Klinická biochemie v pediatrii a gerontologii

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Věk

●&#= 13;

●je jednou ze základních preanalytických neovlivnitelných proměnných

●pro jednotlivé věkové skupiny platí rozdílné referenční intervaly

●řada speciálních metod je využívána pouze určitou věkovou skupinou

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Věkové skupin= y v klin. biochemii

●&#= 13;

`= 79;novorozenci (vč. nedonošených)

`= 79;děti (do ukončení puberty)

`= 79;dospělí

`= 79;stárnoucí skupina (u žen po menopaze)

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Novorozenec – specifika vzorků

●&#= 13;

`= 79;pupečníková krev

●kapilárn&= iacute; krev z paty, venózní z žil na hlavě nebo i.v. katetrů

`= 79;křehkost erytrocytů

● <= /div>

`= 79;význam POCT

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Novorozenec= = – specifika testů

●= hemoglobin

●= bilirubin

●= protein

●= enzymy (CK, ALT, AST, GMT)

●= acidobazická rovnováha

●= glukóza

●= lipidy

●= fyziologický hyperthyroidismus

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Novorozenec= – hemoglobin

●&#= 13;

●po porodu klesá počet ery a koncentrace Hb do několika dní po porodu

●&#= 13;

●donošení novorozenci mají 80% HbF a 20% HbA a na konci prvního roku je přítomen = pouze HbA

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Novorozenec= – bilirubin

= ●koncentrace je fyziologicky nízká v pupečníkové krvi, koncentrace fyziologicky přechodně stoupá jako důsledek zvýšeného obratu Hb (vrchol 3-5 dní) s maximem 205 umol/l (nedonošenci 274)

●&#= 13;

●hemolytická nemoc novoroze= nce, jádrový ikterus

●&#= 13;

●vzhledem k nezralosti jater je bilirubin nekonjugovaný

●&#= 13;

●= Přím&e= acute; stanovení bilirubinu (A454 – A540<= /span>)

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= Novorozenec= –= protein, enzymy, glukóza <= /span>

●Proteinémie po porodu stoupá z důvodu přesunu tekutin do intersticia (změna tlakových poměrů).

●&#= 13;

●Enzymy (CK, ALT, AST, GMT) mají v séru vyšší katalytické koncentrace.

●&#= 13;

●Glykémie je nižší vzhledem k malým zásobám glykogenu.

●&#= 13;

●

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Novorozenec= –= ABR, ionty

●Saturace O2 je nízká a během hodin stoupá. První den je fyziologická metabolická acidóza (kys. mléčná), která ustoupí do 24 h.

●&#= 13;

●První poporodní den je zvýšený sodk, draslík (až 7 mmol/l), chloridy, vápník.

●&#= 13;

●Lipidy jsou nízké a stoupají po začátku příjmu potravy.

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Vrozen&e= acute; vady metabolismu

`= 79;Závažné

`= 79;Vzácné

`= 79;AR dědičnost

●Různý klinický obraz (intoxikace vs. neprospívání)

`= 79;Nutná specializovaná vyšetření

●Rutinní vyšetření: ABR, glukóza, amoniak, laktát, v moči ketony

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VVM R= 11; základní skupiny <= /span>

●&#= 13;

`= 79;Poruchy metabolismu aminokyselin

●&#= 13;

`= 79;Organické acidurie

●&#= 13;

`= 79;Poruchy oxidace mastných kyselin

●&#= 13;

`= 79;Poruchy cyklu močoviny

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VVM - vy= šetření<= span lang=3DEN-US style=3D'font-family:Arial;mso-ascii-font-family:Arial;mso-sp= ecial-format: lastCR;display:none'>

`= 79;Plasma

–= ;Aminokyseliny

–= ;Karnitin, acylkarnitiny

–= ;Mastné kyseliny

–&#= 13;

`= 79;Moč

–= ;Organické kyseliny

–= ;Acylglyciny

–

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Skríning novorozenců

●z kapky krve na filtračním papře

●&#= 13;

`= 79;Fenylketonurie - Guthrieho test

●&#= 13;

`= 79;Hypothyreóza

●&#= 13;

–

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Klinick<= /span>á biochemie poruch = růstu

●&#= 13;

`= 79;Růst a růstový hormon

●&#= 13;

`= 79;Fyziologie GH u dítěte a dospělého

`= 79;Fyziologie IGF –I a IGF-II

`= 79;Fyziologie IGFBG-3

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Klinick<= /span>á biochemie poruch = růstu

●= vyš<= span lang=3DCS style=3D'font-family:"Times New Roman";mso-ascii-font-family:"Ti= mes New Roman"'>etřovány GH (bazálně i po stimulaci), IGF-I, IGFBP-3

●

`= 79;Deficit GH (méně)

`= 79;Necitlivost ke GH (častěji, obvykle částečná)

●&#= 13;

●Akromegalie a supresní test GH (jako oGTT)

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Nej= častější autosomálně recesivní choroba u bělošských populací postihující= ~ 1 / 3 000 novorozenců

Klinicky= se manifestuje progresivním obstuk= čním sino-= pulmoná= rní= m onemocněním, dysfunkcí exogenní sekrece pankreatu, zvýšenými Cl- v potu (> 60mM) a <= /b>mužskou infertilitou&#= 13;

Onemocn= ění významně zkracuje život postižených. <= /b>

Cystická fibróza

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„3D“ model CFTR proteinu=

Scientific American XII/95

Mutace = DF508=

Polysacharidy

Buněčná membrána

ATP

TM1 / TM2

Cl- ionty

Iontový kanál

ATP

Regulač= ní doména

Cytoplasma

Lumen

Nucleotide Binding

Domain 1 –NBD1

Nucleotide Binding

Domain 2- NBD2

Proteinkináza

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mmvldl9gUOj7/4CBgoOEhYaHF4kZi4qNjBkJADs= ------=_NextPart_01CAAFFC.BB47E170 Content-Location: file:///C:/B134650B/vek_soubory/slide0029.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinická biochemie v pediatrii a gerontologii

Funkce CFTR proteinu

R

NBD1

NBD2

PKA<= /span>

cAMP

Outwardly-

Rectified

Cl- Channel

TM1

TM2

CFTR

Cl-

Cl-

ENaC=

P2

Na+

ATP

ATP

ATP

Lum= en

Cyt= oplasma

G<= /span>

E= pitelový

Sod&iacu= te;kový

(Na+) Kanál

(Channel)

ORCC=

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------=_NextPart_01CAAFFC.BB47E170 Content-Location: file:///C:/B134650B/vek_soubory/slide0030.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii" Klinická biochemie v pediatrii a gerontologii

Hlavní role CFTR proteinu

=

=

`= 79;Transport vody a iontů (Cl-)- chloridový kanál

`= 79;transport ATP -> regulace ORCC, EN= aC

●Sensor intracelulárních hl= adin ATP

●Úloha v endocytóze a exocytóze

●Acidifikace lysozomů

`= 79;Internalizační receptor pro P. aeruginosa? =

`= 79;alkalinizace pankreatických štáv (HCO3 /&nb= sp; Cl- ) <= /span>

●Role při apoptóze= 3;

●Metabolismus NO

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=

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Hlavní mutace CFTR genu - DF508

504

508<= /span>

512

Norma = E N I = I F = G V S

D<= /b>F508 = N I I = G V S = Y D

Pr= ůměrná četnost mutace D<= span lang=3DCS style=3D'font-family:Arial;mso-ascii-font-family:Arial;mso-hansi= -font-family: Arial;color:white;mso-color-index:0'>F508 na všech CF chromosomech

u evropských populací je ~ 66.8%. Od roku 1989 bylo nalezeno

p<= /span>řes 900 mutací CFTR genu (www.genet.sickkids.on.ca/cftr).

