Elderly-onset primary Sjögren's syndrome focused on clinical and salivary gland ultrasonographic features

ObjectiveTo assess the clinical, laboratory, and salivary gland ultrasound (SGUS) characteristics of elderly-onset of primary Sjögren's syndrome (EopSS).MethodsWe included pSS patient from two referral hospitals over a 4-year period. The SGUS scores (0–48) and SG volumes were assessed. Clinical, laboratory, and SGUS data were compared according to age at onset: EopSS (≥ 65 years), adult-onset (AopSS) (≥ 40 and < 65 years), and young-onset (YopSS) (< 40 years).ResultsThis cross-sectional study included a total of 221 patients, 43 (19.5%) of which had EopSS. Subjective sicca symptoms, results of the Schirmer's test, and unstimulated salivary flow rate revealed no significant differences between the groups. EopSS patients presented a significantly higher frequency of interstitial lung disease (ILD) (EopSS: 51.2% vs. AopSS: 13.5% vs. YopSS: 8.7%, P < 0.001) and lower frequency of arthritis (7% vs. 22.6% vs. 39.1%, P < 0.01). They also had significantly lower positivity of anti-Ro/SSA (51.2%) and anti-La/SSB (7.0%) and lower levels of rheumatoid factor, C4, and IgG. The EopSS group had significantly lower SGUS positivity (defined as total scores ≥ 14) (44.2% vs. 64.5% vs. 78.3%, P < 0.05), lower SGUS scores, and smaller submandibular gland volume.ConclusionWe report a specific phenotype of EopSS, characterised by high prevalence of ILD, less involvement of the peripheral joint, and low biological activity. SGUS evaluation showed less parenchymal abnormalities but more atrophic changes in major SGs in EopSS patients. Considering the low positivity of anti-Ro/SSA and SGUS in EopSS, SG biopsy remains the only way to confirm the diagnosis of pSS, especially in elderly patients.     

Squamous cell carcinoma antigen-IgM (SCCA-IgM) is associated with interstitial lung disease in systemic sclerosis

ObjectiveInterstitial lung disease (ILD) is the major determinant of prognosis in patients with systemic sclerosis (SSc). Squamous Cell Carcinoma Antigen (SCCA1) is a serin protease inhibitor which plays a pivotal role in inflammation and fibrosis. SCCA1 is overexpressed in pulmonary tissue of patients with idiopathic pulmonary fibrosis and can be detectable in serum as circulating immune complex bound to IgM (SCCA-IgM). We aimed to investigate the association between SCCA-IgM and clinical features of patients with SSc.MethodsNinety-seven patients with SSc (ACR/EULAR criteria) were consecutively enrolled in the study. Clinical and serological variables and organ involvement were recorded. Pulmonary involvement was investigated by high-resolution CT (HRCT) and respiratory function tests. SCCA-IgM serum levels were measured by a validated ELISA assay (Hepa-IC, Xeptagen, Venice, Italy). We set the cut-off value for serum levels of SCCA-IgM > 200 AU/ml, calculated as mean + 3 standard deviations in 100 healthy subjects.ResultsForty-one (42.3%) patients were affected with ILD. SCCA-IgM values were significantly higher in patients with ILD than in those without: 218 (80-402) vs. 87.5 (59-150) AU/mL, P = 0.003. Patients with positive SCCA-IgM had more frequently ILD (69.7% vs. 28.1%, P ≤ 0.0001) and a lower total lung capacity (TLC) (P = 0.024) compared with negative ones. No differences were found in any other clinical and serological features. At multivariate analysis, SCCA-IgM was found to be associated with ILD diagnosis (OR 10.6, IC 2.9-38.4, P = 0.001).ConclusionSCCA-IgM is associated with interstitial lung disease in scleroderma patients and might be used in the assessment of SSc-ILD.     

Is there a link between inflammation and abnormal lipoprotein profile in Sjögren's syndrome?

