Anévrismes infectieux de l’aorte thoracique : présentation de 7 cas et revue de la littérature

Purpose

Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms.

Patients and methods

Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series.

Results

Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N = 1), Salmonella enteritidis (N = 3) and Candida albicans (N = 1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N = 5), that increased rapidly in size (N = 5), or presented an inflammatory aspect of the aortic wall (N = 4). Management was both medical and interventional: surgery (N = 3) or endoluminal repair (N = 4). Outcome was favourable in six patients; one patient died from aneurysm-related complications.

Conclusion

Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

Intérêt de l’imagerie par résonance magnétique nucléaire au cours de l’atteinte cardiaque du syndrome de Churg-Strauss. Trois observations et revue de la littérature

Purpose

The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15–92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods

We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results

Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n = 1) and retrosternal pain (n = 2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000–11 000/mm³); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n = 3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n = 2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n = 3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n = 2); one patient also underwent plasma exchanges.

Conclusion

Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.     

Caractéristiques cliniques,paracliniques et profil évolutif de l’atteinte aortique de la maladie de Horton : à propos de 26 cas d’aortite parmi 63 cas de maladie de Horton

Purpose

Aortic involvement that occurs in temporal arteritis is probably underestimated because it is usually asymptomatic. The characteristics of giant cell arteritis with aortic involvement are still poorly described and the relationship between aortitis and vascular outcome of the disease has not been clearly delineated. The objective of this retrospective study of 63 patients with giant cell arteritis, including 26 with aortic involvement, was to compare the features of patients with and without aortitis, and to assess the contribution of CT-scan and FDG-PET-scan in screening for vascular disease, monitoring, and therapeutic management of patients.

Methods

This retrospective study was conducted in the internal medicine department of the university hospital in Marseille, France, from January 1, 2005 to September 30, 2011. Patients had at least three out of the five American College of Rheumatology criteria for temporal arteritis and aortic involvement was investigated in all patients using CT-scan. Aortic wall thickness greater or equal to 3 mm was considered to be abnormal.

Results

Of 63 patients diagnosed with giant cell arteritis, 26 (41.3%) had aortic involvement diagnosed by aortic CT-scan. Age at diagnosis was significantly younger (66.8 vs 73.8 years; P = 0.002) in the group with aortitis. Inflammatory dorsal and low back pain, signs of vascular disease of the upper limbs (P = 0.009), and higher level of acute phase reactants were associated with aortitis. Aneurysmal lesions of the aorta were significantly more frequent in the group with aortitis. Twenty patients had both aortic CT-scan and FDG-PET-scan. For patients in whom aortic involvement was not demonstrated with CT-scan, FDG-PET-scan was always non-contributive. With corticosteroids, aortitis resolved within 6 months in all patients as evaluated by aortic CT-scan. However, aortitis persisted in 80% of cases at 6 months when evaluated with FDG-PET-scan, and in 66% of cases at 12 months, without influencing the treatment.

Conclusion

This case series shows no specific features of aorta and its main roots involvement in giant cell arteritis, justifying a systematic screening by CT-scan. The high frequency of this arterial involvement could help physicians in the diagnosis of giant cell arteritis. Aortitis seems to be associated with vascular complications as highlighted by the frequency of aortic aneurysm and a case of early aortic dissection. Finally, the role of PET-CT-scan for screening vascular disease and therapeutic monitoring remains to be clarified.     

Amylose cardiaque : à propos d’une série de 14 patients,description et facteurs pronostiques

Purpose

Cardiac amyloidosis is rare. The objective of this study was to report on a case series of 14 patients with cardiac amyloidosis and to study the prognostic factors.

Methods

Monocentric retrospective study of all adult patients who presented with cardiac amyloidosis, diagnosed at the Georges-Pompidou European hospital in Paris between 2003 and 2011.

