• Title/Summary/Keyword: 선천적

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Treatment of Systemic Arterial Supply to Lower Lobe of Left Lung (Operation vs. Embolotherapy): Comparison of Two Cases and Literature Review (좌측 하폐엽에 공급하는 비정상적인 체동맥의 치료 (수술과 색전술의 비교))

  • Jeon Eui-Yong;Rhee Gwang-Woo;Goo Dong-Erk;Kim Eung-Soo
    • Journal of Chest Surgery
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    • v.39 no.3 s.260
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    • pp.230-235
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    • 2006
  • Systemic arterialization of lung with/without sequestration (Sequestration/Anomalous Origin of Left Pulmonary Artery, AOLPA) is a rare form of congenital anomalous systemic arterial supply to the lungs. In this anomaly, the arterial supply of one or more arteries of the basal segments of the lower lobe derives from an aberrant vessel arising from the aorta. We report two adult cases of systemic arterialization of normal basal segments of left lower lobe lung with/without sequestration. The one (AOLPA) was treated by left lower basal segmentectomy and the other (Sequestration) by therapeutic angiographic embolization. Based on the favorable follow-up result in our patients, although lobectomy (segmentectomy) is the basic treatment modality, embolotherapy could also be a mode of treatment that could be selectively applied to elderly, infirm patients or high risk patients with poor pulmonary function.

Surgical Management of Critical Pulmonary Stenosis -A case report- (중증 폐동맥협착증의 외과적 치료 -1례 보고-)

  • Jung, Tae-Yeol;Ban, Dong-Gyu;Kim, Hyuck;Kim, Young-Hak;Chung, Won-Sang;Kang, Jeong-Ho;Jee, Heng-Ok;Lee, Chul-Bum;Kim, Nam-Su;Seoh, Jung-Kuk
    • Journal of Chest Surgery
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    • v.33 no.12
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    • pp.963-967
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    • 2000
  • 정상심실중격의 폐동맥협착은 흔한 선천성 심장질환이지만 신생아기에 심한 증세를 보이는 위기적 폐동맥 협착은 드물며 예후도 대단히 나쁘다. 경피적 풍선 판막성형술의 발달과 함께 폐동맥협착의 외과적 치료는 더욱 줄어드는 추세이다. 본 증례는 정상심실중격의 위기적 폐동맥협착증으로 진단받은 생후 2일된 남자 신생아로 심한 청색증과 저산소증을 보여 응급실로 내원하였다. 환아는 산소공급 및 Prostaglandin E$_1$을 투여 후 동맥혈 산소 분압이 19 mmHg에서 54mmHg로 증가하였다. 경피적 풍선 판막성형술을 시도하였으나 유도도관(Guide wire)이 판막의 개구부를 통과하지 못하였고 시술도중 심낭내로 조영제가 고이는 소견을 보여 우심실 천공이 의심되었으므로 응급으로 정상체온의 체외순환하에서 폐동맥 절개후 폐동맥 판막절개술을 시행하였다. 수술 후 중환자실에서의 수술경과는 양호하였고 현재 수술 6개월째 외래 추적관찰중이다.

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Immunostimulatory Effects of Purple Bamboo Salts Composed with Rubus coreanus in Raw264.7 Cells and Mouse Peritoneal Macrophages (복분자 자죽염의 마우스 대식세포주 및 복강 대식세포에 대한 면역증진 효과)

  • Park, Heejeon;Kim, Sokho;Jeong, Sohee;Park, Heeran;Kim, Jin-Hyung;Song, Jiyoung
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.46 no.3
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    • pp.306-313
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    • 2017
  • Purple bamboo salt (PuBS) is commonly used as a medicinal food in Korea and has beneficial potentials such as antioxidant and anti-inflammatory effects. Rubus coreanus is called Bokbunja, which is used as a traditional medicine for treating asthma, impotence, and allergic diseases in Korea. The aim of present study was to investigate the immunostimulatory effect of PuBS composed with Rubus coreanus (PuBS-R). We performed comparative analysis between PuBS and PuBS-R in Raw264.7 cells, which is a mouse macrophage cell line, and peritoneal macrophages isolated primarily from the mouse peritoneal cavity. We evaluated cytotoxicity and the immune cytokine response in PuBS- and PuBS-R-treated cells. Both PuBS and PuBS-R did not have any cytotoxicity in Raw264.7 cells up to $500{\mu}g/mL$. Gene and protein levels of immune cytokines such as tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$), $interferon-{\gamma}$ ($IFN-{\gamma}$), interleukin (IL)-10, and IL-12 were significantly elevated by PuBS-R more than PuBS in Raw264.7 cells. Moreover, we evaluated the immunostimulatory effects of PuBS-R on mouse primary peritoneal macrophages. Protein levels of inducible nitric oxide synthase, $TNF-{\alpha}$, $IFN-{\gamma}$, IL-10, and IL-12 were significantly higher in PuBS-R-treated peritoneal macrophages than PuBS-treated peritoneal macrophages. These results suggest the potential immunostimulatory effect of PuBS-R for immunity against harmful infection.

