Sulle tracce dei pastori-allevatori pre-protostorici nel Carso (e altrove): esempi di uso integrato di indicatori diversi

The Classical Karst is a plateau of low rounded hills and low mountains ranging from 100-200 m to 800-900 m above sea level, that covers the eastern most part of northern Italy and the southwestern part of Slovenia. The outcropping rocks are chiefly limestones, crossed by two flysch (marl and sandstone) belts, a dozen kilometres wide. The area is not suitable for agriculture, and since historical times pastoralism / herding of sheep and goats has been one of the main economic activities. Its importance also in prehistoric times – hinted at already at the end of the 19th century by Marchesetti – was confirmed in the late 1990s, when sedimentological and soil micro-morphological analyses of some cave deposits indicated that these sites, at least in the investigated areas, had been used by prehistoric shepherds for stabling their flocks. The re-examination of the archaeological materials found in the same caves suggested that the pastoral use presumably started in the Neolithic and continued till the Bronze or Iron Age. In the course of millennia the agents and the ways of using the caves would have clearly changed: on the grounds of the available data, and still referring to the models proposed by Brochier c. 20 years ago, at present the hypothesis that most of the Karst caves were basically used as “grottes-bergeries” – i.e. as stables almost without a contemporaneous human presence –, rather than “habitats-bergeries”, is likely, though not beyond doubt. Our interpretation combines direct and indirect indicators from different scientific fields: besides sedimentology, soil micromorphology and archaeology, at the basis of the renewed interest in pastoralism, archaeometry, geomorphology, archaeozoology, ethnohistory and ethnography. To test the potentiality of the use of data derived in particular from the last two fields, we have recently extended our research to other geographical areas, close to the Karst, immediately to the north, in the northeastern part of the region Friuli Venezia Giulia, and far away, in Sardinia

Transfusional approach in multi-ethnic Sickle Cell patients: real-world practice data from a Multicenter survey in Italy

Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Gold standard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC) transfusion regimens to limit disease progression. Here, we report a retrospective study on 1,579 SCD patients (median age 23 years; 802 males/777 females), referring to 34 comprehensive Italian centers for hemoglobinopathies. Although we observed a similar proportion of Caucasian (47.9%) and African (48.7%) patients, Italian SCD patients clustered into two distinct overall groups: children of African descent and adults of Caucasian descent. We found a subset of SCD patients requiring more intensive therapy with a combination of HC plus chronic transfusion regimen, due to partial failure of HC treatment alone in preventing or reducing sickle cell-related acute manifestations. Notably, we observed a higher use of acute transfusion approaches for SCD patients of African descent when compared to Caucasian subjects. This might be related to (i) age of starting HC treatment; (ii) patients' low social status; (iii) patients' limited access to family practitioners; or (iv) discrimination. In our cohort, alloimmunization was documented in 135 patients (8.5%) and was more common in Caucasians (10.3%) than in Africans (6.6%). Alloimmunization was similar in male and female and more frequent in adults than in children. Our study reinforces the importance of donor-recipient exact matching for ABO, Rhesus, and Kell antigen systems for RBC compatibility as a winning strategy to avoid or limit alloimmunization events that negatively impact the clinical management of SCD-related severe complications

Risultati preliminari degli Studi Prospettico e Retrospettivo (OEP 2015-01 e 2015-02) sull’Anemia Emolitica Autoimmune (AEA) del bambino di nuova diagnosi

