Palatal shape covariation in extraction versus nonextraction borderline patients: A geometric morphometric study.

INTRODUCTION: This study aimed to evaluate changes in palatal shape after orthodontic treatment from a borderline sample of extraction and nonextraction patients with a Class I relationship. METHODS: A borderline sample regarding premolar extractions was obtained through discriminant analysis and comprised 30 nonextraction and 23 extraction patients. The digital dental casts of these patients were digitized with 3 curves and 239 landmarks placed on the hard palate. Procrustes superimposition and principal component analysis were implemented to assess group shape variability patterns. RESULTS: The success of the discriminant analysis in identifying a borderline sample regarding the extraction modality was validated using geometric morphometrics. Concerning palatal shape, no sexual dimorphism was found (P = 0.78). The first 6 principal components that were statistically significant accounted for 79.2% of the total shape variance. Palatal changes were 61% more pronounced in the extraction group, which exhibited a decrease in palatal length (P = 0.02; 10,000 permutations). In contrast, the nonextraction group showed an increase in the palatal width (P <0.001; 10,000 permutations). Intergroup comparisons indicated that the nonextraction group exhibited longer palates, whereas the extraction group exhibited higher palates (P = 0.02; 10,000 permutations). CONCLUSIONS: Considerable changes in palatal shape were seen for the nonextraction and extraction treatment group, with the latter exhibiting more pronounced changes, mainly in terms of palatal length. Further investigations are needed to clarify the clinical significance of the palatal shape changes in borderline patients after extraction and nonextraction treatment.

Life-threatening antineutrophil cytoplasmic antibody-associated vasculitis after influenza A H1N1 infection requiring veno-venous extracorporeal membrane oxygenation.

INTRODUCTION: Antineutrophil cytoplasmic autoantibody-associated vasculitis is an immune-mediated necrotizing vasculitis, affecting small- and medium-sized vessels. CASE REPORT: A 22-year-old female patient with free medical history presented with life-threatening pulmonary hemorrhage due to antineutrophil cytoplasmic autoantibody-associated vasculitis, temporarily associated with influenza A H1N1 infection. Due to rapidly worsening respiratory failure, despite conventional management, veno-venous peripheral extracorporeal membrane oxygenation was initiated and continued for 26 days, with subsequent renal replacement therapy. DISCUSSION: We present a case of severe antineutrophil cytoplasmic autoantibody-associated pulmonary vasculitis, managed with veno-venous extracorporeal membrane oxygenation at the initial phase. Despite the significant challenges raised with the use of extracorporeal membrane oxygenation in pulmonary hemorrhage cases, extracorporeal membrane oxygenation may have a significant impact on outcome in this setting, by providing adequate time for a successful immunosuppressive treatment.

A study of the mechanical properties of as-received and intraorally exposed single-crystal and polycrystalline orthodontic ceramic brackets.

BACKGROUND: Although ceramic brackets have been extensively used for decades in orthodontics there is not till today any study focusing on the possible deterioration of mechanical properties after in vivo ageing. OBJECTIVES: To determine whether the mechanical properties of alumina orthodontic brackets change after intraoral ageing thereby assessing the validity of a theoretical model established for the performance of ceramics in wet environments. MATERIALS AND METHODS: Two alumina brackets, one single crystal (Radiance, American Orthodontics, Sheboygan, WI) and one polycrystalline (Clarity, 3M, St. Paul, MN) were included in this study. Ten brackets for each group were collected from different patients after a minimum of 3-month intraoral exposure, whereas as-received brackets of the same manufacturers were used as controls. The specimens were subjected to Raman spectroscopy and were then embedded in epoxy resin and metallographic ground and polished. The mechanical properties of four groups (radiance control: RAC, radiance-retrieved RAR, clarity control: CLC and clarity-retrieved CLR) were determined using instrumented indentation testing according to ISO 14577-2002. The mechanical properties tested were Martens hardness (HM), indentation modulus (EIT), the ratio of elastic to total work, commonly known as elastic index (ηIT), and fracture toughness (KIC). The numerical results were statistically analysed employing two-way analysis of variance (ANOVA) and Tukey multiple comparison test at a = 0.05. RESULTS: Raman analysis revealed that both brackets are made of a-Al2O3 (corundum). No statistically significant differences were found for HM (N/mm2): RAC = 7249 (1507), RAR = 6926 (1144), CLC = 8052 (1360), CLR = 7390 (2393), or for EIT (GPa): RAC = 141 (27), RAR = 139 (23), CLC = 139 (28), CLR = 131 (47). However, significant differences were identified between the two alumina brackets tested for ηIT (%): RAC = 55.7 (4.2), RAR = 54.0 (3.5), CLC = 62.5 (4.4), CLR = 61.8 (4.7), while KIC was measured only for the polycrystalline bracket (Clarity) because of the complicated fractured pattern of the single-crystal bracket. Both brackets share equal HM and EIT before and after orthodontic intraoral ageing. LIMITATIONS: Whereas the study assessed the changes after intraoral exposure per theoretical model, which describes the reduction of critical stress to induce fracture after wetting, long-term intraoral ageing could have induced more pronounced effects. CONCLUSIONS/IMPLICATIONS: The results of this study indicate that 3 months of intraoral ageing do not change the mechanical properties of single-crystal and polycrystalline orthodontic brackets tested, thus indicating that the Griffith theory may not be applied to the case of manufactured ceramic brackets owing possibly to internal defects.