The Norwood Operation in Infants with Complex Congenital Heart Disease (복잡 선천성 심기형 환자에서의 Norwood 술식)

  • 박정준;김용진
    • Journal of Chest Surgery
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    • v.30 no.3
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    • pp.263-269
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    • 1997
  • From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

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Long-Term Treatment with Enalapril Depresses Endothelin and Neuropeptide Y-induced Vasoactive Action in Spontaneously Hypertensive Rats (선천성 고혈압흰쥐에서 Endothelin과 Neuropeptide Y에 의한 심혈관계 반응에 Enalapril 장기처치가 미치는 영향)

  • Kim, Kwon-Bae;Sohn, Uy-Dong;Kim, Choong-Young
    • The Korean Journal of Pharmacology
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    • v.28 no.1
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    • pp.49-60
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    • 1992
  • This study was designed to evaluate the responses of cardiovascular system to endothelin (ET) and neuropeptide Y (NPY) in 12 week-old SHR treated with or without enalapril (ENP) for 6 weeks. The diastolic blood pressure and heart rate were lower in ENP-treated SHR than in control. The pressor response to intravenous, but not intracerebroventricular, ET or NPY was attenuated by ENP treatment. The chronotropic action induced by electrical stimulation was attenuated by ENP or ET. The negative chronotropic action of ET was blocked by yohimbine. The increase in aortic tension induced by electrical field stimulation (EFS) was depressed in ENP-treated group as compared with non-treated group, and enhanced by ET, but not NPY, in the non-treated group. The ET-induced increase in tension was enhanced by removal of endothelium in the control group but not in ENP-treated group. The plasma concentration of norepinephrine and ET-induced increase in concentration of norepinephrine and epinephrine in plasma were decreased in ENP-treated group. These results suggest that preventive effect of enalapril on the development of hypertension may result from depressing vasoactive action of endothelin and neuropeptide Y, and sympathetic neurotransmission at peripheral nervous system.

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THE MEDIAL SIGMOID DEPRESSION: Its Anatomic and Radiographic Considerations (하악골과 방사선사진상에서의 하악절흔 내측 함요)

  • Kang Byung-Cheol
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.21 no.1
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    • pp.7-13
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    • 1991
  • An anatomic radiolucency called medial sigmoid depression can be observed on a number of panoramic and mandibular oblique lateral views in the ramus just below and anterior to the mandibular sigmoid notch. The radiolucency may be unilateral or bilateral. Seventy-eight mandibles of dry skulls were visually examined and radiographed by panoramic machine. The observable incidence, the location center, and the mean size of the medial sigmoid depression were studied. Additionally, the radiographic incidence of the depression was determined using 500 panoramic radiographs. Normal anatomic radiolucent areas in the jaws are encountered often in the radiographs taken for the usual dental practice purpose. Some of these radiolucencies can be misdiagnosed as pathologic entities. But in most cases, the size, location, and appearance of the radiolucent area, and the presence or absence of symptoms can lead one to the correct diagnosis before surgery is undertaken. And this can be secured if diagnosticians are aware of the common and varied appearances of the normal anatomic radiolucencies A depression in the ramus just below and slightly anterior to the most inferior aspect of mandibular sigmoid notch shows occasionally foramen-like or notch-like radiolucency on some panoramic and oblique lateral views. This radiolucency was named medial sigmoid depression by Langlais et a1. in 1983. Since there're no reports about this medial sigmoid depression after that, (The author retrieved the Mediline from 1983 to 1990: Cambridge scientific abstracts, (7200 Wisconsin Ave Bethesda MD 20814 U.S.A.) National Library of Medicine) the author studied the size and the location of the depression of the mandible specimens, and determined the relationship between the observable anatomic incidence and its reproducibility on the radiographs.

