53 research outputs found

    Modeling of multiple filamentation of terawatt laser pulses on a hundred-meter air path

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    The results of numerical simulation of multiple filamentation of terawatt femtosecond pulse Ti:Sapphire laser performed on the experimental data obtained in the airway of a length of 106 m when changing the initial spatial focusing and laser power

    ГСнСтичСскиС аспСкты ΠΏΠ°Ρ‚ΠΎΠ³Π΅Π½Π΅Π·Π° систСмной красной Π²ΠΎΠ»Ρ‡Π°Π½ΠΊΠΈ Ρƒ Π΄Π΅Ρ‚Π΅ΠΉ

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    The paper presents data on the pathogenesis of systemic lupus erythematosus (SLE), and depicts various molecular mechanisms for the development of SLE and lupus-like syndromes. It describes groups of diseases, such as apoptotic defects; NETosis; interferonopathies; complement deficiency; autotolerance disorders associated with mutations in the RAG1/RAG2 genes; hereditary metabolic diseases (prolidase deficiency, deficiency of adenosine deaminase 2; lysinuric protein intolerance; and Ξ±-mannosidase deficiency). The table summarizes clinical data on most of the known lupus-like syndromes and their molecular mechanisms.The pathogenesis of many forms of monogenic lupus-like diseases is being studied. The main sign suggesting in favor of the possible monogenic disease in a patient with SLE is its onset in infancy, especially in males. Attention should be also paid to a compromised family history, including to the marriage between close relatives, the resistance of disease to standard therapy, as well as atypical symptoms.Β Π’ ΡΡ‚Π°Ρ‚ΡŒΠ΅ ΠΏΡ€ΠΈΠ²Π΅Π΄Π΅Π½Ρ‹ Π΄Π°Π½Π½Ρ‹Π΅ ΠΎ ΠΏΠ°Ρ‚ΠΎΠ³Π΅Π½Π΅Π·Π΅ систСмной красной Π²ΠΎΠ»Ρ‡Π°Π½ΠΊΠΈ (Π‘ΠšΠ’), описаны Ρ€Π°Π·Π»ΠΈΡ‡Π½Ρ‹Π΅ молСкулярныС ΠΌΠ΅Ρ…Π°Π½ΠΈΠ·ΠΌΡ‹ развития Π‘ΠšΠ’ ΠΈ Π²ΠΎΠ»Ρ‡Π°Π½ΠΎΡ‡Π½ΠΎΠΏΠΎΠ΄ΠΎΠ±Π½Ρ‹Ρ… синдромов. ΠžΠΏΠΈΡΠ°Π½Ρ‹ Ρ‚Π°ΠΊΠΈΠ΅ Π³Ρ€ΡƒΠΏΠΏΡ‹ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ, ΠΊΠ°ΠΊ Π΄Π΅Ρ„Π΅ΠΊΡ‚Ρ‹ Π°ΠΏΠΎΠΏΡ‚ΠΎΠ·Π°, Π½Π΅Ρ‚ΠΎΠ·, ΠΈΠ½Ρ‚Π΅Ρ€Ρ„Π΅Ρ€ΠΎΠ½ΠΎΠΏΠ°Ρ‚ΠΈΠΈ, Π΄Π΅Ρ„ΠΈΡ†ΠΈΡ‚ систСмы ΠΊΠΎΠΌΠΏΠ»Π΅ΠΌΠ΅Π½Ρ‚Π°, Π½Π°Ρ€ΡƒΡˆΠ΅Π½ΠΈΡ систСмы аутотолСрантности, связанныС с мутациями Π² Π³Π΅Π½Π°Ρ… RAG1/RAG2, наслСдствСнныС Π±ΠΎΠ»Π΅Π·Π½ΠΈ ΠΎΠ±ΠΌΠ΅Π½Π° (Π΄Π΅Ρ„ΠΈΡ†ΠΈΡ‚ ΠΏΡ€ΠΎΠ»ΠΈΠ΄Π°Π·Ρ‹, Π΄Π΅Ρ„ΠΈΡ†ΠΈΡ‚ Π°Π΄Π΅Π½ΠΎΠ·ΠΈΠ½Π΄Π΅Π·Π°ΠΌΠΈΠ½Π°Π·Ρ‹ 2-Π³ΠΎ Ρ‚ΠΈΠΏΠ°, лизинуричСская Π½Π΅ΠΏΠ΅Ρ€Π΅Π½ΠΎΡΠΈΠΌΠΎΡΡ‚ΡŒ Π±Π΅Π»ΠΊΠ°, Π΄Π΅Ρ„ΠΈΡ†ΠΈΡ‚ Ξ±-ΠΌΠ°Π½Π½ΠΎΠ·ΠΈΠ΄Π°Π·Ρ‹). Π’ Π²ΠΈΠ΄Π΅ Ρ‚Π°Π±Π»ΠΈΡ†Ρ‹ прСдставлСны ΠΎΠ±ΠΎΠ±Ρ‰Π΅Π½Π½Ρ‹Π΅ клиничСскиС Π΄Π°Π½Π½Ρ‹Π΅ ΠΎ Π±ΠΎΠ»ΡŒΡˆΠΈΠ½ΡΡ‚Π²Π΅ извСстных Π²ΠΎΠ»Ρ‡Π°Π½ΠΎΡ‡Π½ΠΎΠΏΠΎΠ΄ΠΎΠ±Π½Ρ‹Ρ… синдромов ΠΈ ΠΈΡ… молСкулярных ΠΌΠ΅Ρ…Π°Π½ΠΈΠ·ΠΌΠ°Ρ….