Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 169
Filter
1.
Am J Cardiol ; 205: 63-68, 2023 10 15.
Article in English | MEDLINE | ID: mdl-37586123

ABSTRACT

Coronary artery ectasia (CAE) is defined as local or generalized aneurysmal dilatation of the coronary arteries. CAE likely represents an exaggerated form of excessive vascular wall remodeling in different clinical settings such as atherosclerosis, vasculitides, connective tissue disorders, hereditary collagen defects, bacterial infections, and congenital malformations. In the present case-control study, we investigated whether the incidental finding of CAE in patients who undergo coronary angiography is associated with presence of autoimmune reactivity. From 2019 to 2022, we identified all consecutive patients with CAE (n = 319) on elective or emergency coronary angiography (n = 7,458). We furthermore included 90 patients with nonectatic coronary arteries as a control group. Antinuclear antibody (ANA) titer was measured in both groups using the indirect immunofluorescence method from peripheral blood samples. The prevalence of CAE in our study cohort was 4.3%. Among patients with CAE (n = 319), presence of positive Antinuclear antibody (ANA) titer was identified in 128 patients (40%). Only 18 patients (20%) from the control group had positive ANA titer. There was a statistically significant greater percentage of patients with positive ANA titer among patients with CAE than among controls (chi-square = 12.39; p <0.001), with an odds ratio of 2.68. Among patients with CAE, there is an increased prevalence of positive ANA titer, suggesting an underlying autoimmune disease. Screening for autoimmune reactivity could be a reasonable diagnostic strategy in patients who undergo coronary angiography with an incidental finding of coronary ectasia because the number needed to screen for positive ANA titer in this subgroup of patients is only 5.


Subject(s)
Autoimmune Diseases , Coronary Aneurysm , Coronary Artery Disease , Humans , Dilatation, Pathologic/epidemiology , Coronary Vessels/diagnostic imaging , Case-Control Studies , Antibodies, Antinuclear , Cross-Sectional Studies , Coronary Aneurysm/epidemiology , Coronary Angiography/methods , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases/epidemiology , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology
2.
Tohoku J Exp Med ; 261(2): 165-171, 2023 Oct 24.
Article in English | MEDLINE | ID: mdl-37558418

ABSTRACT

Coronary artery ectasia (CAE) is defined as segmental dilatation with a diameter of 1.5-fold greater than that of an adjacent normal segment. Whether CAE is a unique clinical finding or results from other clinical entities remains to be determined. The purpose of the study was to investigate the prevalence, and clinical and angiographic characteristics of CAE in patients with coronary artery disease (CAD). Among the 8,845 coronary angiograms reviewed between the years 2014 and 2022, 142 patients had CAE yielding a detection rate of 4.9% among 2,870 CAD angiograms, and 28 patients had isolated CAE showing a detection rate of 0.32% (28/8,845) among total coronary angiography procedures. Overall, the incidence of CAE was 1.92% (170/8,845). The most commonly affected coronary artery by ectasia was the right coronary artery (RCA) (46.28%) among CAE coexisting with CAD cohort. The proportion of obesity, family history of CAD, and the proportion of hyperlipidemia in CAD patients who had ectasia were significantly higher than that in CAD patients who did not have ectasia (P < 0.05). In conclusion, CAE is an uncommon finding in coronary angiography, most commonly affecting the RCA. The obesity, family history of CAD, and the coexistence of hyperlipidemia were independent variables associated with CAE in CAD patients.


Subject(s)
Coronary Aneurysm , Coronary Artery Disease , Hyperlipidemias , Humans , Coronary Artery Disease/complications , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology , Retrospective Studies , Dilatation, Pathologic/complications , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/epidemiology , Coronary Vessels/diagnostic imaging , Prevalence , Coronary Angiography/methods , Obesity
3.
Eur Heart J ; 44(33): 3152-3164, 2023 09 01.
Article in English | MEDLINE | ID: mdl-37288540

ABSTRACT

AIMS: International guidelines recommend screening of first-degree relatives (FDR) of people with bicuspid aortic valves (BAVs). However, the prevalence of BAV and of aortic dilatation amongst family members is uncertain. METHODS AND RESULTS: A systematic review and meta-analysis of original reports of screening for BAV. Databases including MEDLINE, Embase, and Cochrane CENTRAL were searched from inception to December 2021 using relevant search terms. Data were sought on the screened prevalence of BAV and aortic dilatation. The protocol was specified prior to the searches being performed, and standard meta-analytic techniques were used. Twenty-three observational studies met inclusion criteria (n = 2297 index cases; n = 6054 screened relatives). The prevalence of BAV amongst relatives was 7.3% [95% confidence interval (CI) 6.1%-8.6%] overall and per family was 23.6% (95% CI 18.1%-29.5%). The prevalence of aortic dilatation amongst relatives was 9.4% (95% CI 5.7%-13.9%). Whilst the prevalence of aortic dilatation was particularly high in relatives with BAV (29.2%; 95% CI 15.3%-45.1%), aortic dilatation alongside tricuspid aortic valves was a more frequent finding, as there were many more family members with tricuspid valves than BAV. The prevalence estimate amongst relatives with tricuspid valves (7.0%; 95% CI 3.2%-12.0%) was higher than reported in the general population. CONCLUSION: Screening family members of people with BAV can identify a cohort substantially enriched for the presence of bicuspid valve, aortic enlargement, or both. The implications for screening programmes are discussed, including in particular the substantial current uncertainties regarding the clinical implications of aortic findings.


