Stopové prvky - Pavol Jozef Šafárik University

Stopové prvky - Pavol Jozef Šafárik University

Poruchy metabolizmu stopovch prvkov kolsk rok 2009/10 2012/13 Oliver Rcz, stav patologickej fyziolgie UPJ LF Koice 30/10/2012 stop09.ppt 1 Stopov prvky - osnova Prvky ivota a stopov prvky Sasn stav problematiky a nezodpovedan otzky Metabolizmus eleza Zinok a me Ostatn kovy Nekovy - seln, jd a flr

30/10/2012 stop09.ppt 2 Poruchy metabolizmu stopovch prvkov I. vod a veobecn prehad 30/10/2012 stop09.ppt 3 Prvky ivota Hlavn biognne prvky - 6 C, H, N, O, ale aj P a S

Elektrolyty Na+, K+, Mg++, Ca++ oproti Cl- (?) Stopov < 1 g s dvoma vnimkami kovy: Fe, Zn, Cu, Mn, Mo, Cr, Co, V, Sn nekovy: F, I, Se, Si, B 30/10/2012 stop09.ppt 4 Prvky ivota - pozor! Nezodpoved zloeniu udskho tela: As, Au, Pb, Hg. Siln selekcia: Zemsk kra - O, Si, Al, Fe, Ca

Morsk voda ? More v ase vzniku ivota ? Prvkov zloenie rastln sa viac podob na pdu ivot je ahk - prv polovica tabuky, len 4 maj atmov . > 30: 34Se (79) ,42Mo (96), 50Sn (119), 53I (127) 30/10/2012 stop09.ppt 5 Stopov prvky - sasn stav poznatkov - 1 Prvkov zloenie tela, tkanv, buniek, O.K. Ltkov forma - kovy len ako komplexy! Stabiln s znme (hm, molybdoptern, at) Menej stabiln sa ako skmaj

Pridvaj sa k biochemickm truktram po ich syntze - vnimka je Se 30/10/2012 stop09.ppt 6 Stopov prvky - sasn stav poznatkov - 2 Cykly v biosfre - prrodn a po udskom zsahu (ekolgia) vplyv na zdravie Cykly v udskom tele - vea otznikov Klinickobiochemick diagnostika Okrem eleza (a jdu) nie s rutinn vyetrenia O om hovoria plazmatick hladiny ??? Nepriame markery - napr. GPX pre Se 30/10/2012

stop09.ppt 7 Uiton alebo toxick ? +---------------------------------------------------------------------+ BIOGNNE PRVKY +-----------------------------------------+--------------------- HLAVN ELEKTROLYTY STOPOV TOXICK

a b +-----+--------+-----------+-------------+------+--------------------- I 1H 11Na 19K 29Cu 47Ag 79Au II 12Mg 20Ca 30Zn 4Be 48Cd 56Ba 80Hg III 5B

5B 13Al IV 6C 50Sn 14Si 82Pb V 7N 15P 23V 33As 73Ta 83Bi VI 8O 16S 24Cr 42Mo 34Se 24Cr 34Se 42Mo VII 17Cl 25Mn 9F 53I 9F 35Br

VIII 26Fe27Co28Ni 26Fe 27Co 28Ni +---------------------------------------------------------------------+ 30/10/2012 stop09.ppt 8 Koko ? Ktor ? RDA = recommended daily allowance od 1949, pre stopov prvky od 1989. Sta na prevenciu deficity Nov systm - DRI = daily recommended intake

Estimated average requirement RDA Adequate intake Upper limit NO - Fe, Zn, Cr, Se, I, F (nie vdy a u kadho) NIE - Cu, V, Mn, Ni, Co, Sn, Si (zdra mo) 30/10/2012 stop09.ppt 9 Poruchy metabolizmu stopovch prvkov II. Metabolizmus eleza

30/10/2012 stop09.ppt 10 Distribcia eleza C e lk o v m n o s tv o 4000 m g 100 % H e m o g lo b n 2500 63 M y o g lo b n

