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Background/purpose: We explored determinants of success in a large cohort of patients with pectus excavatum submitted to vacuum bell treatment and compared groups with satisfactory versus unsatisfactory outcomes., Methods: Retrospective case-control study in a single center between May 2013 and January 2020, including patients with pectus excavatum treated with vacuum bell. We classified patients according to their status at closure of data registry (surveillance; withdrawal; complete correction; failure) and according to Obermeyer's classification of degrees of pectus excavatum correction. Determinants of success were calculated using receiver operating characteristic curves., Results: Overall, 186 patients were included. Complete correction was achieved by 17% of the cases, while 45% remained under surveillance. Failure rates were low (n = 9; 5%), whereas withdrawal rates were 34%. Based on Obermeyer's classification of degree of excavation correction, 35% had excellent/good, 25% fair, and 40% poor/worse results. When comparing patients with good/excellent results with those with unsatisfactory results, patients with good/excellent results had a longer treatment duration [19.0 (13.0; 28) months vs. 13.0 (6.5; 22.5) months, p<0.0001], and lower initial pectus depth [1.6 (1.2; 2.0) cm, vs. 2.0 (1.5; 2.6) cm, p = 0.001]. Using ROC curves, the best determinants of success were an initial pectus depth ≤ 1.8 cm and a length of treatment > 12 months., Conclusion: One-third of patients in treatment with a vacuum bell achieved excellent or good outcomes in our cohort. Determinants of success included an initial pectus depth of 1.8 cm or less and a minimum length of treatment of 12 months., Type of Study: retrospective comparative study LEVEL OF EVIDENCE: III., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Immune-mediated thrombotic thrombocytopenic purpura is a rare and challenging hematological disease caused by the antibody anti-ADAMTS13. Though the mortality rate has decreased considerably in recent years, fatalities still remain unacceptable. This study aimed at further adding to the existing knowledge of this medical challenge. We enrolled 89 consecutive patients observed in six Italian centers (from 8 August 2013 to 28 May 2021) with a diagnosis of immune-mediated thrombotic thrombocytopenic purpura. Clinical information and blood parameters were collected for all patients. We describe clinical manifestations and laboratory data, possible risk factors and the therapeutic management of first episodes or relapses. A total of 74 first episodes and 19 relapses (median 3 years (interquartile range (IQR): 2-7)) were recorded. Seventy percent of patients enrolled at the first episode showed neurological signs and/or symptoms. All the patients enrolled at the first episode were treated with plasma exchange (median = 12; IQR: 8-19.5) and methylprednisolone (1 mg/kg/day). Rituximab (375 mg/m 2 weekly for four weeks) and caplacizumab were given to 15 (20.2%) and 2 patients (2.6%), respectively. We observed an overall mortality of 5.4% in the follow-up (median 60 months; IQR: 36.0-103.5). All fatalities occurred after a diagnostic delay. Present data point to the importance of the early detection of factors mostly associated with poor outcomes. It is likely that use of caplacizumab could improve the prognosis in those patients.