ObjectivesTo investigate the lipoprotein profile of patients with primary Sjögren's syndrome (pSS) and its association with laboratory tests, including markers of inflammation.MethodsThis is a cross-sectional study among patients with pSS and healthy controls. We analyzed the lipoprotein profile of 73 pSS patients compared to 65 healthy individuals in the control group. We further evaluated possible associations between dyslipidemia in pSS patients and laboratory parameters including: hypergammaglobulinemia, autoantibodies [antinuclear antibodies (ANA), rheumatoid factor (RF), anti-Ro, anti-La], and acute-phase reactants [C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)].ResultsPatients and controls were comparable regarding the demographic variables. Lipoprotein profile was similar between pSS patients and controls: total cholesterol (204.0 ± 43.39 versus 206.5 ± 42.76 mg/mL, P = 0.73), LDL fraction (131.6 ± 37.38 versus 130.62 ± 38.24 mg/dL, P = 0.88) and HDL fraction (49.7 ± 13.5 versus 51 ± 11.5 mg/dL, P = 0.56), triglycerides (129.3 ± 81.0 versus 116.8 ± 53.5 mg/dL, P = 0.29). However, patients with pSS had a strong trend to present dyslipidemia when compared to healthy individuals (76.7% versus 61.5%, P = 0.06). The presence of dyslipidemia in pSS was associated with increased ESR (44.05 ± 28.07 versus 28.28 ± 18.00, P = 0.03), but not with other laboratory markers of the disease and inflammation.Discussion/ConclusionpSS patients frequently present abnormal lipid profile, which are associated with higher levels of ESR. Thus, similar to other systemic inflammatory diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), lipid profile should be evaluated in pSS patients, with the aim of initiating specific therapeutic strategy for prevention of cardiovascular events.     

Entheses ultrasound assessment in primary Sjogren's syndrome

ObjectiveMusculoskeletal pain is a common complaint among patients with primary Sjogren's syndrome (pSS). Joints clinical examination is oftenly normal. A periarticular origin of this pain may be possible. Since clinical examination lacks sensitivity and precision, the use of musculoskeletal ultrasound (US) is more interesting in the evaluation of the entheses involvement, as it is shown to be a more sensitive tool. Our objective was to assess, by an ultrasonographic study, the entheses involvement in the widespread pain of patients with pSS.MethodsThis is a prospective study including 25 women with pSS and 25 age and sex matched healthy controls. An ultrasound examination, using grey scale and Doppler US, of five enthesitic sites (distal quadricipital, proximal patellar, distal patellar, distal Achillian and distal brachial tricipital) sought bilaterally the following lesions: hypoechogenicity, thickening, loss of fibrillar structure, erosions, enthesophytes, calcifications or Doppler hypervascularisation. A final score was calculated by summing the abnormalities scores of all entheses.ResultsThe mean age was 53.2 ± 11.3 years in the pSS group and 50.6 ± 9.7 years in the control group. The mean number of pathological entheses on ultrasound was 3.92 ± 1.93 in the pSS group versus 4.52 ± 2.27 in the control group (P > 0.05). The total score for enthesitis abnormalities was 4.96 ± 2.59 versus 5.72 ± 2.92 (P > 0.05), respectively. There was a positive correlation between total score of ultrasound enthesitic abnormalities and age in both groups.ConclusionIn patients with pSS, clinically painful sites were more frequently found than in US. Musculoskeletal pain was not due to enthesitis.     

Time-course of ultrasound abnormalities of major salivary glands in suspected Sjögren's syndrome

Objective

To evaluate whether major salivary-gland ultrasonography (SGUS) abnormalities change over time in patients with suspected primary Sjögren's syndrome (pSS) during the natural course of the disease.

Associations between ocular and extra-ocular assessment in primary Sjögren's syndrome*

