• 제목/요약/키워드: failure causes

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Renal Sympathetic Denervation Induces Acute Myocardial Inflammation through Activation of Caspase-1 and Interleukin-1β (교감신경절제술(RDN) 후 caspase-1과 interleukin-1β 활성화로 인해 유발된 염증성 급성심근손상)

  • Lee, Dong Won;Kim, Il Young;Kwak, Ihm Soo
    • Journal of Life Science
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    • v.28 no.2
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    • pp.240-246
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    • 2018
  • Efferent and afferent sympathetic nerves are closely related to the development of hypertension and heart failure. Catheter-based renal sympathetic denervation (RDN) is implemented as a strategy to treat resistant hypertension. We investigated whether RDN procedure causes inflammatory damage on myocardium in the early phase of sympathetic denervation. Twenty-five female swine were divided into 3 groups: normal control (Normal, n=5), sham-operated control (Sham, n=5), and RDN groups (RDN, n=15). The RDN group was further subdivided into 3 subgroups according to the time of sacrifice: immediately (RDN-0, n=5), 1 week (RDN-1, n=5), and 2 weeks (RDN-2, n=5) after RDN. There were no significant changes in the clinical parameters between the normal control and sham-operated group using contrast-media. In the myocardium, inflammatory cytokines, $IL-1{\beta}$ and $TNF-{\alpha}$ increased at the first week, and then decreased at the second week after RDN. Anti-inflammatory cytokine, IL-10 increased immediately, and then decreased at the second week after RDN. Caspase-1 activity and apoptosis-associated speck-like protein containing a caspase recruitment domain (ASC) expression increased immediately after RDN until the second week. However, nucleotide-binding oligomerization domain, leucine rich repeat and pyrin domain containing protein 3 (NLRP3) expression did not show any significant differences among the groups. The RDN can cause acute myocardial inflammation through activation of caspase-1 and $IL-1{\beta}$. We should pay attention to protecting against early inflammatory myocardial damage after RDN.

Nutritional Status of Continuous Ambulatory Peritoneal Dialysis Patients (지속성복막투석 환자의 영양상태에 관한 연구)

  • Park, Jin-Kyung;Son, Sook-Mee
    • Journal of Nutrition and Health
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    • v.39 no.7
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    • pp.624-640
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    • 2006
  • Dialysis patients are at risk of malnutrition not only because of losses of nutrients during peritoneal dialysis but also because of anorexia that results in inadequate nutrient intakes. The aim of this study was to estimate the nutritional status of 154 patients receiving continuous ambulatory peritoneal dialysis (CAPD), especially focused on protein-energy malnutrition and vitamin and mineral status. The mean age of the subjects was $5.12\;{\pm}\;12.4\;y$ with educational years of $12.3\;{\pm}\;0.4\;y$ for male and $9.6\;{\pm}\;0.4\;y$ for female. The mean duration of dialysis was $22.7\;{\pm}\;21.7\;mo$. The causes of renal failure included diabetes (32.7), chronic glomerulonephritis (15.0%), and hypertension (8.5%). The main complications associated with chronic renal failure were hypertension (86.1%), diabetes (35.4%) and liver disease (9.0%). The mean daily energy intake was $1216.8\;{\pm}\;457.3\;kcal$ and increased to $1509.2\;{\pm}\;457.2\;kcal$ when added with the energy from dextrose in dialysate. The latter was still much lower than estimated energy requirement but energy intake per kg of body weight (28.1 kcal/1 g) was within the range of that recommended for CAPD patients' diet therapy (25 - 30 kcal/kg). The average daily intake of protein was $49.2\;{\pm}\;25.1\;g$ with 37.6% of the patients showing their intakes less than Estimated Average Requirement. The average protein intake per kg of weight was 0.9 g/kg, which is less than that recommended for CAPD patients (1.2-1.5g/kg) with mean serum albumin level $3.2\;{\pm}\;0.5\;g/dl$. The proportion of the patients with dietary calcium intake less than EAR was 90.9%, but when added with supplementary calcium (phosphorus binder), most patients showed their total calcium intake between EAR and UL. Fifty percent of the patients were observed with dietary iron intake less than EAR, however most patients revealed their total iron intake with supplementation above UL. The addition of folic acid with supplementation increased mean total folic intake to $1126.0\;{\pm}\;152.4\;{\mu}g$ and ninety eight percent of the subjects showed their total folic acid intake above UL. The prevalence of anemia was 83.1 % assessed with hemoglobin level, even with high intakes of iron with supplementation. Thirty four percent of the patients showed their fasting blood glucose was not under control $(\geq\;126\;mg/dl)$ even with medication or insulin probably due to dextrose from dialysate. The mean blood lipid levels were within the reference levels of hyperlipidemia, but with 72.1 % of the patients showing lower HDL-C. In conclusion, Fairly large proportion of the patients were observed with protein malnutrition with low intake of protein and serum albumin level. Few patients showed their vitamins and minerals intake less than EAR with supplementation. For iron and folic acid, their intakes were increased to above UL for large proportion of he patients. However, more than eighty percent of the patients were still anemic associated with decreased renal function. The serum blood glucose and lipid level were not under control for some patients with medication. It seems that supplementation and medications that patients are taking should be considered for dietary consulting of CAPD patients.

