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Mahlinovskaya, G.K. Lapshina, S.G.
Advantages and disadvantages of serial and parallel data transmission fluid. 9.6 Which type (or types) of serial transmission is appropriate for video transmission? Have data to send? For a keyboard connection to a computer?
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]. Rukovodstvo po ekspluatacii un 053 number. 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. 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. Privodim sobstvennoe nablyudenie. 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.