Evaluation of functionality-mobility in patients with skeletal dysplasias. Application of the STEMS tool ("everyday symptoms and mobility screening tool for skeletal dysplasias").

Individuals with differing forms of skeletal dysplasias (SD) frequently report impaired mobility and symptoms. With the objetive to evaluate mobility and associated symptoms in people with SD at an Argentinian pediatric hospital, using an Argentinian version of the Screening Tool for Everyday Mobility and Symptoms (STEMS), a simple questionnaire that allows clinicians to quickly identify the presence of symptoms associated with mobility in people with SD, while considering different environmental settings and the use of assistive devices, an analytical study of a consecutive sample of patients older than 5 years with SD and their affected relatives was carried out.Diagnosis, comorbidities, socioenvironmental, therapeutic, auxological and mobility variables were recorded. The presence and intensity of symptoms was noted through use of both the STEMS and validated scales. Descriptive, association and correlation analyzes were performed. One hundred and nineteen individuals with SD were enrolled in the study and divided into groups: Osteogenesis Imperfecta (OI, n = 55), Achondroplasia (ACH, n = 36) and Other SD resulting in disproportionate short stature (n = 28). Mobility assistive devices were almost exclusively used by individuals with OI. They were more frequently used by individuals with overweight and obesity, more severe form of the disease and in the outdoor settings. Two thirds (66.4%) of the individuals assessed in this study reported pain, 87.4% reported fatigue, and 58.8% reported both pain and fatigue. The intensity of symptoms was similar between groups and correlated with age and auxological variables. The STEMS was clear, easy and quick to use for identifying presence of pain and fatigue in this population group. The STEMS proved to be a simple and useful tool for evaluating functional mobility and associated symptoms in our population of individuals with SD.

A Logical Modeling of Severe Ignorance.

In the logical context, ignorance is traditionally defined recurring to epistemic logic. In particular, ignorance is essentially interpreted as "lack of knowledge". This received view has - as we point out - some problems, in particular we will highlight how it does not allow to express a type of content-theoretic ignorance, i.e. an ignorance of φ that stems from an unfamiliarity with its meaning. Contrarily to this trend, in this paper, we introduce and investigate a modal logic having a primitive epistemic operator I, modeling ignorance. Our modal logic is essentially constructed on the modal logics based on weak Kleene three-valued logic introduced by Segerberg (Theoria, 33(1):53-71, 1997). Such non-classical propositional basis allows to define a Kripke-style semantics with the following, very intuitive, interpretation: a formula φ is ignored by an agent if φ is neither true nor false in every world accessible to the agent. As a consequence of this choice, we obtain a type of content-theoretic notion of ignorance, which is essentially different from the traditional approach. We dub it severe ignorance. We axiomatize, prove completeness and decidability for the logic of reflexive (three-valued) Kripke frames, which we find the most suitable candidate for our novel proposal and, finally, compare our approach with the most traditional one.

Growth in height and body proportion from birth to adulthood in hereditary hypophosphatemic rickets: a retrospective cohort study.

PURPOSE: Patients with hereditary hypophosphatemic rickets are short and disproportionate and very little information is available on segmental growth, but the body disproportion at adulthood leads us to think that the growth velocity of legs is slower. METHODS: A total of 96 children were included and molecular testing was carried out in 42. Children who reached adult height were classified into two groups according to their compliance to conventional treatment (phosphate supplement and calcitriol). Individual growth records of height and sitting height/height were plotted using Argentine reference data in 96 children and growth curves were estimated by fitting Preece-Baines Model 1 in 19 of the children. RESULTS: Molecular testing revealed sequence deleterious alterations or large deletions in 36/42 patients. During childhood, 76% of children grew below - 1.88 standard deviation score (SDS) and 97% had body disproportion. During adolescence, the mean peak height velocity for the good and poor compliance to treatment groups was 7.8 (0.6) and 5.4 (0.4) cm/year in boys and 7.0 (0.7) and 5.2 (0.8) cm/year in girls, respectively. At adulthood, the median sitting height/height ratio was 2.32 and 6.21 SDS for the good and poor compliance to treatment groups, respectively. The mean pubertal growth spurt of the trunk was -0.8 (1.4) SDS, with a short pubertal growth spurt of - 1.8 (0.4) SDS for limbs in the good compliance group. Median adult height in 13/29 males and 30/67 females was -4.56 and -3.16 SDS, respectively. CONCLUSION: For all patients the growth spurt was slower, secondary to a short growth spurt of limbs, reaching a short adult height with body disproportion that was more prominent in the poor compliance group.