Treatment of Isoniazid-Resistant Pulmonary Tuberculosis (Isoniazid 내성 폐결핵의 치료실태와 치료성적)

  • Koh, Won-Jung;Kwon, O Jung;Yu, Chang-Min;Jeon, Kyeongman;Kim, Kyung Chan;Lee, Byoung-Hoon;Hwang, Jung Hye;Kang, Eun Hae;Suh, Gee Young;Chung, Man Pyo;Kim, Hojoong
    • Tuberculosis and Respiratory Diseases
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    • v.56 no.3
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    • pp.248-260
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    • 2004
  • Background : As an effective regimen for isoniazid (INH)-resistant pulmonary tuberculosis, several treatment regimens have been recommended by many experts. In Korea, a standard regimen has not been established for INH-resistant tuberculosis, and the treatment by individual physicians has been performed on an empirical bases. The purpose of the present study was to retrospectively describe the treatment characteristics and evaluate the treatment outcomes of patients with INH-resistant tuberculosis. Materials and Methods : Sixty of 69 patients reported to have INH-resistant tuberculosis from 1994 to 2001 were retrospectively analyzed. Exclusion criteria included: death from other causes, with the exceptions of tuberculosis and incomplete treatment, including a patient's transfer-out. Results : A previous tuberculosis history was found in 28 (46.7%) patients. The sputum smear for acid-fast bacilli was positive in 44 (73.3%) patients, and 30 (50.0%) had cavitary disease. Streptomycin resistance coexisted in 25.0% of isolates. INH was to be prescribed continuously, even after INH resistance was reported, in 86.0% of patients. The treatment regimens were diverse between the patients according to drug regimen composition and treatment duration. The most frequent prescribed regimen included rifampin, ethambutol and pyrazinamide, with and without INH, for the full 12-month term of treatment. Treatment failure occurred in 13 (21.7%) patients. Cavitary disease (p=0.005) and a treatment regimen with second-line drugs, excluding rifampin (p=0.015), were associated with treatment failure. One patient experienced a relapse. Conclusions : Standardized treatment guidelines will be needed in Korea to improve the treatment efficacy for INH-resistant tuberculosis.

EFFECT OF THE EXPONENTIAL CURING OF COMPOSITE RESIN ON THE MICROTENSILE DENTIN BOND STRENGTH OF ADHESIVES (복합레진의 exponential 중합법이 상아질접착제의 미세인장접착강도에 미치는 영향)