ObjectivesTo assess associations between ophthalmological features and the main systemic biomarkers of primary Sjögren's Syndrome (pSS), and to identify systemic biomarkers associated with severe keratoconjunctivitis sicca (KCS) in pSS patients.MethodsIn this cross-sectional study, data was retrospectively extracted from the monocentric cohort of the French reference centre for pSS. We analysed data from the initial visit of patients admitted for suspicion of pSS and included patients validating pSS ACR/EULAR classification criteria. Ophthalmological assessment included Schirmer's test, tear break-up time, ocular staining score (OSS), and visual analogue scale (DED-VAS) for dry eye disease (DED) symptoms. Results of minor salivary gland biopsy, unstimulated whole salivary flow rate, anti-SSA/Ro antibodies, anti-SSB/La antibodies, and rheumatoid factor (RF) were collected.ResultsA total of 253 patients (245 females) with confirmed pSS, aged 56.6 ± 13.0 years, were included, among which 37% had severe KCS. Multivariate analysis showed that the presence of anti-SSA/Ro antibodies, anti-SSB/La antibodies and RF were associated with conjunctival OSS (odds ratio–OR– = 1.25 per OSS unit increase; confidence interval–CI–95% = 1.05–1.49; P = 0.01; OR = 1.31 per OSS unit increase; CI95% = 1.09–1.58, P = 0.002, and OR = 1.34 per OSS unit increase; CI95% = 1.12–1.59; P = 0.001, respectively). Both anti-SSB/La antibodies and DED-VAS ≥ 5 were significantly associated with severe KCS (OR = 2.03; CI95% = 1.03–4.00; P < 0.05 and OR = 2.52, CI95% = 1.31–4.90; P < 0.01, respectively).ConclusionAssociation between conjunctival OSS and systemic biomarkers of pSS indicate the crucial importance of conjunctival staining when pSS is suspected as a cause of DED. Conversely, patients with anti-SSB and DED-VAS ≥ 5 features should be prioritized for extensive evaluation by an ophthalmologist due to their association with severe KCS.     

Incomplete thymic involution in systemic sclerosis and rheumatoid arthritis

ObjectiveThe thymus plays a crucial role in immune system homeostasis. Thymic abnormalities have been reported in many autoimmune diseases, but data for systemic sclerosis (SSc) and rheumatoid arthritis (RA) are sparse. The aim of this study was to evaluate the prevalence and correlates of radiological incomplete involution of the thymus in SSc and RA patients, and in a non-autoimmune group of controls.MethodsAll patients were at least 40 years old: 96 SSc patients (median age 59 years, 80% women) and 65 RA patients (median age 57 years, 88% women) were compared with 32 control individuals (median age 63 years, 62% women). Pulmonary CT-scans performed for lung assessment were available for all individuals. For the purpose of our study, complete involution of the thymus was defined as the absence of a residual thymus or a gland thickness, corresponding to the short axis on the axial slice, of less than 7 mm. We defined incomplete involution of the thymus as a residual thymic tissue more than 7 mm thick.ResultsThe frequency of incomplete thymus involution was significantly higher in SSc and RA patients (respectively 15 and 14%) than in the control group (0%; P < 0.05). Incomplete thymus involution was associated with pulmonary restrictive syndrome in SSc patients, and with biotherapy and an absence of antinuclear antibodies in RA patients.ConclusionOur findings show that two autoimmune diseases, SSc and RA, are associated with incomplete thymus involution.     

Evaluation of carotid intima media thickness in children with idiopathic nephrotic syndrome

AimAim of the study was to determine if carotid intima media thickness in children with idiopathic nephrotic syndrome is greater than in healthy subjects, and to assess whether carotid intima media thickness in children with nephrotic syndrome is associated with clinical (including disease duration, cumulative dose of steroids, number of relapses) and biochemical parameters.MethodsA cross-sectional study included 40 patients with nephrotic syndrome (mean age 11.7 ± 4.7 years). Steroid dependent nephrotic syndrome was established in 32 patients (80%), while 8 (20%) had steroid resistant nephrotic syndrome. Control group consisted of 20 age and gender matched healthy children. Blood pressure based on 24-h ambulatory blood pressure monitoring (ABPM), carotid intima media thickness, fasting glucose, insulin, HbA1c, lipid concentrations were measured in all children.ResultsA significant difference was detected in carotid intima media thickness values (P = 0.036). Children with nephrotic syndrome had significantly greater carotid intima media thickness compared with healthy children (0.42 ± 0.06 and 0.38 ± 0.03 mm). Carotid intima-media thickness was positively associated with duration of nephrotic syndrome (r = 0.45; P = 0.004), body mass index (r = 0.48; P = 0.002), daytime systolic blood pressure (r = 0.46; P = 0.003) and night-time systolic blood pressure (r = 0.52; P = 0.001). Multiple linear regression showed that duration of nephrotic syndrome was the only independent predictor of carotid intima media thickness in children with nephrotic syndrome (R² = 0.244; β=0.327; P = 0.037).ConclusionThe findings of the present study suggest subclinical vascular damage in patients with nephrotic syndrome. Duration of nephrotic syndrome was the only independent predictor of carotid intima media thickness.     

