Antitripsine

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INTRODUCTION AND OBJECTIVE: REDAAT, the Spanish Registry of Patients with Alpha-1 Antitrypsin Deficiency, was set up in order to improve knowledge of this disease. This study is an evaluation of the registry and an analysis of its patient population. METHODS: The registry has a database hosted on the website www.redaat.es. It collects clinical and functional data on patients with PiSZ, ZZ phenotypes and other rare variants. RESULTS: Thanks to the collaboration of 124 physicians, the registry currently contains information on 511 individuals from 103 healthcare centers...

Western blot of synovial fluid has been widely used for osteoarthritis (OA) research and diagnosis, but there is no ideal loading control for this purpose. Although β-actin is extensively used as loading control in western blot, it is not suitable for synovial fluid because it is not required in synovial fluid as a cytoskeletal protein. A good loading control for synovial fluid in OA studies should have unchanged content in synovial fluids from normal and OA groups, because synovial fluid protein content can vary with changes in synovial vascular permeability with OA onset...

Protein-loosing enteropathy in patients with inflammatory bowel diseases (IBDs) is an uncommon complication, but should be considered in any patient with hypoproteinemia in whom other causes have been excluded such as concomitant hepatic disease, severe malnutrition or proteinuria. The diagnosis is based on determination of fecal alpha-1 antitripsin clearance and stool analysis. Prognosis depends upon the patient and the disease location, severity and complication. Treatment is directed at control of the underlying IBDs but also includes albumin infusion in the most severe cases of hypoalbuminemia and fluid retention, dietary modifications for recovery and maintenance of nutritional status and supportive care to prevent further complication like deep venous thrombosis...

BACKGROUND & AIMS: Wilson's disease (WD) is an autosomal recessively inherited copper storage disorder due to mutations in the ATP7B gene that causes hepatic and neurologic symptoms. Current treatments are based on lifelong copper chelating drugs and zinc salts, which may cause side effects and do not restore normal copper metabolism. In this work we assessed the efficacy of gene therapy to treat this condition. METHODS: We transduced the liver of the Atp7b(-/-) WD mouse model with an adeno-associated vector serotype 8 (AAV8) encoding the human ATP7B cDNA placed under the control of the liver-specific α1-antitrypsin promoter (AAV8-AAT-ATP7B)...

INTRODUCTION: The low-grade inflammation and insulin resistance are two events that could be present in varying degrees, on obesity and chronic diseases. The degree of subclinical inflammation can be gauged by measuring the concentrations of some inflammatory biomarkers, including the hepatic origin ones. Some of those biomarkers are sialic acid, α1-antitrypsin and the C-terminal fragment of alpha1-antitrypsin, ceruloplasmin, fibrinogen, haptoglobin, homocystein and plasminogen activator inhibitor-1...

Recently, the share of children with verified neonatal hepatitis induced by genetic predisposition, malformations of biliary tracts, inborn infections with affection of hepatobiliary system increased. The comprehensive biochemical examination of 62 children aged from 1.5 months to 2 years old with diagnosis of neonatal hepatitis. The changes of standard indicators of cytolysis, cholestasis and protein metabolism were on average moderate in group with reliable increase of protein concentration of acute phase of inflammation...

The integral indices based on element ratio of hemogram as well as alpha-1 antitripsin, interleukins 2 and 10 contents in the adolescents living in industrial town with a high pollution level are represented in this paper. The increase in the processes of the nonspecific phagocytic protection and exertion of adaptive mechanisms has been revealed.

We delineate a complication of hypoalbuminemia in dentatorubral-pallidoluysian atrophy (DRPLA), which we have found to be common in this disorder. In addition, we explored the pathogenesis of this phenomenon through clinical and histological examinations. Clinical course and laboratory findings of nine patients with childhood-onset DRPLA (aged 6-49 years; CAG repeat length 62-93) were retrospectively reviewed. Autopsied specimens from three patients were examined by histopathological and immunohistochemical analyses...

The content of alpha-macroglobulin associated with pregnancy, alpha2-glycoprotein, alpha1-antitripsin, and lactolerrin in blood serum of pregnant women and umbilical serum under hydramnion and risk of development of intrauterine infection of fetus is investigated. It is demonstrated that in case ofpresence in blood of pregnant woman of G-antibodies to C. trachomatis under low titers (1:20, 1:40) the increase of levels of alpha-macroglobulin, alpha2-glycoprotein, al-antitripsin and especially of lactoferrin in serum of pregnant women testifies the high risk of presence of intrauterine infection of fetus and probability of birth of child with low values on Apgar scale...

This article presence the connection between paradontitis and cardiovascular diseases, and definition of the maintenance of acute phase proteins in an oral fluid at patients with acute myocardial infarction is obviously important for clinic. Results of own researches of change of the maintenance of three acute phase proteins: ceruloplasmin, alpha1-antitripsin and orosomucoid in an oral fluid and blood plasma at paradontitis and myocardial infarction allow to consider the paradontitis as one more risk factor of a cardiovascular pathology, except well-known hypertensions, smoking, a diabetes...