  • Seong, So-Rae;Seo, Duck-kyu;Lee, In-Bog;Son, Ho-Hyun;Cho, Byeong-Hoon
    • Restorative Dentistry and Endodontics
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    • v.35 no.2
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    • pp.125-133
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    • 2010
  • Objectives: Rapid polymerization of overlying composite resin causes high polymerization shrinkage stress at the adhesive layer. In order to alleviate the shrinkage stress, increasing the light intensity over the first 5 seconds was suggested as an exponential curing mode by an LED light curing unit (Elipar FreeLight2, 3M ESPE). In this study, the effectiveness of the exponential curing mode on reducing stress was evaluated with measuring microtensile bond strength of three adhesives after the overlying composite resin was polymerized with either continuous or exponential curing mode. Methods: Scotchbond Multipurpose Plus (MP, 3M ESPE), Single Bond 2 (SB, 3M ESPE), and Adper Prompt (AP, 3M ESPE) were applied onto the flat occlusal dentin of extracted human molar. The overlying hybrid composite (Denfil, Vericom, Korea) was cured under one of two exposing modes of the curing unit. At 48h from bonding, microtensile bond strength was measured at a crosshead speed of 1.0 mm/min. The fractured surfaces were observed under FE-SEM. Results: There was no statistically significant difference in the microtensile bond strengths of each adhesive between curing methods (Two-way ANOVA, p > 0.05). The microtensile bond strengths of MP and SB were significantly higher than that of AP (p < 0.05). Mixed failures were observed in most of the fractured surfaces, and differences in the failure mode were not observed among groups. Conclusion: The exponential curing method had no beneficial effect on the microtensile dentin bond strengths of three adhesives compared to continuous curing method.

Open Heart Surgery after Pulmonary Artery Banding in Children (소아에서 폐동맥밴딩술후의 개심술 치료)

  • 김근직;천종록;이응배;전상훈;장봉현;이종태;김규태
    • Journal of Chest Surgery
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    • v.32 no.9
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    • pp.781-789
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    • 1999
  • Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.

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A Study on the Guidelines on the Insertion of Metal Stiffeners in the Restoration of Stone Cultural Heritages (석조문화재 복원을 위한 금속보강재 매입방법 표준화 연구)

  • Lee, Dong-sik;Kim, Hyun-yong;Kim, Sa-dug;Hong, Seong-geol
    • Korean Journal of Heritage: History & Science
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    • v.46 no.3
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    • pp.212-228
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    • 2013
  • Stone cultural heritages are repaired by the use of metal stiffeners. The problem is that this type of repair has been based on the experience of workers without specific guidelines and has caused various problems. This is to suggest the structural reinforcement and behavioral characteristics of metal rods to minimize the secondary damage of materials and have the specimens tested and verified to establish the guidelines on how to insert metal stiffeners. When only epoxy resin is applied to the cut surface, only 70% of the properties of the parent material are regenerated and it is required to structurally reinforce the metal stiffener for the remaining 30%. The metal rod is under the structural behavior after the brittle failure of stone material and the structural behavior does not occur when the metal stiffener is below 0.251%. When it accounts for over 0.5%, it achieves structural reinforcement, but causes secondary damage of parent materials. The appropriate ratio of metal stiffener for the stone material with the strength of $1,500kgf/cm^2$, therefore, should be between 0.283% and 0.377% of the cross section of attached surface to achieve reversible fracture and ductility behavior. In addition, it is more effective to position the stiffeners at close intervals to achieve the peak stress of metal rod against bending load and inserting the stiffener into the upper secions is not structurally supportive, but would rather cause damage of the parent material. Thus, most stiffeners should be inserted into the lower part and some into the central part to work as a stable tensile material under the load stress. The dispersion effect of metal rods was influenced by the area of reinforcing rods and unrelated to their diameter. However, it ensures stability under the load stress to increase the number of stiffeners considering the cross section adhered when working on large-scale structures. The development length is engineered based upon the diameter of stiffener using the following formula: $l_d=\frac{a_tf_y}{u{\Sigma}_0}$. Also, helically-threaded reinforcing rods should be used to perform the behaviors as a structural material.

Results of Hyperfractionated Radiation Therapy in Bulky Stage Ib, IIa, and IIb Uterine Cervical Cancer (종괴가 큰 병기 Ib, IIa, IIb 자궁경부암에서 다분할 방사선치료의 결과)

