Differences in clinicopathological features and distribution of risk factors in Italian melanoma patients.

BACKGROUND: No studies are available in the literature on the distribution of different melanoma features and risk factors in the Italian geographical areas. OBJECTIVE: To identify the differences in clinical-pathological features of melanoma, the distribution of risk factors and sun exposure in various Italian macro-areas. METHODS: Multicentric-observational study involving 1,472 melanoma cases (713 north, 345 centre, 414 south) from 26 referral centres belonging to the Italian Multidisciplinary Group for Melanoma. RESULTS: Melanoma patients in northern regions are younger, with thinner melanoma, multiple primaries, lower-intermediate phototype and higher counts of naevi with respect to southern patients; detection of a primary was mostly connected with a physician examination, while relatives were more involved in the south. Northern patients reported a more frequent use of sunbeds and occurrence of sunburns before melanoma despite sunscreen use and a lower sun exposure during the central hours of the day. CONCLUSIONS: The understanding of differences in risk factors distribution could represent the basis for tailored prevention programmes.

Validation of an original questionnaire for patients with psoriatic arthritis: the Psoriatic Arthritis Impact Profile (PAIP).

BACKGROUND AND AIMS: There is a wide evidence that Psoriatic Arthritis (PsA) as well as Psoriasis (Ps) lead to significant health problems and interfere with the patient quality of life (QoL). Even though a validated questionnaire for Ps is available, no questionnaire for PsA is currently present in literature. The aim of our work has been to confirm the efficacy of our original questionnaire as well as to validate it, through the comparison with other existing recognised and accepted questionnaires, such as MOF-SF36, HAQ, McGill Pain Questionnaire, and Zeung Self-Rating Depression and Anxiety Scales. MATERIALS AND METHODS: We have realized a questionnaire for PsA (Psoriatic Arthritis Impact Questionnaire, PAIP), in terms of psychological and rheumatological evaluation, QoL, social and economic assets. RESULTS: The statistical comparisons between PAIP and the accepted questionnaires (see above) confirm that PAIP is widely validated and represents a useful tool suitable for clinical evaluation and management of patients with PsA. CONCLUSIONS: The indexes of the correlation among the different parts of PAIP and the other questionnaires have shown positive correlations. Moreover, PAIP presents a dedicated unit for the economical and therapeutic parameters, The short time for compilation (15 minutes), the easy of comprehension of the questions, and - above all - the validation of PAIP, make our questionnaire a useful tool, suitable for the clinical management of the patients with PsA.

Risk of multiple primary cancers following melanoma and non-melanoma skin cancer.

BACKGROUND: The relationship between cutaneous malignancies and successive primary cancers has been studied since several years, but it still remains controversial. OBJECTIVE: The aim of this study was to evaluate the excess risk of multiple primary cancer among the population of Umbria, Italy, that survived a skin cancer. METHODS: The data registered in the Umbrian Population Cancer Registry from 1994 to 2006 were collected, recorded, and analysed in accordance to the standard methods recommended for cancer registries. Among skin cancer patients, those with multiple cutaneous and non-cutaneous cancers were selected. Only sites with a frequency of more than five cases were considered. The expected number of cases was obtained from indirect standardization with regional incidence rates in several sites that incurred in the overall period. The significance of the observed/expected ratios and the corresponding 95% CI were based on the Poisson distribution. RESULTS: In men, a significant standardized incidence ratio (SIR) was found for melanoma (2.21), non-melanoma skin cancers (1.86), Hodgkin's (4.95) and non-Hodgkin lymphoma (1.82), and tongue/mouth cancer (2.47). In women, melanoma, non-melanoma skin and breast cancer showed a significant high SIRs (4.13, 1.55 and 1.28 respectively). All other cancers showed a non-significant SIR. Considering all sites combined and all sites except skin cancers, the analysis showed a significant excess in men, whereas a significant risk was observed in women only when also skin cancers were considered. CONCLUSIONS: Data from the whole of Umbrian population revealed that skin cancer patients experience marked excess risk of further primary cancers. The main risk in both genders is skin melanoma and other skin cancers. The excess of lymphomas and tongue/mouth cancers is also significant in men, and breast cancer in women. These observations prompt us to include a screening for these cancers in the follow-up of our patients surviving a skin malignancy.

Localized Wegener's granulomatosis.

