PURPOSE: The main limitation of the National Institutes of Health (NIH) consensus criteria is the biological and prognostic heterogeneity of tumors while the utility of the newly proposed UICC TNM staging system has not yet been validated. Our object is to compare the overall and disease-free survival (DFS) of patients with gastric gastrointestinal stromal tumors (GIST) using the UICC TNM staging system and the NIH consensus criteria and to determine the optimal risk stratification system for GIST.METHODS: Retrospective analysis of 164 patients who underwent operative management for primary gastric GIST between 1994 and 2004.RESULTS: The overall five-year survival rate was 89.6%. In the 149 patients who underwent an R0 resection, the five-year DFS rate was 86.3%. According to the NIH consensus criteria, the five-year survival rate after resection was 100% for patients in the very low-, low-, and intermediate-risk groups and 71.9% for patients in the high-risk group. The UICC TNM stages IA, IB, and II had the same survival rates (100%) and stage IIIA (75.0%) and stage IIIB (69.5%) showed no significant difference in survival compared to the NIH criteria, which precluded better risk stratification. The patients who were included in the high-risk group by NIH consensus criteria (>10 cm, ≤5/50 high power field) and were stage II by the UICC TNM staging system had a 100% five-year survival rate.CONCLUSION: Using the current schemes, one system does not demonstrate superior prognostic ability over the other. However, the high-risk group (T4/low mitotic rate) and stage II patients appear to be appropriately classified.
Classification ; Consensus ; Disease-Free Survival ; Gastrointestinal Stromal Tumors ; Humans ; National Institutes of Health (U.S.) ; Neoplasm Staging ; Population Characteristics ; Retrospective Studies ; Survival Rate

PURPOSE: The effect of different peritoneal dialysis (PD) modalities on the decline in residual renal function (RRF) is unclear due to inconsistencies among studies. In particular, the effect of automated peritoneal dialysis (APD) modalities [continuous cyclic peritoneal dialysis (CCPD) and nightly intermittent peritoneal dialysis (NIPD)] on RRF has not been examined in a large cohort. MATERIALS AND METHODS: We conducted a single-center retrospective study to investigate the association between PD modalities and decline in RRF in 142 incident PD patients [34 on CCPD, 36 on NIPD, and 72 on continuous ambulatory peritoneal dialysis (CAPD)]. RRF was measured within 2 months from PD start and at 1 year after PD initiation. RESULTS: The RRF at 1 year after PD initiation was 1.98+/-2.20 mL/min/1.73 m2 in CCPD patients and 3.63+/-3.67 mL/min/1.73 m2 in NIPD patients, which were moderately lower than 4.23+/-3.51 mL/min/1.73 m2 in CAPD patients (p=0.064). Moreover, there was no significant difference in the 1-year rate of decline of RRF between CCPD and NIPD patients, although APD patients had a faster 1-year RRF decline rate than CAPD patients (CCPD and NIPD vs. CAPD: -45.68 and -36.69 vs. 1.17%/year, p=0.045). APD was associated with a more rapid decline in RRF in patients with end-stage renal disease undergoing PD, although multivariate analysis attenuated the significance of this finding (beta=-31.50; 95% CI, -63.61 to 0.62; p=0.052). CONCLUSION: Our results suggest that CAPD might be more helpful than APD for preserving RRF during the first year of dialysis therapy, although there was no significant difference in the 1-year rate of decline of RRF between the two APD modalities.
Adult ; Female ; Glomerular Filtration Rate/physiology ; Humans ; Kidney/pathology/physiopathology ; Kidney Failure, Chronic/*therapy ; Male ; Middle Aged ; Peritoneal Dialysis/*adverse effects ; Retrospective Studies

A 74-year-old woman presented with edema in the lower extremities. Laboratory tests revealed anemia, thrombocytopenia, hypoalbuminemia, hypercholesterolemia, and nephrotic-range proteinuria. Myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia (MDS-RCMD) was confirmed by bone marrow biopsy. Renal biopsy demonstrated membranous glomerulonephritis (MGN), stage I. Based on these clinicopathologic results, she was diagnosed as having MGN with MDS-RCMD. This is a rare case report of MGN in a parient with MDS-RCMD featuring nephrotic syndrome.
Aged ; Anemia ; Biopsy ; Bone Marrow ; Edema ; Female ; Glomerulonephritis, Membranous* ; Humans ; Hypercholesterolemia ; Hypoalbuminemia ; Lower Extremity ; Myelodysplastic Syndromes ; Nephrotic Syndrome ; Proteinuria ; Thrombocytopenia

