scholarly journals P1505 Echocardiographic assessment of different pulmonary hypertension groups

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
M Trepa ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Interventricular Septum ◽  
Pulmonary Vascular Disease ◽  
Eccentricity Index ◽  
Group 4 ◽  
Global Strain ◽  
Group 3 ◽  
Group 2 ◽  
Rv Function ◽  
Group 1

Abstract Introduction Noninvasive echocardiography evaluation of the right ventricle (RV) has been shown to have prognostic value in patients with pulmonary hypertension (PH). Different etiology groups might have different echocardiographic phenotypes. In this study, we aimed to study echocardiographic characterization of the different PH groups and its ability to predict pulmonary vascular disease severity. Methods We collected echocardiographic and right heart catheterization (RHC) data from 97 (75% female, age 65 ± 15 years) consecutive patients referred to an expert tertiary care referral PH centre from 12/2016 to 11/2018. Echocardiographic analysis was performed using Echo-Pac software from GE Healthcare®. Group 3 and 5 were not included in the group comparison analysis due to few patients included. Results Group 2 PH was the most frequent etiology of PH (35), followed by group 1 (26), group 4 (18), group 5 (3) and group 3 (2). The echocardiographic evaluation of this population as a whole showed borderline parameters of RV dysfunction (tricuspid annular plane systolic excursion (TAPSE) 18 ± 4 mm, fractional area change (FAC) 33 ± 10% and S’ tricuspid wave 10 ± 3 cm/sec). Mean RV global strain was -15 ± 5 and RV free wall strain was -17 ± 7. PH group 1 had a significantly lower FAC (26 ± 4%, p = 0.0025), higher eccentricity index (IE) (1.5 ± 0.1, p = 0.01), and more frequently RV outflow tract (RVOT) notching than other groups (62%, p = 0.012). Group 4 presented an intermedium echocardiographic phenotype between group 1 and 2, and showed more abnormal strain values than the other groups. Group 2 had fewer patients in sinus rhythm (atrial fibrillation in 34% of patients, p = 0.02), presented a thicker interventricular septum (11.3 ± 1.8, p = 0.014), a higher FAC (35 ± 3%, p = 0.0025), higher E mitral wave velocity (72 ± 6 cm/s, p < 0.001) and E/E’ ratio (12.7 ± 10.2, p = 0.006), and larger left (45 ± 3 cm3/m3, p < 0.01) and right atria (25 ± 2 cm2, p = 0.03). PH groups 1 and 4 had higher Pulmonary Vascular Resistance (PVR) and Pulmonary Mean Arterial Pressure (PMAP) values than group 2, which significantly correlated with echocardiographic RV function parameters as TAPSE, FAC, RV global strain and IE. In PH group 2, eccentricity index was the only predictor of PVR (β=4.1, p = 0.018). In this population, a left atria volume < 32.7 cm3/m2 (OR 4.25, CI 1.71 - 10.55) and a E/e’ ratio < 12 (OR 4.72, CI 2.05 - 10.87) predicted PECP < 15 mmHg. RV global strain > -17.1 predicted PVR > 3 wood (OR 3.46, CI 1.50 - 8.02) and PMAP > 20 mmHg (OR 4.92, CI 1.67 - 14.51). TAPSE < 18 mm predicted PVR > 3 wood (OR 7.41, CI 2.99 - 18.36, AUC 0.72). Conclusion Different PH groups present mild echocardiographic differences between them. PH group 1 presented with more echocardiographic signs of RV disfunction, and PH group 2 had higher FAC, E/E’ and larger right and left atria. RV function parameters predicted PVR in PH groups 1 and 4, and EI was the only predictor of PVR in PH group 2. Abstract P1505 Figure.

scholarly journals Peripheral Arterial Stiffness in Acute Pulmonary Embolism and Pulmonary Hypertension at Short-Term Follow-Up