160 4 E n z m y ( k a ta l z a a i.) 8 0 ,2 Z s o b y (fe ritn ) 1350 33 T ran sp o rt (tra n s fe rn ) 5 0 ,1 2

30/10/2012 stop09.ppt 11 Bilancia eleza Rozpad erytrocytov (0,8 % denne 20 ml) 20 - 25 mg denne 90 - 95 % recyklcia! Straty len 1 - 2 mg/d Ben strava 10 - 15 mg denne kontrolovan rezorbcia 1 - 2 mg/d Fyziologicky zven straty: Mentrucia: 30 mg, gravidita 300 mg, laktcia 180 mg Darcovstvo krvi = 200 ml Er = 200 mg eleza, 15 % zsob

30/10/2012 stop09.ppt 12 30/10/2012 stop09.ppt 13 Ako na to ? Anmia - podozrenie na sideropniu elezo v sre (m: 14 - 29, : 12 - 23 mol/l) je o niom !!! Vzbov kapacita (TIBS, total iron binding capacity) je jednoduch a lacn meranie Meranie feritnu ? ak mte dos peaz, je to dobr ukazovate celkovch zsob (plazma: 40 - 50 ng/l)

Meranie transfernu ? (2 - 3 mg/l), kole Solubiln transfernov receptor 30/10/2012 stop09.ppt 14 Vzbov kapacita (TIBC) 1. Meranie eleza zo sra (16) Pridanie eleza k sru - nastenie transfernu na 100 % Odstrnenie neviazanho eleza 2. Meranie eleza zo sra (64) Saturan index = 16/64 = 0,25 (25 %) < 0,2 = sideropnia; > 0,55 = nadbytok 20-02-11 30/10/2012 stop09.ppt

15 Nedostatok a nadbytok eleza Sideropnia je ast u ien v rozvojovch krajinch. ast prody, infekcie, nedostaton stravovanie. U ns - choroby GIT (achlrhydria), choroby spojen s chronickmi stratami krvi (obliky, gynekologick, vredov choroba v minulosti) Prsni vegetarini deti 3 stupne, na konci je mikrocytov hypochrmna anmia (eny na obrazoch starch holandskch majstrov) 30/10/2012 stop09.ppt 16

1. Deplcia zsob krvn obraz a parametre OK 2. Deplcia eleza krvn obraz OK, parametre nie 3. Sideropenoick anmia 30/10/2012 stop09.ppt 17 Nedostatok a nadbytok eleza Popeye (staton nmornk) a pent Omyl - pent nie je dobrm zdrojom eleza (mso) Ete v omyl - nadbytok eleza je rizikovm faktorom ischemickej choroby srdca a rakoviny (?) Primrna hemochromatza - dedistvo E spred 10 - 15 tisc rokmi Sekundrna hemochromatza aj s anmiou! Naprklad po opakovanch transfziach pre

hemolytick anmie 30/10/2012 stop09.ppt 18 Hemochromatza a regulcia rezorbcie Fe OMIM *235200; carrier frequency 4,5 7,1 % (!) Homozygotes 2 - 5/1000 HFE gene in HLA region, 1 common mutation A in gny, in mutcie

1999 HFE2 - long arm, ch. 1 2000 HFE3 - 7q22, transferrin receptor 2 2001 HFE4 - ch 2; SCL40A1 gene for ferroportin 2003 HAMP gene; ch 1 for hepcidin 2004 HJV gene for hemojuvelin ??? 2025 ??? 30/10/2012 stop09.ppt 19 Hemochromatza a regulcia rezorbcie Fe Pre praktick medicnu zriedkav formy hemochromatzy nie s dleit, ale poznatky zskan na zklade tdia tchto chorb pomu objasni fyziologick regulciu rezorbcie eleza

Hlavnm regultorom je hepcidn z peene zven expresia v experimente - Fe deficit znen expresia pri mutcii alebo pri mutcii v gne pre hemojuvelin - nadbytok Fe A akumulcia Fe v substantia nigra pri Parkinsonovej chorobe preo, ako ? 30/10/2012 stop09.ppt 20 20-02-11 30/10/2012 stop09.ppt 21 Transfernov receptor a solubiln

receptor v krvi 30/10/2012 stop09.ppt 22 Feritn multimr, v strede vek mnostve atmov Fe 30/10/2012 stop09.ppt 23 Klinick manifestcia hemochromatzy