BACKGROUND: Wegener's granulomatosis (WG) is a rare granulomatous necrotizing vasculitis of small and medium vessels which has predilection for upper airways, lungs and kidney. However, any other organ, including the skin and oral cavity, can be involved. Although mucocutaneous lesions are relatively common, they have only rarely been reported as localized manifestation of the disease. OBJECTIVES: Our aim was to evaluate the type and sites of skin and mucosal lesions, clinical course and response to treatment, histologic features and laboratory findings in localized WG. METHODS: The medical records of three patients (two women and one man) with localized WG followed up at our hospitals for a mean time of 10 years were studied. RESULTS: All patients presented with facial plaques infiltrating the nasal and palatal mucosae and cartilages and, in one case, perforating the palatal bone. Anti-neutrophil cytoplasmic antibodies, which are the marker for multisystem WG, were negative. The disease, refractory to various immunosuppressants, responded well, albeit incompletely, to prednisone plus cyclophosphamide. LIMITATIONS: The limited number of patients is counterbalanced by the rarity of the disease. CONCLUSIONS: Our cases may represent a rare distinctive subset of WG limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction.

Olive oil mill effluents as a feedstock for production of biodegradable polymers.

The aim of the present paper was to study the feasibility of using olive oil mill effluents (OMEs) as a substrate in biodegradable polymer production. OMEs were anaerobically fermented to obtain volatile fatty acids (VFAs), which are the most highly used substrate for polyhydroxyalkanotes (PHAs) production. The anaerobic fermentation step was studied both without pretreatment and with different pretreatments (i.e., centrifugation, bentonite addition, and bentonite addition followed by centrifugation) and at various concentrations (28.5, 36.7 and 70.4 g CODL(-1)). During fermentation, VFA concentration was determined (7-16 g CODL(-1)) as well as the corresponding yield with respect to initial COD (22-44%). At all initial concentrations, centrifugation pretreatment (with or without previous addition of bentonite) significantly increased the final VFA concentration and yield, whereas the addition of bentonite alone had no influence. Moreover, centrifugation pretreatment led to a different acid distribution, which affected the hydroxyvalerate (HV) content within the obtained copolymer poly beta-(hydroxybutyrate-hydroxyvalerate) [P(HB-HV)]. OMEs were tested for PHA production by using a mixed culture from an aerobic SBR. Centrifuged OMEs, both with or without fermentation, were tested. PHAs were produced from both matrices, but with fermented OMEs PHA production was much higher, because of the higher VFA concentration. The initial specific rate of PHA production obtained with fermented OMEs was approximately 420 mg COD g COD(-1)h(-1) and the maximum HV content within the copolymer was about 11% (on a molar basis). The HV monomer was produced only until propionic acid remained present in the medium.

Competitive transport of cadmium and lead through a natural porous medium: influence of the solid/liquid interface processes.

Contaminated groundwater typically contains different metal contaminants which may compete with each other for the same adsorption sites. Understanding the fate of these micro-pollutants is of primary importance for the assessment of the risk associated with their dispersion in the environment and for the evaluation of the most appropriate remediation technology. In this regard, column techniques can be considered as useful tools both to perform transport experiments and to obtain equilibrium adsorption data without any perturbation of the actual solid/liquid interface. Cd and Pb monocomponent step column experiments were used to obtain adsorption isotherms on a natural aquifer material. A General Composite approach was used to define the equilibrium adsorption model characterized by two types of sites (ion-exchange and surface complexation sites). Coupling the adsorption model with the Advection-Dispersion equation (by IMPACT code) allowed us to well represent the monocomponent step experiments. The model was successfully used to predict the competitive Cd and Pb transport behaviour. Cd peaks of concentration due to Pb competition were experimentally observed and simulated by the model. This behaviour can be described only by an accurate modelling of the interaction and cannot be predicted by simple isotherms (such as Langmuir or Freundlich type).

Dermatomyositis in 132 patients with different clinical subtypes: cutaneous signs, constitutional symptoms and circulating antibodies.

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottron's papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynaud's phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.

Removal of molecular weight fractions of COD and phenolic compounds in an integrated treatment of olive oil mill effluents.

