type visuals, they showed 'real type', 'narrative-metaphor type', and 'shadow effect' among socio-semiotic features. Such correlations implied that knowing the type of the visual image may help determining the role of the image in the textbook to some extent, and also corresponding socio-semiotic characteristics. As a result, it was possible to infer how accessible certain visual images are to students. The above results have implications for the effective use of visual images in future textbooks.

  • PDF

Expression of Jun and p53 Genes from the Brain of Rats Irradiated with $^{60}Co{\gamma}$-ray (감마선 조사에 의한 뇌조직의 Jun 및 p53유전자 발현)

  • Kim Yong Seok;Woo Chong Kyu;Lee Yong Sung;Koh Jai Kyung;Chun Ha Chung;Lee Myung Za
    • Radiation Oncology Journal
    • /
    • v.14 no.4
    • /
    • pp.265-279
    • /
    • 1996
  • Damage produced by radiation elicits a complex response in mammalian cells, including growth rate changes and the induction of a variety of genes associated with growth control and apoptosis. At doses of 10,000 cGy or greater, the exposed individual was killed in a matter of minutes to a couple of days, with symptoms consistent with pathology of the central nervous system(CNS) including degenerative changes. The nature of the damage in irradiated cells underlies the unique hazards of ionizing radiation. Radiation injury to CNS is a rare event in clinical medicine, but it is catastrophic for the patient in whom it occurs. The incidence of cerebral necrosis has been reported as high as 16% for doses greater than 6,000 cGy. In this study, the effect of radiation on brain tissue was studied in vivo. Jun and p53 genes in the rat brain were induced by whole body irradiation of rat with 600Co in doses between 1 Gy and 100 Gy and analyzed for expression of jun and p53 genes at the postirradiation time up to 6 hours. Northern analyses were done using 1.8 Kb & 0.8 Kb-pGEM-2-JUN/Eco RI/Pst I fragments, 2.0 Kb-php53B/Bam HI fragment and ,1.1 Kb-pBluescript SK--ACTIN/Eco RI fragment as the digoxigenin or [${\alpha}^{32}P$] dCTPlabeled probes for Jun, p53 and ${\beta}$-actin genes, respectively. Jun gene seemed to be expressed near the threshold levels in 1 hour after irradiation of $^{60}$Co in dose less than 1 Gy and was expressed in maximum at 1 hour after irradiation of $^{60}$Co in dose of 30 Gy. Jun was expressed increasingly with time until 5 or 6 hours after irradiation of $^{60}$Co in doses of 1 Gy and 10 Gy. After irradiation of $^{60}$Co in dose between 20 Gr and 100 Gy, the expression of Jun was however increased to peak in 2 hours and decreased thereafter. p53 gene in this study also seemed to be expressed near the threshold levels in 1 hour after irradiation of $^{60}$Co in dose less than 1 Gy and was expressed in maximum at 6 hours after irradiation of $^{60}$Co in dose of 1 Gy, p53 was expressed increasingly with time until 5 or 6 hours after irradiation of $^{60}$Co in dose between 1 Gy and 40 Gy. After irradiation of $^{60}$Co in doses of 50 Gy and 100 Gy, the expression of p53 was however increased to peak in 2 hours and decreased thereafter. The expression of Jun and p53 genes was not correlative in the brain tissue from rats. It seemed to be very important for the establishment of the optimum conditions for the animal studies relevant to the responses of genes inducible on DNA damage to ionizing radiation in mammalian cells. But there are many limitations to the animal studies such as the ununiform patterns of gene expression from the tissue because of its complex compositions. It is necessary to overcome the limitations for development of in situ Northern analysis.

  • PDF

Hemolytic-uremic Syndyome Associated with Bloody Diarrhea (혈변을 동반한 용혈성 요독 증후군 5례)

  • Kim Jung-Sim;Park Eun-Jung;Chung So-Hee;Ko See-Hwan;Uhm Mee-Ryung;Park Moon-Su;Lee Heung-Jea;Jin Dong-Kyu
    • Childhood Kidney Diseases
    • /
    • v.1 no.2
    • /
    • pp.170-175
    • /
    • 1997
  • Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

  • PDF

A Study on the Evaluation of Patient Dose in Interventional Radiology (중재적방사선검사에서 환자 피폭선량에 관한 연구)