Results

Fourteen patients were identified (10 men and four women). Median age at diagnosis was 66.5 years. Twelve patients were diagnosed with AL amyloidosis, one with AA amyloidosis, and one with transthyretin amyloidosis. All patients presented cardiac manifestations: heart failure (n = 9), rhythm disorders (n = 6). Eight patients presented extra-cardiac manifestations of amyloidosis: renal (n = 8), gastrointestinal (n = 5). Troponin serum level was increased in eight patients and BNP level was superior to 400 pg/L in 12 patients. When performed, the cardiac magnetic resonance imaging (MRI) showed, in six patients out of seven, chamber dilatation, concentric hypertrophy or late enhancement. Among patients with cardiac failure at diagnosis (n = 9), seven died with a median survival of 1 month duration. Factors of poor prognosis were, in our study, heart failure, elevated levels of troponin and BNP, and the AL amyloidosis subtype.

Conclusion

Cardiac amyloidosis, especially the AL type, has a very poor prognosis, essentially because of an underlying multiple myeloma and heart failure.     

Sclérites,aspects cliniques, étiologiques et thérapeutiques : à propos d’une série de 32 observations

Purpose

To report on the various clinical presentations, etiological diagnosis, prognosis and treatment of patients with scleritis evaluated at a tertiary care eye center.

Methods

Retrospective, monocentric study on a series of 32 patients in a tertiary center.

Results

The mean age of included patients with scleritis was 46.8 years (range, 22 to 77 years). Nineteen patients were women and 13 were men. Twenty-six patients (81%) had anterior scleritis (15 nodular, 8 diffuse and 3 necrotizing), six (19%) had posterior scleritis. Unilateral inflammation was present in 24 patients (75%). Twelve out of the 32 patients (37.5%) had an underlying systemic disease: granulomatosis with polyangiitis (n = 3), Behçet's disease (n = 2), unspecified inflammatory arthritis (n = 2), psoriatic arthritis (n = 1), ankylosing spondylitis (n = 1), sarcoidosis (n = 1), Cogan's syndrome (n = 1) and ulcerative colitis (n = 1). Six patients (18.8%) were suspected of having infectious disease with herpes virus: clinical context and positive treatment response with oral valacyclovir. Systemic agents and topical agents were required in 28 patients (87.5%). The first line therapy was mainly oral non-steroidal anti-inflammatory drugs in 15 patients (47%) and oral corticosteroids in 8 (25%). Immunosuppressive drugs were required in 6 patients. The mean follow-up was 16.3 months. Six patients (19%) had a decrease in visual acuity.

Conclusion

The number of systemic disease in our series is similar to the main series in the literature. Treatment with valaciclovir might be effective in patients with suspected herpes simplex scleritis.     

Particularités cliniques de l’embolie pulmonaire chez la personne âgée : étude comparative de 64 patients

Purpose

To date only a few studies regarding pulmonary embolism (PE) in elderly have been published. The aim of this study was to determine the clinical features of PE in elderly patients (≥ 75 years).

Methods

All patients hospitalized for PE in our internal medicine department from January 2005 to December 2010 were included in the study. The aim was to compare the features of PE in elderly patients (≥ 75 years) to those of patients younger than 75 years. The following data were recorded: past medical history, risk factors for venous thrombo-embolism (VTE), clinical features, and PE etiologies.

Results

The population was composed of 64 patients (women 56%) with a median age of 82 years (IQR: 13.5). There was no statistical difference for risk factors of VTE. Syncope was more frequent in elderly patients (33% versus 7%, P = 0.04) whereas thoracic pain predominated in younger patients (36,5% versus 7%, P = 0.005). Chronic obstructive pulmonary disease was more frequent in the past medical history of elderly patients. The diagnostic of PE was less suspected in elderly patients (47% versus 72%, P = 0.035). The etiologies were similar between the two groups.

Conclusion

Our study highlights the frequency of syncope as the presenting feature of PE in elderly, whereas thoracic pain is uncommon. We confirmed the difficulty to diagnose PE in elderly population.     

Validation de la reproductibilité de la mesure de la surface de l’orifice régurgitant par planimétrie dans l’insuffisance aortique : une étude préliminaire d’échocardiographie trans-œsophagienne tridimensionnelle

Background

Aortic regurgitation is mainly evaluated by trans-thoracic echocardiography using multi-parametric qualitative and semi quantitative tools. All those parameters can fail to meet expectations, resulting in an imperfect diagnostic reliability and assessment of aortic regurgitation severity can be challenging.