Subject(s)
Aortic Diseases , Bicuspid Aortic Valve Disease , Heart Valve Diseases , Humans , Heart Valve Diseases/epidemiology , Heart Valve Diseases/genetics , Heart Valve Diseases/diagnosis , Dilatation , Aortic Valve , Aortic Diseases/diagnosis , Dilatation, Pathologic/epidemiology , Retrospective Studies
4.
Angiology ; 74(9): 876-880, 2023 10.
Article in English | MEDLINE | ID: mdl-36113406

ABSTRACT

Coronary artery ectasia (CAE) is defined as abnormal dilation of a coronary artery ≥1.5 times the normal segment. We aimed to determine the prevalence and clinical predictors of CAE. This was a prospective analysis performed on 6465 patients undergoing coronary angiography. Patients were divided based on the presence or absence of CAE and compared for angiographic characteristics and clinical risk factors. The prevalence of CAE was 7%, CAE associated with coronary artery stenosis was 5.4%, and isolated CAE was 1.6%. The mean age of presentation in CAE patients was 60 years, with male predominance (83.8%) and stable angina was the most common presentation. The left anterior descending artery (LAD) (51.7%) was the most commonly involved vessel, with diffuse ectasia more commonly seen in right coronary artery and discrete ectasia in LAD. Type 4 CAE was the most common type (92.4%). Hypertension, diabetes, smoking, dyslipidemia, and obesity were found in 62.4%, 35.3%, 45.3%, 54.9%, and 23.3%, respectively in CAE patients, with significant association with smoking (Odds Ratio = 3.06). The prevalence of CAE was 7% and was frequently associated with atherosclerotic coronary disease. Smoking was a significant predisposing factor for CAE.


Subject(s)
Coronary Disease , Coronary Vessels , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/pathology , Coronary Vessels/diagnostic imaging , Coronary Vessels/pathology , Coronary Angiography , Humans , Male , Female , Middle Aged , Prevalence , Sex Factors , Coronary Disease/diagnostic imaging , Coronary Disease/epidemiology , Prospective Studies , India/epidemiology
5.
Optom Vis Sci ; 99(7): 560-567, 2022 07 01.
Article in English | MEDLINE | ID: mdl-35446302

ABSTRACT

SIGNIFICANCE: The article highlights various topographic patterns and their prevalence in a large spectrum of ectatic corneal diseases (ECDs). Knowledge of these patterns can help clinicians for quicker diagnosis and selection of appropriate contact lens design. PURPOSE: This study aimed to determine various corneal topography patterns and their prevalence in patients with ECDs who visited a tertiary eye hospital in western India. METHODS: Keratoconus, pellucid marginal degeneration, keratoglobus, and post-refractive surgery progressive corneal ectasia are considered under ECDs. This cross-sectional retrospective study reviewed records of 632 consecutive patients with clinical ECDs at their first presentation. The right eye was considered for pattern analysis. In cases with suspected or forme fruste ectasia in the right eye, the fellow eye was considered. A sagittal map with standard scale of Atlas 9000 topographer (Carl Zeiss Meditec AG, Jena, Germany) was used for pattern analysis. They were classified into 18 categories and grouped under five groups. The prevalence of these patterns was calculated and assessed with 95% confidence interval (CI). RESULTS: The mean ± SD age of patients was 23.6 ± 8.2 years. The highest prevalence was of asymmetric patterns (39.6% [95% CI, 35.7 to 43.5%]; asymmetric bowtie [AB] with steepest radial axis index [SRAX], 18.8%; AB with inferior steep, 16.0%; AB with superior steep [SS], 3.2%; symmetric bowtie with SRAX, 1.6%) and of central or paracentral patterns (28.6% [95% CI, 25.1 to 32.3%]; inferior steep, 12.2%; heart, 7.4%; oval, 4.1%; symmetric bowtie, 2.4%; round, 1.6%; irregular, 0.9%) followed by advanced patterns (17.3% [95% CI, 14.4 to 20.4%; nonmeasurable, 5.4%; globus, 4.9%; indiscriminate, 7.0%). The peripheral patterns were 11.7% (95% CI, 9.3 to 14.4%) (claw, 6.3%; junctional, 3.2%; butterfly, 1.9%; SS, 0.3%). Rare patterns were 2.8% (95% CI, 1.7 to 4.5%) (superior [junctional, claw, and heart], AB with SS with SRAX, and AB with SRAX located temporally). CONCLUSIONS: Asymmetric and central or paracentral are the most common patterns in our study. The higher prevalence of advanced patterns indicates the need for earlier diagnosis of ECDs in our population. The peripheral patterns also have significant prevalence.