2021 ◽  
Vol 10 (14) ◽  
pp. 3008
Author(s):  
Silvia Papa ◽  
Cristiano Miotti ◽  
Giovanna Manzi ◽  
Gianmarco Scoccia ◽  
Federico Luongo ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Arterial Stiffness ◽  
Hospital Discharge ◽  
Acute Pulmonary Embolism ◽  
Systemic Hypertension ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and under-recognized complication of acute pulmonary embolism (PE). Forty consecutive patients with acute PE (Group 1), predominantly female (22, 55%) with a mean age of 69 ± 15 years, were matched for demographic data with 40 healthy subjects (Group 2), 40 systemic hypertension patients (Group 3) and 45 prevalent idiopathic pulmonary arterial hypertension (IPAH) patients (Group 4). The baseline evaluation included physical examination, NYHA/WHO functional class, right heart catheterization (RHC) limited to IPAH patients, echocardiographic assessment and systemic arterial stiffness measurement by cardio-ankle vascular index (CAVI). Patients with PE underwent an echocardiographic evaluation within 1 month from hospital discharge (median 27 days; IQR 21–30) to assess the echo-derived probability of PH. The CAVI values were significantly higher in the PE and IPAH groups compared with the others (Group 1 vs. Group 2, p < 0.001; Group 1 vs. Group 3, p < 0.001; Group 1 vs. Group 4, p = ns; Group 4 vs. Group 2, p < 0.001; Group 4 vs. Group 3, p < 0.001; Group 2 vs. Group 3, p = ns). The predicted probability of echocardiography-derived high-risk criteria of PH increases for any unit increase of CAVI (OR 9.0; C.I.3.9–20.5; p = 0.0001). The PE patients with CAVI ≥ 9.0 at the time of hospital discharge presented an increased probability of PH. This study highlights a possible positive predictive role of CAVI as an early marker for the development of CTEPH.

scholarly journals The Challenge to Decide between Pulmonary Hypertension Due to Chronic Lung Disease and PAH with Chronic Lung Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 311
Author(s):  
Horst Olschewski
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Chronic Lung Disease ◽  
Diagnostic Procedures ◽  
Disease Group ◽  
Group 4 ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Chronic lung diseases are strongly associated with pulmonary hypertension (PH), and even mildly elevated pulmonary arterial pressures are associated with increased mortality. Chronic obstructive pulmonary disease (COPD) is the most common chronic lung disease, but few of these patients develop severe PH. Not all these pulmonary pressure elevations are due to COPD, although patients with severe PH due to COPD may represent the largest subgroup within patients with COPD and severe PH. There are also patients with left heart disease (group 2), chronic thromboembolic disease (group 4, CTEPH) and pulmonary arterial hypertension (group 1, PAH) who suffer from COPD or another chronic lung disease as co-morbidity. Because therapeutic consequences very much depend on the cause of pulmonary hypertension, it is important to complete the diagnostic procedures and to decide on the main cause of PH before any decision on PAH drugs is made. The World Symposia on Pulmonary Hypertension (WSPH) have provided guidance for these important decisions. Group 2 PH or complex developmental diseases with elevated postcapillary pressures are relatively easy to identify by means of elevated pulmonary arterial wedge pressures. Group 4 PH can be identified or excluded by perfusion lung scans in combination with chest CT. Group 1 PAH and Group 3 PH, although having quite different disease profiles, may be difficult to discern sometimes. The sixth WSPH suggests that severe pulmonary hypertension in combination with mild impairment in the pulmonary function test (FEV1 > 60 and FVC > 60%), mild parenchymal abnormalities in the high-resolution CT of the chest, and circulatory limitation in the cardiopulmonary exercise test speak in favor of Group 1 PAH. These patients are candidates for PAH therapy. If the patient suffers from group 3 PH, the only possible indication for PAH therapy is severe pulmonary hypertension (mPAP ≥ 35 mmHg or mPAP between 25 and 35 mmHg together with very low cardiac index (CI) < 2.0 L/min/m2), which can only be derived invasively. Right heart catheter investigation has been established nearly 100 years ago, but there are many important details to consider when reading pulmonary pressures in spontaneously breathing patients with severe lung disease. It is important that such diagnostic procedures and the therapeutic decisions are made in expert centers for both pulmonary hypertension and chronic lung disease.

scholarly journals 364 Pulmonary hypertension: survival and prognostic factors by subgroups

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Lucia Soriente ◽  
Valeria Visco ◽  
Chiara Aliberti ◽  
Michele Ciccarelli ◽  
Gennaro Galasso ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Arterial Hypertension ◽  
Nyha Class ◽  
Group 4 ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
Clinical Conditions ◽  
Group 1