Past history of stage I/II solid tumor malignancy impacts considerably on sepsis mortality: a propensity score matching analysis from the hellenic sepsis study group.

BACKGROUND: Whether past history of solid stage I/II inactive cancer has an impact on 28-day mortality of sepsis remains unclear. We aimed to determine the impact of history of stage I or II solid tumor malignancy in complete remission the last 3 years on sepsis outcome. METHODS: Using the database of the Hellenic Sepsis Study Group from 1553 patients with sepsis admitted in the ICU, 83 patients with sepsis by Sepsis-3 definition with past-history of stage I/II inactive solid malignancy the last 3 years were depicted. A comparator group of 83 patients fully matched for age, severity, type of infection and comorbidities was selected by propensity score matching. RESULTS: Mortality after 28 days was 37.3% in the comparator group and 54.2% in the solid tumor stage I/II group (odds ratio for death 1.98; p: 0.030). Following step-wise forward Cox regression analysis, septic shock (hazard ratio 1.80), acute renal injury (hazard ratio 2.06), history of coronary heart disease (hazard ratio 0.36) and history of stage I/II solid tumor malignancy (hazard ratio 1.79) were the only independent variables associated with 28-day mortality. Serum levels of procalcitonin and of soluble urokinase plasminogen activator receptor were similar between the two groups of comparisons. CONCLUSIONS: Past history of stage I/II solid malignancy is an independent risk factor for unfavorable outcome from sepsis the first 28 days.

Platelet and coagulation disorders in newly diagnosed patients with pulmonary arterial hypertension.

There is a complex and not fully elucidated association between pulmonary arterial hypertension (PAH) and coagulation disorders. The goal of this study was to evaluate platelet function, coagulation and fibrinolysis in PAH patients at diagnosis, before PAH-specific treatment initiation. We enrolled 20 healthy controls and 30 PAH patients (20 with connective tissue disease (CTD-PAH) and 10 idiopathic (iPAH)). None of the participants was on any antiplatelet or anticoagulation therapy. Blood samples from PAH patients were collected during the initial right heart catheterization. All subjects were assessed with platelet function analyzer-100 (PFA-100), epinephrine (Epi) and ADP-induced light transmission aggregometry (LTA), thromboelastometry (ROTEM) and endogenous thrombin potential (ETP). Our results showed that Epi and ADP-LTA values were significantly lower in newly diagnosed PAH patients compared to controls. Disaggregation was present in 73% of patients, a characteristic not seen in healthy individuals. In ROTEM assay, CT and CFT measurements were significantly higher and a angle lower compared to controls. ETP testing revealed significantly reduced outcomes in AUC, Cmax and Tmax. When CTD-PAH and iPAH patient groups were compared, iPAH ADP-LTA values were significantly decreased compared to CTD-PAH. In conclusion, newly diagnosed PAH patients presented with decreased platelet aggregation, clot propagation and thrombin generation, along with delayed initiation of the coagulation process. These hemostatic deficits could indicate an "exhaustion" of the coagulation process that could be caused by endothelial dysfunction and chronic activation of the procoagulant pathways. Further studies are warranted to confirm these laboratory findings and assess their potential clinical significance.