PURPOSE: We undertook an observational study to investigate the effects of immunosuppressive treatment on proteinuria and renal function in 179 Korean idiopathic membranous nephropathy patients with nephrotic syndrome. MATERIALS AND METHODS: The primary outcome was regarded as the first appearance of remission and the secondary outcomes as a decline in estimated glomerular filtration rate (eGFR) >50% or initiation of dialysis, and all-cause mortality. Seventy-two (40.2%) and 50 (27.9%) patients were treated with corticosteroids alone (C) and corticosteroids plus cyclosporine (C+C), respectively, whereas 57 (31.8%) did not receive immunosuppressants (NTx). Cyclosporine was added if there was no reduction in proteinuria of >50% from baseline by corticosteroids alone within 3 months. RESULTS: There were no differences in baseline renal function and the amount of proteinuria among the three groups. Overall, complete remission (CR) was achieved in 88 (72.1%) patients by immunosuppressants. In a multivariate analysis adjusted for covariates associated with adverse renal outcome, the probability of reaching CR was significantly higher in the C [hazard ratio (HR), 4.09; p<0.001] and C+C groups (HR, 2.57; p=0.003) than in the NTx group. Kaplan-Meier analysis revealed that 5-year CR rates of C, C+C, and NTx groups were 88.5%, 86.2%, and 56.7% (p<0.001). Ten-year event-free rates for the secondary endpoints in these three groups were 91.7%, 79.9%, and 57.2% (p=0.01). CONCLUSION: Immunosuppressive treatment was effective in inducing remission and preserving renal function in these patients. Therefore, stepwise treatment using corticosteroids alone and in combination with cyclosporine is warranted in these patients.
Adrenal Cortex Hormones/adverse effects/*therapeutic use ; Adult ; Aged ; Cyclosporine/adverse effects/*therapeutic use ; Drug Administration Schedule ; Female ; Glomerular Filtration Rate/drug effects ; Glomerulonephritis, Membranous/*drug therapy/mortality ; Humans ; Immunosuppressive Agents/adverse effects/*therapeutic use ; Kaplan-Meier Estimate ; Kidney/drug effects/physiology ; Male ; Middle Aged ; Proteinuria/chemically induced ; Treatment Outcome

Exclusively dopamine producing retroperitoneal paragangliomas are extremely rare. We have experienced the first Korean case managed successfully based on the proper evaluation. A 26-year-old female patient came to our attention after the accidental detection of an adrenal mass. She had no symptoms and denied any family history. Laboratory evaluations were normal but serum dopamine (425 ng/L) and 24-hour urine dopamine levels (1,565.3 microg/day) were elevated. She underwent laparoscopic right adrenalectomy. Histopathological diagnosis was a paraganglioma. After operation, dopamine levels in serum and 24-hour urine dropped to 0.09 ng/L and 388.4 microg/day. Dopamine producing paraganglioma elicit no clinical symptoms. Only the dopamine level is elevated in serum and 24-hour urine samples. Surgical resection without using preoperative alpha blockage is the treatment of choice. The prognosis for patients with this tumor tends to be poor because the diagnosis is usually delayed due to lack of symptoms.
Adrenal Glands ; Adrenalectomy ; Adrenergic alpha-Antagonists ; Adult ; Dopamine ; Female ; Humans ; Korea ; Paraganglioma ; Pheochromocytoma ; Porphyrins ; Prognosis

