BackgroundErdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with characteristic radiological and histological features. This entity is defined by a mononuclear infiltrate consisting of lipid-laden, foamy histiocytes that stain positively for CD68 and negatively for CD1a. Osseous involvement is constant and characteristic. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits.MethodsBoth radiography and technetium-99m bone scintigraphy may reveal osteosclerosis of the long bones, which is a typical finding in ECD. For visceral involvement, computed tomography (CT) is most useful, while magnetic resonance (MR) imaging is more sensitive for cardiovascular lesions; 2-[fluorine-18] fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET)/CT scanning is useful in assessing the extension of ECD lesions.ResultsThe prognosis is extremely variable and is often worse when there is cardiovascular system involvement. Diagnosis is based on the combination of radiographic, CT, MR imaging and nuclear medicine features and a nearly pathognomonic immunohistochemical profile.ConclusionThe aims of this work are to perform a systematic review of Erdheim-Chester disease as seen on imaging of the chest, abdomen and musculoskeletal system and to discuss the diagnostic workup and differential diagnoses according to the imaging presentation.Teaching points• Bone involvement is usually present in patients, and the imaging findings are pathognomonic of ECD.• The circumferential periaortic infiltration may extend to its branches, sometimes becoming symptomatic.• Cardiac involvement—the pericardium, right atrium and auriculoventricular sulcus—worsens its prognosis.• Perirenal infiltration extending to the proximal ureter is highly suggestive of this disease.
Gastrointestinal (GI) bleeding is a common clinical condition that is increasingly seen in an aging population and frequently requires hospitalization and intervention, with significant morbidity and mortality. Obscure GI bleeding (OGIB) is defined as loss of blood with no source identified after upper endoscopy and colonoscopy. Whether an obscure site of bleeding is clinically evident or silent, it constitutes a diagnostic and therapeutic challenge for the clinician. Gastroenterology and radiology provide the essential diagnostic tools used to evaluate suspected OGIB, each with its strengths and weaknesses. Small bowel series and conventional enteroclysis have a limited role in OGIB. Computed tomographic (CT) enterography and CT enteroclysis are noninvasive techniques with promising results in evaluation of small bowel disease and silent OGIB. CT angiography is a useful triaging tool for diagnosing or excluding active GI hemorrhage, localizing the site of bleeding, and guiding subsequent treatment. Tagged red blood cell scanning is the most sensitive technique for detection of active GI bleeding and allows imaging over a prolonged period, making it useful for detecting intermittent bleeding. Capsule endoscopy has emerged as an important tool for investigating OGIB, but it may soon have competition from double-balloon enteroscopy, a diagnostic technique that can also facilitate therapy.
Objectives The left atrium (LA) modulates left ventricular filling through reservoir, conduit and booster pump functions. Only limited data exist on LA involvement in type 2 diabetes mellitus (DM2). This study sought to assess LA function in asymptomatic DM2 with cardiac MRI. We hypothesized that cardiac MRI can detect LA dysfunction in asymptomatic DM2. Methods Forty-five patients with asymptomatic DM2 and 24 normoglycaemic controls were studied. MRI cine imaging was performed to measure LA maximal and minimal volumes. A flow-sensitive phase-contrast gradient-echo sequence was used for flow measurements perpendicular to the orifice of the mitral valve, to quantify active LA stroke volume. LA total, passive and active emptying volumes and fractions were calculated. Results LA reservoir function, namely LA total ejection fraction, was significantly greater in controls compared to patients with DM2 (62.2±5.2 vs 57.0±7.6 %, P=0.004). LA passive ejection fraction was also greater in the controls (26.2±9.5 vs 16.1±11.0 %, P<0.001). Regarding parameters of LA booster pump function, LA active ejection fraction was not significantly different between groups. DM2 was demonstrated to be an independent determinant of LA function.