A geometric morphometric evaluation of hard and soft tissue profile changes in borderline extraction versus non-extraction patients.

OBJECTIVES: To evaluate the hard tissue and facial profile changes in matched extraction and non-extraction Class I patients by the use of geometric morphometrics. SUBJECTS AND METHODS: From a parent sample of 542 Class I patients, previously subjected to discriminant analysis, a subsample of 68 borderline cases was obtained, 34 treated with extraction and 34 without extraction of 4 first premolars. Geometric morphometric methods (Procrustes superimposition and Principal Component Analysis) were applied on cephalometric tracings to assess the validity of the discriminant analysis in successfully identifying a morphologically homogeneous group and to evaluate inter- and intra-group skeletal and facial profile shape changes. RESULTS: No significant pre-treatment shape difference between the two groups was found, thus validating the discriminant analysis. The non-extraction group showed increase in hard tissue facial height (P < 0.001), with slight lower lip retrusion and upper lip protrusion (P = 0.027). The extraction group showed retraction of the hard tissue and facial profile outline (P < 0.001). Permutation tests for post-treatment inter-group differences resulted in P = 0.054 for the soft tissue outline and P = 0.078 for the hard tissue skeletal component. CONCLUSIONS: The evidence indicates that borderline cases treated with four premolar extractions will exhibit lip retrusion compared to non-extraction treatment.

Soft tissue changes following extraction vs. nonextraction orthodontic fixed appliance treatment: a systematic review and meta-analysis.

The aim of this systematic review was to assess the effect of systematic extraction protocols during orthodontic fixed appliance treatment on the soft tissue profile of human patients. Nine databases were searched until December 2016 for controlled clinical studies including premolar extraction or nonextraction treatment. After elimination of duplicate studies, data extraction, and risk-of-bias assessment according to the Cochrane guidelines, random-effects meta-analyses of mean differences (MD) or standardized mean differences (SMD) and their 95% CIs were performed, followed by subgroup, meta-regression, and sensitivity analyses. Extraction treatment was associated with increased lower lip retraction (24 studies; 1,456 patients; MD = 1.96 mm), upper lip retraction (21 studies; 1,149 patients; MD = 1.26 mm), nasolabial angle (21 studies; 1,089 patients; MD = 4.21°), soft-tissue profile convexity (six studies; 408 patients; MD = 1.24°), and profile pleasantness (three studies; 249 patients; SMD = 0.41). Patient age, extraction protocol, and amount of upper incisor retraction during treatment were significantly associated with the observed extraction effects, while the quality of evidence was very low in all cases due to risk of bias, baseline confounding, inconsistency, and imprecision. Although tooth extractions seem to affect patient profile, existing studies are heterogenous and no consistent predictions of profile response can be made.

Torturous path of an elastic gap band: Interdisciplinary approach to orthodontic treatment for a young patient who lost both maxillary central incisors after do-it-yourself treatment.

Spacing between the maxillary anterior teeth is a common concern for young patients and their parents. Patients often consider a diastema to be an annoying but minor problem; they might consult their general dentists for help, or try to address the issue themselves by applying elastic "gap bands." Moreover, parents, without any informed consent, sometimes accept this erroneous method as an easy and inexpensive treatment approach. A 9-year-old boy had severe acute periodontitis involving the maxillary central incisors caused by the placement of an elastic band and its apical migration. Despite periodontal and surgical interventions, the maxillary central incisors were finally extracted, and the patient started orthodontic treatment. The orthodontic treatment plan included maxillary lateral incisor substitution to replace the lost central incisors and mesialization of the maxillary posterior dentition. An interdisciplinary approach with excellent cooperation among the orthodontist, general dentist, and other dental specialists obtained an esthetically pleasing and optimized functional result. Treating the diastema between the anterior teeth with elastic gap bands and without fixed orthodontic appliances should be avoided. Patients should seek proper orthodontic advice for even small-scale orthodontic problems to prevent catastrophic outcomes, as exhibited in this case report.

Arch-width changes in extraction vs nonextraction treatments in matched Class I borderline malocclusions.