Objectives

We sought to evaluate feasibility and intra- and inter-observer reproducibility of aortic regurgitant orifice area measured by planimetry with tridimensional trans-esophageal echocardiography on patients with at least grade 2/4 aortic regurgitation.

Patients and methods

Consecutive patients with at least grade 2/4 aortic regurgitation measured by trans-thoracic echocardiography and referred for trans-esophageal echocardiography for any reason were included. Planimetric reconstructions of regurgitant orifice area were studied and reproducibility indexes between senior and junior observers were calculated.

Results

Twenty-three patients were included in this study. Intra- and inter-observer reproducibility were excellent with an ICC of 0.95 [0.88–0.98], P < 0.0001 and 0.91 [0.79–0.96], P < 0.0001, respectively. Mean length of the measurement was 6.6 ± 0.9 min [CI95% 6.23–7.01].

Conclusion

Planimetric measurement of the aortic regurgitant orifice using tridimensional trans-esophageal echocardiography seems to be feasible and has great intra- and inter-observer reproducibility. Reconstruction durations were compatible with a daily use. There is a need now to investigate the reliability of this measurement as compared with the reference technique.     

Prévalence et valeur diagnostique des anticorps antinucléaires de spécificité antigénique indéterminée : étude rétrospective à propos d’une série de 90 patients

Purpose

The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique.

Patients and methods

Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis.

Results

Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n = 6), scleroderma (n = 5), Sjögren's syndrome (n = 3), SLE (n = 4), rheumatoid arthritis (n = 6) and antiphospholipid syndrome (n = 4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation.

Conclusion

Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as “gold standard” test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.     

Myopathies inflammatoires et anticorps anti-PM-Scl : à propos d’une série et revue de la littérature

Purpose

The objectives of this study were to evaluate: (1) the prevalence of anti-PM-Scl antibodies within the framework of antinuclear antibodies detection; and (2) the clinical features and outcome of patients with isolated polymyositis/dermatomyositis.

Methods

Nine thousand and sixty-four consecutive antinuclear testing data allowed us to evaluate anti-PM-Scl antibody prevalence. Second, we also assessed the characteristics of patients with isolated dermatomyositis/polymyositis and associated anti-PM-Scl antibody.

Results

Over 9064 consecutive antinuclear samples tested for antinuclear antibodies, 3263 (36%) were positive; anti-PM-Scl antibody were positive in nine patients: 0.1% of all sera, 0.2% of sera positive for antinuclear antibodies, 1.2% of sera positive for anti-ENA antibodies. Four of the nine patients with anti-PM-Scl antibody had dermatomyositis (n = 3) and polymyositis (n = 1). Patients with dermatomyositis/polymyositis and anti-PM-Scl antibody exhibited severe complications, as follows: ventilatory insufficiency (n = 2) requiring mechanical ventilation in one case, esophageal involvement requiring enteral feeding (n = 1); also, two of these patients had cancer.

Conclusion

Our case series suggests that the presence of anti-PM-Scl antibody is not a favorable prognostic factor in patients with dermatomyositis/polymyositis. This type of antibody appears to be associated with lung and esophageal involvement; in addition, anti-PM-Scl antibody may co-exist with malignancy in PM/DM patients. Taken together, we suggest that patients with dermatomyositis/polymyositis and anti-PM-Scl antibody require both initial evaluation for lung/digestive manifestations and cancer and close surveillance.     

Uvéites révélant une sarcoïdose : caractéristiques cliniques à propos de 23 cas

Purpose

Uveitis may rarely reveal sarcoidosis in Caucasian patients. Our objective was to analyze the clinical manifestations, and the outcome in a group of patients in whom uveitis was the presenting manifestation of sarcoidosis.

Methods

Retrospective study including 23 patients (mean age: 50.3 ± 14.5 years) diagnosed with sarcoidosis after an episode of uveitis. Granulomatous lesions were documented in 14 patients.

