29 research outputs found
Observation Of Very High Energy Cosmic-ray Families In Emulsion Chambers At High Mountain Altitudes (i)
Characteristics of cosmic-ray hadronic interactions in the 1015 - 1017 eV range are studied by observing a total of 429 cosmic-ray families of visible energy greater than 100 TeV found in emulsion chamber experiments at high mountain altitudes, Chacaltaya (5200 m above sea level) and the Pamirs (4300 m above sea level). Extensive comparisons were made with simulated families based on models so far proposed, concentrating on the relation between the observed family flux and the behaviour of high-energy showers in the families, hadronic and electromagnetic components. It is concluded that there must be global change in characteristics of hadronic interactions at around 1016 eV deviating from thise known in the accelerator energy range, specially in the forwardmost angular region of the collision. A detailed study of a new shower phenomenon of small-pT particle emissions, pT being of the order of 10 MeV/c, is carried out and its relation to the origin of huge "halo" phenomena associated with extremely high energy families is discussed as one of the possibilities. General characteristics of such super-families are surveyed. Β© 1992.3702365431Borisov, (1981) Nucl. Phys., 191 BBaybrina, (1984) Trudy FIAN 154, p. 1. , [in Russian], Nauka, MoscowLattes, Hadronic interactions of high energy cosmic-ray observed by emulsion chambers (1980) Physics Reports, 65, p. 151Hasegawa, ICR-Report-151-87-5 (1987) presented at FNAL CDF Seminar, , Inst. for Cosmic Ray Research, Univ. of TokyoCHACALTAYA Emulsion Chamber Experiment (1971) Progress of Theoretical Physics Supplement, 47, p. 1Yamashita, Ohsawa, Chinellato, (1984) Proc. 3rd Int. Symp. on Cosmic Rays and Particle Physics, p. 30. , Tokyo, 1984, Inst. for Cosmic Ray Research, Univ. of Tokyo(1984) Proc. 3rd Int. Symp. on Cosmic Rays and Particle Physics, p. 1. , Tokyo, 1984Baradzei, (1984) Proc. 3rd Int. Symp. on Cosmic Rays and Particle Physics, p. 136. , Tokyo, 1984Yamashita, (1985) J. Phys. Soc. Jpn., 54, p. 529Bolisov, (1984) Proc. 3rd Int. Symp. on Cosmic rays and Particle Physics, p. 248. , Tokyo, 1984, Inst. for Cosmic Ray Research, Univ. of TokyoTamada, Tomaszewski, (1988) Proc. 5th Int. Symp. on Very High Energy Cosmic-Ray Interactions, p. 