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The article focuses on treating mastocytosis disorders in children and adults with vaccination. Topics discussed include characterization of the disease through presence of clonal mast cells (MC) in cutaneous tissues and extracutaneous organs; risk factors related to patients with mastocytosis such as anaphylaxis with hymenoptera venom (HV); epidemiological data on frequency and severity of mastocytosis; and presence of idiopathic anaphylaxis among children's.

Systemic Mastocytosis has been long identified as a potential cause of osteoporosis; nevertheless, data regarding longitudinal variation of bone mineral density (BMD) in patients with indolent systemic mastocytosis (ISM) are missing . We studied BMD variation at lumbar spine and proximal hip after 30-month (±6 months) follow-up in a large cohort of patients (83) with ISM without osteoporosis, supplementated with vitamin D and/or calcium when needed. We also analyzed the correlation between variation of BMD, basal serum tryptase levels and bone turnover markers (BTM). Sixty-four percent of our population was male; mean age was 52.1 (±11.5) years. Vitamin D insufficiency (serum levels of 25-OH-vitamin D, 25OHD, lower than 75 nmol/L) was found in more than 70 % of patients. After a follow-up of 30 ± 6 months with only vitamin D (5000-7500 IU weekly of oral cholecalciferol) or calcium (500 mg/die) supplementation when needed, we observed 2.1 % increase in BMD at lumbar spine, with no significant changes at hip. At the end of follow-up, almost 60 % of patients showed 25OHD serum levels still lower than recommended, despite vitamin D supplementation. Reduction in BMD after follow-up significantly correlated with high C-telopeptide of type I collagen serum levels at the time of diagnosis. In patients with ISM without osteoporosis, a routinary BMD evaluation within a time <2 years is not justified, except in the presence of elevated BTM. In these patients, vitamin D supplementation is frequently needed. [ABSTRACT FROM AUTHOR]

Mastocytosis is a rare condition characterized by abnormal mast cell proliferation and a broad spectrum of manifestations, including various organs and tissues. Osteoporosis is one of the most frequent manifestations of systemic mastocytosis, particularly in adults. Osteoporosis secondary to systemic mastocytosis is a cause of unexplained low bone mineral density that should be investigated when accompanied by suspicious clinical elements. Bone involvement is often complicated by a high recurrence of fragility fractures, mainly vertebral, leading to severe disability. The mechanism of bone loss is the result of different pathways, not yet fully discovered. The main actor is the osteoclast with a relative or absolute predominance of bone resorption. Among the stimuli that drive osteoclast activity, the most important one seems to be the RANK-RANKL signaling, but also histamine and other cytokines play a significant role in the process. The central role of osteoclasts made bisphosphonates, as anti-resorptive drugs, the most rational treatment for bone involvement in systemic mastocytosis. There are a few small studies supporting this approach, with large heterogeneity of drug and administration scheme. Currently, zoledronate has the best evidence in terms of gain in bone mineral density and bone turnover suppression, two surrogate markers of anti-fracture efficacy. [ABSTRACT FROM AUTHOR]

Mastocytosis is a clonal disorder characterized by the proliferation and accumulation of mast cells ( MC) in different tissues, with a preferential localization in skin and bone marrow ( BM). The excess of MC in mastocytosis as well as the increased releasability of MC may lead to a higher frequency and severity of immediate hypersensitivity reactions. Mastocytosis in adults is associated with a history of anaphylaxis in 22-49%. Fatal anaphylaxis has been described particularly following hymenoptera stings, but also occasionally after the intake of drugs such as nonsteroidal anti-inflammatory drugs, opioids and drugs in the perioperative setting. However, data on the frequency of drug hypersensitivity in mastocytosis and vice versa are scarce and evidence for an association appears to be limited. Nevertheless, clonal MC disorders should be ruled out in cases of severe anaphylaxis: basal serum tryptase determination, physical examination for cutaneous mastocytosis lesions, and clinical characteristics of anaphylactic reaction might be useful for differential diagnosis. In this position paper, the ENDA group performed a literature search on immediate drug hypersensitivity reactions in clonal MC disorders using MEDLINE, EMBASE, and Cochrane Library, reviewed and evaluated the literature in five languages using the GRADE system for quality of evidence and strength of recommendation. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to determine how socio-demographic and clinical variables affect quality of life ( Qo L) and to assess the validity of a 20-item scale in a sample of Italian subjects with colostomy, ileostomy and multiple stomata. Method A cross-sectional multicentre survey was carried out in Italy between 2009 and 2010 in 73 stoma centres coordinated by the University of Padova. Patients aged 18 years old and above with a history of nontemporary stoma were included in the study. The Stoma Care Qo L scale was measured and validated using a Rasch model. Socio-demographic and clinical characteristics were considered in the analyses. Results Two hundred and fifty-one patients were recruited for the study; the mean age was 62 years, 58% were men, 72% had colostomy and 25% ileostomy; approximately 70% of patients had intestinal cancer requiring a stoma, 13% a complication and 10% an inflammatory disease. No significant differences were observed throughout strata in the Stoma Care Qo L scale index, except for geographical area, where subjects from south Italy showed a significantly lower index than subjects living in other parts of Italy ( P < 0.01). Colostomy and ileostomy patients reported very similar Qo L. Cronbach's alpha for the Stoma Care Qo L scale was 0.90 (95% CI 0.88-0.92). Rasch analysis supported the viability of the Stoma Care Qo L scale questionnaire and showed acceptable goodness-of-fit. Three under-fitted items were observed. Conclusion The study confirms the validity of the 20-item Stoma Care Qo L scale questionnaire as a research tool for stoma patients but the number of items could be reduced. [ABSTRACT FROM AUTHOR]

