Clinical and morphological phenotypes in intrathoracic sarcoidosis

Aim. To study clinical and morphological phenotypes in different variants of the course of intrathoracic sarcoidosis and isolate new phenotypes.Materials and methods. The study included 121 patients with intrathoracic sarcoidosis aged 21–66 years (50.4% were men, 49.6% were women, the average age at the time of the disease onset was 38 years) over the period 2007– 2019. During the examination, patients’ complaints were studied thoroughly, and the diagnosis was histologically verified in all cases. During an extended histological examination, the quantitative and qualitative composition of biopsy specimens was investigated. The number of granulomas in the field of vision and the content of giant cells, macrophages, lymphocytes, neutrophils, and eosinophils in them were studied. Qualitative parameters were assessed for the presence of hyalinosis, Schaumann bodies, necrosis, stamping, calcification, fibrosis, and vasculitis. All patients were retrospectively divided into two clinical groups depending on the outcomes of the disease: group 1 included patients with a favorable course of sarcoidosis, proceeding without relapses and signs of progression; group 2 encompassed patients with an unfavorable course of the disease with relapses and progression, requiring long-term administration of systemic glucocorticoids.Results. The analysis showed that among all general clinical manifestations, only the presence of dyspnea, skin manifestations, and weight loss occurred significantly more often in the patients with an unfavorable course of intrathoracic sarcoidosis (р = 0.04; 0.02; and 0.01, respectively). Among morphological parameters, a large number of macrophages was significantly more frequent in the biopsy specimens in this group of patients (р < 0.01).

Download Full-text

This Is a Title : Long Term succsesfull Prophylaxis in 190 Patients with Severe Congenital Factor XIII Deficiency, a Four Years Experience. Here Is the Sub-Title: Succsefull Prophylaxis in Factor XIII Deficiency

Blood ◽

10.1182/blood.v124.21.2851.2851 ◽

2014 ◽

Vol 124 (21) ◽

pp. 2851-2851

Author(s):

Majid Naderi ◽

Akbar Dorgalaleh ◽

Shadi Tabibian ◽

Shaban Alizadeh ◽

Maryam Sadat Hosseini ◽

...

Keyword(s):

