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

Serum a-1-antitrypsin (a-1-AT) was determined in 40 patients with chronic viral hepatitis. The assay is based on the method used for quantitative determination of a-1-AT by rate nephelometry of antigen antibody complex. The results indicate that serum a-1-AT levels of patients chronic viral hepatitis is greater than of normal subjects. The linear correlation were found between serum a-1-AT levels and ALAT, ASAT activities, total bilirubin with r = 0.03; 0.43; 0.45 respectively.
Hepatitis, Chronic ; alpha 1-Antitrypsin

Hemorrhage ; genetics ; Humans ; Mutation ; Recurrence ; alpha 1-Antitrypsin ; genetics

Malignant fibrous histiocytoma(MFH) is well known as a soft tissue tumor of the extremities and retroperitoneum, but MFH of the skull is very rare. We report a case of MFH arising from the temporal bone in a 27-year-old male. This tumor presented as an osteolytic lesion and soft tissue mass on the left temporal bone without obvious invasion of the underlying brain parenchyma. The patient underwent tumor and bone removal, follwed by radiation therapy. Hostologic examination disclosed pleomorphic spindle cells in a storiform pattern and tumor cells showed positive reaction for vimentin, lysozyme, alpha 1-antitrypsin and (1-antichymotrypsin in immunohistochemical stain.
Adult ; alpha 1-Antitrypsin ; Brain ; Extremities ; Histiocytoma, Malignant Fibrous* ; Humans ; Male ; Muramidase ; Skull ; Temporal Bone* ; Vimentin

The study reports a boy with alpha1-antitrypsin Pittsburgh mutation. The boy was admitted into the hospital because of recurrent joint hematoma. The laboratory examinations revealed that prothrombin time and activated partial thromboplastin time were prolonged and cannot be corrected by 1:1 fresh plasma. The inhibitor of factor VIII, anticardiolipin antibody and lupus anticoagulant were all negative. Platelet aggregation test indicated the existence of the inhibitor of thrombin. Alpha1-antitrypsin Pittsburgh mutation was confirmed by genomic sequencing. The clinical manifestations, diagnosis and treatment of this disorder are discussed in this paper.
Child ; Hematoma ; epidemiology ; Humans ; Male ; Mutation ; Recurrence ; alpha 1-Antitrypsin ; genetics

Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as diffuse nodular thickening of the small bowel and as linear hypodense streaking densities in the small bowel caused by dilated lymphatic channels. Demonstration of increased enteric protein loss using 51Cr-, 131I- or 99mTc-labeled albumin, timed measurement of fecal excretion of radioactivity or by measuring fecal clearance of alpha 1-antitrypsin can also help the diagnosis. We experienced a rare case of intestinal lymphangiectasia in an eight year old boy who presented with facial edema, abdominal distension and intermittent diarrhea. We report a patient with intestinal lymphangiectasia, in whom abdominal CT, 99mTc-labeled albumin scintitigraphy, and stool alpha 1-antitrypsin measurement played key roles in determining the diagnosis. A brief review of literature was made.
alpha 1-Antitrypsin ; Biopsy ; Diagnosis ; Diarrhea ; Edema ; Humans ; Hypoalbuminemia ; Male ; Radioactivity ; Tomography, X-Ray Computed

Child ; Child, Preschool ; Humans ; alpha 1-Antitrypsin Deficiency ; diagnosis ; genetics ; therapy

OBJECTIVETo investigate whether pretreatment with alpha(1)-antitrypsin (AAT) can attenuate acute lung injury (ALI) in rabbits induced with endotoxin.

METHODSThirty-two healthy adult New Zealand rabbits were anaesthetized, tracheotomized and mechanically ventilated. They were then randomly divided into four groups (n = 8): (1) Infusion of Escherichia coli endotoxin [Lipopolysaccharide (LPS) 500 microg/kg] without AAT (Group LPS). (2) Infusion of AAT 120 mg/kg at 15 minutes after LPS (Group LAV). (3) Infusion of AAT 120 mg/kg without endotoxin (Group AAT). (4) Infusion of saline 4 ml/kg as control (Group NS). Arterial blood gases, peripheral leukocyte counts and airway pressure were recorded every hour for eight hours. Physiologic intrapulmonary shunting (Qs/Qt) was measured every four hours. After eight hours, blood samples were collected for measurement of plasma concentration and activity of AAT. Then, the animals were sacrificed, and bronchoalveolar lavage fluid (BALF) was collected for measurement of concentrations of total protein (TP), interleukin-8 (IL-8), tumor necrosis factor (TNF alpha, the activities of NE and AAT, total phospholipids (TPL) and disaturated phosphatidylcholine (DSPC). In addition, the wet-to-dry lung weight ratio (W/D) was measured.

RESULTSThe infusion of endotoxin induced decreases in arterial oxygen pressure (PaO(2)), peripheral leukocyte counts, total respiratory compliance (TLC) and the increases in peak pressure (P(peak)), Qs/Qt compared with the baseline values (P < 0.05). The increased plasma concentration but reduced activity of AAT was also found in contrast to that in Group NS (P < 0.05). In the BALF, the activity of AAT, TPL, DSPC/TPL were lower than those in Group NS (P < 0.05), but the concentrations of albumin, IL-8, TNF alpha, the activity of NE and the ratio of W/D were higher than those in Group NS (P < 0.05). The pretreatment of AAT attenuated the deterioration of oxygenation, the reduction of compliance and the deterioration of other physiological and biochemical parameters mentioned above.

CONCLUSIONPretreatment with AAT could attenuate endotoxin-induced lung injury in rabbits. Those beneficial effects of AAT might be due, in part, to reduction in the levels of mediators that could activate neutrophils, in addition to the direct inhibitory effect on neutrophil elastase.

Animals ; Endotoxins ; toxicity ; Lung Diseases ; prevention & control ; Rabbits ; Random Allocation ; alpha 1-Antitrypsin ; pharmacology ; therapeutic use

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*

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