HISTOMORPHOMETRIC PARAMETERS OFTHE CARDIAC CONDUCTION SYSTEM AND THE MYOCARDIUM: CORRELATING RESULTS OF POSTMORTEM FORENSIC ANALYSIS ON ALCOHOLIC CARDIOMYOPATHY AND CORONARY HEART D

The article presents results of a study on the correlation of histomorphometric parameters of the heart in case of death from alcoholic cardiomyopathy (ACM), ischemic heart disease (IHD) and mechanical injury (control group) by the method of correlation analysis. The previous studies show that normally with age, the myocardium of the sinoatrial node (SAN) is most worn out and fibrosed. In case of IHD the His bundles are most worn out and fibrosed. In case of ACM, histomorphological changes are uniform in all areas of the myocardium. This indicator can be used in practice when providing differential diagnosis of the above-described types of pathology and causes of death. In addition, the results of our study confirm that myocardial lipomatosis is more typical of ACM, which increases its diagnostic value.

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Causes of death in beef cattle in southern Brazil

Journal of Veterinary Diagnostic Investigation ◽

10.1177/10406387211007952 ◽

2021 ◽

pp. 104063872110079

Author(s):

Franciéli A. Molossi ◽

Bianca S. de Cecco ◽

Camila B. Pohl ◽

Rogério B. Borges ◽

Luciana Sonne ◽

...

Keyword(s):

Beef Cattle ◽

Causes Of Death ◽

Diagnostic Category ◽

Diagnostic Value ◽

Parasitic Diseases ◽

Porto Alegre ◽

Pathology Laboratory ◽

Regional Location ◽

Young Cattle ◽

Postmortem Examinations

We determined the prevalence of diseases and pathogens associated with mortality in beef cattle in the State of Rio Grande do Sul, Brazil, based on pathology laboratory submissions. Postmortem examinations were conducted on 1,277 beef cattle that died between 2008 and 2018. Information regarding age, time of the year, breed, and regional location were analyzed statistically. Most cattle were from the surrounding region of Porto Alegre, and 78.7% of the analyzed cases had diagnostic value. The diagnostic category with most cases was infectious and/or parasitic diseases (60%), followed by toxic and toxicoinfectious (25%). Most cases occurred in the fall. Major disease conditions identified included hemoprotozoal infection (18.2%), rabies (8.2%), and plant intoxications by Senecio spp. (8.5%) and Pteridium arachnoideum (4.6%). Hemoprotozoal infection occurred at a higher frequency in young cattle, mainly in animals up to 1 y old. Intoxication by Senecio spp. was more frequent in cattle 2–3 y old.

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Solid Swallow Examination During High Resolution Manometry and EGJ-Distensibility Help Identify Esophageal Outflow Obstruction in Non-obstructive Dysphagia

Dysphagia ◽

10.1007/s00455-021-10260-0 ◽

2021 ◽

Author(s):

Fritz Ruprecht Murray ◽

Lara Maria Fischbach ◽

Valeria Schindler ◽

Larissa Schnurre ◽

Juliane Marie Hente ◽

...

Keyword(s):