The balance between tissue-type plasminogen activator (t-PA) and plasminogen activator inhibitor type 1 (PAI-1) regulates fibrinolysis. PAI-1 expression increases in atherosclerotic arteries and vascular smooth muscle cells (VSMCs) are one of major constituents of atheroma. We investigated the impact of lysophosphatidylcholine (lysoPC), an active component of oxidized low-density lipoprotein, on the plasminogen activator system of the rat VSMCs. The lysoPC stimulated the protein and gene expressions of PAI-1 but did not affect the protein expression of t-PA. Fibrin overlay zymography revealed that lysoPC increased the activity of PAI-1 in the conditioned media, while concurrently decreasing that of free t-PA. Vitamin E inhibited the lysoPC-induced PAI-1 expression. Further, lysoPC increased the intracellular reactive oxygen species (ROS) formation. Caffeic acid phenethyl ester, an inhibitor of NF-kappaB, blocked this lysoPC effect. Indeed, lysoPC induced the NF-kappaB-mediated transcriptional activity as measured by luciferase reporter assay. In addition, genistein, an inhibitor of protein-tyrosine kinase (PTK), diminished the lysoPC effect, while 7,12-dimethylbenz[a]anthracene, a stimulator of PTK, stimulated PAI-1 production. In conclusion, lysoPC does not affect t-PA expression but induces PAI-1 expression in the VSMC by mediating NF-kappaB and the genistein-sensitive PTK signaling pathways via oxidative stress. Importantly, lysoPC stimulates the enzyme activity of PAI-1 and suppresses that of t-PA.
Animals ; Benz(a)Anthracenes/pharmacology ; Caffeic Acids/pharmacology ; Cells, Cultured ; Genistein/pharmacology ; Lipoproteins, LDL/metabolism ; Lysophosphatidylcholines/*pharmacology ; Muscle, Smooth, Vascular/cytology/*drug effects/metabolism ; NF-kappa B/antagonists & inhibitors/metabolism ; Oxidative Stress/drug effects ; Phenylethyl Alcohol/analogs & derivatives/pharmacology ; Plasminogen Activator Inhibitor 1/agonists/genetics/*metabolism ; Protein Kinase Inhibitors/pharmacology ; Protein-Tyrosine Kinases/antagonists & inhibitors/metabolism ; Rats ; Rats, Sprague-Dawley ; Reactive Oxygen Species/metabolism ; Signal Transduction/drug effects ; Tissue Plasminogen Activator/*metabolism ; Transcription, Genetic/drug effects ; Up-Regulation/drug effects ; Vitamin E/pharmacology

Calcifying aponeurotic fibroma is a rare soft tissue tumor that mostly occurs in the distal extremities of children and adolescents. We report here on a case of calcifying aponeurotic fibroma of the right elbow in an 8-year-old boy, and the tumor was diagnosed by surgical excision. The patient complained of painless swelling and mild limitation of the range of motion of the elbow joint. Radiologically, the mass was ill-defined and showed stippled calcification with shallow bony erosion. Microscopically, the tumor was composed of spindle cells with nodular deposits of hyalination and calcification, and these deposits were surrounded by palisading polygonal plump cells. Immunohistochemically, the tumor showed a diffuse positive expression for CD99 and negativity for smooth muscle actin, S-100 protein and CD34. The patient has been well with no signs of recurrence during the 42 months after surgery.
Actins ; Adolescent ; Child ; Elbow ; Elbow Joint ; Extremities ; Fibroma ; Humans ; Hyalin ; Muscle, Smooth ; Range of Motion, Articular ; Recurrence ; S100 Proteins ; Soft Tissue Neoplasms

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

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

This study aimed to investigate the existence of GABA receptor and the mechanisms of action of GABA and diazepam of the trachealis muscle isolated from dog. Horizontal muscle strips of 2mm×15mm were prepared from canine trachea, and isometric myography in isolated muscle chamber bubbled with 95/5%-O₂/CO₂ at 36℃, at the pH of 7.4 was performed. Muscle strips contracted responding to the electrical field stimulation (ESP) by 2~20 Hz, 20 msec, monophasic square wave of 60 VDC. GABA and diazepam suppressed the EFS-induced contractions to the similar extent, significantly. (p<0.05). Bicuculline, a GABA(A) receptor antagonist blocked both GABA- and diazepam-inhibitions; but DAVA, a GABA(B) receptor antagoinst did not affect either of them. These results suggest than in the canine trachealis muscle, there may be only GABA(A) receptor, and GABA and diazepam inhibit the contractility via GABA(A) receptor.
Animals ; Bicuculline ; Diazepam ; Dogs ; gamma-Aminobutyric Acid* ; Hydrogen-Ion Concentration ; Myography ; Receptors, GABA ; Receptors, GABA-A ; Trachea