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1.

Background

Symptoms at suspicion of necrotizing enterocolitis (NEC) are often nonspecific and several biomarkers have been evaluated for their discriminative power to both diagnose and predict the course from NEC suspicion to complicated disease requiring surgical intervention. Thus, we aimed to assess the utility of interleukin-6 (IL-6) to predict surgical intervention in infants suffering from NEC and, furthermore, to discriminate infants with starting NEC or late-onset sepsis (LOS).

Methods

IL-6 serum levels at disease onset were retrospectively analyzed in 24 infants suffering from NEC as well as 16 neonates with LOS.

Results

IL-6 serum levels at disease onset were significantly higher in infants suffering from NEC necessitating surgical intervention in the disease course compared to infants with medical NEC (5000 [785–5000] vs. 370 [78–4716] pg/ml, p?=?0.0008) as well as gram-positive LOS (5000 [785–5000] vs. 84 [12–269] pg/ml, p?=?0.0001). Infants suffering from gram-negative LOS exhibited elevated IL-6 serum levels at disease onset comparable to infants with surgical NEC (5000 [1919–5000] vs. 5000 [785–5000] pg/ml, p?=?1.00).

Conclusion

The proinflammatory cytokine IL-6 appears to be a promising marker to distinguish surgical NEC from medical NEC at the onset of disease but cannot discriminate between surgical NEC and gram-negative LOS.

Level of evidence

II  相似文献   

2.

Background

The effects of lumacaftor-ivacaftor therapy on glycemia have not been thoroughly investigated. Continuous glucose monitoring (CGM) provides detailed information about glycemic patterns and detects glucose abnormalities earlier than traditional screening tools for diabetes.

Methods

CGM measures, HbA1c, and oral glucose tolerance test (OGTT) results were collected and within-subject results compared in F508del homozygous youth with CF before and after initiation of lumacaftor-ivacaftor using the Wilcoxon signed-rank test.

Results

Nine youth with CF (6 males, median age 12.7?years) were enrolled. CGM was performed in all participants before (median 26?weeks) and after lumacaftor-ivacaftor (median 29?weeks). HbA1c and fasting plasma glucose increased (p?=?.02) after lumacaftor-ivacaftor initiation. No changes in OGTT 1?h or 2?h glucose nor CGM measures were observed overall. When analyzed by sex, males showed lower glycemic variability, as reflected by the mean amplitude of glycemic excursions, on the post-treatment CGM.

Conclusions

Glycemic abnormalities persisted in CF patients treated with lumacaftor-ivacaftor, although sex-dependent differences in glycemic response to treatment may exist.  相似文献   

3.

Objectives

Current studies concentrate on the cytokine network and its role in the pathogenesis of spondyloarthritis (SpA). In this study, we analyzed whether the serum cytokine profile (interleukins: IL-10, IL-11, IL-12, IL-15, IL-17, IL-23 and IL-33) correlates with demographic data, clinical manifestations, disease activity and treatment outcome in a group of patients with axial spondyloarthritis.

Material and methods

Forty-nine patients with an established diagnosis of axial spondyloarthritis (aSpA) and 19 healthy volunteers as controls were enrolled in the study. Clinical evaluation included patient''s medical history, 44 joint count, back pain intensity and global disease activity in the preceding week (VAS), the duration of morning stiffness and blood tests. Disease activity was assessed using BASDAI and ASDAS-CRP. Serum concentration of IL-10, IL-11, IL-12, IL-15, IL-17, IL-23 and IL-33 was determined.

Results

In patients with aSpA, elevated serum concentration of IL-10, IL-15, IL-17 and IL-23 was detected. In the aSpA group we detected higher values of serum concentration of IL-23 and IL-33 in the subgroup with anterior uveitis (83.1 ±184.0 pg/ml vs. 14.0 ±17.1 pg/ml, p < 0.0001 and 45.5 ±71.9 pg/ml vs. 18.4 ±14.3 pg/ml, p < 0.0001, respectively). Additionally, in the subgroup with peripheral arthritis, elevation of serum concentration of IL-12 (249.3 ±246.9 pg/ml vs. 99.9 ±105.9 pg/ml, p = 0.0001) was detected. Patients with preradiological SpA had higher serum concentration of IL-17 than patients with established diagnosis of AS (6.37 ±8.50 pg/ml vs. 2.04 ±2.98 pg/ml, p = 0.0295). No differences in serum concentration of analyzed cytokines were found between the subgroup with low to moderate disease activity and the subgroup with high to very high disease activity.

