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Ataxia telangiectasia (AT) is a rare neurodegenerative genetic disorder due to bi-allelic mutations in the Ataxia Telangiectasia Mutated (ATM) gene. The aim of this paper is to better define the immunological profile over time, the clinical immune-related manifestations at diagnosis and during follow-up, and to attempt a genotype–phenotype correlation of an Italian cohort of AT patients. Retrospective data of 69 AT patients diagnosed between December 1984 and November 2019 were collected from the database of the Italian Primary Immunodeficiency Network. Patients were classified at diagnosis as lymphopenic (Group A) or non-lymphopenic (Group B). Fifty eight out of 69 AT patients (84%) were genetically characterized and distinguished according to the type of mutations in truncating/truncating (TT; 27 patients), non-truncating (NT)/T (28 patients), and NT/NT (5 patients). In 3 patients, only one mutation was detected. Data on age at onset and at diagnosis, cellular and humoral compartment at diagnosis and follow-up, infectious diseases, signs of immune dysregulation, cancer, and survival were analyzed and compared to the genotype. Lymphopenia at diagnosis was related per se to earlier age at onset. Progressive reduction of cellular compartment occurred during the follow-up with a gradual reduction of T and B cell number. Most patients of Group A carried bi-allelic truncating mutations, had a more severe B cell lymphopenia, and a reduced life expectancy. A trend to higher frequency of interstitial lung disease, immune dysregulation, and malignancy was noted in Group B patients. Lymphopenia at the onset and the T/T genotype are associated with a worst clinical course. Several mechanisms may underlie the premature and progressive immune decline in AT subjects.

The chapter develops, within a text devoted to the fiscal money, mainly under an economical point of view, an alternative point of view: the juridical one. The main problem dealed with is the legal feasibility of issuing Certified for Tax Credits. This study is conducted both under a private law approach and under a public law one. At the end, a properly accounting point of view is developped, under the provisions of generally agreed accounting standards and European Regulations on public sector accounts.

The chapter, within a book devoted to the fiscal money, treats of regional fiscal money. In doing this, it focuses two main topics. The former is the exploitation of a regional fiscal money, answering to the question if it is technically possible and economically convenient to issue fiscal money at a lower level than the state one. The latter concerns an Italian region in particular: Sicily, considered under many points of view as an OCA (Optimum Currency Area). The chapter explores, as well, the positive effects of introducing regional fiscal monies in underdevelopped regions and the value of them as a positive experiment, like in a laboratory, before extending them on a national basis.