인슐린 비의존형 당뇨병환자에서 혈관합병증과 Lipoprotein ( a ) 의 관계

김선희(Seon Hee Kim),채희복(Hee Book Chai),박중열(Joong Yeol Park),민원기(Won Ki Min),김우건(Woo Kun Kim),김기수(Ghi Su Kim),이기업(Ki Up Lee) 대한내과학회 1997 대한내과학회지 Vol.52 No.3

N/A Objectives: High serum Lp(a) concentration is associated with a high risk of coronary artery disease(CAD). This study was initiated to determine whether increased Lp(a) levels are associated with diabetic vascular complications in Korean patients with NIDDM. Methods: A total of 183 NDDM patients were studied cross-sectionally for the presence of various vascular complications. Lp(a) levels were measured by 1-step sandwich ELISA method. Results: The patients with overt proteinuria had higher Lp(a) levels than the patients with mormo- albuminuria or microalbuminuria(26.8mg/dl vs 13.8 mg/dl and 17.3mg/dl, p<0.05), The patients with proliferative retinopathy and/or those treated by photocoagulation had higher Lp(a) levels than those without retinopathy or those with background retinopathy(34.1mg/dl vs 13.3mg/dl and 16,9mg/dl, p<0.05), The Lp(a) levels were also higher in the patients with CAD than those without CAD(30.9mg/ dl vs 16.3mg/dl, p<0.05). Multiple logistic regression analysis revealed that high Lp(a) levels were independantly associated with CAD and severe diabetic retinopath3. Conclusion: High Lp(a) levels are associated with CAD and proliferative retinopathy in Korean patients with NIDDM.

비호지킨림프종의 임상상과 예후인자

김태원(Tae Won Kim),이정신(Jung Shin Lee),최성준(Sung Jun Choi),최종수(Jong Soo Choi),장대영(Dai Young Zang),이제환(Je Hwan Lee),김성배(Sung Jun Choi),김상위(Sang We Kim),서철원(Cheol Won Suh),이규형(Kyoo Hyung Lee),김우건(Woo Kun Ki 대한내과학회 1997 대한내과학회지 Vol.52 No.2

N/A Objectives: The advent of intense combination chemotherapy has transformed aggressive non-Hod-gkins lymphoma from a disease that was once uniformly fatal to one that is now often curable. Remission rates and survival may be improved by using intensive chemotherapy regimens. However, this more aggressive approach is inevitably associated with increased toxicity, and an accurate pretreament prognostic assessment of patients is required to guide the physician in selecting the most appropriate therapeutic regimen. Many studies have reported prognostic factors of non-Hodgkins lymphoma in western countries, but there are few reports on prognostic factors in Koreans and it is suggested that clinical characteristcs of non-Hodgkins lymphoma in Korea differ from those in western countries. The purpose of this study was to illustrate clinical characteristics, prognostic factors and treatment outcome in non-Hodgkins lymphoma in Korea. Methods: Clinical features of 151patients (age over 15years) with non-Hodgkins lymphoma registered at Asan Medical Center from March 1989 to December 1993 were retrospectively reviewed. Prognostic factors and treatment outcome were evaluated among 121previously untreated patients. Multi variate analysis of potential pretreatment prognostic factors was performed using Coxs proportional hazards model. Results: Of the 151patients evaluated, 55% had diffuse large cell type, while low-grades were encountered in less than 1% of the patients. Extranodal involvement was noted in 76% of the patients. Cental nervous system was the commonest primary extranodal site, followed by stomach. Complete remission was achieved in 73 of 121patients (60%). The median follow-up for 121patients was 24months and the actuarial overall survival was 48% at 3years and 44N at 5years with a median overall survival of 33months. At the median followup of 32months, the actuarial 5year disease-free survival rate among 73patient with complete remission was 65% and median remission duration was not reached. Presence of systemic B symptoms and advanced clinical stages were associated with a low complete remission rate. None turned out to be associated with the remission duration. The Coxs proportional hazards model identified age above 60years, presence of systemic B symptoms and elevated LDH level as significant independent poor prognostic factors influencing overall survival. Conclusion: This study reveals a low prevalence rate of the low-grades lymphoma and a higher propensity of diffuse large cell type. These results suggest that clinical characteristics of non-Hodgkins lymphoma in Korea are different from those in the western countries. Our data also show that certain pretreatment clinical factors can help in predicting survival and in planning treatment.

x-Light Chain Monoclonal Gammopathy에 병발된 성인형 Fanconi 증후군과 골연화증 1예

박수길,김철희,김홍규,김우건,김기수,정시정,고정민 대한내분비학회 1998 Endocrinology and metabolism Vol.13 No.1

The Fanconi syndrome is a complex tubulopathy, which is characterized by urinary hyperexcretion of amino acids of all classes, phosphate, glucose, bicarbonate, calkium, potassium, and otherions, and proteins with molecular weights under 50,000 daltons. This metabolic disease leads to hypophospatemia, hypokalemia, growth failure, metabolic acidasis, and rickets/osteomalacia. Fanconi syndrome may be inherited or acqulred. Most cases of adult Fanconi syndrome are acquired, and the acquired syndrome is associated with thermal burns, exposure to heavy metals or drugs, vitmnin D deficiency, renal transplantation, or light chain deposition. The most common cause of adult Fanconi syndrome is multiple myeloma. We describe here a case of adult Fanconi syndrome and osteomalacia associated with x-light chain monoclonal gammopathy. A 47-year-old woman presented with multiple bane pain and proximal muscle weakness for 2 years. Laboratory findmgs showed hypophosphatemia, mild hypocalcemia, marked elevation of serum alkaline phosphatase, metabolic acidosis, low 25-OH- vitamm D level and secondary hyperparathyroidism. Urinary excretion of protein, uric acid, phosphate, and glucose was mcreased, and tubular reabsorption of phosphate was decreased to 50%. Protein immunofixation electrophoresis of serum and urine showed x-light chain type monoclonal gammopathy. Bone marrow examination was normal except moderate elevation of plasma cell component(8.8%). The skeletal radiography showed fractures of both lower ribs and pseudofracture in right femoral lesser trochanter. We treated the patient with calcium, 1.25-(OH)2-vitamin D, phosphorus, bicarbonate, and potassium, and her clinical symptoms were gradually relieved. (J Kor Soc Endocrinol 13:99-107, 1998)

