• Title/Summary/Keyword: 증후군

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Primary Repair of Boerhaave's Syndrome (Boerhaave 증훈군에 대한 일차 봉합술)

  • 김재현;김삼현;박성식;임수빈;서필원
    • Journal of Chest Surgery
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    • v.34 no.11
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    • pp.879-882
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    • 2001
  • advancement in the treatment of esophageal perforation due to the development of ICU care and antibiotics. There were controversies in the treatment of esophageal perforation when diagnosed after 24hrs. From 1995 to 2000, we performed a buttressed primary repair and mediastinal drainage in 6 Boerhaave\`s syndrome patients among 13 esophageal perforation patients. Two patients died(33%). They died because of pneumonia, ARDS and sepsis on 38th, 39th post-operative day respectively. Two patients had leak at the site of repair which was treated completely with conservative treatment. We report on the result of a buttressed primary repair and mediastinal drainage for 6 Boerhavve\`s syndrome patients.

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Three Cases of Obesity-Hypoventilation Syndrome (Pickwickian Syndrome) (비만성 저환기 증후군 (Pickwickian 증후군) 3예)

  • Jung, Ji-Hyun;Lee, Sang-Haak;Choi, Young-Mee;Kwon, Soon-Seog;Kim, Young-Kyoon;Kim, Kwan-Hyoung;Moon, Hwa-Sik;Song, Jeong-Sup;Park, Sung-Hak
    • Tuberculosis and Respiratory Diseases
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    • v.53 no.5
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    • pp.561-568
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    • 2002
  • Severe obesity can produce a marked impairment of respiratory function. The obesity hypoventilation or Pickwickian syndrome comprises of extreme obesity, alveolar hypoventilation, somnolence, plethora, pulmonary hypertension and right heart failure. It is sometimes associated with obstructive sleep apnea but can be distinguished from obstructive sleep apnea by the presence of awake $CO_2$ retention. Alt hough uncommon, it is important to recognize this syndrome because due to its potential life threatening nature and because can be reversed by appropriate treatment. Here, we report 3 cases of obesity hypoventilation syndrome.

A Case of Multiple Basal Cell Carcinomas in Nevoid Basal Cell Carcinoma Syndrome

  • Ji, So Young;Lee, Seong Pyo;Suhk, Jeong Hoon;Yang, Wan Suk
    • Archives of Craniofacial Surgery
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    • v.11 no.1
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    • pp.23-27
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    • 2010
  • 목적: 모반양 기저세포암 증후군 (Nevoid basal cell carcinoma syndrome) 또는 골린-골츠 증후군은 한국에서는 흔하지 않은 증후군으로 주로 상염색체 우성으로 유전하고 다기관 장애가 나타날 수 있으며 높은 표현율과 다양한 표현도를 특징으로 한다. 모반양 기저세포암 증후군의 진단 기준에는 다발성 기저세포암, 이소성 석회화(ectopic calcification), 손 또는 발바닥 오목 (palma or plantar pits), 치성 각화 낭종(odontogenic keratocysts), 가족력 및 골격계, 신경계, 안, 비뇨생식계 및 심장혈관의 이상 등이 있다. 본원에서 주로 두부의 다발성 기저세포암을 가진 모반양 기저세포암 증후군 환자를 경험하여 보고하고자 한다. 방법: 환자는 2007년 4월 두부의 색소성 모반으로 피부과에서 시행한 펀치 생검에서 기저세포암을 진단받고 의뢰되었으며, 이후 2009년 7월까지 14회의 추가적인 절제 및 조직 검사를 시행하였다. 환자는 갑상샘 유두암종의 재발로 인해 갑상샘 절제술을 2회 시행한 과거력이 있었으며 이학적 검사와 일반 혈액, 소변, 간 기능 및 갑상선 기능 검사를 시행하였고, 흉부와 늑골 방사선 검사, 심전도와 안면부 및 두부 컴퓨터단층촬영과 유전자 검사를 시행하였다. 결과: 두부와 안면부에서 절제한 27개의 병변 중 23개(85%)가 기저세포암으로 진단되었으며, 치성 각화 낭종과 대뇌겸 석회화, 이학적 검사에서 손바닥 오목이 발견되었다. 하복부 초음파에서 난소 낭종이 발견되었으나 조직 검사는 시행되지 않았다. 결론: 한국에서 모반양 기저세포암 증후군에 대한 연구는 주로 치과와 피부과 영역에서 국한되었으며, 특히 치과 영역에서의 보고는 치성 각화 낭종 및 손바닥 오목에 초점을 둔 것이 대부분이었다. 이에 본원에서는 주로 두부의 다발성 기저세포암을 가진 모반양 기저세포암의 환자를 경험하였으며, 초기에 발견된 작은 병변의 제거 시 2mm의 정상 조직을 포함하여 절제하였어도 3년간의 경과관찰 중 재발없이 좋은 결과를 얻을 수 있어 이를 보고하는 바이다.

