The objective of our study was to compare the cellular and extracellular matrix (ECM) structure and the biomechanical properties of human pericardium (HP) with the normal human aortic heart valve ...(NAV). HP tissues (from 12 patients) and NAV samples (from 5 patients) were harvested during heart surgery. The main cells in HP were pericardial interstitial cells, which are fibroblast-like cells of mesenchymal origin similar to the valvular interstitial cells in NAV tissue. The ECM of HP had a statistically significantly (p < 0.001) higher collagen I content, a lower collagen III and elastin content, and a similar glycosaminoglycans (GAGs) content, in comparison with the NAV, as measured by ECM integrated density. However, the relative thickness of the main load-bearing structures of the two tissues, the dense part of fibrous HP (49 ± 2%) and the lamina fibrosa of NAV (47 ± 4%), was similar. In both tissues, the secant elastic modulus (Es) was significantly lower in the transversal direction (p < 0.05) than in the longitudinal direction. This proved that both tissues were anisotropic. No statistically significant differences in UTS (ultimate tensile strength) values and in calculated bending stiffness values in the longitudinal or transversal direction were found between HP and NAV. Our study confirms that HP has an advantageous ECM biopolymeric structure and has the biomechanical properties required for a tissue from which an autologous heart valve replacement may be constructed.
Narcolepsy-cataplexy (N-C) is a focal neurodegenerative disease with a genetic predisposition and autoimmune etiology; the pathogenesis of narcolepsy without cataplexy (Nw/oC) is less clear. One ...hundred and forty eight patients underwent clinical face-to face interviews, polysomnography, multiple sleep latency testing and HLA-DQB1*0602 typing. The cohort was divided into four age groups: children and adolescents under 19 years (
N
= 31), adults aged 20–39 years (
N
= 51), 40–59 years (
N
= 28) and over 60 years (
N
= 38). N-C was found in 93 adults (79.5 %) compared with 16 pediatric patients (51.6 %) (
p
< 0.01), suggesting that at least some of the children were candidates for developing cataplexy in the future. Statistical evaluation showed an increasing age-related proportion of associated sleep disorders—obstructive sleep apnea, periodic leg movements and restless leg syndrome (
p
< 0.001). Nw/oC patients showed sleep comorbidities less frequently than N-C group. A close connection with N-C was found particularly in REM behavior disorder (RBD) (
p
< 0.05). RBD affected a third of the patients in the youngest as well as in the oldest groups. However, association with other sleep disorders had no significant effect on nocturnal sleep (with the exception of obstructive sleep apnea), and the sleep comorbidities under study had no noticeable effect on daytime sleepiness.
Background
We describe data on 10,472 renal biopsies gathered by the Czech Registry of Renal Biopsies over a period of 18 years.
Methods
We assessed the main demographic, clinical and histological ...data of individuals who underwent renal biopsies of native kidneys in 31 centers in the Czech Republic (population 10.3 million) during the period 1994–2011.
Results
We evaluated 10,472 renal biopsies: males 57.8 %, children (≤15 years) 13.6 %, elderly (>60 years) 19.1 %. The most frequent biopsy-proven diseases were primary (55.7 %) and secondary (29.1 %) glomerulonephritides (GN). Tubulointerstitial nephritis (TIN) was observed in 3.4 % and vascular diseases in 4.1 %. The samples were non-diagnostic in 4.2 %. Among primary GN the most frequent diagnoses were IgA nephropathy (IgAN) (37.4 %), membranous GN (MGN) (13 %) and focal segmental glomerulosclerosis (FSGS) (12.6 %). Among secondary GN, systemic lupus erythematosus (SLE) represented 23.2 %, hereditary diseases 19.8 % and necrotizing vasculitis (NV) 19.4 %. Among adults, mild renal insufficiency serum creatinine (SCr) 111–200 μmol/l was present in 24.7 %, advanced renal insufficiency (SCr 201–400 μmol/l) in 15.3, and 12.3 % of patients had SCr > 400 μmol/l. The most common diseases in patients with nephrotic proteinuria were minimal change disease (MCD) (39.7 %) among children, IgAN (26.2 %) in adults aged 16–60 years and amyloidosis (42.7 %) among the elderly. The mean annual incidence (per million population) was: primary GN 30.9, secondary GN 18.1, IgAN 11.6, MGN 4.0, SLE 4.0, FSGS 3.9, MCD 3.4, NV 3.2, diabetic nephropathy 2.3, thin basement membrane glomerulopathy 2.0, mesangioproliferative GN 1.9, and TIN 1.9. Ultrasound needle guidance was used in 66.8 %. The frequency of serious complications (symptomatic hematoma, gross hematuria, blood transfusion) was approximately 3.2 %.
Conclusions
This report provides representative population-based data on native biopsy-proven renal diseases in the Czech Republic. Over the 18 years of nationwide biopsy survey, we noted an increase of the mean age of renal biopsy cases, an increasing proportion of elderly, and a cardinal change in biopsy technique towards ultrasonography needle guidance.
