• Title/Summary/Keyword: 장기부전

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Trace Elements Deficiency and the Diagnostic Usefulness of Hair Mineral Analysis in Children with Chronic Gastrointestinal Disease (만성 소화기 질환 환아에서 미량원소 결핍과 모발 검사의 유용성)

  • Hong, Jea-Na;Lee, Jung-Hwa;Lee, Ran;Shin, Jee-Youn;Ko, Jae-Sung;Seo, Jeong-Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.122-129
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    • 2008
  • Purpose: Patients with chronic gastrointestinal disease are at risk for trace element deficiency due to impaired absorption and gastrointestinal loss. The aim of this study was to evaluate the trace element status of patients with gastrointestinal disease by blood and hair analysis, and to determine the usefulness of hair mineral analysis for diagnosing trace element deficiency not detected by a blood test. Methods: An analysis of hair minerals was performed and compared with blood mineral analysis in 13 patients with chronic gastrointestinal disease. The concentration of each element in the hair and blood was compared in the subgroups based on parenteral nutritional support or clinical symptoms. Results: Almost all patients had trace element deficiency. The trace elements deficient in the blood or hair analysis included zinc, selenium and copper. The hair zinc concentration was significantly lower in the group receiving parenteral nutritional support. The hair selenium concentration was statistically associated with the clinical symptoms of hair loss, brittle hair and loss of hair pigmentation. Conclusion: The results of this study suggest that patients with chronic gastrointestinal disease should receive adequate zinc and selenium replacement to avoid trace element deficiency especially when treated with long-term parenteral nutrition. Hair mineral analysis is useful as a complementary tool for the detection of a trace element deficiency.

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The Ornithine Transcarbamylase (OTC) Deficiency Identified by a Novel Mutation (새로운 유전자 돌연변이로 확진된 Ornithine Transcarbamylase (OTC) 결핍증 1례)

  • Song, Ari;Lee, Kiwuk;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.148-154
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    • 2016
  • A urea cycle disorder is a condition caused by a defect of the enzymes in the urea cycle, and deficiency of ornithine transcarbamylase (OTC), which converts carbamoyl phosphate and ornithine into citrulline, is the most common type of the disorder. OTC deficiency induces the accumulation of precursors of urea, ammonia, and glutamine, leading to neurological symptoms including hypotonia, respiratory failure, seizure, lethargy, and coma and sometimes to death. Because OTC deficiency is inherited in an X-linked manner, typical symptoms such as vomiting, poor feeding, and lethargy appear mainly in male neonates. We recently had a case that presented with neonatal onset lethargy, vomiting, and apnea in a 4-day-old boy. He was diagnosed with OTC deficiency by biochemical phenotype, including hyperammonemia and an increased orotic acid level in the urine. Genetic analysis of the OTC gene showed a novel mutation c.780_781insCAGGCAGTGT (p.Ile261Glnfs*35). He was treated for hyperammonemia using continuous venovenous hemofiltration (CVVH) at 118 hours after birth. After 4 days of CVVH, his consciousness and blood ammonia concentration were normalized, and he was discharged at the age of 53 days. At around 12 months of age, bilateral femur fractures and osteomyelitis occurred in this patient. Two months after the fracture, he died of septic shock, insulin-resistant hyperglycemia, and multi-organ failure.

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Cause Analysis on Dry of stream flow for the Ui-Cheon (우이천의 건천화 원인분석)

