“ “ Alagille Alagille ov ov s s i i ndrom ndrom bez bez žutice žutice ” ” kviz kviz Irena Barbarić

4.SyAlagille

Download PPT Report

Upload
misel-satrak
View
220
Download
6

Embed Size (px)

DESCRIPTION

SyAlagille

Citation preview

Alagilleov sindrom bez uticekvizIrena Barbari
Alagille sy. uvodautosomno dominantno nasljeivanjegen (JAG1) - 20p12-jagged-1 locus poremeaji u jetri, srcu, kostima, oku, bubrezima uz karakteristini izgled lica moe biti prisutna i blaga do umjerena mentalna retardacija
N.E, 4-mjeseca staro muko dojenenormalna, druga trudnoanesrodni roditelji terminsko, PT 2700g, PD 47cm, Apgar 10/10cijepljenje i profilaksa vitaminom D redovitoPrikaz djeteta
premjeten iz druge bolnice zbog:vruicejake sumnje na UTInenapredovanja na teini

boja stolice i urina kau urednaAnamneza
urednog stanja svijesti, blago poremeenog opeg stanja, kardio-respiratorno kompenziranmanji za dob (TT 4800g;
Laboratorijski nalazi* kliniki vidljiva utica iznad 40-50 mol/L
UZ abdomena rtg kraljenicenefroloki pregledokulistiki pregledneuropedijatrijski pregled

genetsko testiranje ?Ostale pretrage}N
Kardioloki pregledpluna stenoza

obje glavne grane PA su uedesno 2.6mm, lijevo 3.2 mmgradijent tlaka kroz PA cca 70 mmHg
kronina kolestaza i smanjen broj interlobularnih unih vodovaballooning i giant cell promjene na hepatocitimaduktalna proliferacija uz upaluBiopsija jetreA. Note the prominent hepatocytic degeneration, intracellular and canalicular cholestasis (Hematoxylin & eosin stain).B. Extensive periportal and pericellular fibrosis A
N.E. (desno) i stariji brat
vitamini topivi u mastimaminerali (kalcij, fosfor, cink)ursodeoksikolna kiselinapraenje zbog komplikacijazastoj u rastufibroza i ciroza jetreportalna hipertenzijaPraenje
genska analiza indicirana u nejasnim sluajevima

timski rad

edukacija roditelja o kronino bolesnom djetetu

razvoj variksa jednjakaPraenje
Praenjeokultno krvarenjeEGDS

proteinurija
znaajno zaostajanje u rastu i teiniusporen linearni rast i niska tjelesna teinapri roenju zaostaju 1SD, a kasnije 2-3SD od normalne populacije

testbanklive.com /! 4! ( ! ( " #$4 5#'%4 # ' 4! 4 #( # #"! 4! ! # ! $ 5 %* 8 ' ! 4 4 ! # + 8 ' ! 4 4 ! # 8 ' ! 4 4 ! # 8, ' ! 4 4 ! # -, /! 4! ( ! ( " #$4 5#'%4 # ' 4! 4 #( # #"! 4!

Documents

It En Fr En Fr Transpallet · 2585 2500 75 520 1507 378 1071 1510 2300 2515 ø1500 800 1200 E-DISCOVERY 4 4 4 4 4 4---OPTIONAL 4 4 4 4 4 4 4 4 4 DISCOVERY 4 4 4 4 4 4 4 4 4 4 4 4

Documents

No. 1 Two Hand / Five Note Sight Reading - Michael Kravchukmichaelkravchuk.com/.../01/...Finger-Sight-Reading.pdf · 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 &∑ Two Hand / Five Note

Documents

Hänsel und Gretel€¦ · ã ã ã & bb bb bb bb # # b b bb bb bb bb bb bb bb 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 44 4 4 44 4 4 44 4 4 4 4 44 4 4 4 4 44 4 4 4 4 4 4 44 Piccolo

Documents

Concerto Grosso nº 4 Elegía - WordPress.com · 2016-09-20 · ã &? & & B?? ## # 44 4 4 4 4 44 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 Flute Oboe Clarinet in Bb Bassoon

Documents

Sandu Score Print - matrixmusic.com · ã &? b b b b b b bbb bbb bbb bbb bbb bbb bbb bbb bbb 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 Alto Sax 1

Documents

(Canção Natalina) Autor: Irving Belin Arr.: Paulo€¦ · ã bbb b b b b b b b b b b bb bb bbb bbb bbb bbb bbb bbb 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

Sandu Score Print - matrixmusic.commatrixmusic.com/assets/jazz/scores/JJZ203_s.pdfã &? b b b b b b bbb bbb bbb bbb bbb bbb bbb bbb bbb 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

Capa Frente - Baião · 2013. 7. 5. · ã bbbb bb bb bb b bb b bb bb bb bbb bbb bbb bbbb bbbb bbbb bbbb bbbb 4 4 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 4 4 4 4

Documents

Giuseppe Verdi (Le Roncole, 10 ottobre 1813 – Milano, 27 ... · ã? ã V? &? &? & & & # # # # # # # # # # # # # # # # # # # # 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 4 4 4 4 4 4