324. , Lodz, 1988, Inst. for Cosmic Ray Research, Univ. of Tokyo, PolandHasegawa, (1989) ICR-Report-197-89-14, , Inst. for Cosmic Ray Research, Univ. of TokyoCHACALTAYA Emulsion Chamber Experiment (1971) Progress of Theoretical Physics Supplement, 47, p. 1Okamoto, Shibata, (1987) Nucl. Instrum. Methods, 257 A, p. 155Zhdanov, (1980) FIAN preprint no. 45, , Lebedev Physical Institute, MoscowSemba, Gross Features of Nuclear Interactions around 1015eV through Observation of Gamma Ray Families (1983) Progress of Theoretical Physics Supplement, 76, p. 111Nikolsky, (1975) Izv. Akad. Nauk. USSR Ser. Fis., 39, p. 1160Burner, Energy spectra of cosmic rays above 1 TeV per nucleon (1990) The Astrophysical Journal, 349, p. 25Takahashi, (1990) 6th Int. Symp. on Very High Energy Cosmic-ray Interactions, , Tarbes, FranceRen, (1988) Phys. Rev., 38 D, p. 1404Alner, The UA5 high energy simulation program (1987) Nuclear Physics B, 291 B, p. 445Bozzo, Measurement of the proton-antiproton total and elastic cross sections at the CERN SPS collider (1984) Physics Letters B, 147 B, p. 392Wrotniak, (1985) Proc. 19th Cosmic-Ray Conf. La Jolla, 1985, 6, p. 56. , NASA Conference Publication, Washington, D.CWrotniak, (1985) Proc. 19th Cosmic-Ray Conf. La Jolla, 1985, 6, p. 328. , NASA Conference Publication, Washington, D.CMukhamedshin, (1984) Trudy FIAN, 154, p. 142. , Nauka, Moscow, [in Russian]Dunaevsky, Pluta, Slavatinsky, (1988) Proc. 5th Int. Symp. on Very High Energy Cosmic-Ray Interactions, p. 143. , Lodz, 1988, Inst. of Physics, Univ. of Lodz, PolandKaidalov, Ter-Martirosyan, (1987) Proc. 20th Int. Cosmic-Ray Conf., Moscow, 1987, 5, p. 141. , Nauka, MoscowShabelsky, (1985) preprints LNPI-1113Shabelsky, (1986) preprints LNPI-1224, , Leningrad [in Russian]Hillas, (1979) Proc. 16th Int. Cosmic-Ray Conf., Kyoto, 6, p. 13. , Inst. for Cosmic Ray Research, Univ. of TokyoBorisov, (1987) Phys. Lett., 190 B, p. 226Hasegawa, Tamada, (1990) 6th Int. Symp. on Very High Energy Cosmic-Ray Interactions, , Tarbes, FranceSemba, Gross Features of Nuclear Interactions around 1015eV through Observation of Gamma Ray Families (1983) Progress of Theoretical Physics Supplement, p. 111Ren, (1988) Phys. Rev., 38 D, p. 1404Dynaevsky, Zimin, (1988) Proc. 5th Int. Symp. on Very High Energy Cosmic-Ray Interaction, p. 93. , Lodz, 1988, Inst. of Physics, Univ. of