Early childhood vaccination coverage and timeliness by macro-area of origin in children born to foreign women residing in Italy.

OBJECTIVES: Country of origin might affect vaccine uptake in children born to immigrants. We aimed to evaluate differences in childhood vaccination coverage (VC) and timeliness by macro-area of origin of foreign mothers residing in Italy. STUDY DESIGN: Multicentre retrospective birth cohorts. METHODS: We analysed data of 23,287 children born in 2009-2014 to foreign women in the cities of Rome, Turin and Treviso. We retrieved data through record-linkage of the population, vaccination and birth registries. We estimated VCs at different ages for vaccines against tetanus, measles and meningococcal group-C, using the Kaplan-Meier method. Factors associated with vaccine uptake were evaluated using multilevel Poisson models. RESULTS: Estimates of VC at any age and for all antigens were significantly lower in children born to women from Asia and higher in children born to women from Africa, as compared to other macro-areas. Similar differences by area of origin were observed for timeliness; independently of mother's sociodemographic characteristics and neonatal outcomes, the probability of delay vaccination after 2 years of age for each antigen was highest in children born to women from Asia. The risk of missed vaccination for all antigens was significantly higher in children born to younger and unemployed women. CONCLUSIONS: Factors related to area of origin (e.g., cultural habits, language skills) are likely to affect parents' decision to vaccinate their children. These factors, as well as sociodemographic characteristics, should be adequately investigated and addressed to increase vaccine uptake in foreign children, especially those born to Asian women.

Pubertal growth in height, sitting height and leg length in achondroplasia.

Background: Children with achondroplasia (ACH) appear to lack a pubertal growth spurt in height.Aim To explore the growth spurt in height and its segments sitting height and leg length, in a large sample of ACH cases using growth curve modelling.Subjects and methods: Height and sitting height were measured longitudinally in ACH children, and the data were analysed using the SITAR (SuperImposition by Translation and Rotation) growth model, which estimates a mean growth curve and random effects for individuals defining differences in size, pubertal timing and intensity.Results: Out of 402 ACH children, 85 boys and 75 girls aged 7-20 years had respectively 529 and 454 measurements of height and sitting height, with leg length calculated by difference. SITAR analysis identified peaks in mean height velocity at 13.3 and 11.3 years in boys and girls, with peak velocities of 4.3 and 4.4 cm/year. Mean peak velocity for sitting height was 3.0 cm/year, but leg length showed no peak. The SITAR models explained 92% to 99% of the cross-sectional variance.Conclusion: ACH children do experience a growth spurt in puberty, but only half that of control children. The spurt is due entirely to sitting height, with no leg length spurt.

[Mortality results in SENTIERI Project]. / Risultati dell'analisi della mortalità nel Progetto SENTIERI.