만성골수성백혈병에서의 동종골수이식 : 국내 경험의 후향적 분석 Retrospective Analysis of Korean Experience

이규형,이제환,이정신,김상희,김우건,김춘추,이종욱,김병국,박선양,이홍기,윤성수,김효철,남동기,한지숙,민유홍,현명수,황태주,정익주,박영석,이정애 대한조혈모세포이식학회 1998 대한조혈모세포이식학회지 Vol.3 No.2

연구방법: 국내에서 시행된 만성골수백혈병의 치료를 위한 가족간 동종골수이식의 임상적 특징을 후향적으로 분석하였다. 9개의 병원에서 1986년 9월부터 1997년 6월까지 시행한 137예의 가족간 동종골수이식을 보고하였다. 결과: 전체 환자 중 62%인 85예가 남자였고 연령은 15세에서 50세까지 분포하였다(중앙치 30세). 95예(71%)의 환자는 이식 당시 만성골수성백혈병 만성기의 상태였고 38예(28%)는 가속기 또는 모세포성발증기의 상태였다. 70예(51%)의 환자는 이식전처치로 busulfan+cyclophosphamide (BuCy) 치료를 받았고 42예(31%)의 환자는 cyclophosphamide+전신방사선 조사(Cy-TBI)를 받았다. 환자에게 이식된 단핵구의 수는 0.06-9.5×10^(8)/kg(중앙치 1.2×10^*/kg)였다. 1 예를 제외한 모든 환자에서 이식 후 8-100일(중앙치 17일)에 중성구 500/㎕ 이상의 착상이 관찰되었다. 급성 및 만성이식편대숙주질환은 각각 46%와 43%의 환자에서 관찰되었다. 중앙추적기간 20개월(범위 3-135개월) 경과 후 137예의 환자 중 52예의 환자가 사망하였다. 사망 원인은 감염(27%), 백혈병의 지속 또는 재발(23%), 이식편대숙주질환(21%), 치료독성(15%), 그리고 이식편거부(8%) 등이었다. 환자들의 event-free survival에 영향을 주는 인자로는 이식전 병의 상태(p<0.001)와 환자의 혈청 HBsAg 양성여부(p=0.012)가 중요하였다. Overall survival에 영향을 주는 인자도 이식전 병의 상태와 환자 혈청 HBsAg 양성여부로 나타났다. 결론: 본 연구에서 국내 만성골수성백혈병에 대한 동종골수이식술은 그 시행 예가 급격히 증가하고 있음을 보여주었다. 환자들의 임상 특성과 생존 성적은 외국의 성적과 유사하였다. B형 간염 감염환자의 경우 예후가 유의하게 불량하였고, 이는 추후의 임상연구에서 확인이 필요한 소견으로 사료된다. Background and Methods: Clinical features of allogeneic bone marrow transplantation (BMT) performed for patients with chronic myelocytic leukemia (CML) in Korea were analyzed retrospectively. Nine hospitals reported a total of 137 cases of allogeneic BMT performed between September, 1986 and June, 1997. Results: There were 85 male (62%) and 52 female patients with median age of 30 years (range 15-50). Ninety five patients (71%) were in chronic phase and 38 patients (28 %) were in accelerated/blastic phase of the disease. Seventy patients (51%) received busulfan-cyclophosphamide (BuCy) regimen for preparation and 42 patients (31%) received cyclophosphamide-total body irradiation (Cy-TBI) regimen. The median mononuclear cell dose given to patients was 1.2×10^(8)/kg (range, 0.06-9.5×10^(8)/kg). All but one patient achieved absolute neutrophil count of 500/μl after median 17 days after BMT (range, 8-100 days). Acute and chronic graft-versus-host disease (GVHD) were reported in 61/133 (46%) and in 57/134 (43%) patients, respectively. The median follow-up time of survivors was 20 months (range, 3-135 months). There were 52 deaths (38%). The causes of death were infection (14 patients, 27%), recurrent/persistent disease (12 patients, 23%), GVHD (11 patients, 21%), regimen related toxicity (8 patients, 15%), graft rejection/failure (4 patients, 8%), and unknown (3 patients, 6%). The factors influencing event free survival of the patients were disease status before BMT (p<0.001, by log-rank test) and serum hepatitis B surface antigen status (p=0.012). Longer duration of disease before BMT showed poorer outcome but the difference did not reach statistical significance. The factors affecting overall survival of the patients were disease status before BMT (p=0.009) and hepatitis B surface antigen status (p=0.036). The donor hepatitis B antigen status did not influence event free survival or overall survival of the patients. Conclusion: Clinical features and survival patterns of patients with CML treated with allogeneic BMT in Korea were similar to historical data from other countries. Further data analyses are warranted for the role of hepatitis B virus infection influencing the outcome of allogeneic BMT for CML.