Factor Xiii ◽

Clinical Manifestations ◽

Bleeding Diathesis ◽

Factor Xiii Deficiency ◽

Before And After ◽

Bleeding Episodes ◽

Group 2 ◽

Fxiii Activity ◽

Group 1

Abstract Background: factor XIII deficiency (FXIIID) is an extremely rare bleeding disorder with an estimated incidence of 1 per 1 to 3 million that transmitted in an autosomal recessive manner. The disorder is characterized by severe bleeding diathesis, impaired wound healing and recurrent spontaneous miscarriage. Sistan and Baluchistan Province in south east of Iran with high rate of consanguineous marriages has the highest incidence of FXIIID around the world. The aim of this study was to evaluate clinical manifestations and prophylaxis therapy in FXIII deficient patients with life threatening complications and to assess the risk of FXIII inhibitor development in patients with long term prophylaxis. We also investigated prophylaxis treatment in neonates with FXIIID. Methods: This study was conducted on 190 patients with FXIIID from southeast of Iran. Diagnosis of FXIIID was based on factor activity and molecular investigation of Trp187Arg which is the only reported mutation of FXIII in this region. We also selected some cases for sequencing to confirm the molecular results.Patients underwent regular prophylaxis and the efficacy of prophylaxis was assessed by prospective follow-up in duration of 4 years. The frequency of bleeding episodes as described by Tosetto et al. was recorded before and after prophylaxis each month. Patients with Intracranial hemorrhage (ICH) received Fibrogammin P® in a dose of 30 IU/Kg every 4 day as prophylaxis and in a dose of 10-26 IU/kg when ICH occurred. Patients with miscarriage underwent regular prophylaxis with FXIII concentrate with a dose of 10 IU/Kg every 2 weeks during pregnancy and also received the same dose as prophylaxis before gestation in 4 week intervals.In cases that had a history of prophylaxis for more than 4 years or had more than 50 injections, development of FXIII inhibitor was investigated by Bethesda assay. We evaluated plasma FXIII activity and FXIII inhibitor in day 28 after the last prophylaxis administration by Cobas Mira device. This assay was performed on a large amount of population for the first time. If the result of inhibitor was negative the test was replicated with 3 dilutions.We also enrolled 34 neonates with FXIIID to the study and divided them into two groups. Group 1 (17 neonates) received a standard dose of Fibrogammin (10-26 IU/Kg) while group 2 (17 neonates) received a dose of 60-80 IU/Kg. We followed both groups for 36 months and the frequency of bleeding episodes was recorded for each group and compared using independent t-test. Neonates in group 2 were assessed for thrombotic events promptly after administration of Fibrogammin P®, the following day and one week later. Results: Plasma FXIII activity in all patients was undetectable which is suggestive of severe deficiency. Genetic analysis revealed a homozygous Trp187Arg mutation in all patients and it was in agreement with the sequencing results of selected cases. Clinical manifestation of patients and the bleeding score of different diathesis before and after prophylaxis are indicated in table 1. Forty nine patients had the defined criteria for investigation of FXIII inhibitor development. Among them 14 had experienced ICH. Despite long term prophylaxis in these patients none of them was detected to develop FXIII inhibitors. No patient with ICH experienced this phenomenon after prophylaxis treatment. The majority of patients with miscarriage only experienced this diathesis once (62.5%). The prophylaxis program was successful in management of pregnancy in all 8 patients. Comparison of two neonate groups revealed that bleeding episodes in group 2 were significantly lower than group 1 (p<0.05). We did not observe any thrombotic event in group 2, which is an important finding in safety of Fibrogammin P® also in prevention of potential loss of drug. Conclusion: Fibrogammin P® is effective in the management of FXIIID and surprisingly long term prophylaxis in the patients did not lead to development of FXIII inhibitor. Furthermore higher dose of Fibrogammin P® is safe and effective in neonates. Table 1. Clinical manifestations and Tosetto bleeding scores of different bleeding diathesis before and after prophylaxis. Number of affected patients (%) Score before prophylaxis Score after prophylaxis Umbilical bleeding 157(82.5) 12 3 Hematoma 101 (53) 12 2 ICH 32(17%) 14 3 Epistaxis 26(14%) 11 2 Ecchymosis 25 (12.5%) 12 2 Hemarthrosis 7 (4%) 13 2 Miscarriage 8 (5%) - - Disclosures No relevant conflicts of interest to declare.

Download Full-text

Direct Pharmacological Correction of Oxidative Stress in Rat Kidneys Does Not Facilitate Diabetic Nephropathy

International Journal of Biomedicine ◽

10.21103/article11(3)_oa8 ◽

2021 ◽

Vol 11 (3) ◽

pp. 296-300

Author(s):

A. Yu. Zharikov ◽

S.O. Filinova ◽

O. N. Mazko ◽

O. G. Makarova ◽

I. P. Bobrov

Keyword(s):

Free Radical Oxidation ◽

Renal Tissue ◽

Alpha Tocopherol ◽

Citrate Buffer ◽

Radical Oxidation ◽

Term Administration ◽

Male Wistar Rats ◽

Group 2 ◽

Group 1

The aim of this study was to evaluate the effect of alpha-tocopherol acetate (ATA) on the activity of free-radical oxidation (FRO) in renal tissue and renal function in rats with experimental streptozotocin (STZ)-induced diabetes mellitus (DM). Methods and Results: Experiments were conducted on 22 male Wistar rats aged 60-100 days and weighing 250-300g. The animals were divided into two groups (Group 1 (control) and Group 2 (experimental. To induce DM, the animals were injected intraperitoneally 1ml of STZ solution in the citrate buffer at a dose of 65mg/kg. For more selective modeling of type 2 DM, the rats were previously injected with an intraperitoneal solution of cytoflavin based on a nicotinamide dose of 115mg/kg In Croup 2, ATA was administered in the period from the fifth to eighth weeks, inclusive, intragastrically through a tube at a daily dose of 300mg/kg. Experiments showed that after a 4-week course of ATA, the concentration of thiobarbiturate-reactive products in the kidney tissues of the rats in Group 2 was 5.3 times lower than in Group 1. The activity of all antioxidant enzymes did not differ between the two groups. In both groups, during all 8 weeks of the experiment, the levels of renal excretion of glucose, protein, and creatinine significantly exceeded the initial level, while the level of diuresis remained stable. Conclusion: The long-term administration of ATA in experimental streptozotocin (STZ)-induced DM is accompanied by a significant suppression of the activity of the FRO processes in the kidneys, but does not lead to an improvement in the course of DN.

