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Lehrnbecher T.,Goethe University Frankfurt | Aplenc R.,Childrens Hospital of Philadelphia | Pereira F.R.,Argentina Treatment Group of Acute Leukemias GATLA Argentina | Lassaletta A.,Hospital Nino Jesus | And 4 more authors.
Haematologica | Year: 2012

Background Standardization in clinical practice may lead to improved outcomes. Unfortunately, little is known about the variability of non-pharmacological anti-infective measures in children with cancer. Design and Methods A web-based survey assessed institutional recommendations regarding restrictions of social contacts, pets and food and instructions on wearing face masks in public for children with standard- risk acute lymphoblastic leuk emia and acute myeloid leukemia during intensive chemotherapy. Results A total of 336 institutions in 27 countries responded to the survey (range, 1-76 institutions per country; overall response rate 61%). Most institutions recommend that patients with acute myeloid leukemia avoid indoor public places and daycare, kindergarten and school, whereas recommendations for patients with acute lymphoblastic leukemia differ considerably by institution. In terms of restrictions related to pets, there was a wide variability between institutions for both acute lymphoblastic and acute myeloid leukemia patients. Most, but not all institutions do not allow children with either acute lymphoblastic or acute myeloid leukemia to eat raw meat, raw seafood or unpasteurized milk. Whereas most institutions do not routinely recommend that patients with acute lymphoblastic leukemia wear face masks in public, advice on this matter varies for patients with acute myeloid leukemia. Conclusions The survey demonstrates that there is a wide variation in recommendations on non-pharmacological anti-infective measures between different institutions, countries and continents. This information may be used to encourage harmonization of supportive care practices and future clinical trials. © 2012 Ferrata Storti Foundation.

De Masi S.,Azienda Ospedaliero Universitaria Meyer
Epidemiologia e prevenzione | Year: 2014

The Italian's experience of the guidelines development group is discussed through the evaluation of its ten years of activity. Focus is placed on the Italian guidelines working group organization and on the kind of documents developed. The horizontal architecture of the system and the several partnerships settled over time allowed the definition of a small coordinating group connected with a multitude of territorial stakeholders, such as scientific societies and local health units pertaining to the Italian National Health System. Different kinds of documents were produced, as adaptations of already existing guidelines elaborated by international institutions, short reviews addressing specific clinical issues and consensus conferences aimed at providing clinical governance on issues which lack on evidence. The steps needed to produce a high quality guideline are presented, considering and comparing all the different international experiences, to define and discuss a common and well-structured methodology, and to face the ethical and epistemological implications of each method. The multidisciplinary of the working groups, the importance of the active surveillance on conflicts of interests, the definition of a minimum set of rules to be followed during the whole activity and the transparency of all the steps are the milestones of the Italian experience. The lack of a continuous and stable source of funding and the subsequent instability of the central structure are endangering all the knowledge and the experience gained during these years of activity. It is therefore crucial to guarantee and safeguard the role of a national, independent and public institution in the supervision of the guidelines development process and the provision of clinical governance.

The children nursing profession: attitudes and motivations. A cross-sectional study Introduction In the main European health systems with the profession of General Care Nurse (GN) exists the profession of the Children Nurse (CN). A debate has developed about the relationship between the 2 professions and their respective roles in Paediatric wards, however studies about the Italian situation are scanty. Materials and methods This cross-sectional study describes with the use of a questionnaire, features, attitudes and motivations of a population of registered nurses working in all the Paediatrics wards of the Province of Florence, Italy. Results 299 nurses were examined (70% GNs, 30% CNs). CNs self-evaluated their own preparedness with higher scores than GNs (p<0.00001). The intention of leaving the profession among GN was three times higher than among GPs (p=0.05). More than one half of CNs stated that they would never work with adults. Conclusions The study shows some interesting differences between the two nursing professions. In particular, that the children nursing profession has a motivational basis largely different from that of the general nursing profession.

Bryceson Y.T.,Karolinska University Hospital | Pende D.,Istituto di Ricovero e Cura rattere Scientifico Azienda Ospedaliera Universitaria | Maul-Pavicic A.,University Hospital Freiburg | Gilmour K.C.,Immunology | And 16 more authors.
Blood | Year: 2012

Familial hemophagocytic lymphohistiocytosis (FHL) is a life-threatening disorder of immune regulation caused by defects in lymphocyte cytotoxicity. Rapid differentiation of primary, genetic forms from secondary forms of hemophagocytic lymphohistiocytosis (HLH) is crucial for treatment decisions. We prospectively evaluated the performance of degranulation assays based on surface up-regulation of CD107a on natural killer (NK) cells and cytotoxic T lymphocytes in a cohort of 494 patients referred for evaluation for suspected HLH. Seventy-five of 77 patients (97%) with FHL3-5 and 11 of 13 patients (85%) with Griscelli syndrome type 2 or Chediak-Higashi syndrome had abnormal resting NK-cell degranulation. In contrast, NK-cell degranulation was normal in 14 of 16 patients (88%) with X-linked lymphoproliferative disease and in 8 of 14 patients (57%) with FHL2, who were identified by diminished intracellular SLAM-associated protein (SAP), X-linked inhibitor of apoptosis protein (XIAP), and perforin expression, respectively. Among 66 patients with a clinical diagnosis of secondary HLH, 13 of 59 (22%) had abnormal resting NK-cell degranulation, whereas 0 of 43 had abnormal degranulation using IL-2-activated NK cells. Active disease or immunosuppressive therapy did not impair the assay performance. Overall, resting NK-cell degranulation below 5% provided a 96% sensitivity for a genetic degranulation disorder and a specificity of 88%. Therefore, degranulation assays allow a rapid and reliable classification of patients, benefiting treatment decisions. © 2012 by The American Society of Hematology.

Andreoli E.,Laboratorio Of Microbiologia E Virologia | Savelli S.,Azienda Ospedaliero Universitaria Meyer | Guidi S.,Azienda Ospedaliero Universitaria Careggi | Pecile P.,Laboratorio Of Microbiologia E Virologia
Journal of Pediatric Hematology/Oncology | Year: 2012

Toxoplasma gondii is an opportunistic parasite, which very unusually may cause acute encephalitis in patients undergoing chemotherapy or hematopoietic stem cell transplant. The prognosis is usually dismal also because of late diagnosis, depending on the limited availability of specific diagnostic tools. An early diagnosis allows effective intervention with specific antibiotics, which may provide a chance for cure. We report 2 cases of cerebral toxoplasmosis in which the use of polymerase chain reaction on cerebrospinal fluid allowed a prompt diagnosis and specific therapy, which was followed by clinical response and negativization at follow-up studies of T. gondii genome on cerebrospinal fluid by polymerase chain reaction and by brain imaging. Copyright © 2012 by Lippincott Williams & Wilkins.

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