Lodz, PolandDynaevsky, (1990) Proc. 6th Int. Symp. on Very High Energy Cosmic-Ray Interactions, , Tarbes, France(1989) FIAN preprint no. 208, , Lebedev Physical Institute, Moscow(1990) Proc. 21st Int. Cosmic-Ray Conf., Adelaide, 8, p. 259. , Dept. Physics and Mathematical Physics, Univ. of Adelaide, AustraliaHasegawa, (1990) ICR-Report-216-90-9, , Inst. for Cosmic-Ray Research, Univ. of TokyoTamada, (1990) Proc. 21st Int. Cosmic-Ray Conf., Adelaide, 1990, 8. , Dept. Physics and Mathematical Physics, Univ. of Adelaide, AustraliaTamada, (1990) ICR-Report-216-90-9(1981) Proc. 17th Int. Cosmic-Ray Conf., Paris, 5, p. 291(1990) Proc. Int. Cosmic-Ray Conf., Adelaide, 1990, 8, p. 267. , Dept. Physics and Mathematical Physics, Univ. of Adelaide, Australia(1989) Inst. Nucl. Phys. 89-67/144, , preprint, Inst. Nucl. Phys., Moscow State UnivSmilnova, (1988) Proc. 5th Int. Sym. on Very High Energy Cosmic-Ray Interactions, p. 42. , Lodz, 1988, Inst. of Physics, Univ. of Lodz, PolandGoulianos, (1986) Proc. Workshop of Particle Simulation at High Energies, , University of Wisconsin, Madison, USAIvanenko, (1983) Proc. 18th Int. Cosmic-Ray Conf., Bangalore, 1983, 5, p. 274. , Tata Inst. Fundamental Research, Bombay, IndiaIvanenko, (1984) Proc. Int. Symp. on Cosmic-Rays and Particle Physics, p. 101. , Tokyo, 1984, Inst. for Cosmic Ray Research, Univ. of Tokyo(1988) 5th Int. Symp. on Very High Energy Cosmic-Ray Interactions, p. 180. , Lodz, 1988, Inst. of Physics, Univ. of Lodz, Poland(1990) Proc. 21st Int. Cosmic-Ray Conf., Adelaide, 1990, 8, p. 251. , Dept. Physics and Mathematical Physics, Univ. of Adelaide, Australia(1991) Izv. AN USSR No. 4, , to be publishedNikolsky, Shaulov, Cherdyntseva, (1990) FIAN preprint no. 140, , Lebedev Physical Institute, Moscow, [in Russian](1987) Proc. 20th Int. Cosmic-Ray Conf., Moscow, 1987, 5, p. 326. , Nauka, Mosco
Observation Of A High-energy Cosmic-ray Family Caused By A Centauro-type Nuclear Interaction In The Joint Emulsion Chamber Experiment At The Pamirs
