The acute effects of variable dos rates to total body irradiation (TBI) were investigated with 600 cgy of single exposure in the mice as a preclinical model. Total 80 mice (ICR) were used. Twenty of which served as controls, receiving no irradiation. All irradiated mice showed a universal decline in their weight and white blood cell count. The degree of weight loss and leukopenia were similar at 3 different dos rate but slightly prominent with 15 cgy/minute group. The degree of recovery among the groups showed no dose rate dependence. Our results suggest that TBI with 15 cgy/minute may be applicable for clinical therapy with careful evaluation of patient's condition.
Animals ; Leukocyte Count ; Leukopenia ; Mice* ; Weight Loss ; Whole-Body Irradiation*

Multiple epiphyseal dysplasia (MED) is a clinically and genetically heterogeneous chondroplasia, characterized by delayed development of the ossification centers and, deformities of the extremities that involve only the epiphysis and result in mild short stature. Mutations in the cartilage oligomeric matrix protein (COMP) gene are most commonly found, and most of the mutations are located in the calmodulin-like repeats and the C-terminal domain. We report a Korean kindred of?12 family members with MED in four generations who were found to have a novel mutation in the COMP gene. A pedigree showed early onset osteoarthritis requiring arthroplasty that was an autosomal dominant inherited trait. Radiological examinations demonstrated the presence of osteochondral defects in the medial femoral condyles, and the knee and hip joints showed variable degrees of precocious degenerative changes. Mutation analysis of the COMP gene in the proband and five other affected family members identified a novel missense mutation, c.1280G>C (p.Gly427Ala) in exon 12, which was not found in three unaffected family members. Direct sequencing of the COMP gene may yield pathogenic mutations in dominantly inherited MED cases, and may provide opportunities of carrier detection among high-risk family members, leading to genetic counseling for early diagnosis and intervention before the onset of complications.
Achondroplasia ; Arthroplasty ; Cartilage ; Congenital Abnormalities ; Early Diagnosis ; Epiphyses ; Exons ; Extracellular Matrix Proteins ; Extremities ; Family Characteristics ; Genetic Counseling ; Glycoproteins ; Hip Joint ; Humans ; Knee ; Mutation, Missense ; Osteoarthritis ; Osteochondrodysplasias ; Pedigree

This in vitro study examined the effect of surface defects on cutting blades on the extent of the cyclic fatigue fracture of HEROShaper Ni-Ti rotary files using fractographic analysis of the fractured surfaces. A total of 45 HEROShaper (MicroMega) Ni-Ti rotary files with a #30/.04 taper were divided into three groups of 15 each. Group 1 contained new HEROShapers without any surface defects. Group 2 contained HEROShapers with manufacturing defects such as metal rollover and machining marks. Group 3 contained HEROShapers that had been clinically used for the canal preparation of 4-6 molars. A fatigue-testing device was designed to allow cyclic tension and compressive stress on the tip of the instrument whilst maintaining similar conditions to those experienced in a clinic. The level of fatigue fracture time was measured using a computer connected the system. Statistical analysis was performed using a Tukey's test. Scanning electron microscopy (SEM) was used for fractographic analysis of the fractured surfaces. The fatigue fracture time between groups 1 and 2, and between groups 1 and 3 was significantly different (p < 0.05) but there was no significant difference between groups 2 and 3 (p > 0.05). A low magnification SEM views show brittle fracture as the main initial failure mode. At higher magnification, the brittle fracture region showed clusters of fatigue striations and a large number of secondary cracks. These fractures typically led to a central region of catastrophic ductile failure. Qualitatively, the ductile fracture region was characterized by the formation of microvoids and dimpling. The fractured surfaces of the HEROShapers in groups 2 and 3 were always associated with pre-existing surface defects. Typically, the fractured surface in the brittle fracture region showed evidence of cleavage (transgranular) facets across the grains, as well as intergranular facets along the grain boundaries. These results show that surface defects on cutting blades of Ni-Ti rotary files might be the preferred sites for the origin of fatigue fracture under experimental conditions. Furthermore, this work demonstrates the utility of fractography in evaluating the failure of Ni-Ti rotary files.
Edible Grain ; Fatigue* ; Fractures, Stress* ; Microscopy, Electron, Scanning ; Molar