Conclusions

We report that in aSpA patients, compared to controls, elevated serum concentrations of IL-10, IL-15, IL-17 and IL-23 were observed. Some cytokines may predispose to a more severe course of aSpA.  相似文献   

4.

Objective

Disruption of the enterohepatic circulation of bile acids (BAs) is part of the gastrointestinal phenotype of cystic fibrosis (CF). Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, improves pulmonary function in CF patients with class III gating mutations. We studied the effect of ivacaftor on the enterohepatic circulation by assessing markers of BA homeostasis and their changes in CF patients.

Methods

In CF patients with an S1251N mutation (N?=?16; age 9–35?years S125N study/NTR4873) or a G551D mutation (N?=?101; age 10–24?years; GOAL study/ NCT01521338) we analyzed plasma fibroblast growth factor 19 (FGF19) and 7α-hydroxy-4-cholesten-3-one (C4) levels, surrogate markers for intestinal BA absorption and hepatic synthesis, respectively, before and after treatment with ivacaftor.

Results

At baseline, median FGF19 was lower (52% and 53%, P?<?.001) and median C4 higher (350% and 364%, P?<?.001), respectively, for the S1251?N and G551D mutation patient groups compared to healthy controls. Treatment with ivacaftor significantly increased FGF19 and reduced C4 levels towards normalization in both cohorts but this did not correlate with CFTR function in other organs, as measured by sweat chloride levels or pulmonary function.

Conclusions

We demonstrate that patients with CFTR gating mutations display interruption of the enterohepatic circulation of BAs reflected by lower FGF19 and elevated C4 levels. Treatment with ivacaftor partially restored this disruption of BA homeostasis. The improvement did not correlate with established outcome measures of CF, suggesting involvement of modulating factors of CFTR correction in different organs.  相似文献   

5.
6.

Background

We retrospectively evaluated the relationship between cytokine gene polymorphisms and development of postoperative pneumonia after esophagectomy.

Methods

In 120 patients who underwent esophagectomy, serum samples were obtained to measure levels of serum interleukin (IL)-6 and IL-10 at four time points (preoperatively, postoperative day (POD)0, POD1, and POD3). DNA extracted from peripheral blood in all patients was analyzed to determine polymorphisms of cytokines such as tumor necrosis factor-α -1031 T/C, IL-1β -511C/T, IL-6 -634C/G, and IL-10 -819 T/C.

Results

Postoperative pneumonia arose in 34 patients (28.3 %). Perioperative serum IL-10 levels were significantly higher for IL-10 -819 C/T?+?C/C genotypes than for T/T genotypes (POD0 16.7?±?2.84 vs. 8.54?±?0.87 pg/ml, p?=?0.0002; POD1 14.0?±?2.64 vs. 8.8?±?0.87 pg/ml, p?=?0.0143; POD3 8.9?±?2.67 vs. 4.4?±?0.52 pg/ml, p?=?0.0076). The frequency of the IL-10 -819 T/T genotype was significantly higher in patients with postoperative pneumonia than in patients without pneumonia (p?=?0.0323). Multivariate analysis of factors such as sex, smoking, length of operation, field of lymph node dissection, and IL-10 polymorphism identified IL-10 polymorphism as independent predictor of postoperative pneumonia.

Conclusions

Patients with IL-10 -819 T/T genotype may be at high risk for postoperative pneumonia after esophagectomy.  相似文献   

7.

Background

Hypoglycemia in cystic fibrosis (CF) patients during the oral glucose tolerance test (OGTT) has been reported; however, these patients have not been well-characterized. Few studies have examined whether hypoglycemia during the OGTT increases the risk of developing CF-related diabetes (CFRD). Objectives of this study were to describe the characteristics of CF patients with hypoglycemia during the OGTT and to determine the incidence and time to development of CFRD in those with hypoglycemia.