Effect of Postoperative Restrictive Fluid Therapy in the Recovery of Patients with Abdominal Vascular Surgery

ObjectiveTo compare the outcome of the postoperative administration of a restricted or standard intravenous fluid regimen in patients who underwent elective abdominal vascular surgery. The primary end point was postoperative hospital stay.DesignProspective observer-blinded, randomised controlled trial.Material and methodsPatients were considered eligible if they underwent transperitoneal aorto-iliac approach with infrarenal graft repair. During the operation and intensive care unit stay, fluids were prescribed by the anaesthetists who were unaware of the details of the study. In the vascular surgical ward, patients in the standard group (SG) received 2500 ml of fluids per day, whereas patients in the restricted group (RG) received 1500 ml of fluids per day. All the patients were evaluated on an intention-to-treat basis.ResultsForty patients were randomised to the RG (n = 20) or SG (n = 20). No significant differences were observed in the recovery of gastrointestinal function. However, the postoperative hospital stay was shorter in the RG (8 days) than in the SG (12 days) (p = 0.003).ConclusionsThe use of a restrictive postoperative fluid protocol significantly reduces the duration of hospital stay in patients who have undergone major elective abdominal vascular surgery.     

Le cyclophosphamide dans le syndrome néphrotique idiopathique : résultats et perspectives

IntroductionCyclophosphamide (CYP) has been used for over 40 years in patients with steroid-sensitive nephrotic syndrome (NSSS) presenting frequent relapses (NSRF) or steroid dependence (NSSD). However, the long-term success of treatment with cyclophosphamide is difficult to predict. The objectives of this study are to determine long-term outcomes of cyclophosphamide and identify the factors associated with sustained remission.MethodsWe retrospectively studied the data from 50 patients with idiopathic nephrotic syndrome, treated by oral cyclophosphamide and followed at service of pediatric for more than 8 years for idiopathic nephrotic syndrome and related factors for survival without relapse were evaluated by univariate analysis.ResultsThe median age at the time of diagnosis was 4.3 years, and median follow-up time was 1.7 years with the median of 8 years at the first use of CYC. Patients had received a median cumulative dose of 168 mg/kg. At the end of follow-up, 38% of patients entered into remission after using CYC while 62% failed to respond and further relapses then occur. The median time of stopping corticosteroid therapy was three month. The survival without relapse was respectively 56% (28 patients), 52% (26 patients), 48% (24 patients), and 38% (19 patients), at 6 months, one year, two years and more than two years. In univariate analysis, the survival without relapse was related to the age at the moment of starting the therapy par CYC (the median was 5 months for an age  <  8 years and 41 months for an age ≥ 8 years; P = 0.049), the type of nephrotic syndrome [36 months for SNRF, 4 months for NSSD and nephrothic syndrome steroid resistant (NSSR); P = 0.068], and the histological lesion (6 months for diffuse mesangial proliferation, 2 months for segmental glomerulosclerosis; P = 0.009). The age at the moment of diagnosis, the sex and the cumulative dose of CYC did not have significant influence.ConclusionThe results presented in this study suggest the use of oral cyclophosphamide for short period remain second line effective therapy. Further well-designed trials are required to evaluate the efficacy of other steroid-sparing agents.     

Diagnostic utility of a second minor salivary gland biopsy in patients with suspected Sjögren's syndrome: A retrospective cohort study

