Primary sarcomas of the liver are rare. A case of primary malignant fibrous histiocytoma of the liver is reported. A 55-year-old male was admitted with epigastric pain. An abdominal computed tomographic scan disclosed a 10cm, low-density area in the left lobe of the liver. Histological examination of the resected tumor showed bundles of spindle cells arranged in a storiform pattern. In some areas, many bizarre giant cells were scattered. Immunohistochemically, tumor cells were positive for alpha 1-antitrypsin and alpha 1-antichymotrypsin, and weakly positive for vimentin. The tumor cells did not express cytokeratin, desmin or alpha fetoprotein.
alpha 1-Antichymotrypsin ; alpha 1-Antitrypsin ; alpha-Fetoproteins ; Desmin ; Giant Cells ; Histiocytoma, Malignant Fibrous* ; Humans ; Keratins ; Liver* ; Male ; Middle Aged ; Sarcoma ; Vimentin

Alpha-1 antitrypsin (AAT), an alpha globulin glycoprotein, is a member of the serine protease inhibitor (serpin) superfamily. The clinical significance of AAT is highlighted by AAT deficiency. Genetic deficiency of AAT can present as several neutrophilic diseases associated with emphysema, liver cirrhosis, panniculitis, and systemic vasculitis. Recently, animal and human studies have shown that AAT can control inflammatory, immunological, and tissue-protective responses. In addition, AAT treatment can prevent overt hyperglycemia, increase insulin secretion, and reduce cytokine-mediated apoptosis of pancreatic β-cells in diabetes. These multifunctional roles of AAT draw attention to the glycoprotein's therapeutic potential for many inflammatory and autoimmune diseases beyond AAT deficiency. As underlying mechanisms, recent studies have suggested the importance of serine protease inhibitory activity of AAT in obesity-associated insulin resistance, chronic obstructive pulmonary disease, and cystic fibrosis. In this review, we explore the multiple functions of AAT, in particular, the anti-inflammatory and serine protease inhibitory functions, and AAT's therapeutic potential in a variety of human diseases through published literature.
alpha 1-Antitrypsin ; Alpha-Globulins ; Animals ; Apoptosis ; Autoimmune Diseases ; Cystic Fibrosis ; Diabetes Mellitus ; Emphysema ; Glycoproteins ; Humans* ; Hyperglycemia ; Insulin ; Insulin Resistance ; Liver Cirrhosis ; Neutrophils ; Panniculitis ; Pulmonary Disease, Chronic Obstructive ; Serine Proteases ; Systemic Vasculitis ; Therapeutic Uses

OBJECTIVETo observe and compare the effects of Hanfangji Compound and IFN-gamma on expressions of transthyretin (TTR) , inter-alpha inhibitor H1 (ITIH1) and serpin peptidase inhibitor clade F member 2 (SERPINF2) of hepatic stellate cells (HSC-T6).

METHODHanfangji Compound and IFN-gammaof different concentrations were used in hepatic stellate cell-T6 (HSC-T6) for 48 h. Flow cytometer was used to detect the effects of Hanfangji Compound and IFN-gamma on HSC proliferation. RT-PCR method was adopted to detect mRNA expressions of TFR, ITIH1 and SERPINF2. TTR, ITIH1 and SERPINF2 secretions were detected by ELISA. The protein localizations of TTR, ITIH1 and SERPINF2 were examined by immune fluorescence. The protein expression of TfR and ITIHI were determined by Western blot.

RESULTAfter Hanfangji Compound and IFN-gamma were adopted in HSC-T6, compared with the control group, the cell proliferation was inhibited obviously (P < 0. 05) , protein expressions of TTR, ITIH1 and SERPINF2 and mRNA expression increased significantly, with certain correlation with concentrations of Hanfangji Compound. The 2. 5 g L-I Hanfangji Compound group was superior to the IFN-gamma group (P <0. 05).

CONCLUSIONHanfangji Compound can inhibit HSC proliferation, upregulated TTR, ITIH1 and SERPINF2 proteins and mRNA expression, which may be one of mechanisms of anti-hepatic fibrosis of Hanfangji Compound.

Alpha-Globulins ; genetics ; metabolism ; Blotting, Western ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Drugs, Chinese Herbal ; pharmacology ; Enzyme-Linked Immunosorbent Assay ; Hepatic Stellate Cells ; cytology ; drug effects ; metabolism ; Humans ; Receptors, Albumin ; genetics ; metabolism ; alpha-2-Antiplasmin ; genetics ; metabolism

