Maria Silvia Mangano

Background: Mastocytosis (MS) is a rare disease that can involve various organs, including the bone. Given the incidence of the disease in the global population, MS poses a challenge for physicians, and early therapeutic intervention in the initial stages could significantly impact the quality of life of affected patients. Objective: The aim of this scoping review was to provide an overview of secondary osteoporosis in systemic mastocytosis (SM), focusing on the heterogeneity of its manifestations, the benefits of early diagnosis, and appropriate pharmacological treatment. Design: A technical expert panel (TEP) consisting of 8 physicians with expertise in metabolic bone diseases conducted the review following the PRISMA-ScR model. A strength of this study is that it provides various therapeutic approaches for patients with bone involvement in SM, although the limited available literature on the topic constituted a limitation. The TEP sought evidence regarding the following diagnostic and therapeutic modalities in the management of SM: “bisphosphonate therapy”, “zoledronic acid therapy”, “denosumab therapy”, “IFN-alpha therapy”, and “IFN-alpha therapy in combination with pamidronate”. Results: Clinical data showed a correlation between densitometric outcomes, serum tryptase levels, and mast cell infiltration in the bone marrow, between increased bone mineral density and the presence of osteosclerosis in cases of advanced SM, between the severity of osteoporosis and hypertryptasemia, and also provided results on the long-term effects of bisphosphonate therapy, the therapeutic efficacy of zoledronic acid administration, the positive effect of denosumab on the reduction of serum tryptase levels (even if is proved in a limited numbers of cases) and the prevention of new fractures, and the effect of IFN-alpha in more severe cases of SM, either alone or in combination with pamidronate. Conclusions: Studies have demonstrated the effectiveness of various treatments depending on the form of mastocytosis, whether indolent systemic or advanced systemic, in the prognosis of the disease. However, this role should be further investigated in additional clinical studies, considering the limited data on the use of these interventions.

Telemedicine was shown to be indispensable during the SARS-CoV-2 pandemic to ensure continuity of care for fragile patients. We compared a telerehabilitation program versus an outpatient rehabilitation program in women with breast cancer undergoing quadrantectomy surgery. There were 56 women with breast cancer divided into two groups: the treatment group (TG), made up of 24 patients undergoing a remote rehabilitation project program; and the control group (CG), composed of 32 patients subjected to the same rehabilitation project program in an outpatient setting. At the time of enrollment (T0) and the end of the 8 weeks of treatment (T1), the following questionnaire scores were considered: numerical rating scale (NRS), Disabilities of the Arm, Shoulder and Hand questionnaire (Quick-DASH), Piper fatigue scale (PFS)m and Breast Cancer Therapy Functional Rating Scale (FACT-B). We observed that the CG showed greater improvements than the TG in upper limb function (7.8 ± 4.2 vs. 10.9 ± 4.9; p < 0.05) and quality of life (27.9 ± 7.2 vs. 40.0 ± 3.3; p < 0.05). No difference in efficacy between the two groups was observed for pain (2.2 ± 0.6 vs. 2.3 ± 0.9; p = 0.64) and fatigue (3.2 ± 1.1 vs. 3.2 ± 0.6; p = 0.66). Telerehabilitation is a valid tool in the management of women with breast cancer in the postoperative phase. However, face-to-face rehabilitation treatment may be preferred because it is more effective as it allows the construction of a specific, personalized, and targeted rehabilitation program.

This narrative review aims to give an overview of some postural evaluation systems currently used in patients with haemophilia. Among them, first, we analyse the HJHS scale, recognized as a specific evaluation tool for haemophilic arthropathy. Second, we focus on other systems usually used in non-haemophilic patients that have also shown good applicability in this patient category, such as gait analysis, stabilometry, and baropodometric examination. This review underlines the use these tools could have in clinical practice to identify the early postural alterations in patients with haemophilia and set up personalised rehabilitation programs.

Even though vaccination is easy to access in Europe, it has been facing several outbreaks caused by vaccine preventable diseases. Trying to balance the right to health of the community with individual self-determination, in Italy was mandate vaccination for ten vaccine preventable diseases in 2017. The project “Sportello Vaccinale” provided a counseling service to parents in kindergarten of Palermo. The main objective of the study was to investigate knowledge and compliance on vaccination by children’s parents after the implementation of mandatory vaccination. A cross sectional study was conducted among parents accessing to the “Sportello Vaccinale” using a questionnaire. The questionnaire consisted of 34 items divided into three sections: socio-demographic data; knowledge and attitude on mandatory vaccination using Health Belief Model (HBM); Health Literacy (HL) level. A total of 95 questionnaires were garnered in the kindergartens from respondents with a mean age of 36 (±SD=6) years. The respondents who delayed hexavalent or Measles, Mumps, Rubella and Varicella (MMRV) vaccinations were 8 (8%) and the more frequent cause was fear of adverse reaction (87%). The sample had more often high level of health literacy (37%), and 55% had higher HBM score. The factor significantly associated with higher HBM score was the age of respondents (OR= 1.14, p=0.006) after controlling for other variables. Consent to vaccination practice was widely represented, but the results of the study highlighted the need for parents to have more information and more time devoted to the practice of vaccination counseling. Implementing counseling activities at school or at the vaccination service, could support an aware decision process of the parents on vaccination topic.