High Resolution ◽

Diagnostic Yield ◽

Diagnostic Value ◽

Control Group ◽

Clinical Response Rate ◽

Interventional Treatment ◽

Outflow Obstruction ◽

Motility Disorders ◽

High Resolution Manometry ◽

Solid Swallows

AbstractSingle water swallow (SWS) high-resolution manometry (HRM) may miss relevant esophageal motility disorders. Solid test meal (STM) during HRM and lately the functional lumen imaging probe (FLIP) have been shown to be of diagnostic value in the assessment of motility disorders. We aimed to assess the diagnostic yield of STM and FLIP in non-obstructive dysphagia (NOD). Patients assessed for dysphagia with both HRM and FLIP between April 2016 and August 2019 were analyzed for signs of non-obstructive EGJ outflow obstruction (EGJOO) according to Chicago Classification 3.0 (CCv3) and CC adapted for the use with solid swallows (CC-S), followed by an individual group-specific analysis. Five subjects without dysphagia served as control group. Standard HRM- and FLIP-values as well as esophagograms and Eckardt Scores were analyzed. Forty-two patients were identified (male/female, 14/36, median age 62). Twenty-five (59.5%) were diagnosed with EGJOO during STM only (= SWS-negative patients; CC-S). The EGJ distensibility index (EGJ-DI) of symptomatic patients was significantly lower compared to the control group (p = 0.006). EGJ-DI was < 3mm2/mmHg in 67% and 88% of patients diagnosed according to CC-S and CCv3, respectively. The IRP during STM showed a significant association to the corresponding EGJ-DI values (p < 0.001). Seventy-six percent of patients received treatment because of additional STM evaluation with a favorable clinical response rate of 89%. STM and FLIP identify EGJOO in symptomatic patients with normal SWS during HRM. STM resembles an inexpensive and clinically meaningful option to diagnose motility disorders and helps to select patients for interventional treatment.

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MiR-206 regulates the progression of osteoporosis via targeting HDAC4

European Journal of Medical Research ◽

10.1186/s40001-021-00480-3 ◽

2021 ◽

Vol 26 (1) ◽

Author(s):

Zhiyuan Lu ◽

Dawei Wang ◽

Xuming Wang ◽

Jilong Zou ◽

Jiabing Sun ◽

...

Keyword(s):

Cell Proliferation ◽

Cell Apoptosis ◽

Diagnostic Value ◽

Expression Level ◽

Luciferase Reporter ◽

Control Group ◽

Luciferase Reporter Gene ◽

Mineral Density ◽

Gene Assay

Abstract Background More and more studies have confirmed that miRNAs play an important role in maintaining bone remodeling and bone metabolism. This study investigated the expression level of miR-206 in the serum of osteoporosis (OP) patients and explored the effect and mechanism of miR-206 on the occurrence and development of osteoporosis. Methods 120 postmenopausal women were recruited, including 63 cases with OP and 57 women without OP. The levels of miR-206 were determined by qRT-PCR technology. Spearman correlation coefficient was used to evaluate the correlation of miR-206 with bone mineral density (BMD). An ROC curve was used to evaluate the diagnostic value of miR-206 in osteoporosis. The effects of miR-206 on cell proliferation and cell apoptosis of hFOBs were measured by CCK-8 assay and flow cytometry, respectively. Luciferase reporter gene assay was used to confirm the interaction of miR-206 and the 3′UTR of HDAC4. Results Serum miR-206 had low expression level in osteoporosis patient group compared with control group. The expression level of serum miR-206 had diagnostic value for osteoporosis, and the serum miR-206 levels were positively correlated with BMD. The down-regulated miR-206 could inhibit cell proliferation and promote cell apoptosis. Luciferase analysis indicated that HDAC4 was the target gene of miR-206. Conclusions MiR-206 could be used as a new potential diagnostic biomarker for osteoporosis, and in in vitro cell experiments, miR-206 may regulate osteoblast cell proliferation and apoptosis by targeting HDAC4.

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The diagnostic value of EBV-DNA and EBV-related antibodies detection for nasopharyngeal carcinoma: a meta-analysis

Cancer Cell International ◽

10.1186/s12935-021-01862-7 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Weixing Liu ◽

Gui Chen ◽

Xin Gong ◽

Yingqi Wang ◽

Yaoming Zheng ◽

...

Keyword(s):