Background Previous research has focused on physician's perspectives of end-of-life ( EOL) decision making as well as patient and family EOL decision making. There is a lack of research pertaining to the EOL treatment preferences of nurses and especially nurses working in a variety of care settings. Aim The aim of this study was to compare nurses' EOL treatment preferences in Hong Kong, Ireland, Israel, Italy and the USA. Methods A comparative descriptive design was used with a convenience sample of nurses (n = 1089). A survey questionnaire using EOL hypothetical clinical case scenarios was used to collect data between June 2011 and July 2012. Results Nurses in every country consistently chose a more aggressive option for patients than for themselves or for a parent. The treatment preferences of nurses varied from country to country. Lack of knowledge of patients' wishes and duty of care were the main influencing factors on treatment preferences. Study Limitations The study was limited to the hypothetical nature of the scenarios; however, the study highlights numerous future research questions. Conclusions This study is the first to examine and compare nurses' preferred EOL treatment choices in five countries from three different continents. The findings of this study raise several important questions for healthcare researchers, for policy development, and highlight the need for further international collaboration. [ABSTRACT FROM AUTHOR]

Accessible summary The aim of the research was to examine the thoughts and feelings experienced by Italian nurses when caring for distressed and aggressive patients in acute psychiatric settings., Fear was the prevalent feeling experienced by nurses: fear of being harmed by the patient and fear of harming the patient and fear of damaging the therapeutic relationship., When managing distressed and aggressive patients, the nursing team have to share similar feelings and be emotionally in tune with each other., The paper offers clear explanations of nurses' feelings, the reasons for their fear and the importance of effective team working during a crisis., Abstract This paper reports on a qualitative study to identify Italian nurses' feelings and emotions towards the management of distressed and disturbed psychiatric patients in acute inpatient settings. Four focus groups were carried out involving 33 nurses from seven acute psychiatric services in a region of Italy. Data were analysed using content analysis. Findings highlight that fear is the prevailing feeling experienced by nurses in the management of distressed patients. This includes both fear of being physically harmed and concerns about doing harm or damage to patients. The unpredictability of aggressive behaviour from unknown patients was described as especially frightening. Known patients, namely those previously admitted to the unit, induced less fear, as their behaviour was considered more predictable. Patients with dual diagnosis were also a worry for many nurses, who considered themselves lacking in the personal relational skills needed to manage an individual in crisis. Nurses reported that they try to understand the reasons for a patient's aggressive behaviour and are empathetic to his or her suffering. When such empathy is lacking, nurses experience conflicting feelings of counter-aggressiveness. The paper concludes by emphasizing the need to increase availability of education related to patient management, in particular team working and its internal relationships. [ABSTRACT FROM AUTHOR]

Background Systemic mastocytosis ( SM) may be associated with hymenoptera allergy. In such cases, immunotherapy is a life-saving treatment, but a circumstantiated diagnosis is needed for its prescription. Patients with SM and previous reactions to stings, but with negative tests represent a diagnostic dilemma. The basophil activation test ( BAT) may be helpful in refining the diagnosis. Objective We assessed the usefulness of BAT in subpopulations of mastocytosis patients, including those with negative tests for insect allergy. Methods Within a population of patients with mastocytosis and previous stings, we studied by BAT and augmented intradermal test ( IDT) (10 µg/ml) two groups: (1) with reactions to stings and negative tests; (2) without reactions and negative tests. Basophil activation test was performed with different venoms, assessing at flow cytometry basophils' activation. Results Sixty-three patients had mastocytosis and 52 had reactions to previous hymenoptera stings. Of them, seven proved negative to diagnostic tests. In six of seven of those patients, BAT was negative with all venoms, and in one, basophils resulted activated also with the negative control. In six patients without previous reactions and negative tests, BAT was totally negative in five of six patients and weakly positive to Hornet in one. Finally, the IDT at 10 µg/ml venom produced nonspecific positive results in most cases. Conclusion In patients with mastocytosis, the negative results of standard tests are reliable, because BAT and IDT at higher concentration do not add useful information. [ABSTRACT FROM AUTHOR]