SENTIERI Project (Mortality study of residents in Italian polluted sites) studies mortality of residents in 44 sites of national interest for environmental remediation (Italian polluted sites, IPS). The epidemiological evidence of the causal association between causes of death and exposures was a priori classified into one of these three categories: Sufficient (S), Limited (L) and Inadequate (I). In these sites various environmental exposures are present. Asbestos (or asbestiform fibres as in Biancavilla) has been the motivation for defining six sites as IPSs (Balangero, Emarese, Casale Monferrato, Broni, Bari-Fibronit, Biancavilla). In five of these, increases in malignant neoplasm or pleura mortality are detected; in four of them, results are consistent in both genders. In six other sites (Pitelli, Massa Carrara, Aree del Litorale Vesuviano, Tito, "Aree industriali della Val Basento", Priolo), where other sources of environmental pollution in addition to asbestos are reported, mortality from malignant neoplasm of pleura is increased in both genders in Pitelli, Massa Carrara, Priolo, "Litorale vesuviano". In the time span 1995-2002, a total of 416 extra cases of malignant neoplasm of pleura are detected in the twelve asbestos-polluted sites. Asbestos and pleural neoplasm represent an unique case. Unlike mesothelioma, most causes of death analyzed in SENTIERI have multifactorial etiology; furthermore, in most IPSs multiple sources of different pollutants are present, sometimes concurrently with air pollution from urban areas: in these cases, drawing conclusions on the association between environmental exposures and specific health outcomes might be complicated. Notwithstanding these difficulties, in a number of cases an etiological role could be attributed to some environmental exposures. The attribution could be possible on the basis of increases observed in both genders and in different age classes, and the exclusion of a major role of occupational exposures was thus allowed. For example, a role of emissions from refineries and petrochemical plants was hypothesized for the observed increases in mortality from lung cancer and respiratory diseases in Gela and Porto Torres; a role of emissions from metal industries was suggested to explain increased mortality from respiratory diseases in Taranto and in Sulcis-Iglesiente-Guspinese. An etiological role of air pollution in the raise in congenital anomalies and perinatal disorders was suggested in Falconara Marittima, Massa-Carrara, Milazzo and Porto Torres. A causal role of heavy metals, PAH's and halogenated compounds was suspected for mortality from renal failure in Massa Carrara, Piombino, Orbetello, "Basso bacino del fiume Chienti" and Sulcis-Iglesiente-Guspinese. In Trento-Nord, Grado and Marano, and "Basso bacino del fiume Chienti" increases in neurological diseases, for which an etiological role of lead, mercury and organohalogenated solvents is possible, were reported. The increase for non-Hodgkin lymphomas in Brescia was associated with the widespread PCB pollution. Mortality for causes of death with a priori Sufficient or Limited evidence of association with the environmental exposure exceeds the expected figures, with a SMR of 115.8% for men (90% IC 114.4-117.2; 2 439 extra deaths) and 114.4% for women (90% CI 112.4-116.5; 1 069 extra deaths). These excesses are also observed when analysis is extended to all the causes of death (i.e. with no restriction to the ones with a priori Sufficient or Limited evidence): for a total of 403 692 deaths (both men and women), an excess of 9 969 deaths is observed, with an average of about 1 200 extra deaths per year. Most of these excesses are observed in IPSs located in Southern and Central Italy. The procedures and results of the evidence evaluation are presented in a 2010 Supplement of Epidemiology & Prevention devoted to SENTIERI.

[Effect of misclassification of the place of residence on incidence estimates: the example of child cancer in a local health authority in Rome, Italy]. / Misclassificazione della residenza nelle schede di Dimissione Ospedaliera ed effetti sulle stime di incidenza: l'esempio dei tumori in età pediatrica nella ASL RMD.

A study on cancer of the lymphatic and hematopoietic tissue among residents aged 0-14 years was conducted by the Local Health Unit RMD (Rome, Italy; period 2003-09; codes of the International Classification of Diseases, Ninth Revision, Clinical Modification: 200-208). Age and gender Standardized Mortality and Hospitalization Ratios were computed in order to compare observed and expected cases, using municipal rates as reference. Place of residence at the time of admission, as recorded in the Hospital Registry, was compared with the information recorded in the Municipal Registers and the correlation between the two sources was calculated by Cohen's Kappa. No mortality nor morbidity excesses were observed in the study area. Although 14% of children were not confirmed as being resident at the time of admission, the Cohen's Kappa indicates a strong correlation between the Municipal Registry and the Hospital Registry (84%). The analyses restricted to children with ascertained residence did not yield different results. For those whose residence was not confirmed, the mismatch of information between the Municipality Registry and the Hospital Registry needs to be clarified.