Results

Ophthalmological examination revealed anterior uveitis (n = 5), intermediate uveitis (n = 2), posterior uveitis (n = 25) and panuveitis (n = 11). Ocular inflammation was bilateral in 16 patients (69,6%), typical aspects of granulomatous uveitis were found in only 16 eyes over 39 (41%), posterior uveitis was found in 18 eyes (46.2%), with an averaged visual acuity of 5/10. Macular oedema was noted in five patients. Suggestive signs of ocular sarcoidosis were present in 43% of the patients. Stage 1 or 2 pulmonary involvement (n = 22), musculoskeletal (22%), skin (13%), or spleen (9%) involvements were the most common findings. Oral corticosteroids were necessary in 91.3% of the patients, immunosuppressive agents in 26.1%, with a prolonged treatment greater than two years in 58%. The visual prognosis was good, with visual acuity greater than 6/10 in 96% of the cases if the ocular inflammation spared retina and choroid. However, a visual acuity less than 6/10 was observed in 44% of the cases when the posterior segment was involved.

Conclusion

Sarcoidosis may be revealed by an intraocular inflammation, with typical patterns in only 43% of the cases. Sarcoidosis should therefore be included in the differential diagnosis of every uveitis. Oral corticosteroids are required in almost all cases, owing to ocular involvement rather than visceral involvement.     

Intérêt de l’échocardiographie trans-œsophagienne dans l’optimisation de la stratégie thérapeutique après un accident vasculaire cérébral ischémique

Aim of the study

In the setting of ischemic stroke, the place of transesophageal echocardiography (TEE) is still matter of debate. The aim of the study is to evaluate the therapeutic impact provided by TEE and to characterize patients in whom TEE is warranted.

Patients and method

Three hundred and fifty-nine consecutive patients were included in the study. “Decisive TEE” (DTEE) was defined by echographic findings resulting in a change of treatment, whereas “informative TEE” (ITEE) was defined by TEE revealing a potential cardiac or aortic source of embolism.

Results

Three hundred and forty-one patients underwent TEE. Twenty-eight patients (8.2%) had DTEE and 184 (53.9%) had ITEE. DTEE were as follows: thrombus in the left atrial appendage in 6 patients, complex aortic plaques in 10 patients, patent foramen ovale (PFO) associated with atrial septal aneurism (ASA) and an important right to left shunt (3 patients), FOP associated with ASA and lower limb phlebitis (1 patient), 4 cases of endocarditis and 4 patients with intense spontaneous echo contrast in the left atrium. In most cases of DTEE (67.8%), the patient was given anticoagulation drugs. Left atrial dilatation (P = 0.005) and multivessel territory stroke (P = 0.018) were statistically predictive of DTEE.

Conclusions

In the setting of ischemic stroke, TEE provides important additional informations, but modifies therapeutic strategy in less than 10% of cases. Multivessel territory stroke, and left atrial dilatation were predictive of DTEE.     

Use of covered Cheatham-Platinum stents in congenital heart disease

Background

Controversy remains regarding the use of covered stents in congenital heart disease (CHD). We evaluate the possibilities and safety of covered Cheatham-Platinum (CCP) stents in CHD.

Methods

Single-center retrospective CHD-database study of all CCP stents, 2003–2012. Three study groups: aortic coarctation (CoA), right ventricular outflow tract pre-stenting for percutaneous revalvulation (RVOT), and miscellaneous. Continuous data expressed as median (range).

Results

114 CCP stents in 105 patients, age 16.8 years (4.2–71.2).CoA group: 54 CCP stents in 51 patients: 3/54 for aneurysm exclusion, in 51/54 covering used “prophylactically” because of increased risk for vessel tear. Overall, CCP stenting increased the coarctation diameter from 6 mm (0–15) to 15 mm (10–20) (p < 0.001).RVOT group: 39 CCP stents in 37 patients (34 with RVOT graft, 3 with transannular patch): the graft lumen had shrunken from nominal 21 mm (10–26) to 13 mm (5–22); with the CCP stent the RVOT was redilated to 22 mm (16–26, p < 0.001 vs stenosis).Miscellaneous group: 21 CCP stents in 17 patients: closure of Fontan-circuit fenestration (n = 5), restoration of superior caval vein (n = 2) or pulmonary artery (n = 3) patency, relief of supra-pulmonary stenosis (n = 2), exclusion of aberrant pulmonary arteries (n = 1), cavopulmonary conduit expansion (n = 2), Blalock–Taussig shunt flow reduction (n = 1), and defibrillator lead protection from sharp stents (n = 1). Hybrid procedures performed in 3/17 patients. CCP stent was used as rescue treatment in 2/patients to seal iatrogenic bleeding.