ObjectiveTo determine whether repeated minor salivary gland biopsy (MSGB) has a clinical diagnostic utility in patients with suspicion of Sjögren's syndrome (SS).MethodsClinical, biological, pathological data and physician's diagnosis after each MSGB from patients with suspected primary or secondary SS who had benefited from 2 MSGB at Brest University Hospital between January 1st, 1990 and January 14th, 2015, were retrospectively collected. We compared the characteristics of patients with and without first positive MSGB, concordance between the MSGB, and analyzed the modifications of diagnosis after the second MSGB.ResultsNinety-three patients were included, first MSGB was positive for 23 and negative for 70. Patients with first positive MSGB had more often renal involvement (P < 0.05) and hypergammaglobulinemia (P = 0.01), anti-SSA antibodies (P < 0.05) and positive second biopsy with focus score ≥ 1 or Chisholm > 2 (P < 0.01). The mean time between the 2 MSGB was 5.7 ± 4.3 years. The concordance between the results of the 2 biopsies was low (κ = 0.34). MSGB influenced diagnostic's change in 10 cases where the second MSGB was always guided by new specific clinical manifestations.ConclusionWe observed a low concordance between 2 MSGB in patients with suspected pSS in our study. Despite this variability, performing a second MSGB changed the initial diagnosis in only a minority of the patients and was particularly useful when clinical manifestations had deeply evolved.     

Intravesical botulinum toxin for overactive bladder syndrome without detrusor overactivity

ObjectiveTo report our experience of intravesical botulinum toxin for idiopathic overactive bladder syndrome (OAB) without detrusor overactivity (DOA) on urodynamic assessment.Patients and methodsData regarding presentation, diagnosis, urodynamic findings, date and dose of treatment, and outcomes were recorded prospectively for 94 patients undergoing intravesical botulinum toxin injection for idiopathic overactive bladder syndrome at our institution. The cohort included 19 patients without DOA on urodynamics. A positive response to treatment was defined as patient-reported improvement without the need for further treatment. ICIQ-OAB and UI scores, and bladder diary parameters were also recorded. Rates of urinary retention requiring intermittent or indwelling catheterisation were noted.ResultsThe overall response rate to treatment was 82% (n = 94). Patients without DOA (n = 19) had a response rate of 89%, which compared favourably with a response rate of 81% in patients with DOA (n = 75).Overall, 29% of patients who were voiding normally prior to treatment required intermittent self-catheterisation after the procedure. The requirement for self-catheterisation did not appear to be influenced by urodynamic findings.ConclusionThese preliminary, non-randomised data suggest that intravesical botulinum toxin injection may be efficacious in patients with OAB symptoms without DOA. Further evaluation by means of a randomised, controlled trial is suggested.     

Sarcoidosis presenting with and without Löfgren's syndrome: Clinical,radiological and behavioral differences observed in a group of 691 patients

ObjectivesJust a few series of Löfgren's syndrome have been reported. Our aim was to describe the epidemiology and clinical profile of sarcoidosis patients presenting with Löfgren's syndrome vs. non-Löfgren's syndrome.MethodsRetrospective cohort study of 691 consecutive patients with sarcoidosis diagnosed at the Bellvitge University Hospital in Barcelona, Spain, between 1976 and 2018.ResultsThree hundred and nine patients (44.7%) were diagnosed with Löfgren's syndrome and 382 with non-Löfgren's syndrome (55.3%). The mean age at diagnosis was 39.8 years-old (SD 11.7) vs. 46.6 (SD 14.5) (P < 0.001). 249 patients (80.6%) vs. 218 (57.1%) were female (P < 0.001), and mostly Caucasians (304, 98.4% vs. 351, 91.9%, P = 0.002). Out of the total 309, Löfgren's syndrome patients developed more frequently fever and articular involvement, and 45 (14.6%) presented with isolated periarticular ankle inflammation. When compared, radiological stages at diagnosis were more advanced in non-Löfgren's syndrome patients: stage 0 (2.9% vs. 14.7%), stage I (82.5% vs. 41.4%), stage II (14.6% vs. 29.3%), and stage III/IV (0 vs. 14.7%) (P < 0.001). Chronic trend > 2 years was more prevalent in non-Löfgren's syndrome (66, 22.6% vs. 233, 67.4%; P < 0.001), as well as the proportion of patients in whom treatment was needed (58, 18.8% vs. 224, 58.6%; P < 0.001). Risk factors related to chronic trend > 2 years were older age, stage II at diagnosis and the need of treatment.ConclusionsLöfgren's syndrome is a well-differentiated form of sarcoidosis with persuasive different epidemiological, clinical, radiological and prognostic features.     