  • Kim, Jin-Hee;Kim, Ok-Bae
    • Radiation Oncology Journal
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    • v.15 no.4
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    • pp.349-356
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    • 1997
  • Purpose : To evaluate the efficacy of hyperfractionated radiation therapy in carcinoma of the cervix, especially on huge exophytic and endophytic stage Ib, IIa and IIb Materials and Materials : Fourty one patients with carcinoma of the cervix treated with hyperfractionated radiation therapy at the Department of Therapeutic Radiology, Dongsan Hospital, Keimyung University. School of Medicine from Jul, 1991 to Apr, 1994. According to FIGO s1aging system, therewere stage Ib (3 patients) IIa (6 patients) with exophytic ($\geq$5cm in dinmeter) and huge endophytic mass. and IIb (32 patients) with median age of 55 yeavs old. Radiation therapy consisted of hyperfractionated external irradition to the whole pelvis (120cGy/fraction, 2 fraction/day (minimum interval of 6 hours), 3600-5520cGy) and boost parametrial doses (for a total of 4480-6480cGy) with midline shield $(4\times10cm)$, and combined with intracavitary irradiation (up to 7480-8520cGy in Ib, IIa and 8480-9980cGy in IIb to point A). The maximum and mean follow up durations were 70 and 47 months respectively . Results : Five year local control rate was $78\%$ and the actuarial overall five year survival rate was $66.1\%$ for all patients, $44.4\%$ for stage Ib, IIa and $71.4\%$ for stage IIb. In bulky IIb (above 5cm in tumor size, 11 patients) five year local control rate and five rear survival rate was $88.9\%,\;73\%$ respectively Pelvic lymph node status (negative : $74\%,\;positive:25\%$, p=0.0015) was significant Prognostic factor affecting to five rear survival rate. There was marginally significant survival difference by total dose to A point ($>84Gy\;:\;70\%,\;>84Gy\;:\;42.8\%$, p=0.1). We consider that the difference of total dose to A point by stage (mean Ib,IIa : 79Gy. IIb 89Gy P=0.001) is one of the causes in worse local control and survival of Ib,IIa than IIb The overall recurrence rate was $39\%$ (16/41). The rates of local failure alone. distant failure alone. and combined local and distant failure were $9.7\%,\;19.5\%,\;and\;9.7\%$, respectively. Two Patients developed leukopenia ($\geq$ grade 3) and Three patients develoued grade 3 gastrointestinal complication. Above grade 3 complication was not noted. There was no treatment related death noted. Conclusion : We thought that it may be necessary to increase A point dose to more than 85Gy in hyperfractionated radiotherapy of huge exophytic and endophvtic stage Ib,IIa. We considered that hyperfractionated radiation therapy may be tolerable in huge exophytic and endophytic stage IIb cervical carcinoma with acceptable morbidity and possible survival gain but this was results in small patient group and will be confirmed by long term follow up in many patients.

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A Study for Improvement of Erythropoietin Responsiveness in Hemodialysis Patients (혈액 투석 환자에서 조혈 호르몬 치료 효과 향상에 대한 연구)

  • Park, Jong-Won;Do, Jun-Yeung;Yoon, Kyung-Woo
    • Journal of Yeungnam Medical Science
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    • v.18 no.2
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    • pp.226-238
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    • 2001
  • Background: Anemia in chronic renal failure plays an important role in increasing morbidity of dialysis patients. The causes of the anemia are multifactorial. With using of erythropoietin(EPO) most of uremia-induced anemia can be overcome. However, about 10% of renal failure patients shows EPO-resistant anemia. Hyporesponsiveness to EPO has been related to many factors: iron deficiency, aluminum intoxication, inflammations, malignancies and secondary hyperparathyroidism. So I evaluated the improvement of EPO responsiveness after correction of above several factors. Materials and Methods: Seventy-two patients on hemodialysis over 6 months were treated with intravenous ascorbic acid(IVAA, 300 mg t.i.w. for 12 weeks), After administration of IVAA for 12 weeks, patients were classified into several groups according to iron status, serum aluminum levels and i-PTH levels. Indivisualized treatments were performed: increased iron supplement for absolute iron deficiency, active vitamin D3 for secondary hyperparathyroidism and desferrioxamine(DFO, 5 mg/kg t.i.w.) for aluminum intoxication or hyperferritinemia. Results: 1) Result of IVAA therapy for 12 weeks on all patients(n=72). Hemoglobin levels at 2, 4, 6 week were significantly elevated compared to baseline, but those of hemoglobin at 8, 10, 12 week were not significantly different. 2) Result of IVAA therapy for 20 weeks on patients with 100 ${\mu}g/l$ ${\leq}$ ferritin < 500 ${\mu}g/l$ and transferrin saturation(Tsat) below 30%(n=30). After treatment of IV AA for 12 weeks, patients were evaluated the response of therapy according to iron status. Patients with 100 ${\mu}g/l$ ${\leq}$ ferritin < 500 ${\mu}g/l$ and Tsat below 30% showed the most effective response. These patients were treated further for 8 weeks. Hemoglobin levels at 2, 4 week were significantly increased compared to baseline with significantly reduced doses of EPO at 2, 4, 6, 10, 12, 16, 20 week. Concomitantly significantly improvement of Tsat at 2, 6, 16, 20 week compared to baseline were identified. 3) Result of IVAA therapy for 12 weeks followed by DFO therapy for 8 weeks on patients with serum aluminum above 4 ${\mu}g/l$(n=12) Hemoglobin levels were not significantly increased during IVAA therapy for 12 weeks but dosages of EPO were significantly decreased at 2, 4, 6, 8 week during DFO therapy compared to pre-treatment status. Conclusion: IVAA can be helpful for the treatment of the anemia caused by functional iron deficiency and can reduce the dosage of EPO for anemia correction. And administration of low dose DFO, in cases of increased serum aluminum level, can reduce the requirement of EPO.