Nasopharyngeal Carcinoma ◽

Test Performance ◽

Meta Analysis ◽

Diagnostic Value ◽

Cochrane Library ◽

Control Group ◽

Summary Receiver Operating Characteristic ◽

Ebv Dna ◽

The Difference ◽

Sensitivity Specificity

Abstract Background Numerous individual studies have investigated the diagnostic value of EBV-DNA, EA-IgA, VCA-IgA, EBNA1-IgA and Rta-IgG detection for nasopharyngeal carcinoma (NPC), but the conclusions remain controversial. This meta-analysis aimed to determine the value of EBV-DNA, EA-IgA, VCA-IgA, EBNA1-IgA and Rta-IgG detection in the diagnosis of NPC. Methods PROSPERO registration number: CRD42019145532. PubMed, EMBASE, Cochrane Library, and Chinese data libraries (Wanfang, CNKI, and CBM) were searched up to January 2019. The pooled sensitivity, specificity, and positive likelihood, negative likelihood, and diagnostic odds ratios were conducted in this meta-analysis. Summary receiver operating characteristic curves evaluated the test-performance global summary. Publication bias was examined by Deek’s funnel plot asymmetry test. Results Forty-seven studies with 8382 NPC patients (NPC group) and 15,089 individuals without NPC (Control group) were included in this meta-analysis. The sensitivity, specificity, positive likelihood (+ LR), negative likelihood (-LR), DOR and AUC of EBV-DNA in diagnosis of NPC were: 0.76 (95% CI 0.73–0.77), 0.96 (95% CI 0.95–0.97), 14.66 (95% CI 9.97–21.55), 0.19 (95% CI 0.13–0.28), 84 (95% CI 50.45–139.88), 0.96 (SE: 0.001), and 0.55 (95% CI 0.54–0.57), 0.96 (95% CI 0.96–0.97), 12.91 (95% CI 9.55–17.45), 0.35 (95% CI 0.29–0.43), 39.57 (95% CI 26.44–59.23), 0.94 (SE: 0.002) for the EA-IgA, and 0.85 (95% CI 0.84–0.85), 0.89 (95% CI 0.88–0.89), 6.73 (95% CI5.38–8.43), 0.17 (95% CI 0.12–0.23), 43.03 (95% CI 31.51–58.76), 0.93 (SE: 0.007) for the VCA-IgA, and 0.86 (95% CI 0.85–0.88), 0.87 (95% CI 0.88–0.90), 7.55 (95% CI 5.79–9.87), 0.16 (95% CI 0.13–0.19), 50.95 (95% CI 34.35–75.57), 0.94 (SE: 0.008) for the EBNA1-IgA, and 0.70 (95% CI 0.69–0.71), 0.94 (95% CI 0.94–0.95), 9.84 (95% CI 8.40–11.54), 0.25 (95% CI 0.21–0.31), 40.59 (95% CI 32.09–51.35), 0.95 (SE: 0.005) for the Rta-IgG. The EBV-DNA had larger AUC compared with other EBV-based antibodies (P < 0.05), while the difference between EA-IgA, VCA-IgA, EBNA1-IgA and Rta-IgG was not statistically significant (P > 0.05). Conclusions EBV-DNA, VCA-IgA, EBNA1-IgA and Rta-IgG detection have high accuracy in early diagnosis NPC. In addition, EBV-DNA detection has the higher diagnosis accuracy in NPC. On the other hand, EA-IgA is suitable for the diagnosis but not NPC screening.

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POS1405 OPTICAL SPECTRAL TRANSMISSION (HANDSCAN) IN PATIENTS WITH PSORIATIC ARTHRITIS: FIRST DATA ON DIAGNOSTIC VALUE AND ASSOCIATIONS WITH CLINICAL AND ULTRASOUND DISEASE ACTIVITY MARKERS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3670 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 985.1-985

Author(s):

K. Triantafyllias ◽

S. Liverakos ◽

C. Noack ◽

A. Schwarting

Keyword(s):