Síndrome metabólico en los cálculos de oxalato cálcico: ¿es tan importante en este tipo de litiasis?

Introduction and objectiveThe association of metabolic syndrome with lithogenesis has been described, especially in uric acid stones.The aim of the work was to analyze the role of the metabolic syndrome in oxalocalcic lithogenesis.Materials and methodsMetabolic evaluation of 151 patients including biochemical, hormonal and 24-urine urine parameters, as well as characteristics associated with metabolic syndrome.The relationship between characteristics associated with metabolic syndrome and those related to lithogenesis was evaluated using Spearman's correlation coefficient (SCC), Student's t test and Fisher's exact test.ResultsThe average body mass index (BMI) was 25.9 (SD 3.7). The median age was 51 years (18.6-84.8) and 64.9% were men.There were no statistically significant differences between hypertension and estradiol, testosterone, triglycerides, or cholesterol (P = .191, .969, .454, .345, respectively). Regarding glucose, mean value was 114.5 and 93.5 mg/dl in patients with and without hypertension (P = .000). Glucose, estradiol, testosterone, or cholesterol levels did not vary with proteinuria (P = .518, P = .227, P = .095, P = .218, respectively). Mean triglycerides were 185.6 and 108.2 mg/dl in patients with and without proteinuria (P = .001). Hypertension and proteinuria were not associated (P = .586). BMI correlated with serum and urinary uric acid and urinary creatinine.ConclusionsThere are few associations between the characteristics of metabolic syndrome and abnormalities related to lithogenesis. Metabolic syndrome does not seem to have a relevant role in the development of oxalocalcic stones.     

Evaluación de los parámetros funcionales espermáticos en individuos infértiles normozooespérmicos

ObjectiveThis study evaluated the integrity of the chromatin structure, reactive oxygen species (ROS) levels, mitochondrial membrane potential (MMP), DNA damage and lipid peroxidation of semen samples from infertile men classified as unexplained infertility.MethodsBetween February 2010 and July 2011 semen parameters and functional tests were evaluated in 10 subjects with proven fertility, 10 that belong to general population and 8 with idiopathic infertility. In addition to the conventional semen analysis, the following unconventional seminal analysis were conducted: evaluation of ROS, MMP, sperm chromatin structure assay (SCSA) by flow cytometry, assessment of sperm membrane lipid peroxidation by spectrophotometry, and alkaline comet assay by electrophoresis.ResultsWe observed a significant increase (P < .05) in the production of ROS and the fragmentation or sperm DNA damage in the population of infertile men. There were no statistically significant differences (P > .05) in the analysis of sperm membrane integrity between the groups. Moreover, we observed significant correlations (P < .05) between SCSA and comet assay (r = 0.86) and the production of intracellular ROS (r = -0.588).ConclusionThe sperm from individuals with idiopathic infertility showed high levels of intracellular ROS and increased levels of DNA fragmentation in the sperm. These results suggest that these two parameters are related to unexplained infertility and therefore have clinical importance as a possible diagnostic and prognostic tool in the evaluation of idiopathic male infertility.     

Relación del síndrome metabólico con el cáncer de mama posmenopáusico

IntroductionMetabolic syndrome is associated with an increased risk of diabetes mellitus (DM) and coronary heart disease. It may also be associated with a higher risk of some common cancers. The objective of this study was to determine the relationship between metabolic syndrome and breast cancer in postmenopausal women.MethodsWe present a prospective cohort study of postmenopausal women. This cohort was divided into two groups: the «benign diagnosis group», including women who were studied after breast cancer screening; and the «malignant tumor group», including patients with breast cancer that had been diagnosed by biopsy. Age, weight, height, body mass index (BMI), abdominal perimeter, serum glucose, LDL, HDL and insulin levels were analyzed as variables under study. The HOMA-IR homeostatic model formula was used to assess insulin resistance. The differences were considered statistically significant when P < .05.ResultsTwo hundred women with a mean age of 61.5 ± 9.6 (range: 37-93) were enrolled in the study, consisting of 150 (75%) patients with a benign diagnosis and 50 (25%) patients with a malignant tumor. BMI and abdominal perimeter were higher in the group with a malignant tumor (P < .05). The incidence of DM and metabolic syndrome was higher in the malignant tumor group (P < .005). In the malignant tumor group, much higher incidences correlated with fasting glycemic levels > 100 mg/dL, insulin levels > 10 mIU/L and HOMA-IR scores > 2.7 (P < .05).ConclusionsThere is a relationship between metabolic syndrome and postmenopausal breast cancer. More studies are needed to establish methods for the prevention of breast cancer in women with metabolic syndrome.     

