Heparin-Induced Thrombocytopenia (HIT)

Heparin-induced thrombocytopenia (HIT I) is a severe, life-threatening, and immunological drug reaction. According to the clinical-laboratory characteristics, there are two types of HIT: type I (HIT I) and type II (HIT II). HIT I is the result of non-immunologic, direct interaction of heparin with the platelet surface. Contrary, HIT II is immunologically induced (antibody-mediated) and life-threatening side effect of heparin therapy, often associated with thromboembolic complications. All patients receiving heparin are exposed to the development of anti-heparin antibodies, irrespective of the heparin dosage, type, and method of administration. HIT most commonly develops in intensive care patients, dialyzed patients, and cardiosurgical and orthopedic patients. It commonly develops after 5–10 days of heparin therapy. Platelet count decreases by more than 50% from the baseline and ranges from 20 × 109/L to 100 × 109/L. In HIT II, thromboembolic complications usually include deep-vein thrombosis and pulmonary embolism, but they also include arterial occlusion of the extremities, myocardial infarction, stroke, and necrosis and organ damage. Clinical assessment of the HIT probability using 4T´s score system, systematic monitoring of platelet number in heparin-receiving patients, and specific laboratory diagnosis of anti-heparin antibodies substantially contribute to the final confirmation of the diagnosis, enable timely administration of direct non-heparin thrombin antagonists, and reduce mortality from thromboembolic complications

Hemothorax as the first manifestation of idiopathic pulmonary arteriovenous malformation

Background: Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies and hemothorax as a presenting feature of PAVM is a very rare occurrence. Case presentation: A 45-year old woman presented with chest pain and breathlessness. A chest x-ray showed left-sided pleural effusion. An emergency MSCT scan with contrast showed no signs of pulmonary embolism but instead a probable AV malformation was shown. Diagnostic thoracocentesis revealed hemorrhagic exudate with negative cytology and microbiology findings. Thoracic drainage was performed resulting with complete regression of hemothorax. Three months later, patient was treated with transcatheter embolization of PAVM with good clinical outcome. Conclusions: We have shown that management of PAVM related hemothorax initially by thoracic drainage followed by later on performed catheter embolization of the PAVM could lead to a successful outcome

Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Background: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history. Methods: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels < 11 μM and/or proteinase inhibitor genotypes PI*ZZ, PI*SZ and compound heterozygotes or homozygotes of other rare deficient variants. We describe the characteristics of the individuals included from February 2020 to May 2022. Results: A total of 1044 individuals from 15 countries were analysed. The most frequent genotype was PI*ZZ (60.2%), followed by PI*SZ (29.2%). Among PI*ZZ patients, emphysema was the most frequent lung disease (57.2%) followed by COPD (57.2%) and bronchiectasis (22%). Up to 76.4% had concordant values of FEV1(%) and KCO(%). Those with impairment in FEV1(%) alone had more frequently bronchiectasis and asthma and those with impairment in KCO(%) alone had more frequent emphysema and liver disease. Multivariate analysis showed that advanced age, male sex, exacerbations, increased blood platelets and neutrophils, augmentation and lower AAT serum levels were associated with worse FEV1(%). Conclusions: EARCO has recruited > 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function.Funding: The International EARCO registry is funded by unrestricted grants of Grifols, CSL Behring, Kamada, pH Pharma and Takeda to the European Respiratory Society (ERS). Acknowledgements: The authors would like to thank the patients who participated in this study and the EARCO study investigators (listed below). We wish to acknowledge Elise Heuvelin from the ERS ofce (Lausanne, Switzterland) for her support in the management of EARCO, and Gemma Vilagut and Christina Founti (Bioclever, Barcelona, Spain) for their support in EARCO data monitoring. We also acknowledge the participation of Eduardo Loeb (Barcelona, Spain) in the development of the database and the monitoring of the data. List of EARCO study investigators: Georg-Christian Funk (Austria), Wim Jans sens, Silvia Pérez-Bogerd (Belgium), Leidy Prada (Colombia), Ana Hecomovic (Croatia), Eva Bartosovska, Jan Chlumsky, (Czech Republic), Alan Altraja, Jaanus Martti (Estonia), Angelo G. Corsico, Ilaria Ferrarotti, Simone Scarlata, Mario Malerba (Italy), Jan Stolk, Emily F van’t Wout (Netherlands), Joanna Chorowstoska-Wyminko (Poland), Catarina Guimaraes, Maria Sucena, Ana Caldas Raquel Marçoa, Isabel Ruivo dos Santos, Bebiana Conde, Maria Joana Reis Amado Maia Da Silva, Rita Boaventura (Portugal), Ruxandra Ulmeanu (Romania), María Torres-Duran, Marc Miravitlles, Miriam Barrecheguren, Juan Luis Rodriguez-Hermosa, Myriam Calle-Rubio, José María Hernández-Pérez, José Luis López-Campos, Francisco Casas-Maldonado, Ana Bustamante, Carlota Rodriguez-García, Cristina Martinez-González, Cruz González, Eva Tabernero, Lourdes Lázaro, Virginia Almadana, Mar Fernández-Nieto, Francisco Javier Michel de la Rosa, Carlos Martíez-Rivera, Layla Diab, María Isabel Parra (Spain), Hanan Tanash, Eeva Piitulainen (Sweden), Christian F. Clarenbach (Switzerland), Serap Argun Baris, Dilek Karadogan, Sebahat Genç (Turkey), Alice M. Turner, Beatriz Lara, David G. Parr (United Kingdom). EARCO Steering committee: Christian F Clarenbach and Marc Miravitlles (Co-chairs), Robert Bals, Jan Stolk, Joanna Chorostowska-Wynimko, Karen O’Hara, Marion Wilkens, José Luis López-Campos, Alice M. Turner, Ilaria Ferrarotti, Gerry McElvaney and Robert A. Stockle

