Snímek 1 - BPP

Snímek 1 - BPP

Invazivn mukormykza Kazuistika spn lby infekce u pacienta s leukmi Hamal P., Novk Z., Zlek B., Hoza J., Michl P., Krahulk D., Svobodov L. Podpoeno Intern grantovou agenturou Univerzity Palackho v Olomouci (grant . IGA_LF_2017_031) Mukormykza Etiologie - taxonomie d Mucorales Rody Mucor M. circinelloides, M. hiemalis

Rhizopus R. arrhizus, R. microsporus Rhizomucor R. pusillus Lichtheimia (Absidia) L. corymbifera, L. ramosa Cunninghamella C. bertholletiae Saksenaea S. vasiformis

Apophysomyces A. elegans Syncephalastrum S. racemosum Mukormykza Etiologie evidence (databze) Mukormykza Etiologie srovnvac studie Mukormykza Etiologie u dt Mukormykza

Epidemiologie Zdroj infekce Penos infekce respiran cestou (inhalace spor) nepmo pdou (traumatick implantace spor) Vstupn brna infekce a patogeneze

ubikvitn oportunn patogeny, bn se vyskytujc v prod (zajiuj rozklad organickch substrt), lovka infikuj jen vzcn HCD, nosn sliznice monost en do paranazlnch dutin, orbity, mozku plce poruen ke angioinvazivita hyf trombzy, infarkty, ischemick nekrza Vnmav jedinci pacienti s imunosupres

granulocytopenie lba kortikosteroidy krevn choroby (leukmie, lymfomy) stavy spojen s malnutric a ketoacidzou (ulcerzn kolitida, dekompenzovan diabetes) Mukormykza Klinick formy 1) Rinocerebrln (rhinoorbitln, mozkov absces) vskyt spojen s klinickou trias: projevy zkladnho onemocnn (dekompenzovan DM, jatern cirhza, hemoblastza) + orbitocelulitida + meningoencefalitida prognza asto infaustn 2) Plicn (bronchopneumonie, mukormycetom, endobronchiln

mukormykza) ast komplikace akutnch hemoblastz perakutn prbh s trvalou horekou, masivn hemoptzou, dunost prognza velmi vn Mukormykza Klinick formy 3) Primrn kon komplikace rozshlch traumat nebo poplenin lokln otok a nekrza ke 4) Gastrointestinln

vzcn forma u podvyivench a tce imunosuprimovanch dt ulcerace a eroze nejastji v aludku a tlustm stev 5) Diseminovan nejastj komplikace plicn infekce hematogenn rozsev metastatick lze pedevm v mozku, ale i v srdci, stev, jtrech, ledvinch perakutn prbh s infaustn prognzou Mukormykza Diagnostika

Agresivn povaha systmov infekce nutno rychle stanovit diagnzu! Klinick pznaky Zobrazovac metody HRCT, MRI Mikrobiologick postupy Materil tk, sekret z drnu, str z resekovan tkn, BAL, krev na PCR Vyetovac metody

Mikroskopie louhov prepart pravohle se vtvc neseptovan mycelium Kultivace Sabouraudv agar identifikace sklkov kultura endogenn sporangiospry

interpretace nlezu cave laboratorn kontaminace! Molekulrn genetika PCR (+ sekvenace, hybridizace ) Mukormykza Diagnostika v mykologick laboratoi - doporuen Mukormykza Lba Stabilizace zkladnho onemocnn

prevence ketoacidzy, stabilizace diabetu, redukce imunosupresivnch lk, zven potu granulocyt Farmakologick (systmov antimykotika) Prvn volba: lipidov formy AmB (5 mg/kg/den) ABLC (v lipidovm komplexu, Abelcet) uvoluje se pi intra- i extracelulrnm kontaktu s houbovou bukou L-AMB (v lipozomu, Ambisome) uvolnn ve fagolysozomu likvidace fagocytovanch hub nzk aktivita bhem neutropenie velmi inn pi mykzch CNS, plic ABCD (v koloidn disperzi, Amphocil) vnj vazba na lipidov nosi snadn uvolnn pi kontaktu s houbovou bukou lep inek extracelulrn

Alternativa: posakonazol (400 mg 2x denn), isavukonazol Chirurgick (radikln odstrann nekrotick infikovan hmoty) rhinocerebrln resekce elisti, orbity plicn lobektomie, segmentln resekce resekce steva Mukormykza Lba v pediatrii - doporuen Mukormykza Lba v pediatrii pehled studi Invazivn mukormykza u pacienta s leukmi