Abstract Aims The definition of pulmonary hypertension (PH) requires the documentation of mean pulmonary arterial pressure (PAPm) ≥20 mmHg at rest, assessed by right cardiac catheterization. This condition can characterize multiple clinical conditions with different pathophysiological and haemodynamic aspects. Specifically, in pulmonary arterial hypertension (IAP) (Group 1), the increase in PAP is due to an intrinsic pathology of the pulmonary microcirculation; Group 2 includes the forms of IP associated with a pathology of the left heart; Group 3 includes all pathologies of the pulmonary parenchyma and/or hypoxic conditions that lead to a secondary impairment of the small circulation; Group 4 identifies patients with chronic thromboembolic pulmonary heart (CPCTE); finally, Group 5 includes rare clinical conditions in which IP is linked to direct involvement or ab extrinsic compression of the pulmonary vessels. Methods and results To examine the number of deaths and the differences between the various subgroups, we analyzed the follow-up of approximately 76 patients (64.30 ± 13.20 years, 37% male) enrolled in the Pulmonary Hypertension Clinic of the San Giovanni AOU Dio and Ruggi d’Aragona of Salerno from 2014 to 2020 excluding patients with IP under definition and those ‘screened’ who did not show pulmonary hypertension at rest. At each visit, the patients were subjected to anamnestic data collection, physical examination, measurement of blood pressure, heart rate, arterial saturation, transthoracic cardiac echo color Doppler at rest, attribution of the functional class NYHA, evaluation of functional capacity by performing the test of the 6-min walk test (6MWT) and possible programming of right cardiac catheterization. From the data analysis it was found that 26 patients (34.21%, 55.81 ± 13.90 years, 27% males) were affected by IP group 1; 15 patients (19.48%, 74.12 ± 6.26 years, 20% male) were affected by IP group 2; 14 patients (18.18%, 63.34 ± 11.52 years, 71% male) were affected by IP group 3; 12 patients (15.58%, 67.22 ± 11.53 years, 33% male) were affected by IP group 4; 2 patients (2.60%, 71.57 ± 12.48 years, 0% male) were affected by IP group 5; 7 patients (9.09%, 70.07 ± 8.27 years, 57% male) were affected by group 2–3 mixed IP. Analysing the number of deaths, of the 76 patients, 17 deaths were recorded in total (22.37%). All patients in group 1 had been treated with specific therapy and survival was 88% at 3 years. Of the three deaths (12%) in this group, one patient had idiopathic PAH non-responder to pulmonary vasoreactivity test, one patient belonged to the IAP subgroup associated with congenital heart shunt but with concomitant lung disease, and the third patient belonged to the IAP subgroup associated with connective tissue disease (specifically Takayasu’s arteritis). From the analysis of the idiopathic IAP subgroup it emerged that the patients were all women, with an average age of 50.81 ± 3.98 years, and that the deceased patient was distinguished at the first visit from the other patients for: a history of arterial hypertension, dysthyroidism, and obesity; worst NYHA class (III vs. II), elevated heart rate (102 vs. 70.00 ± 7.07 b.p.m.) and blood pressure (SBP: 150.00 vs. 127.50 ± 10.61; DBP 90.00 vs. 75.00 ± 7.07 mmHg) at rest at the clinic visit. Transthoracic echocardiography revealed elevated PAPs values (100.00 vs. 42.50 ± 20.51 mmHg), low TAPSE values (20.00 vs. 26.50 ± 3.54 mm), reduced pulmonary acceleration time (ACT 60 ms), enlargement of the right atrium (area 22 cm2), worst exercise tolerance parameters (6MWT 300 m and 86% final SO2 vs. 427.50 ± 74.25 m and 96.50 ± 0.71% final SO2). Conclusions The percentage of deaths in the different groups appears very heterogeneous, especially if we consider the six deaths (42.86%) in group 3 and three deaths (42.86%) in patients with mixed IP groups 2 and 3. In these two groups, age and advanced NYHA class were the most representative prognostic factors. On the other hand, analysing patients belonging to the idiopathic IAP subgroup, a worse prognosis is entrusted to the negativity of the vasoreactivity test, to the presence of cardiovascular comorbidities (arterial hypertension and dysthyroidism in our case), to worse echocardiographic values (PAPs, TAPSE, ACT, atrial area right) and reduced functional capacity at the 6MWT. However, early treatment and innovative drugs together with a careful strategy have been allowed.

