BACKGROUND: Decay accelerating factor (DAF/CD55), regulates the complement system by accelerating decay of the C3 convertase, has been described in several malignancies, however, the clinicopathologic significance of CD55 and its receptor CD97 has not been fully investigated. We examined the expression patterns of both CD55 and CD97 and their association with clinicopathologic parameters in colorectal cancers (CRCs). METHODS: Expression patterns of CD55 and CD97 in the stroma and tumor cells at tumor center and invasive front were examined in 130 CRCs, and their significance was statistically evaluated. RESULTS: CD55-high stroma was correlated with tumor border (p=0.006) and invasion depth (p=0.013). CD55-high tumor cells at tumor center and invasive front were correlated with histologic grade, and CD55-high tumor cells at invasive front with tumor, node and metastasis (TNM) stage (p<0.05). CD97-high stroma was correlated with lymph node metastasis (p=0.016) and TNM stage (p=0.030). CD97-high tumor cells at tumor center and invasive front were correlated with tumor size and CD97-high tumor cells at tumor center with tumor border (p<0.05). Patients with CD55-high stroma showed poor overall and recurrence-free survival (p<0.05) in univariate analysis, and were independently associated with short recurrence-free survival (p=0.025) in multivariate analysis. CONCLUSIONS: Stromal CD55 overexpression would be an indicator of adverse clinical outcome and a useful prognostic factor.
Antigens, CD55 ; Calcium Hydroxide ; Colorectal Neoplasms ; Complement C3-C5 Convertases ; Complement System Proteins ; Humans ; Immunohistochemistry ; Lymph Nodes ; Neoplasm Metastasis ; Zinc Oxide

BACKGROUND: In vitro levels of complement C3 and C4 proteins are sensitive to storage conditions. To avoid in vitro complement activation when testing is delayed, serum should be frozen at -20degrees C within 2 hr of venipuncture. However, this is impractical in routine laboratory work. Therefore, we investigated alterations in C3 and C4 levels in refrigerated specimens over time and derived formulae to estimate initial levels of complement concentrations in delayed testing. METHODS: Ten fresh specimens were measured for C3 and C4 concentrations and were refrigerated at 4degrees C. We measured C3 and C4 levels in refrigerated samples daily for 4 days using an automated nephelometer (Beckman Coulter Inc., USA). RESULTS: C3 and C4 levels were significantly increased over time in refrigerated specimens (P<0.001, P<0.001, respectively). The increments in C3 and C4 levels were described by the equations: C3 (mg/dL)=3.55x+87.18 (r=0.9909), and C4 (mg/dL)=0.72x+22.3 (r=0.9395), where x=the number of days samples were refrigerated before testing. Increases in C3 and C4 concentrations were described on a percentage basis by the equations: DeltaC3 (%)=4.14x+1.07 (r=0.9903), and DeltaC4 (%)=3.57x+2.48 (r=0.9405). CONCLUSIONS: As the measured C3 and C4 concentrations increased by 3.55 mg/dL (4.1%) and 0.72 mg/dL (3.6%) per day in refrigerated specimens, the levels of C3 and C4 should be adjusted in delayed testing. We proposed that the formulae presented be used to back-calculate initial levels of C3 and C4 concentrations.
Complement Activation ; Complement C3* ; Complement C4 ; Complement System Proteins ; Phlebotomy

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.
Aminopeptidases ; Autoantibodies ; Complement C3 Nephritic Factor ; Complement Pathway, Alternative ; Complement System Proteins ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Hemolytic-Uremic Syndrome ; Humans ; Immunoglobulins ; Light ; Mass Screening ; Microscopy ; Microscopy, Electron ; Microscopy, Fluorescence ; Proteinuria

