Performance of enhanced liver fibrosis plasma markers in asymptomatic individuals with ZZ α1-antitrypsin deficiency.

Objectives: Alpha1-antitrypsin deficiency (AATD) is a common genetic cause of chronic liver disease. According to retrospective studies, up to 25% of those with homozygous ZZ (Glu 342 to Lys) AATD suffer from liver cirrhosis and/or liver cancer in late adulthood. We hypothesized that the plasma markers for liver fibrosis, necrosis, and apoptosis may identify AATD individuals at higher risk for liver diseases.

Plasma levels of alpha1-antichymotrypsin and secretory leukocyte proteinase inhibitor in healthy and chronic obstructive pulmonary disease (COPD) subjects with and without severe alpha1-antitrypsin deficiency.

Background: Individuals with severe Z alpha1-antitrypsin (AAT) deficiency have a considerably increased risk of developing chronic obstructive lung disease (COPD). It has been hypothesized that compensatory increases in levels of other protease inhibitors mitigate the effects of this AAT deficiency. We analysed plasma levels of AAT, alpha1-antichymotrypsin (ACT) and secretory leukocyte protease inhibitor (SLPI) in healthy (asymptomatic) and COPD subjects with and without AAT deficiency.

Chronic intestinal pseudo-obstruction due to buserelin-induced formation of anti-GnRH antibodies.

Background & Aims: A 30-year-old woman, treated with buserelin, an analogue of gonadotropin-releasing hormone (GnRH) (also called luteinizing hormone-releasing hormone, LH-RH), developed chronic intestinal pseudo-obstruction (CIPO). The sudden onset of this disease in a previously healthy woman perplexed us. CIPO refers to a gastrointestinal disorder that can have a variety of causes, such as drugs, among others.

Analysis of systemic biomarkers in COPD patients.

The finding that alphal-antitrypsin (AAT) deficiency, PiZZ, a well-established genetic risk factor for COPD, is related to high levels of circulating AAT polymers, prompted us to measure serum levels of such polymers and selected markers of inflammation in age- and gender-matched patients with stable COPD and control subjects with and without severe AAT deficiency, and to assess their relationship with each other and with the genetic AAT-variant. We found that COPD individuals (n= 20), independent of AAT-variant, had significantly higher serum levels of AAT and its polymers, MMP-9, sICAM-1, VEGF and sE-selectin than controls (n=30). Subjects with PiZZ COPD (n= 10) showed significantly elevated serum levels of AAT-polymers, sE-selectin and sICAM-1, while patients with PiMM COPD (n= 10) showed higher levels of MMP-9, VEGF, IL-8 and MCP-1 than controls.

Secreted leukocyte protease inhibitor is present in aqueous humours from cataracts and other eye pathologies.

Previous studies identified serine, cysteine and metalloproteases in normal aqueous humours (AH) and suggested that a balance between proteases and their inhibitors may play a role in the modulation of the AH outflow. We aimed to determine whether secretory leukocyte protease inhibitor (SLPI), a serine protease inhibitor, is present in AH of patients with cataract and other eye pathologies. AH was collected from 117 cataract patients of which 55 were diagnosed with more when one eye disease: cataract only (n=62), pseudoexfoliation (PEX) (n=26), glaucoma (n=6), diabetes retinopathy (n=4), iritis-uveitis (n=4) and macular degeneration (n=28).

Increased plasma levels of serine proteinase inhibitors in lung cancer patients.

Background: Tumor growth and invasiveness occur through infiltration of tumor cells into the host cells and by angiogenesis, which is modulated by proteinases and antiproteinases released from tumor cells that carry out tissue remodelling. A number of studies have revealed variations in the plasma levels of serine proteases and their inhibitors among tumor types.

Patients And Methods: By immunological methods we analysed the levels of serine protease inhibitors AAT, ACT and SLPI in newly diagnosed lung cancer patients (n=14) compared to non-smoker and smoker, age- and gender-matched control groups (n=16), and also in an expanded group of lung cancer patients with local tumors (n=14) and with metastasis (n=18).