  • Yi, Choong Sung;Choi, Seung An;Kim, Hung Soo;Shim, Myung Pil
    • Proceedings of the Korea Water Resources Association Conference
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    • 2004.05b
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    • pp.753-758
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    • 2004
  • 건천화란 하천이 건천이 되어가는 과정이라고 할 수 있다. 그러나 이의 정량적 접근이 어려운 이유는 건천이 되어가는 과정에 대한 현상을 지속적으로 관찰하기 어렵고, 순수한 자연현상이라기보다 인위적 요소가 간섭한 결과이기 때문이다. 본 연구는 건천이 되어가는 하천 중에서도 도시화에 의한 건천화의 영향이 큰 것으로 보이는 우이천 유역에 대해 건천화의 원인을 분석하여 몇 가지 결론과 대책을 제시하였다. 우이천은 유역면적 $27.67km^2$, 유로연장 11.75km의 비교적 좁은 면적 내에 인구 461,351명이 거주하고 주변 행정구역 총 인구가 604,282명인 지방2급하천이다. 유역 내 북부와 북서부의 산악지역을 통과하는 상류는 대부분 계류(溪流)로서 자연상태를 유지하고 있으나, 중${\cdot}$하류는 양안의 셋방, 복개, 직강화, 보 및 낙차공 등 인공구조물로 인해 하천형상이 크게 왜곡되어 있는 전형적인 도시하천유역이다. 본 연구는 우이천에 대한 건천화 원인분석을 위해 크게 4가지 방봅으로 접근하였는데 반분포형 유출모형인 TOPMODEL에 의한 장기유출분석, 수문${\cdot}$기상자료에 의한 분석, 인문${\cdot}$사회자료의 분석, 지하수위 분석 등이었다. 분석결과 우이천 유역과 같이 장기간에 걸쳐 도시화가 진행된 유역은 수문순환계의 왜곡이 돌이킬 수 없는 수준이므로, 적절한 건천화 방지대책의 방향은 건천화의 원인이 되는 인자를 제거함으로써 줄어든 유량을 회복하는 방안보다는 인위적이고 강제적인 방식으로 추가적인 하천수량을 확보하는 것이 타당하다 사료된다. 하천의 건천화는 하나의 주된 원인이 지배적일 수도 있지만 간접적인 영향이 많고, 하나의 원인이 다른 인자에 영향을 주어 연쇄적인 영향을 미칠 수도 있는 복잡한 현상이다. 따라서 다양한 각도에서 다양할 접근방식으로 방지대책을 수립하여야 할 것이다.적에 따른 임계지속기간과 유출량의 변화를 검토해 보았다.이를 각각의 경우의 해석해 결과와 비교${\cdot}$분석하였다. 후방추적 퍼프모형은 전방추적 퍼프모형에 비하여 사용된 퍼프수와 관계없이 작은 오차를 발생하였으며, 전체적으로 퍼프 모형이 입자모형보다는 훨씬 적은 수의 계산을 통해서도 작은 오차를 나타낼 수 있다는 것을 알 수 있었다. 그러나 Gaussian 분포를 갖는 퍼프모형은 전단흐름에서의 긴 유선형 농도분포를 모의할 수 없었고, 이에 관한 오차는 전단계수가 증가함에 따라 비선형적으로 증가하였다. 향후, 보다 다양한 흐름영역에서 장${\cdot}$단점 분석 및 오차해석을 수행한 후에 각각의 Lagrangian 모형의 장점만을 갖는 모형결합 방법을 제시할 수 있을 것으로 판단된다.mm/$m^{2}$로 감소한 소견을 보였다. 승모판 성형술은 전 승모판엽 탈출증이 있는 두 환아에서 동시에 시행하였다. 수술 후 1년 내 시행한 심초음파에서 모든 환아에서 단지 경등도 이하의 승모판 폐쇄 부전 소견을 보였다. 수술 후 조기 사망은 없었으며, 합병증으로는 유미흉이 한 명에서 있었다. 술 후 10개월째 허혈성 확장성 심근증이 호전되지 않아 Dor 술식을 시행한 후 사망한 예를 제외한 나머지 6명은 특이 증상 없이 정상 생활 중이다 결론: 좌관상동맥 페동맥이상 기시증은 드물기는 하나, 영유아기에 심근경색 및 허혈성 심근증 또는 선천성 승모판 폐쇄 부전등을 초래하는 심각한 선천성 심질환이다. 그러나 진단 즉시 직접 좌관상동맥-대동맥 이식술로 수술적 교정을 해줌으로써 좋은 성적을 기대할 수 있음을 보여주었다.특히 교사들이 중요하게 인식하는 해방적 행동에 대한 목표를 강조하여 적용할 필요가 있음을 시사하고 있다.교하여 유의한 차이가 관찰되지 않았다. 또한 HSP 환자군에서도 $IL1RN^{*}2$ allele 빈도와 carria

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Operation of Ascending Aorta and/or Aortic Arch (상행대동맥 및 대동맥궁의 수술)