The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO

Background: Although bronchiectasis has been recognised as a feature of some patients with Alpha1-Antitrypsin deficiency the prevalence and characteristics are not widely known. We wished to determine the prevalence of bronchiectasis and patient characteristics. The first cohort of patients recruited to the EARCO (European Alpha1 Research Collaboration) International Registry data base by the end of 2021 was analysed for radiological evidence of both emphysema and bronchiectasis as well as baseline demographic features. Results: Of the first 505 patients with the PiZZ genotype entered into the data base 418 (82.8%) had a reported CT scan. There were 77 (18.4%) with a normal scan and 38 (9.1%) with bronchiectasis alone. These 2 groups were predominantly female never smokers and had lung function in the normal range. The remaining 303 (72.5%) ZZ patients all had emphysema on the scan and 113 (27%) had additional evidence of bronchiectasis. Conclusions: The data indicates the bronchiectasis alone is a feature of 9.1% of patients with the PiZZ genotype of Alpha1-antitrypsin deficiency but although emphysema is the dominant lung pathology bronchiectasis is also present in 27% of emphysema cases and may require a different treatment strategy

Antioxidant Capacity of Teas and Herbal Infusions: Polarographic Assessment

Hydrogen peroxide scavenging (HPS) activity of unfermented (green, yellow, and white), partially fermented (oolong), and completely fermented (black) tea (Camellia sinensis), mate (Ilex paraguariensis), and various herbal infusions, as well as individual compounds (flavan-3-ols, flavonols, cinnamic and benzoic acids, and methylxanthines), was assessed by recently developed direct current (DC) polarographic assay. Correlations of tea and herbal infusion HPS activity with total phenolic content determined using the Folin-Ciocalteu assay (FC-GAE) (0.81 and 0.93), ferric reducing/antioxidant power (FRAP) (0.97 and 0.92), 1,1-diphenyl-2-picrylhydrazyl (DPPH) (0.77 and 0.80), and 2,2'-azinobis(3-ethylbenzthiazoline-6-sulfonic acid) (ABTS) scavenging (0.86 and 0.86) were statistically significant. Correlations between relative antioxidant capacity index (RACI), calculated by assigning all applied assays equal weight, and HPS (0.98), FRAP (0.97), ABTS (0.89), and DPPH (0.89) confirmed DC polarographic assay reliability when applied individually. Correlation analysis, ANOVA, and Levene and Tukey's HSD tests unequivocally confirmed this reliable, rapid, and low-cost assay validity, clearly demonstrating its advantages over spectrophotometric assays applied

Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency : EARCO international registry

Background: Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history. Methods: The EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function. Trial registrationwww.clinicaltrials.go