Post on 25-Nov-2015
MUCOVISCIDOZA
(FIBROZA CHISTICA)
Prof. Dr. EVELINA MORARUProf. Dr. EVELINA MORARU
UMF UMF Gr. T. PopaGr. T. Popa IASIIASI
Mai 2011Mai 2011
MUCOVISCIDOZA
DEFINITIE
Cea mai frecventa anomalie monogenica A.R.
Rasa caucaziana evolutie cronica potential letala.
Frecventa medie Europa 1/2500 nou nascuti.
Problema de sanatate publica pe plan mondial.
DOROTHY ANDERSONcaracterul vascos al
mucusului1938
Actualitati in FC:-Bio-predictori ai supravietuirii si ameliorariea calitatii vietii-Cresterea sperantei de viata
-Terapia genica si perspectiva altor terapii specifice-Modularea transportului ionic: transportul Cl prin alte canale decat CFTR
Proteoliza
Inflamatie
Pancreas normal
Pancreas cu FC
Secretie HCO3-normala
Secretie HCO3-scazuta
Secretieenzimatica
Celulesecretoare
GENETICA MUCOVISCIDOZEI
Gena defectiva brat lung cromozom 7 Implicate foarte multe mutatii (Alele) peste
1000
Cea mai frecventa mutatie DF 508 EXON 10 (media europeana 70%)
Gena defectiva proteina patologica cu rol in transportul clorului la nivelul membranei celulare.
CFTR Cystic fibrosis conductance regulator. (Reglator al conductantei transmembranare ).
Factori genetici
Factori genetici
Factori genetici
Clasificare genotip FC dupa clasa de
mutatii functionale
Clasa mutatii I, II, III rata mortalitate
crescuta
Clasa mutatii IV, V rata mortalitate
scazuta
Factori genetici
Corelatii intre aspectul initial al bolii
si evolutia clinica
Bolnavi initial cu simptome (respiratorii
si gastrointestinale)
Supravietuire scurta
Functie pulmonara deteriorata
Incidenta crescuta Pseudomonas
aeruginosa
Problema mucoviscidozei in Romania
Mucoviscidoza nu este suficient cunoscuta
Boala nediagnosticata
Diagnostic tardiv
Complicatii ireversibile
Eficienta terapiei scazuta
In Romania
la 200.000 nasteri/an
80 90 cazuri noi mucoviscidoza
In realitate numar cazuri nou diagnosticate inacceptabil de mic.
SUBSTRATUL FIZIOPATOLOGIC SI RELATIA CU TABLOUL CLINIC
Consecinta anomaliei genetice blocarea si functionarea defectuoasa a canalelor de clor la nivel celular implicit ClNa si apa.
Secretiile organe si sisteme
cu continut sarac in apa
vascoase
aderente la epiteliile canaliculilor excretorii greu de eliminat
SUBSTRATUL FIZIOPATOLOGIC SIRELATIA CU TABLOUL CLINIC
Acumularea secretiilor alterarea functiilor organelor si distructia lor:
Plaman
pancreas
ficat
intestin
organe reproducere
Tegumente concentratie foarte crescuta in sare
Tablou clinic polimorf:
Semne majore:
Suferinta respiratorie cronica tuse cronica
Diaree cronica cu steatoree
Falimentul cresterii
FIBROZA CHISTICA / BOALA CRONICA FIBROZA CHISTICA / BOALA CRONICA DEBUT PRECOCEDEBUT PRECOCE
CF MUTATIE GENETICA
DISFUNCTIE CFTR
TRANSPORT IONIC ANORMAL
MUCUS VASCOS, ADERENT
INFLAMATIE OBSTRUCTIE
INFECTIE
INSUFICIENTA PULMONARA
DISTRUCTIE CAI AERIENE PULMONARE
MOARTE PRECOCE
CERC VICIOS: OBSTRUCTIE, INFECTIE, INFLAMATIEPROGRESIV, IN FINAL PERMANENTA, DISTRUCTIVA, SCADE SPERANTA DE VIATA
CICLUL VICIOS SE INSTALEAZA PRECOCE, ANTERIOR SIMPTOMELOR EVIDENTE
RUPEREA ACESTOR SECVENTE PRIN STRATEGII TERAPEUTICE PRECOCE. OFERA SANSA PROGRESIEI LENTE, SUPRAVIETUIRII MAI MARI, Q V AMELIORATA
KONSTAN, Am. J. KONSTAN, Am. J. RespResp. . CritCrit. Care Med. 1995. Care Med. 1995TIDDENS, TIDDENS, PediatrPediatr. . PulmonolPulmonol. 2002. 2002
ROLUL CLEARANCEROLUL CLEARANCE--ULUI MUCOULUI MUCO--CILIARCILIAR
Epiteliul cailor aeriene adaptat
Autocuratirea pulmonului fiziologic se realizeaza prin mucus
F.C. transforma un mecanism fiziologic de aparare intr-o amenintare permanenta!
