We report a case of 46-year-old women who suffered from delayed transfusion hemolytic anemia due to anti-Jkb antibody after renal transplantation. The patient had been treated with hemodialysis and had a past history of multiple transfusion. On the second postoperative day, she received 2 units of packed red cell. During transfusion, she complained of mild chest tightness only, but 10 days later, anemia of unknown origin developed. Irregular antibody was found in her serum and identified as anti- Jkb antibody. Together with other serologic findings, she was diagnosed as delayed hemolytic transfusion reaction due to anti -Jkb antibody. We thought that this reaction might be the amnestic response to previous exposure during delivery or remote multiple transfusion. Our patient responded to steroid and plasmapheresis and recovered without severe hemolytic transfusion reaction. In conclusion, antibody screening tests and identification test might be considered as a routine pretransfusion test for all renal recipients for safe transfusion practices.
Anemia ; Anemia, Hemolytic ; Blood Group Incompatibility* ; Female ; Humans ; Kidney Transplantation ; Mass Screening ; Middle Aged ; Plasmapheresis ; Renal Dialysis ; Thorax ; Transplantation*

No abstract available.
Drug Therapy, Combination* ; Lymphoma, Non-Hodgkin*

Recent clinical trials showed that bortezomib, a novel proteasome inhibitor, had therapeutic activity in multiple myeloma. However, there was no data about the feasibility of bortezomib in Korean patients. We performed a pilot study of bortezomib in patients with relapsed or refractory myeloma (1.3 mg/m2 twice weekly for 2 week in a 3-week cycle). Seven patients were enrolled. The median age of patients was 59 yr. All patients previously received VAD (vincristine, doxorubicin and dexamethasone) and thalidomide chemotherapy. Three patients previously received alkylator-containing chemotherapy and 4 patients, autologous stem cell transplantation. Bortezomib monotherapy resulted in 3 partial remissions (43%), 3 no changes (43%) and 1 progressive disease (14%). One patient who had no response to bortezomib monotherapy experienced partial remission after addition of dexamethasone to bortezomib. The most common serious toxicity was thrombocytopenia (grade 3/4, 10 of 20 cycles (50%)) and grade 3 peripheral neuropathy was developed in 2 of 20 cycles (10%). Drug-related adverse event led to discontinuation of bortezomib in 1 patient. There was no treatment related mortality. Overall, bortezomib seems to be effective and feasible. Conduction of larger clinical studies on Korean patients is necessary to characterize clinical efficacy and safety of bortezomib more precisely.
Aged ; Antineoplastic Agents/administration & dosage/adverse effects/*therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/adverse effects/therapeutic use ; Boronic Acids/administration & dosage/adverse effects/*therapeutic use ; Dexamethasone/administration & dosage/adverse effects ; Disease Progression ; Drug Resistance, Neoplasm ; Female ; Humans ; Korea ; Male ; Middle Aged ; Multiple Myeloma/*drug therapy ; Neoplasm Recurrence, Local ; Pilot Projects ; Pyrazines/administration & dosage/adverse effects/*therapeutic use ; Research Support, Non-U.S. Gov't ; Survival Analysis ; Thrombocytopenia/chemically induced ; Time Factors

A 52-year-old male admitted the hospital because of the spontaneous pneumothorax. About twenty years ago, he had experienced the pulmonary tuberculosis but he did not medicine the antituberculosis regulary. The pleural biopsy during right lobectomy and closed thoracotomy revealed the pleural tuberculosis and we instituted the re-trial of antituberculosis medication Acute oliguric renal failure occurred after the medications for seven days. The blood smear showed the evidence of microangiopathic hemolysis. We regarded the HUS deveoloped in this patient and four times of plasma exchage was accomplished. But the renal biopsy revealed the acute tubular necrosis due to the pigment nephropathy with interstitial inflammation and the careful laboratory evaluation showed the massive hemolysis with the compensated DIC. The rifampicin dependent antibodies were identified by indirect antiglobulin test. The microangiopathic finding would be due to DIC. We regarded that the renal failure was due to the hemolysis and, in minor part, was due to interstitial nephritis. Total plasma exchage, hemodialysis and prednisolone teatment resolved the hemolysis and the renal failure in this patient.
Anemia, Hemolytic* ; Antibodies* ; Biopsy ; Coombs Test ; Dacarbazine ; Hemolysis ; Hemolytic-Uremic Syndrome* ; Humans ; Inflammation ; Male ; Middle Aged ; Necrosis* ; Nephritis, Interstitial ; Plasma ; Pneumothorax ; Prednisolone ; Renal Dialysis ; Renal Insufficiency ; Rifampin* ; Thoracotomy ; Tuberculosis, Pleural ; Tuberculosis, Pulmonary