Previous works (Beccari et al. 1999b; Beccari et al. 2001a; Beccari et al. 2001b) on the anaerobic treatment of olive oil mill effluents (OME) have shown: (a) a pre-treatment based on the addition of Ca(OH)2 and bentonite was able to remove lipids (i.e. the most inhibiting substances present in OME) almost quantitatively; (b) the mixture OME-Ca(OH)2-bentonite, fed to a methanogenic reactor without providing an intermediate phase separation, gave way to high biogas production even at very low dilution ratios; (c) the effluent from the methanogenic reactor still contained significant concentrations of residual phenolic compounds (i.e. the most biorecalcitrant substances present in OME). Consequently, this paper was aimed at evaluating the fate of the phenolic fractions with different molecular weights during the sequence of operations (adsorption on bentonite, methanogenic digestion, activated sludge post-treatment). The results show that a very high percentage (above 80%) of the phenolic fraction below 500 D is removed by the methanogenic process whereas the phenolic fractions above 1,000 D are significantly adsorbed on bentonite; the 8-day activated sludge post-treatment allows an additional removal of about 40% of total filtered phenolic compounds. The complete sequence of treatments was able to remove more than the 96% of the phenolic fraction below 500 D (i.e. the most toxic fraction towards plant germination). Preliminary respirometric tests show low level of inhibition exerted by the effluent from the methanogenic reactor on aerobic activated sludges taken from full-scale municipal wastewater plants.

Enhancement of anaerobic treatability of olive oil mill effluents by addition of Ca(OH)2 and bentonite without intermediate solid/liquid separation.

Previous work on the anaerobic treatment of olive oil mill effluents (OME) have shown: (a) lipids, even if more easily degraded than phenols, were potentially capable of inhibiting methanogenesis more strongly; (b) a pretreatment based on addition of Ca(OH)2 and bentonite removed lipids almost quantitatively; (c) preliminary biotreatability tests performed on the pretreated OME showed high bioconversion into methane at very low dilutions ratios, especially when the mixture (OME, Ca(OH)2 and bentonite) was fed to the biological treatment without providing an intermediate phase separation. This paper was directed towards two main aims: (a) to optimize pretreatment: the best results in terms of methane production were obtained by addition of Ca(OH)2 up to pH 6.5 and of 10 g L-1 of bentonite; (b) to evaluate the enhancement of anaerobic treatability of OME pretreated under optimized conditions in a lab-scale continuous methanogenic reactor fed with the substrate without intermediate solid/liquid separation: very satisfactory performances were obtained (at an organic load of 8.2 kg COD m-3 d-1 and at a dilution ratio of 1:1.5 total COD removal was 91%, biogas production was 0.80 g CH4 (as COD)/g tot. COD, lipids removal was 98%, phenols removal was 63%). The results confirm the double role played by bentonite (adsorption of the inhibiting substances and release of the adsorbed biodegradable matter in the methanogenic reactor).

Clinical evidence for myocardial derecruitment downstream from severe stenosis: pressure-flow control interaction.

To verify the interaction between coronary pressure (CP) and blood flow (CBF) control, we studied nine candidates for angioplasty of an isolated lesion of the left anterior descending coronary artery [i.e. , percutaneous transluminal coronary angioplasty (PTCA)]. CBF (i.e., flow velocity x coronary cross-sectional area at the Doppler tip) and CP were monitored during washout of 2-5 mCi of (133)Xe after bolus injection into the left main artery before and after PTCA. Xe mean transit time (MTT) was calculated as the area under the time-activity curve, acquired by a gamma camera, divided by the dose obtained from a model fit of the Xe curve in the anterior wall. CBF response to intracoronary adenosine (2 mg) was also assessed. PTCA increased baseline CBF (from 14.5 +/- 9.4 to 20 +/- 8 ml/min, P < 0.01), coronary flow reserve (from 1.52 +/- 0.24 to 2.33 +/- 0.8, P < 0.01), and CP (from 64 +/- 9 to 100 +/- 10 mmHg, P < 0.05). MTT decreased from 89 +/- 32 to 70 +/- 19 s (P < 0.05) after PTCA; however, MTT and CBF changes were not correlated (r = -0.09, not significant). Inasmuch as MTT is the ratio of distribution volume to CBF, MTT x CBF was used as an index of perfused myocardial volume. Volume increased after PTCA from 23 +/- 18 to 56 +/- 30 ml. A direct correlation was observed between the percent increase in distal CP and percent increase in perfused volume (r = 0.91, P < 0.01). Thus low CP was not associated with exhaustion of flow reserve but, rather, with reduction of perfused myocardial volume. These data suggest that, in the presence of a severe coronary stenosis, derecruitment of vascular units occurs that is proportional to the decrease in driving pressure. Residual perfused units maintain a vasomotor tone, thus explaining the paradoxical persistence of coronary reserve.

