porfirini

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powerpoint presentation porfirini – promejutochnie produkti sinteza gema. gem predstavlyaet soboy kompleksnoe soedinenie dvuxvalentnogo jeleza s porfirinom porfirini yavlyayutsya silnimi fotosensibiliruyushimi veshestvami. spektr poglosheniya porfirinov vklyuchaet dva diapazona: 400-410 nm i 580-660 nm gruppa nasledstvennix zabolevaniy, obuslovlennix narusheniyami sinteza gema s povisheniem soderjaniya porfirinov v krovi i tkanyax ar- autosomno-retsessivniy, ad – autosomno-dominantniy, a - kolichestvo izvestnix mutatsiy defekt fermenta lokus mutatsiia nachalo bolezni chastota vstrechaemosti 1. porfiriya, vizvannaya defitsitom aminolevulinat-degidratazi alk-degidrotaza ~ 5% normi ap 9 q34 7 mutatsiy variabelno opisano lish neskolko sluchaev 2. ostraya peremejayushayasya porfiriya pfb-dezaminaza, 50% normi ad 11q23.3 >227mutatsiy redko do zrelogo vozrasta laplandiya 1:1000, vezde 1,5:100000 3. vrojdennaya eritropoeticheskaya porfiriya uroporfirino-gen-3-kosintaza ap 10 q25.2-26.3 v utrobe i pri rojdenii ˂ 200 opisannix sluchaev 4. kojnaya porfiriya uroporfirinogen dakarboksilaza priobretennaya takje ad lp34 30-40 let chasto v ssha i evrope 5. gepatoeritro-poeticheskaya porfiriya uroporfirinogen dakarboksilaza ar do 2 let ˂ 20 opisannix sluchaev 6. …

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gormoni (estrogeni,progesteron, v nekotorix sluchayax – peroralnie kontratseptivi), infektsii, golodanie. simptomi povrejdeniya vegetativnix otdelov nervnoy sistemi vklyuchayut abdominalnie boli, zapori (inogda ponosi),taxikardiyu, slyunotechenie, prexodyashuyu arterialnuyu gipertoniyu, ortostaticheskuyu gipotoniyu, spazm arteriy setchatki, sosudistiy spazm v koje konechnostey. inogda nablyudayutsya polinevrit, vyalaya tetraplegiya, rasstroystva chuvstvitelnosti rasstroystva so storoni tsns : bulbarnie paralichi, mozjechkovie narusheniya, gipotalamicheskaya disfunktsiya, ostrie i xronicheskie psixozi, koma vovlechenie mejrebernix i diafragmalnix nervov mojet privodit k dixatelnomu paralichu – naibolee chastoy prichine smerti otmechayutsya giponatriemiya, gipomagniemiya, obichno vizivayushaya tetaniyu, povishenie urovney xolesterina ( u 40-50% bolnix). na svetu mocha okrashivaetsya v krasniy tsvet v moche povishen uroven predshestvennikov – porfobilinogena i aminolevulinovoy kisloti. snijena aktivnost fermenta uroporfirinogensintetazi. diagnosticheskie priznaki: uvelichenie uroporfirina v moche poyavlenie v krovi normoblastov uvelichenie urovnya uroporfirina v eritrotsitax fotodermatoz anomalnaya okraska zubov na otkritix uchastkax imeyutsya oblasti giperpigmentatsii i depigmentatsii. v bolshinstve sluchaev nablyudaetsya gemoliz. vo vremya gemoliza: povisheno soderjanie urobilinogena v kale normoblasticheskaya giperplaziya …