Adhesiveness of dental resin-based restorative materials investigated with atomic force microscopy.

This study aimed to show that the polymerization contraction of dental methacrylate-based materials, when used as adhesives on hard substrate, produces voids at the material-substrate interface. This phenomenology is closely related with the nanoleakage and the sealing ability of these materials. One prime/bond system, three restorative composite resins, and one orthodontic bonding system were cured by using mirror-like glass slides as a compliance-free reference substrate. The adhesive surface was analyzed by atomic force microscopy, and the polymerization contraction of bulk material was tested by laser beam-scanning method. Nanoperiodic structure of three-dimensional (3D) images, section analysis, and roughness characteristics (R(a) and R(z)) indicated that polymerization contraction produced voids at the interface. When the adhesive surface was exposed to oral simulating fluids (water, ethanol, and lactic acid solutions), hydrolytic degradation involved some hundreds of nanometers in depth. In visible light-cured (VLC) materials, the interface porosity decreased when an irradiation pause ( approximately 2 min) was carried out during gelation.

Porosity in composite resins.

Porosity in the pastes of composite resins before and after polymerization has been measured. The influence of the diameter of the extrusion orifices and the spatulation of the chemically-activated pastes has been evaluated. Considering that thin layers of these materials are transparent, the investigation has been carried out mainly by analysis of approximately 300 microns or lower thickness layers, that are still transparent under the microscope. Generally, there was a significant dependence of the porosity on the orifice diameter. However, this rule showed some exceptions. As far as the chemically-activated pastes were concerned, in all examined cases the number of small size (10-40 microns) porosities increased after spatulation.

Polymerization shrinkage of microfilled composites determined by laser beam scanning.

An He-Ne scanning laser beam was used to measure for the first time the linear shrinkage of light-cured microfilled composites. A low-power beam, which has a wavelength (632.8 nm) different from the polymerization wavelength (approximately 450 nm), was used. In these conditions no shrinkage is induced by the laser light. This method of measurement makes it possible to analyse small samples with a very low error (1.1 microns). Five different materials were tested using 10-20 mg specimens, and the shrinkage process was examined in detail over 2 days. All these materials showed shrinkage of more than 50% of their original length after 1 min of irradiation and approximately 99% of the total shrinkage occurred 4 h after irradiation.

Study of dental materials by laser beam scanning.

Several properties of dental materials are characterized by dimensional variation induced by different parameters. In this work a non-contact method used for measurement of linear dimensional variation is shown. The accuracy is +/- 1 micron for sample length ranging from 1 to 70 mm.

Structure of dental glass-ionomer cements by confocal fluorescence microscopy and stereomicroscopy.

The microstructure of four cements, setting by different mechanisms (acid-base, dual cure, triple cure), was studied. The porosity of unpolymerized materials was detected by stereomicroscopy. After polymerization and storage in water or lactic acid solution, the porosity, filler distribution and gel layer, which was formed at the filler/matrix interface of polymerized materials, were examined by confocal laser microscopy. For this purpose, the specimens were treated with fluorescent dye solution before the test. The results showed that hydrolytic degradation (pH 7) mainly involved the resin matrix, and the acid erosion (pH 3.5) involved the gel layer too. As regards the filler, materials with different setting mechanism released the glass particles in different times. The loss of the filler particles occurred quicker in acid-base setting cements, and slower in triple-cured material.

Polymerization of dental composite resins using plasma light.

Five visible light-cured composite resins used as restoration or adhesive materials in dentistry, were irradiated with high energy plasma light (1300 mW/cm2), and contraction, rate of contraction, irradiation-induced temperature were analysed. A comparison was carried out with the same materials irradiated with a conventional halogen light (400 mW/cm2). The exposure to the photoactivating lights was either continuously or sequentially in three or more intervals with 10 min between intervals. Comparing the lengths of exposure of both lights, which induced the same contraction in a given material, it was found that the exposure length to the plasma light was greatly reduced, when compared with the exposure length of the halogen light (1:10). Frequently, the final contraction of plasma-irradiated materials was lower, whereas the rate of contraction, as indicated by the linear dimensional variation curves obtained by laser beam scanning method, did not show significant differences between the two lights. The temperature increase induced by plasma light on the material did not exceed the temperature induced by conventional light.