Download Full-text

AB1047 IgG4-RELATED DISEASE PRESENTATION REQUIRES ADMISSION TO THE EMERGENCY DEPARTMENT IN THE MAJORITY OF CASES

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2919 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1814.1-1815

Author(s):

G. Mancuso ◽

E. Della Torre ◽

M. Lanzillotta ◽

G. A. Ramirez ◽

L. Dagna

Keyword(s):

Emergency Department ◽

Disease Onset ◽

Diagnostic Delay ◽

Clinical Manifestations ◽

General View ◽

Related Disease ◽

Acute Presentation ◽

Igg4 Related Disease ◽

Group 2 ◽

Group 1

Background:IgG4-related disease (IgG4-RD) is generally considered a chronic fibro-inflammatory condition with insidious presentation and subclinical course. Our clinical experience, however, suggests that a sizable proportion of patients experience multiple accesses to the emergency department (ED), either at disease onset or during the disease course.Objectives:In the present study we aimed (i) to assess the prevalence of acute manifestations of IgG4-RD at disease onset requiring referral to the ED, and (ii) to calculate the diagnostic delay from the initial acute presentation.Methods:We revised our database and identified patients admitted to the ED because of symptoms lately attributed to IgG4-RD onset (Group 1) and those that were referred to our outpatient clinic without previous urgent manifestations (Group 2). Acute manifestations were clustered based on the anatomical district affected by IgG4-RD. Epidemiological, clinical, and serological features of Group 1 and Group 2 were compared.Results:The study included 141 patients with IgG4-RD. 76 (54%) presented to the ED at disease onset. The most common clinical manifestations requiring admission to the ED were jaundice (53%), abdominal pain (41%), and fever (10%). Gastrointestinal involvement was the most frequent cause of referral to the ED (71% of cases), followed by involvement of the retroperitoneum (14.5%), and of the nervous system (6.6%). Pancreato-biliary involvement was significantly more frequent in Group 1. Head, neck, salivary and lacrimal gland involvement was more frequent in Group 2. The diagnostic delay was significantly shorter in Group 1 than in Group 2.Conclusion:Clinical manifestations associated with IgG4-RD onset require referral to the ED in the majority of cases. This finding contrasts with the general view of IgG4-RD as a condition with non-acute presentation.References:[1]Bledsoe JR, Della-Torre E, Rovati L, Deshpande V. IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. APMIS. 2018;126:459-476.[2] Della-Torre E, Lanzillotta M, Doglioni C. Immunology of IgG4-related disease. Clin Exp Immunol.2015;181:191-206.[3]Lanzillotta M, Campochiaro C, Trimarchi M, Arrigoni G, Gerevini S, Milani R, et al. Deconstructing IgG4-related disease involvement of midline structures: Comparison to common mimickers. Mod Rheumatol. 2017;27:638-645.[5]Della-Torre E, Stone JH. “How I manage” IgG4-Related Disease. J Clin Immunol. 2016;36:754-763.[6]Perugino CA, Mattoo H, Mahajan VS, Maehara T, Wallace ZS, Pillai S, et al. Emerging Treatment Models in Rheumatology: IgG4-Related Disease: Insights Into Human Immunology and Targeted Therapies. Arthritis Rheumatol. 2017;69:1722-1732.[7]Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539-51.[8]Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385:1460-71[9]Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21-30.Disclosure of Interests:Gaia Mancuso: None declared, Emanuel Della Torre: None declared, Marco Lanzillotta: None declared, Giuseppe Alvise Ramirez: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Download Full-text

POS0888 EPIDEMIOLOGICAL AND CLINICAL DIFFERENCES BETWEEN ANTI-MDA5 PHENOTYPES: DATA FROM A LARGE COHORT (MEDRA5) STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3837 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 700.1-700

Author(s):

E. Trallero-Araguás ◽

F. Romero ◽

I. Castellví ◽

V. Ortiz-Santamaria ◽

S. Castañeda ◽

...

