mukovistsidoz

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mukovistsidoz-chastoe monogennoe zabolevanie,obuslovlennoe mutatsiey gena mvtr(transmembrannog regultora mv), xarakterizuyusheesya porajeniem ekzokrinnix jelez jiznenno vajnix orgnov i sistem i imeyushee obichno tyajeloe techenie i prognoz. sledstviem mutatsii gena yavlyae mukovistsidoz osiyo xalqaro universiteti mukovistsidoz chastoe monogennoe zabolevanie, obuslovlennoe mutatsiey gena mvtr (transmembrannogo regulyatora mv), xarakterizuyusheesya porajeniem ekzokrinnix jelez jiznenno vajnix organov i sistem, i imeyushee obichno tyajeloe techenie i prognoz. sledstviem mutatsii gena yavlyaetsya narushenie strukturi i funktsii belka-transmembrannogo regulyatora mv, reguliruyushego elektrolitniy (preimushestvenno xloridniy) transport mejdu kletkami i mejkletochnoy jidkostyu. etot belok nazivayut xlornim kanalom. vozmojnie klinicheskie proyavleniya mukovistsidoza otiti evstaxeiti sinusiti bronxiti pnevmonii parotiti (ne virusnie) kishechnie koliki vipadenie pryamoy kishki jeltuxa neproxodimost distrofiya oteki malokrovie mozgovaya koma v letnee vremya (elektrolitnie narusheniya). izmeneniya v podjeludochnoy jeleze pri mukovistsidoze bronxi pri mukovistsidoze serdtse pri mukovistsidoze tip nasledovaniya pri mukovistsidoze autosomno-retsessivniy otets (getero zigota) mat (getero zigota) zdorov nositel (getero zigota) nositel (getero zigota) bolnoy zvenya patogeneza mukovistsidoza: narusheniya elektrolitnogo …

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ichini vyazkosti mokroti pri mukovistsidoze: narushenie elektrolitnogo obmena, chto vedet k degidratatsii sekreta dnk degenerirovannix neytrofilov, kotorie privlekayutsya v bolshom kolichestve v dixatelnie puti formi mukovistsidoza osnovnie: smeshannaya s porajeniem jkt i bronxolegochnoy sistemi (75-80%); preimushestvenno legochnaya (15-20%); preimushestvenno kishechnaya (5%). stadiynost izmeneniy v legkix pri mukovistsidoze stadiya xarakter protsessa dlitelnost techeniya 1-ya stadiya retsidiviruyushiy bronxit 10 let 2-ya stadiya xronicheskiy bronxit 1-15 let 3-ya stadiya diffuzniy skleroz, bronxoektazi, kisti, virajennaya dn, serdechnaya nedostatochnost 3-5 let 4-ya stadiya tyajelaya serdechno-dixatelnaya nedostatochnost letalniy isxod v techenie neskolkix mesyatsev rentgenologicheskaya kartina sindrom porajeniya interstitsialnoy tkani legkix – usilenie, tyajistost, yacheistost legochnogo risunka za schet razrastaniya soedinitelnoy tkani. peribronxialnie i perivaskulyarrnie izmeneniya, rasshirenie teni korney legkix. sindrom narusheniya bronxialnoy proxodimosti povishenie prozrachnosti legochnoy tkani, nizkoe stoyanie diafragmi, uvelichenie peredne-zadnego razmera grudnoy kletki, dolkovie i segmentarnie atelektazi. sindrom zapolneniya alveol – infiltratsiya, alveolyarniy otek. sindrom “sotovogo legkogo” – krupnoyacheistaya deformatsiya legochnogo risunka s obrazovaniem …

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kterialnoy floroy (preimushestvenno sinegnoynoy palochkoy) v rezultate u rebenka razvivaetsya dlitelno tekushiy protsess s yavleniyami obstruktsii, atelektazami, obstruktivnoy emfizemoy, vtorichnim pnevmosklerozom, deformatsiey bronxov, bronxoektazami, pristupami astmi. porajenie jkt pri mukovistsidoze pri mukovistsidoze porajaetsya preimushestvenno podjeludochnaya jeleza, kishechnik i pechen zatrudnenie ottoka vyazkogo sekreta privodit k ego zastoyu v prosvete ekzokrinnix jelez, v rezultate chego vivodnie protoki rasshiryayutsya, a jelezistaya tkan postepenno atrofiruetsya; razvivaetsya progressiruyushiy fibroz tkani jelezi. porajenie ekzokrinnix jelez pishevaritelnoy sistemi obuslovlivaet narushenie protsessov perevarivaniya i vsasivaniya substratov, privodyashee k razvitiyu sindroma malabsorbtsii. izmeneniya v podjeludochnoy jelezi xarakterizuyutsya diffuznim fibrozom, razrastaniem soedinitelnoy tkani vokrug protokov, dolek i vnutri nix, kistoznimi izmeneniyami melkix i srednix protokov, s gustoy slizyu, inogda eozinofilnogo xaraktera s inkrustatsiey kaltsiya vnutri kist, atrofiey atsinusov, vovlecheniem v patologicheskiy protsess ostrovkov langergansa (na bolee pozdnix stadiyax zabolevaniya). izmeneniya v tonkoy kishke proisxodit kistoznoe pererojdenie bokalovidnix kletok i liberkyunovix jelez, utolshenie slizistoy obolochki za schet razrastaniya soedinitelnoy tkani, …