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

F508

71.57 %

CFTRdele2,3/21kb/

4.64 %

G551D

4.03 %

N1303K

3.02 %

G542X

2.22 %

1898+1 G->A

2.02 %

2143delT

1.21 %

R347P

0.81 %

W1282X

0.60 %

4374+1 G->T

0.40 %

Nejčastější mutace CFTR genu v ČR

Paleolitická

Slovanská

Keltská

Středomoří

„Fénická“

Keltská-Wales
Slovanská

Germánská

Ashk. Židov.

Slovanská=

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Starší dětský pacien= t

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χ= 9;Proteinémie stoupá na dospělé hodnoty v 10 letech

`= 79;Stoupá kreatininémie (růst svalové hmoty)

●Kyselina močová poklesá do 7 let a následně stoupá do 16 let na dospělé hodnoty

`= 79;ALP odpovídá činnosti osteoblastů a růstu

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Puberta<= /span>

=

=

`= 79;Začátek biochemických odlišností mezi pohlavími

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`= 79;Nástup cirkamenzálních rytmů u dívek

●&#= 13;

●Vyšetření hormonů u pubertas praecox, opožděného nástupu puberty

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Dospělost

=

=

`= 79;Tvoří referenční populaci

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`= 79;Větš<= span lang=3DCS style=3D'font-family:"Times New Roman";mso-ascii-font-family:"Ti= mes New Roman"'>ina biochemických ukazatelů stabilní

●&#= 13;

●Stoupá glykmie po ztěži, klesá glomerulární filtrace (clearance kreatininu), stoupá cholesterol

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T= ěhotenství

=

=

`= 79;Hormony: estrogeny, progesteron, hCG, hPL

●Nárůst intravaskulárního objemu a diluční pokles koncentrace hemoglobinu (absolutn= í množství stoupá) a albuminu

`= 79;Renální funkce: nárůst GF o 40-50%

●Metabolismus glukózy: inzulinová rezistence, snížení renlního prahu, nárůst postprandiálních glykémií

`= 79;Hyperkoagulační stav

●

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Skr&iacu= te;ning vrozených vývojových vad

=

= ● upozorňuje na zvýšené riziko minimálně tří nejdůležitějších vývojových vad:

–trizomie 21. chromosomu (M.Down)<= /span>

–trizomie 18. chromosomu (M.Edward= s)

–defektu neurální trubice (NTD) <= /div>
–&#= 13;

–Interpretace laboratorních výsledk<= /span>ů vyžaduje přesnou znalost gest= ačního věku (UZ), je prováděna pomocí software a vlastním = výsledkem je odhad rizika výše uvedených onemocnění. =

●

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Skr&iacu= te;ning VVV

=

=

●laboratorní markery pro I. trimestr – PAPP-A (případně free-beta hCG), odběr v 10.týdnu těhotenství

●

●laboratorní markery pro II. trimestr – hCG, AFP (případně uE3), odběr v 15.-17. týdnu těhotenství

●

●ultrazvukové markery I. trimestru – NT (nuchální trasnlucence), NB (nosní kůstka), provádí se v 11.-12. týdnu těhotenství

●

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St&aacut= e;rnoucí populace

=

=

●Menopauza, andropauza =

●Ledviny &= #8211; pokles koncentrační schopnosti, pokles clearance kreatininu, snížení glukózového prahu

●Pokles hladin IgG a IgM

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●Vzestup cholesterolu <= /span>

●&#= 13;

●Vyšetření u stavů častějších u stárnoucí populace (dehydratace, malnutrice, deficity vitamínů a stopových prvků)