PURPOSE: Urosepsis in adults comprises approximately 25% of all sepsis cases, and is due to complicated urinary tract infections in most cases. However, its mechanism is not fully clarified. Urosepsis is a very complicated disease with no effective strategy for early diagnosis and treatment. This study aimed to identify possible target-related proteins involved in urosepsis using proteomics and establish possible networks using bioinformatics. METHODS: Fifty patients admitted to the Urology Unit of Lanzhou General PLA (Lanzhou, China), from October 2012 to October 2015, were enrolled in this study. The patients were further divided into shock and matched-pair non-shock groups. 2-DE technique, mass spectrometry and database search were used to detect differentially expressed proteins in serum from the two groups. RESULTS: Six proteins were found at higher levels in the shock group compared with non-shock individuals, including serum amyloid A-1 protein (SAA1), apolipoprotein L1 (APOL1), ceruloplasmin (CP), haptoglobin (HP), antithrombin-III (SERPINC1) and prothrombin (F2), while three proteins showed lower levels, including serotransferrin (TF), transthyretin (TTR) and alpha-2-macroglobulin (A2M). CONCLUSION Nine proteins were differentially expressed between uroseptic patients (non-shock groups) and severe uroseptic patients (shock groups), compared with non-shock groups, serum SAA1, APOL1,CP, HP, SERPINC1and F2 at higher levels, while TF, TTR and A2M at lower levels in shock groups.these proteins were mainly involved in platelet activation, signaling and aggregation, acute phase protein pathway, lipid homeostasis, and iron ion transport, deserve further research as potential candidates for early diagnosis and treatment. (The conclusion seems too simple and vague, please re-write it. You may focus at what proteins have been expressed and introduce more detail about its significance.).
Adult ; Aged ; Antithrombin III ; Apolipoprotein L1 ; blood ; Ceruloplasmin ; Female ; Haptoglobins ; Humans ; Male ; Middle Aged ; Prealbumin ; Pregnancy-Associated alpha 2-Macroglobulins ; Proteomics ; Prothrombin ; Sepsis ; blood ; diagnosis ; etiology ; genetics ; Serum Amyloid A Protein ; Transferrin ; Urinary Tract Infections ; complications

The exact pathomechanism of anti-epidermal cell pemphigus antibodies in developing acantholytic changes is unknown. Recent investigations have suggested that pemphigus antibodies, after binding to the antigenic site, induce activation of epidermal plasminogen activator. This increased activity of the plasminogen activator converts plasminogen to plasmin in high level degrades intercellular bridges resulting in loss of adhesion between epidermal cells. Author examined, by modified direct immunofluorescence, the deposition of plasmin and its inhibitor proteins such as alpha 1-antitrypsin and alpha 2-macroglobulin, with the early lesional skin specimens from 5 patients of pemphigus All these lesional skin demonstrated intense deposits of plasmin and aIpha 2-mscrogIobulin, and to a less degree alpha l-antitrypsin, all having indentical patterns to that of IgGautoantibodies. These proteins were also stained at the dermoepidermal junction and upper dermis, but less intensely. The identification of these particular proteins ; plasmin, alpha 1 antitrypsin, and alpha 2 macroglobulin, could be an alternate mean for the enzyme-histologic diagncsis of pemphigus.
alpha 1-Antitrypsin ; alpha-Macroglobulins ; Antibodies ; Dermis ; Fibrinolysin* ; Fluorescent Antibody Technique, Direct ; Humans ; Pemphigus* ; Plasminogen ; Plasminogen Activators ; Skin*

OBJECTIVETo study the value of the determination of serum and urine haptoglobin (HP) and alpha 1-antitrypsin (AAT) in predicting the response to glucocorticoid therapy in children with primary nephrotic syndrome (PNS).

METHODSA total of 84 children with PNS were classified to steroid-sensitive nephrotic syndrome (SSNS) (n=58) and steroid-resistant nephrotic syndrome (SRNS) groups (n=26). Forty healthy children were randomly selected for the control group. HP and AAT levels in blood and urinary samples were determined using ELISA. The efficiency of HP and AAT in predicting the response to glucocorticoid treatment of PNS was evaluated by the receiver operating characteristic (ROC) curve.

RESULTSCompared with the control group, both the SSNS and SRNS groups had significantly higher serum HP concentrations and urine AAT/Cr ratio before treatment (P<0.05); compared with the SSNS group, the SRNS group had significantly higher serum HP concentrations and urine AAT/Cr ratio before treatment and after one week and four weeks of treatment (P<0.05). Serum HP had the highest efficiency in predicting the response to glucocorticoid treatment of PNS at the concentration of 37.935 mg/mL, with the sensitivity and specificity being 92.3% and 86.2% respectively. Urine AAT/Cr ratio had the highest prediction efficiency at 0.0696, with the sensitivity and specificity being 100% and 79.3% respectively. ROC curve analysis of serum HP combined with urine AAT/Cr ratio showed a better prediction efficiency, with the sensitivity and specificity being 92.3% and 96.6% respectively.

CONCLUSIONSThe increase in serum HP level or urine AAT/Cr ratio may indicate glucocorticoid resistance in the early stage of PNS. A combination of the two can achieve better efficiency in the prediction of SRNS.