Keyword(s):

Internal Medicine ◽

Statistical Significance ◽

Disease Onset ◽

Amyopathic Dermatomyositis ◽

Clinically Amyopathic Dermatomyositis ◽

San Sebastian ◽

Group 3 ◽

Group 2 ◽

Hospital General ◽

Group 1

Background:Idiopathic inflammatory myopathies are a heterogenous group of systemic autoimmune diseases. Several phenotypes have been linked to specific autoantibodies. Clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease, the most severe form of ILD, is associated with the anti-MDA5 antibodies. However not all the patients with dermatomyositis and anti-MDA5 positive antibodies develop this severe condition.Objectives:We aim to define different phenotypes from a large cohort of patients diagnosed with dermatomyositis who were positive to anti-MDA5 antibodies.Methods:We retrospective analyzed the clinical and immunological data of 90 anti-MDA5 patients [50 female, 55.6%, mean (SD) age at diagnosis 47 (15.4) yrs.] with dermatomyositis recruited from a multicenter register in Spain (MEDRA5) including 30 hospitals. All the patients fulfill de International Myositis Classification Criteria (EULAR/ACR) for dermatomyositis (score >90%). Anti-MDA5 were detected by means of commercial immunoblot (EUROIMMUN©). The chi-square test was used to assess the relationships between qualitative variables. The Kruskal-Wallis test was used to compared medians between groups.Results:Sixty-six patients (73.3%) were diagnosed with clinically amyopathic dermatomyositis. Three different phenotypes linked with the anti-MDA5 antibody were identified. Group 1: patients with rapidly-ILD phenotype (28 patients, 31.1%), group 2: antisynthetase-like phenotype (23 patients, 25.5%), and group 3: non-ILD phenotype (39 patients, 43.3%). Clinical and immunological comparison between the groups disclosed that age at disease onset was higher (median, IQR) in patients from group 1 [53 (43-60)] vs. group 2 [46 (40-56)] or group 3 [42(41-51)] (p=0.01); disease onset was more frequent in spring in patients from group 1 (46.5%) than in the rest of the groups (21.7% and 28.9%) (p<0.01). Cancer was detected in 7 patients, only associated with myositis in 3 cases (3 years interval between cancer and dermatomyositis) without significant differences between phenotypes. Vasculitis (one case ANCA positive) was detected in 9 cases (6 limited to skin, 1 renal and 1 intestinal), 6 of them in the group 3 (statistical significance, in comparison with group 1 and 2, p<0.01). Mortality rate was higher in group 1 (51.9%, 16 out of 17 due to refractory respiratory failure) vs group 2 (12.5%) or 3 (0%) (p<0.001). Anti Ro52 positivity was more frequent in group 1 (65.4%) vs. group 2 (25%) or 3 (35.5%) (p<0.017), although it did not reach statistical significance in terms of mortality (p=0.173) or patients admitted in the intensive care unit (p=0.173). Mechanic hands were more frequent in group 2 (40.6%) than in groups 1 (25%) and 3 (34.4%) (p=0.05). Fever was significantly most frequent in group 1(52.6%) than in group 2 (21.1%) and 3 (26.3%) (p=0.001). Other clinical or immunological features such as arthritis, myositis, or the number of characteristic skin lesions among others were not more frequent in one group or another.Conclusion:Three different phenotypes of patients positive to anti-MDA5 were identified. The presence or not of ILD, or the different type (rapidly progressive or not) of ILD were the main feature that allow to differentiate these phenotypes, which are relevant in clinical practice.References:[1]Allenbach Y, Uzunhan Y, Toquet S, et al; French Myositis Network. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020;95: e70-e78.Acknowledgements:List of contributors of MEDRA5 group: Aguilar-García J (Internal Medicine, Hospital Costa del Sol, Marbella), Carrión-Barberá I (Rheumatology, Hospital del Mar, Barcelona), Cobo-Ibañez T (Rheumatology, Hospital Infanta Sofía, San Sebastián de los Reyes), de Escalante-Yangüela B (Internal Medicine, Hospital Clínico Lozano Blesa, Zaragoza), Fonseca-Aizpuru EM (Internal Medicine, Hospital de Cabueñes, Gijón), González-Cubillo L (Intensive Medicine, Hospital Universitario de Cruces, Barakaldo), González-Gay MA (Rheumatology, Hospital Marqués de Valdecilla, Santander), Prieto-González S (Internal Medicine, Hospital Clinic, Barcelona), Ruiz-Román A (Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla), Calero-Paniagua I (Internal Medicine, Hospital Virgen de la Luz, Cuenca), Callejas-Rubio JL (Internal Medicine, Hospital Clínico San Cecilio, Granada), Gil-Vila A (Internal Medicine, Hospital Vall d’Hebron, Barcelona), de Miguel-Campo B (Internal Medicine, Hospital Doce de Octubre, Madrid), García-Sevilla R (Pneumology, Hospital General Universitario de Alicante, Alicante), Iriarte-Fuster A (Internal Medicine, Hospital de Bellvitge, Hospitalet de Llobregat), Jovani-Casano V (Rheumatology, Hospital General Universitario de Alicante, Alicante), Lozano-Rivas N (Rheumatology, Hospital Virgen de la Arritxaca, Murcia), Martín-Gascón M (Internal Medicine, Hospital Morales Meseguer, Murcia), Martinez-González O (Rheumatology, Hospital Universitario de Salamanca, Salamanca), Monteagudo-Jiménez M (Internal Medicine, Hospital Parc Taulí, Sabadell), Mora-Ortega GM (Pneumology, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes), Moral-Moral Pedro (Internal Medicine, Hospital Universitari i Politecnic La Fe, Valencia), Pérez-De Pedro I (Interna Medicine, Hospital Regional Universitario de Málaga, Málaga), Picazo-Talavera MR (Rheumatology, Hospital del Sureste, Madrid), Rubio-Rivas M (Internal Medicine, Hospital de Bellvitge, Hospitalet de Llobregat)Disclosure of Interests:None declared