In-vivo evaluation of the effect of cyanoacrylate on prosthetic vascular graft infection – does cyanoacrylate increase the severity of infection?

VASA ◽  
2020 ◽  
Vol 49 (4) ◽  
pp. 281-284
Author(s):  
Atıf Yolgosteren ◽  
Gencehan Kumtepe ◽  
Melda Payaslioglu ◽  
Cuneyt Ozakin
Keyword(s):  
Vascular Graft ◽  
Graft Infection ◽  
Vascular Graft Infection ◽  
Group 4 ◽  
Significant Difference ◽  
Group 3 ◽  
Group 2 ◽  
Prosthetic Vascular Graft Infection ◽  
Group 1

Summary. Background: Prosthetic vascular graft infection (PVGI) is a complication with high mortality. Cyanoacrylate (CA) is an adhesive which has been used in a number of surgical procedures. In this in-vivo study, we aimed to evaluate the relationship between PVGI and CA. Materials and methods: Thirty-two rats were equally divided into four groups. Pouch was formed on back of rats until deep fascia. In group 1, vascular graft with polyethyleneterephthalate (PET) was placed into pouch. In group 2, MRSA strain with a density of 1 ml 0.5 MacFarland was injected into pouch. In group 3, 1 cm 2 vascular graft with PET piece was placed into pouch and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. In group 4, 1 cm 2 vascular graft with PET piece impregnated with N-butyl cyanoacrylate-based adhesive was placed and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. All rats were scarified in 96th hour, culture samples were taken where intervention was performed and were evaluated microbiologically. Bacteria reproducing in each group were numerically evaluated based on colony-forming unit (CFU/ml) and compared by taking their average. Results: MRSA reproduction of 0 CFU/ml in group 1, of 1410 CFU/ml in group 2, of 180 200 CFU/ml in group 3 and of 625 300 CFU/ml in group 4 was present. A statistically significant difference was present between group 1 and group 4 (p < 0.01), between group 2 and group 4 (p < 0.01), between group 3 and group 4 (p < 0.05). In terms of reproduction, no statistically significant difference was found in group 1, group 2, group 3 in themselves. Conclusions: We observed that the rate of infection increased in the cyanoacyrylate group where cyanoacrylate was used. We think that surgeon should be more careful in using CA in vascular surgery.

Does Perioperative Hemoglobin A1c Level Affect the Incidence, Pattern and Mortality of Lower Extremity Amputation?

2019 ◽  
Vol 17 (4) ◽  
pp. 354-364
Author(s):  
Hassan Al-Thani ◽  
Moamena El-Matbouly ◽  
Maryam Al-Sulaiti ◽  
Noora Al-Thani ◽  
Mohammad Asim ◽  
...  
Keyword(s):  
Glycemic Control ◽  
Lower Extremity ◽  
Hazard Ratio ◽  
Lower Extremity Amputation ◽  
Group 4 ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
Extremity Amputation ◽  
Group 1

Background: We hypothesized that perioperative HbA1c influenced the pattern and outcomes of Lower Extremity Amputation (LEA). Methods: A retrospective analysis was conducted for all patients who underwent LEA between 2000 and 2013. Patients were categorized into 5 groups according to their perioperative HbA1c values [Group 1 (<6.5%), Group 2 (6.5-7.4%), Group 3 (7.5-8.4%), Group 4 (8.5-9.4%) and Group 5 (≥9.5%)]. We identified 848 patients with LEA; perioperative HbA1c levels were available in 547 cases (Group 1: 18.8%, Group 2: 17.7%, Group 3: 15.0%, Group 4: 13.5% and Group 5: 34.9%). Major amputation was performed in 35%, 32%, 22%, 10.8% and 13.6%, respectively. Results: The overall mortality was 36.5%; of that one quarter occurred during the index hospitalization. Mortality was higher in Group 1 (57.4%) compared with Groups 2-5 (46.9%, 38.3%, 36.1% and 31.2%, respectively, p=0.001). Cox regression analysis showed that poor glycemic control (Group 4 and 5) had lower risk of mortality post-LEA [hazard ratio 0.57 (95% CI 0.35-0.93) and hazard ratio 0.46 (95% CI 0.31-0.69)]; this mortality risk persisted even after adjustment for age and sex but was statistically insignificant. The rate of LEA was greater among poor glycemic control patients; however, the mortality was higher among patients with tight control. Conclusion: The effects of HbA1c on the immediate and long-term LEA outcomes and its therapeutic implications need further investigation.

