V poslednie dni prisoedinilas' pnevmoniya. V vozraste 5. Istoriya bolezni yavlyaetsya yarkoi illyustraciei prizhiznennoi diagnostiki bolezni Volmana. Rossiiskii vestnik perinatologii i pediatrii, N4-2000, s.40-42. Write something about yourself. No need to be fancy, just an overview. No Archives Categories.

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.

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.

Vladimir Ruzicka played in four leagues over the course of his career. Learn more about them from these League History pages. Czechoslovakia League. National Hockey League. Switzerland National League A. Czech Republic League. Team Information. Klyuch aktivacii dlya movavi slajd shou download.

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. 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. Edinichnye limfocity imeyut vakuol'nyi vid. Bilirubin - 30 mkmol'/l, svyazannaya frakciya - 25 mkmol'/l. Holesterin - 6,8 mkmol'/l, -lipoproteidy - 78 ed.