Download Full-text

Prognostic value of mitral regurgitation in patients with asymmetric hypertrophic cardiomyopathy

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.394 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

M Tesic ◽

L Travica ◽

V Giga ◽

D Trifunovic ◽

I Jovanovic ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Mitral Valve ◽

Mitral Regurgitation ◽

Prognostic Value ◽

Volume Index ◽

Common Finding ◽

Adequate Treatment ◽

Group 2 ◽

Group 1

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Since mitral regurgitation (MR) is a very common finding in patients with hypertrophic cardiomyopathy (HCM), the evaluation of the mitral valve anatomy and the degree of MR is of utmost importance in this population. However, data regarding the prognostic value of different degrees of MR in HCM remains scarce. Purpose The aim of this study was to determine whether the presence of a higher degree of MR affects: 1) long term prognosis; 2) clinical and echocardiographic presentation of HCM patients. Material and Methods We included prospectively 102 patients, diagnosed with primary asymmetric HCM. The degree of MR was determined echocardiographicaly according to current recommendations of the American Association of Echocardiography. According to the MR severity, patients were divided into 2 groups: Group 1 (n = 52) with no/trace or mild MR and Group 2 with moderate or moderate to severe MR. All patients had clinical and echocardiographic examination, 24-hour Holter ECG and NT pro BNP analysis performed. The primary outcome was a composite of: 1) HCM related death or sudden death; 2) hospitalization due to acute heart failure; 3) sustained ventricular tachycardia; 4) ischemic stroke. Results Patients with higher MR degree had more frequent chest pain (p = 0.039), syncope (p = 0.041) and NYHA II functional class (p < 0.001). Group 2 patients had mostly obstructive form of HCM (p < 0.001) with more frequent presence of previous atrial fibrillation (AF) (p = 0.032), as well as the new onset of AF (p = 0.014) compared to patients in Group 1. Patients with higher MR degree had significantly more SAM (p < 0.001) resulting in a more frequent eccentric MR jet (p < 0.001), along with calcified mitral annulus (p = 0.007), enlarged left atrial volume index (p < 0.001), and elevated right ventricular pressure (p = 0.001). As a result of higher MR grade, Group 2 had higher E/e" values (p < 0.001), elevated LV filling pressure (lateral E/e’ >10), as well as higher levels of NT pro BNP (p = 0.001). By Kaplan-Meier analysis we demonstrated that the event free survival rate during follow up of median 75 (IQR 48-103) months was significantly higher in Group 1 compared to the Group 2 (79% vs. 46%, p < 0.001), Figure 1. After adjustment for relevant confounders, moderate/moderate to severe MR remained as an independent predictor of adverse outcome (hazard ratio 2.58, 95% CI: 1.08-6.13, p < 0.001). Conclusion Presence of moderate, or moderate to severe MR was associated with poor long-term outcome of HCM patients. These results indicate the importance of an adequate MR assessment and detailed evaluation of the mitral valve anatomy in the prediction of complications and adequate treatment of patients with HCM. Abstract Figure.

