Survey on the usage of therapeutic erythrocytapheresis in transfusion services in Italy for the treatment of polycythemia vera, secondary erythrocytosis and hemochromatosis.

INTRODUCTION: Erythrocytapheresis, an apheresis treatment which selectively removes red blood cells, is an alternative to therapeutic phlebotomy, over which it has several advantages. Actually there is a high degree of variability in the use of this treatment. This prompted SIdEM (Italian Society of Hemapheresis and Cell Manipulation) to conduct a survey on the use of erythrocytapheresis in the Italian Transfusion Services. The purpose is to monitor this activity in the treatment of Polycythemia Vera (pv), secondary erythrocytosis and hemochromatosis. MATERIALS AND METHODS: A data collection file was sent to the SIdEM regional delegates who, in turn, involved the Transfusion Centers in the areas they cover. The data collected were processed on a Microsoft Excel spreadsheet. RESULTS: 75 centers from 14 Italian regions responded to the Survey: 36 centers (48 %) use erythrocytapheresis (35 centers perform therapeutic apheresis and 1 center only donor apheresis), 39 centers (52 %) do not (15 centers perform therapeutic apheresis, 18 centers only donor apheresis and 6 centers do not perform either therapeutic apheresis or donor apheresis). Although most centers have a substantially uniform attitude concerning the indications for which erythrocytapheresis is used, the survey shows that there are still differences more evident in the treatment of secondary erythrocytosis than in the treatment of pv or hemochromatosis. CONCLUSIONS: This survey has been useful to document the current Italian reality and to raise awareness about the need for improvement in optimizing and standardizing the use of a therapy with a great potential to exploit properly.

Predictability of a New Orthodontic Extrusion Technique for Implant Site Development: A Retrospective Consecutive Case-Series Study.

In clinical daily practice, there are situations in which implant sites have vertical and/or horizontal bone defects and often we must improve their morphology and dimensions before fixture insertion. It is crucial to carefully evaluate the surgical site as regards the characteristics of both hard and soft tissues. The orthodontic extrusion technique can be used for nonsurgical augmentation of the implant site as an alternative to traditional regenerative/reparative surgical therapies. The orthodontic extrusion is based on a biological mechanism that uses the portion of periodontal ligament, still present on the root before the tooth extraction, for the increase of hard and soft tissues. In the literature, there is no evidence of common guidelines for this technique but only tips based on personal experience and/or previous studies. The aim of this study was to investigate and to validate the reliability of a new orthodontic extrusion technique (MF Extrusion Technique, by Dr. Mauro Fadda) by means of a retrospective consecutive case-series study. After we have done a review of the literature, we evaluated the X-rays of twelve consecutively treated patients before the orthodontic extrusion (T0) and after the stabilization period (T1), in order to quantify, by two different measurements, area and linear, the bone gain obtained by the application of the new technique. All the patients examined showed a significant increase in bone areas with an average value of 31.575 mm2. The linear bone gain had an average value of 4.63 mm. Data collected were statistically analysed by the Wilcoxon signed-rank test. The results obtained both from area and linear measurements at T0 and at T1 times showed that there was a statistically significant bone gain with p < 0.01.

Secondary desmoplastic fibroma-like tissue changes in mandibular fibrous dysplasia: clinicopathological and molecular study of a case.

Fibrous dysplasia may show locally aggressive behaviour reflecting secondary intralesional changes, extension to soft tissue, or malignant transformation. We report the case of a patient with polyostotic fibrous dysplasia who had a giant mandibular lesion consisting of histologically typical, genotypically-confirmed, fibrous dysplasia merged with a fibrotic and hypocellular desmoplastic fibroma-like tissue in which the same Gsα-R201H mutation was detected. The occurrence of the same mutation in both the fibrous dysplasia and areas of desmoplastic fibroma suggests that the fibroma-like tissue reflects an unusual secondary tissue change within an otherwise typical fibrous dysplasia. To the best of our knowledge, only four cases of fibrous dysplasia with desmoplastic fibroma-like tissue changes have been reported.

Our experience in the surgical management of craniofacial fibrous dysplasia: what has changed in the last 10 years?

The mainstay of treatment of craniofacial dysplasia (CFD) remains surgery once clinical observation has been excluded. Nevertheless, disagreement remains about the type of surgical intervention (remodelling versus radical resection). The aim of this paper is to present our experience until 2013 comparing CFD management between 1980 and 2002 and between 2003 and 2013 and to propose our surgical algorithm. From January 2003 to December 2013, 41 new patients (18 males and 23 females) with histologically demonstrated CFD presented to our Department. Data were compared with those of 95 patients observed and/or treated between 1980 and 2002. Considering the last period, we noted that observation (26/41 patients) was the most used method; radical resection was performed in most cases (8/15 patients), but in proportion the numbers of patients undergoing bone shaving has increased (6% between 1980 and 2002 vs 15% between 2003 and 2013), while a decrease in the number of patients undergoing excision was seen (63% between 1980 and 2002 vs. 19% between 2003 and 2013). On this basis, we believe that radical resection is the only technique to obtain resolution of fibrous dysplasia. Wait-and-see is indicated in case of stable lesions. Reconstructive techniques allow obtaining adequate aesthetical and functional results; nevertheless, in most cases adjunctive surgical refinements are required and recovery time is higher than with surgical shaving, so that most patients prefer to perform remodelling. Nevertheless, in case of aggressive lesions radical resection is mandatory, except in paediatric patients with residual large defects in which it can be acceptable to try to resolve symptoms via bone shaving, reserving more aggressive treatments in case of relapse or after skeletal maturity.