An exotic cosmic-ray family event is observed in the large emulsion chamber exposed by the joint at the Pamirs (4360 m above sea level). The family is composed of 120Ξ³-ray-induced showers and 37 hadron-induced showers with individual visible energy exceeding 1 TeV. The decisive feature of the event is the hadron dominance: Ξ£EΞ³, Ξ£E(Ξ³) h, γEΞ³, γE(Ξ³) hγ, γEΞ³Β·RΞ³γ and γE(Ξ³)Β·Rhγ being 298 TeV, 476 TeV, 2.5 TeV, 12.9 TeV, 28.6 GeV m and 173 GeV m, respectively. Most probably the event is due to a Centauro interaction, which occured in the atmosphere at βΌ700 m above the chamber. The event will constitute the second beautiful candidate for a Centauro observed at the Pamirs. Β© 1987.1901-2226233Bayburina, (1981) Nucl. Phys. B, 191, p. 1Lattes, Fujimoto, Hasegawa, Hadronic interactions of high energy cosmic-ray observed by emulsion chambers (1980) Physics Reports, 65, p. 151(1984) Trudy FIAN, 154, p. 1Borisov, (1984) Proc. Intern. Symp. on Cosmic rays and particle physics, p. 3. , TokyoRen, (1985) 19th Intern. Cosmic ray Conf., 6, p. 317. , La JollaYamashita, (1985) 19th Intern. Cosmic ray Conf., 6, p. 364. , La JollaTamada, (1977) Nuovo Cimento, 41 B, p. 245T. Shibata et al., to be publishedHillas, (1979) 16th Intern. Cosmic ray Conf., 6, p. 13. , KyotoBattiston, Measurement of the proton-antiproton elastic and total cross section at a centre-of-mass energy of 540 GeV (1982) Physics Letters B, 117, p. 126UA5 Collab., G.J. Alner et al., preprint CERN-EP/85-62Taylor, (1976) Phys. Rev. D, 14, p. 1217Burnett, (1984) Proc. Intern. Symp. on Cosmic rays and particle physics, p. 468. , Toky
ΠΠΠΠΠΠ Π’ΠΠΠΠΠΠ¦ΠΠ ΠΠ‘ΠΠ₯ΠΠΠΠΠΠ§ΠΠ‘ΠΠΠ₯ Π‘Π’Π ΠΠ’ΠΠΠΠ Π‘ΠΠ’ΠΠΠΠ ΠΠΠ’ΠΠΠΠΠ‘Π’Π Π‘Π’Π£ΠΠΠΠ’ΠΠ
This article is devoted to the analysis of trends in psychological strategies of network behavior at the student age. The article discusses the strategies of activity, attitudes towards the Internet, and self-presentation in Internet communication among students.ΠΠ°Π½Π½Π°Ρ ΡΡΠ°ΡΡΡ ΠΏΠΎΡΠ²ΡΡΠ΅Π½Π° Π°Π½Π°Π»ΠΈΠ·Ρ ΡΠ΅Π½Π΄Π΅Π½ΡΠΈΠΉ ΠΏΡΠΈΡ
ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΉ ΡΠ΅ΡΠ΅Π²ΠΎΠ³ΠΎ ΠΏΠΎΠ²Π΅Π΄Π΅Π½ΠΈΡ Π² ΡΡΡΠ΄Π΅Π½ΡΠ΅ΡΠΊΠΎΠΌ Π²ΠΎΠ·ΡΠ°ΡΡΠ΅. Π ΡΡΠ°ΡΡΠ΅ ΡΠ°ΡΡΠΌΠ°ΡΡΠΈΠ²Π°ΡΡΡΡ ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΈ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ, ΡΡΡΠ°Π½ΠΎΠ²ΠΊΠΈ ΠΏΠΎ ΠΎΡΠ½ΠΎΡΠ΅Π½ΠΈΡ ΠΊ ΠΠ½ΡΠ΅ΡΠ½Π΅ΡΡ ΠΈ ΡΠ°ΠΌΠΎΠΏΡΠ΅Π·Π΅Π½ΡΠ°ΡΠΈΡ Π² ΠΠ½ΡΠ΅ΡΠ½Π΅Ρ-ΠΎΠ±ΡΠ΅Π½ΠΈΠΈ Ρ ΡΡΡΠ΄Π΅Π½ΡΠ΅ΡΠΊΠΎΠΉ ΠΌΠΎΠ»ΠΎΠ΄Π΅ΠΆΠΈ
ΠΠΠΠΠ‘ΠΠ«Π Π£ΠΠΠΠΠΠΠΠ― Π‘Π’Π£ΠΠΠΠ’ΠΠ Π Π‘ΠΠ’ΠΠΠΠ ΠΠΠΠΠΠΠΠΠ