Idiopathic nodular glomerulosclerosis is an unusual entity with histopathologic features resembling nodular diabetic glomerulosclerosis but occurs in non diabetic patients and predominantly in older males with smoking and long standing hypertension. It is characterized histologically by nodular mesangial sclerosis, glomerular basement membrane thickening, and arteriolosclerosis in the absence of immune-type deposit, and clinical presentations typically include renal insufficiency and severe proteinuria with relatively poor prognosis. We experienced a rare case of idiopathic nodular glomerulosclerosis presenting rapid progressive renal failure in a 42-year-old man without clinical evidence of diabetes. He was admitted with peripheral edema and renal insufficiency. He had a history of hypertension for 2 years and chronic hepatitis B for 5 years. Funduscopy and renal ultrasound were normal. HbA1c and oral glucose tolerance test were normal and on serum and urine electrophoresis, M-spike was not found. Light microscopic examination demonstrated the characteristic features of lobular glomerulonephritis showing glomerular hypertrophy and nodular mesangial sclerosis. Immunofluorescence was negative for immune-type deposits. Electron microscopy showed marked increase of mesangial matrix, mesangial cell proliferation, moderate effacement of foot processes, and glomerular basement thickening without electron-dense deposits or other specific fibrils. Idiopathic nodular glomerulosclerosis was diagnosed. After discharge, he continued smoking and his blood pressure was not controlled. Three months after first biopsy, second biopsy was performed because of increasement of serum creatinine and specimens showed progression of tubulointerstitial change and nodular mesangial sclerosis. Subsequent serum creatinine was progressively increased and hemodialysis was started 13 months after diagnosis.
Adult ; Arteriolosclerosis ; Biopsy ; Blood Pressure ; Creatinine ; Diabetic Nephropathies* ; Diagnosis ; Edema ; Electrophoresis ; Fluorescent Antibody Technique ; Foot ; Glomerular Basement Membrane ; Glomerulonephritis ; Glucose Tolerance Test ; Hepatitis B, Chronic ; Humans ; Hypertension ; Hypertrophy ; Male ; Mesangial Cells ; Microscopy, Electron ; Prognosis ; Proteinuria ; Renal Dialysis ; Renal Insufficiency* ; Sclerosis ; Smoke ; Smoking ; Ultrasonography

Ischemic nephropathy is defined as renal dysfunction due to renal hypoperfusion mainly through renal artery stenosis. It is a common cause of chronic renal failure in old patients with atherosclerosis and is also a potentially correctable cause of renal failure if diagnosed earlier. We experienced a case of sudden Rt. main renal artery occlusion and renal failure after femoral artery angioplasty in an aged male patient with underlying atherosclerotic bilateral renovascular stenosis associated with ischemic nephropathy. He received successful Lt. renal artery angioplasty with stenting and restored Lt. renal artery blood flow. His blood pressure was more easily controlled with fewer antihypertensive drugs after renal artery revascularization. His renal function was recovered to his baseline level and became stabilized thereafter. Early diagnosis of ischemic nephropathy with MRA and timely renal angioplasty with stenting are beneficial to avoiding progression to irreversible renal failure.
Angioplasty* ; Antihypertensive Agents ; Atherosclerosis ; Blood Pressure ; Constriction, Pathologic ; Early Diagnosis ; Femoral Artery ; Humans ; Kidney Failure, Chronic ; Male ; Renal Artery Obstruction* ; Renal Artery* ; Renal Insufficiency* ; Stents*