Chronic Compartment Syndrome and Stress Fracture (만성구획증후군 및 스트레스 골절)

  • Choi, Chang-Hyuk;Baek, Seung-Hoon;Jang, Il-Woong
    • Journal of Korean Orthopaedic Sports Medicine
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    • v.9 no.1
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    • pp.16-21
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    • 2010
  • The prevalence of overuse syndrome in the lower extremity including chronic compartment syndrome and stress fracture is increasing with popularity of sports activities. Chronic compartment syndrome is defined as elevation of the interstitial pressure during exertional activities in a closed osseofascial compartment that results in microvascular compromise and operative procedures can be necessary if conservative treatments fail. Stress fracture can be classified as fatigue and insufficiency fracture; stress fracture occurs by repeated strain under abnormal conditions from the patient's activity whereas insufficiency fracture does by those from a process intrinsic to the bone. Most stress fractures occur in the lower extremity, most commonly in the tibial region. Fatigue fractures begin in athletes with the change in their training programs. The radiographic findings are usually diagnostic or at least strongly suggestive and MRI has proven to be a beneficial diagnostic tool for difficult diagnostic cases. Fatigue fractures are treated with a decrease in activity, but surgical procedure may be necessary in those in anterior cortex of the tibial diaphysis.

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Jagged1 mutation analysis in Alagille syndrome patients (Alagille 증후군에서 Jagged1 돌연변이)

  • Ko, Jae Sung;Yang, Hye Ran;Kim, Kyung Mo;Seo, Jeong Kee
    • Clinical and Experimental Pediatrics
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    • v.49 no.5
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    • pp.519-522
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    • 2006
  • Purpose : Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has been found mutated in Alagille syndrome. The aim of the study was to investigate the mutation analysis of JAG1 gene in Korean patients with Alagille syndrome. Methods : Genomic DNA was extracted from peripheral leukocytes of 6 patients. The 26 exons of JAG1 gene were amplified and PCR products were directly sequenced. Results : Two novel frameshift mutations were found. 118delC in exon 2 was found in a patient who developed hepatocellular carcinoma at 4 years of age. 999-1000delTG was identified in exon 7. Conclusion : Mutations identified in this study are expected to give rise to truncated proteins.

Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children (소아에서 가역성 후두부 백질 뇌증 증후군의 임상적, 방사선학적 특성에 대한 고찰)

  • Lim, Hae-Ri;Seo, Hye-Eun;Kwon, Sun-Hak
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.901-904
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    • 2007
  • Purpose : Reversible posterior leukoencephalopathy syndrome is a complex disorder with characteristic clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. Methods : A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were retrospectively reviewed and analyzed. Results : Fifteen pateints were involved. (9 males and 5 females). The patients' ages ranged from 2-20 years (mean age : 10 years). The brain MRI revealed fairly symmetric areas of increased T2 signal involving both white and gray matter of parieto-occipital regions. The condition seemed to be associated with cyclosporin A and steroid therapy or a variety of other conditions in which blood pressure rises acutely. Conclusion : Reversible posterior leukoencephalopathy syndrome is a complicated neurological condition, but a better understanding of this complex syndrome may obviate unnecessary investigations and lead to prompt and appropriate management of the associated problems.