Conclusion

CCP stents can safely be applied in CHD patients. The covering allows adequate sealing of existing or expected tears, thereby increasing the safety margin with more complete dilation.     

Granulomatoses rénales : étude rétrospective de 44 observations

Purpose

Granulomatous interstitial nephritis (GIN) are identified in 0.5 to 1,3% of all renal biopsies. Renal outcome and treatment modalities are not clearly established in the literature.

Methods

We retrospectively analyzed a case series of 44 GIN identified among all renal biopsies performed between 1984 and 2005 in the Rhône-Alpes area.

Results

The study population included 25 men and 19 women with a mean age of 56 years, and mean diagnostic delay was 11 months. Renal function was severely impaired (mean creatinine clearance 24 mL/min). Proteinuria was observed in 77% (mean value 0,9 g/24 h) of the patients and associated with microscopic hematuria and leukocyturia in 30% and 25%, respectively. The most common diagnosis was sarcoidosis (25%, n = 11), followed by drug-induced GIN (9%, n = 4), tuberculosis (6,8%, n=3), hemopathy-related paraneoplastic GIN (6,8%, n = 3), HIV infection (n = 1) and chronic renal allograft rejection (n = 1). In other patients, no aetiology was found (48%, n = 21). Severity of renal failure justified hemodialysis in 34% (n = 15) of the patients. Three patients underwent renal transplantation. Nonetheless, renal outcome was generally favorable: renal function improved in 41% (n = 18) and stabilized in 34% (n = 15) of patients.

Conclusions

Sarcoidosis, drug-induced and infections represent the main causes of GIN. Histologic features are not specific enough to determine the aetiology. Corticosteroids is the gold standard in sarcoidosis, drug-induced, and idiopathic GIN. Treatment is etiologic in the other cases.     

Aumento de la expresión de las moléculas CD11c y CD103 en neutrófilos de sangre periférica tratados con una formulación de ribosomas bacterianos y proteoglicanos de Klebsiella pneumoniae

Aim

To evaluate the effect of a preparation with bacterial ribosomes and proteoglycans from Klebsiella pneumoniae «R» on the in vitro expression of CD11c and CD103 molecules in neutrophils from peripheral blood.

Methods

Isolation of neutrophils from peripheral blood with Ficoll-Paque, incubation with R and detection of CD11c and CD103 through flow cytometry.

Results

Six hours after the incubation period, CD11c expression increased significantly compared with the control with 125 and 500 μg/ml of R (P = .017 and P = .006, respectively). CD103 expression induced with 125 μg/ml of R after 6 hours was significantly higher than that observed after 4 hours at the same concentration (P = .014) and that found with 62.5 μg/ml (P = .017) of R.

Conclusions

The increased expression of CD11c and CD103 induced by R in the neutrophils could contribute to the R mechanism against respiratory pathogens.     

High homocysteine and low folate concentrations in acute aortic dissection

Background

Biomarkers for monitoring progression and prognosis of thoracic aneurysm are of great interest. Homocysteine (Hcy) induces elastolysis in arterial media and may directly affect fibrillin-1 or collagen whereas lipoprotein (Lp) (a) inhibits elastolysis by reducing activation of matrix metallopeptidase-9.

Methods

We studied 31 consecutive patients with acute aortic dissection (AAD) admitted for emergency surgery (group I, 60 ± 13 years old, 25 men), 30 consecutive patients with chronic aneurysms of the ascending aorta (group II, 67 ± 12 years old, 24 men) and 20 healthy controls (group III, 58 ± 15 years old, 14 men). We evaluated Hcy, folate, B12, Lp(a) and methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism at baseline.