INFLAMATIA PULMONARA IN FC
Ratjen et al. Ratjen et al. DNA DNA concentrations in BAL fluid of CF concentrations in BAL fluid of CF pacientspacients with early lung disease: with early lung disease: influence of treatment with influence of treatment with dornasedornase alpha, Pediatric alpha, Pediatric PulmonologyPulmonology 2005;39:12005;39:1--44
Inflamatie
Obstructiecai aeriene
Colonizare bacteriana
Declinul functiei pulmonare
AderentaAderenta mucoasamucoasa in FC:in FC:frumoasafrumoasa dardar mortalamortala
CurbeCurbe de de referintareferinta pentrupentru percentilulpercentilul FEV1 in FEV1 in functiefunctie de de varstavarsta la la pacientipacienti cu FCcu FC
Masculin Feminin
Michal Kulich, Margaret Rosenfeld, Jonathan Campbell, Richard Kronmal, Ron L. Gibson, Christopher H. Goss and Bonnie Ramsey Disease-specific Reference Equations for Lung Function in Patients with Cystic Fibrosis, American Journal of Respiratory and Critical Care Medicine Vol 172. pp. 885-891, (2005)
IMPLICATIILE DECLINULUI FUNCTIEI IMPLICATIILE DECLINULUI FUNCTIEI PULMONAREPULMONARE
SCADEREA FEV1 CU 10 % IN 2 ANI DUBLEAZA RISCUL DECESULUI
MORTALITATE MARE LA CEI CU FEV1 LA VARSTE MICI (6 17 ANI) MAI MULT DECAT LA ADULTI (>17 ANI)
HRCT PREDICTIV PRECIS AL PROGRESIEI BOLII (ANUAL)
RABIN. HR, BUTLER SM RABIN. HR, BUTLER SM Pulmonary exacerbation in cystic fibrosisPulmonary exacerbation in cystic fibrosisPediatrPediatr. . PulmonolPulmonol. 2004; 37. 2004; 37--400400--406 406
PREDICTIA ETR PREDICTIA ETR SCADEREA FEVSCADEREA FEV11 Fiecare ETR are impact asupra supravietuirii scazind FEV1 cu 12% 4 ETR pe an echivaleaza cu inf. cu BURKHOLDERIA CEPACIA
5 ANI
SUPRAVIETUIRE
1 ETR = scade FEV1 cu 12%
4 ETR (un an) = scade FEV1 cu 48%
VIRSTA
ETR ANUAL
B.C. SINDROM
INFECTII (STAF,Py0)
DIABET ZAHARAT
FEV1
SEX(F)
MALNUTRITIA (SCOR Z)
FUNCTIE PANCREATICA
Liou Survivorship model
Inflamatia pulmonara si exacerbarile ei Inflamatia pulmonara si exacerbarile ei initiaza modificari fiziopatologice si initiaza modificari fiziopatologice si metabolice care au impact nutritional metabolice care au impact nutritional ((anorexieanorexie))
Casexia este determinata multifactorial: Casexia este determinata multifactorial: constelatia citokinica proinflamatorie are constelatia citokinica proinflamatorie are efect asupra statusului nutritional: TNFa efect asupra statusului nutritional: TNFa (casexina), IL1, IL6, iL8 (casexina), IL1, IL6, iL8 -- > prabusire > prabusire metabolicametabolica
Solubilizarea biofilmului prin dornaza alfa Solubilizarea biofilmului prin dornaza alfa scade rata de infectiiscade rata de infectii
PATOGENIEPATOGENIE
MANIFESTARI RESPIRATORII IN FCMANIFESTARI RESPIRATORII IN FC
DEBUT SIMPTOMATICPESTE 6 LUNI
(90% LA UN AN)
WHEEZING
BRONSIOLITA PERSISTENTA
ATOPIE (ABPA) 8%
BOALA PULMONARA OBSTRUCTIVA
INFECTII TRENANTE
BRONSIECTAZIE
SINUZITE (PANSINUZITE)
RINITE POLIPOZA NAZALA
SURDITATE TRANSMISIE
EPISTAXIS RECIDIVANT
HRCT PREDICTIV EVOLUTIVLBA - PREDICTIV INFECTIE
C
O
N
S
E
C
I
N
T
E
DECLIN
FUNCTIONAL
PNEUMOTORAX RECIDIVANT 5-8 %
HEMOPTIZIA (MINORA, MASIVA)
BRONSIECTAZIA
ATOPIA 8 10 %
DECLIN FEV1
HIPOXIE SEVERA
PERTURBARI GAZOMETRIE
REFLUX G E (25 %)
INSUFICIENTA RESPIRATORIE
MALNUTRITIE MULTIFACTORIALA
MOARTE
COMPLICATII:
INVESTIGATII AFECTARE RESPIRATORIE IN FCINVESTIGATII AFECTARE RESPIRATORIE IN FC
Diagnostic confirmare Monitorizare
- fisa clinica (puls oximetrie)- fisa familie (tuse, secretii, dispnee)- atopia (Ig E si Aspergillus)- teste functionale (CV, FEV1)- ORL, Audiograma- Rg clasica- LBA (Bronhoscopie)- HRCT- Densitometrie osoasa
RegulaRegula: : complexitatecomplexitate, , secventialitatesecventialitate!!