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Congenital Dropsy in Korean-native Calf (한우 송아지의 선천성 전신수종)

  • 강문일;박영석;한동운
    • Korean Journal of Animal Reproduction
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    • v.23 no.1
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    • pp.63-68
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    • 1999
  • A 4-year old Korean-native cow of third parturition delivered an deformed calf with congenital anasarca and hypoplasia of the extremity at Muan County in Chonnam Province. At parturition, the dam showed amniotic fluid approximately two times more than that at the normal and also occurred severe dystocia. The anomaly died before parturition was 82 cm long and weighed 25 kg. It was difficult to recognize the neck and face of the anomaly due to severe edema and the stretched tongue through mouth was found. The shape of the extremity was recognizable and the systemic hypoplasia of the body with marked edema was found. At necropsy, there was a large amount of serous fluid both in pleural and peritoneal cavity. Also the fluid was contained some of yellowish granular materials and diffusely was fulfilled in all subcutaneous tissue. There was a partial opening in diaphragm which was resulted in the protrusion of approximately half of liver toward peritoneal cavity. There were two kidneys (15${\times}$21 and 13${\times}$18 cm) on the left which were enlarged and relatively larger than the right one (13${\times}$9 cm). In addition, there were found slight to moderate hepatomegaly and splenomegaly with slight congestion, and diffuse edema of intestinal serosa. Lung (32${\times}$49 mm) with marked hypoplasia was smaller than the size of heart (56${\times}$45mm). Serological test to the dam showed relatively high antibody titer (>64) to Akabane virus which might be involved in the outbreak of the deformed Korean-native calf as one of many causative agents.

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Jitter and Shimmer of the Deaf Voice (농자 음성의 주파수 변동율 및 진폭 변동율)

  • Ok-ran Jeong
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.7 no.1
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    • pp.39-42
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    • 1996
  • The present study analyed jitter and shimmer of the deaf in 4 different voicing conditions. Thirty-two male subjects and 27 female subjects participated in the study on a voluntary basis. The age ranged from 6 to 18 for male and 8 to 21 for female subjects. The subjects were either congenitally or prelingually deaf The four different voicing conditions included /a/ prolongation, counting, reading, and conversation. The experiment utilized CSL Visi-Pitch Model 6095(Kay Elemetrics Corp.) to sample and analyze the data. Both jitter and shimmer means were higher than the threshold values(normative data) reported. In addition, this investigation performed two separate 2-factor ANOVAs in order to determine if jitter and shimmer change as a function of gender and voicing condition. The results showed the following. First of all there was the gender effect on shimmer but not on jitter, in that male subjects 'shimmer was higher than females'. secondly, there was the voicing condition effect both on jitter and shimmer. /a/ prolongation and reading produced lower jitter than counting and conversation. /a/ prolongation produced lower shimmer than the remaining conditions. Finally, no interaction between gender and voicing condition existed.

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A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung (14세 여아에서 발견된 선천성 낭종성 선종성 기형 1예)

  • Lee, Myung In;Sohn, So Hee;Lee, Dae Joon;Ha, Dong Yul;Jee, Young Koo;Lee, Kye Young;Kim, Keun Youl;Choi, Young Hi;Cho, Jeong Hi;Seo, Pil Weon;Kim, Sam Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.5
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    • pp.805-811
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    • 1996
  • Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

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DENTAL TREATMENT IN A PATIENT WITH CONGENITAL PANHYPOPITUITARISM UNDER GENERAL ANESTHESIA (선천성 범뇌하수체저하증(Congenital panhypopituitarism) 환자의 전신마취 하 치과치료)

  • Kim, Hyuntae;Song, Ji-Soo;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Shin, Teo Jeon
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.15 no.1
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    • pp.70-73
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    • 2019
  • Congenital panhypopituitarism is an uncommon condition, present from birth, characterized by the decreased secretion of most of the hormones produced by the pituitary. The purpose of this case report is to present a case about caries treatment of a 26-month-old female patient with congenital panhypopituitarism under general anesthesia. A 26-month-old girl with congenital panhypopituitarism visited Seoul National University Dental Hospital for caries treatment of anterior primary teeth. Because of the child's age and underlying systemic disease, dental treatment under general anesthesia was considered. Prior to the dental procedure, 30mg of cortisol was administered intravenously in order to prevent possible adrenal crisis by stressful events. The dental procedure was successfully performed under general anesthesia. This case report suggests that general anesthesia may be useful for the dental treatment with congenital panhypopituitarism. Hormone deficiency should be assessed prior to dental procedure and, if necessary, stress hormone replacement therapy should be considered.