Methods

This cohort study included 466 adults with CF at the Toronto Adult CF Clinic between 1996 and 2015. Subjects were classified into two groups based on their plasma glucose (PG) level 2?h after a 75?g OGTT: hypoglycemia (PG?≤?3.9?mmol/L) or no hypoglycemia (PG?>?3.9?mmol/L). Clinical and demographic data were collected from the clinic visit closest to the OGTT. Differences between groups were assessed using Fisher's exact test or Mann-Whitney-Wilcoxon test.

Results

138 patients (29.6%) experienced hypoglycemia during the OGTT. More males experienced hypoglycemia compared to no hypoglycemia (69.6% vs. 54.6% respectively; p?=?0.003). Those who were heterozygous deltaF508 were more likely to experience hypoglycemia (p?=?0.006). Subjects who experienced hypoglycemia were less likely to develop CFRD at ten years compared to no hypoglycemia (12.0% vs. 42.1%, respectively; p?<?0.001).

Conclusions

Hypoglycemia following OGTT is common in CF however the 10?year risk of developing CFRD in these patients was low. Males and those who were heterozygous deltaF508 were at higher risk for hypoglycemia.  相似文献   

8.

Background

With improved prognosis of CF, comorbidities including chronic kidney disease (CKD) are becoming increasingly important. Identification of those at highest CKD risk is hence a priority.

Methods

In this cross-sectional study, adults with CF attending the Copenhagen CF Centre at Rigshospitalet with ≥ 2 measurements of serum creatinine from 2013 to 2015 were included. Data was obtained from an electronic CF database, which contains anonymised clinical and laboratory data on all individuals attending the clinic. CKD was defined as a confirmed (≥ 3 months apart) estimated glomerular filtration rate  60 mL/min/1.73m2.

Results

Of 181 individuals, the CKD prevalence was 2.7% and increased to 11% after inclusion of lung transplanted patients. Individuals with CKD were generally older (median 39 (IQR, 36–45) vs. 31 (IQR, 24–39) years; p < 0.001), diabetic (86% vs. 41%, p < 0.001), with longer median duration of chronic pulmonary infection (28.3 (20.0–35.8) vs. 20.0 (9.9–34.7) years; p = 0.008) and with longer intravenous aminoglycosides use (606 (IQR, 455–917) vs. 273 (IQR, 91–826) days, p = 0.005).

Conclusions

The CKD prevalence is high and related to age, diabetes, chronic infection, transplantation and aminoglycosides use. These observations call for longitudinal studies investigating CKD predictors in adults with CF.  相似文献   

9.

Background

We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.

Methods

In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1–6?months after commencement of ivacaftor, and were correlated with FEV1 (% predicted), sweat chloride, C-reactive protein (CRP) and body mass index (BMI).

Results

After 1?month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6?months. A significant inverse correlation between absolute and delta values of HE4 and FEV1 (r?=??0.5376; P?<?.001 and r?=??0.3285; P?<?.001), was retrospectively observed in pooled groups, including an independent association of HE4 with FEV1 by multiple regression analysis (β?=??0.57, P?=?.019). Substantial area under the receiver operating characteristic curve (ROC-AUC) value was determined for HE4 when 7% mean change of FEV1 (0.722 [95% CI 0.581–0.863]; P?=?.029) were used as classifier, especially in the first 2?months of treatment (0.806 [95% CI 0.665–0.947]; P?<?.001).

Conclusions

This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy.  相似文献   

10.

Background

Resistin is an immunometabolic mediator that is elevated in several inflammatory disorders. A ligand for Toll-like receptor 4, resistin modulates the recruitment and activation of myeloid cells, notably neutrophils. Neutrophils are major drivers of cystic fibrosis (CF) lung disease, in part due to the release of human neutrophil elastase- and myeloperoxidase-rich primary granules, leading to tissue damage. Here we assessed the relationship of resistin to CF lung disease.