  • 구본원;허동명;전상훈;장봉현;이종태;김규태;이응배
    • Journal of Chest Surgery
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    • v.29 no.11
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    • pp.1212-1217
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    • 1996
  • From Dec. 1993 to May 1995, 9 male and 5 female patients ranging in age from 25 to 65 years, were operated on for ascending aorta and/or aortic arch diseases. Six patients had acute aortic dissection, type A(ruptured in 4 cases); four had ruptured ascending aortic aneurysm; three had annuloaortic ectasia(ruptured in 1 cases); one had aortic arch aneurysm. The diagnostic procedures were echo cardiography and dynamic CT scan in all patients having acute dissection or rupture. The aortic angiography was performed in two cases. Indications for operations were rupture in five cases, acute aortic dissection in five cases, severe congestive heart failure in two cases, progressive aortic insufficiency in one case and impending rupture in one case. The emergent repair was performed in ten cases(71%). The surgical treatment consisted of 6 Cabrol operations, a Cabrol operation combined with arch replacement, a modified Bentall operation, 4 replacement of ascending aorta, a replacement of aortic arch, and a replacement of ascending aorta and aortic arch. Complications were a hypoxic encephalopathy, two atrial fibrillations, a sternal deheiscence, and a mediastinitis. Two early mortality(14%) were due to intractable bleeding and multiple organ failure, and one late mortality(7%) was due to ventricular arrhythmia. In eleven survivors, follow-up period was from 2 months to 12 months and the course was uneventful.

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The 10 Years Experience of Lung Transplantation (폐 이식 수술의 10년 치험)

  • Paik, Hyo-Chae;Hwang, Jung-Joo;Kim, Do-Hyung;Joung, Eun-Kyu;Kim, Hae-Kyoon;Lee, Doo-Yun
    • Journal of Chest Surgery
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    • v.39 no.11 s.268
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    • pp.822-827
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    • 2006
  • Background: Lung transplantation is a definitive therapy for a variety of end stage lung diseases. Since 1996, we have performed thirteen cases of lung transplantation including two retransplantations, and we analyzed the outcomes, complications, and survivals of these patients. Material and Method: We retrospectively analyzed the medical records of thirteen cases from July, 1996 to July, 2005. Result: During the period, 11 patients had undergone 43 lung and heart-lung transplantations, and two patients had retransplantation due to allograft failure. Mean age of recipients were $45.2{\pm}10.7$ years(range, $25{\sim}59$). Early complications were bleeding, reperfusion injury, and infection and late complications were mainly infection and post-transplantation lymphoproliferative disease. Excluding the operative mortality, the mean survival period was 16.5 months($2{\sim}60$ months). Two retransplantations had been performed 2 weeks and 13 months after single lung transplantations. Conclusion: In order to achieve long term survival, early detection of complications and proper treatment in addition to surgical skills are necessary, and these efforts can promote better lung transplantation programs in the near future.

Clinical Efficacy of the Transradial Approach in Percutaneous Intervention for a Malfunctioning Arteriovenous Fistula (혈액 투석 동정맥루의 기능 부전에 대한 인터벤션 치료 시 경요골 동맥 접근법의 유용성)

  • Hyun Young Choi;Gyoo-Sik Jung;Hee Kang;Ye Na Kim;Hyung Hwan Moon;Jong Hyouk Yun
    • Journal of the Korean Society of Radiology
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    • v.83 no.3
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    • pp.658-668
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    • 2022
  • Purpose To evaluate the effectiveness of the transradial artery approach (TRA) for treating malfunctioning arteriovenous fistulas (AVFs) in patients on hemodialysis. Materials and Methods A retrospective analysis was conducted in this single-center study of TRA endovascular procedures in 73 patients (43 male and 30 female; mean age of 67.4 years (range, 42-92 years) with malfunctioning AVFs, between January 2008 and April 2019. Patients' baseline and lesion characteristics, technical and clinical success, and complications were evaluated, and functional patency was analyzed using the Kaplan-Meier method. Results Radial artery approaches were successful in all patients. Angioplasty performed using the TRA achieved technical and clinical success rates of 98.6%(72/73) and 91.7%(67/73), respectively. The median primary patency time was 18.8 ± 15.9 months. The primary functional patency rates at 3, 6, and 12 months were 82.1%, 68.6%, and 63.9%, respectively. There were no major complications or adverse events, such as hand ischemia, related to the radial artery approach. Conclusion In selected cases, the TRA can be used complementary to the transvenous approach to treat malfunctioning AVFs.