Subject(s)
Corneal Diseases , Keratoconus , Adolescent , Adult , Cornea , Corneal Diseases/diagnosis , Corneal Diseases/epidemiology , Corneal Topography , Cross-Sectional Studies , Dilatation, Pathologic/epidemiology , Humans , Keratoconus/diagnosis , Keratoconus/epidemiology , Prevalence , Retrospective Studies , Young Adult
6.
Sci Rep ; 11(1): 22436, 2021 11 17.
Article in English | MEDLINE | ID: mdl-34789864

ABSTRACT

In this study, corneal findings regarding keratoconus (KC) and early KC among mothers with Down syndrome children (MDS) and a group of age-at-delivery-matched mothers with normal children (MNC) were compared. KC was diagnosed based on the presence of a clinical sign and at least one abnormal tomographic or biomechanical criterion. Early KC was defined as having no clinical sign in the presence of at least one abnormal tomographic or biomechanical criterion. The normal subgroups in each group were compared in terms tomographic and biomechanical parameters. In MDS and MNC, the prevalence rates were 6.5% and 1.6% for KC (P = 0.047), and 30.9% and 14.3% for early KC (P = 0.014), respectively. Comparison between the two normal subgroups showed significant differences in mean index of height asymmetry, irregularity index, anterior asphericity, pentacam random forest index, corneal stiffness parameters at first applanation, deformation amplitude ratios, integrated radius-1 mm, highest concavity deflection amplitude, biomechanical corrected IOP, peak distance, and radius (all P < 0.05). This study showed that MDS are more likely to have KC and also to have thinner, steeper and softer corneas compared to MNC. This results support the need for further work for determining the risk of delivering a child with DS.


Subject(s)
Down Syndrome/epidemiology , Keratoconus/diagnosis , Keratoconus/epidemiology , Mothers , Adult , Child , Cornea/pathology , Corneal Pachymetry/methods , Corneal Topography/methods , Dilatation, Pathologic/diagnosis , Dilatation, Pathologic/epidemiology , Female , Humans , Iran/epidemiology , Middle Aged , Prevalence , Retinoscopy/methods
7.
Eur J Vasc Endovasc Surg ; 62(1): 26-35, 2021 Jul.
Article in English | MEDLINE | ID: mdl-34090782

ABSTRACT

OBJECTIVE: Aortic neck dilatation (AND) occurs after endovascular aneurysm repair (EVAR) with self expanding stent grafts (SESs). Whether it continues, ultimately exceeding the endograft diameter leading to abdominal aortic aneurysm (AAA) rupture, remains uncertain. Dynamics, risk factors, and clinical relevance of AND were investigated after EVAR with standard SESs. METHODS: All intact EVAR patients treated from 2000 to 2015 at a tertiary institution were included. Demographic, anatomical, and device related characteristics were investigated as risk factors for AND. Outer to outer diameters were measured at a single standardised aortic level on reconstructed computed tomography (CT) images. RESULTS: A total of 460 patients were included (median follow up 5.2 years, interquartile range [IQR] 3.0, 7.7 years; CT imaging follow up 3.3 years, IQR 1.3, 5.4). Baseline neck diameter was 24 mm (IQR 22, 26) and increased 11.1% (IQR 1.5%, 21.9%) at last CT imaging. Endograft oversizing was 20.0% (IQR 13.6, 28.0). AND was greater during the first year (5.2% [IQR 0, 11.7]) decreasing subsequently (two to four years to 1.4%/year [IQR 0.0, 4.5%], p ≤ .001) and was associated with suprarenal fixation endografts (t value = 7.9, p < .001) and oversizing (t value = 4.4, p < .001). AND exceeding the endograft was 3.5% (95% CI 2.2% - 4.8%) and 14.4% (95% CI 11.0% - 17.8%) at five and eight years, respectively. Excessive AND was associated with baseline neck diameter (OR 1.2/mm, 95% CI 1.05 - 1.41) while the Excluder endograft had a protective effect (OR 0.15, 95% CI 0.04 - 0.58). Excessive AND was associated with type 1A endoleak (HR 3.3, 95% CI 1.1 - 9.7) and endograft migration > 5 mm (HR 3.1, 95% CI 1.4 - 6.9). CONCLUSION: AND after EVAR with SES is associated with endograft oversizing and radial force but decelerates after the first post-operative year. Baseline aortic neck diameter and suprarenal stent bearing endografts were associated with an increased risk of AND beyond nominal stent graft diameter. However, it remains unclear whether patient selection, differences in endograft radial force or the suprarenal stent are accountable for this difference.