Sjogren's syndrome (SS) is an autoimmune disorder in which lymphocytes infiltrate the exocrine glands, resulting in the development of sicca symptoms. Lymphocytes may also invade various other organs and cause diverse symptoms. Interstitial pneumonia has been observed frequently in SS patients. Typically, the pneumonia responds well to systemic steroids, and fatal cases are rare. We experienced a case of lymphocytic pneumonia accompanied by SS and treated with cyclophosphamide pulse therapy, and we present details of the case herein.
Adult ; Humans ; Lung/*pathology ; Lung Diseases, Interstitial/drug therapy/*pathology ; Lymphocytes/*pathology ; Male ; Plasma Cells/pathology ; Sjogren's Syndrome/*pathology

BACKGROUND: The relationship between bile duct proliferation and portal fibrosis in obstructive liver diseases remains unclear. The purpose of this study is to analyze the relationship between hepatic stellate cells (HSC), hepatocytes and bile ductule proliferation in obstructive liver disease using immunoreactivity for alpha-SMA (alpha-smooth muscle actin), CK7, and CK19. METHODS: We used 20 human tissue samples with hepatic fibrosis due to intrahepatic stones and liver cirrhosis. Immunohistochemical staining was performed using the streptavidin-biotin method. RESULTS: Proliferations of bile ductules at the periphery of the hepatic lobules, and diffuse HSC activation in the perisinusoidal spaces were observed in all cases. Immunoreactivity of the hepatocytes for CK7 and CK19 suggested a possible phenotypic transformation into bile duct epithelium during fibrogenesis. Immunohistochemical-analyses of alpha-SMA expression profiles showed that intralobular HSCs and some hepatocytes underwent early phenotypic changes, and that the accumulation of collagen coincides with that of alpha-SMA-labeled myofibroblasts around portal/septal ductular structures. CONCLUSIONS: Our results showed the possibility of a phenotypic transformation of hepatocytes into bile ductular epithelium. It is suggested that hepatocytes might play a role in bile ductule proliferation in obstructive liver disease.
Bile ; Bile Ducts ; Collagen ; Epithelium ; Fibrosis ; Hepatic Stellate Cells ; Hepatocytes ; Humans ; Liver ; Liver Cirrhosis ; Liver Diseases ; Muscles ; Myofibroblasts

BACKGROUND: If acute renal failure develops in patients with acute pyelonephritis, it is most commonly due to hypovolemia, sepsis, drug therapy or urinary obstruction. But there have been reported many cases about patients of acute renal failure derived from acute pyelonephritis itself without any predisposing factor. To find out the predisposing related factors, we analysed the clinical patterns of patients of acute pyelonephritis with acute renal failure compared to that of patients of acute pyelonephritis without acute renal failure. METHODS: From January 1996 to December 2000, the authors identified 172 patients older than 16 years of age who admitted to Korea University Hospital for acute pyelonephritis. Among them, patients whose serum creatinine level had been less than 1.5mg/dL before admission and who did not have any of chronic renal failure, diabetic nephropathy or hypertensive nephropathy were recruited. According to the level of serum creatinine at admission, the patients were divided into two groups. If ones serum creatinine level at admission was less than 1.5mg/dL, he or she included to control group, if more than 1.5 mg/dL to acute renal failure group. And we compared their clinical features and laboratory values. RESULTS: The patients with acute pyelonephritis complicated to renal failure showed the tendency of older age, more dehydration, more inflammation, more frequent abnormal findings in abdominal ultrasonography and more frequency of chronic systemic disease than those without renal failure. Acute renal failure due to acute pyelonephritis might be recovered rapidly by general supportive care like fluid replacement and adequate antimicrobial therapy. CONCLUSION: Acute pyelonephritis should be considered rare cause of acute renal failure. Especially in case of old age, severe dehydration, severe inflammation and comorbidity with chronic systemic disease involved in kidney, it would be helpful to observe the clinical course closely.
Acute Kidney Injury* ; Causality ; Comorbidity ; Creatinine ; Dehydration ; Diabetic Nephropathies ; Drug Therapy ; Humans ; Hypovolemia ; Inflammation ; Kidney ; Kidney Failure, Chronic ; Korea ; Pyelonephritis* ; Renal Insufficiency ; Sepsis ; Ultrasonography