Methods

Resistin levels were measured in plasma and sputum from three retrospective CF cohorts spanning a wide range of disease. We also assessed the ability of neutrophils to secrete resistin upon activation in vitro. Finally, we constructed a multivariate model assessing the relationship between resistin levels and lung function.

Results

Plasma resistin levels were only marginally higher in CF than in healthy control subjects. By contrast, sputum resistin levels were very high in CF, reaching 50–100 fold higher levels than in plasma. Among CF patients, higher plasma resistin levels were associated with allergic bronchopulmonary aspergillosis, and higher sputum resistin levels were associated with CF-related diabetes. Mechanistically, in vitro release of neutrophil primary granules was concomitant with resistin secretion. Overall, sputum resistin levels were negatively correlated with CF lung function, independently of other variables (age, sex, and genotype).

Conclusions

Our data establish relationships between resistin levels in the plasma and sputum of CF patients that correlate with disease status, and identify resistin as a novel mechanistic link between neutrophilic inflammation and lung disease in CF.  相似文献   

11.

Background

Treatment of hypocalcemia after thyroidectomy consists of an individual substitution, prophylaxis or a daily administration of calcium/vitamin D3.

Objective

Does prophylactic therapy combined with a risk-adapted substitution of calcium and vitamin D3 reduce symptomatic hypocalcemia compared to individual substitution?

Material and methods

After implementation of a new algorithm, patient data were prospectively documented and analyzed compared to a historical patient population. The algorithm consisted of a single prophylactic i.?v. administration of calcium (1 g calcium gluconate 10% in 250?ml saline) and a risk-adapted oral administration of calcium and vitamin D3 after surgery. Patients without risk (parathyroid hormone, PTH?>?15?pg/ml) were not treated. Patients with a low risk (PTH?≥?6?≤?15?pg/ml and Ca?>?2.0?mmol/l) received 3?g calcium, patients with a high risk (PTH?≥?6?≤?15?pg/ml and Ca?<?2.0?mmol/l) received 3?g calcium and 2?×?0.5?µg vitamin D3 and patients with a very high risk (PTH?<?6?pg/ml) got 4?g calcium and 2?×?0.5?µg vitamin D3.

Results

In this study 415 patients were included (230 prospectively and 185 retrospectively). Serum calcium of patients with individual substitution increased significantly at day 1 (p?=?0.0001) and the number of patients with critical hypocalcemia (Ca?<?2.0?mmol/l) decreased by half (27% vs. 12.2%; p?=?0.0001). There was a significantly lower rate of symptomatic patients (24.9% vs 13.0%; p?=?0.002) and a clear reduction of patients with a prolonged hospitalization (10.8% vs. 6.5%; p?=?0.08). The rate of permanent hypocalcemia was comparable (2.2% vs. 2.1%). In the risk groups there was a significantly different rate of hypocalcemia: patients without risk (n?=?170) in 2.2%, patients with low risk (n?=?36) in 25%, patients with high risk (n?=?13) in 69.2% and patients with very high risk (n?=?11) in 71%.

Conclusion

This new treatment regimen is practicable, significantly lowers the symptoms, also in comparison to the literature and shows a clear differentiation between the risk groups.
  相似文献   

12.

Objective

Antibiotic eradication treatment is the standard-of-care for cystic fibrosis (CF) patients with early Pseudomonas aeruginosa (Pa)-infection; however, evidence from placebo-controlled trials is limited.

Methods

This double-blind, placebo-controlled trial randomised CF patients <7?years (N?=?51) with early Pa-infection to tobramycin inhalation solution (TOBI 300?mg) or placebo (twice daily) for 28?days with an optional cross-over on Day 35. Primary endpoint was proportion of patients having throat swabs/sputum free of Pa on Day 29.

Results

On Day 29, 84.6% patients in the TOBI versus 24.0% in the placebo group were Pa-free (p?<?0.001). At the end of the cross-over period, 76.0% patients receiving TOBI in the initial 28?days were Pa-free compared to 47.8% receiving placebo initially. Adverse events were consistent with the TOBI safety profile with no differences between TOBI and placebo.

Conclusion

TOBI was effective in eradicating early Pa-infection with a favourable safety profile in young CF patients.Trial registration number: NCT01082367  相似文献   

13.