Subject(s)
Aortic Aneurysm, Abdominal/surgery , Aortic Rupture/epidemiology , Dilatation, Pathologic/epidemiology , Endoleak/epidemiology , Endovascular Procedures/adverse effects , Foreign-Body Migration/epidemiology , Aged , Aorta, Abdominal/diagnostic imaging , Aorta, Abdominal/surgery , Aortic Aneurysm, Abdominal/complications , Aortic Rupture/etiology , Aortography , Computed Tomography Angiography , Dilatation, Pathologic/diagnosis , Dilatation, Pathologic/etiology , Endoleak/diagnosis , Endoleak/etiology , Endovascular Procedures/instrumentation , Female , Foreign-Body Migration/etiology , Humans , Male , Neck , Retrospective Studies , Risk Factors , Stents/adverse effects , Treatment Outcome
9.
Gac Med Mex ; 157(6): 604-609, 2021.
Article in English | MEDLINE | ID: mdl-35108253

ABSTRACT

INTRODUCTION: Coronary ectasia has a low prevalence in the general population. Its risk factors may differ from those of coronary artery disease. OBJECTIVE: To identify the prevalence of coronary ectasia in patients with acute myocardial infarction (AMI) and cardiovascular risk factors (CVRFs). METHODS: Retrospective, cross-sectional study. Out of 3,254 cardiac catheterizations for AMI during one year, 2,975 had no coronary ectasia. We included 558 patients with coronary ectasia on coronary angiography and, as controls, subjects with similar characteristics except for coronary ectasia, and CVRFs were recorded. Descriptive statistics, bivariate and multivariate analysis were used; odds ratio (OR) was calculated. RESULTS: 279 patients with and without coronary ectasia were studied. The prevalence of coronary ectasia was 8.5 %. The platelet/lymphocyte ratio (PLR) was higher in patients with ectasia than in those without ectasia (p = 0.003). In the bivariate analysis, associated CVRFs were overweight, obesity and diabetes, and in the multivariate analysis, hypercholesterolemia (OR: 3.90; p = 0.0001) and exposure to herbicides (OR: 6.82; p = 0.020). CONCLUSIONS: A high prevalence of coronary ectasia was found, with the main risk factors being a history of herbicide use and hypercholesterolemia. PLR was found to be elevated in these patients. Early detection is important due to its association with acute coronary events.


INTRODUCCIÓN: La ectasia coronaria tiene baja prevalencia en población general, los factores de riesgo pueden diferir de la enfermedad arterial coronaria. OBJETIVO: Identificar la prevalencia de ectasia coronaria en pacientes con infarto agudo de miocardio (IAM) y factores de riesgo cardiovascular (FRCV). MÉTODOS: Estudio retrospectivo, transversal. De 3,254 cateterismos cardiacos por IAM durante un año, 2,975 no presentaron ectasia coronaria. Se incluyeron 558 pacientes clasificados como portadores de ectasia coronaria en coronariografía y controles aquellos con características similares exceptuando la ectasia coronaria y se registraron los FRCV. Empleamos estadística descriptiva, análisis bivariante, multivariante y calculamos el odds ratio (OR). RESULTADOS: Se estudiaron 279 pacientes con y sin ectasia coronaria. La prevalencia de ectasia coronaria fue del 8.5%. El índice plaqueta/linfocito (IPL) se encontró más elevado en pacientes con ectasia que en aquellos sin ectasia (p = 0.003). En el análisis bivariante los FRCV asociados fueron sobrepeso, obesidad y diabetes, y en el multivariante la hipercolesterolemia (OR: 3.90; p = 0.0001) y exposición a herbicidas (OR: 6.82; p = 0.020). CONCLUSIONES: Encontramos alta prevalencia de ectasia coronaria, los principales factores de riesgo fueron el antecedente de uso de herbicidas e hipercolesterolemia. Identificamos el IPL elevado en estos pacientes. Es importante la detección oportuna debido a su asociación con eventos coronarios agudos.


Subject(s)
Cardiovascular Diseases , Myocardial Infarction , Cross-Sectional Studies , Dilatation, Pathologic/epidemiology , Heart Disease Risk Factors , Humans , Myocardial Infarction/epidemiology , Retrospective Studies , Risk Factors
10.
Eur J Endocrinol ; 183(4): 463-470, 2020 Oct.
Article in English | MEDLINE | ID: mdl-32822316

ABSTRACT

OBJECTIVE: Turner syndrome (TS) is a rare disorder affecting 1/2500 female newborn. Aortic dilatation (AD) and aortic dissection represent a major concern in TS. The aims of our study were to describe the aortic root growth, potential aortic dilatation (AD) risk factors and cardiovascular outcomes in a cohort of patients with TS. METHODS: Among 204 adult patients included, 197 were studied using a standardized 1.5 Tesla MRI protocol. AD was defined as an aortic diameter ≥20 mm/m2 at the Valsalva sinuses and/or at the ascending aorta, when indexed to body surface area. RESULTS: At baseline, AD was present in 81/197 (41.1%) and 32/197 (16.2%) of patients, at the levels of Valsalva and ascending aorta, respectively. The aortic Valsalva diameter was larger in patients treated for thyroiditis (P < 0.001). Potential risk factors of AD were aging (P < 0.001) and the presence of bicuspid aortic valve (BAV) (P = 0.002). The hazard ratio (HR) of AD occurrence in the presence of BAV was 2.2 (95% CI: 1.33-3.71). After a median follow-up period of 5.1 years (n = 143), AD was present in 58/143 (40.6%) and 25/143 (17.5%) of patients at the levels of Valsalva and ascending aorta, respectively. The median aortic growth of the Valsalva sinuses remained stable. At the ascending aorta, it increased by 0.14 ± 0.61 mm/year. Only one aortic-related death was observed. CONCLUSION: AD is common in adult patients with TS. However, our results are rather reassuring, as the median aortic diameters remained stable after 5.1 years and few aortic events were observed.