Rheumatoid Arthritis ◽

Psoriatic Arthritis ◽

Disease Activity ◽

Power Doppler ◽

Patient Characteristics ◽

Diagnostic Value ◽

Control Group ◽

Spectral Transmission ◽

Grey Scale ◽

Assessment Of Disease Activity

Background:Valid assessment of disease activity leads to improvement of long-term outcomes in patients with inflammatory arthritis (1). Optical spectral transmission (OST) is a modern diagnostic tool able to assess the blood-specific absorption of light transmitted through a tissue, promising quantification of inflammation in the finger and wrist joints of patients with rheumatoid arthritis (RA) (commercial device: HandScan – Demcon/Hemics, The Netherlands) (2). Even though an increasing number of studies have evaluated diagnostic value of this new technology in RA patients (2,3), no data exist regarding psoriatic arthritis (PsA).Objectives:To examine for the first time the diagnostic value of OST in detecting inflammation in patients with PsA and to evaluate its relationship with disease activity markers and various epidemiological and anthropometric patient characteristics.Methods:OST-Measurements were performed in a group of PsA patients and a group of healthy controls. The difference between OST in the two groups was statistically examined and relationships of OST with clinical (tender / swollen joint counts, disease activity on a visual analogue scale) and serological disease activity markers were evaluated. Moreover, joint ultrasound (US) examinations were performed in a subgroup of PsA patients and OST associations with a Power Doppler- and a Grey Scale-US score were examined. Finally, relationships of OST with various anthropometric and epidemiologic parameters (BMI, hand-size, gender, age) were assessed.Results:We recruited 49 PsA patients [65.3% female; mean age 53.3 years (± 11.8 SD)] and 114 control subjects [77.2% female; mean age 46 years (± 12.8 SD)]. OST was statistically significantly higher in the patient group, compared to the control group [14.95 (12.04 - 17.18, IQR) vs. 10.31 (7.84 – 13.79, IQR); p<0.001]. OST correlated moderately-strongly with both examined US scores (Power Doppler-score: r = 0.5; p = 0.026 and Grey Scale-score: r = 0.52; p = 0.028). Moreover, OST showed a moderate, statistically significant association with C-reactive protein (CRP) (r = 0,298; p = 0,037). Finally, males had significantly higher OST values than females and OST associated moderately-weakly with body mass index (BMI) in the control group (rho = 0.24; p< 0.001).Conclusion:This is the first report of a possible diagnostic value of OST in patients with PsA. OST correlated with ultrasound and serological activity markers and may thus prove to be a useful tool of disease activity assessment, next to well established diagnostic modalities, such as the joint US. Correlations of OST with patient characteristics implicate the need to take also anthropometric and epidemiological patient characteristics into account when interprenting OST results in order to avoid confounding.References:[1]Katchamart W, et al. Systematic monitoring of disease activity using an outcome measure improves outcomes in rheumatoid arthritis. J Rheumatol 2010;37:1411–1415.[2]Triantafyllias, et al. Diagnostic value of optical spectral transmission in rheumatoid arthritis: associations with clinical characteristics and comparison with joint ultrasonography. J Rheumatol 2020 1;47(9):1314-1322.[3]Onna M Van, et al. Assessment of disease activity in patients with rheumatoid arthritis using optical spectral transmission measurements, a non-invasive imaging technique. Ann Rheum Dis 2016;75:511–518.Disclosure of Interests:Konstantinos Triantafyllias Speakers bureau: Pfizer, Novartis, Janssen, Chugai, Stefanie Liverakos: None declared, Claudia Noack: None declared, Andreas Schwarting: None declared

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Diagnostic Value of Vaginal Microecology, Serum miR-18a, and PD-L1 for Identifying HPV-Positive Cervical Cancer

Technology in Cancer Research & Treatment ◽

10.1177/1533033821995281 ◽

2021 ◽

Vol 20 ◽

pp. 153303382199528

Author(s):

Yumei Zhang ◽

Sujuan Qiu ◽

Yueli Guo ◽

Jiaqin Zhang ◽

Xiaoqing Wu ◽

...

Keyword(s):

Cervical Cancer ◽

Area Under The Curve ◽

Serum Levels ◽

Diagnostic Value ◽

High Serum ◽

Control Group ◽

Observation Group ◽

Positive Group ◽

Microbial Dysbiosis ◽

Programmed Death

Objective: We aimed to investigate the diagnostic value of the vaginal microecology, serum miR-18a, and programmed death ligand-1 (PD-L1) for human papillomavirus (HPV)-positive cervical cancer. Methods: Eighty-four patients with HPV-positive cervical cancer were assigned to the observation group, 107 HPV-positive patients without cervical cancer were assigned to the positive group, and 191 healthy women were assigned to the control group. Vaginal microecology and serum levels of miR-18a and PD-L1 on the surface of CD4+ and CD8+ T cells were compared among the 3 groups. The observation group was further divided into subgroups according to patients’ characteristics for comparison. The diagnostic value of miR-18a and PD-L1 for HPV-positive cervical cancer was investigated. Results: Women in the control group had better vaginal microecology and lower levels of miR-18a and PD-L1 than those in the observation and the positive groups (all P < 0.05). Compared with the positive group, the observation group had similar vaginal microecology (all P > 0.05) but higher levels of miR-18a and PD-L1 (all P < 0.05). Moreover, the patients at stage III had higher levels of miR-18a and PD-L1 than those at stage I and II (all P < 0.05). The values of area under the curve for miR-18a and PD-L1 in the diagnosis of HPV-positive cervical cancer were over 0.8 (all P < 0.001). Conclusion: Patients with HPV-positive cervical cancer have vaginal microbial dysbiosis and high serum levels of miR-18a and PD-L1. miR-18a and PD-L1 have diagnostic value for identifying HPV-positive cervical cancer.