Background: Extrinsic allergic alveolitis (EAA) patients form heterogenous group with different clinical manifestation and different prognosis. We aimed to determine how to phenotype distinct EAA ...subgroups. Predictive role of the bronchoalveolar lavage fluid (BALF) IL-4Rα concentration at the time of diagnosis with regard to the clinical behavior in EAA patients was studied. Methods: Concentrations of MMP-7, IL-4Rα, TNF-α, and PAR-2 were measured in the BALF od 71 EAA patients at the time of diagnosis. Lung functions and outcome data were assessed at 12 months after the diagnosis. Correlations between the BALF protein concentration, cell profile, lung functions and patient outcome were determined. Results: We found positive correlations between BALF IL-4Rα concentration and BALF eosinophils (p = 0,006), negative correlation between IL-4Rα BALF concentration and diffusing capacity (DLco) (p = 0,003), negative correlation between IL-4Rα BALF concentration and forced vital capacity (FVC) (p = 0,004) and negative correlation between IL-4Rα concentration and BALF lymphocytes (p = 0,04). The BALF concentration of IL-4Rα was significantly higher in acute exacerbation patients (p = 0,0032) and in patients progressing despite corticosteroid treatment (p = 0,04). We observed a positive correlation between MMP-7 BALF concentration and the BALF lymphocytes (p = 0.05), negative correlation between the PAR-2 BALF concentration and DLco (p = 0.04) and a negative correlation between the BALF TNF-α concentration and DLco (p = 0.03). Conclusions: Specific subgroup of EAA patients with more severe functional impact, distinct BALF cell profile and higher IL-4Rα BALF concentration can be differentiated. Correlations between the BALF concentrations of PAR-2, MMP-7 and TNF-α with clinical parameters may reflect the role of inflammation in the pathogenesis of EAA.
Abstract Objective To evaluate sleep macrostructure, sleep disorders incidence and daytime sleepiness in attention-deficit/hyperactivity disorder (ADHD) affected children compared with controls. ...Methods Thirty-one patients (26 boys, 5 girls, mean age 9.3 ± 1.7, age range 6–12 years) with ADHD diagnosed according to DSM-IV criteria, without comorbid psychiatric or other disorders, as never before pharmacologically treated for ADHD. The controls were 26 age- and sex-matched children (22 boys, 4 girls, age range 6–12 years, mean age 9.2 ± 1.5). Nocturnal polysomnography (PSG) was performed for two nights followed by the multiple sleep latency test (MSLT). Results No differences between the two groups comparing both nights were found in the basic sleep macrostructure parameters or in the time (duration) of sleep onset. A first-night effect on sleep variables was apparent in the ADHD group. Occurrence of sleep disorders (sleep-disordered breathing SDB, periodic limb movements in sleep PLMS, parasomnias) did not show any significant differences between the investigated groups. A statistically significant difference ( p = 0.015) was found in the trend of the periodic limb movement index (PLMI) between two nights (a decrease of PLMI in the ADHD group and an increase of PLMI in the control group during the second night). While the mean sleep latency in the MSLT was comparable in both groups, children with ADHD showed significant (sleep latency) inter-test differences (between tests 1 and 2, 1 and 4, 1 and 5, p < 0.01). Conclusion After the inclusion of adaptation night and exclusion of psychiatric comorbidities, PSG showed no changes in basic sleep parameters or sleep timing, or in the frequency of sleep disorders (SDB, PLMS) in children with ADHD compared with controls, thus not supporting the hypothesis that specific changes in the sleep macrostructure and sleep disturbances are connected with ADHD. A first-night effect on sleep variables was apparent only in the ADHD group. Though we found no proof of increased daytime sleepiness in children with ADHD against the controls, we did find significant vigilance variability during MSLT in the ADHD group, possibly a sign of dysregulated arousal.
Abstract Aims Diabetes mellitus and decreased renal function are important risk factors for contrast-induced nephropathy (CIN) in which oxidative stress damage may play a role. Alkalinization with ...sodium bicarbonate (NaHCO3 ) has been proposed as a means of reducing free-radical mediated renal injury; however, the effectiveness of NaHCO3 treatment to prevent CIN in high-risk patients remains uncertain. Methods We performed a prospective, randomized, double blind, sodium chloride (NaCl) hydration-controlled study of NaHCO3 in 120 diabetic patients with impaired renal function (serum creatinine ≥100 μmol/L) undergoing an elective procedure with use of low-osmolar contrast media. The primary endpoint was the incidence of CIN defined as creatinine increase of ≥25% and/or ≥44 μmol/L within 2 days after contrast. Secondary end-points were maximal changes in serum creatinine and estimated glomerular filtration rate. Urine F2 -isoprostane levels were also assessed as measure of oxidative stress. Results There were no significant group differences in baseline characteristics except for the marginally lower age of the NaHCO3 treated patients (63 ± 11 vs. 67 ± 10 years; p = 0.05). CIN occurred in 7 (11.5%) and 5 (8.5%) patients of the NaHCO3 and NaCl groups, respectively ( p = 0.76; incidence rate ratio 1.35; 95% CI 0.37–5.41). No significant differences were seen in secondary outcome measures and changes in the parameter of oxidative stress. Conclusions In diabetic patients with renal function impairment sodium bicarbonate does not confer protection against contrast-induced nephropathy greater than sodium chloride-based hydration. Its specific role in mitigating oxidative stress damage in CIN is also not supported by our data.