Subject(s)
Aortic Diseases/epidemiology , Turner Syndrome/epidemiology , Adult , Aorta/diagnostic imaging , Aorta/pathology , Aortic Diseases/complications , Aortic Diseases/diagnosis , Aortic Valve/abnormalities , Aortic Valve/pathology , Bicuspid Aortic Valve Disease , Cohort Studies , Dilatation, Pathologic/complications , Dilatation, Pathologic/diagnosis , Dilatation, Pathologic/epidemiology , Disease Progression , Female , France/epidemiology , Heart Valve Diseases/complications , Heart Valve Diseases/diagnosis , Heart Valve Diseases/epidemiology , Heart Valve Diseases/pathology , Humans , Male , Prevalence , Turner Syndrome/complications , Young Adult
11.
Am J Cardiol ; 128: 210-215, 2020 08 01.
Article in English | MEDLINE | ID: mdl-32534732

ABSTRACT

The clinical and imaging differences between bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) patients with medically managed asymptomatic moderate-to-severe aortic stenosis (AS) have not been studied previously. We aim to characterize these differences and their clinical outcomes in this study. A retrospective observational study was conducted on 836 consecutive cases of isolated asymptomatic moderate-to-severe AS, with median follow-up of 3.4 years. Clinical and echocardiographic characteristics were compared between BAV and TAV patients. Subgroup analysis stratified by AS severity were performed. Survival analysis of all-cause mortality was performed using Kaplan-Meier curves and Cox proportional hazards model. Compared to BAV patients, TAV patients were older (76 ± 11 vs 55 ± 16 years, p <0.001) and had more co-morbidities including hypertension (78% vs 56%; p <0.001), diabetes (41% vs 24%; p <0.001), and chronic kidney disease (20% vs 3%; p = 0.001). TAV patients had less severe aortic valve disease than BAV patients, with a higher aortic valve area index (0.71 ± 0.20 cm2/m2 vs 0.61 ± 0.18 cm2/m2, p <0.001) and less aortic dilation (sinotubular junction: 23.7 ± 4.0 mm vs 26.9 ± 4.8 mm, p <0.001; mid-ascending aorta: 31.4 ± 4.7 mm vs 36.3 ± 6.3 mm, p <0.001). TAV patients were more likely to have eccentric left ventricular hypertrophy and less likely to have a normal geometry (p = 0.003). Competing risk analysis identified increased age (hazard ratio 1.03, 95% confidence interval 1.02 to 1.05, p <0.001) and LVEF (hazard ratio 0.98, 95% confidence interval 0.97 to 0.99, p <0.001) as independent risk factors of all-cause mortality. Valve morphology was not a significant independent risk factor for aortic valve replacement or mortality. In conclusion, asymptomatic TAV patients had more cardiovascular risk factors, less severe aortic valve disease, less sinotubular and mid-ascending aortic dilation, more severe LV remodeling.


Subject(s)
Aortic Valve Stenosis/diagnostic imaging , Aortic Valve/abnormalities , Asymptomatic Diseases , Heart Valve Diseases/diagnostic imaging , Adult , Age Factors , Aged , Aged, 80 and over , Aortic Diseases/diagnostic imaging , Aortic Diseases/epidemiology , Aortic Diseases/physiopathology , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Aortic Valve Stenosis/complications , Aortic Valve Stenosis/epidemiology , Aortic Valve Stenosis/physiopathology , Bicuspid Aortic Valve Disease , Case-Control Studies , Cause of Death , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/physiopathology , Echocardiography , Female , Heart Valve Diseases/complications , Heart Valve Diseases/epidemiology , Heart Valve Diseases/physiopathology , Heart Valve Prosthesis Implantation/statistics & numerical data , Humans , Hypertrophy, Left Ventricular/diagnostic imaging , Hypertrophy, Left Ventricular/epidemiology , Hypertrophy, Left Ventricular/physiopathology , Kaplan-Meier Estimate , Male , Middle Aged , Mortality , Proportional Hazards Models , Retrospective Studies , Risk Factors , Severity of Illness Index , Stroke Volume
12.
Cardiol Young ; 30(5): 663-667, 2020 May.
Article in English | MEDLINE | ID: mdl-32290873

ABSTRACT

BACKGROUND: Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity. METHODS: A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical-instrumental-genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve. RESULTS: Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004). CONCLUSIONS: In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis.