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Long-term Effect of Desferrioxamine to rHuEPO Resistant Anemia in Hemodialysis Patients (혈액 투석 환자에서 나타나는 rHuEPO 저항성 빈혈에 대한 Desferrioxamine의 장기 효과)

  • Lim, Sang-Woo;Jung, Hang-Jae;Bae, Sung-Wha;Do, Jun-Young;Yoon, Kyung-Woo
    • Journal of Yeungnam Medical Science
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    • v.14 no.2
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    • pp.399-414
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    • 1997
  • There are several factors concerning to anemia in chronic renal failure patients. But when rHuEPO is used, most of these factors can be overcome, and the levels of hemoglobin are increased. However, about 10% of the renal failure patients represent rHuEPO-resistant anemia eventhough high dosage of rHuEPO. For these cases, desferrioxamine can be applied to correct rHuEPO resistnacy, and many mechanism of DFO are arguing. So we are going to know whether DFO can be applied to correct anemia of the such patients, how long its effect can be continued. The seven pateients as experimental group(DFO+EPO) who represent refractoriness to rHuEPO and the other seven patients as control group(EPO) were included. Experimental group had lower than 9 g/dL of hemoglobin levels despite high rHuEPO dosage (more than 4000U/Wk) and showed normocytic normochromic anemia. There were no definitve causes of anemia such as hemorrhage or iron deficiency. Control group patients had similar characteristics in age, mean dialysis duration but showed adequate response to rHuEPO. DFO was administered to experimental group for 8 weeks along with rHuEPO(the rHuEPO individual mean dosage had been determined by mean dosage of the previous 6 months. Total mean dosage; 123.5 U/Kg/Wk). After 8 weeks of DFO administration, the hemoglobin and rHuEPO dosage levels were checked for 15 consecutive months. It should be noted that the patients determined their own rHuEPO dosage levels according to hemoglobin levels and economic status. In conrol group, rHuEPO was administered by the same method used in experimental group without DFO through the same period. Fifteen months of observation period after DFO trial were divided as Time I(7 months after DFO trial) and Time II(8 months after Time I). The results are as follows: Before DFO trial, mean hemoglobin level of experimental group was 7.8 g/dL, which is similar level(p>0.05) to control group(mean Hb; 8.2 g/dL). But in experimental group, significantly(p<0.05) higher dosages of rHuEPO(mean; 123.5 U/Kg/Wk) than control group (mean; 41.6 U/Kg/Wk) had been used. It means resistancy to rHuEPO of experimental group. But after DFO trial, the hemoglobin levels of the experimental group were increased significantly(p<0.05), and these effect were continued to Time II.(Time I; mean 8.6g/dL, Time II; mean 8.6g/dL) The effects of DFO to hemoglobin were continued for 15 months after DFO trial with similar degree through Time I, Time II. Also, rHuEPO dosages used in the experimental group were decreased to similar levels of the control group after DFO trial and these effect were also continued for 15 months(Time I; mean 48.1 U/Kg/Wk. Time II; mean 51.8 U/Kg/Wk). In the same period, hemoglobin levels and rHuEPO dosages used in the control group were not changed significantly. Notibly, hemoglobin increment and rHuEPO usage decrement in experimental group were showed maxilly in the 1st month after DFO trial. That is, after the use of DFO, erythopoiesis was enhanced with a reduced rHuEPO dosage. So we think rHuEPO reisistancy can be overcome by DFO therapy. In conclusion, the DFO can improve the anemia caused by chronic renal failure at least over 1 year, and hence, can reduce the dosage of rHuEPO for anemia correction. Additional studies in order to determine the mechanism of DFO on erythropoiesis and careful attention to potential side effects of DFO will be needed.

