Kaposiform hemangioendothelioma (KHE) is an aggressive disease with high morbidity and mortality. The aim of this study was to retrospectively evaluate the efficacy and safety of sirolimus for the treatment of progressive KHE. A multicenter, retrospective cohort study was conducted in patients with progressive KHE treated with sirolimus. A total of 52 patients were analyzed. Thirty-seven (71%) patients exhibited Kasabach-Merritt phenomenon (KMP) and were significantly younger than the patients without KMP [95% confidence interval (CI), 14.39-41.61; p < 0.001]. Patients without KMP were all treated with sirolimus alone, whereas 21 KMP patients with severe symptoms received short-term combination therapy with prednisolone. Overall, 96% and 98% of patients showed improved relief of notable symptoms and/or improved complications at 6 and 12 months after treatment, respectively. After sirolimus treatment, significant decreases in mean severity scores occurred at 6 months (95% CI, 2.23-2.54, p < 0.001) and 12 months (95% CI, 1.53-1.90, p < 0.001). Compared to KMP patients, patients without KMP showed a response that was similar to but less pronounced during the 12 months of treatment (95% CI, 40.87-53.80; p < 0.001). For subgroup analysis of KMP patients, there were no significant differences in tumor shrinkage between those treated with combination therapy and those receiving sirolimus alone (95% CI, 18.11-25.02; p > 0.05). No patients permanently discontinued treatment due to toxicity-related events, and no drug-related deaths occurred. Sirolimus was effective and safe for the treatment of progressive KHE. Sirolimus may be considered as a first-line therapy or as part of a multidisciplinary approach for the treatment of KHE.
We report metallurgy on the nanoscale
to generate metal nanoparticles
and their simultaneous patterning in a single step. This is achieved
by the self-reduction of porous metal–organic framework crystals
using nanosecond pulsed laser irradiation. Metal nanoparticles of
Fe, Co, Ni, Cu, Zn, Cd, In, Bi, and Pb with uniform sizes (controllable
between 3 to 200 nm) and gaps (as narrow as 2 nm) are produced by
nine different metal–organic frameworks, where atomically dispersed
non-noble metal ions are reduced and gathered across the pores. The
instant light absorption and cooling at local positions by a laser
allows for precise and efficient patterning of metal nanoparticles.
This new method is suitable for device fabrication at a speed of 15
mm2 s–1 on glass, consuming only 1.5
W of power. A large variety of metal nanoparticle three-dimensional
architectures are demonstrated, among which one architecture exhibits
an enhanced plasmonic effect homogeneously across the entire pattern
for the detection of molecules at an extremely low concentration (10–12 M). These architectures are extremely stable under
air and humidity during production, use, and storage, without altering
the oxidation state, for 6 months.
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