Background

We aim to clarify if frailty affects severe postoperative complications in elective colorectal surgery.

Methods

Consecutive 269 colorectal cancer patients older than 65 years undergoing curative surgery were enrolled in this study. The relevance of the frailty and sarcopenia to postoperative outcome was assessed. Clinical frailty (CF) was defined as clinical frailty scale (CFS)?≥?4. Sarcopenia was assessed by measuring skeletal muscle area using computed tomography.

Results

Seventy-eight patients (29%) had CF and 159 patients (59%) had sarcopenia. CF was significantly associated with older age (P?=?0.0008), postoperative severe complications (P?=?0.001), and postoperative in-hospital stay (P?<?0.0001), although sarcopenia was not. Logistic regression analysis revealed that low anterior resection and CF were independent predictors of severe postoperative complications (P?=?0.038 and P?=?0.001, respectively).

Conclusion

CF, but not sarcopenia, is a robust predictor of severe postoperative complications in patients with colorectal cancer.  相似文献   

14.

Background

Hepatic steatosis is a common manifestation of CF-related liver disease(CFLD). Controlled attenuation parameter(CAP) measurement during transient elastography(TE) semiquantifies liver steatosis. We examined the relationship between CAP and CFLD severity, clinical factors and liver stiffness measurements(LSM).

Methods

This is a cross-sectional study of CF patients seen for outpatient care between January 2013–March 2014. CFLD severity was categorized as no CFLD, CFLD without portal hypertension(PHTN) and CFLD with PHTN, based on published criteria.

Results

129 patients (median 18.4y; 57% male) had valid CAP. 70(54%) had no CFLD, 44(34%) CFLD without PHTN, and 15(12%) CFLD with PHTN. The median CAP was 210?dB/m (IQR 181–239). Steatosis(CAP ≥230?dB/m) was seen in 27% of subjects without CFLD, 48% in CFLD but no PHTN, and 20% in with CFLD and PHTN(P?=?.04). CAP was higher for subjects with CFLD without PHTN (P?<?.05). There was no CAP difference between subjects with no CFLD and those with CFLD and PHTN (P?≥?.65). LSM was not different between no CFLD and CFLD without PHTN (P?=?.07), but each of these groups had lower LSM compared to subjects with CFLD and PHTN(P?<?.001 for each). Except for direct bilirubin, CAP was not associated with clinical markers of liver disease.

Conclusion

CAP was normal in 86(67%) of patients with CF and was not associated with standard clinical markers of liver disease. CAP was higher in patients with liver disease, which could possibly reflect the loss of steatosis observed with progression to cirrhosis and portal hypertension.  相似文献   

15.

Background

Dornase alfa (DNase) is one of the commonest cystic fibrosis (CF) treatments and is often used for many years. However, studies have not evaluated the effectiveness of its long-term use. We aimed to use UK CF Registry data to investigate the effects of one-, two-, three-, four- and five-years of DNase use on lung function to see if the benefits of short-term treatment use are sustained long term.

Methods

We analysed data from 4,198 people in the UK CF Registry from 2007 to 2015 using g-estimation. By controlling for time-dependent confounding we estimated the effects of long-term DNase use on percent predicted FEV1 (ppFEV1) and investigated whether the effect differed by ppFEV1 at treatment initiation or by age.

Results

Considering the population as a whole, there was no significant effect of one-year's use of DNase; change in ppFEV1 over one year was ?0.1% in the treated compared to the untreated (p?=?0.51) and this did not change with long-term use. However, treatment was estimated to be more beneficial in people with lower lung function (p?<?0.001); those with ppFEV1?<?70% at treatment initiation, showed an increase in lung function over one year that was sustained out to five years. The estimated effect of DNase did not depend on age (p?=?0.35).

Conclusions

DNase improved lung function in individuals with reduced lung function, bringing a step-change in lung function, but no change in the slope of decline. There was no evidence for a benefit in lung function in those initiating treatment with ppFEV1?>?70%.  相似文献   

16.