Congenital hypomyelination neuropathy with Ser72Leu substitution in PMP22.

We describe a patient with congenital hypomyelination neuropathy. The pathological and morphometrical findings in the sural nerve biopsy were consistent with a defect of myelin formation and maintenance. Direct sequence analysis of the genomic regions coding the peripheral myelin proteins P0 and PMP22 disclosed a heterozygous missense point mutation that leads to a Ser72Leu substitution in the second transmembrane of PMP22. Codon 72 mutations of PMP22 are associated with different phenotypes encompassing the Dejerine-Sottas syndrome and including congenital hypomyelination neuropathy.

Nodular secondary syphilis.

Nodular secondary syphilis in a 23-year-old Indian man was characterized by numerous papular, nodular and plaque skin lesions, without involvement of the mucous membranes. The histopathology showed sarcoid-like granulomata with lymphocytes, histiocytes, eosinophils, plasma cells and multinucleated giant cells. The differential diagnosis included deep mycoses, leprosy, tuberculosis, sarcoidosis and lymphoma. The results of serological tests and the rapid response to penicillin indicated a correct diagnosis.

An unusual neck mass: the Warthin tumor.

The cystoadenolymphoma or Warthin's tumor is a benign lesion of the salivary glands and principally of the parotid gland. We report a case of a 62 year old white male seen at our Institute for evaluation of an asymptomatic right neck mass located 2 cm below the mandible at the anterior margin of the sternocleidomastoid muscle. The echography showed a lymphoid structure simulating a lymphoproliferative process. Histologic examination of the surgical specimen revealed a Warthin's tumor. We discuss the importance of considering Warthin's tumor in the differential diagnosis of an isolated neck mass, and report our experience over a ten year period in the surgical management of neck masses. Additionally, the literature concerning extraparotid Warthin's tumors is reviewed.

[Allergic fungal sinusitis: is this rare disease an allergy or infection?]. / Sinusite Allergica Micotica: una rara affezione allergica o infettiva?

Allergic Fungal Sinusitis (AFS) is a newly recognized form of benign, non invasive sinusitis the histopathologic features of which are similar to those of allergic bronchopulmonary aspergillosis. AFS is a rare condition. However, because treatment and prognosis vary widely, it is important that this disorder be recognized and differentiated from chronic bacterial sinusitis and other forms of fungal sinusitis. AFS does not discriminate by age although it is primarily found in young adults. AFS patients are usually atopic, often having a history of asthma and nasal polyposis. Many have suffered from the symptoms of chronic sinusitis for years while others have had multiple sinus surgery. Radiographs reveal the involvement of multiple sinuses, often with bone destruction. Laboratory findings support an allergic state with a marked increase in eosinophilia and total IgE. At times RAST testing proves positive for fungi and immediate cutaneous reactivity to fungi is also present. Histologic review of the sinus contents reveals characteristic "allergic mucin", with numerous eosinophiles, Charcot-Leyden crystals and fungal hyphae, without any fungi tissue invasion. A wide variety of fungal agents has been implicated, although the majority belong the Dematiacee family. Those patients with allergic mucin but no documented fungi are indicated as having AFS-like syndrome. The pathogenesis of AFS is uncertain. There is controversy in the literature as to what role hypersensitivity (Gell and Coombs type I and type III responses) in infection play. To date current therapeutic recommendations include complete exenteration of all allergic mucin. Adjunctive, short-term systemic steroids often prove useful and nasal steroid spray should be continued for long term. Systemic antifungal agents are not recommended in AFS. Recurrence is common and thus close clinical, endoscopic and radiographic follow-up is important. The clinicopathologic features of one patient with AFS are reported and etiopathogenetic problems are discussed. The presented case showed a positive culture with negative immunological testing (RAST-positive and immediate cutaneous reactivity to fungal antigen), thus confirming the pathogenetic hypothesis of the saprophytic fungal growth in an atopic patient.

Scrapie strain infection in vitro induces changes in neuronal cells.