Association between cervical disc disease and lesions of multiple sclerosis

2021 ◽  
pp. 197140092098356
Author(s):  
Marwan Alkrenawi ◽  
Michael Osherov ◽  
Azaria Simonovich ◽  
Jonathan Droujin ◽  
Ron Milo ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Disc ◽  
Cervical Disc Disease ◽  
Disc Disease ◽  
Group 4 ◽  
Demyelinating Lesions ◽  
Grade Ii ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Background Cervical discopathy and demyelinating lesions often co-exist in patients with multiple sclerosis (MS). Our study examines the possible association between these two pathologies. Methods Medical records and cervical magnetic resonance imaging scans of MS patients with cervical discopathy who were seen at our MS clinic during 2018 were retrospectively reviewed. The severity of the disc disease was classified as grade I (no compression), grade II (compression of the dural sac) and grade III (cord compression). The spinal cord in each scan was divided into six segments corresponding to the intervertebral space of the spine (C1–C6). Each segment was defined as containing demyelinating lesion and disc pathology (group 1), demyelinating lesion without disc pathology (group 2), disc pathology without demyelinating lesion (group 3) and no demyelinating lesion or disc pathology (group 4). Fisher’s exact test was used to test the association between demyelinating lesions and disc pathology. Results Thirty-four MS patients with cervical discopathy were included in the study (26 females; average age 42.9 ± 13.7 years; average disease duration 8.4 ± 5.4 years). A total of 204 spinal cord segments were evaluated. Twenty-four segments were classified as group 1, 27 segments as group 2, 52 segments as group 3 and 101 segments as group 4. There was no association between demyelinating lesions and the grade of disc disease ( p = 0.1 for grade I, p = 0.3 for grade II and p = 1 for grade III disc disease). Conclusion Our study did not find any association between cervical disc disease and demyelinating spinal cord lesion.

scholarly journals Association between serum oestradiol level on the hCG administration day and neonatal birthweight after IVF-ET among 3659 singleton live births

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yu Liu ◽  
Jing Li ◽  
Wanyu Zhang ◽  
Yihong Guo
Keyword(s):  
Birth Weight ◽  
Ovarian Stimulation ◽  
Group 4 ◽  
Group 6 ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
The Impact ◽  
Ivf Et ◽  
Group 1

AbstractOestradiol, an important hormone in follicular development and endometrial receptivity, is closely related to clinical outcomes of fresh in vitro fertilization-embryo transfer (IVF-ET) cycles. A supraphysiologic E2 level is inevitable during controlled ovarian hyper-stimulation (COH), and its effect on the outcome of IVF-ET is controversial. The aim of this retrospective study is to evaluate the association between elevated serum oestradiol (E2) levels on the day of human chorionic gonadotrophin (hCG) administration and neonatal birthweight after IVF-ET cycles. The data of 3659 infertile patients with fresh IVF-ET cycles were analysed retrospectively between August 2009 and February 2017 in First Hospital of Zhengzhou University. Patients were categorized by serum E2 levels on the day of hCG administration into six groups: group 1 (serum E2 levels ≤ 1000 pg/mL, n = 230), group 2 (serum E2 levels between 1001 and 2000 pg/mL, n = 524), group 3 (serum E2 levels between 2001 and 3000 pg/mL, n = 783), group 4 (serum E2 levels between 3001 and 4000 pg/mL, n = 721), group 5 (serum E2 levels between 4001 and 5000 pg/mL, n = 548 ), and group 6 (serum E2 levels > 5000 pg/mL, n = 852). Univariate linear regression was used to evaluate the independent correlation between each factor and outcome index. Multiple logistic regression was used to adjust for confounding factors. The LBW rates were as follows: 3.0% (group 1), 2.9% (group 2), 1.9% (group 3), 2.9% (group 4), 2.9% (group 5), and 2.0% (group 6) (P = 0.629), respectively. There were no statistically significant differences in the incidences of neonatal LBW among the six groups. We did not detect an association between peak serum E2 level during ovarian stimulation and neonatal birthweight after IVF-ET. The results of this retrospective cohort study showed that serum E2 peak levels during ovarian stimulation were not associated with birth weight during IVF cycles. In addition, no association was found between higher E2 levels and increased LBW risk. Our observations suggest that the hyper-oestrogenic milieu during COS does not seem to have adverse effects on the birthweight of offspring after IVF. Although this study provides some reference, the obstetric-related factors were not included due to historical reasons. The impact of the high estrogen environment during COS on the birth weight of IVF offspring still needs future research.