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eltenie skler i koji nachinaetsya s kontsentratsii bilirubina v krovi 26 mkmol/l i trebuet vremeni 5-8 dney bilirubin obrazuetsya v retikuloendotelialnoy sisteme, osobenno v selezenke i kupferovskix kletkax pecheni pri raspade jelezosoderjashix belkov: gemoglobina, katalazi, mioglobina, tsitoxromov. v techenie sutok raspadaetsya ot 6 do 8 g gemoglobina i obrazuetsya okolo 240-430 mg jelchnix pigmentov. bilirubin ploxo rastvorim v vode, yavlyaetsya toksicheskim veshestvom. bilirubin, popadaya iz kletok res v sivorotku krovi, elektrostaticheski vzaimodeystvuet s albuminom i transportiruetsya v pechen. eta fraktsiya nosit nazvanie nekon'yugirovanniy ( svobodniy ) bilirubin. laboratornoe nazvanie takogo bilirubina – nepryamoy. eto obuslovleno tem, chto bilirubin svyazan s albuminom i dlya ego opredeleniya neobxodimo predvaritelno provesti reaktsiyu deproteinizatsii. gepatotsiti aktivno zaxvativayut bilirubin iz krovi – za 18 minut iz krovi ischezaet 50% ekzogennogo bilirubina. pogloshenie gepatotsitami iz krovi nekon'yugirovannogo bilirubina – aktivniy protsess, v kotorom prinimayut uchastie ligand i spetsificheskiy belok protein z. v gepatotsitax karboksilnie gruppi bilirubina …

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soderjitsya bilirubin, svyazanniy s glyukuronovoy kislotoy ( pryamoy ) i bilirubin, ne svyazanniy s glyukuronovoy kislotoy ( nepryamoy). norma: obshiy bilirubin novorojdennie 1 sut 6,8-68,0mkmol/l novorojdennie 3 sut 17,7-171 mkmol/l novorojdennie 1 mes 5,2-17,1mkmol/l deti i vzroslie 3,4-17,0mkmol/l bilirubin kon'yugirovanniy (pryamoy) vzroslie < 5 mkmol/l bilirubin obnarujivaetsya v kale pri disbakterioze v svyazi s tem, chto vosstanovlenie bilirubina proisxodit s uchastiem degidrogenazi mikroflori kishechnika gemoliticheskie anemii ostrie i xronicheskie v12- defitsitnaya anemiya talassemiya obshirnie gematomi bilirubinemiya pri mexanicheskoy jeltuxe yavlenie postoyannoe. snijenie soderjaniya bilirubina v moche ili ego polnoe ischeznovenie ukazivaet na chastichnoe ili polnoe vosstanovlenie proxodimosti jelchnix putey. istinnaya (pechenochnaya ) jeltuxa voznikaet pri razlichnix porajeniyax pecheni. nablyudaetsya pri: ostriy virusniy gepatit tsitomegalovirusnaya infektsiya, infektsionniy mononukleoz amebniy abstsess pecheni, opistorxoz, aktinomikoz sifilis vtorichniy tsirroz pecheni,xolangit, pervichniy rak pecheni, metastaticheskie porajeniya pecheni pervichniy biliarniy tsirroz pecheni toksicheskoe povrejdenie pecheni: chetirexxloristiy uglerod, xloroform, trixloretilen, alkogol lekarstvennie otravleniya: paratsetamol, izoniazid,rifampitsin, xlorpromazin vsledstvie …

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kolichestva sterkobilina. sootnoshenie kolichestva sterkobilina i urobilinovix tel s mochoy pri tyajelix porajeniyax pecheni dostigaet 1:1( norma 10:1 ili 20:1)

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powerpoint presentation porfirini – promejutochnie produkti sinteza gema. gem predstavlyaet soboy kompleksnoe soedinenie dvuxvalentnogo jeleza s porfirinom porfirini yavlyayutsya silnimi fotosensibiliruyushimi veshestvami. spektr poglosheniya porfirinov vklyuchaet dva diapazona: 400-410 nm i 580-660 nm gruppa nasledstvennix zabolevaniy, obuslovlennix narusheniyami sinteza gema s povisheniem soderjaniya porfirinov v krovi i tkanyax ar- autosomno-retsessivniy, ad – autosomno-dominantniy, a - kolichestvo izvestnix mutatsiy defekt fermenta lokus mutatsiia nachalo bolezni chastota vstrechaemosti 1. porfiriya, vizvannaya defitsitom aminolevulinat-degidratazi alk-degidrotaza ~ 5% normi ap 9 q34 7 mutatsiy variabelno opisano lish neskolko sluchaev 2. ostraya peremejayushayasya porfiriya pfb-dezami...

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