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Mahlinovskaya, G.K. Lapshina, S.G. Kulakova, T.D. Tihonova Detskaya gorodskaya klinicheskaya bol'nica N 9 im. Speranskogo, Moskva Klyuchevye slova: - redkoe zabolevanie, otnositsya k gruppe boleznei, svyazannyh s narusheniem obmena lipidov [1, 2].

Metabolicheskim defektom yavlyaetsya otsutstvie kisloi fosfatazy, vedushee k nakopleniyu holesterina, ego efirov, trigliceridov v teh tkanyah, gde naibolee intensivno proishodit ih sintez, - v kore nadpochechnikov, pecheni, slizistoi kishechnika [3- 7]. Otlozhenie lipidov soprovozhdaetsya uvelicheniem pecheni, selezenki, limfouzlov.

-borby-s-diabetom-chto-nuzhno-znat-o-bolezni/ 2012-11-14T10:15:00+02:00. Free Online Website Malware Scanner - quttera.com.

Patognomonichnym yavlyaetsya nakoplenie bol'shogo kolichestva kal'ciya v zonah zhirovyh nekrozov v nadpochechnikah, chto delaet vozmozhnym prizhiznennuyu diagnostiku etogo zabolevaniya rentgenologicheskimi metodami (obzornyi spisok organov bryushnoi polosti, komp'yuternaya tomografiya). V perifericheskoi krovi poyavlyayutsya vakuolizirovannye limfocity, neredko anemiya, v kostnom mozge - penistye kletki. Alazhillya, klinicheskie simptomy razvivayutsya v pervye dni ili nedeli zhizni [1]. Rvota, diareya bystro privodyat k gipotrofii, cherez neskol'ko nedel' otmechaetsya razvernutaya kartina:, uvelichenie zhivota, pecheni i selezenki. Zabolevanie fatal'no, letal'nyi ishod nastupaet obychno v pervye 6 mes zhizni, nasledovanie po autosomno-recessivnomu tipu. Instrukciya po pozharnoj bezopasnosti dlya masterskoj. Privodim sobstvennoe nablyudenie.

Steinberg absolute vst instrument collection free. Steinberg Absolute 2 vst instrument collection review and how to get it. When it comes to software for making music, it’s often the case that you’re going to have to pay a premium price for a premium piece of software. Absolute 4 VST Sound Instrument Collection. Consisting of the top range of Steinberg's acclaimed VST instruments, Absolute 4 VST Instrument Collection offers over 6,800 presets and over 100 GB of first-class sounds, VST technology and advanced sound design. Absolute 2 VST Instrument Collection from Steinberg overview. Absolute 2 VST Instrument Collection from Steinberg overview. No thanks Try it free. Absolute 2 VST Instrument Collection Updates and Downloads. The full installer for Absolute 2 is available via the Steinberg Download Assistant. VSTi AU Patcher.

Gospitalizirovana v Detskuyu gorodskuyu klinicheskuyu bol'nicu N 9 v vozraste 3 mes po povodu gepatosplenomegalii,, gipotrofii. Devochka rodilas' u molodyh zdorovyh roditelei, ot pervoi beremennosti, protekavshei s anemiei, ugrozoi preryvaniya vo vtoroi polovine, otmechalas' kal'cinaciya placenty. Rody v srok, massa tela 3300 g, dlina 52 sm. V svyazi s podozreniem na aspiraciyu molokom v vozraste 2 ned rebenok nahodilsya v stacionare, gepatosplenomegaliya i anemiya togda otsutstvovali. Vskarmlivalas' molochnymi smesyami, otmechalas' umerennaya gipotrofiya. Psihomotornoe razvitie sootvetstvovalo vozrastu.

V 3-mesyachnom vozraste vyyavlena anemiya (87 g/l), uvelichenie pecheni i selezenki. Pri gospitalizacii devochka aktivnaya, veselaya, appetit ne narushen, srygivaniya redkie, otmechalas' subikterichnost' kozhi i skler. Vedushimi byli priznaki portal'noi gipertenzii, gipotrofii II stepeni i anemiya. Zhivot rezko uvelichen v ob'eme, na perednei poverhnosti zhivota razvita venoznaya set', pechen' massivnaya, plotnaya, s rovnym kraem, uvelicheniem obeih dolei, vystupala iz-pod kraya rebernoi dugi po perednei aksillyarnoi linii na 7 sm, po sredneklyuchichnoi linii - na 6 sm, po srednei linii zhivota - na 2/3. Selezenka plotnoi konsistencii pal'pirovalas' na 3 sm iz-pod kraya rebernoi dugi. Stul 3-4 raza v sutki neperevarennyi, zhidkii, s nebol'shim kolichestvom slizi, okrashennyi. Mocha svetlaya, diurez adekvatnyi.

Analiz mochi normal'nyi. V analize krovi: Hb 102 g/l, er. 3,1•10 12l -1, rc. 180•10 9l -1, l.

10,2•10 9l -1, p. 2%; SOE 39 mm/ch.