●

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MakeNotesVis() ) return runAnimations =3D _InitAnimations(); if( IsWin("PPTSld") ) parent.SldUpdated(GetSldId()) g_origSz=3DparseInt(SlideObj.style.fontSize) g_origH=3Dsld.style.posHeight g_origW=3Dsld.style.posWidth g_scaleHyperlinks=3D(document.all.tags("AREA").length>0) if( g_scaleHyperlinks ) InitHLinkArray() if( g_scaleInFrame||(IsWin("PPTSld") && parent.IsFullScrMode() ) ) document.body.scroll=3D"no" _RSW() if( IsWin("PPTSld") && parent.IsFullScrMode() ) FullScrInit(); =09 MakeSldVis(); ChkAutoAdv() if( runAnimations ) { if( document.all("NSPlay") ) document.all("NSPlay").autoStart =3D false; if( sld.filters && sld.filters.revealtrans ) setTimeout( "document.body.start()", sld.filters.revealtrans.duration * = 1000 ); else document.body.start(); } } function MakeSldVis() { var fTrans=3Dg_showAnimation && SldHasTrans() if( fTrans ) { if( g_bgSound ) { idx=3Dg_bgSound.indexOf(","); pptSound.src=3Dg_bgSound.substr( 0, idx ); pptSound.loop=3D -(parseInt(g_bgSound.substr(idx+1))); } SlideObj.filters.revealtrans.Apply() } SlideObj.style.visibility=3D"visible" if( fTrans ) SlideObj.filters.revealtrans.Play() } function MakeNotesVis() { if( !IsNts() ) return false SlideObj.style.display=3D"none" nObj =3D document.all.item("NotesObj") parent.SetHasNts(0) if( nObj ) { nObj.style.display=3D"" parent.SetHasNts(1) } return 1 } function ChkAutoAdv() { if(SldHasTrans()) SlideObj.onfilterchange=3DAutoAdv else AutoAdv() } function AutoAdv() { if(!IsWin("PPTSld") || !gUseSldTimings )return var sld=3DGetCurSld() if( (sld.mAdvDelay>0) && !parent.IsFramesMode() ) setTimeout("parent.GoToNextSld()",sld.mAdvDelay) } function GetObj(id) { if(g_supportsPPTHTML) return document.all(id); else return document.getElementById(id); } function SldHasTrans() { return SlideObj.style.filter !=3D ""; } function GetSldId() { return sId=3Dlocation.href.substring(location.href.la= stIndexOf('/')+1) } function HideMenu() { if( frames["PPTSld"] && PPTSld.document.all.item("ctx= tmenu") && PPTSld.ctxtmenu.style.display!=3D"none" ) { PPTSld.ctxtmenu.styl= e.display=3D'none'; return true } return false } function IsWin( name ) { return window.name =3D=3D name } function IsNts() { return IsWin("PPTNts") } function IsSldOrNts() { return( IsWin("PPTSld")||IsWin("PPTNts") ) } function SupportsPPTAnimation() { return( navigator.platform =3D=3D "Win32"= && navigator.appVersion.indexOf("Windows")>0 ) } function SupportsPPTHTML() { var appVer=3Dnavigator.appVersion, msie=3DappVer.indexOf("MSIE "), ver=3D0 if( msie >=3D 0 ) ver=3DparseFloat( appVer.substring( msie+5, appVer.indexOf(";",msie) ) ) else ver=3DparseInt(appVer) return( ver >=3D 4 && msie >=3D 0 ) } function _RSW() { if( !g_supportsPPTHTML || IsNts() || ( !g_scaleInFrame && (!IsWin("PPTSld") || !parent.IsFullScrMode()) ) ) return var padding=3D0; if( IsWin("PPTSld") && parent.IsFramesMode() ) padding=3D6 cltWidth=3Ddocument.body.clientWidth-padding cltHeight=3Ddocument.body.clientHeight-padding factor=3D(1.0*cltWidth)/g_origW if( cltHeight < g_origH*factor ) factor=3D(1.0*cltHeight)/g_origH newSize =3D g_origSz * factor if( newSize < 1 ) newSize=3D1 s=3DSlideObj.style s.fontSize=3DnewSize+"px" s.posWidth=3Dg_origW*factor s.posHeight=3Dg_origH*factor s.posLeft=3D(cltWidth-s.posWidth+padding)/2 s.posTop=3D(cltHeight-s.posHeight+padding)/2 if( g_scaleHyperlinks ) ScaleHyperlinks( factor ) } function _InitAnimations() { animRuntimeInstalled =3D ''+document.body.localTime !=3D 'undefined'; isFullScreen =3D (window.name =3D=3D "PPTSld") && !parent.IsFramesMode(); g_animUseRuntime =3D g_showAnimation && animRuntimeInstalled && !(isFullSc= reen && parent.IsSldVisited()); if( g_animUseRuntime ) { collSeq =3D document.all.tags("seq"); if( collSeq !=3D null ) { for(ii=3D0;ii numSlds ) gSldJumpIdx =3D numSlds; if ( gSldJumpIdx >=3D 0 ) { if ( gSldJumpIdx =3D=3D 0 ) gSldJumpIdx =3D 1; var jumpTo =3D parseInt(gSldJumpIdx); gSldJump =3D 0; gSldJumpIdx =3D ""; win.GoToSld( parent.GetSldList().mList[jumpTo-1].mSldHref ) } } } function _KDH() { if( event.keyCode =3D=3D 8 ) { event.returnValue =3D 0; parent.GoToPrevSld(); } } function DocumentOnClick() { if( IsNts() || parent.HideMenu() ) return; if( ( g_allowAdvOnClick && !parent.IsFramesMode() ) || (event && (event.keyCode=3D=3D32) ) ) parent.GoToNextSld(); } var g_supportsPPTHTML =3D SupportsPPTHTML(), g_scaleInFrame =3D 1, gId=3D""= , g_bgSound=3D"", g_scaleHyperlinks =3D false, g_allowAdvOnClick =3D 1, g_showInBrowser = =3D 0, gLoopCont =3D 0, gUseSldTimings =3D 1; var g_showAnimation =3D g_supportsPPTHTML && SupportsPPTAnimation() && ( (w= indow.name=3D=3D"PPTSld" && !parent.IsFramesMode()) || g_showInBrowser );va= r g_animManager =3D null; var g_animUseRuntime =3D false; var g_animItemsToHide, g_animInteractiveItems, g_animSlideTime; var g_animMainSequence =3D null; var ENDSHOW_MESG=3D"Konec prezentace, klepnutím ukončíte= předvádění.", SCREEN_MODE=3D"Frames", gIsEndShow= =3D0, NUM_VIS_SLDS=3D30, SCRIPT_HREF=3D"script.js", FULLSCR_HREF=3D"fullscr= een.htm"; var gCurSld =3D gPrevSld =3D 1, g_offset =3D 0, gNtsOpen =3D gHasNts =3D gO= tlTxtExp =3D 0, gHasNarration =3D 0, gOtlOpen =3D true window.gPPTHTML=3DSupportsPPTHTML() var gMainDoc=3Dnew Array(new hrefList("slide0001.htm",1,-1,1),new hrefList(= "slide0002.htm",1,-1,1),new hrefList("slide0003.htm",1,-1,1),new hrefList("= slide0004.htm",1,-1,1),new hrefList("slide0005.htm",1,-1,1),new hrefList("s= lide0006.htm",1,-1,1),new hrefList("slide0007.htm",1,-1,1),new hrefList("sl= ide0009.htm",1,-1,1),new hrefList("slide0010.htm",1,-1,1),new hrefList("sli= de0008.htm",1,-1,1),new hrefList("slide0012.htm",1,-1,1),new hrefList("slid= e0011.htm",1,-1,1),new hrefList("slide0013.htm",1,-1,1),new hrefList("slide= 0014.htm",1,-1,1),new hrefList("slide0015.htm",1,-1,1),new hrefList("slide0= 022.htm",1,-1,1),new hrefList("slide0023.htm",1,-1,1),new hrefList("slide00= 24.htm",1,-1,1),new hrefList("slide0028.htm",1,-1,1),new hrefList("slide002= 9.htm",1,-1,1),new hrefList("slide0030.htm",1,-1,1),new hrefList("slide0031= .htm",1,-1,1),new hrefList("slide0033.htm",1,-1,1),new hrefList("slide0017.