ΠŸΠ°Ρ‚ΠΎΠ³Π΅Π½Π΅Π· ΠΌΠ½ΠΎΠ³ΠΈΡ… Ρ„ΠΎΡ€ΠΌ ΠΌΠΎΠ½ΠΎΠ³Π΅Π½Π½Ρ‹Ρ… Π²ΠΎΠ»Ρ‡Π°Π½ΠΎΡ‡Π½ΠΎΠΏΠΎΠ΄ΠΎΠ±Π½Ρ‹Ρ… Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ находится Π² процСссС изучСния. ΠžΡΠ½ΠΎΠ²Π½Ρ‹ΠΌ ΠΏΡ€ΠΈΠ·Π½Π°ΠΊΠΎΠΌ, ΡΠ²ΠΈΠ΄Π΅Ρ‚Π΅Π»ΡŒΡΡ‚Π²ΡƒΡŽΡ‰ΠΈΠΌ Π² ΠΏΠΎΠ»ΡŒΠ·Ρƒ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΠ³ΠΎ ΠΌΠΎΠ½ΠΎΠ³Π΅Π½Π½ΠΎΠ³ΠΎ заболСвания Ρƒ больного Π‘ΠšΠ’, являСтся Π΅Π³ΠΎ Π½Π°Ρ‡Π°Π»ΠΎ Π² Ρ€Π°Π½Π½Π΅ΠΌ дСтском возрастС, особСнно Π² сочСтании с муТским ΠΏΠΎΠ»ΠΎΠΌ. Π’Π°ΠΊΠΆΠ΅ слСдуСт ΠΎΠ±Ρ€Π°Ρ‰Π°Ρ‚ΡŒ Π²Π½ΠΈΠΌΠ°Π½ΠΈΠ΅ Π½Π° отягощСнный сСмСйный Π°Π½Π°ΠΌΠ½Π΅Π·, Π² Ρ‚ΠΎΠΌ числС Π½Π° Ρ„Π°ΠΊΡ‚ близкородствСнного Π±Ρ€Π°ΠΊΠ°, ΡƒΡΡ‚ΠΎΠΉΡ‡ΠΈΠ²ΠΎΡΡ‚ΡŒ заболСвания ΠΊ стандартной Ρ‚Π΅Ρ€Π°ΠΏΠΈΠΈ ΠΈ Π½Π΅Ρ‚ΠΈΠΏΠΈΡ‡Π½ΡƒΡŽ симптоматику.

    Filamentation of focused and collimated laser beams in liquids

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    Experimental results of investigations into the transformation of the spectral and spatial characteristics of femtosecond collimated and focused Ti:Sapphire-laser beams with wavelengths of 800 and 400 nm upon filamentation in continuous liquid media are presented. It is shown that broadening of the laser pulse spectrum due to phase self-modulation in the medium with a cubic nonlinearity depends on the pulse power and beam diameter. Dependences of the number of filaments, width of laser radiation spectrum, nonlinear focusing distance, and diameter of the filamentation region on the laser pulse power are measured. The existence of a relative power interval in which the explosive growth of the number of filaments occurs, is established. Β© (2015) COPYRIGHT Society of Photo-Optical Instrumentation Engineers (SPIE). Downloading of the abstract is permitted for personal use only

    Preventive medicine of von Hippel-Lindau disease-associated pancreatic neuroendocrine tumors

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    Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel-Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10-75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2\u2009cm; P\u20091.5\u2009cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs <2.8\u2009cm vs 652.8\u2009cm (94% vs 85% by 10 years; P\u2009=\u20090.020; 80% vs 50% at 10 years; P\u2009=\u20090.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8\u2009cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs
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