Deficiency of the purine salvage enzyme adenosine deaminase leads to SCID (ADA-SCID). Hematopoietic cell transplantation (HCT) can lead to a permanent cure of SCID; however, little data are available on outcome of HCT for ADA-SCID in particular. In this multicenter retrospective study, we analyzed outcome of HCT in 106 patients with ADA-SCID who received a total of 119 transplants. HCT from matched sibling and family donors (MSDs, MFDs) had significantly better overall survival (86% and 81%) in comparison with HCT from matched unrelated (66%; P < .05) and haploidentical donors (43%; P < .001). Superior overall survival was also seen in patients who received unconditioned transplants in comparison with myeloablative procedures (81% vs 54%; P < .003), although in unconditioned haploidentical donor HCT, nonengraftment was a major problem. Long-term immune recovery showed that regardless of transplant type, overall T-cell numbers were similar, although a faster rate of T-cell recovery was observed after MSD/MFD HCT. Humoral immunity and donor B-cell engraftment was achieved in nearly all evaluable surviving patients and was seen even after unconditioned HCT. These data detail for the first time the outcomes of HCT for ADA-SCID and show that, if patients survive HCT, long-term cellular and humoral immune recovery is achieved. (Blood. 2012;120(17):3615-3624)
Background Individuation and management of the contacts of confirmed COVID-19 infection cases allows to quickly identify and isolate any secondary cases and interrupt the chain of transmission of the infection. Contact tracing is unquestionably very useful in stemming the spread of the pandemic from SARS-Cov 2 however there are no pediatric data that have measured the effectiveness of this method.MethodsFrom 30 October 2020 to 30 April 2021 ,during the second wave of the Covid pandemic, 19 primary care pediatricians reported suspected cases of SAR Cov 2 to the Public Health Service of the Piacenza district via a digital platform relating to a total pediatric population of about 17,500 children. All reported cases underwent a molecular PCR test as soon as possible and in any case within 48 hours. At the same time Public Hygiene Service implemented contact tracing in the same pediatric population in all children who were in close contact with positive cases .Results On the basis of clinical suspicion 1306 subjects underwent a molecular PCR nose pharyngeal swab for Covid -19, 107 of them tested positive (8,2% of reports-0.6% of the monitored population). In the same period, the Public Hygiene Service, through contact-tracing, identified another 1244 positive subjects in the same population of 17,500 children (7,1% of the monitored population).ConclusionsOur experience confirm that contact tracing is strongly raccomended for the managment of the SAR Cov 2 pandemic .It was about eleven times more sensitive than the clinical criteria for identifying positive subjects in pediatric age, often asymptomatic, during the second wave of the Covid pandemic.
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