PC12 cells, in the presence of nerve growth factor (NGF), support replication of the mouse-derived scrapie strains 139A and ME7, with the former yielding 100-1000-fold higher levels of infectivity. Infectivity remained cell-associated and cells did not show any gross morphological alterations, although changes were observed by electron microscopy in the form of an increased number of lipid droplets in 139A-infected cultures. Analysis of phospholipid metabolism in 139A infected cells indicated that scrapie replication did not change the inositol phosphate levels, but did stimulate phosphoinositide synthesis. Replication was not detected in PC12 cells infected with either the hamster-derived 263K or rat-derived 139R scrapie strains. Since scrapie-infected cultures did not exhibit cell death or any gross changes, any scrapie-induced effects would probably be manifested in nonvital cellular functions. When compared to controls, infection with the 139A scrapie strain resulted in decreased activity of the cholinergic pathway-related enzymes, as well as the GABA synthetic pathway; however, the adrenergic pathway was unaffected by scrapie infection. The effects of the 139A scrapie strain on the cholinergic system appeared to be dose-dependent and were first detected prior to the detection of scrapie agent replication in these cells. No neurotransmitter-related enzymatic changes were detected in 263K- or 139R-infected PC12 cells. The enzymatic changes observed in ME7-infected PC12 cells and in Chandler agent-infected mouse neuroblastoma cells suggest that the significant changes in neurotransmitter levels in cultures exhibiting low infectivity titers must involve factors other than, but not excluding, replication of the agent. The role of additional factors is also suggested in studies of protein kinase C activity in 139A- and 139R-infected PC12 cells. These studies emphasize the value of the PC12 cell model system in examining the scrapie strain-host cell interaction and, in addition, support the concept of variation among scrapie strains.

Cholesterol granuloma of the frontal sinus. A case report.

A rare case of cholesterol granuloma of the frontal sinus is reported. The pathogenetical mechanisms and the tumour's behaviour are discussed on the basis of the patient's, the radiologic examination and the pathologist's finding's. A follow-up of three years is given.

Demonstration of scrapie strain diversity in infected PC12 cells.

Scrapie strain replication in the nerve growth factor-induced, differentiated PC12 cell culture system was examined. Differences in replication between mouse-derived agents were demonstrated, with the 139A scrapie strain yielding 100- to 1000-fold higher levels of infectivity than the ME7 scrapie strain. Replication was not detected in PC12 cells infected with either the hamster-derived 263K or rat-derived 139R scrapie strains. Studies on the neurotransmitters in infected PC12 cells demonstrated that the adrenergic pathway was unchanged but the cholinergic pathway was altered. Furthermore, the degree of alteration correlated with the level of scrapie strain replication. Comparison of infectivity titres and enzymatic changes in ME7-infected PC12 cells with those in Chandler agent-infected mouse neuroblastoma cells suggests that the significant changes in neurotransmitter levels in cultures exhibiting low titres of infectivity involve factors in addition to strain replication. The variation in the range of scrapie strain replication in PC12 cells is discussed in relationship to species barrier, cell targeting, genetic susceptibility and species strain specificity. These studies further emphasize the value of the PC12 cell model system in examining the scrapie strain-host cell interaction and in addition support the concept of variation among scrapie strains.

Alterations in neurotransmitter-related enzyme activity in scrapie-infected PC12 cells.

Enzyme activities associated with the neurotransmitter pathways in nerve growth factor-treated, 139A scrapie strain-infected PC12 cells were examined. Since these cells show no morphological alterations during the time of agent replication, any scrapie-induced effects would have to be associated with non-vital cellular functions. When compared to controls, infection with the 139A scrapie strain resulted in decreased activity of the cholinergic pathway-related enzymes, choline acetyltransferase and acetylcholinesterase. However, the adrenergic pathway was unaffected by scrapie infection as evidenced by unaltered tyrosine hydroxylase activity, the putative rate-limiting enzyme in the synthesis of catecholamines. The effects of the 139A scrapie strain on the cholinergic system appeared to be dose-dependent and were first detected prior to the detection of scrapie agent replication in these cells. Furthermore, the altered enzymic activities observed were not the result of contaminating material in the scrapie brain homogenate because similar results were obtained when partially purified scrapie preparations were used as the inoculum. These scrapie agent-induced alterations in specific neuronal properties suggest a mechanism for the clinical manifestations observed in scrapie and perhaps other related central nervous system disorders.