Subject(s)
Bicuspid Aortic Valve Disease/epidemiology , Marfan Syndrome/complications , Medical History Taking , Sinus of Valsalva/pathology , Adolescent , Bicuspid Aortic Valve Disease/etiology , Child , Child, Preschool , Cohort Studies , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/etiology , Echocardiography , Female , Humans , Male , Young Adult
13.
Ophthalmic Epidemiol ; 27(4): 289-297, 2020 08.
Article in English | MEDLINE | ID: mdl-32172662

ABSTRACT

PURPOSE: To investigate the corneal topometric and tomographic findings that can be used in the diagnosis of subclinical keratoconus. METHODS: A retrospective cohort study. The study group was selected from patients with clinically evident keratoconus in one eye and subclinical keratoconus without evident topographic findings in fellow eye. The age-matched control group was selected from patients who were candidates for laser in situ keratomileusis (LASIK) and did not develop ectasia after LASIK surgery at least 1-year follow-up. All subjects underwent topographic, topometric and tomographic (Belin-Ambrósio Enhanced Ectasia Display III) analyses via a Pentacam HR rotating Scheimpflug camera (Oculus, Germany, version 1.20r.98) before LASIK surgery. RESULTS: The study group consisted of 151 patients (69 male and 82 female, mean age of 24.8 ± 7.2 years) and the control group also consisted of 150 patients (70 male and 80 female, mean age of 26.0 ± 6.3 years). There were statistically significant differences in all measured topometric (p˂.05) and tomographic (p˂.001) parameters between the eyes with subclinical keratoconus and those of the control group. In discriminating eyes with subclinical keratoconus from normal eyes, final D showed the highest area under curve value (0.858, sensitivity 85.2%, specificity 66.7%), followed by maximum pachymetric progression index (0.809, sensitivity 81.9%, specificity 69.4%) and average pachymetric progression index (0.796, sensitivity 81.9%, specificity 68.1%) in receiver operating characteristic analysis. CONCLUSION: Topometric and tomographic parameters might be useful for early detection of keratoconus, but the sensitivity and specificity of any parameter are not high enough to be used alone.


Subject(s)
Cornea/diagnostic imaging , Corneal Topography/methods , Keratoconus/diagnosis , Tomography/methods , Adolescent , Adult , Area Under Curve , Case-Control Studies , Cornea/pathology , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/etiology , Early Diagnosis , Female , Follow-Up Studies , Humans , Keratomileusis, Laser In Situ/adverse effects , Keratomileusis, Laser In Situ/methods , Male , ROC Curve , Retrospective Studies , Sensitivity and Specificity , Young Adult
15.
Transplantation ; 104(1): 145-153, 2020 01.
Article in English | MEDLINE | ID: mdl-31343566

ABSTRACT

BACKGROUND: Urological obstructive complications (UOC) affect up to 15% of kidney transplants (KTX). Most cases are excluded by ultrasonography (US); however, accuracy may be limited in the early transplant phase. Features of acute tubular injury (ATI) in KTX biopsy may be informative but histological features indicating UOC are ill defined. Tubular ectasia (TE) was shown to be associated with UOC in experimental data. We evaluated the association of histomorphological features, particularly TE, with occult (=without relevant hydronephrosis in US) UOC and renal outcomes. METHODS: We included all recipients with an early indication biopsy (976 of 1537 consecutive KTX). The biopsy finding of TE classified as "suspicious of UOC" was compared with the following endpoints: delayed graft function, estimated glomerular filtration rate, and occult UOC. Additionally, histopathological features of ATI were reevaluated by a single pathologist to increase diagnostic accuracy. RESULTS: Fifty-eight (5.9%) patients presented with TE, which was not related to delayed graft function or estimated glomerular filtration rate. Forty percent of patients had a UOC (most frequently ureteral stenosis) close to biopsy. Comparing these biopsies to matched controls, TE was significantly associated with UOC (odds ratio 2.69; P = 0.018). After histopathological reevaluation of these biopsies including additional features of ATI, we developed a final multivariate model with a highly significant relationship to UOC (Receiver operating characteristic-area under the curve: 0.77; P = 0.001). The model provides a specificity of 78% and negative predictive value of 73%. CONCLUSIONS: TE together with additional signs of ATI indicates occult UOC. This histological phenotype should trigger more detailed evaluation for UOC when there is no evidence of relevant hydronephrosis in the ultrasonography.


Subject(s)
Allografts/pathology , Kidney Transplantation/adverse effects , Kidney Tubules/pathology , Postoperative Complications/epidemiology , Ureteral Obstruction/epidemiology , Adult , Allografts/physiopathology , Biopsy , Delayed Graft Function/epidemiology , Delayed Graft Function/physiopathology , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/etiology , Dilatation, Pathologic/pathology , Female , Glomerular Filtration Rate/physiology , Humans , Kidney , Kidney Tubules/physiopathology , Male , Middle Aged , Models, Statistical , Multivariate Analysis , Postoperative Complications/etiology , Postoperative Complications/pathology , Postoperative Complications/physiopathology , Predictive Value of Tests , Retrospective Studies , Risk Assessment , Transplantation, Homologous/adverse effects , Ureteral Obstruction/etiology , Ureteral Obstruction/pathology , Ureteral Obstruction/physiopathology
16.
J Fr Ophtalmol ; 43(1): 18-24, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31831272