EXACERBARILE PULMONAREEXACERBARILE PULMONARE
1. SIMPTOME1. SIMPTOME A TUSE (frecventa, durata, intensitate)
B SPUTA (creste sau apare)
C CULOAREA SPUTEI (devine verde)
D HEMOPTIZIA (recidiva, amploare)
E TOLERANTA EFORT (scade)
F OBOSEALA PERMANENTA
2. SEMNE FIZICE2. SEMNE FIZICE A RETRACTIE INTERCOSTALA (muschi accesorii)
B POLIPNEE
C CREPITATII TORACICE
D EMFIZEM
E FEBRA
F MALNUTRITIE
3. LABORATOR3. LABORATOR A SCADE FEV1 CU 10%
B INFILTRATE CONDENSARI
C LEUCOCITOZA
D SCADE SATURATIA O2
PROGNOSTIC REZERVATPROGNOSTIC REZERVAT
Atingere multiviscerala
Sex feminin
Anomalii radiologice persistente
Colonizare bacteriana multipla
Hemoptizie recidivanta
Cord pulmonar cronic
Intreruperea cresterii staturo - ponderale
OBIECTIVELE TERAPIEI FCOBIECTIVELE TERAPIEI FC
Tratamentul
infectiilor
Combaterea
obstructiei prindrenaj secretii
Interventie
nutritionala
Controlul
inflamatiei cailor
aeriene
TRATAMENT ANTIINFECTIOSTRATAMENT ANTIINFECTIOS
Utilizarea antibioticelor
Oral exacerbari usoare
Topic TOBRA inhalator Intravenos secvente de 14 zile
Administrarea secventiala, lunara, alternativa
Utilizarea chinolonelor (1/2 doza intravenos in ETR)
Asocierea antibioticelor cu mucolitice (DN asa) amelioreaza FEV1 cu 11, 28%
Asocierea cu kinetoterapia
Vaccinarea si imunoterapia
Imunoglobuline intravenoase
--Richard B. Moss,Richard B. Moss,BonniewBonniew RamseyRamsey
REGULI ANTIBIOTERAPIE IN FCREGULI ANTIBIOTERAPIE IN FC
500 mg intravenously 500 mg intravenously every 6 h OR 1 g every 6 h OR 1 g
intravenously every 12 intravenously every 12 hh
15 mg/kg intravenously 15 mg/kg intravenously every 6 hevery 6 h
MethicillinMethicillin--resistant resistant S. S. aureusaureus
2 g intravenously every 2 g intravenously every 6 h6 h
252550 mg/kg 50 mg/kg intravenously every 6 hintravenously every 6 h
OROR
1 g intravenously every 1 g intravenously every 8 h8 h
30 mg/kg intravenously 30 mg/kg intravenously every 8 hevery 8 h
CefazolinCefazolinStaphylococcus Staphylococcus aureusaureus
ADULT DOSEADULT DOSE**PEDIATRIC DOSEPEDIATRIC DOSE**ANTIBIOTICANTIBIOTICPREVALENT PREVALENT BACTERIABACTERIA
ANTIBIOTICS FOR THE TREATMENT OF BACTERIA ASSOCIATED WITH PULMONARY EXACERBATIONS:
557.5 mg/kg 7.5 mg/kg intravenously every 8hintravenously every 8h
557.5 mg/kg 7.5 mg/kg intravenously every 8 hintravenously every 8 hAmikacinAmikacin****
3 mg/kg intravenously 3 mg/kg intravenously every 8 hevery 8 h
3 mg/kg intravenously 3 mg/kg intravenously every 8 hevery 8 hTobramycinTobramycin
PLUS PLUS aminoglycosideaminoglycoside(choose 1):(choose 1):
2 g intravenously every 2 g intravenously every 8 h8 h
50 mg/kg intravenously 50 mg/kg intravenously every 8 hevery 8 hAztreonamAztreonam
2 g intravenously every 2 g intravenously every 8 h8 h
40 mg/kg intravenously 40 mg/kg intravenously every 8 hevery 8 hMeropenemMeropenem||||
500 mg500 mg1 g 1 g intravenously every 6 hintravenously every 6 h