Child ; Child, Preschool ; Creatinine ; urine ; Female ; Glucocorticoids ; therapeutic use ; Haptoglobins ; analysis ; urine ; Humans ; Male ; Nephrotic Syndrome ; blood ; drug therapy ; urine ; alpha 1-Antitrypsin ; analysis ; blood ; urine

OBJECTIVE: To observe the characteristics of serum protein in patients with benign paroxysmal positional vertigo (BPPV) during the symptomatic period. METHOD: Fifty-five patients with BPPV and 58 control subjects were enrolled in the study. All the patients underwent the Dixe-Hallpike and Roll maneuver to confirm the type of BPPV. The average time of onset was (1.0 ± 0.8)days in the group of BPPV. The clinical features and the laboratory tests of serum protein electrophoresis, blood counts, and liver and kidney function were performed in both groups. RESULT: The levels of serum albumin (Alb), α1 globulin, α2 globulin and β globulin of BPPV group did not differ statistically in the two groups (P > 0.05); The γ-globulin levels were significantly higher in patients with BPPV than in controls (P < 0.05). CONCLUSION The γ-globulin levels are increased when BPPV is attacking.
Alpha-Globulins ; analysis ; Benign Paroxysmal Positional Vertigo ; blood ; diagnosis ; Beta-Globulins ; analysis ; Case-Control Studies ; Humans ; Serum Albumin ; analysis ; gamma-Globulins ; analysis

1-AT is a potent inhibitor of protease in human serum. 1-AT was successfully purified by a 5 step procedure: (1) 34%-80% ammonium sulfate precipitation, (2) affinity chromatography on Cibacron blue sepharose column, (3) ion-exchange chromatography on DEAE-sephadex A50, (4) 80% ammonium sulfate precipitation and HPLC. with the arginin-HCL 10% buffer, pH 6.0; (3) ion-exchange on DEAE-sephadex A50 and (5) HPLC. A molecular weight and isoelectropoint of the albumin are similar with those of the 1-AT. That is why, affinity chromatography on cibacron blue sepharose column to remove the serum albumin is a very important step of the 1-AT purification. The purified 1-AT is in 91-fold purification with recovery of 32.1% specific inhibitory activity of 32978.4 per gam protein
Plasma ; alpha 1-Antitrypsin

BACKGROUND: Effective treatment and monitoring of tuberculosis (TB) requires biomarkers that can be easily evaluated in blood samples. The aim of this study was to analyze the serum proteome of patients with TB and to identify protein biomarkers for TB. METHODS: Serum samples from 26 TB patients and 31 controls were analyzed by using nano-flow ultra-performance liquid chromatography coupled to quadrupole time-of-flight mass spectrometry in data-independent mode, and protein and peptide amounts were calculated by using a label-free quantitative approach. The generated data were analyzed by using principal component analysis and partial least squares discriminant analysis, a multivariate statistical method. RESULTS: Of more than 500 proteins identified, alpha-1-antitrypsin was the most discriminative, which was 4.4 times higher in TB patients than in controls. Peptides from alpha-1-antitrypsin and antithrombin III increased in TB patients and showed a high variable importance in the projection scores and coefficient in partial least square discriminant analysis. CONCLUSIONS: Sera from patients with TB had higher alpha-1-antitrypsin levels than sera from control participants. Alpha-1-antitrypsin levels may aid in the diagnosis of TB.
Adult ; Aged ; Antithrombin III/analysis ; Biological Markers/blood ; Chromatography, High Pressure Liquid ; Discriminant Analysis ; Female ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Proteome/*analysis ; *Proteomics ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; Tuberculosis/*blood/genetics/metabolism ; alpha 1-Antitrypsin/analysis

BACKGROUND: After multiple trauma, blood coagulation activity is enhanced and fibrinolytic activity is suppressed. Due to high tissue thromboplastin concentration in cerebral tissue, more serious coagulation and fibrinolytic abnormalities may occur when concomitant head trauma is present. The aim of this study was to determine the changes in coagulation and fibrinolysis after trauma and the effects of head trauma on coagulation and fibrinolysis. METHODS: This study includes 35 trauma patients: 16 patients with head trauma (group A) and 19 patients without head trauma (group B). We measured the plasma levels of functional protein C, antithrombin III (AT III), thrombin antithrombin III complex (TAT), plasmin alpha 2 plasmin inhibitor complex (PIC), tissue plasminogen activator antigen (t-PA), and plasminogen activator inhibitor-1 antigen (PAI-1) on admission and on days 1, 2, 4, and 6 after the trauma. RESULTS: The TAT and the TAT/PIC were significantly higher in group A than in group B on all days. PIC was significantly lower in group A than in group B on all days except the day of admission. Over the course of time, the TAT and the TAT/PIC decreased in both groups and PIC increased. On admission, the PAI-1 of both groups was increased, but it decreased over the course of time. The t-PA was increased on admission, was suppressed on the 1st day, and then increased again. The PAI-1 and the t-PA showed no significant difference between the two groups. CONCLUSIONS: After multiple trauma, coagulation was enhanced and fibrinolysis was suppressed. Enhanced coagulation and suppressed fibrinolysis were significantly greater in group A than in group B.
alpha-2-Antiplasmin ; Antithrombin III ; Blood Coagulation ; Craniocerebral Trauma ; Fibrinolysin ; Fibrinolysis* ; Humans ; Multiple Trauma* ; Plasma ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators ; Protein C ; Thrombin ; Thromboplastin ; Tissue Plasminogen Activator