ABSTRACT

PURPOSE: To determine the indications, frequency, influential factors and clinical outcomes of resuturing in an atraumatic setting after penetrating keratoplasty (PK). METHODS: Medical records of all patients who underwent resuturing in the absence of traumatic wound dehiscences after PK between January 1, 2007 and December 31, 2015 were reviewed. The cases were divided into 2 groups: patients with suture-related problems underwent mandatory resuturing (mandatory group), and patients with post-PK ectasia or a progressive increase in K values and surgically induced astigmatism underwent optional resuturing (Optional group). Patient demographics and surgical indications for PK, reasons for and frequency of resuturing, time between PK and resuturing, and clinical outcomes were evaluated. RESULTS: The frequency of resuturing was 9.03% (59 of 633), and the mean age was 39.15±17.80 years. The most common indication for PK was keratoconus (42.4%) and the interval between PK and resuturing ranged from 0.03 to 32 months. The underlying cause leading to resuturing was suture-related problems in 43 eyes (72.9%), development of ectasia or progressive steepening of the K values and surgically induced astigmatism in 16 eyes (27.1%). The mean visual acuity increased, the K value and astigmatism decreased significantly following resuturing in both the mandatory group and the Optional group (P≤0.2). The decrease in astigmatism and K values was more marked in the Optional group, as expected (P≤0.001). CONCLUSION: While resuturing is essential in order to obtain wound integrity in the setting of dehiscence, it is effective in terms of achieving higher visual acuities and lower astigmatism and K values in high astigmatism and post-PK ectasia cases.


Subject(s)
Astigmatism/surgery , Keratoplasty, Penetrating , Reoperation , Suture Techniques , Adolescent , Adult , Aged , Aged, 80 and over , Astigmatism/epidemiology , Cornea/surgery , Dilatation, Pathologic/epidemiology , Dilatation, Pathologic/surgery , Female , Humans , Keratoconus/surgery , Keratoplasty, Penetrating/adverse effects , Keratoplasty, Penetrating/statistics & numerical data , Male , Middle Aged , Postoperative Complications/epidemiology , Postoperative Complications/surgery , Reoperation/methods , Reoperation/statistics & numerical data , Retrospective Studies , Suture Techniques/adverse effects , Suture Techniques/statistics & numerical data , Sutures , Wound Healing/physiology , Young Adult
18.
J Vasc Surg ; 70(6): 1801-1808, 2019 12.
Article in English | MEDLINE | ID: mdl-31327605

ABSTRACT

OBJECTIVE: The prevalence of intracranial aneurysms (IAs) is higher in patients with aortic aneurysms. However, there are lack of studies investigating prevalence of thoracic aortic aneurysms (TAAs) in patients with IAs. The objective of this study was to evaluate the prevalence and risk factors for thoracic aortic dilatations (TADs) and TAAs in patients with IAs. METHODS: We retrospectively reviewed data from 1777 patients with diagnosed IAs at our institution between 2006 and 2016. We included 411 patients with saccular IAs and available imaging studies (computed tomography or magnetic resonance imaging) of all thoracic aortic segments. TAD was defined according to age- and sex-matched normograms, and TAA as a diameter of greater than 4.0 cm. RESULTS: A total of 83 patients (20%) had TAD or TAA. The prevalence of TADs and TAAs were 18% (n = 74) and 8% (n = 31) without significant difference between unruptured and ruptured IAs (P = .7). Of the 74 patients with TAD, 22 (30%) had multiple TADs and 66% of the TADs located in the aortic arch. Older age (odds ratio [OR], 1.04; P = .006), rheumatic disease (OR, 4.73; P = .009) and alcohol abuse (OR, 4.77; P = .01) were significant risk factors for TAD/TAA. CONCLUSIONS: The prevalence of TADs and TAAs is considerably greater in patients with IAs compared with reports from the general population, suggesting that IAs might be associated with aortopathy and might share a similar pathogenetic background with TADs/TAAs. Especially patients with IAs and a history of rheumatic disease and/or alcohol abuse are at high risk for TADs/TAAs.


Subject(s)
Aortic Aneurysm, Thoracic/complications , Aortic Aneurysm, Thoracic/epidemiology , Aortic Diseases/epidemiology , Intracranial Aneurysm/complications , Aged , Aortic Diseases/pathology , Dilatation, Pathologic/epidemiology , Female , Humans , Male , Middle Aged , Prevalence , Retrospective Studies , Risk Factors
19.
Can J Cardiol ; 35(7): 892-898, 2019 07.
Article in English | MEDLINE | ID: mdl-31292088