INTRODUCTION: The aims of this study were to identify a sample of borderline Class I extraction and nonextraction patients and to investigate posttreatment changes in arch-width and perimeter measurements. METHODS: A parent sample of 580 Class I patients was subjected to discriminant analysis, and a borderline subsample of 62 patients, 31 treated with extraction of 4 first premolars and 31 treated without extractions, was obtained. The patients' plaster casts were digitally scanned, and the maxillary and mandibular intercanine and intermolar widths and perimeters were assessed. RESULTS: The extraction group showed increases in maxillary and mandibular intercanine widths (P <0.001) and decreases in mandibular intermolar width and in maxillary and mandibular perimeters (P <0.001). The nonextraction group showed increases in all 4 arch-width measurements (P ≤0.003), whereas the maxillary and mandibular perimeters were maintained. The posttreatment differences between the 2 groups showed significant differences in the maxillary (P <0.001) and mandibular intermolar widths (P <0.001). Also, the comparison of the arch perimeters between the 2 treatment groups showed adjusted differences of -8.51 mm (P <0.001) and -8.44 mm (P <0.001) for the maxillary and mandibular arches, respectively. The intercanine widths showed no changes between the 2 treatment groups. CONCLUSIONS: Borderline Class I patients treated with extraction of 4 first premolars had decreased maxillary and mandibular intermolar and perimeter measurements compared with nonextraction patients. The maxillary and mandibular intercanine widths showed no significant difference between the 2 treatment groups.

University clinic and private practice treatment outcomes in Class I extraction and nonextraction patients: A comparative study with the American Board of Orthodontics Objective Grading System.

INTRODUCTION: The aim of this study was to compare treatment outcomes in university vs private practice settings with Class I patients using the American Board of Orthodontics Objective Grading System. METHODS: A parent sample of 580 Class I patients treated with and without extractions of 4 first premolars was subjected to discriminant analysis to identify a borderline spectrum of 66 patients regarding the extraction modality. Of these patients, 34 were treated in private orthodontic practices, and 32 were treated in a university graduate orthodontic clinic. The treatment outcomes were evaluated using the 8 variables of the American Board of Orthodontics Objective Grading System. RESULTS: The total scores ranged from 10 to 47 (mean, 25.44; SD, 9.8) for the university group and from 14 to 45 (mean, 25.94; SD, 7.7) for the private practice group. The university group achieved better scores for the variables of buccolingual inclination (mean difference, 2.28; 95% confidence interval [CI], 0.59, 3.98; P = 0.01) and marginal ridges (mean difference, 1.32; 95% CI, 0.28, 2.36; P = 0.01), and the private practice group achieved a better score for the variable of root angulation (mean difference, -0.65; 95% CI, -1.26, -0.03; P = 0.04). However, no statistically intergroup differences were found between the total American Board of Orthodontics Objective Grading System scores (mean difference, -0.5; 95% CI, -3.82, 4.82; P = 0.82). CONCLUSIONS: Patients can receive similar quality of orthodontic treatment in a private practice and a university clinic. The orthodontists in the private practices were more successful in angulating the roots properly, whereas the orthodontic residents accomplished better torque control of the posterior segments and better marginal ridges.

Treatment outcomes after extraction and nonextraction treatment evaluated with the American Board of Orthodontics objective grading system.

INTRODUCTION: A controversy exists regarding better treatment outcomes when patients treated with extractions and without extractions are evaluated. The aims of this study were to use the American Board of Orthodontics objective grading system (ABO-OGS) to evaluate and compare treatment outcomes in extraction vs nonextraction Class I patients and to determine whether the treatment choice was a significant predictor of success according to the ABO examination. METHODS: Discriminant analysis was applied to a sample of 542 patients, and a borderline sample of 55 patients was obtained. Of these patients, 25 were treated with extractions and 30 without extraction of the 4 first premolars. Treatment results were then assessed using the 8 variables of the ABO-OGS. RESULTS: The total scores ranged from 11 to 41 (mean, 27.04; SD, 6.3) for the extraction group and from 16 to 44 (mean, 29.07; SD, 7.1) for the nonextraction group. The variable of buccolingual inclination had the highest scores in both groups (8.44 [SD, 3.3] for the extraction group; 8.90 [SD, 3.8] for the nonextraction group; mean difference, 0.46; 95% CI, -1.44, 2.37; P = 0.63). However, no statistically significant intergroup differences were found, either between the scores of the 8 ABO-OGS variables or between the total ABO-OGS scores. Regarding the success rates of the ABO examination, no significant difference was found between the 2 treatment groups (odds ratio, 2.55; 95% CI, 0.74, 0.85; P = 0.14). CONCLUSIONS: For a patient with a borderline Class I malocclusion, extraction and nonextraction treatment can achieve the same quality of results as assessed by the ABO-OGS. Additionally, in these Class I patients, the treatment modality (extraction or nonextraction) is not a significant predictor of passing the ABO examination.

