Pre-Production and Quality Assurance of the Mu2e Calorimeter Silicon Photomultipliers

The Mu2e electromagnetic calorimeter has to provide precise information on energy, time and position for $\sim$100 MeV electrons. It is composed of 1348 un-doped CsI crystals, each coupled to two large area Silicon Photomultipliers (SiPMs). A modular and custom SiPM layout consisting of a 3$\times$2 array of 6$\times$6 mm$^2$ UV-extended monolithic SiPMs has been developed to fulfill the Mu2e calorimeter requirements and a pre-production of 150 prototypes has been procured by three international firms (Hamamatsu, SensL and Advansid). A detailed quality assurance process has been carried out on this first batch of photosensors: the breakdown voltage, the gain, the quenching time, the dark current and the Photon Detection Efficiency (PDE) have been determined for each monolithic cell of each SiPMs array. One sample for each vendor has been exposed to a neutron fluency up to $\sim$8.5~$\times$~10$^{11}$ 1 MeV (Si) eq. n/cm$^{2}$ and a linear increase of the dark current up to tens of mA has been observed. Others 5 samples for each vendor have undergone an accelerated aging in order to verify a Mean Time To Failure (MTTF) higher than $\sim$10$^{6}$ hours.Comment: NDIP 2017 - New Developments In Photodetection, 3-7 July 2017, Tours (France

Epidermolysis Bullosa in children: the central role of the pediatrician

Epidermolysis bullosa (EB) is a severe hereditary disease characterized by defective epithelial adhesion causing mucocutaneous fragility. The major types are EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB) and more than 35 EB subtypes. Another very rare type of EB is Kindler EB (KEB). Clinically, it is a very heterogeneous disease which ranges from localized to extensive skin lesions with frequent multisystem extra cutaneous involvement. The role of a pediatrician-dermatologist cooperation within a multidisciplinary team is fundamental for both the diagnosis and management contributing to these patients' better life expectancy. Aim of this study is to describe clinical and laboratory characteristics of the main EB subtypes focusing on nutritional and gastrointestinal aspects, providing information to aid the paediatric management of children with EB. This retrospective study reviewed the cases of 160 pediatric EB patients (76 male and 84 female): 31 patients affected by EBS (mean age +/- SD: 4.37 +/- 7.14), 21 patients affected by JEB (mean age +/- SD: 9.26 +/- 17.30) and 108 with DEB (mean age +/- SD: 11.61 +/- 13.48). All patients were admitted at the Bambino Gesu Children's Hospital in Rome, between June 2005 to June 2020. The reduced gastrointestinal absorption, chronic losses, esophageal stenosis and chronic inflammatory state, represent the basis of nutritional problems of EB patients. In particular, anemia represents one of the most important complications of DEB patients which could require transfusion-dependent patterns. Malnutrition, vitamin deficiencies and anemia have been related to growth delay in EB patients. A specific diet with a balance of all macronutrients is required and improving caloric intake with sugar limitations is fundamental to prevent dental caries and tooth decay typical of EB patients. While sepsis proved to be the major cause of morbidity and mortality in younger patients, squamous cell carcinoma was mostly observed in older patients, especially those affected by DEB. Patients with EB require regular monitoring for complications and sequelae with a frequency of evaluations which varies based on age and EB subtypes. Cooperation among medical teams involving paediatricians, dermatologists, specialist clinicians including nutritionists such as families and patient's association is fundamental to approach the disease and improve the quality of life of these patients

naevoid basal cell carcinoma syndrome in a 22 month old child presenting with multiple basal cell carcinomas and a fetal rhabdomyoma

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Tailored treatments in inborn errors of immunity associated with atopy (IEIs-A) with skin involvement

inborn errors of immunity associated with atopy (IEIs-A) are a group of inherited monogenic disorders that occur with immune dysregulation and frequent skin involvement. several pathways are involved in the pathogenesis of these conditions, including immune system defects, alterations of skin barrier and metabolism perturbations. current technological improvements and the higher accessibility to genetic testing, recently allowed the identification of novel molecular pathways involved in IEIs-A, also informing on potential tailored therapeutic strategies. compared to other systemic therapy for skin diseases, biologics have the less toxic and the best tolerated profile in the setting of immune dysregulation. Here, we review IEIs-A with skin involvement focusing on the tailored therapeutic approach according to their pathogenetic mechanism

Crilin: A CRystal calorImeter with Longitudinal InformatioN for a future Muon Collider

The measurement of physics processes at new energy frontier experiments requires excellent spatial, time, and energy resolutions to resolve the structure of collimated high-energy jets. In a future Muon Collider, the beam-induced backgrounds (BIB) represent the main challenge in the design of the detectors and of the event reconstruction algorithms. The technology and the design of the calorimeters should be chosen to reduce the effect of the BIB, while keeping good physics performance. Several requirements can be inferred: i) high granularity to reduce the overlap of BIB particles in the same calorimeter cell; ii) excellent timing (of the order of 100 ps) to reduce the out-of-time component of the BIB; iii) longitudinal segmentation to distinguish the signal showers from the fake showers produced by the BIB; iv) good energy resolution (less than 10%/sqrt(E)) to obtain good physics performance, as has been already demonstrated for conceptual particle flow calorimeters. Our proposal consists of a semi-homogeneous electromagnetic calorimeter based on Lead Fluoride Crystals (PbF2) readout by surface-mount UV-extended Silicon Photomultipliers (SiPMs): the Crilin calorimeter. In this paper, the performances of the Crilin calorimeter in the Muon Collider framework for hadron jets reconstruction have been analyzed. We report the single components characterizations together with the development of a small-scale prototype, consisting of 2 layers of 3x3 crystals each