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Abstract 16851: ST Segment Variability as a Predictor of Clinical Deterioration in Hypoplastic Left Heart Syndrome

Circulation ◽

10.1161/circ.130.suppl_2.16851 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Eric L Vu ◽

Craig G Rusin ◽

Dan J Penny ◽

Kathy K Kibler ◽

Ronald B Easley ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Clinical Deterioration ◽

Cardiac Conduction ◽

Control Group ◽

High Risk Population ◽

Left Heart ◽

Hypoplastic Left Heart ◽

St Segment ◽

Icu Discharge ◽

Left Heart Syndrome

Introduction: The interstage mortality rate for hypoplastic left heart syndrome (HLHS) is high at 12%. Modalities to predict clinical decline would be useful in this high risk population. Due to cardiac conduction system anomalies in HLHS, electrocardiogram (ECG) monitoring of the ST segment is confounded. Our aim is to develop a monitor of ST variability that predicts deterioration despite the presence of an abnormal ST segment baseline. Hypothesis: Increased ST variability is associated with clinical deterioration in HLHS. Methods: A prospective, observational study was conducted at Texas Children’s Hospital using interstage recordings of 5 lead ECG. From January 2013 to January 2014, 25 subjects were admitted with HLHS; 21 had requisite ECG data for inclusion. In 11 subjects, there were 17 deterioration events, defined as rapid response team (RRT) activation. Events included 13 instances of respiratory failure with intubation and 4 instances of chest compressions. The control group included 10 subjects with no deterioration. An approach to quantify variability was developed where the ST segment vector was resolved using 3 orthogonal leads (II, V5, and aVF) and variability quantified with two metrics: 1) Displacement: distance between ST vector tips at 30 s intervals and 2) Component range: sum of ST vector tip movement in 3 dimensions over 10 min intervals. Comparison was made with the four hour window prior to RRT activation in the deterioration group against the four hour window prior to ICU discharge in the control group. Results: Four hours prior to clinical deterioration, increased ST variability was noted in both methods. For the 30 s displacement method, subjects with deterioration had a median value of 0.059 mm [IQR: 0.039 - 0.075] compared with 0.037 mm [IQR: 0.025 - 0.053] in the control group (p = 0.023). For the 10 min component range method, subjects with deterioration had a median value of 3.0 mm [IQR: 1.8 - 4.5] compared with 1.9 mm [IQR: 1.0 - 2.8] in the control group (p = 0.031). Conclusions: ST variability is increased in HLHS subjects prior to deterioration. Although conduction abnormalities limit the utility of ST monitoring in this population, quantification of ST variability may have a role in advanced systems to monitor clinical deterioration.

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The Clock Drawing Test: Performance differences between the free-drawn and incomplete-copy versions in patients with MCI and dementia

Dementia & Neuropsychologia ◽

10.1590/s1980-5764-2016dn1003009 ◽

2016 ◽

Vol 10 (3) ◽

pp. 227-231 ◽

Cited By ~ 2

Author(s):

Bárbara Costa Beber ◽

Renata Kochhann ◽

Bruna Matias ◽

Márcia Lorena Fagundes Chaves

Keyword(s):