Orthodontic Risk Perspectives among Orthodontists during Treatment: A Descriptive Pilot Study in Greece and Slovakia.

This study explores orthodontists' perspectives on risks associated with orthodontic treatment, as described by Greek and Slovak orthodontists. Informed by the foundational importance of effective communication of risk perspectives in health sciences, particularly in facilitating valid consent and shared decision-making, this research addresses gaps identified in the literature concerning the consistent communication of potential treatment risks based on demographic and cultural characteristics. This study identifies 15 potential critical risks during orthodontic treatment. These risks include root resorption; temporary undesired changes to the occlusion; sleep difficulties; not achieving an ideal result; development of black triangles between teeth; taking additional X-rays; speech difficulties; using a protective splint during sports; duration of treatment; number of visits; transmission of infectious diseases; and swallowing orthodontic appliances. A questionnaire, distributed electronically to orthodontists in Greece (N1 = 570) and Slovakia (N2 = 210) from September 2022 to December 2022, aimed to assess risk communication practices, taking into consideration socio-demographic factors, such as country, gender, age, and academic-degree-related variations. A total of 168 valid questionnaires (91 from Slovakia and 77 from Greece) were obtained, indicating significant disparities in the risks emphasized and preferred forms of consent. The Greek orthodontists focused more on the risks involved, such as relapse, root resorption, temporal occlusal changes, and failure of desired movement, while the Slovak practitioners tended to be more interested in sleeping difficulties, temporal occlusal changes, and not achieving an ideal result. They also obtained written or digital consent from patients or their parents/guardians more frequently than the Greek team. Male orthodontists discussed specific risks more frequently, including relapse and extractions, whereas females preferred written or digital consent. PhD-trained orthodontists prioritized certain risks, indicating the need for tailored approaches. This study underscores the dynamic nature of risk assessment in orthodontic practice, emphasizing its ethical and strategic dimensions. The findings advocate for tailored risk communication strategies that recognize individual, contextual, and cultural factors, and the need for an orthodontic informed consent protocol for a tailored communication approach for patients to elevate the standard of care in European orthodontics. The reliance on digital tools reflects contemporary trends in enhancing patient understanding, thereby supporting ongoing innovation in orthodontic practices.

Senescent human periodontal ligament fibroblasts after replicative exhaustion or ionizing radiation have a decreased capacity towards osteoblastic differentiation.

Loss of teeth increases with age or after genotoxic treatments, like head and neck radiotherapy, due to periodontium breakdown. Periodontal ligament fibroblasts represent the main cell type in this tissue and are crucial for the maintenance of homeodynamics and for its regeneration. Here, we have studied the characteristics of human periodontal ligament fibroblasts (hPDLF) that became senescent after replicative exhaustion or after exposure to ionizing radiation, as well as their ability for osteoblastic differentiation. We found that senescent hPDLF express classical markers of senescence, as well as a catabolic phenotype, as shown by the decrease in collagen type I and the increase of MMP-2 expression. In addition, we observed a considerably decreased expression of the major transcription factor for osteoblastic differentiation, i.e. Runx2, a down-regulation which was found to be p53-dependent. In accordance to the above, senescent cells have a significantly decreased alkaline phosphatase gene expression and activity, as well as a reduced ability for osteoblastic differentiation, as found by Alizarin Red staining. Interestingly, cells from both type of senescence express similar characteristics, implying analogous functions in vivo. In conclusion, senescent hPDLF express a catabolic phenotype and express a significantly decreased ability towards an osteoblastic differentiation, thus probably affecting tissue development and integrity.

Extracorporeal Membrane Oxygenation (ECMO)-Associated Coagulopathy in Adults.

Extracorporeal membrane oxygenation (ECMO) is used for the management of severe respiratory and cardiac failure and as a bridge to achieve definite treatment or transplantation. ECMO-associated coagulopathy (EAC) is a frequent complication leading to high rates of thrombosis or severe haemorrhage, contributing to morbidity and mortality among patients. Understanding the pathophysiology of EAC is substantial for effectively managing patients on ECMO. We analyse the underlying mechanism of EAC and discuss the monitoring of the coagulation profile, combining the viscoelastic point-of-care assays with the conventional coagulation laboratory tests.