L’anemia emolitica autoimmune (AEA) è una emopatia rara in età pediatrica nella quale i criteri diagnostici e le strategie terapeutiche non sono ancora ben standardizzate. Il GdL AIEOP “Patologie del globulo rosso” ha affrontato tali problematiche producendo un documento, fruibile sul sito AIEOP dal 1 Novembre 2013, che possa essere di aiuto nella gestione della patologia e ha dato inizio ad uno Studio sia retrospettivo che prospettico multicentrico di raccolta dati, per valutare se e come le indicazioni fornite possano aver cambiato la gestione dell’emopatia. Vengono presentati i risultati preliminari riguardanti 159 bambini, 48 con diagnosi precedente il 1 Novembre 2013 e 111 con diagnosi successiva. I pazienti, 56%M e 44%F, con un’età mediana alla diagnosi di 47 mesi, sono stati reclutati da 21 Centri AIEOP. Alla diagnosi, nel 20,8% dei pazienti, si è riscontrata la presenza di patologie immunologiche e/o ematologiche precedenti o concomitanti l’AEA mentre nel 15,1% era documentata un’infezione isolata. Il valore medio di Hb era 6,1 grammi, quasi tutti i pazienti presentavano segni di emolisi. Nella Tabella 1 sottostante sono riportate le indagini immunoematologiche. Il trattamento nelle AEA, escludendo le forme da IgM fredde, è stato iniziato in 140/146 pz (95,9%) ed ha previsto il PDN a dosaggi convenzionali nel 94,4%. Il 51,4% ha trasfuso Emazie concentrate, il 59,7% HD di PDN, il 49,7% HD di Immunoglobuline, l’1,4% PlasmaExchange. Il 9,5% dei pazienti ha necessitato di terapie alternative già nelle prime 4 settimane. La Remissione Completa è stata raggiunta dal 55,4% dei pazienti al 21° giorno, dal 20,9% al 28° giorno, dal 9,4% al 42° giorno mentre il 14,3% era in RP o Resistente al 42° giorno. La differenza più significativa tra lo studio prospettico e quello retrospettivo è stata la durata della terapia di prima linea: 6 mesi o più (per steroidodipendenza) nel 71,6% dei pazienti dello studio prospettico verso il 52,3% del retrospettivo (p=0.037) e la percentuale di recidive: 5,5% vs 29,8%. Quest’ultimo dato, anche se significativo, è da valutare nel tempo. Nel follow up nel 10,4% dei pazienti sono comparse patologie immunitarie e/o oncoematologiche

Association of immune thrombocytopenia and celiac disease in children: A retrospective case control study

Objective: The association between celiac disease (CD) and immune thrombocytopenia (ITP) is still uncertain. The aim of this study was to characterize the coexistence of these two diseases in Italian children. Materials and Methods: This is a retrospective multicenter study investigating the occurrence of CD in 28 children with ITP diagnosed from January 1, 2000, to December 31, 2019. Results: The first diagnosis was ITP in 57.1% and CD in 32.1% of patients. In 3 patients (10.7%), the two diagnoses were simultaneous. All the potential and silent cases of CD in our cohort were diagnosed in the groups of \u201cITP first\u201d and \u201csimultaneous diagnosis\u201d. In all children ITP was mild, and in 2 out of 8 not recovered from ITP at the time of CD diagnosis a normalization of platelet counts (>100,000/\ub5L) occurred 3 and 5 months after starting a gluten-free diet, respectively. Conclusion: We think that screening for CD should be considered in children with ITP regardless of the presence of gastrointestinal symptoms. Furthermore, some patients may recover from ITP after starting a gluten-free diet

Late-onset and long-lasting autoimmune neutropenia: An analysis from the Italian Neutropenia Registry

Primary autoimmune neutropenia (pAN) is typified by onset in early infancy and a mild/ moderate phenotype that resolves within 3 years of diagnosis. In contrast, secondary AN is classically an adult disease associated with malignancy, autoimmunity, immunodeficiency, viral infection, or drugs. This study describes a cohort of 79 children from the Italian Registry who, although resembling pAN, did not fully match the criteria for pAN because neutropenia either appeared after age 5 years (LO-Np) or lasted longer than 3 years (LL-Np). These 2 categories compared with classical pAN showed a far inferior rate of resolution (P < .001), lower severity of neutropenia (P = .03), leukopenia (P < .001), lymphopenia (P < .001) with low B+ (P = .001), increased need of granulocyte colony-stimulating factor (P = .04), and increased frequency of autoimmunity over the disease course (P < .001). A paired comparison between LO-Np and LL-Np suggested that LO-Np had a lower rate of resolution (P < .001) and lower white blood cell (P < .001) and lymphocyte (P < .001) values, higher occurrence of apthae (P = .008), and a stronger association with autoimmune diseases/ markers (P = .001) than LL-Np, thus suggesting a more pronounced autoimmune signature for LO-Np. A next-generation sequencing panel applied in a small subgroup of LO-Np and LL-Np patients identified variants related to immune dysregulations. Overall, these findings indicate that there are important differences among pAN LL-Np and LO-Np. Forms rising after 3 years of age, with low tendency to resolution, require tight monitoring and extensive immune investigations aimed to early identify underlying immunologic disease