  • Park, Hyung-Sin;Lim, Cheong-Hwan;Kang, Byung-Sam;You, In-Gyu;Jung, Hong-Ryang
    • Journal of radiological science and technology
    • /
    • v.35 no.4
    • /
    • pp.299-308
    • /
    • 2012
  • To perform patient dose surveys in major interventional radiography procedures as a mean of inter-institutional comparison and of establishing reference dose levels with the ultimate goal of optimizing patient doses in the field of interventional radiography. We reviewed international patient dose survey data in the literature and measured patient dose in major interventional radiography procedures (TACE, AVF, PTBD, TFCA, GDC embolization). ESD(Entrance Skin Dose) was measured using TLD chips attached to the patient skin and ED(Effective Dose) was calculated using angiography unit-derived DAP. A survey of patient dose in interventional radiography procedures were also performed with a questionnaire for interventional radiologists and we proposed a guideline for optimizing patient doses in the field of interventional radiology. The patient dose survey data in interventional radiography procedures were very rare in literature compared with those in diagnostic radiography procedures. In TACE, the mean ED was 25.43 mSv and the mean ESD was 511.75 mGy. The mean ED of TACE was not high, but the cumulative dose should be checked, due to longer procedure TACE. In TFCA, the mean ED was 22.6 mSv and it was relatively high compared with data of other countries. In GDC embolization, the mean ED was not available, because GDC embolization was performed with old Image-Intensifier-type unit and there has no unit-installed ionization chamber. Also, the mean ESD of GDC embolization was up to 2,264 mGy and further studies are needed to calculate the net ED of GDC embolization. Patient dose occurred during interventional radiography procedures are high related with the difficulty of the procedure, fluoroscopy time, the number of angiographies and the treatment protocol. Therefore, continuous education and efforts should be made to optimize the patient dose in the field of interventional radiology.

Clinical Investigation about the Result of Surgically Treated Myasthenia Gravis (중증 근무력증의 수술적 치료결과에 대한 임상적 고찰)

  • 김대현;황은구;조규석;김범식;박주철
    • Journal of Chest Surgery
    • /
    • v.36 no.1
    • /
    • pp.15-20
    • /
    • 2003
  • Myasthenia gravis is a rare autoimmune disease involving acetylcholine receptor and its autoantibody on neuromuscular junction. The methods of treatment are medical treatment and surgical thymectomy. In this paper we analyzed the result of thymectomy and the factors affecting the postoperative symptom improvement. Material and method : This study obtained medical records of 37 patients who received the thymectomy for myasthenia gravis from March 1986 to December 1998. Result Out of 37 cases, 21 cases(57%) showed improvement, of which 8 cases (50%) in the group of thymoma(n=16), and 13 cases (62%) in the group of thymic hyperplasia(n=21) showed the improvement of symptoms. Postoperative complications were respiratory insufficiency due to aggravation of symptoms after operation, including tracheal intubation for ventilator support in 9 cases, pneumonia in 3 cases, pneumothorax in 2 cases and left vocal cord palsy in 1 case. There was one postoperative mortality. The relation between postoperative improvement and sex(P=0.3222), age(P=0.7642), thymic pathologic variants,(P=0.4335) and classification of thymoma(P=0.20) showed no statistically significant correlation. However, the lower grade of preoperative symptoms can predict the lower grade of postoperative symptoms significantly(P=0.0032). Follow up study to 36 postoperative survivors was performed in October 2002 based on the out-patient records and call with patients. Out of 36 cases, 33 cases(91.7%) could be investigated and 3 cases could not. Mean follow up period was 83.2 months. Out of 33 cases, 25 cases(75.8%) showed symptomatic improvement, of which 8 cases(53.3%) in the group of thymoma(n=15) and 17 cases(94.4%) in the group of thymic hyperplasia(n=18) showed the improvement of$\boxUl$ symptoms. Conclusion : In myasthenia gravis, thymectomy showed the good improvement, and more important factor affecting the improvement of symptoms was the grdae of preoperative symptoms. Also midterm and long term follow up results showed good symptomatic improvement.

Pulmonary Complications after Surgery for Esophageal Cancer (식도암 수술 후 발생한 호흡기 합병증)

  • Lee, Jang-Hoon;Lee, Jung-Cheul
    • Journal of Chest Surgery
    • /
    • v.39 no.2 s.259
    • /
    • pp.134-139
    • /
    • 2006
  • Background: Complications after surgery for esophageal cancer are various and not rare. Among them, pulmonary complication is well known as one of the most important insults which has negative influence on the postoperative course and results in mortality. So we attempted to analyze the factors which may have relation to postoperative pulmonary complication. Material and Method: The retrospective study was undertaken in 87 patients who underwent curative surgery for esophageal cancer from Jan. 1996 to Aug. 2005. We divided them into two groups, patients with pulmonary complication (group A, n=28), without pulmonary complication (group B, n=59). Statistical analysis was performed with Fisher's exact test. Result: The postoperative pulmonary complication developed in 28 patients ($32\%$). There was no difference between two groups in past medical history, preoperative pulmonary function, surgery time, anastomosis method, pathologic stage, and trial of neoadjuvant therapy. Age and incidence of cervical anastomosis were significantly higher in group A (p=0.001, p=0.023). The rate of routine postoperative ventilator care was significantly higher in group S (p=0.007). Chest tube indwelling time and hospital stay were significantly longer in group A (p=0.011, p=0.001). There were 6 postoperative deaths ($6.8\%$) and 5 deaths were related to pulmonary complication. Pneumonia was the most common cause of death and MRSA (methicillin resistant staphylococcus aureus) was the most common organism in sputum culture. Conclusion: Pulmonary complication after esophageal cancer surgery was the most important cause of death. Pulmonary complication was closely related to patient's age and cervical anastomosis. We think postoperative routine ventilator care is helpful for prevention of pulmonary complications, especially MRSA pneumonia, and reducing mortality.