Self-reported adherence to diet and preferences towards type of meal plan in patient with type 2 diabetes mellitus. A cross-sectional study.

BACKGROUND AND AIMS: Few studies have evaluated the attitudes of patients with type 2 diabetes mellitus (T2DM) towards the given dietary plans. In this study, we aimed to evaluate: i) the self-reported adherence of T2DM patients to the prescribed diets; ii) the use of other types of diet schemes; iii) the patients' preferences towards the type of meal plans. METHODS AND RESULTS: A 16 multiple-choice items questionnaire was administered to 500 T2DM patients; 71.2% (356/500) of them had the perception of having received a dietary plan; only 163/356 declared to be fully adherent. The latter had lower BMI (25.8 ± 4.5 vs 29.1 ± 4.5 kg/m2, p < 0.001) than patients who were partly adherent. Among patients not following the given diet, 61.8% was eating in accordance to a self-made diet and 20.9% did not follow any diet. Only a few patients (2.4%) had tried a popular diet/commercial program. Most patients preferred either a "sufficiently free" (201/500) or a "free" (218/500) scheme. The use of supplements attracted younger, obese individuals, with higher education, and most managers. In a multinomial regression model, age and diabetes duration were inversely associated with the choice of a "rigid" scheme, diabetes duration and glycated hemoglobin levels were inversely correlated with a "free" diet choice, obesity was associated with a "strategic" scheme choice, while lower education (inversely) and obesity (directly) correlated with the preference for "supplement use". CONCLUSIONS: Socio-cultural/individual factors could affect attitudes and preferences of T2DM patients towards diet. These factors should be considered in order to draw an individually tailored dietary plan.

Is the timing of caloric intake associated with variation in diet-induced thermogenesis and in the metabolic pattern? A randomized cross-over study.

BACKGROUND/OBJECTIVES: Food-induced thermogenesis is generally reported to be higher in the morning, although contrasting results exist because of differences in experimental settings related to the preceding fasting, exercise, sleeping and dieting. To definitively answer to this issue, we compared the calorimetric and metabolic responses to identical meals consumed at 0800 hours and at 2000 hours by healthy volunteers, after standardized diet, physical activity, duration of fast and resting. SUBJECTS/METHODS: Twenty subjects (age range 20-35 years, body mass index=19-26 kg m(-)(2)) were enrolled to a randomized cross-over trial. They randomly received the same standard meal in the morning and, 7 days after, in the evening, or vice versa. A 30-min basal calorimetry was performed; a further 60-min calorimetry was done 120-min after the beginning of the meal. Blood samples were drawn every 30-min for 180-min. General linear models, adjusted for period and carry-over, were used to evaluate the 'morning effect', that is, the difference of morning delta (after-meal minus fasting values) minus evening delta (after-meal minus fasting values) of the variables. RESULTS: Fasting resting metabolic rate (RMR) did not change from morning to evening; after-meal RMR values were significantly higher after the morning meal (1916; 95% confidence interval (CI)=1792, 2041 vs 1756; 1648, 1863 kcal; P<0.001). RMR was significantly increased after the morning meal (90.5; 95% CI=40.4, 140.6 kcal; P<0.001), whereas differences in areas-under-the-curve for glucose (-1800; -2564,-1036 mg dl(-1) × h, P<0.001), log-insulin (-0.19; -0.30,-0.07 µU ml(-1) × h; P=0.001) and fatty free acid concentrations (-16.1;-30.0,-2.09 mmol l(-1) × h; P=0.024) were significantly lower. Delayed and larger increases in glucose and insulin concentrations were found after the evening meals. CONCLUSIONS: The same meal consumed in the evening determined a lower RMR, and increased glycemic/insulinemic responses, suggesting circadian variations in the energy expenditure and metabolic pattern of healthy individuals. The timing of meals should probably be considered when nutritional recommendations are given.

Use of PCR-restriction fragment length polymorphism analysis for identification of yeast species isolated from bovine intramammary infection.