The article deals with such concepts as "basic beliefs" and "strategies of network activity". The authors present some results of empirical research of students ' basic beliefs in the conditions of virtual communication, reflecting the specifics of the relationship between the preferences of network behavior strategies with different basic beliefs.Π ΡΡΠ°ΡΡΠ΅ ΡΠ°ΡΡΠΌΠ°ΡΡΠΈΠ²Π°ΡΡΡΡ ΡΠ°ΠΊΠΈΠ΅ ΠΏΠΎΠ½ΡΡΠΈΡ, ΠΊΠ°ΠΊ Β«Π±Π°Π·ΠΈΡΠ½ΡΠ΅ ΡΠ±Π΅ΠΆΠ΄Π΅Π½ΠΈΡΒ» ΠΈ Β«ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΈ ΡΠ΅ΡΠ΅Π²ΠΎΠΉ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈΒ». Π ΡΠ°Π±ΠΎΡΠ΅ Π°Π²ΡΠΎΡΠ°ΠΌΠΈ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΠΎΡΠ΄Π΅Π»ΡΠ½ΡΠ΅ ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΡΠΌΠΏΠΈΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ Π±Π°Π·ΠΈΡΠ½ΡΡ
ΡΠ±Π΅ΠΆΠ΄Π΅Π½ΠΈΠΉ ΡΡΡΠ΄Π΅Π½ΡΠΎΠ² Π² ΡΡΠ»ΠΎΠ²ΠΈΡΡ
Π²ΠΈΡΡΡΠ°Π»ΡΠ½ΠΎΠΉ ΠΊΠΎΠΌΠΌΡΠ½ΠΈΠΊΠ°ΡΠΈΠΈ, ΠΎΡΡΠ°ΠΆΠ°ΡΡΠΈΠ΅ ΡΠΏΠ΅ΡΠΈΡΠΈΠΊΡ Π²Π·Π°ΠΈΠΌΠΎΡΠ²ΡΠ·ΠΈ ΠΏΡΠ΅Π΄ΠΏΠΎΡΡΠ΅Π½ΠΈΡ ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΉ ΡΠ΅ΡΠ΅Π²ΠΎΠ³ΠΎ ΠΏΠΎΠ²Π΅Π΄Π΅Π½ΠΈΡ Ρ ΡΠ°Π·Π½ΡΠΌΠΈ Π±Π°Π·ΠΈΡΠ½ΡΠΌΠΈ ΡΠ±Π΅ΠΆΠ΄Π΅Π½ΠΈΡΠΌΠΈ
ΠΠ·Π°ΠΈΠΌΠΎΡΠ²ΡΠ·Ρ ΠΌΠ°ΡΡΠΎΠ²ΠΎΠΉ ΠΊΡΠ»ΡΡΡΡΡ ΠΈ ΠΈΠ½Π΄ΡΡΡΡΠΈΠΈ Π²ΠΈΠ΄Π΅ΠΎΠΈΠ³Ρ: ΠΏΠΎΠ»ΠΈΡΠΈΠΊΠΎ-ΡΠΈΠ»ΠΎΡΠΎΡΡΠΊΠΈΠΉ Π°ΡΠΏΠ΅ΠΊΡ
ΠΠ°ΠΊ ΡΠ»Π΅ΠΌΠ΅Π½Ρ ΠΌΠ°ΡΡΠΎΠ²ΠΎΠΉ ΠΊΡΠ»ΡΡΡΡΡ Π²ΠΈΠ΄Π΅ΠΎΠΈΠ³ΡΡ ΠΏΠΎΠ»ΡΠ·ΡΡΡΡΡ Π±ΠΎΠ»ΡΡΠΈΠΌ ΡΡΠΏΠ΅Ρ
ΠΎΠΌ, ΠΈΠΌΠΈ ΠΈΠ½ΡΠ΅ΡΠ΅ΡΡΡΡΡΡ ΡΠ°Π·Π½ΡΠ΅ ΡΠ»ΠΎΠΈ Π½Π°ΡΠ΅Π»Π΅Π½ΠΈΡ. ΠΠ½ΠΈ Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΡΡΡ Ρ Π±ΠΎΠ»ΡΡΠΈΠΌ ΠΊΠΎΠ»ΠΈΡΠ΅ΡΡΠ²ΠΎΠΌ Π»ΡΠ΄Π΅ΠΉ, ΡΡΠΎ Π΄Π΅Π»Π°Π΅Ρ ΠΈΡ
Π²Π°ΠΆΠ½ΡΠΌ ΠΎΠ±ΡΠ΅ΠΊΡΠΎΠΌ ΠΈΠ·ΡΡΠ΅Π½ΠΈΡ ΠΏΠΎΠ»ΠΈΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΠΌ ΡΠΎΠΎΠ±ΡΠ΅ΡΡΠ²ΠΎΠΌ. ΠΡ
ΠΏΠΎΡΠ΅Π½ΡΠΈΠ°Π» ΠΌΠ°Π»ΠΎΠΈΠ·ΡΡΠ΅Π½ Π² ΠΊΠΎΠ½ΡΠ΅ΠΊΡΡΠ΅ ΠΌΠ°ΡΡΠΎΠ²ΠΎΠΉ ΠΊΡΠ»ΡΡΡΡΡ
NIMANN-PICK TYPE C DISEASE. CLINICAL CASE STUDIES
Nimann-Pick Type C Disease is a hereditary progressive disease of the nervous system from the group of lysosomal accumulation diseases resulting from abnormal subcellular lipid distribution, which leads to the accumulation of cholesterol and glycosphingolipid in the brain and other body tissues. Clinical presentation of the disease is varied and manifested as progressive cerebellar, cognitive disorders, vertical ophthalmoparesis, frequently combined with disorders of the liver, spleen and lungs. This article outlines the key mechanisms of Nimann-Pick type C disease pathogenesis, leading clinical presentations depending on the time the disease manifested it self and key therapy approaches. We described two