Long Term Survival after the Resection of Esophageal Cancer (식도암 절제수술 후 장기 성적)

  • 김영태;성숙환;김주현
    • Journal of Chest Surgery
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    • v.32 no.7
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    • pp.653-659
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    • 1999
  • Background: Despite the recent promising efforts to improve survival in patients with esophageal carcinoma, the long term survival results of patients with esophageal cancer have seldom been reported in Korea. To establish standard control for future studies, we re trospectively analyzed the surgical treatment results of the esophageal cancer patients managed in our department at Seoul National University Hospital. Material and Method: From January 1984 to December 1996, 734 patients were diagnosed with esophageal cancer. Among them, 253 patients underwent surgery in our department. We retrospectively analyzed the operative results and long term survival rates of these patients. Result: The majority of patients(237) had squamous cell histology and only 6 patients had adenocarcinoma. The final TNM stage grouping for these patients was based on the 1988 revised American Joint Commitee on Cancer classification. Twenty one patients were surgically classified as stage I, 109 as stage II, and 107 as stage III. C respiratory failure in 8, sepsis in 1, hepatic failure in 1, bleeding in 1 and unknown etiology in the remaining 3. The actuarial survival of 222 patients in whom the curative resection was accomplished at 1-, 2-, 3- and 5 years was 74.7${\pm}$3.1%, 46.5${\pm}$3.7%, 32.3${\pm}$3.7%, and 19.9${\pm}$3.3%, respectively. CONCLUSION: The poor long term survival rates suggest that an alternative treatment method such as intensive combined modality therapy should be developed for the management of esophageal cancer.

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Operative Treatment of Congenitally Corrected Transposition of the Great Arteries(CCTGA) (교정형 대혈관 전위증의 수술적 치료)

  • 이정렬;조광리;김용진;노준량;서결필
    • Journal of Chest Surgery
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    • v.32 no.7
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    • pp.621-627
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    • 1999
  • Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

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A Case of Primary Signet Ring Cell Carcinoma of the Lung (원발성 기관지 반지세포암 1예)

  • Choi, Won-Il;Sohn, Jeong-Ho;Kwon, Oh-Young;Hur, Jeong-Suk;Hwang, Jae-Seok;Han, Seong-Beom;Song, Hong-Suck;Jeon, Young-June;Kwon, Kun-Young
    • Tuberculosis and Respiratory Diseases
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    • v.41 no.5
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    • pp.562-567
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    • 1994
  • Signet ring cell carcinoma has been previously described in many organs, most frequently in the stomach, and rarely in the colon, rectum, gallbladder, pancreas, breast, nadsal cavity, prostate, urinary bladder and ureter. Signet ring cell carcinomas in the lung, especially, when examined by small biopsies, are generally believed to be metastatic. This case was diagnosed by bronchoscopic biopsy. We also examined various organs by noninvasive method, including UGI series, barium enema and abdomen CT scan, but all studies were nomal. Patient received cisplatin and etoposide combination chemotherapy followed by local radiotherapy as a primary non-small cell lung cancer. Patient died of his disease 6 months after diagnosis. Now we report a case of primary signet ring cell carcinoma of the lung.

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Derodidymus in Korean-Native Calf (한우 송아지의 이두이안체)

  • 강문일;박영석;한동운
    • Korean Journal of Animal Reproduction
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    • v.23 no.1
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    • pp.69-73
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    • 1999
  • A calf with derodidymus was delivered from a 3-year old Korean-native cow with abortion last ime at Jangheong area in Chonnam Province. The anomaly with body weight of 35 kg was born t the normal time of parturition. During the parturition, however, the calf with twin head was onfirmed and the complete amputation between fore and hind limb was inevitably carried out or safety of the dam. The calf had normal skeleton and extremities but had two heads and cervical vertebrae divided from upper thoracic part. At necropsy, there were found totally 3 pairs of ore limbs including two pairs of hypoplastic ones hided in the thoracic limb and also found one air of sternum. One head had incomplete torsion or unilateral hypoplasia of mandible with artly hypoplastic skull. There were marked fusion and torsion from cervical to 3rd thoracic vertebra. No abnormality was found on all organs in the pleural and abdominal cavities except a rectal stricture formed at 5 cm away from the atresia ani.

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