C a r d io p u l m o n a r y I m ag i ng • O r ig i n a l R e s e a rc hThis article is available for credit. This research was financially supported by Project "DoIT," which is cofinanced by the European Community Fund FEDER through COMPETE. rosis [10], which can be detected noninvasively by MDCT.Coronary artery disease is commonly cited as a mechanism underlying diastolic dysfunction [11]. Many of the same factors that contribute to atherosclerosis may also produce LV diastolic dysfunction by either direct mechanisms (e.g., hypertension and age-related vascular stiffening) or secondarily via coronary artery disease progression and resulting changes in myocardial compliance [11].There are limited and controversial published data on the relationship of CAC to LV diastolic function. Edvardsen et al. [12] reported that coronary atherosclerosis is associated with depressed regional LV systolic and diastolic wall strain measured by MRI tagging. Colletti et al. [13] found that a CAC score greater than 100 predicts an increased likelihood of clinically unsuspected LV regional wall motion abnormalities, which are C ardiovascular disease is the most frequent cause of death and disability in patients with type 2 diabetes mellitus (DM2). DM2 is responsible for diverse cardiovascular complications such as increased coronary atherosclerosis and left ventricular (LV) dysfunction. LV diastolic dysfunction is highly prevalent in DM2 [1,2], representing the earliest preclinical manifestation of LV dysfunction in patients with this condition [3][4][5][6].Over the past decade, cardiac MRI has been widely accepted as the reference standard for the assessment of cardiac structure and function because of its high spatial and temporal resolution, excellent image quality, and lack of geometric assumptions [7]. Cardiac MRI offers a variety of alternative approaches for evaluating diastolic function [8,9].The presence of coronary artery calcium (CAC) is indicative of coronary atheroscle-OBJECTIVE. The purpose of this study was to compare cardiac MRI-derived parameters of left ventricular (LV) diastolic function between uncomplicated type 2 diabetes mellitus (DM2) and normoglycemic control subjects and to evaluate whether these parameters of LV diastolic function are related to coronary atherosclerosis. SUBJECTS AND METHODS.We prospectively studied 41 subjects with DM2 and 21 normoglycemic control subjects (30 women and 32 men; mean age, 57.2 ± 7.1 [SD] years) with no evidence of overt cardiovascular disease. We used cardiac MRI to measure LV volumes, LV peak filling rate (PFR), and transmitral flow and CT to determine coronary artery calcium scores.RESULTS. Absolute values of the peak filling rate (PFR) were significantly lower in DM2 patients than in control subjects (mean ± SD, 293.2 ± 51.7 vs 375.7 ± 102.8 mL/s, respectively; p < 0.001). Mitral peak E velocities (mean ± SD, 42.8 ± 10.7 vs 48.8 ± 10.4 cm/s; p = 0.040) and peak E velocity-to-peak A velocity ratios (0.88 ± 0.3 vs 1.1 ± 0.3; p = 0.002) were also lower in DM2 patients com...
Objectives Asymptomatic left ventricular (LV) diastolic dysfunction is increasingly recognised as an important diagnosis. Our goal was to study the prevalence and gender differences in subclinical LV diastolic dysfunction, using cardiovascular magnetic resonance imaging (CMR) at 3 T. Methods We prospectively studied 48 volunteers (19 male and 29 female, mean age 49±7 years) with no evidence of cardiovascular disease. We used CMR to measure left atrium (LA) and LV volumes, LV peak filling rate and transmitral flow.Results The overall prevalence of LV diastolic dysfunction in our cohort varied between 20 % (based on evaluation of LV filing profiles) and 24 % (based on the evaluation of the transmitral flow). The prevalence of diastolic dysfunction was higher in men than in women, independently of the criteria used (P between 0.004 and 0.022). Indexed LV end-diastolic volume, indexed LV stroke volume, indexed LV mass, indexed LA minimum volume and indexed LA maximum volume were significantly greater in men than in women (P<0.05). All the subjects had LV ejection fractions within the normal range. Conclusions It is clinically feasible to study diastolic flow and LV filling with CMR. CMR detected diastolic dysfunction in asymptomatic men and women. Key Points • CMR imaging offers new possibilities in assessing left ventricular diastolic function.• The prevalence of diastolic dysfunction is higher in men than in women.• The prevalence of some diastolic dysfunction in a normal population is 24 %.
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