= htm",1,-1,1),new hrefList("slide0018.htm",1,-1,1),new hrefList("slide0019.h= tm",1,-1,1),new hrefList("slide0020.htm",1,-1,1),new hrefList("slide0034.ht= m",1,-1,1),new hrefList("slide0035.htm",1,-1,1),new hrefList("slide0021.htm= ",1,-1,1)); function FullScrInit() { g_allowAdvOnClick =3D GetCurSld().mAdvOnClk document.body.style.backgroundColor=3D"black" document.oncontextmenu=3Dparent._CM; document.onkeydown =3D _KDH; document.ondragstart=3DCancel document.onselectstart=3DCancel self.focus() } function Redirect( frmId ) { var str=3Ddocument.location.hash,idx=3Dstr.indexOf('#'), sId=3DGetSldId() if(idx>=3D0) str=3Dstr.substr(1); if( window.name !=3D frmId && ( sId !=3D str) ) { obj =3D GetObj("Main-File") window.location.href=3Dobj.href+"#"+sId return 1 } return 0 } var MHTMLPrefix =3D CalculateMHTMLPrefix(); function CalculateMHTMLPrefix() { if ( document.location.protocol =3D=3D 'mhtml:') { href=3Dnew String(document.location.href) Start=3Dhref.indexOf('!')+1 End=3Dhref.lastIndexOf('/')+1 if (End < Start) return href.substring(0, Start) else return href.substring(0, End) } return ''; } function GetTags(base,tag) { if(g_supportsPPTHTML) return base.all.tags(tag); else return base.getElementsByTagName(tag); } function UpdNtsPane(){ if(frames["PPTNts"]) PPTNts.location.replace( MHTMLP= refix+GetHrefObj( gCurSld ).mNtsHref ) } function UpdNavPane( sldIndex ){ if(gNavLoaded) PPTNav.UpdNav() } function UpdOtNavPane(){ if(gOtlNavLoaded) PPTOtlNav.UpdOtlNav() } function UpdOtlPane(){ if(gOtlLoaded) PPTOtl.UpdOtl() } function SetHasNts( fVal ) { if( gHasNts !=3D fVal ) { gHasNts=3DfVal UpdNavPane() } } function ToggleOtlText() { gOtlTxtExp=3D!gOtlTxtExp UpdOtlPane() } function ClearMedia() { if (PPTSld.pptSound) PPTSld.pptSound.loop =3D 0; } function FullScreen() { if ( PPTSld.g_animUseRuntime ) PPTSld.document.body.pause(); ClearMedia(); var href =3D ( document.location.protocol =3D=3D 'mhtml:') ? FULLSCR_HREF = : FULLSCR_HREF+"#"+GetHrefObj(gCurSld).mSldHref; if(PPTNav.event.ctrlKey) { var w =3D (window.screen.availWidth * 1.0) / 2.0 var h =3D w * (PPTSld.g_origH * 1.0) / PPTSld.g_origW win =3D window.open( MHTMLPrefix+href,null,"toolbar=3D0,resizable=3D1,top= =3D0,left=3D0," + "width=3D"+ w + ",height=3D" + h ); if( win.document.body && PPTSld.g_animUseRuntime ) win.document.body.PPTSldFrameset=3Dwindow; } else { win =3D window.open( MHTMLPrefix+href,null,"fullscreen=3Dyes" ); if( win.document.body && PPTSld.g_animUseRuntime ) win.document.body.PPTSldFrameset=3Dwindow; } } function ToggleVNarration() { rObj=3DPPTSld.document.all("NSPlay") if( rObj && !PPTSld.g_animUseRuntime ) { if( (rObj.playState =3D=3D 1)||(rObj.playState =3D=3D 0) ) rObj.Play() else if( rObj.playState =3D=3D 2 ) rObj.Pause() else return; } else if( PPTSld.g_animUseRuntime ) { narObj =3D PPTSld.document.all("narrationID") if( narObj ) narObj.togglePause() } } function GetCurSldNum() { obj=3DGetHrefObj(gCurSld) if( obj.mOrigVis =3D=3D 1 ) return obj.mSldIdx else return gCurSld } function GetNumSlds() { if( GetHrefObj(gCurSld).mOrigVis =3D=3D 1 ) return GetSldList().mNumVisSlds; else return GetSldList().mList.length } function GetSldNum( href ) { for(ii=3D0; ii 1 ) PopSldList(); else if( !IsFramesMode() ) { if( gLoopCont ) GoToFirst() else EndShow() } } function GoToPrevSld() { ii=3DgCurSld-1 if( ii > 0 ) { obj=3DGetHrefObj(ii) while ( obj && ( obj.mVis =3D=3D 0 ) && ( ii>0 ) ) obj=3DGetHrefObj(--ii) if( ii =3D=3D 0 ) ii=3D1 GoToSldNum(ii) } } function GoToFirst(){ GoToSld( GetHrefObj(1).mSldHref ) } function GoToLast() { ii=3DGetSldList().mList.length if( ii !=3D gCurSld ) GoToSld( GetHrefObj(ii).mSldHref ) } function GoToSldNum( num ) { if( PPTSld.event ) PPTSld.event.cancelBubble=3Dtrue obj =3D GetHrefObj( num ) obj.mVis=3D1 gPrevSld=3DgCurSld gCurSld =3D num; PPTSld.location.replace(MHTMLPrefix+obj.mSldHref) if( IsFramesMode() ) { UpdNavPane(); UpdOtlPane(); UpdNtsPane() } } function GoToSld( href ) { if( PPTSld.event ) PPTSld.event.cancelBubble=3Dtrue GetHrefObj( GetSldNum(href) ).mVis=3D1 PPTSld.location.replace(MHTMLPrefix+href) } function SldUpdated( id ) { if( id =3D=3D GetHrefObj(gCurSld).mSldHref ) return gPrevSld=3DgCurSld gCurSld=3DGetSldNum(id) if( IsFramesMode() ) { UpdNavPane(); UpdOtlPane(); UpdNtsPane() } } function PrevSldViewed(){ GoToSld( GetHrefObj(gPrevSld).mSldHref ) } function HasPrevSld() { return ( gIsEndShow || ( gCurSld !=3D 1 && GetHrefO= bj( gCurSld-1 ).mVis =3D=3D 1 )||( GetCurSldNum() > 1 ) ) } function HasNextSld() { return (GetCurSldNum() !=3D GetNumSlds()) } function CloseWindow() { if( HideMenu() ) return; var event =3D PPTSld.event; if( !IsFramesMode() && event && (event.keyCode=3D=3D27 || event.keyCode=3D= =3D32 || event.type=3D=3D"click" ) ) window.close( self ); CatchNumKeys( self, event ); } function Unload() { gIsEndShow=3D0; } function SetupEndShow() { gIsEndShow=3D1; PPTSld.document.body.scroll=3D"no"; PPTSld.document.onkeypress=3DCloseWindow; PPTSld.document.onclick=3DCloseWindow; PPTSld.document.oncontextmenu=3D_CM; } function EndShow() { if( IsFramesMode() ) return if( PPTSld.event ) PPTSld.event.cancelBubble=3Dtrue doc=3DPPTSld.document var dir =3D doc.body.dir if( dir !=3D "rtl" ) dir =3D "ltr"; doc.open() doc.writeln('