Bronzov sfarbenie koe Hepatomeglia, neskr cirhza, aj rakovina peene Bolesti v kboch Pokodenie endokrinnch liaz (bronzov diabetes) Kardiomyopatia Chronick navov syndrm Strata libida, impotencia Oxidan stres a akcelerovan ateroskleza pravdepodobne u v latentnom tdiu Faktory manifestcie: pohlavie (m>), strava (mso), alkohol 30/10/2012 stop09.ppt

24 Poruchy metabolizmu stopovch prvkov III. Zinok a me 30/10/2012 stop09.ppt 25 Zinok, Zn 2 - 3 g; RDA 12 - 15 mg Sas vekho mnostva enzmov (300) a inch bielkovn ( inzuln, transkripn faktory, receptory - a 3000) Chuov a uchov receptory,

inov kanly Stabilizuje priestorov truktru pecilnych domn - zinkov prsty 30/10/2012 stop09.ppt 26 20-02-11 30/10/2012 stop09.ppt 27 Zinok histria Nihil album (ZnO) pouivali u v stredoveku na liebu onch chorb 1746 A.S. Margaff, Nemecko, objav Zn 1869 - 1957 esencialita zinku u rastln a zvierat,

experimenlny a klinick deficit (hovdz dobytok, hydina) 1940 - 1961 prv Zn-metaloenzmy, esencialita u loveka 1974 stanoven RDA 2004 intenzvny vskum - marginlny deficit je ast, deficit v ivonej vrobe je vny problm 20-02-11 30/10/2012 stop09.ppt 28 Zn-metaloenzmy a proteny, funkcie DNA polymerza Zn-Cu SOD Retinoldehydrogenz a

Kolagenza Metalotionen Zn prsty Thymulin Receptory hormnov 30/10/2012 Syntza NK, delenie buniek Antioxidan ochrana Regenercia zrakovho purpuru Spojivo, cievna stena Transport

Transkripn faktory Diferencicia T lymfocytov Endokrinn funkcie stop09.ppt 29 Zinok, 2 - 3 g; RDA 12 - 15 mg/d Zdroje: vntornosti, huby, cvikla Mal zsoby, fytty obilnn zhrouj rezorbciu Marginlny deficit je pravdepodobne ast aj u ns ast infekcie, rastov retardcia ak deficit: Blzky Vchod - cerelie Alkoholici, cirhotici, nefrotick a malabosrpn sy. Vroden porucha absorbcie - acrodermatitis enteropathica (ADEMA u prasiat), Th: ZnO 30/10/2012

stop09.ppt 30 Zinok zdroje Hovdzie mso 6,4 mg/100 g Biely chlieb 0,6 Orechy 2,7 Kva, aj 0,02

Kraby 4,6 Ustrice 79,0 Mlieko 0,3 Ovsen vloky 3,O 30/10/2012 Pitn voda 0,03 stop09.ppt 2,2 31 Me, Cu 2 - 5 mg 100 - 150 mg; RDA Najviac vo svaloch, kostiach a peeni Aktvne centrum mnohch enzmov, predovetkm oxidoreduknch

Cu++ + eCu+ Superoxiddismutza, lyzyloxidza, cytochrmoxidza a i. Ceruloplazmn je transportnou bielkovinou medi 30/10/2012 stop09.ppt 32 Cu-metaloenzmy a proteny Cytochrm-C-oxidza Superoxiddismutza Monoaminooxidza (Katecholamny) Tyroznza (melann) Lyzyloxidza (kolagn) Ceruloplazmn a metalotionen (transport, detoxikcia)