The effect of zinc and vitamin A supplementation on immune response in an older population.

OBJECTIVE: To determine if either supplemental vitamin A, zinc, or both increases cell-mediated immune response in an older population. DESIGN: A double-blind, randomized, controlled trial of supplementation with vitamin A and zinc. SETTING: Casa Di Riposo Roma III, a public home for older people in Rome, Italy. SUBJECTS: The health and nutritional status of 178 residents were evaluated. One hundred thirty-six residents agreed to participate in the trial and were randomized into four treatment groups, and 118 of these residents completed the trial. INTERVENTION: The four treatments consisted of: (1) Vitamin A (800 micrograms retinol palmitate); (2) Zinc (25 mg as zinc sulfate); (3) Vitamin A and Zinc (800 micrograms retinol palmitate and 25 mg as zinc sulfate); (4) Placebo capsules containing starch. MAIN OUTCOME MEASUREMENTS: Immune tests-counts of leucocytes, lymphocytes, T-cell subsets, and lymphocyte proliferative response to mitogens-were measured before and after supplementation. RESULTS: Zinc increased the number of CD4 + DR + T-cells (P = .016) and cytotoxic T-lymphocytes (P = .005). Subjects treated with vitamin A experienced a reduction in the number of CD3 + T-cells (P = .012) and CD4 + T-cells (P = .012). CONCLUSIONS: These data indicate that zinc supplementation improved cell-mediated immune response, whereas vitamin A had a deleterious effect in this older population. Further research is needed to clarify the clinical significance of these findings.

Determinants of tuberculosis in an immigrant population in Rome: a case-control study.

SETTING: An out-patient clinic for immigrants in Rome, Italy. OBJECTIVE: To determine risk factors for tuberculosis among immigrants in Italy. DESIGN: Case-control study. Cases comprised 44 individuals aged 15-55 years who had a first diagnosis of tuberculosis between 1989 and 1994 at the clinic. Controls comprised 264 individuals randomly recruited among immigrants who attended the clinic for other reasons within seven days before or after the case was diagnosed. Subject information included country of origin, date of first arrival in Italy, level of education, knowledge of the Italian language, and legal resident status. RESULTS: An increase of tuberculosis risk was observed with increasing tuberculosis incidence in the country of origin. Multivariate analysis showed an increased risk for those coming from Central and South America (odds ratio [OR] 5.5; 95% confidence interval [CI] 1.6-18.7). The adjusted odds ratio by time since entry in Italy increased during the second year of residence (OR 2.8; 95% CI 1.1-7.0), but decreased after that period. A trend toward increasing risk with decreasing educational level was observed. CONCLUSIONS: These results demonstrate the need for a public health policy in Italy for tuberculosis control among immigrants which includes screening, prophylaxis and treatment.

The possible role of hematin and Mössbauer effect in the inhibition of osteosarcoma cell proliferation.

Hematin, like many hematoporphyrines and porphyrin derivatives, shows a higher affinity and preferential localization for tumor cells. These properties are particularly interesting in view of their possible applications in tumor treatment. The aim of the present work was to test the capability of porphyrines and resonant low energy gamma rays to produce some kind of selective inhibition of tumor cell proliferation. We investigated the role of hematin to potentiate the killing capability of 14.4 keV resonant gamma rays from a 57Co Mössbauer source. Human osteosarcoma cell cultures "MG-63" were incubated in the presence or absence of hematin (10(-4) to 10(-5) M) for 24 hours. They were successively irradiated for 4 hours with 14.4 keV gamma rays from a 3.7 GBq 57Co source. The combined effects of hematin and resonant gamma radiation were then examined and tested statistically for significance. Different degrees of growth inhibition were observed when hematin alone, radiation alone, and hematin plus gamma rays were administered to the cultures. Hematin, at the concentrations of 10(-4) M is capable of inhibiting tumor cells growth. While no significant effect is attributable to irradiation alone, hematin plus irradiation show a larger inhibition than that expected for purely additive effects.