OBJECTIVE: To assess the new quantitative bone scan parameters as markers of Charcot neuroosteoarthropathy (CNO) activity. RESEARCH DESIGN AND METHODS: Forty-two patients with acute (n = 21) and ...nonacute (n = 21) CNO underwent quantitative bone scanning. Patients with acute CNO were followed for 3-12 months and bone scans were repeated after treatment. New quantitative parameters were assessed and compared with markers of bone turnover and with skin temperature difference (STD). RESULTS: Significant correlations between quantitative bone scan parameters and bone turnover markers were observed (all P < 0.05). These parameters decreased after treatment of CNO, and its reduction to the baseline value correlated with differences of bone turnover markers and STD (all P < 0.05). CONCLUSIONS: Our study suggests that bone scanning can be used not only for diagnosis of CNO but also for monitoring disease activity by quantitative bone scan parameters.
A genetic predisposition has been identified in 30% of idiopathic pulmonary fibrosis (IPF) cases. Although it is highly probable that the genotype affects the disease susceptibility and course in ...almost all patients, the specific genotype goes undetected. The aim of the present study was to explore the effects of variants of the genes encoding interleukin-4 (IL-4), mucin 5B (MUC5B), toll interacting protein (TOLLIP), surfactant protein A (SFPTA), transforming growth factor-p (TGF-p) and transporters associated with antigen processing (TAP1 and TAP2) on the course of IPF. A total of 50 patients with IPF were enrolled, and variants of these genes were assessed. Lung function at the time of diagnosis and after 6, 12 and 18 months, and the number of acute exacerbations and deaths in each observation period were measured. ANOVA was used to test the association between gene polymorphisms and the decrease in lung function. There was no significant effect of the gene polymorphisms on the outcomes of patients up to 6 months during the observation period. After 12 months, an effect of an IL-4 single nucleotide polymorphism (SNP) (rs 2070874) on patient outcomes was observed relative risk (RR) for T allele: 5.6; 95% confidence interval (CI), 0.79-390; P=0.053. The RR of progression in patients with the IL-4 SNP (rs 2243250) and the CT and TT genotypes was 4.3 (95% CI, 1.1-175; P=0.046). A total of 18 months after the diagnosis of IPF, an effect of the TOLLIP polymorphism on patient outcome was detected (rs 111521887; risk allele GC; RR: 7.2; 95% CI, 0.97-53.6; P=0.052). Thus, IL-4 and TOLLIP gene polymorphisms may represent disease course-modifying factors, but not drivers of IPF.
Abstract Background Childhood parasomnias are believed to be a benign disorder due to immaturity of some neural circuits, synapses and receptors. The aim of our study was to explore a possible ...connection with other neurological developmental disorders. Methods 72 children (mean age 9.9 ± 5.0 years, 47 boys) were clinically examined and 88 nocturnal v-PSG and 22 v-EEG recordings were evaluated. The most frequent diagnostic findings were: sleepwalking in 24 children, confusional arousal in 21, sleep terror in 8, groaning and enuresis each in 7, non-specific arousal disorder in 4 patients, and REM-related parasomnia in only one child. For statistical evaluation chi-square test, the two-sample t -test and Mann–Whitney rank test were used. Results Perinatal risk history was found in 38% of the cohort. Developmental disorders were diagnosed in 30 children (41.7%), more frequently in combinations with: attention-hyperactivity disorder (30.6%), dyslexia and dysgraphia (13.9%), developmental dysphasia (9.7%), mild motor and/or intellectual dysfunction (6.9%). Abnormal movements in sleep, some of them also regarded as developmental, were diagnosed in 37 children (51.4%). Sleep-related breathing disorders were found in 29 patients (40.3%) –snoring (29.2%) and/or sleep apnea (11.1%). Only 16.7% had no comorbidity. Most of the children (60%) showed 2 or 3, exceptionally up to 5 comorbidities. Children, in whom no parasomnia was found in close relatives, had a mild but non-significant earlier onset of the disease (4.4 ± 4.0 against 6.3 ± 4.3 years). Conclusion Childhood parasomnias are frequently associated with perinatal risk factors and developmental comorbidities, and can be regarded as a disorder of sleep maturation.
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