ABSTRACT

BACKGROUND: Systematic screening for ascending thoracic aortic aneurysms and dilations does not exist currently with unknown prevalence and diagnostic yields. We evaluated the prevalence of ascending thoracic aortic dilation. METHODS: Computed tomography scans including the chest in adult patients carried out during 2016 were reviewed at our institution. Aortic dilation was defined as the ascending thoracic aorta diameter ≥ 4.0 cm, with sensitivity analyses using height-indexed values and thresholds of 3.5, 4.25, and 4.5 cm. The prevalence of aortic dilation was evaluated by age and sex. Potential diagnostic yield along the continuum of age threshold was calculated by sex. RESULTS: Of the 5662 scans from unique patients, the prevalence of aortic dilation was 2.1% overall, 3.2% for males and 0.9% for females. Patients with aneurysms were significantly older (70.2 ± 9.9 vs 58.3 ± 16.4 years, P < 0.001) and more likely to be male (81.0% vs 54.2%, P < 0.001). The highest diagnostic yield of aneurysm ≥ 4.5 cm in females occurred at the age threshold of ≥ 73 years, with the yield of 0.5%. The highest diagnostic yield of aneurysm ≥ 4.5 cm in males occurred at age ≥ 84 years, with the yield of 5.7%. In males, the diagnostic yields at age thresholds of ≥ 50, ≥ 60, and ≥ 70 years were 1.3%, 1.6%, and 2.2%, respectively. CONCLUSIONS: Aortic dilation was identified in 2.8% of individuals with age ≥ 50 years. In females, aneurysm was uncommon. In males, there was an incremental increase in the diagnostic yield with age. Male patients with age ≥ 50 years may be the demographic group with a high prevalence of dilation.


Subject(s)
Aorta, Thoracic/diagnostic imaging , Aortic Aneurysm, Thoracic/diagnostic imaging , Aortic Aneurysm, Thoracic/epidemiology , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/epidemiology , Age Factors , Aged , Connecticut/epidemiology , Cross-Sectional Studies , Dyslipidemias/epidemiology , Female , Humans , Hypertension/epidemiology , Incidental Findings , Male , Middle Aged , Prevalence , Sex Distribution , Tomography, X-Ray Computed
20.
J Pediatr Urol ; 15(4): 341.e1-341.e6, 2019 Aug.
Article in English | MEDLINE | ID: mdl-31164273

ABSTRACT

BACKGROUND: There are limited data on the predictive value of the consensus urinary tract dilation (UTD) score with respect to subsequent clinical diagnoses. We sought to define the relationship between postnatal UTD risk score and clinical outcomes during childhood. METHODS: Complete ultrasound image sets from a random selection of infants aged 0-90 days undergoing initial ultrasound at a single institution for prenatal hydronephrosis between 2012 and 2014 were assigned a UTD score by 1 pediatric urologist and 1 pediatric radiologist. Urinary tract dilation risk score was analyzed for association with a composite outcome comprising urinary tract infection, vesicoureteral reflux (VUR), ureteropelvic junction obstruction, non-refluxing megaureter (NRM), ureterocele, bladder outlet obstruction (BOO), and chronic kidney disease. Surgical intervention and resolution of UTD were evaluated separately. Descriptive and survival analyses were performed. RESULTS: Urinary tract dilation scores for 494 subjects were P0 in 23.5%, P1 in 26.5%, P2 in 23.5%, and P3 in 26.5%. Seventy-four percent were male. Median age at initial imaging was 28 days; median follow-up was 19.8 months. The composite outcome occurred in 138 of 494 patients (27.9%) and varied significantly (p < 0.001) by UTD score: 11.2% for P0, 10.7% for P1, 29.3% for P2, and 58.8% for P3. On survival analysis (Summary Figure), higher UTD grade was significantly associated with the composite outcome (hazard ratio for P3 vs. P0 was 7.4 [95% CI: 3.44-15.92, p < 0.001]). Urinary tract infection and VUR diagnosis varied by UTD score (p = 0.03 and p < 0.001, respectively). Ureteropelvic junction obstruction was diagnosed (based on MAG3 results) in 6.3% of patients, 84% of whom were P3. Non-refluxing megaureter was diagnosed in 7.7%. Ureterocele and BOO were uncommon (1.4%, and 0.6%, respectively). Surgical intervention was also associated with UTD risk, with 46% of P3 undergoing surgery vs. 1% of P0, 1% of P1, and 6% of P2 (p < 0.001). Resolution of UTD occurred in 41% (median 10.1 months) and varied significantly by UTD risk (p < 0.001). DISCUSSION: Urinary tract dilation risk score is associated with clinical events, although ascertainment bias may influence some of the differences in outcomes, particularly for VUR, because VCUG utilization varied by the UTD group. The lack of any significant difference in outcomes between patients with UTD P0 versus P1 suggests that the P1 category could be eliminated as it does not meaningfully distinguish between outcome risk. CONCLUSIONS: Higher UTD risk scores are strongly associated with genitourinary diagnoses during the first two years of life.


Subject(s)
Dilatation, Pathologic/epidemiology , Hydronephrosis/diagnostic imaging , Prenatal Diagnosis , Ultrasonography, Doppler , Urologic Diseases/epidemiology , Age Factors , Cohort Studies , Dilatation, Pathologic/diagnostic imaging , Female , Follow-Up Studies , Humans , Hydronephrosis/pathology , Incidence , Infant, Newborn , Male , Postnatal Care , Pregnancy , Proportional Hazards Models , Retrospective Studies , Risk Assessment , Sex Factors , Urologic Diseases/diagnostic imaging , Urologic Diseases/physiopathology
SELECTION OF CITATIONS
SEARCH DETAIL
...