Background: The preferential association of mastocytosis with hymenoptera sting reactions is well known, but there is no data on the prevalence of clonal mast cell disorders in subjects with severe systemic reactions due to foods or drugs. Methods: Patients with food- or drug-induced severe systemic reactions, including anaphylaxis, and increased serum tryptase were studied for the presence of mastocytosis, and compared with a population of patients with hymenoptera allergy. The aetiological role of foods or drugs was assessed according to current recommendations. Systemic reactions were graded in severity according to the procedure described by Mueller. Serum tryptase was considered increased if the level was >11.4 ng/ml. Subjects with increased tryptase had dermatological evaluation and Bone marrow(BM) aspirate-biopsy, which included histology/cytology, flow cytometry and detection of KIT mutations. Results: A total of 137 subjects (57 male, mean age 42 years) were studied. Of them, 86 proved positive for drugs and 51 for foods. Overall, out of 137 patients, only nine (6.6%) had a basal tryptase >11.4 ng/ml, and only two (1.5%) were diagnosed with mastocytosis. This was clearly different from patients with hymenoptera allergy, where 13.9% had elevated tryptase and 11.1% had a clonal mast cell disorder. Conclusion: The association of clonal mast cell disorders with hymenoptera allergy seems to be more specific than that with food- or drug-induced systemic reactions. [ABSTRACT FROM AUTHOR]

Zeta-associated protein-70 (ZAP-70), mostly assessed by flow-cytometry (FC), recently emerged as reliable prognostic factor in chronic lymphocytic leukaemia (CLL) at presentation. We evaluated ZAP-70 expression in 156 CLL patients by immunohistochemistry (IHC) on formalin-fixed bone marrow (BM) biopsies at diagnosis. At presentation, 117 patients (75%) were with Binet stage A, 27 (17%) stage B and 12 (8%) stage C. Median follow-up was 61 months (range 6–242). ZAP-70 was expressed in neoplastic lymphocytes of 69 patients (44%). Concordance between ZAP-70 by IHC and ZAP-70 by FC, immunoglobulin heavy chain variable genes (IGHV) mutational status and CD38 expression was found in 41/46 (89%), 41/49 (80%) and in 60/88 (68%) tested cases, respectively. ZAP-70 expression significantly correlated with advanced Binet stage (B–C), diffuse BM infiltration, increased lactate dehydrogenase (LDH) and β2-microglobulin serum levels and lymphocyte doubling time <12 months. ZAP-70 positivity was significantly related to poorer time to progression (median 16 months vs 158 of ZAP-70-negative cases) (P<0.0001) and overall survival (median 106 months vs not reached) (P=0.0002); this correlation was confirmed at multivariate analysis. ZAP-70 expression correlated with poorer outcome also when evaluated only in the 117 stage A patients. In conclusion, immunohistological detection of ZAP-70 on formalin-fixed BM biopsies at diagnosis appears a useful methodological approach to identify patients with poor prognosis in CLL.Leukemia (2007) 21, 102–109. doi:10.1038/sj.leu.2404458; published online 2 November 2006 [ABSTRACT FROM AUTHOR]

In an international study, psychometric properties of the Care Dependency Scale (CDS) were examined by analysing data gathered in Dutch, Canadian, Italian and Norwegian nursing homes. For that purpose, from these countries a convenience sample was developed consisting of 525 patients with dementia. The English, Italian and Norwegian research instruments were translations of the original Dutch CDS. Psychometric evaluations of the CDS were carried out for each country separately as well as for the four countries combined. High alpha coefficients between 0.94 and 0.97 were calculated. Subsequent test-retest and inter-rater reliability revealed moderate to substantial Kappa values. Factor analysis resulted in a one-factor solution. The scalability of the CDS was demonstrated by means of Mokken scale analysis. One of the main outcomes of the cross-cultural comparison was that the findings in the four countries show more similarities than differences, so that the scale can be used appropriately in nursing home practice. [ABSTRACT FROM AUTHOR]

On the basis of a previous experience suggesting that daunorubicin dose in induction was an independent prognostic factor in adult ALL, we designed a chemotherapeutic regimen (ALLVR589) characterized by high doses of daunorubicin (270 mg/m2) in induction and by high-dose Ara-C in post-remission. The protocol was otherwise conventional: induction and post-remission therapy were followed by chemo-radio prophylaxis of the central nervous system (CNS) and periodical reinductions over a 3-year maintenance period. Sixty consecutive patients (male 42, female 18, median age 34 years, range 14-71; B-lineage, 35; T-lineage, 25; Ph' and bcr/abl positive, 7) recruited between 1989 and 1996, were evaluated for treatment outcome. Complete remissions were 56 (93%), one patient had refractory disease, early deaths were five (8%); 19/56 (34%) patients relapsed, five of whom were Ph'+. Median time to relapse was 11 months (range 3-47); 68% of relapses occurred within 12 months from CR. No CNS relapses were observed. After a median follow-up of 44 months (1-100), 33/60 (55%) patients remain event-free; 23/60 (38%) are off-therapy in continuous CR (median follow-up from diagnosis: 63 months; range 38-100). These results suggest that increasing DNM dosage in induction is one of the possible approaches to improve the outcome of adult ALL by decreasing the relapse occurrence. [ABSTRACT FROM AUTHOR]

Editorial. Focuses on the opening of national and cultural boundaries to professional collaboration in nursing research. Problems and opportunities of learning a foreign language; Efforts of North American universities to help developing countries; Awareness that scientifically reliable knowledge needs to be validated in more than one cultural setting.