Subphenotypes in Non-Syndromic Orofacial Cleft Patients Based on the Tooth Agenesis Code (TAC).

Background: It was the aim of this study to investigate tooth agenesis patterns, which are expressed to different subphenotypes according to the TAC method in a spectrum of non-syndromic orofacial cleft patients. Methods: A total of 183 orofacial cleft patient records were assessed for tooth agenesis and TAC patterns. The association between TAC and sex, and cleft type was examined, and logistic regression models were additionally applied. Additionally, the distribution of missing teeth by cleft type and the tooth agenesis inter-quadrant association were examined. Results: The most frequent cleft type was CLPL (n = 72; 39.3%), while the maxillary left lateral incisor was the most frequently missing tooth that was strongly dependent on the cleft type (29.5%, p < 0.001). Of the 31 TAC patterns identified, four were the most prevalent and occurred in 80.8% of the sample, while 20 TAC patterns were unique. Cleft type contrary to sex (p = 0.405) was found to play a significant role in TAC distribution (p = 0.001). The logistic regression's results suggested that overall, neither sex nor cleft type were associated with tooth agenesis. Prevalence of tooth agenesis in each quadrant clearly depended on cleft type; and there was a strong association found between tooth agenesis in different quadrants. Conclusions: Thirty-one different subphenotypes were identified in TAC patterns. The first four TAC patterns accounted for the 80.8% of the sample's variability while twenty of the patterns were unique. A strong association was present between TAC pattern and cleft type. No association was found between the sex of the patient, tooth agenesis and TAC patterns. Tooth agenesis depended strongly on the cleft type, and the most frequently missing tooth was the maxillary left lateral incisor. The interquadrant association for tooth agenesis found suggests a genetic link in the etiology of clefts.

Platelet, Fibrinolytic and Other Coagulation Abnormalities in Newly-Diagnosed Patients with Chronic Thromboembolic Pulmonary Hypertension.

The pathophysiological background of chronic thromboembolic pulmonary hypertension (CTEPH) has not been fully elucidated. Evidence suggests that abnormal platelet function and ineffective fibrinolysis may play a key role in the development of the disease. The purpose of this study was to evaluate platelet and coagulation function in CTEPH, using non-conventional global coagulation assays, and platelet activation and endothelial dysfunction laboratory markers. A total of 40 newly-diagnosed CTEPH patients were studied, along with 35 healthy controls. Blood samples from CTEPH patients were taken directly from the pulmonary artery. All subjects were assessed with platelet function analyzer-100, light transmission aggregometry, thromboelastometry, endogenous thrombin potential. von Willebrand antigen and activity, p-selectin, thromboxane A2 and serotonin levels were also assessed. The results showed that CTEPH patients present diminished platelet aggregation, presence of disaggregation, decreased rate of fibrinolysis, defective thrombin generation and increased levels of thromboxane A2, p-selectin, von Willebrand antigen and activity. Serotonin levels did not present any differences between the two groups. The results of this study suggest that CTEPH patients present platelet function, fibrinolytic, thrombin generation and other clot formation abnormalities. Well-designed clinical studies are needed to further evaluate the complex hemostatic abnormalities in the CTEPH setting and assess their potential clinical applications.

Orofacial Muscle Weakening in Facioscapulohumeral Muscular Dystrophy (FSHD) Patients.