' + ENDSHOW_MESG + '
') doc.close() } function SetSldVisited(){ GetSldList().mList[gCurSld-1].mVisited=3Dtrue } function IsSldVisited(){ return GetSldList().mList[gCurSld-1].mVisited } function hrefList( sldHref, visible, advDelay, advClk ) { this.mSldHref=3D this.mNtsHref =3D sldHref this.mOrigVis=3D this.mVis =3D visible this.mVisited=3D false this.mAdvDelay=3D advDelay this.mAdvOnClk=3D advClk } function SldList(arr,curSld,fEnd) { this.mCurSld =3D curSld; this.mList =3D new Array(); var idx =3D 1; for(ii=3D0;ii 0) { PushSldList(sldList,fEnd); gCurSld =3D 1; } else if( PPTSld.event ) PPTSld.event.cancelBubble=3Dtrue } function PushSldList(arr,fEnd) { var ii =3D gSldStack.length; gSldStack[ii] =3D new SldList(arr,gCurSld,fEnd); GoToSld( gSldStack[ii].mList[0].mSldHref ); } function PopSldList() { if (gSldStack[gSldStack.length-1].fEndShow) EndShow() else { gCurSld =3D gSldStack[gSldStack.length-1].mCurSld; gSldStack[gSldStack.length-1] =3D null; gSldStack.length--; var sldList =3D gSldStack[gSldStack.length-1]; GoToSld( sldList.mList[gCurSld - 1].mSldHref ); } } var custShowList=3Dnew Array(); function ImgBtn( oId,bId,w,action ) { var t=3Dthis t.Perform =3D _IBP t.SetActive =3D _IBSetA t.SetInactive=3D _IBSetI t.SetPressed =3D _IBSetP t.SetDisabled=3D _IBSetD t.Enabled =3D _IBSetE t.ChangeIcon =3D null t.UserAction =3D action t.ChgState =3D _IBUI t.mObjId =3D oId t.mBorderId=3D bId t.mWidth =3D w t.mIsOn =3D t.mCurState =3D 0 } function _IBSetA() { if( this.mIsOn ) { obj=3Dthis.ChgState( gHiliteClr,gShadowClr,2 ) obj.style.posTop=3D0 } } function _IBSetI() { if( this.mIsOn ) { obj=3Dthis.ChgState( gFaceClr,gFaceClr,1 ) obj.style.posTop=3D0 } } function _IBSetP() { if( this.mIsOn ) { obj=3Dthis.ChgState( gShadowClr,gHiliteClr,2 ) obj.style.posLeft+=3D1; obj.style.posTop+=3D1 } } function _IBSetD() { obj=3Dthis.ChgState( gFaceClr,gFaceClr,0 ) obj.style.posTop=3D0 } function _IBSetE( state ) { var t=3Dthis GetObj( t.mBorderId ).style.visibility=3D"visible" if( state !=3D t.mIsOn ) { t.mIsOn=3Dstate if( state ) t.SetInactive() else t.SetDisabled() } } function _IBP() { var t=3Dthis if( t.mIsOn ) { if( t.UserAction !=3D null ) t.UserAction() if( t.ChangeIcon ) { obj=3DGetObj(t.mObjId) if( t.ChangeIcon() ) obj.style.posLeft=3Dobj.style.posLeft+(t.mCurState-4)*t.mWidth else obj.style.posLeft=3Dobj.style.posLeft+(t.mCurState-0)*t.mWidth } t.SetActive() } } function _IBUI( clr1,clr2,nextState ) { var t=3Dthis SetBorder( GetObj( t.mBorderId ),clr1,clr2 ) obj=3DGetObj( t.mObjId ) obj.style.posLeft=3Dobj.style.posLeft+(t.mCurState-nextState)*t.mWidth-obj= .style.posTop t.mCurState=3DnextState return obj } function TxtBtn( oId,oeId,action,chkState ) { var t=3Dthis t.Perform =3D _TBP t.SetActive =3D _TBSetA t.SetInactive=3D _TBSetI t.SetPressed =3D _TBSetP t.SetDisabled=3D _TBSetD t.SetEnabled =3D _TBSetE t.GetState =3D chkState t.UserAction =3D action t.ChgState =3D _TBUI t.mObjId =3D oId t.m_elementsId=3D oeId t.mIsOn =3D 1 } function _TBSetA() { var t=3Dthis if( t.mIsOn && !t.GetState() ) t.ChgState( gHiliteClr,gShadowClr,0,0 ) } function _TBSetI() { var t=3Dthis if( t.mIsOn && !t.GetState() ) t.ChgState( gFaceClr,gFaceClr,0,0 ) } function _TBSetP() { if( this.mIsOn ) this.ChgState( gShadowClr,gHiliteClr,1,1 ) } function _TBSetD() { this.ChgState( gFaceClr,gFaceClr,0,0 ) this.mIsOn =3D 0 } function _TBSetE() { var t=3Dthis if( !t.GetState() ) t.ChgState( gFaceClr,gFaceClr,0,0 ) else t.ChgState( gShadowClr,gHiliteClr,1,1 ) t.mIsOn =3D 1 } function _TBP() { var t=3Dthis if( t.mIsOn ) { if( t.UserAction !=3D null ) t.UserAction() if( !t.GetState ) return if( t.GetState() ) t.SetPressed() else t.SetActive() } } function _TBUI( clr1,clr2,lOffset,tOffset ) { SetBorder( GetObj( this.mObjId ),clr1,clr2 ) Offset( GetObj( this.m_elementsId ),lOffset,tOffset ) } function Offset( obj, top, left ){ obj.style.top=3Dtop; obj.style.left=3Dle= ft } function SetBorder( obj, upperLeft, lowerRight ) { s=3Dobj.style; s.borderStyle =3D "solid" s.borderWidth =3D 1 s.borderLeftColor =3D s.borderTopColor =3D upperLeft s.borderBottomColor=3D s.borderRightColor =3D lowerRight } function GetBtnObj(){ return gBtnArr[window.event.srcElement.id] } function BtnOnOver(){ b=3DGetBtnObj(); if( b !=3D null ) b.SetActive() } function BtnOnDown(){ b=3DGetBtnObj(); if( b !=3D null ) b.SetPressed() } function BtnOnOut(){ b=3DGetBtnObj(); if( b !=3D null ) b.SetInactive() } function BtnOnUp() { b=3DGetBtnObj() if( b !=3D null ) b.Perform() else Upd() } function GetNtsState(){ return parent.gNtsOpen } function GetOtlState(){ return parent.gOtlOpen } function GetOtlTxtState(){ return parent.gOtlTxtExp } function NtsBtnSetFlag( fVal ) { s=3Ddocument.all.item( this.m_flagId ).style s.display=3D"none" if( fVal ) s.display=3D"" else s.display=3D"none" } function _BSetA_Border(){ b =3D gBtnArr[this.mObjId]; if( b !