Documents

javierjacinto.files.wordpress.com · 2016-09-20 · & & &? & &?? & & ã &? &? & & B?? ## # 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 Flute Oboe

Documents

Fantasy Cl. e Banda - Michele Mangani Cl. e Banda.pdfã ã bb bb bb bb # # # b b bb bb bb bb bb bb 4 4 44 4 4 44 44 4 4 44 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 44 4 4 4 4 44 4 4

Documents

Of Emerald Shires-score - Amazon S3 · 2015-08-24 · bb bb bb # # b bb bb bb bb bb 4 4 4 4 4 4 44 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 Fl. Ob. Bsn. 1 2 B.Cl

Documents

Officine - gruppoguzzo.it · Idraulica & Coste Ambiente. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 4. 4 ... garantisce la massima efficienza e semplicità

Documents

Rapport annuel 2016 - Armoup€¦ · Assemblée générale 2017 Association romande des maîtres d'ouvrage d'utilité publique 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

ADESTE FIDELES - Musicalbero · b b b ## # # # # ## # ## # b # b b 4 4 44 4 4 44 4 4 44 4 4 44 4 4 4 4 44 4 4 4 4 4 4 44 4 4 44 4 4 4 4 4 4 Flauta Oboe Fagote Clarinete Mi b Requinto

Documents

TABLE OF CONTENTS - Musikhaus Kirstein · 2014. 11. 11. · table of contents i.features ii.panel 2 2 iii.installation iv.functions 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

CONDUCTOR 26893S BACKROW POLITICS - … · 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 44..... 1stEÑAltoSaxophone 2ndEÑAltoSaxophone 1stBÑTenorSaxophone

Documents

Mens q=108 4 œ œ œ ˙.æ - Drums2play · 2017-06-07 · ã ã ã 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 Bells Xylophone 1 Xylophone 2 Vibraphone Marimba 1 Marimba 2 Bass

Documents

Hino Nacional Brasileiro - · PDF fileb b b b ## # # # ## # ## ## # # # b b b b b 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4..... Flauta

Documents

Conductor score ICARO - ALBERTO MANDARINI · V &?? # # # bb bb bb bb bb bb bb bb 4 4 4 4 44 4 4 4 4 44 4 4 44 44 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4..... Alto Saxophone 1

Documents

Star Wars - Main title Partitura con protezione · 2017-07-10 · ã ã bb bb bb bb # # # bb bb b bb bb bb bb bb 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 4 4 4 4

Documents

EFFIE SUITE - Jan Bosveld · 2014. 2. 18. · 44 44 44 44 44 44 44 44 44 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 Solo Bas tuba (Bas Trombone) Fluit C Hobo

Documents

Cond ucte r Music by Henry Mancini · ª ª b b b b b b b b b b b b b b b b b 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 44 4 4 4 4 4 4 44 4 4 4 4 4 4 Flûte Hautbois Basson ClarinetteenSib1

Documents

Nr. 115 für denprodev-unna.de/cms/upload/lageplaene/Haferkamp.pdf4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

Il Barbiere di Siviglia - Jan Bosveld · b b b b b # # # # # # # # # # # # # # # # b b b b b b 44 44 44 44 44 44 44 44 44 44 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4

Documents

444 44 4 - Textbroker...sssssssss ss s 44 44 4 4 4 4 4 44 444 4 444 44 4 444 4 4 4 4 44 4 4 4 4 444 4 4 4 4 4 44 4 4 4 44 4 4 4 4 4 4 444 4 4 4

Documents

Algemene verspreiding contractnr.051988 ANRE ...-10-5 0 5 10 15 20 25 30 35 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4] 0 10 20 30 40 50 60] Buitentemperatuur [°C] Grondwaterdebiet

Documents

Redline Tango q=120 · 2020. 12. 31. · 44 44 4 4 44 4 4 44 44 4 4 44 4 4 44 44 4 4 44 4 4 44 44 4 4 44 4 4 4 4 44 4 4 44 4 4 44 4 4 4 4 44 44 4 4 44 44 4 4 4 4 43 43 4 3 43 4 3

Documents

$odonto42012.files.wordpress.comÏ à¡± á> þÿ j 4 „4 þÿÿÿ 4 4 4 4 4 4 4 4 4 4!4"4#4$4%4&4'4(4)4*4+4,4-4.4/404142434445464748494:4;44=4>4?4@4A4B4C4D4E4F4G4H4I4J4K4L4M4N4O4P4Q4R4S4T4U4V4W4X4Y4Z4[4\4]4^4_4$

odonto42012.files.wordpress.comÏ à¡± á> þÿ j 4 „4 þÿÿÿ 4 4 4 4 4 4 4 4 4 4!4"4#4$4%4&4'4(4)4*4+4,4-4.4/404142434445464748494:4;44=4>4?4@4A4B4C4D4E4F4G4H4I4J4K4L4M4N4O4P4Q4R4S4T4U4V4W4X4Y4Z4[4\4]4^4_4

Documents