Download Full-text

Longitudinal Reproducibility of CO2-Triggered BOLD MRI for the Hemodynamic Evaluation of Adult Patients with Moyamoya Angiopathy

Cerebrovascular Diseases ◽

10.1159/000513301 ◽

2021 ◽

pp. 1-7

Author(s):

Constantin Roder ◽

Uwe Klose ◽

Helene Hurth ◽

Cornelia Brendle ◽

Marcos Tatagiba ◽

...

Keyword(s):

Intraclass Correlation ◽

Collateral Flow ◽

Surgical Revascularization ◽

Bold Mri ◽

Promising Tool ◽

Hemodynamic Evaluation ◽

Group 2 ◽

Group 1

Background and Purpose: Hemodynamic evaluation of moyamoya patients is crucial to decide the treatment strategy. Recently, CO2-triggered BOLD MRI has been shown to be a promising tool for the hemodynamic evaluation of moyamoya patients. However, the longitudinal reliability of this technique in follow-up examinations is unknown. This study aims to analyze longitudinal follow-up data of CO2-triggered BOLD MRI to prove the reliability of this technique for long-term control examinations in moyamoya patients. Methods: Longitudinal CO2 BOLD MRI follow-up examinations of moyamoya patients with and without surgical revascularization have been analyzed for all 6 vascular territories retrospectively. If revascularization was performed, any directly (by the disease or the bypass) or indirectly (due to change of collateral flow after revascularization) affected territory was excluded based on angiography findings (group 1). In patients without surgical revascularization between the MRI examinations, all territories were analyzed (group 2). Results: Eighteen moyamoya patients with 39 CO2 BOLD MRI examinations fulfilled the inclusion criteria. The median follow-up between the 2 examinations was 12 months (range 4–29 months). For 106 vascular territories analyzed in group 1, the intraclass correlation coefficient was 0.784, p < 0.001, and for group 2 (84 territories), it was 0.899, p < 0.001. Within the total follow-up duration of 140 patient months, none of the patients experienced a new stroke. Conclusions: CO2 BOLD MRI is a promising tool for mid- and long-term follow-up examinations of cerebral hemodynamics in moyamoya patients. Systematic prospective evaluation is required prior to making it a routine examination.

Download Full-text

Adiponectin and its receptors in patients with cardiovascular disease

European Journal of Preventive Cardiology ◽

10.1093/eurjpc/zwab061.238 ◽

2021 ◽

Vol 28 (Supplement_1) ◽

Author(s):

E Belik ◽

OV Gruzdeva ◽

YUA Dyleva ◽

EG Uchasova ◽

MYU Sinitsky ◽

...

Keyword(s):

Heart Defects ◽

Post Translational Modification ◽

Fat Depots ◽

Funding Sources ◽

Adiponectin Mrna ◽

Fat Stores ◽

Group 2 ◽

High Level ◽

Group 1

Abstract Funding Acknowledgements Type of funding sources: None. Aim to determine the features of adiponectin expression, secretion of adiponectin and its receptors in local fat depots in CVD. Materials and methods The study included 90 patients with СAD (Group 1) and 60 patients with heart defects (Group 2). Adipocytes were isolated from samples of subcutaneous (SAT), epicardial (EAT) and perivascular (PVAT) adipose tissue obtained during CABG or heart valve replacement. The expression of adiponectin was determined by qPCR using TaqManTM Gene Expression Assays (Applied Biosystems, USA) in the ViiA 7 Real-Time PCR System (Applied Biosystems, USA), the levels of expression products was determined using enzyme immunoassay (Bender MedSystems GmbH, Vienna, Austria). The data were analyzed using the statistical software Statistica 9.0. Results EAT adipocytes were characterized by the lowest adiponectin expression relative to adipocytes of other localization both in Group 1 and Group 2. In patients Group 1 adiponectin expression in EAT was reduced relative in SAT and PVAT (by 1.2 and 1.5 times). In Group 2, the adiponectin mRNA in the EAT was lower than in the SAT and PVAT (1.4 and 1.5 times). The expression of adiponectin in EAT in Group 2 exceeded the same indicator in Group 1 by 1.2 times. The maximum expression of adiponectin was observed in the PVAT culture in patients of both groups. For Group 2, this indicator exceeded the values of Group 1 by 1.2 times. The content of adiponectin in the culture EAT was lower than in the SAT, both in Group 1 and Group 2 (by 1.3 and 1.13 times). The level of this indicator in Group 2 was 1.4 times higher than in Group 1. PVAT adipocytes of patients with CAD were characterized by the lowest level of adiponectin secretion in comparison with adipocytes of other localization. The adiponectin level in the PVAT of Group 2 exceeded that of fat stores of other localization and in Group 1 patients by 1.8 times. There were no statistically significant differences in the expression and concentration of adiponectin in the culture of adipocytes of the SAT between the groups of patients. In Group 1, the lowest level of AdipoR1 was found in the adipocyte culture of the PVAT. Noteworthy is the decrease in the level of AdipoR1 in Group 1 compared to the level of Group 2, observed in the SAT and PVAT: 1.3 and 1.5 times. There were no significant differences in the concentration of the AdipoR1 in the EAT, as well as AdipoR2 in all types of AT between the groups of patients. Conclusion: in CVD the EAT is characterized by minimal expression and secretion of adiponectin, regardless of nosology. In CAD despite the high level of expression of adiponectin, the adipocytes of the PVAT were found to have the lowest content in comparison with adipocytes of other localization. Dysregulation of the adiponectin/AdipoR axis is observed in PVAT, which may be due to low expression of adiponectin receptors and long-term processes of its post-translational modification and oligomerization in CAD.