=3D null ) b.S= etActive() } function _BSetI_Border(){ b =3D gBtnArr[this.mObjId]; if( b !=3D null ) b.S= etInactive() } function _BSetP_Border(){ b =3D gBtnArr[this.mObjId]; if( b !=3D null ) b.S= etPressed() } function _BSetA_BorderImg() { b =3D gBtnArr[this.mBorderId] if( b !=3D null && this.mIsOn && !b.GetState() ) { obj=3Dthis.ChgState( gHiliteClr,gShadowClr,2 ) obj.style.posTop=3D0 } } function _BSetI_BorderImg() { b =3D gBtnArr[this.mBorderId] if( b !=3D null && this.mIsOn && !b.GetState() ) { obj=3Dthis.ChgState( gFaceClr,gFaceClr,1 ) obj.style.posTop=3D0 } } var gHiliteClr=3D"THREEDHIGHLIGHT",gShadowClr=3D"THREEDSHADOW",gFaceClr=3D"= THREEDFACE" var gBtnArr =3D new Array() gBtnArr["nb_otl"] =3D new TxtBtn( "nb_otl","nb_otlElem",parent.ToggleOtlPan= e,GetOtlState ) gBtnArr["nb_otlElem"] =3D new TxtBtn( "nb_otl","nb_otlElem",parent.ToggleOt= lPane,GetOtlState ) gBtnArr["nb_nts"] =3D new ImgBtn( "nb_nts","nb_ntsBorder",10,parent.ToggleN= tsPane ) gBtnArr["nb_nts"].SetActive =3D _BSetA_BorderImg; gBtnArr["nb_nts"].SetInactive =3D _BSetI_BorderImg; gBtnArr["nb_ntsBorder"] =3D new TxtBtn( "nb_ntsBorder","nb_ntsElem",parent.= ToggleNtsPane,GetNtsState ) gBtnArr["nb_ntsElem"] =3D new TxtBtn( "nb_ntsBorder","nb_ntsElem",parent.To= ggleNtsPane,GetNtsState ) gBtnArr["nb_prevBorder"] =3D gBtnArr["nb_prev"]=3D new ImgBtn( "nb_prev","n= b_prevBorder",30,parent.GoToPrevSld ) gBtnArr["nb_nextBorder"] =3D gBtnArr["nb_next"]=3D new ImgBtn( "nb_next","n= b_nextBorder",30,parent.GoToNextSld ) gBtnArr["nb_sldshw"]=3D new ImgBtn( "nb_sldshw","nb_sldshwBorder",18,parent= .FullScreen ) gBtnArr["nb_sldshwBorder"] =3D new TxtBtn( "nb_sldshw","nb_sldshwBorder",pa= rent.FullScreen,null ) gBtnArr["nb_sldshwBorder"].SetActive =3D _BSetA_Border; gBtnArr["nb_sldshwBorder"].SetInactive =3D _BSetI_Border; gBtnArr["nb_sldshwText"] =3D new TxtBtn( "nb_sldshw","nb_sldshwText",parent= .FullScreen,null ) gBtnArr["nb_sldshwText"].SetActive =3D _BSetA_Border; gBtnArr["nb_sldshwText"].SetInactive =3D _BSetI_Border; gBtnArr["nb_voice"] =3D gBtnArr["nb_voiceBorder"] =3D new ImgBtn( "nb_voice= ","nb_voiceBorder",18,parent.ToggleVNarration ) gBtnArr["nb_otlTxtBorder"] =3D gBtnArr["nb_otlTxt"]=3D new ImgBtn( "nb_otlT= xt","nb_otlTxtBorder",23,parent.ToggleOtlText ) gBtnArr["nb_ntsBorder"].m_flagId=3D "nb_nts" gBtnArr["nb_ntsBorder"].SetFlag =3D NtsBtnSetFlag gBtnArr["nb_otlTxt"].ChangeIcon=3D GetOtlTxtState var sNext=3D"Další",sPrev=3D"Předchozí",sEnd=3D"Kon= ec prezentace",sFont=3D"Arial",sArrow=3D"Šipka",sFreeform=3D"Voln&yacu= te; tvar",sRect=3D"Obdélník",sOval=3D"Ovál" function ShowMenu() { BuildMenu(); var doc=3DPPTSld.document.body,x=3DPPTSld.event.clientX+doc.scrollLeft,y= =3DPPTSld.event.clientY+doc.scrollTop m =3D PPTSld.document.all.item("ctxtmenu") m.style.pixelLeft=3Dx if( (x+m.scrollWidth > doc.clientWidth)&&(x-m.scrollWidth > 0) ) m.style.pixelLeft=3Dx-m.scrollWidth m.style.pixelTop=3Dy if( (y+m.scrollHeight > doc.clientHeight)&&(y-m.scrollHeight > 0) ) m.style.pixelTop=3Dy-m.scrollHeight m.style.display=3D"" } function _CM() { if( !parent.IsFullScrMode() ) return; if(!PPTSld.event.ctrlKey) { ShowMenu() return false } else HideMenu() } function BuildMenu() { if( PPTSld.document.all.item("ctxtmenu") ) return var mObj=3DCreateItem( PPTSld.document.body ) mObj.id=3D"ctxtmenu" mObj.style.visibility=3D"hidden" var s=3DmObj.style s.position=3D"absolute" s.cursor=3D"default" s.width=3D"120px" SetCMBorder(mObj,"menu","black") var iObj=3DCreateItem( mObj ) SetCMBorder( iObj, "threedhighlight","threedshadow" ) iObj.style.padding=3D2 CreateMenuItem( iObj,sNext,M_GoNextSld,M_True ) CreateMenuItem( iObj,sPrev,M_GoPrevSld,M_HasPrevSld ) CreateSeparator( iObj ) CreateMenuItem( iObj,sEnd,M_End,M_True ) mObj.style.visibility=3D"visible" } function Cancel() { window.event.cancelBubble=3Dtrue; window.event.returnVa= lue=3Dfalse } function Highlight() { ChangeClr("activecaption","threedhighlight") } function Deselect() { ChangeClr("threedface","menutext") } function Perform() { e=3DPPTSld.event.srcElement if( e.type=3D=3D"menuitem" && e.IsActive() ) e.Action() else PPTSld.event.cancelBubble=3Dtrue } function ChangeClr( bg,clr ) { e=3DPPTSld.event.srcElement if( e.type=3D=3D"menuitem" && e.IsActive() ) { e.style.backgroundColor=3Dbg e.style.color=3Dclr } } function M_HasPrevSld() { return( parent.HasPrevSld() ) } function M_GoNextSld() { if( gIsEndShow ) M_End(); 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charset="us-ascii" Klinická biochemie v pediatrii a gerontologii ------=_NextPart_01CAAFFC.BB47E170 Content-Location: file:///C:/B134650B/vek_soubory/outline.htm Content-Transfer-Encoding: quoted-printable Content-Type: text/html; charset="us-ascii"