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e povishennom appetite i xoroshem uxode. prodoljenie v dalneyshem prisoedinyayutsya jalobi na suxost vo rtu zatrudnenie pri proglativanii pishi, vo vremya edi deti upotreblyayut bolshoe kolichestvo jidkosti po mere narastaniya intoksikatsii snijaetsya appetit inogda v etot period materi obrashayut vnimanie na gorko-soleniy privkus pota rebenka. prodoljenie esli mukovistsidoz ne bil zapodozren, to zabolevanie postepenno vstupaet v sleduyushuyu fazu — period razgara. razvivaetsya vtorichniy sindroma malabsorbtsii — fermentativnaya nedostatochnost podjeludochnoy jelezi i jelez kishechnika, porajenie pecheni. prodoljenie vsledstvie dlitelnogo narusheniya protsessov pishevareniya i razvitiya xronicheskoy gipoksii sostoyanie bolnix uxudshaetsya, virajennost klinicheskix simptomov usilivaetsya, razvivayutsya poligipovitaminoz, obmennie rasstroystva, poyavlyayutsya bol v jivote, vzdutie i uvelichenie ego razmerov. prodoljenie izmeneniya stula xarakterizuyutsya uchasheniem, polifekaliey kal rezko-zlovonniy, blestyashiy, svetlo-seriy (za schet bolshogo kolichestva jira), prichem inogda jir vitekaet iz anusa v vide maslyanistoy jidkosti. odnako inogda kal mojet bit oformlennim, plotnim, daje vida «ovechego». prodoljenie snijayutsya mishechniy tonus i turgor tkaney, poterya massi tela …

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slojneniya mukovistsidoza: invaginatsiya (obichno pri mekonialnom ileuse, no mojet bit i v bolee starshem vozraste) yazvennoe krovotechenie polipoz kishechnika obsledovaniyu na mukovistsidoz podlejat: deti s chastimi obstruktivnimi bronxitami, bronxialnoy astmoy, pnevmoniyami, protekayushimi s sindromom bronxialnoy obstruktsii; mekonealnim ileusom, sindromom malabsorbtsii, retsidiviruyushimi bolyami v jivote, retsidiviruyushimi sinusitami, gipotrofiey. pilokarpinovaya potovaya proba. dostovernim yavlyaetsya kontsentratsiya nacl svishe 40 mmol/l v vozraste do 1 goda i svishe 60 mmol/l u detey starshe goda (1 raz v 6mesyatsev). pilokarpinoviy test s nagruzkoy doksa (pri somnitelnom teste bez nagruzki) nogtevoy test. diagnosticheskoy yavlyaetsya kontsentratsiya natriya v tkani nogtya vishe 80mmol/kg. dnk-analiz genealogicheskiy metod issledovaniya metodi diagnostiki mukovistsidoza na pervom etape diagnostiki provoditsya rasshirennoe koprologicheskoe issledovanie, viyavlyayushee snijenie (1:16—1:8) aktivnosti tripsina vplot do polnogo otsutstviya (rentgenplenochniy test shvaxmana); virajennuyu steatoreyu i tipa — uvelichennoe soderjanie neytralnogo jira vsledstvie narusheniya ego gidroliza v svyazi so vneshnesekretornoy pankreaticheskoy nedostatochnostyu; umerennuyu amiloreyu i kreatoreyu; umerennoe povishenie soderjaniya jirnix kislot …

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mukovistsidoz-chastoe monogennoe zabolevanie,obuslovlennoe mutatsiey gena mvtr(transmembrannog regultora mv), xarakterizuyusheesya porajeniem ekzokrinnix jelez jiznenno vajnix orgnov i sistem i imeyushee obichno tyajeloe techenie i prognoz. sledstviem mutatsii gena yavlyae mukovistsidoz osiyo xalqaro universiteti mukovistsidoz chastoe monogennoe zabolevanie, obuslovlennoe mutatsiey gena mvtr (transmembrannogo regulyatora mv), xarakterizuyusheesya porajeniem ekzokrinnix jelez jiznenno vajnix organov i sistem, i imeyushee obichno tyajeloe techenie i prognoz. sledstviem mutatsii gena yavlyaetsya narushenie strukturi i funktsii belka-transmembrannogo regulyatora mv, reguliruyushego elektrolitniy (preimushestvenno xloridniy) transport mejdu kletkami i mejkletochnoy jidkostyu. etot belok nazivay...

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