Download Full-text

Does Presurgical Nasoalveolar Molding Have a Long-Term Effect on Nasal and Upper Airway Dimensions?

The Cleft Palate-Craniofacial Journal ◽

10.1177/1055665620980234 ◽

2020 ◽

pp. 105566562098023

Author(s):

Ashwina S. Banari ◽

Sanjeev Datana ◽

Shiv Shankar Agarwal ◽

Sujit Kumar Bhandari

Keyword(s):

Upper Airway ◽

P Value ◽

Airway Patency ◽

The Mean ◽

Group 2 ◽

Acoustic Pharyngometry ◽

Nasoalveolar Molding ◽

Area And Volume ◽

Group 1

Objectives: To compare nasal and upper airway dimensions in patients with cleft lip and palate (CLP) who underwent nasoalveolar molding (NAM) with those without NAM during infancy using acoustic pharyngometry and rhinometry. Materials and Methods: Eccovision acoustic pharyngometry and rhinometry (Sleep Group Solutions) was used for assessment of mean area and volume of nasal and upper airway in patients with complete unilateral CLP (age range 16-21 years) treated with NAM (group 1, n = 19) versus without NAM (group 2, n = 22). Results: The mean nasal cross-sectional areas and volume were higher in group 1 compared to group 2 on both cleft ( P value <.001) and noncleft side ( P value >.05). The mean area and volume of upper airway were also significantly higher in group 1 compared to group 2 ( P value <.05). Conclusions: Nasoalveolar molding being one of the first interventions in chronology of treatment of patients with CLP, its long-term outcome on nasal and upper airway patency needs to be ascertained. The results of the present study show that the patients with CLP who have undergone NAM during infancy have better improvement in nasal and upper airway patency compared with those who had not undergone NAM procedure. The basic advantages of being noninvasive, nonionizing and providing dynamic assessment of nasal and upper airway patency make acoustic pharyngometry and rhinometry a diagnostic tool of choice to be used in patients with CLP.

Download Full-text

Chronic kidney disease stage stratifies short- and long-term outcomes after aortic root replacement

Interactive Cardiovascular and Thoracic Surgery ◽

10.1093/icvts/ivaa320 ◽

2020 ◽

Author(s):

Tsuyoshi Yamabe ◽

Yanling Zhao ◽

Paul A Kurlansky ◽

Suzuka Nitta ◽

Saveliy Kelebeyev ◽

...