BACKGROUND: Glomerular diseases of diverse origins are characterized by heavy proteinuria and tubulointerstitial changes in pathology. Numerous studies have recently demonstrated that interstitial fibrosis and tubular atrophy are better predictors of renal disease progression compared with glomerular pathology. One of the important mechanisms of these tubulointerstitial injury is tubulointerstitial damage due to increased protein trafficking across the proximal tubular epithelial cells. We tested the hypothesis that tubular cells exposed to high concentration of protein express TGF-beta, which can be related to tubulointerstitial fibrosis, and Fas antigen, which can be associated with tubular cell apoptosis. METHODS: Cultured human proximal tubular cells were incubated with varying concentrations of BSA (1, 10 mg/mL) and nephrotic range proteinuria, due to diabetic nephropathy (1, 10 mg/mL), with or without inactivation of complement. After 24 hr-incubation period, the expressions of TGF-beta and Fas mRNA were examined by RT-PCR. RESULTS: The amount of expression of TGF-beta was increased in BSA 10 mg/mL group (0.78+/-0.12, p=0.016) and in diabetic proteinuria 10 mg/mL group (0.7+/-0.08, p=0.012) compared to control group which was incubated in medium alone (0.48+/-0.02), and the amount of expression of Fas was increased in BSA 10 mg/mL group (0.97+/-0.09, p=0.021) and showed increased tendency in diabetic proteinuria 10 mg/mL group (0.94+/-0.14, p=0.067) also. Furthermore, the anti TGF-beta antibody ameliorated the increased albumin-induced expression of Fas. CONCLUSION: Collectively, our results showed that protein overload increased the expression of TGF-beta & Fas, which can play an important role in tubulointerstitial atrophy by inducing apoptosis of renal tubular cells.
Antigens, CD95 ; Apoptosis ; Atrophy ; Complement System Proteins ; Diabetic Nephropathies ; Disease Progression ; Epithelial Cells ; Fibrosis ; Humans* ; Pathology ; Protein Transport ; Proteinuria* ; RNA, Messenger ; Transforming Growth Factor beta*

BACKGROUND: Glomerular diseases of diverse origins are characterized by heavy proteinuria and tubulointerstitial changes in pathology. Numerous studies have recently demonstrated that interstitial fibrosis and tubular atrophy are better predictors of renal disease progression compared with glomerular pathology. One of the important mechanisms of these tubulointerstitial injury is tubulointerstitial damage due to increased protein trafficking across the proximal tubular epithelial cells. We tested the hypothesis that tubular cells exposed to high concentration of protein express TGF-beta, which can be related to tubulointerstitial fibrosis, and Fas antigen, which can be associated with tubular cell apoptosis. METHODS: Cultured human proximal tubular cells were incubated with varying concentrations of BSA (1, 10 mg/mL) and nephrotic range proteinuria, due to diabetic nephropathy (1, 10 mg/mL), with or without inactivation of complement. After 24 hr-incubation period, the expressions of TGF-beta and Fas mRNA were examined by RT-PCR. RESULTS: The amount of expression of TGF-beta was increased in BSA 10 mg/mL group (0.78+/-0.12, p=0.016) and in diabetic proteinuria 10 mg/mL group (0.7+/-0.08, p=0.012) compared to control group which was incubated in medium alone (0.48+/-0.02), and the amount of expression of Fas was increased in BSA 10 mg/mL group (0.97+/-0.09, p=0.021) and showed increased tendency in diabetic proteinuria 10 mg/mL group (0.94+/-0.14, p=0.067) also. Furthermore, the anti TGF-beta antibody ameliorated the increased albumin-induced expression of Fas. CONCLUSION: Collectively, our results showed that protein overload increased the expression of TGF-beta & Fas, which can play an important role in tubulointerstitial atrophy by inducing apoptosis of renal tubular cells.
Antigens, CD95 ; Apoptosis ; Atrophy ; Complement System Proteins ; Diabetic Nephropathies ; Disease Progression ; Epithelial Cells ; Fibrosis ; Humans* ; Pathology ; Protein Transport ; Proteinuria* ; RNA, Messenger ; Transforming Growth Factor beta*