This study reports a rapid PCR-based technique using a one-enzyme RFLP for discrimination of yeasts isolated from bovine clinical and subclinical mastitis milk samples. We analyzed a total of 1,486 milk samples collected over 1 yr in south Sardinia and northern Italy, and 142 yeast strains were preliminarily grouped based on their cultural morphology and physiological characteristics. Assimilation tests were conducted using the identification kit API ID 32C and APILAB Plus software (bioMérieux, Marcy l'Etoile, France). For PCR-RFLP analysis, the 18S-ITS1-5.8S ribosomal(r)DNA region was amplified and then digested with HaeIII, and dendrogram analysis of RFLP fragments was carried out. Furthermore, within each of the groups identified by the API or PCR-RFLP methods, the identification of isolates was confirmed by sequencing of the D1/D2 region using an ABI Prism 310 automatic sequencer (Applied Biosystems, Foster City, CA). The combined phenotypic and molecular approach enabled the identification of 17 yeast species belonging to the genera Candida (47.9%), Cryptococcus (21.1%), Trichosporon (19.7%), Geotrichum (7.1%), and Rhodotorula (4.2%). All Candida species were correctly identified by the API test and their identification confirmed by sequencing. All strains identified with the API system as Geotrichum candidum, Cryptococcus uniguttulatus, and Rhodotorula glutinis also produced characteristic restriction patterns and were confirmed as Galactomyces geotrichum (a teleomorph of G. candidum), Filobasidium uniguttulatum (teleomorph of Crypt. uniguttulatus), and R. glutinis, respectively, by D1/D2 rDNA sequencing. With regard to the genus Trichosporon, preliminary identification by API was problematic, whereas the RFLP technique used in this study gave characteristic restriction profiles for each species. Moreover, sequencing of the D1/D2 region allowed not only successful identification of Trichosporon gracile where API could not, but also correct identification of misidentified isolates. In conclusion, the 18S-ITS1-5.8S region appears to be useful in detecting genetic variability among yeast species, which is valuable for taxonomic purposes and for species identification. We have established an RFLP database for yeast species identified in milk samples using the software GelCompar II and the RFLP database constitutes an initial method for veterinary yeast identification.

Safety of hematopoietic stem cell donation in glucose 6 phosphate dehydrogenase-deficient donors.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common RBC enzymatic disorder in humans capable of producing hemolytic events. Recently, concern has been raised about using G6PD-deficienct subjects as hemopoietic stem cell (HSC) donors. In a 10-year period, 101 consecutive HSC donors were submitted to donation procedures for transplantation inside their families in our Center. All donors were tested for G6PD and 19 (19%) turned out to be G6PD-deficient. The donors' safety and the effectiveness of these transplant outcomes were compared with those of the remaining 82 donors. No difference could be observed in any safety parameter between the two groups. No difference was recorded in donors' complications rates, in HSC production, in quantity of growth factor required, in Hb early drop or in Hb recovery. No difference was found in transplant outcome. From this retrospective analysis, we conclude that a G6PD-deficient but otherwise healthy volunteer can be selected as a HSC donor.

Looking for Candida nivariensis and C. bracarensis among a large Italian collection of C. glabrata isolates: results of the FIMUA working group.

Two recently described pathogenic Candida species, C. nivariensis and C. bracarensis, share many phenotypic characteristics with C. glabrata and are easily misidentified as such. The aim of this study was to determine the occurrence of these cryptic species in Italy. One thousand yeast isolates collected in 14 Italian regions and identified as C. glabrata by phenotypic and biochemical methods were included in this study: 928 were screened on CHROMagar and 72 were analysed by a multiplex PCR. None of these cryptic species was identified despite the nationwide distribution and the variety of biological origin of the isolates.

Two clinical cases of prosthetical rehabilitation after a tumor of the upper maxilla.

The most frequent type of treatment for patients diagnosed with a malignant neoplasia of the oral cavity is surgical removal of the tumor. As a result of the resection performed, it is difficult to obtain satisfactory results in the oral rehabilitation of these patients. When possible, fixed prostheses are the best option, because they guarantee stability, but they should be made so that the operator can remove them periodically to check the health of the oral tissues underneath and to intercept any relapse of the tumor. This study analyses two cases of patients surgically treated for a tumor of the upper maxilla at the Oral and Maxillo-Facial Surgery of Sapienza University, in Rome. In the first case the surgical site was covered with local flaps, and the patient was rehabilitated with an implant-supported removable prosthesis. In the second case the maxilla was reconstructed with a fibula vascularized free flap, and the patient was rehabilitated with an implant-supported prosthesis screwed to a titanium bar solidarizing the implants. Therefore, this prosthesis was fixed, but could be removed by the dentist. The different approach to these two cases was influenced by the different anatomic situations after the reconstruction. It is important for the dentist to approach these patients knowing the kind of surgery they received because this aspect will influence rehabilitative choices. Rehabilitation should be planned, when possible, before surgical treatment, in order to cooperate with the maxillo-facial surgeon in choosing the most appropriate restorative treatment.