A 21-year-old male was presented with sudden headache, fever, petechiae and neck stiffness. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics. However the patient developed generalized edema, oliguria, azotemia, and heavy proteinuria in the recovery phase of illness. Low serum C3 level was also noted. A kidney biopsy was performed and showed the features of postinfectious glomerulonephritis and typical subepithelial humps on electron-microscopic examination. His symptoms and laboratory findings were improved, and C3 level returned to normal range after conservative treatment. We suggest that a complement deficiency should be ruled out in patients of glomerulonephritis developed during the recovery phase of meningococcal meningitis. C3 nephritic factor detection and renal biopsy should be carefully considered in these patients.
Anti-Bacterial Agents ; Azotemia ; Biopsy ; Cerebrospinal Fluid ; Complement C3 Nephritic Factor ; Complement System Proteins ; Diagnosis ; Edema ; Fever ; Glomerulonephritis* ; Headache ; Humans ; Kidney ; Male ; Meningitis ; Meningitis, Meningococcal* ; Neck ; Neisseria meningitidis ; Oliguria ; Proteinuria ; Purpura ; Reference Values ; Young Adult

Ten serum samples from the patient with bullous pemphigoid with the baseruent mernbrane zone autoantibody titers of 320 or greater were tested, by the method of in vitro complement immunofluorescence, for their ability to fix Factor B and properdin in addition to Clq, C4 and C3. Five samples yielded positive C3 and properdin staining reaciions while four samples demonstrated positive Factor B stainings. All ten samples yieled positive C3, C4 and Clq staining reactions, Heat inactivation or treatment of the complement source with EDTA, MG2-EGTA abolished both C3, properdin and Factor B staining in all ten cases. This result suggest that pemphigoid antibody will fix properdin and Factor B in addition to Clq, C4 and C3, a phenomenon explained by assembly of the C3b amplification mechanism following activation of the classical pathway of complement system.
Complement Factor B* ; Complement System Proteins ; Edetic Acid ; Fluorescent Antibody Technique ; Hot Temperature ; Humans ; Pemphigoid, Bullous* ; Properdin*

We report a case of hereditary angioedema in a 48-year old female patient. She experidenced facial edema and dyspnea 5 to 6 times for a year. Similar episodes developed on some members of her family, especially her sisters and father. We examined her and her sister's serum complement levels. The results showed decreased levels of C1 esterase inhibitor and C4, compared to normal levels. We treated the patient with danazol effectively.
Angioedemas, Hereditary* ; Complement C1 Inhibitor Protein ; Complement System Proteins ; Danazol ; Dyspnea ; Edema ; Fathers ; Female ; Humans ; Middle Aged ; Siblings

BACKGROUND: Complementary receptors have been suggested to play causative roles in the neuroinflammatory process of Alzheimer's disease (AD). The genetic expressions of the C3a receptor (C3aR), C5a receptor (C5aR) and the protein expressions of the C3aR and C5aR were examined in the human neuroblastoma cell line, SK-N-SH, after the administration of amyloid peptide (A1-42). METHODS: SK-N-SH cells were incubated overnight with a single dose of 20 M of aggregated A (A1-42). An inhibition study was done with actinomycin D (ActD, 2.5 M) or with the administration of cycloheximide (CHX, 2.5 M) to the cell suspension. Messenger RNA expressions of C3aR and C5aR were detected by RT-PCR. The intensity of bands from 6% polyacrylamide electrophoretic gel was analyzed by a bioimage analyzer. The protein production of C3aR and C5aR in the A-treated cells was also measured by flow cytometry. NFB activation after treatment of A in the cells was detected by an electrophoretic mobility-shift assay. RESULTS: A1-42 increased the expression of C3aR and C5aR. ActD inhibited the expression of both anaphylatoxin receptors but CHX only suppressed C5aR mRNA expression. Activated NFB was demonstrated in the A-stimulated cells. CONCLUSIONS: C3aR and C5aR were constitutively expressed in the human neuroblastoma SK-N-SH cell. Expression of these anaphylatoxin receptors was upregulated after A1-42 stimulation, which as a result, may contribute to the complement-mediated neuroinflammation of AD.
Alzheimer Disease ; Amyloid ; Amyloid beta-Peptides ; Anaphylatoxins ; Cell Line* ; Complement C3a* ; Complement System Proteins* ; Cycloheximide ; Dactinomycin ; Flow Cytometry ; Humans* ; Neuroblastoma* ; RNA, Messenger