cases of this disease registered in Russia. It provides a comparative clinical analysis with already published data. To date, a pathogenetic treatment course has been developed for this disease, a timely detection of this pathology is, therefore, important to the burdened family. This representative article on modern data on diagnostics and treatment of NPC is intended for neurologists, pediatricians, psychiatrists and geneticists. Key words: neurodegenerative disease, Nimann-Pick type C disease, convulsions, ataxia, vertical ophthalmoparesis, loss of acquired skills. (Pediatric Pharmacology. β 2010; 7(5):48-53
Π‘Π ΠΠΠΠΠ’ΠΠΠ¬ΠΠΠ ΠΠΠ£Π§ΠΠΠΠ Π€ΠΠ ΠΠΠΠΠΠΠΠΠ’ΠΠΠ ΠΠ€ΠΠΠΠ‘ΠΠ¦ΠΠΠ Π Π‘ΠΠΠΠΠΠΠΠ Π ΠΠΠΠ‘ΠΠΠΠΠ¬ΠΠΠ Π€ΠΠ ΠΠ Π ΠΠΠ‘ΠΠΠ ΠΠΠΠΠ’Π ΠΠ ΠΠΠΠ«Π₯ ΠΠ«Π¨ΠΠ₯ ΠΠ Π ΠΠΠΠΠΠΠΠ Per Os
Aim: to study features of pharmacokinetics of ofloxacin as a part of anion PEGylated niosomes on a basis of sorbitan monostearate (Span 60) to experimental white mice per os. Materials and methods: ofloxacin was entrapped in niosomes consisting of Span 60, cholesterol, PEG 4000 and dicetylphosphate. Sizes of niosomes estimated by means of probe microscopy. Efficiency of inclusion of an antibiotic in niosomes defined after removal of free drug by a centrifugation. The analysis of the quantitative contents of ofloxacin in samples carried out a method of a high performance liquid chromatography. Results: we studied the main pharmacokinetic parameters of ofloxacin when used free and niosomal forms of antibiotic to experimental white mice per os. It is shown that use of oral niosomal forms leads to decrease of maximal concentration in serum and increase of ofloxacin half-life by 7,4 times in average compared to the free form. It is determined that bioavailability of ofloxacin in the niosomal form is 154% relative to the free form of the antibiotic. Conclusions: niosomal microcontainers are perspective technology of encapsulation and the directed transport of antibacterial preparations through biological barriers. Using of niosomal formulation of ofloxacin is able to afford to increase considerably efficiency of treatment in comparison with a free form and significantly decrease negative effects of antibiotic therapy.Π¦Π΅Π»Ρ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ: ΠΈΠ·ΡΡΠΈΡΡ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΡΠ°ΡΠΌΠ°ΠΊΠΎΠΊΠΈΠ½Π΅ΡΠΈΠΊΠΈ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° Π² ΡΠΎΡΡΠ°Π²Π΅ Π°Π½ΠΈΠΎΠ½Π½ΡΡ