Results

Hcy, folate and B12 differed significantly among the 3 studied groups (P = 0.016, P = 0.004 and P = 0.001, respectively). The levels of Hcy and B12 were significantly higher in group I compared to both groups II and III (P = 0.05 and P = 0.002, P < 0.001 and P = 0.017, respectively) and without significant differences between groups II and III (P = 0.083 and P = 0.124). Folate was significantly lower in group I compared to both groups II and III (P = 0.001 and P = 0.006, respectively) and without marked difference between groups II and III (P = 0.409). No significant difference was found in serum levels of Lp (a) (P = 0.074) or among the frequency of MTHFR C677T genotypes.

Conclusions

Patients with AAD present with higher Hcy and lower folate compared to both chronic aneurysms and controls.     

Utilización de procalcitonina y proteína C reactiva como marcadores de infección en la neutropenia febril de pacientes sometidos a trasplante de progenitores hematopoyéticos

Introduction and objective

Neutropenia is a frequent sign in patients who are going to have a haematopoietic stem cell transplant (HSCT). Infection is an important complication in these patients, which is favoured by immunosuppression and the degree of neutropenia. This study aims to evaluate the diagnostic usefulness of procalcitonin (PCT) and C-reactive protein (CRP) in onco-haematological patients undergoing chemotherapy and HSCT to determine the origin of the fever.

Patients and methods

PCT and CRP values were measured in 30 episodes of febrile neutropenia: before starting chemotherapy, appearance of neutropenia, onset of fever, days 1, 2, 3 and 6 after the onset of fever, and when the febrile episode ended. The episodes were classified as 5 bacteraemia, 3 microbiologically documented infections, 10 clinical infections, and 12 fevers of unknown origin.

Results

The highest PCT mean values corresponded to the group of patients with bacteraemia. Statistically significant differences (P = .04) were found on the second day after the onset of fever. The cut-off point of 0.5 ng/ml showed a sensitivity of 66% and a specificity of 75%. PCR results showed statistically significant differences on days 1, 2 and 3 after the onset of fever (P = .01, P = .003, and P = .002, respectively). The cut-off point of 7.5 mg/L had a sensitivity of 88% and a specificity of 58%.

Conclusions

The combination of PCT and CRP is an insufficient method to detect bacterial infections and may not replace the proper clinical and microbiological diagnosis.     

Aortic coarctation and the retinal microvasculature

Background

Aortic coarctation has been associated with generalized vascular disease, yet little is known about retinal vascular patterns and their changes over time.

Objectives

The aim of this study is to characterize the nature and extent of retinal vascular disease in adults with aortic coarctation, and explore age-related effects and associations with cardiovascular outcomes.

Methods

A prospective cross-sectional seroepidemiological study was conducted on 60 consecutive adults with repaired aortic coarctation, age 42.4 ± 14.1 years, 61.7% male. In addition to detailed questionnaires, imaging studies, and laboratory testing, high-quality retinal images were acquired by 45° nonmydriatic digital funduscopy.

Results

No patient had evidence of hypertensive retinopathy. A distinctive vascular pattern characterized by bilaterally symmetric tortuosity of retinal arteries and veins was observed. Arterial tortuosity was abnormal in 98.3% of patients and decreased with age (P = 0.0005). In patients ≥ 45 years, a 1-point increase in the arterial tortuosity score was associated with a 1.5-fold higher risk of cardiovascular complications (i.e., acute coronary syndrome, stroke, cerebral aneurysm, aortic dissection/rupture) [odds ratio 1.50, 95% CI (1.01, 2.24), P = 0.0496]. Abnormal venous tortuosity was present in 75.0% of patients and non-significantly correlated with higher levels of serum inflammatory markers (C-reactive protein, fibrinogen, interleukin-6, and tumor necrosis factor-alpha). A higher venous tortuosity score was likewise associated with an increased risk of cardiovascular complications [odds ratio 1.86, 95% CI (1.03, 3.35), P = 0.0392].

Conclusions

Adults with repaired aortic coarctation exhibit a unique retinal vascular pattern characterized by excessive arterial and venous tortuosity that regresses with age. Greater tortuosity is associated with adverse cardiovascular outcomes in patients ≥ 45 years.