BACKGROUND: Facioscapulohumeral muscular dystrophy is the third most commonly found type of muscular dystrophy. The aim of this study was to correlate the D4Z4 repeat array fragment size to the orofacial muscle weakening exhibited in a group of patients with a genetically supported diagnosis of FSHD. METHODS: Molecular genetic analysis was performed for 52 patients (27 female and 25 male) from a group that consisted of 36 patients with autosomal dominant pedigrees and 16 patients with either sporadic or unknown family status. The patients were tested with the southern blotting technique, using EcoRI/Avrll double digestion, and fragments were detected by a p13E-11 telomeric probe. Spearman's correlation was used to compare the fragment size with the degree of muscle weakening found in the forehead, periocular and perioral muscles. RESULTS: A positive non-significant correlation between the DNA fragment size and severity of muscle weakness was found for the forehead (r = 0.27; p = 0187), the periocular (r = 0.24; p = 0.232) and the left and right perioral (r = 0.29; p = 0.122), (r = 0.32; p = 0.085) muscles. CONCLUSIONS: Although FSHD patients exhibited a decrease in muscular activity related to the forehead, perioral, and periocular muscles the genotype-phenotype associations confirmed a weak to moderate non-significant correlation between repeat size and the severity of muscle weakness. Orofacial muscle weakening and its association with a D4Z4 contraction alone may not have the significance to serve as a prognostic biomarker, due to the weak to moderate association. Further studies with larger sample sizes are needed to determine the degree of genetic involvement in the facial growth in FSHD patients.

The Role of Exercise Doppler Echocardiography to Unmask Pulmonary Arterial Hypertension in Selected Patients with Systemic Sclerosis and Equivocal Baseline Echocardiographic Values for Pulmonary Hypertension.

Recently, a lower mean pulmonary arterial pressure (PAP) cutoff of >20 mmHg for pulmonary hypertension (PH) definition has been proposed. We examined whether exercise Doppler echocardiography (EDE) can unmask PA hypertension (PAH) in systemic sclerosis (SSc) patients whose baseline echocardiography for PH is equivocal. We enrolled 49 patients with SSc who underwent treadmill EDE. Tricuspid regurgitation (TR) velocity was recorded immediately after EDE. Inotropic reserve of right ventricle (RV) was assessed by the change (post-prior to exercise) of tissue Doppler imaging-derived peak systolic velocity (S) of tricuspid annulus. Inclusion criteria comprised preserved left and RV function, and baseline TR velocity between 2.7 and 3.2 m/s. All patients had right-heart catheterization (RHC) within 48 h after EDE. From 46 patients with good quality of post-exercise TR velocity, RHC confirmed PAH in 21 (45.6%). Post-exercise TR velocity >3.4 m/s had a sensitivity of 90.5%, a specificity of 80% and an accuracy of 84.8% in detecting PAH. Inotropic reserve of RV was positively correlated with maximum achieved workload in METs (r = 0.571, p < 0.001). EDE has a good diagnostic accuracy for the identification of PAH in selected SSc patients whose baseline echocardiographic measurements for PH lie in the gray zone, and it is also potentially useful in assessing RV contractile reserve.

Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

A Severe COVID-19 Case Complicated by Right Atrium Thrombus.

BACKGROUND Recent studies demonstrated evidence of coagulation dysfunction in hospitalized patients with severe coronavirus disease 2019 (COVID-19) due to excessive inflammation, hypoxia, platelet activation, endothelial dysfunction, and stasis. Effective anticoagulation therapy may play a dominant role in the management of severe COVID-19 cases. CASE REPORT A 73-year-old man with a 6-day history of fever up to 38.5°C, dyspnea, cough, and fatigue was diagnosed with COVID-19. He had a past medical history significant for hypertension and coronary artery bypass grafting. Two days after hospital admission, the patient developed acute respiratory failure, requiring intubation, mechanical ventilation, and transfer to the intensive care unit (ICU). He received treatment including antibiotics, hydroxychloroquine, tocilizumab, vasopressors, prone positioning, and anticoagulation with enoxaparin at a prophylactic dose. After a 15-day ICU stay, the patient was hemodynamically stable but still hypoxemic; a transthoracic echocardiogram at that time, followed by a transesophageal echocardiogram for better evaluation, revealed the presence of a right atrium thrombus without signs of acute right ventricular dilatation and impaired systolic function. Since the patient was hemodynamically stable, we decided to treat him with conventional anticoagulation under close monitoring for signs of hemodynamic deterioration; thus, the prophylactic dose of enoxaparin was replaced by therapeutic dosing, which was a key component of the patient's successful outcome. Over the next few days he showed significant clinical improvement. The follow-up transesophageal echocardiogram 3 weeks after effective therapeutic anticoagulation revealed no signs of right heart thrombus. CONCLUSIONS The presented COVID-19 case, one of the first reported cases with evidence of right heart thrombus by transesophageal echocardiography, highlights the central role of diagnostic imaging strategies and the importance of adequate anticoagulation therapy in the management of severe COVID-19 cases in the ICU.