Keyword(s):

Chronic Kidney Disease ◽

Kidney Disease ◽

Confidence Interval ◽

Aortic Root Replacement ◽

Mortality Hazard ◽

Group 3 ◽

Group 2 ◽

Mortality Hazard Ratio ◽

Group 1

Abstract OBJECTIVES Chronic kidney disease (CKD) is prevalent in patients undergoing cardiovascular surgery, and it negatively impacts procedural outcomes; however, its influence on the outcomes of aortic surgery has not been well studied. This study aims to elucidate the importance of CKD on the outcomes of aortic root replacement (ARR). METHODS Patients who underwent ARR between 2005 and 2019 were retrospectively reviewed (n = 882). Patients were divided into 3 groups based on the Kidney Disease: Improving Global Outcomes criteria: Group 1 [estimated glomerular filtration rate (eGFR) ≥ 60 ml/min/1.73 m2, n = 421); Group 2 (eGFR = 30–59 ml/min/1.73 m2, n = 424); and Group 3 (eGFR < 30 ml/min/1.73 m2, n = 37). To reduce potential confounding, a propensity score matching was also performed between Group 1 and the combined group of Group 2 and Group 3. The primary end point was 10-year survival. Secondary end points were in-hospital mortality and perioperative morbidity. RESULTS Severe CKD patients presented with more advanced overall chronic and acute illnesses. Kaplan–Meier analysis showed a significant correlation between CKD stage and 10-year survival (log-rank P < 0.001). The number of events for Group 1 was 15, Group 2 was 49 and Group 3 was 11 in 10 years. Group 3 had significantly higher in-hospital mortality (13.5% vs 3.5% in Group 2 vs 0.7% in Group 1, P < 0.001) and stroke (8.1% vs 7.1% vs 1.2%, P < 0.001) as well as introduction to new dialysis (27.0% vs 5.4% vs 1.7%, P < 0.001). eGFR was shown to be an independent predictor of mortality (hazard ratio, 0.98; 95% confidence interval, 0.96–0.99). Comparison between propensity matched groups showed similar postoperative outcomes, and eGFR was still identified as a predictor of mortality (hazard ratio, 0.97; 95% confidence interval, 0.95–0.99). CONCLUSIONS Higher stage in CKD negatively impacts the long-term survival in patients who are undergoing ARR.

Download Full-text

The Systematic Effect of Mesenchymal Stem Cell Therapy in Critical COVID-19 Patients: A Prospective Double Controlled Trial

Cell Transplantation ◽

10.1177/09636897211024942 ◽

2021 ◽

Vol 30 ◽

pp. 096368972110249

Author(s):

G Adas ◽

Z Cukurova ◽

K Kart Yasar ◽

R Yilmaz ◽

N Isiksacan ◽

...

Keyword(s):

Growth Factors ◽

Critically Ill ◽

Inflammatory Cytokines ◽

Clinical Manifestations ◽

Systematic Effect ◽

Cytokine Storm ◽

Group 3 ◽

Anti Inflammatory ◽

Group 2 ◽

Group 1

The aim of this clinical trial was to control the cytokine storm by administering mesenchymal stem cells (MSCs) to critically-ill COVID-19 patients, to evaluate the healing effect, and to systematically investigate how the treatment works. Patients with moderate and critical COVID-19 clinical manifestations were separated as Group 1 (moderate cases, n = 10, treated conventionally), Group 2 (critical cases, n = 10, treated conventionally), and Group 3 (critical cases, n = 10, treated conventionally plus MSCs transplantation therapy of three consecutive doses on treatment days 0, 3, and 6, (as 3 × 106 cells/kg, intravenously). The treatment mechanism of action was investigated with evaluation markers of the cytokine storm, via biochemical parameters, levels of proinflammatory and anti-inflammatory cytokines, analyses of tissue regeneration via the levels of growth factors, apoptosis markers, chemokines, matrix metalloproteinases, and granzyme-B, and by the assessment of the immunomodulatory effects via total oxidant/antioxidant status markers and the levels of lymphocyte subsets. In the assessment of the overall mortality rates of all the cases, six patients in Group-2 and three patients in Group-3 died, and there was no loss in Group-1. Proinflammatory cytokines IFNγ, IL-6, IL-17A, IL-2, IL-12, anti-inflammatory cytokines IL-10, IL-13, IL-1ra, and growth factors TGF-β, VEGF, KGF, and NGF levels were found to be significant in Group-3. When Group-2 and Group-3 were compared, serum ferritin, fibrinogen and CRP levels in Group-3 had significantly decreased. CD45 +, CD3 +, CD4 +, CD8 +, CD19 +, HLA-DR +, and CD16 + / CD56 + levels were evaluated. In the statistical comparison of the groups, significance was only determined in respect of neutrophils. The results demonstrated the positive systematic and cellular effects of MSCs application on critically ill COVID-19 patients in a versatile way. This effect plays an important role in curing and reducing mortality in critically ill patients.

Download Full-text