Popis ppadu Mlad mu, 16 let OA od 6 let dispenzarizovan v alergologick poradn (bronchitida) RA negativn leden 2013 3. 1. diagnostikovna T-ALL bezprostedn pot zahjena chemoterapie podle protokolu AIEOP-BFM, rameno HRG po 33 dnech dosaeno remise Bhem lby recidivujc perianln absces (izolovny mimo jin ESBL+ kmeny Citrobacter, Klebsiella, 1 z hemokultury izolovn Enterobacter) postupn terapie PPT, AMI, MER, COT, TIG, profylakticky VOR i.v. jen 2013 pacient v remisi zkladnho onemocnn

tk neutropenie (0,5 109/l) + hyperferritinmie (ferritin = 4193 g/l), dlouhodob kortikoidy g /l), dlouhodob kortikoidy diabetes mellitus pznaky rinosinusitidy (bolest oka, neuralgie n. V vlevo, konjunktivitis) oftalmoplegie a protruze bulbu vlevo 15. 10. provedena chirurgick sanace PND s odbrem sekvestru na histologick a stru na mikrobiologick vyeten + zahjena empiricky lba VOR i.v. (6 mg/kg/den) + pedasn ukonena intenzivn chemoterapie T-ALL Invazivn mukormykza u pacienta s leukmi jen 2013 Popis ppadu histologie v prepartu nalezena houbov vlkna metodou PCR in situ bylo zjitno, e se jedn o Lichtheimia corymbifera dal zchyt houbovch vlken se stejnm vsledkem identifikace byl z frontlnho laloku mozkov tkn (odbr 5.11.)

mykologie 18. 10. ve stru z paranazln dutiny kultivan zachycena vlknit houba 23. 10. vlknit houba identifikovna jako Rhizomucor sp. (pozdji byla identifikace houby sekvenn analzou zmnna na Lichtheimia ramosa + stanovena citlivost in vitro k AMB a POS) dal zchyt stejnho druhu houby ze stejnho materilu 22., 23. a 30. 10. 23. 10. byla na zklad identifikace houby antimykotick lba zmnna na ABLC (5 mg/kg/den) 25. 10. pidn POS p.o. (3 400 mg) Invazivn mukormykza u pacienta s leukmi Popis ppadu listopad 2013 zjitno loisko ve frontlnm mozkovm laloku ABLC zmnn na L-AMB na dobu 2 msc, pot nvrat k ABLC (celkov doba systmov lby lipidovmi AMB 10 msc), stle v kombinaci s POS (celkov doba lby POS 12 msc) zjitno loisko na plicch pidn lokln cAMB (inhalace)

provedena exenterace lev orbity (8. 11.) opakovan sanace PND 11. 11. bakteriln sepse (pvodce Pseudomonas aeruginosa) MER + AMI Invazivn mukormykza u pacienta s leukmi Popis ppadu prosinec 2013 odstranno loisko z frontlnho mozkovho laloku extrakce zub vlevo nahoe mykologick nlezy negativn orbita kolonizovna Pseudomonas aeruginosa a enterokokem TEI, AMI, PPT, COL, CIP, TIG, MER ervenec 2014

rozshl chirurgick vkon (14 hodin) resekce horn elisti vlevo, kryt defektu patra a laterln stny nosn dutiny vlevo tpem z oblasti m. latissimus dorsi, prava cvnho zsoben, retrakce vek nyn pacient v 1. remisi T-ALL dispenzarizace plastick chirurg modelace orbity pro on protzu stomatochirurg nhrada resekovan poloviny horn elisti Invazivn mukormykza u pacienta s leukmi etnost a pozice etiologickho agens Mukormykza Zvry Pomrn vzcn onemocnn vysok mra mortality

Na oddlench s rizikovmi pacienty je nutn na tuto infekci diferenciln diagnosticky myslet zk spoluprce klinickho lkae a mykologa Pro spnou lbu je nutn rychl diagnostika nespecifick symptomy velmi mlo rychlch a spolehlivch diagnostickch metod omezen spektrum antimykotik Lebn strategie nezvis na druhu etiologickho agens je komplexn (stabilizace zkladnho onemocnn + radikln chirurgick vkon + antimykotick farmakoterapie)

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