Zinc supplementation and plasma lipid peroxides in an elderly population.

OBJECTIVE: To determine the effect of vitamin A, zinc or both on plasma lipid peroxides in a healthy elderly population. DESIGN: Double-blind randomized controlled trial supplementation of vitamin A and zinc. SETTING: Public home for elderly people, in Rome, Italy. SUBJECTS: A total of 178 residents of a Public home for elderly people were evaluated regarding health and nutritional status. 136 gave a written consensus to participate in the trial and were randomized in four groups of treatment. 118 elderly completed the trial. INTERVENTIONS: Three months supplementation of the following treatments: (1) vitamin A (800 micrograms retinol palmitate); (2) zinc (25 mg zinc as sulphate); (3) vitamin A and zinc (800 micrograms retinol palmitate and 25 mg zinc as sulphate); (4) placebo (starch containing capsules). MAIN OUTCOME MEASURES: Plasma lipid peroxides (TBA-RS) were measured before and after supplementation. RESULTS: Zinc supplementation was associated with a decrease in plasma lipid peroxides (beta = -0.19; 95% confidence levels: -0.37, -0.002; p-value = 0.05) after adjusting for sex, smoking habits, baseline plasma lipid peroxides and vitamin A plasma levels. CONCLUSIONS: Zinc supplementation decreased plasma lipid peroxides while vitamin A had no effect in this elderly population. Adequate zinc intake or supplementation could play an important role in the prevention and/ or modulation of diseases in the elderly people.

Occupational and environmental exposures and lung cancer in an industrialised area in Italy.

AIMS: To investigate the effects of occupational exposures and residence near to industrial sites on lung cancer mortality in an area in Italy. METHODS: 234 cases of lung cancer and 729 controls matched by sex, age, and date of death were enrolled. Environmental exposure was evaluated using historical residence data. A geographical information system was used to compute distances from residence to pollution source (cement factory, power plants, harbour) and an average distance was computed for each subject. Odds ratios (OR) and 95% confidence intervals (CI) in a logistic regression model were used to estimate the relative risk of lung cancer associated with the risk factors (smoking habits and occupational exposure) collected by questionnaire; ORs for distances from pollution sources and from city centre were computed, adjusting for smoking habits, education, and occupation. RESULTS: Smoking habits (< or =10 cigarettes/day, OR = 2.28; 11-20, OR = 4.64; >20, OR = 6.61) and occupational exposure to asbestos (OR = 3.50) were significantly associated with lung cancer risk. Reported traffic level of area of residence and residence near the four sources were not associated with increased risk of lung cancer. There was a significantly increased risk for those residing outside the city centre, in the southern outskirts (OR = 1.51). CONCLUSIONS: The increased lung cancer risk observed in the area can partly be explained by occupational exposures. The increased risk in the outskirts of the city is consistent with the results of dispersion models that indicate high levels of pollutant deposition in the same area.

Dimensional stability of silicone-based impression materials.

This study attempts to demonstrate that the polymerization reaction is not the only factor that affects the shrinkage of silicone-based impression materials because evaporation of the constituents also contributes to the shrinkage. These factors can be evaluated by the study of time-dependent dimensional changes. This is shown both by chemical kinetics and by experimental testing of condensation and addition polymerizing impression materials with different viscosities. Comparison of the different materials shows that the two contributions, polymerization shrinkage, and evaporation shrinkage, can be assessed separately by analysis of the time-dependent shrinkage diagrams. The instability due to the polymerization reaction is complete after a few hours, but the contribution of the constituent evaporation, if present, can have a significant long-term role.