30/10/2012 stop09.ppt 33 Metabolizmus medi, deficit a nadbytok Zdroje: orechy, ustrice, morsk ryby Viae sa na albumn, transcupren a v peeni na ceruloplazmn Vyluuje sa lou Deficit - experimentlne a pri akej malnutrcii - anmia, leukopnia, poruchy kost Hromadenie pri obtruknom iktere, je mon aj intoxikcia - hnaky, pokodenie peene 30/10/2012 stop09.ppt

34 Poruchy metabolizmu medi m. Wilson je vroden deficit ceruloplazmnu hepatolenikulrna degenercia AR dedinos Kayser a Fleischer 1903 (cornea), Wilson 1912 Von kov pokodzuje tkaniv Mierne a ak formy (len transaminzy a KF prstenec a po ak pokodenie peene, neurologick a psychiatrick spt., hemolza, obliky... 200 rznych mutci, 1 ast (ch 13, 1985, ATP7B 13q14) 1/30 000 30/10/2012 stop09.ppt 35 m. Wilson Ukazovate

Norma m. Wilson Ceruloplazmn 1,8 2,5 mol/l < 1,8 mol/l Cu plazma 16 31 mol/l < 16 mol/l Cu pee 30 50 g/g 100 150 g/g Cu mo stopy

100 g/d 30/10/2012 stop09.ppt 36 m. Wilson - kazuistika 22 ron mu, zven transaminzy pri vyetren na darcovstvo krvi Po roku (vylenie inch monch prin) Cielen laboratrne vyetrenia - m. Wilson, peeov forma Dve sestry - asymptomatick forma Kayser - Fleischerov prstenec nemali Myslie na to! - molekulovo-genetick dg. je mon 20-02-11 30/10/2012

stop09.ppt 37 m. Wilson - kazuistika Myslie na to! - molekulovo-genetick dg. je mon Najastejia manifestcia 8 - 20 r. Vinou peeov forma, pri cirhze a psychickch prznakoch je neskoro! Dysartria, dysfgia, rigidita a tremor, aj psychiatrick zmeny zmena osobnosti, zhoren pam Th: penicilamin, Zn, trietyltetramin, v prpade fulminantnej formy transplantcia peene 30/10/2012 stop09.ppt

38 Wilsonova choroba vek Otec 59 S-Cu mol/l 16,1 Matka 56 12,0 0,42

0,24 Syn 22 2,7 1,50 0,00 Dcra1 31 2,6 0,88 0,03

Dcra2 29 15,7 0,54 0,39 Dcra3 25 3,8 1,9 0,00

30/10/2012 stop09.ppt U-Cu mol/d 0,35 CPL g/l 0,37 39 Menkes kinky hair syndrome Zriedkav, ale fatlne ochorenie X chromosome related, ATP7A (1/250 000) disorder of intracellular transport of Cu Low activity of key copper enzymes (SOD, Cytochromoxidase, etc.)

Severe fatal disease with progressive neurological spts., connective tissue, skin, digestion 20-02-11 30/10/2012 stop09.ppt 40 Poruchy metabolizmu stopovch prvkov IV. Ostatn kovy 30/10/2012 stop09.ppt 41

Chrm, Cr kles 1 mg, vekom Silne toxick kov Vo forme komplexov (pikolint a i.) zlepuje citlivos tkanv na inzuln Prevencia poruenej tolerancie glukzy Pomocn lieba pri diabete 2. typu V roku 2003 bol objasnen inok aktivuje jeden z krokov intracelulrnej signalizanej cesty po vzbe inzulnu na jeho receptor 30/10/2012 stop09.ppt 42 Vand, mangn, nikel, kobalt a

molybdn Vand Neexistuje deficit, intoxikcia je mon Inzulnu podobn inok ? Mangn a nikel Neexistuje deficit Kobalt silne toxick aditvum do piva kardiomypatia jedine ako sas vitamnu B12 Molybdn (molybdoptern) Neexistuje deficit, intoxikcia je mon 30/10/2012 stop09.ppt 43

