Background: More than 70% of elderly people age 80 and older are experiencing problems in personal mobility. Assistive robotics can represent a concrete support providing also a support for caregivers, clinicians and nurses by reducing their burden. Methods: A total of 20 older people and 34 caregivers (formal and informal) were interviewed in Italy and the Netherlands to investigate and prioritize their needs concerning the personal mobility domains and their attitudes towards assistive robots. The data were analysed from a user point of view by means of thematic content analysis by underlying recurrent topics. Results: The results revealed four categories of needs from the perspective of the older individuals: instrumental needs, rehabilitation needs, personal safety and indoor activities of daily life. Additionally, the results underline how personal mobility issues influence different aspects of daily life. Complementarily, three categories of caregiver needs were also distinguished: instrumental needs, rehabilitation monitoring needs and checkup needs. The highest percentage of participants showed a positive expectation towards assistive robotics. Conclusions: The results were clustered according to the robot abilities (i.e., motion, interaction, manipulation, decision support and perception abilities) as a list of functional and technical requirements that should be developed to address all the needs related to the personal mobility. Robotic developer teams that work in this context could take advantage of this research. Additionally, this work can be used as a basis for clinicians and nurses working in geriatric units to understand how the robots can support and enhance their work.The incidence of personal mobility limitations affects 35% of adults age 70 and older and 72% of people over 80 years of age. Assistive robots can support elderly people during daily tasks: they could promote their personal mobility acting as a supporting tool. The results of the needs analysis revealed four categories of needs from the perspective of the older individuals: instrumental needs, rehabilitation needs, personal safety, and indoor activities of daily life. Three categories of caregiver needs were also distinguished: instrumental needs, rehabilitation monitoring needs, and check-up needs.

Background: Inherited defects in adenosine deaminase (ADA) cause severe immune deficiency, which can be corrected by ADA enzyme replacement therapy (ERT). Additionally, ADA-deficient patients suffer from hearing impairment. We hypothesized that ADA-deficient (–/–) mice also exhibit hearing abnormalities and that ERT from an early age will improve the hearing and immune defects in these mice.Methods: Auditory brainstem evoked responses, organ weights, thymocytes numbers, and subpopulations, lymphocytes in peripheral blood as well as T lymphocytes in spleen were analyzed in ADA–/– and ADA-proficient littermate post-partum (pp). The cochlea was visualized by scanning electron microscopy (SEM). The effects of polyethylene glycol conjugated ADA (PEG-ADA) ERT or 40% oxygen initiated at 7 days pp on the hearing and immune abnormalities were assessed.Results: Markedly abnormal hearing thresholds responses were found in ADA–/– mice at low and medium tone frequencies. SEM demonstrated extensive damage to the cochlear hair cells of ADA–/– mice, which were splayed, short or missing, correlating with the hearing deficits. The hearing defects were not reversed when hypoxia in ADA–/– mice was corrected. Progressive immune abnormalities were detected in ADA–/– mice from 4 days pp, initially affecting the thymus followed by peripheral lymphocytes and T cells in the spleen. ERT initiated at 7 days pp significantly improved the hearing of ADA–/– mice as well as the number of thymocytes and T lymphocytes, although not all normalized.Conclusions: ADA deficiency is associated with hearing deficits and damage to cochlear hair cells. Early initiation of ERT improves the hearing and immune abnormalities.

Inherited defects that abrogate the function of the adenosine deaminase (ADA) enzyme and consequently lead to the accumulation of toxic purine metabolites cause profound lymphopenia and severe combined immune deficiency. Additionally, neutropenia and impaired neutrophil function have been reported among ADA-deficient patients. However, due to the rarity of the disorder, the neutrophil developmental abnormalities and the mechanisms contributing to them have not been characterized. Induced pluripotent stem cells (iPSC) generated from two unrelated ADA-deficient patients and from healthy controls were differentiated through embryoid bodies into neutrophils. ADA deficiency led to a significant reduction in the number of all early multipotent hematopoietic progenitors. At later stages of differentiation, ADA deficiency impeded the formation of granulocyte colonies in methylcellulose cultures, leading to a significant decrease in the number of neutrophils generated from ADA-deficient iPSCs. The viability and apoptosis of ADA-deficient neutrophils isolated from methylcellulose cultures were unaffected, suggesting that the abnormal purine homeostasis in this condition interferes with differentiation or proliferation. Additionally, there was a significant increase in the percentage of hyperlobular ADA-deficient neutrophils, and these neutrophils demonstrated significantly reduced ability to phagocytize fluorescent microspheres. Supplementing iPSCs and methylcellulose cultures with exogenous ADA, which can correct adenosine metabolism, reversed all abnormalities, cementing the critical role of ADA in neutrophil development. Moreover, chemical inhibition of the ribonucleotide reductase (RNR) enzyme, using hydroxyurea or a combination of nicotinamide and trichostatin A in iPSCs from healthy controls, led to abnormal neutrophil differentiation similar to that observed in ADA deficiency, implicating RNR inhibition as a potential mechanism for the neutrophil abnormalities. In conclusion, the findings presented here demonstrate the important role of ADA in the development and function of neutrophils while clarifying the mechanisms responsible for the neutrophil abnormalities in ADA-deficient patients.