ΠΠΠ-ΡΠΎΠ΄Π΅ΡΠΆΠ°ΡΠΈΡ
Π½ΠΈΠΎΡΠΎΠΌ Π½Π° ΠΎΡΠ½ΠΎΠ²Π΅ ΡΠΎΡΠ±ΠΈ-ΡΠ°Π½Π° ΠΌΠΎΠ½ΠΎΡΡΠ΅Π°ΡΠ°ΡΠ° (Span 60) ΠΏΡΠΈ ΠΏΠ΅ΡΠΎΡΠ°Π»ΡΠ½ΠΎΠΌ Π²Π²Π΅Π΄Π΅Π½ΠΈΠΈ ΡΠΊΡΠΏΠ΅ΡΠΈΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΠΌ Π±Π΅Π»ΡΠΌ ΠΌΡΡΠ°ΠΌ. ΠΠ°ΡΠ΅ΡΠΈΠ°Π»Ρ ΠΈ ΠΌΠ΅ΡΠΎΠ΄Ρ: ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½ Π²ΠΊΠ»ΡΡΠ°Π»ΠΈ Π² Π½ΠΈΠΎΡΠΎΠΌΡ, ΡΠΎΡΡΠΎΡΡΠΈΠ΅ ΠΈΠ· Span 60 Ρ
ΠΎΠ»Π΅ΡΡΠ΅ΡΠΈΠ½Π°, ΠΠΠ 4000 ΠΈ Π΄ΠΈΡΠ΅ΡΠΈΠ»ΡΠΎΡΡΠ°ΡΠ°. Π Π°Π·ΠΌΠ΅ΡΡ Π½ΠΈΠΎΡΠΎΠΌ ΠΎΡΠ΅Π½ΠΈΠ²Π°Π»ΠΈ Ρ ΠΏΠΎΠΌΠΎΡΡΡ Π·ΠΎΠ½Π΄ΠΎΠ²ΠΎΠΉ ΠΌΠΈΠΊΡΠΎΡΠΊΠΎΠΏΠΈΠΈ. ΠΡΡΠ΅ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ Π²ΠΊΠ»ΡΡΠ΅Π½ΠΈΡ Π°Π½ΡΠΈΠ±ΠΈΠΎΡΠΈΠΊΠ° Π² Π½ΠΈΠΎΡΠΎΠΌΡ ΠΎΠΏΡΠ΅Π΄Π΅Π»ΡΠ»ΠΈ ΠΏΠΎΡΠ»Π΅ ΡΠ΄Π°Π»Π΅Π½ΠΈΡ ΡΠ²ΠΎΠ±ΠΎΠ΄Π½ΠΎΠ³ΠΎ Π΄Π΅ΠΉΡΡΠ²ΡΡΡΠ΅Π³ΠΎ Π²Π΅ΡΠ΅ΡΡΠ²Π° ΠΏΡΡΠ΅ΠΌ ΡΠ΅Π½ΡΡΠΈΡΡΠ³ΠΈΡΠΎ-Π²Π°Π½ΠΈΡ. ΠΠ½Π°Π»ΠΈΠ· ΠΊΠΎΠ»ΠΈΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ³ΠΎ ΡΠΎΠ΄Π΅ΡΠΆΠ°Π½ΠΈΡ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° Π² ΠΎΠ±ΡΠ°Π·ΡΠ°Ρ
ΠΎΡΡΡΠ΅ΡΡΠ²Π»ΡΠ»ΠΈ ΠΌΠ΅ΡΠΎΠ΄ΠΎΠΌ Π²ΡΡΠΎΠΊΠΎΡΡΡΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΉ ΠΆΠΈΠ΄ΠΊΠΎΡΡΠ½ΠΎΠΉ Ρ
ΡΠΎΠΌΠ°ΡΠΎΠ³ΡΠ°-ΡΠΈΠΈ. Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ: ΠΈΠ·ΡΡΠ΅Π½Ρ ΠΎΡΠ½ΠΎΠ²Π½ΡΠ΅ ΡΠ°ΡΠΌΠ°ΠΊΠΎΠΊΠΈΠ½Π΅ΡΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΏΠ°ΡΠ°ΠΌΠ΅ΡΡΡ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° ΠΏΡΠΈ Π²Π²Π΅Π΄Π΅Π½ΠΈΠΈ ΡΠΊΡΠΏΠ΅ΡΠΈΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΠΌ Π±Π΅Π»ΡΠΌ ΠΌΡΡΠ°ΠΌ per os ΡΠ²ΠΎΠ±ΠΎΠ΄Π½ΠΎΠΉ ΠΈ Π½ΠΈΠΎΡΠΎΠΌΠ°Π»ΡΠ½ΠΎΠΉ ΡΠΎΡΠΌΡ Π°Π½ΡΠΈΠ±ΠΈΠΎΡΠΈΠΊΠ°. ΠΠΎΠΊΠ°Π·Π°Π½ΠΎ, ΡΡΠΎ ΠΏΡΠΈ ΠΏΠ΅ΡΠΎΡΠ°Π»ΡΠ½ΠΎΠΌ Π²Π²Π΅Π΄Π΅Π½ΠΈΠΈ Π½ΠΈΠΎΡΠΎΠΌΠ°Π»ΡΠ½ΠΎΠΉ ΡΠΎΡΠΌΡ Π½Π°Π±Π»ΡΠ΄Π°Π΅ΡΡΡ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ ΠΌΠ°ΠΊΡΠΈΠΌΠ°Π»ΡΠ½ΠΎΠΉ ΠΊΠΎΠ½ΡΠ΅Π½ΡΡΠ°ΡΠΈΠΈ Π² ΡΡΠ²ΠΎΡΠΎΡΠΊΠ΅ ΠΊΡΠΎΠ²ΠΈ ΠΈ ΡΠ²Π΅Π»ΠΈΡΠ΅Π½ΠΈΠ΅ Π²ΡΠ΅ΠΌΠ΅Π½ΠΈ ΠΏΠΎΠ»ΡΠ²ΡΠ²Π΅Π΄Π΅Π½ΠΈΡ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° Π² ΡΡΠ΅Π΄Π½Π΅ΠΌ Π² 7,4 ΡΠ°Π·Π° ΠΏΠΎ ΡΡΠ°Π²Π½Π΅Π½ΠΈΡ ΡΠΎ ΡΠ²ΠΎΠ±ΠΎΠ΄Π½ΠΎΠΉ ΡΠΎΡΠΌΠΎΠΉ. Π£ΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΎ, ΡΡΠΎ Π±ΠΈΠΎΠ΄ΠΎΡΡΡΠΏΠ½ΠΎΡΡΡ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° Π² Π½ΠΈΠΎΡΠΎΠΌΠ°Π»ΡΠ½ΠΎΠΉ ΡΠΎΡΠΌΠ΅ ΡΠΎΡΡΠ°Π²Π»ΡΠ΅Ρ 154% ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎ ΡΠ²ΠΎΠ±ΠΎΠ΄Π½ΠΎΠΉ ΡΠΎΡΠΌΡ Π°Π½ΡΠΈΠ±ΠΈΠΎΡΠΈΠΊΠ°. ΠΡΠ²ΠΎΠ΄Ρ: Π½ΠΈΠΎΡΠΎΠΌΠ°Π»ΡΠ½ΡΠ΅ ΠΌΠΈΠΊΡΠΎΠΊΠΎΠ½ΡΠ΅ΠΉΠ½Π΅ΡΡ ΡΠ²Π»ΡΡΡΡΡ ΠΏΠ΅ΡΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΉ ΡΠ΅Ρ