151525 mg/kg 25 mg/kg intravenously every 6 hintravenously every 6 hImipenemImipenem||||
3 g intravenously every 3 g intravenously every 6 h6 h
100 mg/kg 100 mg/kg intravenously every 6 hintravenously every 6 hPiperacillinPiperacillin
3 g intravenously every 3 g intravenously every 6 h6 h
100 mg/kg 100 mg/kg intravenously every 6 hintravenously every 6 h
2 g intravenously every 2 g intravenously every 8 h8 h
50 mg/kg intravenously 50 mg/kg intravenously every 8 hevery 8 hCeftazidimeCeftazidime
--lactamlactam (choose 1):(choose 1):Pseudomonas Pseudomonas
aeruginosaaeruginosa
ADULT DOSEADULT DOSE**PEDIATRIC DOSEPEDIATRIC DOSE**ANTIBIOTICANTIBIOTICPREVALENT PREVALENT BACTERIABACTERIA
ADULT DOSEADULT DOSE**PEDIATRIC DOSEPEDIATRIC DOSE**ANTIBIOTICANTIBIOTICPREVALENT PREVALENT BACTERIABACTERIA
Note: Third drug may be Note: Third drug may be added if synergyadded if synergytesting suggests efficacytesting suggests efficacy
445 mg/kg of 5 mg/kg of trimethoprimtrimethoprim
component component intravenously every intravenously every
12 h12 h
445 mg/kg of 5 mg/kg of trimethoprimtrimethoprim
component component intravenously every intravenously every
12 h12 hTrimethoprim/sulfamethoxTrimethoprim/sulfamethox
azoleazole
151520 mg/kg 20 mg/kg intravenously every intravenously every
6 h6 h
151520 mg/kg 20 mg/kg intravenously every intravenously every
6 h6 h
2 g intravenously every 2 g intravenously every 8 h8 h
50 mg/kg intravenously 50 mg/kg intravenously every 8 hevery 8 hCeftazidimeCeftazidime
557.5 mg/kg 7.5 mg/kg intravenously every intravenously every
8 h8 h
557.5 mg/kg 7.5 mg/kg intravenously every intravenously every
8 h8 hAmikacinAmikacin****
100 mg intravenously 100 mg intravenously or orally every 12 hor orally every 12 hMinocyclineMinocycline
PLUS (choose 1):PLUS (choose 1):
2 g intravenously every 2 g intravenously every 8 h8 h
40 mg/kg intravenously 40 mg/kg intravenously every 8 hevery 8 hMeropenemMeropenem
BurkholderiaBurkholderiacepaciacepacia
ADULT DOSEADULT DOSE**PEDIATRIC DOSEPEDIATRIC DOSE**ANTIBIOTICANTIBIOTICPREVALENT PREVALENT
BACTERIABACTERIA
2 g intravenously every 2 g intravenously every 8 h8 h
50 mg/kg intravenously 50 mg/kg intravenously every 8 hevery 8 hAztreonamAztreonam
3 g of 3 g of ticarcillinticarcillincomponent component
intravenously every intravenously every 6 h6 h
100 mg/kg of 100 mg/kg of ticarcillinticarcillincomponent component
intravenously every intravenously every 6 h6 h
Ticarcillin/clavulanateTicarcillin/clavulanatePLUSPLUS
OROR
445 mg/kg of 5 mg/kg of trimethoprimtrimethoprim
component component intravenously every intravenously every
12 h12 h
445 mg/kg of 5 mg/kg of trimethoprimtrimethoprim
component component intravenously every intravenously every
12 h12 hTrimethoprim/sulfametTrimethoprim/sulfamet
hoxazolehoxazole
OROR
3 g of 3 g of ticarcillinticarcillincomponent component