No abstract available.
Angioedema* ; Angioedemas, Hereditary* ; Complement C1 Inhibitor Protein* ; Complement C1s*

The authors evaluated the stress response to cardiopulmonary bypass by measuring plasma Ig G, A, M and C3, C4concentrations. Anesthesia was induced with fentanyl, and all patients were underwent open heart surgery using bubble type heart-lung machine. Blood samples were obtained pre-induction, sternotomy, 10 min. and 30 min. after cardiopulmonary bypass, and 10 min. after termina tion of cardiopulmonary bypass and after transport to L.C.U. IgG, A, M and complement C3, C4concentrations were measured by Turbidmeter using Behring Turbidquant. The results were as follows: 1) Plasma concentrations of IgG, A, M and C3, C4 at preinduction period were 1345. 00+/-194. 42, 289. 10+/-100.4, 177.43+/-72.65, 59.20+/-16.33, 20.50+/-7.54 mg/dl., respectively. 2) Ten min. after cardiopulmonary bypass, the plasma concentration was significantly decreased when compared with the baseline values (p<0.01). 3) Thirty min. after cardiopulmonary bypass, the plasma concentration of the complenent C3, C4 were significantly decreascd (p<0.01). 4) In the 1ntensive care unit, Ig and complement C3, C4 were increased, but did not reach control values. Considering the above results, we conclude that the decrease of plasma Ig G, A, M and complenent C3, C4concentrations are related to mechanical stress of the cardiopulmonary bypass, and to certain activators such as denaturated protein, heterogenous RBCs, and foreign materials, etc.
Anesthesia* ; Cardiopulmonary Bypass* ; Complement C3* ; Complement C4 ; Complement System Proteins* ; Fentanyl* ; Heart-Lung Machine ; Humans ; Immunoglobulin G* ; Immunoglobulins* ; Plasma ; Sternotomy ; Stress, Mechanical ; Thoracic Surgery

BACKGROUND: Cardiopulmonary bypass induces an acute systemic inflammatory response mediated by complement activation and cytokine release. This response is likely to cause capillary leak syndrome and organ dysfunction in infants. Removing harmful cytokines and complement anaphylatoxins after cardiopulmonary bypass may attenuate this response. This study was conducted to see if the modified ultrafiltration and postoperative peritoneal dialysis can reduce plasma inflammatory mediators in pediatric cardiac surgery. MATERIAL AND METHOD: 30 infants (age 1.1 to 12.6 months) who underwent closures of ventricular septal defect using cardiopulmonary bypass (CPB) were enrolled in this study. These patients were divided into three groups; 10 patients selected randomly underwent modified ultrafiltration (Group U), 10 with small body weights (C3a and interleukin-6 (IL-6) were measured by radioimmunoassay and enzyme- linked immunosorbent assay respectively. RESULT: There was no differences in CPB time, aortic cross-clamping time, and lowest temperature during CPB. The effluents of peritoneal dialysis contained significant amount of C3a and IL-6, but there was no definitive decrease of serum concentration of C3a and IL-6. The effluents of modified ultrafiltration had some amount of C3a and negligible IL-6, and there was no decrease of serum concentration of these factors. CONCLUSION: The effluents of peritoneal dialysis contained significant amount of proinflammatory cytokine, IL-6 and complement, C3a. However this study failed to elucidate the decrease in serum levels of these factors. The modified ultrafiltration also was not able to reduce the serum levels of C3a or IL-6 in our study as well.
Anaphylatoxins ; Body Weight ; Capillary Leak Syndrome ; Cardiopulmonary Bypass ; Complement Activation ; Complement C3a ; Complement System Proteins ; Cytokines ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Interleukin-6 ; Peritoneal Dialysis* ; Plasma ; Radioimmunoassay ; Thoracic Surgery* ; Ultrafiltration*