Poruchy metabolizmu stopovch prvkov V. Nekovy 30/10/2012 stop09.ppt 44 Seln, Se Toxick ltka (polovodie) m. Keshan (na), ak kardiomyopatia Deficit selnu v pde, rastlinch, potrave Aktvne centrum glutatinperoxidzy (GPX),

kovho enzmu antioxidanej ochrany Zasahuje aj do metabolizmu jdu Selenocysten je zabudovan do bielkovn poas syntzy 30/10/2012 stop09.ppt 45 Seln, Se Zdroje: cesnak, kvasinky, morsk ryby U ns (a v H, PL, CZ) marginlny nedostatok Potrebn dvka m: 70 g; 50 g, Novie a 200 g Odpora sa suplementcia v rmci prevencie aterosklerzy a malignt v roku 2001 tdia dokzala vrazn znenie rakoviny prostaty a hrubho reva

po 10 rokoch suplementcie Nie je zzran liek proti starnutiu a pod. 30/10/2012 stop09.ppt 46 Poruchy metabolizmu jdu Halogn, vyskytuje sa hlavnie ako NaI Dov voda vyplavuje jd z pdy, hlavnm rezervorom je morsk voda Morsk ryby a in ivochy, niektor minerlne vody Deficit ohrozuje 500 milinov 1 miliardu ud Na Slovensku do r. 1950 vysok vskyt endemickej strumy 30/10/2012 stop09.ppt

47 Poruchy metabolizmu jdu Hormny ttnej azy, metabolizmus, ale aj diferencicia tkanv a intrauteriinn rozvoj CNS!!! Jodidovan kuchynsk so Prvod ani dnes nie je idlny (USG ttnej azy) Aj strumigny (kapusta, cigaretov dym, polychlrovan bifenyly) mu narui metabolizmus jdu a ttnej azy 30/10/2012 stop09.ppt 48 Doporuen prvod jdu S k u p in a

R D A , g / d J o d r ia g / l d o s p e l a a d o le s c e n ti te h o tn e n y 150 100 200 200 200 300 n o v o ro d e n c i 90 > 150

d e ti 6 m 6 r 90 180 220 d e ti 6 1 2 r 120 100 200 30/10/2012 stop09.ppt 49 Ukazovatele metabolizmu jdu

J o d r ia , g /l D e fic it < 20 ak 20 50 50 100 100 200 zvan a h k Volumen ttnej azy ml (USG) mui

< 22 ml eny < 18 ml ia d n y deti 30/10/2012 stop09.ppt poda povrchu tela 50 Nsledky deficitu jdu P lo d

a b o r t , p r e n a n ie v v o jo v c h y b y k r e t e n iz m u s p s y c h o s o m a t ic k r e t a r d c ia N o v o ro d e n e c z v e n m rtn o s s tru m a , h y p o ty r e z a p s y c h o s o m a t ic k r e t a r d c ia D ie a s tru m a , h y p o ty r e z a p s y c h o s o m a t ic k r e t a r d c ia D o s p e l s tru m a , h y p o ty r e z a p o r u c h y m e n t ln y c h f u n c k i

30/10/2012 stop09.ppt 51 Opatrenia Jodidcia soli (35 mg/kg KIO3 jodinan) Kmn zmesi v ivonej vrobe (mlieko) Ryby, dary mora Obohaten potravinov vrobky Suplementcia u tehotnch ien a poas laktcie 30/10/2012 stop09.ppt 52 Zven prvod - Japonsko alebo

prehnan suplementcia A miligramy! U strumy - tyreotoxikza Spustenie autoimunity (v experimente) opatrnos u autoimunitnch ochoren Zdrav ttnu azu neohrozuje 30/10/2012 stop09.ppt 53 Flr, F - metabolick jed/prevencia zubnho kazu U 0,1 mmol/l fluoridu blokuje enolzu baktri Fluoroapatit, ktor tvor 10 % zubnej skloviny je ovea rezistentnej na kysl hydrolzu ako hydroxyapatit. Flr pomha pri premene amorfnho

kalciumfosftu na krytalick apatit Zubn pasty, stne vody, tabletky KF, 1 - 2 mg/d 30/10/2012 stop09.ppt 54

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