intravenously every intravenously every 6 h6 h
100 mg/kg of 100 mg/kg of ticarcillinticarcillincomponent component
intravenously every intravenously every 6 h6 hTicarcillin/clavulanateTicarcillin/clavulanate
StenotrophomonasStenotrophomonasmaltophiliamaltophilia
2 gm intravenously 2 gm intravenously every 8 hevery 8 h
40 mg/kg intravenously 40 mg/kg intravenously every 8 hevery 8 hMeropenemMeropenem
500 mg500 mg1 g 1 g intravenously every 6 hintravenously every 6 h
151525 mg/kg 25 mg/kg intravenously every 6 hintravenously every 6 hImipenemImipenem
PLUS (choose 1):PLUS (choose 1):
400 mg intravenously 400 mg intravenously or 500or 500750 mg orally 750 mg orally
every 12 hevery 12 h15 mg/kg intravenously 15 mg/kg intravenously
or orally every 12 hor orally every 12 hCiprofloxacinCiprofloxacin
OROR
100 mg intravenously 100 mg intravenously or orally every 12 hor orally every 12 hPLUS PLUS MinocyclineMinocycline
151520 mg/kg every 6 h20 mg/kg every 6 h151520 mg/kg 20 mg/kg
intravenously every 6 hintravenously every 6 hAchromobacterAchromobacter
xylosoxidansxylosoxidans
ADULT DOSEADULT DOSE**PEDIATRIC DOSEPEDIATRIC DOSE**ANTIBIOTICANTIBIOTICPREVALENT PREVALENT BACTERIABACTERIA
TRATAMENTUL OBSTRUCTIEITRATAMENTUL OBSTRUCTIEI
Principiul initierii terapiei antiinflamatoare sibronhodilatatoare
Drenajul mucusului Dornaza mucolitica inhalanta
Doza 2,5 mg de 1-2 ori pe zi
Scade ETR cu 37% inclusiv la sugari si copii sub 5 ani
Kinetoterapie clasica
moderna
fitness aerobic
vesta vibratii
flutter valve
Modul de viata (fumat, efort)
Masca presiune pozitiva
TRATAMENTUL ANTIINFLAMATORTRATAMENTUL ANTIINFLAMATOR
Corticoterapia nu are eficienta din AB Doze mari efecte secundare Doze mici interfera cresterea
Cromolin Na + mimetice (albuterol) IbuprofenulIbuprofenul ((studiustudiu 4 4 aniani ameliorareameliorare pulmonarapulmonara)) Macrolidele (Claritromicina / Azitromicina) Montelucast sodic (Singulair) R w 1 antitripsina Antielastaze sintetice IL 10 Trialuri in desfasurare
(anti IL 8) IFN Aerosoli
Michael W. Michael W. KonstanKonstan
ALTE TERAPII PENTRU COMPLICATIIALTE TERAPII PENTRU COMPLICATII
Drenaj agresiv Ablatie - pleurodesis chimic
- abraziune pleurala Tratamentul hemoptiziilor vit. K
substitutie embolizarea arterei bronsice
Oxigenoterapia (ventilatie mecanica, masca) Conditia fizica Transplant pulmonar
(supravietuire 84% la 1 an,61% la 3 ani)
Terapia genica
SITUATII SPECIALESITUATII SPECIALE
ComorbiditatiComorbiditati (B. (B. celiacaceliaca, , astmastm bronsicbronsic, reflux G.E., , reflux G.E., diabetdiabet, , cirozaciroza, , hepatitahepatitacronicacronica, etc), etc)
SarcinaSarcina optiuneoptiune -- 1985 = 52 1985 = 52 gravidegravide FCFC
evolutieevolutie -- 1990 = 111 1990 = 111 gravidegravide
tratamenttratament -- 2001 = 200 2001 = 200 gravidegravide
riscrisc alimentatiealimentatie -- 2010 = ?2010 = ?