BACKGROUND: Since the bioavailability of itraconazole capsule is influenced by patients gastric acidity, it results in treatment failure due to its low dissolution and subsequent low absorption when administered in fasting. Itraconazole Melt-Extrusion tablet has been lately developed in order to improve its dissolution profile. It is the first clinical study to evaluate the efficacy and safety of itraconazole Melt-Extrusion tablet in Korea. OBJECTIVE: This study was conducted to evaluate the efficacy and safety of itraconazole melt-extrusion tablet 400mg daily for 1 week(pulse therapy) for hyperkeratotic type of tinea pedis and manus. METHODS: A clinical and mycological investigation was made of 812 outpatients with hyperkeratotic type of tinea pedis and/or tinea manus who had visited at 52 general hospitals under the lead of the Korean Dermatological Association from June to December, 1998. Patients confirmed by clinically and microscopically as hyperkeratotic type of tinea pedis and/or tinea manus were administered 2 tablets twice a day for one week and followed up for 8 weeks from the start of the medication. RESULTS: The results were summarized as follows; 1. Clinical symptoms of hyperkeratotic type of tinea pedis and/or tinea mauns were significantly improved at the end of study, week 8(p<0.001). 2. Clinical response rate, defined as more than 50% decrease of the sum of the clinical symptom scores, was 79.3%(512/646). 3. Mycological cure rate, dafined as both culture and KOH negative at week 8, was 78.2%(244 /312). 4. 40(5.5%) patients, of the 727 patients evaluable for drug safety evaluation, were reported to have adverse event. CONCLUSION: Itraconazole Melt-Extrusion tablet 400mg/day for 1 week (pulse therapy) is effective and safe in the treatment of hyperkeratotic type of tinea pedis and/or tinea manus.
Absorption ; Biological Availability ; Fasting ; Gastric Acid ; Hospitals, General ; Humans ; Itraconazole* ; Korea ; Outpatients ; Tablets ; Tinea Pedis* ; Tinea* ; Treatment Failure

BACKGROUND: Onychomycosis, especially toenail onychomycosis has become one of the common fungal infection and has historically been regarded as a cosmetic rather than medical problem by many patients, even by physicians. Recently, however, there are several reports that this is a refractory disease which may cause a deleterious effect on patients' quality of life (QOL). OBJECTIVE: The purpose of this study was to investigate the impact of toenail onychomycosis on QOL in Korea and to assess the changes of QOL after treatment. METHODS: Total 1004 patients with toenail onychomycosis which was confirmed by clinical findings and KOH preparation were enrolled at 47 dermatologic centers in Korea, and interviewed with standardized QOL questionnaire before and after systemic antifungal treatment. Responses to the questionnaire were scored by f-point scale (0~4) and averaged, and were analyzed for 5 dimensions of emotional impact, social impact, symptom and functional impact, patients' views concerning treatment, and relationship with doctor. RESULTS: 1. Before and after treatment, the most serious impact was emotional dimension showing 1.90 and 1.30 in average score (AS), and social (AS: 1.14 and 0.83) and symptom and functional impact (AS: 1.05 and 0.92) was also affected. 2. In female rather than male, statistically more significant impact on patients' QOL was observed in all dimensions. 3. After treatment, 3 of 5 dimensions were improved significantly - emotional dimension (AS: from 1.90 to 1.30), social dimension (AS: from 1.14 to 0.83), patients' view concerning treatment(AS: from 1.34 to 1.02) 4. The degree of patients' satisfaction at the therapeutic effect was very high - 62.4% (immediately after. treatment) and 65.8% (9 months after initiation of treatment) of patients answered excellent or good. CONCLUSION: This study confirms that toenail onychomycosis has significant Impact on the overall QOL of patients. Also the effect of antifungal therapy on patients' QOL were satisfactory. Therefore, both doctor and patient should pay more attention to the treatment of onychomycosis.
Female ; Humans ; Korea* ; Male ; Nails* ; Onychomycosis* ; Quality of Life* ; Social Change ; Surveys and Questionnaires

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires