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Preddushaya Sleduyushtaya



[] bolezny

Cm. ij metafizarnaya.

Pallistera

Cm. ѳ Shintselya.

ѳ Pallistera W

Nasleduetsya predpolozhitelyno X-stseplennomu retsessivnomu . Haraktern umerennaya umstvennaya otstalosty, sudorogi, , povshenny mshechny rasshelin Neba gub, 볳 ( konchik vsokiy , , chelyusty ) ( verhnih konechnostey, ukorochenie iskrivleniya loktevoy ).

Mogwai voznikaty obespechenii venoznogo predplechiy.

Palytaufa-Shternberga bolezny

Cm. bolezny.

Panarterit mnozhestvenny obliteriruyushtiy

Cm. bolezny.

nasledstvenny

Nasleduetsya -dominantnomu . Proyavlyaetsya opoyasvayushtimi bolyami zhirnoy . soprovozhdayushtiesya toshnotoy rvotoy, narastayut 24-48 stihayut cherez neskolyko dney. obostreniii povshaetsya uroveny amilaz lipaz . Razvivayushtiysya podzheludochnoy zhelez (vozmozhno poyavlenie kalytsifikatov) privodit narusheniyu .. kzokrinnoy ndokrinnoy .

ostry

Obchno voznikaet znachitelynom alkogolya, Zhelcho- , tupoy TRAVEL zadney penetriru. yushtey dvenadtsatiperstnoy . Otmechayutsya povshenie amilaz plazm, vrazhenne vpotnoy , kotory prichinoy dhatelynoy nedostatochnosti, leykotsitoz kishechnaya neprohodimosty. Gipotenziya gipovolemiya svyazan kssudatsiey plazm podzheludochnoy zhelez. dlitelynoy vozmozhna Kics. rezulytate gipokalytsiemii razvivaetsya . Tyazhely gemorragicheskiy vzvaty diabeticheskuyu .

Trebuetsya postoyanny nazogastralyny , korrektsiya vodnolektrolitnh KOS urovnya . Vozmozhn tyazhelaya 쳳 ustranenii ostatochnoy.

hronicheskiy

Chashte vsego hronicheskih alkogolikov. plazm obchno normalynaya, bilirubinaemia (~ 10% bolynh). Zhirovogo belkovogo otmechayutsya porazhenii> 80% zhelez. Obchno soprovozhdaetsya saharnm diabetom, zhirovm pererozhdeniem ..

narushenii vozmozhna povshennaya chuvstvitelynosty preparatam, podvergayushtimsya .

Pannikulit lihoradyashtiy retsediviruyushtiy nenagnaivayushtiysya

Cm. -Krischena bolezny.

Pantsitopenii

Cm. .

bulybarny progressiruyushtiy

Cm. Neyronov motornh bolezny. semeyne periodicheskie

Harakterizuyutsya voznikayushtimi pristupami mshechnoy paralichami. Razlichayut form :. Giperkaliemicheskaya (K +> 5,5 mkv / ) normokaliemicheskaya ( 3 5,5 mkv / ) gipokaliemicheskaya (nepravilyno diagnostirovan holodovaya paramiotoniya Povshenie svorotke otmechaetsya tolyko pristupov promezhutkah uroveny norm.

Otmechaetsya povshennaya chuvstvitelynosty suktsinilholinu. DLYA pizodov mshechnoy obchno ispolyzuyut atsetozolamid takzhe salybutamol . Povshenny trebuet KG. golodanii rekomenduyut glyukoz. Sleduet podderzhivaty normalynuyu temperaturu .

gipokaliemicheskiy semeyny periodicheskiy ( gipokaliemicheskiy periodicheskiy)

Nasleduetsya -dominantnomu . Proyavlyaetsya pristupami obchno vozraste 20-35 Chron. Dlitelynosty kolebletsya neskolyko neskolykih dney. Tyazhesty varyiruet legkogo prehodyashtego otdelynh Grupp mshts polnogo . Dhatelynaya porazhaetsya . Provotsiruyut pristup dlitelyny nagruzki, plotny uglevodnoy glyukoz . postoyannogo gipokaliemicheskogo . vyavlyayutsya vyaly proksimalynoy grupp mshts, , povshenie zubtsa Q zubtsa P KG. svorottse 2-2,5 mkv / .

Korrektsiyu soderzhaniya vpolnity . vzvaty perifericheskiy . Mshechne relaksant Inogda vzvayut ostatochnuyu relaksatsiyu. Imeetsya soobshtenie uspeshnom ispolyzovanii . Trebuetsya neyromshechnogo KG. 糳 isklyuchaty kotore snizity uroveny : metabolicheskits , glyukoz . Rekomenduetsya soderzhaniya dlitelynoy 糳. Sleduet isklyuchity nagruzku uglevodami gipotermiyu. Pochechny kanalytsevy hronicheskaya Mughals takzhe vzvaty mshechnuyu slabosty dannoy 㳳.

normokaliemicheskiy periodicheskiy semeyny

Provotsiruetsya obchno priemom alkogolya, nagruzkoy, motsionalynm stressom. Dlitelynosty mshechnoy varyiruet prodolzhatysya 2 NEDELYO. Pristup soprovozhdayutsya .

provedeniyu 糳 analogichn takovm gipoka / temicheskoy .

drozhatelyny

Cm. bolezny.

Levandovskogo Holodovoy

Cm. Paramiotoniya vrozhdennaya.

periodicheskiy paramiotonicheskiy

Cm. Paramiotoniya vrozhdennaya.

semeyny periodicheskiy

giperkaliemicheskom vozmozhen ⸢ ispolyzovanii relaksantov.

tserebralyny

Politiologicheskaya 㳳 obaedinyayushtaya motornoy rezulytate - perinatalynogo . progressiruyut varyiruyut. Chashte vsego prichinami vnutricherepne krovoizliyaniya, vnutriutrobne toksin, vrozhdenne gipoksiya, novorozhdennh , . Otmechayutsya sleduyushtie rasstroystva: spasticheskie (75%) vovlecheniem odnoy Vseh konechnostey, (20%) ataksiyu (1-2%). Soputstvuyushtie defekt vklyuchayut sudorogi (60%), zaderzhku umstvennogo ; spasticheskuyu tetraplegiyu chuvstitelynosti.

timi detymi bvaet nevozmozhno kontaktirovaty. tom anesteziologu pomochy Lyudi, osushtestvlyayushtie uhod . Anesteziologicheskie problem svyazan lekarstvennoy terapiey. Dlitelynoe antikonvulysantov izmenity nekotorh anestetikov . sozdayut ukladke bolynh operatsionnom . udelyayut prolezhney.

Paramiotoniya vrozhdennaya (ylenburga bolezny, ylenburga paramiotoniya, Levandovskogo Holodovoy, periodicheskiy paramiotonicheskiy)

ylenburg . ( .) (1840-1917) - nemetskiy . Levandovskiy . ( .) (1879-1935) - nemetskiy .

miotonicheskiy nasleduemy -dominantnomu . Simptom miotonii vrazhen , holode. Vozmozhn pizod vyalogo , svyazannogo ohlazhdeniem miotoniey. Pristup soprovozhdayutsya kolebaniyami urovnya svorotki tendentsiey povsheniyu. Zabolevanie progressiruet, gipertrofiya mshts otsutstvuyut.

Otmechaetsya povshennaya chuvstvitelynosty K , vozmozhn lektrolitne . Galotan ispolyzovaty. temperatur 糳 .



Nevrologicheskoe rasstroystvo, soprovozhdayushteesya vegetativnmi narusheniyami, obrazovaniem prolezhney. Vozmozhno vvedeniya suksametoniya. Zhelatelyno monitorirovanie neyromshechnogo . Mnogie problem shodn takovmi (. ), HQ vrazhen menyshey .

bolezny ( drozhatelyny)

D. (Parkinson J.) (1755-1824) - angliyskiy .

Degenerativnoe zabolevanie harakterizuyushteesya poterey dopaminergicheskih istoshteniem bazalynh gangliyah . kstrapiramidne rasstroystva proyavlyayutsya drozhatelynm paralichom. okonchatelyno ustanovlena. Obchno proyavlyaetsya perenesennogo ntsefalita, otravleniya okisyyu dlitelynogo antipsihotropnh . Chashte muzhchin vozraste 40-60 Chron. Otmechayutsya , rigidnosty konechnostey, nepodvizhnosty litsevh mshts, sharkayushtaya , postoyanny ritmichny. Takzhe seborreya, zhirnaya zrachkove defekt, diafragmalyne spazm, . DLYA ispolyzuyut preparat, yavlyayushtiesya predshestvennikami (), antigistaminne preparat.

provedenii 糳 uchitvaty rasstroystva () storon vnutrennih ffekt prinimaemh . otmenyaetsya pered operatsiey. Obchnaya prodolzhatysya operatsionnogo dnya. levodop sostavlyaet 6-12 , otmen vozmozhno mshechnoy narusheniem ventilyatsii. - VREMYA vozmozhn ortostaticheskaya gipotenziya, , gipertenziya. Arterialynuyu gipotenziyu ustranyayut vazopressorami pryamogo . Sleduet pomnity, Chto fenotiazin butirofenon antagonistami bazalynh gangliyah. Potomu sleduet ispolyzovaty Fonet levodop. Induktsiyu osushtestvlyaty ingalyatsionnmi anestetikami. Danne ispolyzovaniyu protivorechiv. tot vzvaty tahikardiyu gipertenziyu. Nezhelatelyno suksametoniya . mshts bty dostignuta povsheniem galotana. - aritmiyam nekotore avtor rekomenduyut vozderzhivatysya primeneniya galotana, mestnh anestetikov, soderzhashtih . Trebuetsya . kskursiy grudnoy sozdavaty problem posleoperatsionnom . Pered kstubatsiey sleduet ubeditysya samostoyatelynogo dhaniya vosstanovlenii dhatelynh .

Patau [Patau] ( 13 hromosom)

Vrozhdennoe zabolevanie, harakterizuyushteesya mikrotsefaliey, mikrognatiey, rasshtelinoy gub Neba, korotkoy sheey, deformatsiyami polidaktiliey, aplaziey mozolistogo gipoplaziey mozzhechka, vrozhdennmi porokami (DMPP, DMZHP) anomaliyami pochek (gidronefroz, ) nezavershennm povorotom kishechnika, defektami polovh . Vozmozhn mikrognatiey Dz rasshtepleniem .

vbore 糳 uchitvayut sistemne rasstroystva. PM 1: 7800.

( nikotinovoy kislot)

Zabolevanie svyazano defitsitom nikotinovoy kislot. Poslednyaya vhodit , prinimaet, a okislitelyno-vosstanovitelynh reaktsiyah . Vozmozhno dannoy 㳳 bolynh kartsinoidom, upotreblyayushtih pishtu kukuruz, hronicheskih alkogolikov narushenii pishtevareniya. Otmechayutsya psihicheskie razdrazhitelynosty, nevrologicheskie rasstroystva. nikotinovoy kislot ustranyaet nevrologicheskie sutok. kishechne rasstroystva proyavlyayutsya ahlorgidriey, tyazheloy diareey, , a privodity lektrolitov. Vozmozhn dermatit, , , .

Spetsificheskih rekomendatsiy provedeniyu 糳 .

Detskaya

Cm. Kvashiorkor.

Pena-Shokeyra, II

Cm. Mozgo-GLASS--skeletny .

ѳ Pendreda ( vrozhdennaya neyrosensornaya )

Nasleduetsya -retsessivnomu . tovidnoy zhelez obchno vozmozhen (. ó), tireoidktomii.

Availability sozdavaty .

Perikardit

GRUPPA zabolevaniy . Ostre perikardit voznikayut rezulytate infektsiy (virusne, bakterialyne, gribkove, tuberkulez), ( Dresslera) travmaticheskogo ( lektrodov kardiostimulyatora, , monitornh kateterov). Mogwai voznikaty metastazirovanii sistemnh zabolevaniyah (revmatoidny sistemnaya , ), - toksicheskogo (, ) radiatsionnogo oblucheniya sredosteniya. simptomaticheskoe, vklyuchaet kortikosteroid. vpota vpotnom . Klinicheskie bstrot nakopleniya vpota. Skoplenie korotkogo 100-200 zhidkosti vzvaty tamponadu . Naibolee informativny - hokardiografiya. Zhidkosty udalyayut pomoshtyyu . hronicheskom sdavlivayushtem otmechayutsya povshenie venoznogo davleniya, davleniya pravom , DZLA, predserdne 쳳, normalynaya, utolshtenie CT. Treatment obchno hirurgicheskoe.

Dlya 糳 ispolyzuyut sochetanie , opioidov, nizkimi dozami galogenosoderzhashtih anestetikov. tselyyu predovrashtenie , venoznogo vozvrata. normalizovaty OCK . arterialynogo venoznogo davleniya pryammi metodami. hirurgicheskom udalenii vozmozhn .

Peromeliya (konechnostey normalynom " )

Cm. Aglossii-adaktilii .

ѳ Pikvikskiy (, Pikvika)

- Kr. ճ (Ch. Heath) 1889 . godu Pikvikskomu klubu, opisannomu izvestnom PM Dikkensom, personazhey kotorogo bl zhirnm, krasnolitsm , pohrapvaya.

tim sindromom ponimayut nozologicheskuyu obstruktivnogo sonnogo apno (.) legko vyavlyaemh priznakov: znachitelynoe ozhirenie, Nochnoy , arterialynaya gipertenziya, giperemiey . Vnezapnaya smerty CH patsientov nablyudaetsya chashte, sravnivaemh gruppah. Dhatelynaya nedostatochnosty znachitelynoy gipoksemiey giperkapniey nablyudaetsya tolyko nochne dnevne chas.

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