Π½ΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ ΠΈΠ½ΠΊΠ°ΠΏΡΡΠ»ΠΈΡΠΎΠ²Π°Π½ΠΈΡ ΠΈ Π½Π°ΠΏΡΠ°Π²Π»Π΅Π½Π½ΠΎΠ³ΠΎ ΡΡΠ°Π½ΡΠΏΠΎΡΡΠ° Π°Π½ΡΠΈΠ±Π°ΠΊΡΠ΅ΡΠΈΠ°Π»ΡΠ½ΡΡ
ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ² ΡΠ΅ΡΠ΅Π· Π±ΠΈΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΠ΅ Π±Π°ΡΡΠ΅ΡΡ. ΠΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠ΅ Π½ΠΈΠΎΡΠΎΠΌΠ°Π»ΡΠ½ΠΎΠΉ ΡΠΎΡΠΌΡ ΠΎΡΠ»ΠΎΠΊΡΠ°ΡΠΈΠ½Π° ΠΌΠΎΠΆΠ΅Ρ ΠΏΠΎΠ·Π²ΠΎ-Π»ΠΈΡΡ Π·Π½Π°ΡΠΈΡΠ΅Π»ΡΠ½ΠΎ ΠΏΠΎΠ²ΡΡΠΈΡΡ ΡΡΡΠ΅ΠΊΡΠΈΠ²Π½ΠΎΡΡΡ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΠΎ ΡΡΠ°Π²Π½Π΅Π½ΠΈΡ ΡΠΎ ΡΠ²ΠΎΠ±ΠΎΠ΄Π½ΠΎΠΉ ΡΠΎΡΠΌΠΎΠΉ, Π° ΡΠ°ΠΊΠΆΠ΅ ΡΠΏΠΎΡΠΎΠ±ΡΡΠ²ΠΎΠ²Π°ΡΡ Π·Π½Π°ΡΠΈΡΠ΅Π»ΡΠ½ΠΎΠΌΡ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΡ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡ Π½Π΅Π³Π°ΡΠΈΠ²Π½ΡΡ
ΡΡΡΠ΅ΠΊΡΠΎΠ² ΠΏΡΠΈ Π°Π½ΡΠΈΠ±ΠΈΠΎΡΠΈΠΊΠΎΡΠ΅ΡΠ°ΠΏΠΈΠΈ.
NIMANN-PICK TYPE C DISEASE. CLINICAL CASE STUDIES
Nimann-Pick Type C Disease is a hereditary progressive disease of the nervous system from the group of lysosomal accumulation diseases resulting from abnormal subcellular lipid distribution, which leads to the accumulation of cholesterol and glycosphingolipid in the brain and other body tissues. Clinical presentation of the disease is varied and manifested as progressive cerebellar, cognitive disorders, vertical ophthalmoparesis, frequently combined with disorders of the liver, spleen and lungs. This article outlines the key mechanisms of Nimann-Pick type C disease pathogenesis, leading clinical presentations depending on the time the disease manifested it self and key therapy approaches. We described two cases of this disease registered in Russia. It provides a comparative clinical analysis with already published data. To date, a pathogenetic treatment course has been developed for this disease, a timely detection of this pathology is, therefore, important to the burdened family. This representative article on modern data on diagnostics and treatment of NPC is intended for neurologists, pediatricians, psychiatrists and geneticists. Key words: neurodegenerative disease, Nimann-Pick type C disease, convulsions, ataxia, vertical ophthalmoparesis, loss of acquired skills. (Pediatric Pharmacology. β 2010; 7(5):48-53