scholarly journals Control of Vulval Competence and Centering in the Nematode Oscheius sp. 1 CEW1

Genetics ◽  
2003 ◽  
Vol 163 (1) ◽  
pp. 133-146 ◽  
Author(s):  
Sophie Louvet-Vallée ◽  
Irina Kolotuev ◽  
Benjamin Podbilewicz ◽  
Marie-Anne Félix
Keyword(s):  
Germ Line ◽  
Specific Mechanism ◽  
C Elegans ◽  
Group 4 ◽  
Cell Ablation ◽  
Group 3 ◽  
Group 2 ◽  
P Cells ◽  
Large Category ◽  
Group 1

Abstract To compare vulva development mechanisms in the nematode Oscheius sp. 1 to those known in Caenorhabditis elegans, we performed a genetic screen for vulva mutants in Oscheius sp. 1 CEW1. Here we present one large category of mutations that we call cov, which affect the specification of the Pn.p ventral epidermal cells along the antero-posterior axis. The Pn.p cells are numbered from 1 to 12 from anterior to posterior. In wild-type Oscheius sp. 1 CEW1, the P(4-8).p cells are competent to form the vulva and the progeny of P(5-7).p actually form the vulva, with the descendants of P6.p adopting a central vulval fate. Among the 17 mutations (defining 13 genes) that we characterize here, group 1 mutations completely or partially abolish P(4-8).p competence, and this correlates with early fusion of the Pn.p cells to the epidermal syncytium. In this group, we found a putative null mutation in the lin-39 HOM-C homolog, the associated phenotype of which could be weakly mimicked by injection of a morpholino against Osp1-lin-39 in the mother’s germ line. Using cell ablation in a partially penetrant competence mutant, we show that vulval competence is partially controlled by a gonadal signal. Most other mutants found in the screen display phenotypes unknown in C. elegans. Group 2 mutants show a partial penetrance of Pn.p competence loss and an abnormal centering of the vulva on P5.p, suggesting that these two processes are coregulated by the same pathway in Oscheius sp. 1. Group 3 mutants display an enlarged competence group that includes P3.p, thus demonstrating the existence of a specific mechanism inhibiting P3.p competence. Group 4 mutants display an abnormal centering of the vulval pattern on P7.p and suggest that a specific mechanism centers the vulval pattern on a single Pn.p cell.

scholarly journals Hypertrophy of unaffected cardiomyocytes correlates with severity of cardiomyopathy in female patients with Fabry disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Cristina Chimenti ◽  
Romina Verardo ◽  
Andrea Frustaci
Keyword(s):  
Fabry Disease ◽  
Wall Thickness ◽  
Left Ventricular ◽  
Female Patients ◽  
Maximal Wall Thickness ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Aim To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy. Findings Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. Diagnosis of FD was based on the presence of pathogenic GLA mutation, Patients were divided in four groups according with LV maximal wall thickness (MWT): group 1 MWT ≤ 10.5 mm, group 2 MWT 10.5–15 mm, group 3 MWT 16–20 mm, group 4 MWT > 20 mm. At histology mosaic of affected and unaffected cardiomyocytes was documented. Unaffected myocytes’ size ranged from normal to severe hypertrophy. Hypertrophy of unaffected cardiomyocytes correlated with severity of MWT (p < 0.0001, Sperman r 0,95). Hypertrophy of unaffected myocytes appear to concur to progression and severity of FDCM. It is likely a paracrine role from neighboring affected myocytes.

scholarly journals THU0227 CAFFEINE INTAKE MODULATES DISEASE ACTIVITY AND CYTOKINES LEVELS IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 340.2-341
Author(s):  
V. Orefice ◽  
F. Ceccarelli ◽  
C. Barbati ◽  
R. Lucchetti ◽  
G. Olivieri ◽  
...  
Keyword(s):  
Disease Activity ◽  
Lupus Erythematosus ◽  
Caffeine Intake ◽  
Caffeine Consumption ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
The Impact ◽  
Group 1