Purpose

Partial nephrectomy (PN) is standard for small renal masses, improving renal function by preserving renal parenchyma compared with radical nephrectomy. Recent work demonstrated that postoperative surgeon assessment of volume preservation (SAVP) and 3D imaging measurements agree and correlate with postoperative function. We hypothesize preoperative assessment of volume preservation (PAVP) with PN based on preoperative imaging will reliably indicate postoperative renal function.

Materials and Methods

Data were collected from 336 patients undergoing PN for suspected renal cancer by 40 surgeons at 12 centers in Europe and the United States within the Surface-Intermediate-Base International Consortium. Surgeons recorded PAVP and SAVP for individual patients; pre- and postoperative glomerular filtration rate (GFR) was estimated by Chronic Kidney Disease Epidemiology Collaboration equations. Correlations between PAVP, SAVP, and postoperative GFR were assessed with linear regression models. Bland–Altman analysis was used to assess agreement between PAVP and SAVP with a significant cutoff of 5%.

Results

Median PAVP was 90% (interquartile range [IQR] 85%–100%) and SAVP was 90% (IQR: 80%–94%). PAVP and SAVP were moderately correlated (R2?=?0.67, P < 0.0001) and deemed “interchangeable” by Bland–Altman analysis at a 5% acceptable rate of difference (95% CI: ?5.4, ?3.1). Median postoperative GFR was 77.3 (IQR: 56.2, 92.0). Both PAVP (R2?=?0.82, P < 0.0001) and SAVP (R2?=?0.83, P < 0.0001) were correlated with postoperative GFR. Multivariable models utilizing volume-adjusted GFR based on PAVP or SAVP significantly and similarly predicted postoperative GFR (R2?=?0.72 for each).

Conclusion

Renal function is closely linked to the amount of parenchymal volume preservation, whether estimated prior to surgery (PAVP) or afterward (SAVP). PAVP provides reasonably accurate information for decision-making in patients considering PN.  相似文献   

17.

Background

Little is known about the role of interleukin (IL)-1 in the pathogenesis of cystic fibrosis (CF) lung disease. This study investigated the relationship between IL-1 signalling, neutrophilic inflammation and structural lung changes in children with CF.

Methods

Bronchoalveolar lavage fluid (BALf) from 102 children with CF were used to determine IL-1α, IL-1β, IL-8 levels and neutrophil elastase (NE) activity, which were then correlated to structural lung changes observed on chest computed tomography (CT) scans.

Results

IL-1α and IL-1β were detectable in BAL in absence of infection, increased in the presence of bacterial infection and correlated with IL-8 (p?<?0.0001), neutrophils (p?<?0.0001) and NE activity (p?<?0.01 and p?<?0.001). IL-1α had the strongest association with structural lung disease (p <?0.01) in the absence of infection (uninfected: p?<?0.01 vs. infected: p?=?0.122).

Conclusion

Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target.  相似文献   

18.

Background

Having motor impairment since childhood and being at risk of osteoporosis and falls, adults with polio would be more likely to suffer a hip fracture (HF) and may experience different epidemiological characteristics from the general population.

Objective

To estimate the risk and incidence of HF in adults with polio.

Design

Using a national database, we conducted a population-based cohort study. We identified patients with polio using the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code of 138. For each patient with polio, we randomly selected five age- and sex-matched control subjects. Those subjects aged <40 years were excluded. We analyzed participants aged 40?64 years (middle-aged) and subjects aged ≥65 years (elderly) separately and recognized subjects who had an HF (ICD-9-CM code, 820) only when they received hospitalization to care for the illness from January 1, 2003 to December 31, 2008.

Results

We identified 403 adults with polio (mean age?±?standard deviation, 47.2?±?8.6 years). Compared to the controls, patients with polio had a higher incidence of HF (all, 4.1 vs. 1.1/1000 person-years, p?=?0.002; middle-aged, 2.3 vs. 0.3/1000 person-years, p?<?0.001; male, 6.2 vs. 0.9/1000 person-years, p?<?0.001); had a younger mean age (±standard deviation) of fracturing a hip (61.0±14.9 vs. 74.4±9.3 years, p?=?0.015); had a lower cumulative HF-free probability (±standard error) before the age of 65 years (0.970±0.017 vs. 0.988±0.007, p<0.001) and throughout the study duration (0.415?±?0.296 vs. 0.682?±?0.158, p<0.001); and had a higher risk of HF, yielding an adjusted hazard ratio (95% confidence interval) of 3.58 (1.45?8.79, p?=?0.006). Patients with polio aged >48.2 years were likely to experience an HF.