The article presents the results of analysis of blood serum from 60 patients (aged 58.9 +/- 1.5 years) with Q-genous complicated and non-complicated cardiac infarction. The study evaluated the content of various proteins of acute phase (alpha-2-macrogloblin, alpha-1-antitripsin. lactofferin) and cytokines inducing their synthesis (IL-6, IL-1beta, L-8). The examinations carried out on 1st, 7th and 14th day of development of cardiac infarction. It is demonstrated that under cardiac infarction complicated by cardiogenic shock on first day of diseases high levels of lactoferrin and lower levels of macroglobulin are detected in blood serum...

BACKGROUND: Constrictive pericarditis (CP) represents a rare cause of protein-losing enteropathy (PLE) resulting from intestinal lymphangiectasia (IL). In this report, we describe an 8-year-old Turkish boy with IL and PLE secondary to CP. METHODS: The boy was introduced to our clinic due to bilateral pretibial edema and swelling of the eyelids caused by hypoproteinemia. Physical examination revealed a distended right jugular vein. Laboratory investigation revealed PLE with fecal concentration of alpha-1 antitripsin of 4...

BACKGROUND: A sudden death of drug addicts is generally attributed to the depressive effect of substances in question on the respiratory center in the CNS. Microscopic morphology of lungs is relatively rarely dealt with. PURPOSE: This study probes into the morphology of individuals who were intoxicated by amphetamine and/or heroin and suddenly died. MATERIAL AND METHODS: The investigated group comprised lung specimens collected in the files of the Department of Forensic Medicine of Medical University of Warsaw in the years 2002-2004...

UNLABELLED: Asymptomatic HPV infection in pregnant women may be connected with changes of pro-inflammatory cytokines and proteolytic enzymes which may condition the persistence of infection during pregnancy. AIM: The main aim of the study was to evaluate the concentration of interleukin-1, TNF-alpha and alpha-1 antitrypsin in pregnant women infected by oncogenic types HPV. MATERIALS AND METHOD: The study was conducted in 2008-2009 at the Department of Obstetrics and Gynecology Medical University Lodz, on pregnant women in the third trimester of pregnancy The study group consisted on 19 pregnant women HPV-16, -18 infected...

Hepatocellular carcinoma (HCC) is the third most common cause of cancer-related death worldwide. Primary hepatocellular carcinoma can be found most frequently (80-90 %) in patients with liver cirrhosis. The most frequent causes of liver cirrhosis are chronic hepatitis B and C virus infections and chronic alcohol consumption. The occurrence of hepatocellular carcinoma is about 3-15 % in patients with alcoholic liver disease. Other predisposing causes can be: non-alcoholic steatohepatitis (NASH), obesity, diabetes mellitus, autoimmune hepatitis, intrahepatic biliary inflammations (primary biliary cirrhosis, primary sclerosing cholangitis), copper and iron metabolic diseases (Wilson-disease, haemochromatosis), congenital alpha-1-antitripsin deficiency...

UNLABELLED: Obesity is associated with increased inflammation. Creactive protein (CRP) and inflammation-sensitive plasma protein (ISPs) are inflammatory markers. Proinflammatory process may be influenced by high saturated fatty acid intake. OBJECTIVE: The aim of the present study was to evaluate the role of saturated fatty acids load on postprandial circulating levels of PCR and ISPs (alpha1-antitrypsin, alpha1-acid glucoprotein, and fibrinogen) in obese women. DESIGN: A total of 15 obese women (age = 31,7 +/- 4,5 years, BMI = 37,9 +/- 7,3 kg/m(2)) and 15 lean controls women (age = 30,6 +/- 4,6 years, BMI = 20,6 +/- 2,6 kg/m(2)) were recruited for this study...

Blood samples taken from test-subjects in the 7-d immersion experiment were analyzed for blood proteins belonging to alpha1- and alpha2-globulins electrophoretic fractions: alphal-antitripsin (alpha1-AT), a1-acidic glycoprotein (alpha1-AGP), ceruplasmin (Cer), haptoglobin (Hp), alpha2-macroglobulin (alpha2-M), apolipoprotein A (ApoA). Immersion was demonstrated to alter concentrations of the studied proteins; besides, it was shown that return to the normal state could also affect protein concentrations. Immersion effects on blood proteins patterned after acute reaction...

BACKGROUND: We have previously reported the successful induction of renal allograft tolerance in non-human primates using a nonmyeloablative conditioning regimen to produce a mixed-chimeric state in the recipient. In the present study, we applied this same technique to lung allotransplantation in cynomolgus monkeys. METHODS: Nine pairs of fully major histocompatibility complex (MHC)-mismatched cynomolgus monkeys were used. The conditioning regimen consisted of total body irradiation, thymic irradiation, and antithymocyte globulin...

UNLABELLED: Obesity is associated with increased inflammation. C-reactive protein (CRP) is a proinflammatory molecule, and alpha1-antitrypsin is an inflammation-sensitive plasma protein. Proinflammatory process may be influenced by postprandial hyperglycemia. OBJECTIVE: The aim of the present study was to evaluate the role of high-glucose load on postprandial circulating levels of PCR and alpha1-antitrypsin in obese women with normal glucose tolerance. DESIGN: A total of 15 obese women (age=34...