AnesteziaAnestezia abordabord vascular (vascular (KorstKorst R.J.)R.J.)
hiperreactivitatehiperreactivitate laringelaringe, TR, BR, TR, BR
polipozapolipoza ((sondasonda nazalanazala))
hiperhiper secretiesecretie (se (se stopeazastopeaza pulmozymepulmozyme))
hemoragiahemoragia
ChirurgiaChirurgia polipozepolipoze, , adeno/amigdalectomiiadeno/amigdalectomii
endoscopieendoscopie
imageriaimageria
patologiepatologie chirurgicalachirurgicala intercurentaintercurenta
STRATEGII TERAPEUTICESTRATEGII TERAPEUTICE
ObiectiveObiective majoremajore::
Reducerea incidentei si severitatii ETR (exacerbari tract respirator)
Mentinerea functiei pulmonare convenabile
Scaderea ratei infectiilor
Ameliorare nutritionala
ReducereaReducerea incidenteiincidentei sisiseveritatiiseveritatii ETR ETR
((exacerbariexacerbari tract respirator)tract respirator)
MentinereaMentinerea functieifunctieipulmonarepulmonare convenabileconvenabile
Scaderea ratei Scaderea ratei infectiilorinfectiilor
Ameliorare Ameliorare nutritionalanutritionala
PREDICTORI DE PROGNOSTICPREDICTORI DE PROGNOSTIC
Leziuni Leziuni radiologiceradiologice persistentepersistente
ColonizareColonizare bacterianabacteriana
Afectarea metabolicaAfectarea metabolica
Declinul nutritionalDeclinul nutritional
InflamatiaInflamatia cailorcailor aerieneaeriene in in fibrozafibroza chisticachistica
efectulefectul tratamentuluitratamentului cu DORNAZA ALFAcu DORNAZA ALFA
Karl Paul, Ernst Karl Paul, Ernst RietschelRietschel, Manfred , Manfred BallmannBallmann, Matthias , Matthias GrieseGriese, Dieter , Dieter WorlitzWorlitz SchSch. . American Journal of American Journal of Respiratory and critical care medicine Respiratory and critical care medicine 2004, vol. 1692004, vol. 169
SS--a a studiatstudiat efectulefectul pepe termentermen lung lung asupraasupra inflamatieiinflamatiei cailorcailor aerieneaeriene((prinprin LBA) la 105 LBA) la 105 pacientipacienti > 5 > 5 aniani cu FC cu FC
Interval de Interval de studiustudiu = 3 = 3 aniani
InvaziaInvazia neutrofilaneutrofila = = identicaidentica
ElastazaElastaza sisi IL 8 = stabile (gr. IL 8 = stabile (gr. tratattratat))
ElastazaElastaza sisi IL8 (gr. IL8 (gr. netratatinetratati))
rhrh DN DN azaaza => => Reduce Reduce viscozitateaviscozitatea
AmelioreazaAmelioreaza functiafunctia pulmonarapulmonara
Reduce Reduce numarulnumarul exacerbarilorexacerbarilor pulmonarepulmonare
PULMOZYME PULMOZYME -- BENEFICIIBENEFICII
OBIECTIVE:OBIECTIVE:
Reduce riscul exacerbarilor pulmonare (ETR)
Mentine functia pulmonara la cei cu FVC > 40%
Exista experienta recenta si la sugari.
BENEFICII:BENEFICII:
Previne ETR! Reduce utilizarea ATB I.V. cu 27% Ameliorarea la 6 luni de utilizare FEV1, cu 5,8% (istoria naturala FEV1 scade
anual cu 2%) FEV1 > 85% Profil de siguranta mare, efecte secundare
minime
RECOMANDARI PEDIATRICERECOMANDARI PEDIATRICE
Pacientii peste 5 ani (beneficiari siguri)-varste tot mai mici si administrare endotraheala!
Doza 2,5 mg (2500 UI) zilnic, sau minim 6 luni de 2 ori pe zi
Studii la copii sub 5 ani cu efecte benefice si faraefecte secundare
PulmozymePulmozyme [package insert] South[package insert] SouthSan San Francisco, California, Francisco, California, GenentechGenentech inc., 2001inc., 2001
SCHEMA OPTIMA SECVENTIALASCHEMA OPTIMA SECVENTIALA
1. Autodrenaj secretii
2. Aerosoli bronho dilatator
3. Aerosoli / nebulizare PULMOZYME 10 minute dimineata (NU inainte de culcare!)
4. Kinetoterapie (20 minute 1 h dupa masa)
5. Aerosoli antibiotic
6. Aerosoli antiinflamator
PROFILAXIE PRECOCE!
Profilaxie primara:
perioada nou nascut - screening neonatal cu tripsina imunoreactiva
perioada postnatala - testul sudorii priniontoforeza pilocarpinica
Profilaxie secundara:
un protocol terapeutic complex cu scopul:
incetinirea progresiei bolii
prevenirea complicatiilor