Conclusions

Adults with polio are at risk of HF. A customized HF prevention program is important for people with polio. The program should be started early in middle-age and should include men.  相似文献   

19.

Summary

In our current adult CF population, low BMD prevalence was only 20 %, lower than that historically described. We found a mild increase of serum RANK-L levels, independent from the bone resorption level. The increased fracture risk in CF may be explained by a lower tibial cortical thickness and total vBMD.

Introduction

Bone disease is now well described in cystic fibrosis (CF) adult patients. CF bone disease is multifactorial but many studies suggested the crucial role of inflammation. The objectives of this study were, in a current adult CF population, to assess the prevalence of bone disease, to examine its relationship with infections and inflammation, and to characterize the bone microarchitecture using high resolution peripheral scanner (HR-pQCT).

Methods

Fifty-six patients (52 % men, 26?±?7 years) were assessed in clinically stable period, during a respiratory infection, and finally 14 days after the end of antibiotic therapy. At each time points, we performed a clinical evaluation, lung function tests, and biochemical tests. Absorptiometry and dorso-lumbar radiographs were also performed. A subgroup of 40 CF patients (63 % men, 29?±?6 years) underwent bone microarchitecture assessment and was age- and gender-matched with 80 healthy controls.

Results

Among the 56 CF patients, the prevalence of low areal BMD (T-score?<??2 at any site), was 20 % (95 % CI: [10.2 %; 32.4 %]). After infections, serum RANK-L (+24 %, p?=?0.08) and OPG (+13 %, p?=?0.04) were increased with a stable ratio. Microarchitectural differences were mostly observed at the distal tibia, with lower total and cortical vBMD and trabecular thickness (respectively ?9.9, ?3.0, and ?5 %, p?<?0.05) in CF patients compared to controls, after adjustment for age, gender, weight, and height.

Conclusions

In this study, bone disease among adult CF patients was less severe than that previously described with only 20 % of CF patients with low BMD. We found a mild increase of biological marker levels and an impaired volumetric density of the tibia that may explain the increased fracture risk in CF population.
  相似文献   

20.

Purpose

The purpose of this study was to determine whether seeding density of placental mesenchymal stromal cells (PMSCs) on extracellular matrix (ECM) during in utero repair of myelomeningocele (MMC) affects motor function and neuronal preservation in the ovine model.

Methods

MMC defects were surgically created in 33 fetuses and repaired following randomization into four treatment groups: ECM only (n?=?10), PMSC-ECM (42?K cells/cm2) (n?=?8), PMSC-ECM (167?K cells/cm2) (n?=?7), or PMSC-ECM (250–300?K cells/cm2) (n?=?8). Motor function was evaluated using the Sheep Locomotor Rating Scale (SLR). Serial sections of the lumbar spinal cord were analyzed by measuring their cross-sectional areas which were then normalized to normal lambs. Large neurons (LN, diameter 30–70?μm) were counted manually and density calculated per mm2 gray matter.

Results

Lambs treated with PMSCs at any density had a higher median SLR score (15 [IQR 13.5–15]) than ECM alone (6.5 [IQR 4–12.75], p?=?0.036). Cross-sectional areas of spinal cord and gray matter were highest in the PMSC-ECM (167?K/cm2) group (p?=?0.002 and 0.006, respectively). LN density was highest in the greatest density PMSC-ECM (250–300?K/cm2) group (p?=?0.045) which positively correlated with SLR score (r?=?0.807, p?<?0.0001).

Conclusions

Fetal repair of myelomeningocele with high density PMSC-ECM resulted in increased large neuron density, which strongly correlated with improved motor function.

Type of study

Basic science.

Level of evidence

N/A  相似文献   

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