Background: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE. The goal of our study is to critically assess the existing literature on outcomes of patients with KHE treated with mTOR inhibitors.

IgA vascultis (IgAV) (also called Henoch-Schönlein purpura) is the most common small-vessel vasculitis in children, characterized by cutaneous, articular, gastrointestinal (GI), and renal involvement.Although pediatric outcomes tend to be positive, long-term prognosis depends mainly on the severity of renal or other organ involvement. 1 We sought to identify initial features in children with IgAV and their association with unfavorable outcomes defined as persistent renal disease at ≥1 year, other severe organ involvement, and the need for additional treatment with immunosuppresants other than corticosteroids. | PATIENTS AND ME THODSThis was a retrospective study approved by the institutional review board and conducted at the National Institute of Pediatrics, Mexico City. We included patients 0-18 years of age who were diagnosed with IgAV according to the European League Against Rheumatism/ Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society (EULAR/PRINTO/PRES) 2008 criteria 2 (presence of purpura without thrombocytopenia with predominance in lower extremities plus one of the following four conditions: (a) abdominal pain, (b) arthritis or arthralgia, (c) histopathology compatible with leukocytoclastic vasculitis, or d) renal manifestations) and had clinical follow-up for ≥1 year, from January 1, 2007, to December 31, 2017. Patients who had pre-existing renal disease or routine medication intake with corticosteroids or immunosuppresants were excluded. We reviewed charts and collected clinical and laboratory data beginning when the diagnosis of IgAV was made and throughout the clinical course for ≥1 year. Statistical analysis using chi-squared or Fisher's exact test to compare groups was done; a p value ≤.05 was considered significant (IBM SPSS Statistics 21.0. Armonk, NY: IBM Corp.) | RE SULTSA total of 106 patients met inclusion criteria, of whom 59 (55.7%) were female, with a mean age at diagnosis of 5.7 years (SD ± 3.2).All had palpable purpura, and 41 (35.8%) had atypical lesions-for example, location above the waist, or bullous or ulcerated lesions.Arthralgias/arthritis was present in 84 (79.2%), abdominal pain in 74 (69.8%), gastrointestinal bleeding in 37 (34.9%), and abnormal urinalysis in 43 (40.6%): isolated hematuria in 25 (23.5%), proteinuria in 4 (3.7%), or both in 14 (13.2%). None of the patients had abnormal creatinine levels. Mean follow-up time was 3.09 years (SD ± 2.78).