4 research outputs found

    Active Region Overheating in Pulsed Quantum Cascade Lasers: Effects of Nonequilibrium Heat Dissipation on Laser Performance

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    Mid IR Quantum cascade lasers are of high interest for the scientific community due to their unique applications. However, the QCL designs require careful engineering to overcome some crucial disadvantages. One of them is active region (ARn) overheating, which significantly affects laser characteristics, even in the pulsed mode. In this work, we consider the effects related to the nonequilibrium temperature distribution when thermal resistance formalism is irrelevant. We employ the heat equation and discuss the possible limitations and structural features stemming from the chemical composition of the ARn. We show that the presence of solid solutions in the ARn structure fundamentally limits the heat dissipation in pulsed and CW regimes due to their low thermal conductivity compared with binary compounds. Also, the QCL postgrowths affect the thermal properties of a device closer to CW mode, while it is by far less important in the short-pulsed mode

    Prospective Study of a Cohort of Russian Nijmegen Breakage Syndrome Patients Demonstrating Predictive Value of Low Kappa-Deleting Recombination Excision Circle (KREC) Numbers and Beneficial Effect of Hematopoietic Stem Cell Transplantation (HSCT)

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    BackgroundNijmegen breakage syndrome (NBS) is a combined primary immunodeficiency with DNA repair defect, microcephaly, and other phenotypical features. It predominantly occurs in Slavic populations that have a high frequency of carriers with the causative NBN gene c.657_661del5 mutation. Due to the rarity of the disease in the rest of the world, studies of NBS patients are few. Here, we report a prospective study of a cohort of Russian NBS patients.Methods35 Russian NBS patients of ages 1–19 years, referred to our Center between years 2012 and 2016, were prospectively studied.ResultsDespite the fact that in 80% of the patients microcephaly was diagnosed at birth or shortly thereafter, the average delay of NBS diagnosis was 6.5 years. Though 80% of the patients had laboratory signs of immunodeficiency, only 51% of the patients experienced significant infections. Autoimmune complications including interstitial lymphocytic lung disease and skin granulomas were noted in 34%, malignancies—in 57% of the patients. T-cell excision circle (TREC)/kappa-deleting recombination excision circle (KREC) levels were low in the majority of patients studied. Lower KREC levels correlated with autoimmune and oncological complications. Fifteen patients underwent hematopoietic stem cell transplantation (HSCT), 10 of them were alive and well, with good graft function. Three patients in the HSCT group and five non-transplanted patients died; tumor progression being the main cause of death. The probability of the overall survival since NBS diagnosis was 0.76 in the HSCT group and 0.3 in the non-transplanted group.ConclusionBased on our findings of low TRECs in most NBS patients, independent of their age, TREC detection can be potentially useful for detection of NBS patients during neonatal screening. KREC concentration can be used as a prognostic marker of disease severity. HSCT is a viable treatment option in NBS and should be especially considered in patients with low KREC numbers early on, before development of life-threatening complications
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