Turtle Neck Syndrome Posture Correction Service Using CNN-based Learning Model (CNN기반의 학습모델을 활용한 거북목 증후군 자세 교정 시스템)

  • Han, Ji-Ye;Park, Jin-Ho
    • The Journal of the Korea Contents Association
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    • v.20 no.7
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    • pp.47-55
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    • 2020
  • Along with the increased use of smart devices, the incidence of turtle neck syndrome among modern people has increased. Turtle neck syndrome is a posture in which the head is forward compared to the torso due to longer front muscles in the neck and shorter upper muscles, and it is more effective to fix the usual posture habits than surgery or medication. Thus, in this paper, a system is proposed to detect and warn posture that can cause turtle neck syndrome in real time. Image data of correct posture and turtle neck posture are collected to create a CNN-based learning model. Using only the webcam(Built-in camera), the sitting position that enters the camera is verified in real time through the learning model, and if it is a turtle neck position, it generates a warning sound and induces the correct posture. The system can induce people to correct their usual posture habits to treat turtle neck syndrome and prevent more serious diseases such as neck discs.

Nevoid Basal Cell Carcinoma Syndrome : A Case Report (기저세포모반증후군 : 증례 보고)

  • Lee, Yoonjung;Park, Jaehong;Choi, Sungchul;Lee, Sooeon;Kim, Kwangchul
    • Journal of the korean academy of Pediatric Dentistry
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    • v.41 no.1
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    • pp.34-39
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    • 2014
  • Nevoid basal cell carcinoma syndrome(NBCCS) is a autosomal dominant disorder, and its major manifestations are multiple basal cell carcinoma, keratocystic odontogenic tumor, rib anomalies, palmer and plantar pits, calcification of the falx cerebri. Keratocystic odontogenic tumor(KCOT) is defined as intraosseous tumor of odontogenic origin with a characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive behavior. We report a case of a 3-year-old patient with nevoid basal cell carcinoma syndrome who initially presented with unilocular keratocystic odontogenic tumor in maxillary canine region. Keratocystic odontogenic tumor was treated by enucleation, and periodic follow-up check will be required for early diagnosis of additional diseases related with this syndrome.

Steroid and enalapril therapy - possible cause of toxic epidermal necrolysis (부신 피질 호르몬제와 안지오텐신 수용체 길항제 사용 후 발생한 독성 표피괴사 증후군)

  • Kim, Dong Wook;Jung, Da Eun;Koo, Ja Wook
    • Clinical and Experimental Pediatrics
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    • v.49 no.3
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    • pp.332-336
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    • 2006
  • Toxic epidermal necrolysis (TEN) is a rare, acute and life-threatening cutaneous drug reaction. TEN is characterized by the sudden onset of extensive necrosis in the epidermis and frequent mucous membrane involvement. The pathogenesis has not yet been elucidated. In addition, no particular treatment for TEN has been established. We report a case of TEN in a 14-year-old-boy, which might have been caused by steroids with enalapril treatment for membranous nephropathy. He recovered after intravenous immunoglobulin therapy.

Convergence Analysis of Metabolic Syndrome Risk and Related Factors among Kidney Transplantation Recipients (신장이식 수혜자의 대사증후군 발생 위험 관련 요인에 대한 융복합적 조사연구)

  • Chong, Hye Jin
    • Journal of Digital Convergence
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    • v.18 no.5
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    • pp.375-382
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    • 2020
  • The purpose of this study was to analyze the prevalence, and determine factors associated with metabolic syndrome risk among kidney transplantation recipients. This study data were collected by means of retrospective chart reviews for 111 kidney recipients at an organ transplantation center in South Korea. Data were analyzed using descriptive statistics, t-test or chi-squared test, and Pearson's correlation or Point biserial correlation. The prevalence of metabolic syndrome in our subjects was 65.8%. Metabolic syndrome was related with age, body mass index of before and after Kidney transplantation, and smoking. Study results indicate that intervention for modifying individual lifestyle behaviors is required to prevent and reduce their prevalence of metabolic syndrome after kidney transplantation.