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Tetralogy of Fallot Associated with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries -Comparison between the different surgical approaches- (폐동맥폐쇄와 주대동맥폐동맥혈관부행지를 동반한 활로씨사징증 -다양한 외과적 치료접근의 비교-)

  • 이정렬;양지혁;김용진;노준량
    • Journal of Chest Surgery
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    • v.32 no.5
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    • pp.471-479
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    • 1999
  • Background: Patients with tetralogy of Fallot(TOF), pulmonary atresia(PA) and major aortopulmonary collateral arteries(MAPCAs) have been managed by heterogenous surgical strategies. We have taken 5 different surgical approaches to this lesion. In this study, relative advantages and disadvantages of each strategy were discussed by analyzing our surgical results. Material and Method: Between January 1986 and June 1998, 50 patients aged 1 to 177(39${\pm}$31) months at a various morphologic spectrum of this lesion were treated at our institution. The groups, which were classified according to the initial approaches, consisted of one-stage unifocalization(A-1, n=9), staged unifocalization(A-2, n=11), right ventricle to pulmonary artery connection(RV-PA)(B-1, n=11), one-stage RV-PA plus unifocalization (B-2, n=11), and one-stage definitive repair(C, n=8). Morphologic charateristics, operative mortalities, and probabilities of definitive repair were compared between the groups and the causes of death, complications and the follow-up results were described. Result: Mean ages at the first operation were 57${\pm}$18(A-1), 42${\pm}$48(A-2), 18${\pm}$14(B-1), 52${\pm}$55(B-2), and 32${\pm}$34(C) months (p<0.05). Mean numbers of MAPCAs were least in group C (A-1=4.3${\pm}$1.0, A-2=4.5${\pm}$1.3, B-1=4.1${\pm}$1.9, B-2=4.1${\pm}$1.6, C=3.4${\pm}$1.8 : p<0.05). The ratios of the direct and the indirect MAPCAs in each group were not different between the grousps (A-1=91%/9%, A-2=78%/22%, B-1=80%/20%, B-2=80%/20%, C=81%/19% : p>0.05). Nineteen patients had more than 1 dependent MAPCAs. True pulmonary arteries were not present 13 patients and they were confluent in 29. A total of 101 operations were performed. Operative mortalities of initial procedures were 33%(3/9, A-1), 18%(2/11, A-2), 0%(0/11, B-1), 36%(5/11, B-2) and 13%(1/8, C) (p<0.05). Percentages of the definitive repair among the patients of each group were 22%(2/9, A-1), 18%(2/11, A-2). 45%(5/11, B-1), 27%(4/11, B-2), and 100%(8/8, C)(p<0.05). Hypoxic respiratory and cardiac failure(6), hypoxic encephalopathy(2) and sepsis(4) were the major causes of death. Phrenic nerve palsy occured in 5 patients. Thirty-one patients among the survivals have been followed up for a mean duration of 74${\pm}$42(3-145) months. One and five year actuarial survival rates were 73% and 73 %. Conclusion: In conclusion, Right ventricle to pulmonary artery connection used in patients with TOF/PA/MAPCA as an inital procedure appeared to be highly successful in enhancing the chance of satisfactory definitive repair without the significant surgical risks. One-stage total repair at an ealier age group could be performed safely with the resonable outcomes. Unifocalization approach, whether it was performed in a single stage or in the multiple stages, resulted in the high operative mortality and the lowest chance of definitive repair, however more tailored selection of the patients and the long follow-up is mandatory to prove the usefulness of this approach.

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