Po= známky

Prezentac= e

Osnova

1

Klinická biochemie v pediatrii a gerontologii

2

Věk

je jednou ze základních preanalytických neovlivnitelných proměnných

pro jednotlivé věkové skupiny platí rozd&i= acute;lné referenční intervaly

řada speciálních metod je využív&aacu= te;na pouze určitou věkovou skupinou

3

Věkov&eacut= e; skupiny v klin. biochemii

novorozenci (vč. nedonošených)

děti (do ukončení puberty)

dospělí

stárnoucí skupina (u žen po menopaze)

4

Novorozenec R= 11; specifika vzorků

pupečníková krev

kapilární krev z paty, venózní z ži= l na hlavě nebo i.v. katetrů

křehkost erytrocytů

význam POCT

5

Novorozenec R= 11; specifika testů

hemoglobin

bilirubin

protein

enzymy (CK, ALT, AST, GMT)

acidobazická rovnováha

glukóza

lipidy

fyziologický hyperthyroidismus

6

Novorozenec R= 11; hemoglobin

po porodu klesá počet ery a koncentrace Hb do někol= ika dní po porodu

donošení novorozenci mají 80% HbF a 20% HbA a na konci prvního roku je přítomen pouze HbA

7

Novorozenec R= 11; bilirubin

koncentrace je fyziologicky nízká v pupeční= ;kové krvi, koncentrace fyziologicky přechodně stoupá jak= o důsledek zvýšeného obratu Hb (vrchol 3-5 dní) s max= imem 205 umol/l (nedonošenci 274)

hemolytická nemoc novorozence, jádrový ikterus<= /li>

vzhledem k nezralosti jater je bilirubin nekonjugovaný

Přímé stanovení bilirubinu (A₄₅₄ – A₅₄₀)

8

Novorozenec –protein, enzymy, glukóza

Proteinémie po porodu stoupá z důvodu přesunu tekutin do intersticia (změna tlakových poměrů= ).

Enzymy (CK, ALT, AST, GMT) mají v séru vyšší katalytické koncentrace.

Glykémie je nižší vzhledem k malým zásobám glykogenu.

9

Novorozenec R= 11; ABR, ionty

Saturace O2 je nízká a během hodin stoupá. První den je fyziologická metabolická acid&oacu= te;za (kys. mléčná), která ustoupí do 24 = h.

První poporodní den je zvýšený sod&= iacute;k, draslík (až 7 mmol/l), chloridy, vápník.

Lipidy jsou nízké a stoupají po začá= ;tku příjmu potravy.

10

Vrozené = vady metabolismu

Závažné

Vzácné

AR dědičnost

Různý klinický obraz (intoxikace vs. neprospívání)

Nutná specializovaná vyšetření

Rutinní vyšetření: ABR, glukóza, amon= iak, laktát, v moči ketony

11

VVM – základní skupiny

Poruchy metabolismu aminokyselin

Organické acidurie

Poruchy oxidace mastných kyselin

Poruchy cyklu močoviny

12

VVM - vyše= tření

Plasma

Aminokyseliny

Karnitin, acylkarnitiny

Mastné kyseliny

Moč

Organické kyseliny

Acylglyciny

13

Skríning novorozenců

z kapky krve na filtračním papíře

Fenylketonurie - Guthrieho test

Hypothyreóza

14

Klinická biochemie poruch růstu

Růst a růstový hormon

Fyziologie GH u dítěte a dospělého

Fyziologie IGF –I a IGF-II

Fyziologie IGFBG-3

15

Klinická biochemie poruch růstu

vyšetřovány GH (bazálně i po stimulaci), IGF-I, IGFBP-3

Deficit GH (méně)

Necitlivost ke GH (častěji, obvykle částeč= ;ná)

Akromegalie a supresní test GH (jako oGTT)

16

17

18

19

20

21

Hlavní r= ole CFTR proteinu

Transport vody a iontů (Cl^-)- chloridový kanál

transport ATP -> regulace ORCC, ENaC

Sensor intracelulárních hladin ATP

Úloha v endocytóze a exocytóze

Acidifikace lysozomů

Internalizační receptor pro P. aeruginosa?

alkalinizace pankreatických štáv (HCO₃/ Cl^- )

Role při apoptóze

Metabolismus NO

22

23

24

Starš&iacu= te; dětský pacient

Proteinémie stoupá na dospělé hodnoty v 10 letech

Stoupá kreatininémie (růst svalové hmoty)<= /li>
Kyselina močová poklesá do 7 let a následn= ě stoupá do 16 let na dospělé hodnoty

ALP odpovídá činnosti osteoblastů a růs= tu

25

Puberta

Začátek biochemických odlišností mezi pohlavími

Nástup cirkamenzálních rytmů u díve= k

Vyšetření hormonů u pubertas praecox, opož= ;děného nástupu puberty

26

Dospělost<= /a>

Tvoří referenční populaci

Většina biochemických ukazatelů stabilní= ;

Stoupá glykémie po zátěži, kles&aacut= e; glomerulární filtrace (clearance kreatininu), stoupá cholesterol

27

Těhotenstv= í

Hormony: estrogeny, progesteron, hCG, hPL

Nárůst intravaskulárního objemu a dilu= 9;ní pokles koncentrace hemoglobinu (absolutní množství stoupá) a albuminu

Renální funkce: nárůst GF o 40-50%

Metabolismus glukózy: inzulinová rezistence, sní= ;žení renálního prahu, nárůst postprandiálních glykémií

Hyperkoagulační stav

28

Skríning vrozených vývojových vad

upozorňuje na zvýšené riziko minimál= ně tří nejdůležitějších vývojových vad:

trizomie 21. chromosomu (M.Down)

trizomie 18. chromosomu (M.Edwards)

defektu neurální trubice (NTD)

Interpretace laboratorních výsledků vyžaduj= e přesnou znalost gestačního věku (UZ), je provád= 3;na pomocí software a vlastním výsledkem je odhad rizika výše uvedených onemocnění.

29

Skríning= VVV

laboratorní markery pro I. trimestr – PAPP-A (př&i= acute;padně free-beta hCG), odběr v 10.týdnu těhotenství=

laboratorní markery pro II. trimestr – hCG, AFP (př= ;ípadně uE3), odběr v 15.-17. týdnu těhotenství

ultrazvukové markery I. trimestru – NT (nuchální trasnlucence), NB (nosní kůstka), provádí se v 11.-12. týdnu těhotenstv&iacu= te;

30

Stárnouc= í populace

Menopauza, andropauza

Ledviny – pokles koncentrační schopnosti, pokles clearance kreatininu, snížení glukózového prahu

Pokles hladin IgG a IgM

Snížení odpovědi řady endokrinní= ch orgánů na stimulaci

Vzestup cholesterolu

Vyšetření u stavů častějš&iacut= e;ch u stárnoucí populace (dehydratace, malnutrice, deficity vitamínů a stopových prvků)

= = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = =
------=_NextPart_01CAAFFC.BB47E170 Content-Location: file:///C:/B134650B/vek_soubory/filelist.xml Content-Transfer-Encoding: quoted-printable Content-Type: text/xml; charset="utf-8" ------=_NextPart_01CAAFFC.BB47E170--

1	Klinická biochemie v pediatrii a gerontologii
2	Věk je jednou ze základních preanalytických neovlivnitelných proměnných pro jednotlivé věkové skupiny platí rozd&i= acute;lné referenční intervaly řada speciálních metod je využív&aacu= te;na pouze určitou věkovou skupinou
3	Věkov&eacut= e; skupiny v klin. biochemii novorozenci (vč. nedonošených) děti (do ukončení puberty) dospělí stárnoucí skupina (u žen po menopaze)
4	Novorozenec R= 11; specifika vzorků pupečníková krev kapilární krev z paty, venózní z ži= l na hlavě nebo i.v. katetrů křehkost erytrocytů význam POCT
5	Novorozenec R= 11; specifika testů hemoglobin bilirubin protein enzymy (CK, ALT, AST, GMT) acidobazická rovnováha glukóza lipidy fyziologický hyperthyroidismus
6	Novorozenec R= 11; hemoglobin po porodu klesá počet ery a koncentrace Hb do někol= ika dní po porodu donošení novorozenci mají 80% HbF a 20% HbA a na konci prvního roku je přítomen pouze HbA
7	Novorozenec R= 11; bilirubin koncentrace je fyziologicky nízká v pupeční= ;kové krvi, koncentrace fyziologicky přechodně stoupá jak= o důsledek zvýšeného obratu Hb (vrchol 3-5 dní) s max= imem 205 umol/l (nedonošenci 274) hemolytická nemoc novorozence, jádrový ikterus<= /li> vzhledem k nezralosti jater je bilirubin nekonjugovaný Přímé stanovení bilirubinu (A₄₅₄ – A₅₄₀)
8	Novorozenec –protein, enzymy, glukóza Proteinémie po porodu stoupá z důvodu přesunu tekutin do intersticia (změna tlakových poměrů= ). Enzymy (CK, ALT, AST, GMT) mají v séru vyšší katalytické koncentrace. Glykémie je nižší vzhledem k malým zásobám glykogenu.
9	Novorozenec R= 11; ABR, ionty Saturace O2 je nízká a během hodin stoupá. První den je fyziologická metabolická acid&oacu= te;za (kys. mléčná), která ustoupí do 24 = h. První poporodní den je zvýšený sod&= iacute;k, draslík (až 7 mmol/l), chloridy, vápník. Lipidy jsou nízké a stoupají po začá= ;tku příjmu potravy.
10	Vrozené = vady metabolismu Závažné Vzácné AR dědičnost Různý klinický obraz (intoxikace vs. neprospívání) Nutná specializovaná vyšetření Rutinní vyšetření: ABR, glukóza, amon= iak, laktát, v moči ketony
11	VVM – základní skupiny Poruchy metabolismu aminokyselin Organické acidurie Poruchy oxidace mastných kyselin Poruchy cyklu močoviny
12	VVM - vyše= tření Plasma Aminokyseliny Karnitin, acylkarnitiny Mastné kyseliny Moč Organické kyseliny Acylglyciny
13	Skríning novorozenců z kapky krve na filtračním papíře Fenylketonurie - Guthrieho test Hypothyreóza
14	Klinická biochemie poruch růstu Růst a růstový hormon Fyziologie GH u dítěte a dospělého Fyziologie IGF –I a IGF-II Fyziologie IGFBG-3
15	Klinická biochemie poruch růstu vyšetřovány GH (bazálně i po stimulaci), IGF-I, IGFBP-3 Deficit GH (méně) Necitlivost ke GH (častěji, obvykle částeč= ;ná) Akromegalie a supresní test GH (jako oGTT)
16
17
18
19
20
21	Hlavní r= ole CFTR proteinu Transport vody a iontů (Cl^-)- chloridový kanál transport ATP -> regulace ORCC, ENaC Sensor intracelulárních hladin ATP Úloha v endocytóze a exocytóze Acidifikace lysozomů Internalizační receptor pro P. aeruginosa? alkalinizace pankreatických štáv (HCO₃/ Cl^- ) Role při apoptóze Metabolismus NO
22
23
24	Starš&iacu= te; dětský pacient Proteinémie stoupá na dospělé hodnoty v 10 letech Stoupá kreatininémie (růst svalové hmoty)<= /li> Kyselina močová poklesá do 7 let a následn= ě stoupá do 16 let na dospělé hodnoty ALP odpovídá činnosti osteoblastů a růs= tu
25	Puberta Začátek biochemických odlišností mezi pohlavími Nástup cirkamenzálních rytmů u díve= k Vyšetření hormonů u pubertas praecox, opož= ;děného nástupu puberty
26	Dospělost<= /a> Tvoří referenční populaci Většina biochemických ukazatelů stabilní= ; Stoupá glykémie po zátěži, kles&aacut= e; glomerulární filtrace (clearance kreatininu), stoupá cholesterol
27	Těhotenstv= í Hormony: estrogeny, progesteron, hCG, hPL Nárůst intravaskulárního objemu a dilu= 9;ní pokles koncentrace hemoglobinu (absolutní množství stoupá) a albuminu Renální funkce: nárůst GF o 40-50% Metabolismus glukózy: inzulinová rezistence, sní= ;žení renálního prahu, nárůst postprandiálních glykémií Hyperkoagulační stav
28	Skríning vrozených vývojových vad upozorňuje na zvýšené riziko minimál= ně tří nejdůležitějších vývojových vad: trizomie 21. chromosomu (M.Down) trizomie 18. chromosomu (M.Edwards) defektu neurální trubice (NTD) Interpretace laboratorních výsledků vyžaduj= e přesnou znalost gestačního věku (UZ), je provád= 3;na pomocí software a vlastním výsledkem je odhad rizika výše uvedených onemocnění.
29	Skríning= VVV laboratorní markery pro I. trimestr – PAPP-A (př&i= acute;padně free-beta hCG), odběr v 10.týdnu těhotenství= laboratorní markery pro II. trimestr – hCG, AFP (př= ;ípadně uE3), odběr v 15.-17. týdnu těhotenství ultrazvukové markery I. trimestru – NT (nuchální trasnlucence), NB (nosní kůstka), provádí se v 11.-12. týdnu těhotenstv&iacu= te;
30	Stárnouc= í populace Menopauza, andropauza Ledviny – pokles koncentrační schopnosti, pokles clearance kreatininu, snížení glukózového prahu Pokles hladin IgG a IgM Snížení odpovědi řady endokrinní= ch orgánů na stimulaci Vzestup cholesterolu Vyšetření u stavů častějš&iacut= e;ch u stárnoucí populace (dehydratace, malnutrice, deficity vitamínů a stopových prvků)