Erythromycin improves gastric emptying half-time in adult cystic fibrosis patients with gastroparesis

BackgroundGastrointestinal manifestations are frequently encountered in cystic fibrosis patients. Gastroparesis evidenced by a variety of diagnostic methods has been described in patients with cystic fibrosis, predominantly in children and in individuals with advanced lung disease. The presence of gastroparesis in adult patients with different degrees of lung involvement and its response to the acute and chronic administration of macrolides have not been reported.MethodsUsing the University of Florida Cystic Fibrosis database we identified symptomatic patients who had gastroparesis confirmed by a prolonged half-time during gastric emptying scintigraphy.ResultsOf 86 cystic fibrosis patients, periodically followed in our institution, we found five who had classical symptoms and prolonged gastric emptying half-time. Age 25.2 ± 8 years, 80% females, BMI 22 ± 9 kg/m², HbA1c 5.8 ± 0.6 g/dl, FEV1 53.2 ± 15% of predicted. Gastric emptying half-time was 191.4 ± 91.4 min (range 100–300 min) and decreased to 12.2 ± 6 min (range 5–20 min) after IV administration of erythromycin (p = 0.043). Patients were followed up for 3 ± 2.1 years. All patients but one, who was taking opiods, had good clinical response to PO macrolides.ConclusionsGastroparesis occurs in patients with cystic fibrosis, even in patients with relatively preserved lung function and in those without cystic-fibrosis related diabetes. Macrolides may be an effective therapy in cystic fibrosis patients with gastroparesis when administered acutely or chronically.     

Quantification of diffuse myocardial fibrosis using CMR extracellular volume fraction and serum biomarkers of collagen turnover with histologic quantification as standard of reference

PurposeTo compare the assessment of diffuse interstitial myocardial fibrosis in valvular diseases using cardiac magnetic resonance (CMR) extracellular volume fraction (ECV) quantification and serum biomarkers of collagen turnover using results of myocardial biopsy as standard of reference.Materials and methodsThis prospective monocentric study included consecutive patients before aortic valvular replacement. All patients underwent: i), 1.5 T CMR with pre and post contrast T₁ mapping sequence and ECV computation; ii), serum quantification of matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs) and iii), myocardial biopsies were collected during surgery to assess collagen volume fraction (CVF). Patients with coronary artery disease were excluded. Correlation between native T1, ECV, CVF and serum biomarkers were assessed using Pearson correlation test. Agreement between basal anteroseptal ECV with global ECV was assessed using Bland-Altman test.ResultsTwenty-one patients, 16 with aortic stenosis and 5 with aortic regurgitation were included. There were 12 men and 9 women with a mean age of 74.1 ± 6.8 (SD) years (range: 32–84 years). Mean global ECV value was 26.7 ± 2.7 (SD) % (range: 23.4–32.5%) and mean CVF value was 12.4 ± 9.7% (range: 3.2–25.7%). ECV assessed at the basal anteroseptal segment correlated moderately with CVF (r = 0.6; P = 0.0026). There was a strong correlation and agreement between basal anteroseptal ECV and global ECV, (r = 0.8; P < 0.0001; bias 5.4 ± 6.1%) but no correlation between global ECV and CVF (r = 0.5; P = 0.10). Global ECV poorly correlated with serum TIMP-1 (r = 0.4; P = 0.037) and MMP-2 (r = 0.4; P = 0.047). No correlation was found between serum biomarkers and basal anteroseptal- ECV or native T1.ConclusionIn patients with severe aortic valvulopathy, diffuse myocardial fibrosis assessed by anterosepto-basal ECV correlates with histological myocardial fibrosis. Anteroseptobasal ECV strongly correlates with global ECV, which poorly correlates with TIMP-1 and MMP-2, serum biomarkers involved in the progression of heart failure.