Intramural duodenal hematoma is a rare finding in the adult, especially when related to iatrogenic complications of ulcer treatment, it can lead to biliary obstruction and pancreatitis, which can be fatal in severe case. We report one case of intramural duodenal hematoma complicated with pancreatitis after endoscopic hemostasis in a chronic renal failure patient with maintenance hemodialysis. He had a duodenal ulcer bleeding treated with endoscopic epinephrine injection and electro-coagulation therapy, but on the second day, he complained of persistent abdominal pain, nausea and vomiting. Abdominal ultrasound showed acute, edematous pancreatitis and a mass with low echodensity in the wall of the 2nd portion of the duodenum. Symptom and laboratory findings were persistent under conservative therapy, 7 days later, gastric resection, hematoma evacuation was carried out, subsequently the patient recovered from the pancreatitis but the patient died of septic shock and multiple organ dysfunction.
Abdominal Pain ; Adult ; Duodenal Ulcer ; Duodenum ; Epinephrine ; Hematoma* ; Hemorrhage ; Hemostasis, Endoscopic* ; Humans ; Kidney Failure, Chronic* ; Nausea ; Pancreatitis* ; Renal Dialysis* ; Shock, Septic ; Ulcer ; Ultrasonography ; Vomiting

BACKGROUND: Blood-dialyzer membrane interaction in hemodialysis has the potential to activate blood coagulation and fibrinolysis, and it might elicit production of inflammatory cytokine such as TNF-alpha by monocytes activation. The aim of the present study was to; i) assess changes in coagulation status, fibrinolytic activity and plasma level of TNF-alpha during hemodialysis; ii) determine whether the extent of activation is dependent on the dialyzer material used. METHODS: Twenty-five end-stage renal failure patients who had undergone maintenance hemodialysis were included in the study. Patients were randomly divided into two groups; one using hemophan dialyzer membrane (n=13) and the other using polysulfone dialyzer membrane (n=12). On sixth dialysis session, blood samples were obtained before and at the end of hemodialysis. Thrombin-antithrombin complex (TAT) and D-dimer, each reflecting in vivo thrombin generation and fibrin degradation product respectively, were measured for coagulatory and fibrinolytic activity. Tissue plasminogen activator (tPA), plasminogen activator inhibitor-1(PAI-1), and fibrinogen were measured. Activated partial thromboplastin time (aPTT) was measured for efficancy of anticoagulant. Plasma level of TNF-alpha was also measured. RESULTS: During hemodialysis, plasma level of TNF-alpha did not change. Between hemophan dialysis and polysulfone dialysis group, the change in plasma level of TNF-alpha (delta TNF-alpha ) was not different. Significant changes were observed in aPTT, fibrinogen, TAT, D-dimer, tPA during hemodialysis (p<0.05) except in PAI-1 (p=0.71). Between two groups, changes in aPTT, fibrinogen, D-dimer, PAI-1 and tPA (delta aPTT, delta fibrinogen, delta D-dimer, delta PAI-1, delta tPA) were not different (p>0.05). However, the change in TAT (delta TAT) was significantly lower in polysulfone dialysis group (p=0.049). CONCLUSION: Hemodialysis enhances coagulatory activity despite the use of anticoagulant and also enhances fibrinolytic activity, which is likely the result of tPA release. In activation of coagulatory system, biocompatibility of polysulfone membrane is superior to that of hemophan membrane. Plasma level of TNF-alpha did not change during hemodialysis, further study should be considered.
Blood Coagulation* ; Dialysis ; Fibrin ; Fibrinogen ; Fibrinolysis ; Humans ; Kidney Failure, Chronic ; Membranes* ; Monocytes ; Partial Thromboplastin Time ; Plasma ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators ; Renal Dialysis ; Thrombin ; Tissue Plasminogen Activator ; Tumor Necrosis Factor-alpha*