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease mainly affecting women of childbearing age. The interplay between genetic and environmental factors may contribute to disease pathogenesis1. At today, no robust data are available about the possible contribute of diet in SLE. Caffeine, one of the most widely consumed products in the world, seems to interact with multiple components of the immune system by acting as a non-specific phosphodiesterase inhibitor2.In vitrodose-dependent treatment with caffeine seems to down-regulate mRNA levels of key inflammation-related genes and similarly reduce levels of different pro-inflammatory cytokines3.Objectives:We evaluated the impact of caffeine consumption on SLE-related disease phenotype and activity, in terms of clinimetric assessment and cytokines levels.Methods:We performed a cross-sectional study, enrolling consecutive patients and reporting their clinical and laboratory data. Disease activity was assessed by SLE Disease Activity Index 2000 (SLEDAI-2k)4. Caffeine intake was evaluated by a 7-day food frequency questionnaire, including all the main sources of caffeine. As previously reported, patients were divided in four groups according to the daily caffeine intake: <29.1 mg/day (group 1), 29.2-153.7 mg/day (group 2), 153.8-376.5 mg/day (group 3) and >376.6 mg/day (group 4)5. At the end of questionnaire filling, blood samples were collected from each patient to assess cytokines levels. These were assessed by using a panel by Bio-Plex assays to measure the levels of IL-6, IL-10, IL-17, IL-27, IFN-γ, IFN-α and Blys.Results:We enrolled 89 SLE patients (F/M 87/2, median age 46 years, IQR 14; median disease duration 144 months, IQR 150). The median intake of caffeine was 195 mg/day (IQR 160.5). At the time of the enrollment, 8 patients (8.9%) referred a caffeine intake < 29.1 mg/day (group 1), 27 patients (30.3%) between 29.2 and 153.7 mg/day (group 2), 45 patients (51%) between 153.8 and 376.5 mg/day (group 3) and 9 patients (10.1%) >376.6 mg/day (group 4). A negative correlation between the levels of caffeine and disease activity, evaluated with SLEDAI-2K, was observed (p=0.01, r=-0.26). By comparing the four groups, a significant higher prevalence of lupus nephritis, neuropsychiatric involvement, haematological manifestations, hypocomplementemia and anti-dsDNA positivity was observed in patients with less intake of caffeine (figure 1 A-E). Furthermore, patients with less intake of caffeine showed a significant more frequent use of glucocorticoids [group 4: 22.2%,versusgroup 1 (50.0%, p=0.0001), group 2 (55.5%, p=0.0001), group 3 (40.0%, p=0.009)]. Moving on cytokines analysis, a negative correlation between daily caffeine consumption and serum level of IFNγ was found (p=0.03, r=-0.2) (figure 2A); furthermore, patients with more caffeine intake showed significant lower levels of IFNα (p=0.02, figure 2B), IL-17 (p=0.01, figure 2C) and IL-6 (p=0.003, figure 2D).Conclusion:This is the first report demonstrating the impact of caffeine on SLE disease activity status, as demonstrated by the inverse correlation between its intake and both SLEDAI-2k values and cytokines levels. Moreover, in our cohort, patients with less caffeine consumption seems to have a more severe disease phenotype, especially in terms of renal and neuropsychiatric involvement. Our results seem to suggest a possible immunoregulatory dose-dependent effect of caffeine, through the modulation of serum cytokine levels, as already suggested byin vitroanalysis.References:[1]Kaul et alNat. Rev. Dis. Prim.2016; 2. Aronsen et alEurop Joul of Pharm2014; 3. Iris et alClin Immun.2018; 4. Gladman et al J Rheumatol. 2002; 5. Mikuls et alArth Rheum2002Disclosure of Interests:Valeria Orefice: None declared, Fulvia Ceccarelli: None declared, cristiana barbati: None declared, Ramona Lucchetti: None declared, Giulio Olivieri: None declared, enrica cipriano: None declared, Francesco Natalucci: None declared, Carlo Perricone: None declared, Francesca Romana Spinelli Grant/research support from: Pfizer, Consultant of: Novartis, Gilead, Lilly, Sanofi, Celgene, Speakers bureau: Lilly, cristiano alessandri Grant/research support from: Pfizer, Guido Valesini: None declared, Fabrizio Conti Speakers bureau: BMS, Lilly, Abbvie, Pfizer, Sanofi

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