Cognitive Impairment ◽

Test Performance ◽

Diagnostic Value ◽

Control Group ◽

Cognitive Screening ◽

Scoring Method ◽

Clock Drawing Test ◽

Clock Drawing ◽

Healthy Elderly ◽

Drawing Test

ABSTRACT Background: The Clock Drawing Test (CDT) is a brief cognitive screening tool for dementia. Several different presentation formats and scoring methods for the CDT are available in the literature. Objective: In this study we aimed to compare performance on the free-drawn and "incomplete-copy" versions of the CDT using the same short scoring method in Mild Cognitive Impairment (MCI) and dementia patients, and healthy elderly participants. Methods: 90 participants (controlled for age, sex and education) subdivided into control group (n=20), MCI group (n=30) and dementia group (n=40) (Alzheimer's disease - AD=20; Vascular Dementia - VD=20) were recruited for this study. The participants performed the two CDT versions at different times and a blinded neuropsychologist scored the CDTs using the same scoring system. Results: The scores on the free-drawn version were significantly lower than the incomplete-copy version for all groups. The dementia group had significantly lower scores on the incomplete-copy version of the CDT than the control group. MCI patients did not differ significantly from the dementia or control groups. Performance on the free-drawn copy differed significantly among all groups. Conclusion: The free-drawn CDT version is more cognitively demanding and sensitive for detecting mild/early cognitive impairment. Further evaluation of the diagnostic value (accuracy) of the free-drawn CDT in Brazilian MCI patients is needed.

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Hedgehog Signaling is Associated with Liver Response to Fractionated Irradiation in Mice

Cellular Physiology and Biochemistry ◽

10.1159/000452543 ◽

2016 ◽

Vol 40 (1-2) ◽

pp. 263-276 ◽

Cited By ~ 3

Author(s):

Jieun Kim ◽

Sihyung Wang ◽

Jeongeun Hyun ◽

Cynthia D. Guy ◽

Youngmi Jung

Keyword(s):

Murine Model ◽

Hedgehog Signaling ◽

Control Group ◽

Late Response ◽

Response Model ◽

Fractionated Irradiation ◽

Control Groups ◽

Radiation Induced ◽

Histomorphological Changes ◽

Liver Response

Background/Aims: Radiation-induced liver disease (RILD) is a major obstacle in treating liver cancer; however, the mechanisms underlying RILD development remain unclear. Hedgehog (Hh) orchestrates liver response to injury. Herein, we investigated the liver response with Hh to fractionated irradiation (FI) using a small murine model for RILD. Methods: Male mice exposed to liver-targeted FI with 6Gy in 5 consecutive weekly fractions were sacrificed at one day after weekly irradiation and 6 or 10 weeks post 5th FI for the acute and late response model, respectively. Results: The levels of ALT/AST and apoptosis were elevated in all radiation groups. The expression of Hh ligand, Sonic and Indian Hh, and Hh activator, smoothened and gli2, was higher in the acute groups than the control group. Pro-fibrogenic markers were also up-regulated in this model compared with the control group. Histomorphological changes and ballooned hepatocytes were observed in the late response model. Both the expression of Hh and profibrotic genes and the fibrosis level increased in this model compared with the control groups. Conclusion: Enhanced Hedgehog signaling and liver injury with fibrosis in RILD murine model suggests hedgehog as the potential regulator in RILD progression and the suitability of this model for studying RILD.

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The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with Granulomatosis with Polyangiitis

International Journal of Rheumatology ◽

10.1155/2016/7831410 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 8

Author(s):

M. Y. Pervakova ◽

V. L. Emanuel ◽

O. N. Titova ◽

S. V. Lapin ◽

V. I. Mazurov ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Laboratory Data ◽

Clinical Information ◽

Diagnostic Value ◽

Control Group ◽

Serum Samples ◽

Chronic Lung Diseases ◽

Alpha 1 Antitrypsin Deficiency ◽

Group 2 ◽

Alpha 1 Antitrypsin

The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener’s granulomatosis). The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS), incidence of lung involvement, anti-proteinase 3 (PR3) antibodies concentrations, and other laboratory data were collected in 38 GPA patients. We also studied serum samples obtained from 46 healthy donors. In all collected samples A1AT phenotyping by isoelectrofocusing (IEF) and turbidimetric A1AT measurement were performed. Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%). The mean A1AT concentration in samples with atypical A1AT phenotypes was significantly lower (P=0.0038) than in normal A1AT phenotype. We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration. We conclude that A1AT deficiency should be considered in all patients with GPA.

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