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Pedi > Fibroza chistica > Flashcards

Fibroza chistica Flashcards

1
Q

Ce este CFTR?

A

canal de Cl reglat de AMPc imp pt transportul prin MC a Na,Cl, HCO3

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2
Q

Cea mai importanta complicatie a bolii

A

degradarea functiei pulmonare

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3
Q

cum apare degradarea fct pulmonare?

A

prin infectii cronice si colonizare CR cu oportunisti

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4
Q

relatia genotip-fenotip

A

puternica-fenotipul pancreatic, ileus meconial, diabet
variabila-afectarea pulmonara

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5
Q

Modulatori genetici

A

TGF, MBL, NOS, glutation,IL, mucine

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6
Q

functia CFTR

A

absorbtia Na si Cl din lumen in celula, daca e absenta ramane in lumen

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7
Q

teoria low volume

A

creste activitatea ENAC in ep cailor aeriene=>ingrosare mucus

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8
Q

manifestari clasice

A

malnutritie, diaree cu steatoree, tuse productiva persistenta

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9
Q

manifestari prenatale

A

eco:hiperecogenitate la nivel intestinal, dilatatii intestinale, mase hiperecogene, +/- peritonita meconiala

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10
Q

manifestari clinice la nou nascut

A

-ileus meconial
-falimentul cresterii prin -malabsorbtie
-icter prelungit
-gust sarat al sudorii
-deficit de vitamine liposolubile ADK
-deshidratare
-scaune grasoase
-acrodermatitis atrophicans
-wheezing
-maini de spalatoreasa

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11
Q

semnul chitului

A

palparea unei anse pline cu meconiu

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12
Q

tablou clinic in ileus meconial

A

meteorism abdominal

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13
Q

manifestari clinice respiratorii sugar si copil

A

tuse cronica, pneumonii, wheezing, sinuzita cronica, polipoza nazala

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14
Q

manifestari non respiratorii sugar si copil

A

falimentul cresterii, prolaps rectal, anemie, edeme, deshidratare, colestaza

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15
Q

manifestari la adolescent si adult

A

cele respiratorii de la sugar, bronsiectazii, hipocratism digital, pansinuzita cronica, polipoza nazala, inf cu germeni atipici, ABPA

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16
Q

hipocratism digital stadiu 1

A

fluctuenta si inmuierea patului unghial

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17
Q

hipocratism digital stadiu 2

A

alterarea ungiului Lovebond peste 165 de grade

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18
Q

hipocratism digital stadiu 3

A

creste convexitatea patului unghial

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19
Q

hipocratism digital stadiu 4

A

ingrosarea partii distale a degetului

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20
Q

hipocratism digital stadiu 5

A

aspect stralucitor si striat

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21
Q

pentru cine se face testul sudorii?

A

screening neonatal pozitiv, semne clinice, infertilitate B, frati/surori

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22
Q

Cand se face testul sudorii?

A

se face dupa 3 saptamani de viata si G>3kg

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23
Q

La ce valoare testul sudorii este pozitiv?

A

> 60mmol/l

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24
Q

Cand nu se face Testul Sudorii?

A

febra, deshidratare, fluide i.v, leziuni cutanate, corticoterapie, edeme

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25
Q

Cine face testare genetica?

A

TS pozitiv
semne clinice dar TS-
fara semne clinice dar TS+
screening neonatal +

26
Q

cum se face screening-ul neonatal?

A

IRT (tripsinogen imunoreactiv)

27
Q

Cum se procedeaza daca screening ul e pozitiv?

A

are nevoie de confirmare prin testul sudorii

28
Q

Care sunt cei mai frecventi agenti patogeni implicati in afectarea pulmonara?

A

SA, HI, PA

29
Q

stadiu timpuriu afectare pulmonara

A

asimptomatic sau tuse seaca/wheezing, tahipnee

30
Q

stadiu intermediar afectare pulmonara

A

tuse productiva, PEX

31
Q

tratament stadiu timpuriu af pulm

A

fizioterapie respiratorie, mucolitici
norme de igiena
vaccinare antigripala
inf bact

32
Q

tratament stadiu intermediar af pulm

A

ATB
antiinflamatori
supraveghere

33
Q

stadiu avansat af pulm

A

ABPA, atelectaza lobara, hemoptizie, pneumotorax, IR cronica

34
Q

tratament stadiu avansat af pulm

A

tratamentul afectiunii specifice
optimizarea tratamentului de fond
intocmire dosar transplant pulmonar FEV1<30% din prezis

35
Q

Manifestari clinice ABPA

A

-wheezing
-constrictie toracica asthma like
-febra si alterarea starii generale
-sputa maronie/neagra cu consistenta crescuta

36
Q

Criterii dg ABPA

A

-deteriorare clinica fara cauza identificata
-IgE total seric >500kU/L (200-500 retestare dupa 1-3 luni)
-prick test(cutanat) la Aspergillus
-anticorpi anti AF IgG+
-anomalii rx recente:deget de manusa

37
Q

tratament ABPA

A

corticosteroizi sistemici
+/- antifungici (itraconazol)

38
Q

cate semne din criteriile fuchs confirma PEX?

A

4/12

39
Q

Criteriile FUCHS de PEX

A

-modif aspect sputa
-hemoptizie noua/grad mare
-creste frecv tusei sau modif caract
-dispnee/accentuarea ei
-modif starii generale, oboseala, letargie
-febra>38
-anorexie, scadere ponderala
-durere/sens la niv sinusurilor
-modificari in ex fizic ap resp
-scaderea fct pulmonare cu 10% sau >
-modificari RX sugestive de inf pulm

40
Q

Ce terapie inhalatorie se foloseste in afectarea pulmonara din FC?

A

-bronhodilatatori
-clorura de Na hipertona
-Pulmozyme
-steroizi (la aia cu astm)
-ATB

41
Q

Ce mucolitice se folosesc?

A

-NaCl hiperton (RA:tuse, odinofagie)
-Pulmozyme (RA: raguseala, eruptii cutanate)
-Mannitol(Bronchitol)(RA:tuse)

42
Q

Cu ce se trateaza primo-infectia SA/HI?

A

ATB oral 2-4 saptamani in doze mai mari decat uzuale

43
Q

Cu ce se trateaza primo-infectia PA?

A

trat de URGENTA
Tobramicina inhalator 4 sapt/ ATB orale

44
Q

Cu ce se trateaza primo-infectia MRSA?

A

2 ATB orale: rifampicina+acid fusidic sau cotrimoxazol 2-4 sapt

45
Q

cu ce se trateaza inf cronice de SA, HI?

A

atb termen lung oral co-amoxiclav in regim on/off

46
Q

Cum se trateaza inf cronica cu PA?

A

ATB termen lung inhalator:
-colistin/tobramicina on/off
-colistin alternatic cu tobramicina
se prefera ap de nebulizare e-flow/iNeb sau pulbere inhalatorie

47
Q

Cu ce se asociaza PEX?

A

cu declinul functiei pulmonare

48
Q

Cum se trateaza PEX?

A

ATB oral/inhalator/i.v 2 sapt
combinatie de 2 ATB: cefalosporina de generatia a3a+aminoglicozid
ANTIINFLAMATORI
-azitromicina-induce rezistenta la SA, SP, Mycob; reducere eficacitatea TOBRAMICINEI; se face ECG inainte de initiere ptc prelungeste intervalul QTc
-ibuprofen doza inalte
-corticosteroizi inhalatori daca are astm sau oral/sistemic in ABPA, terminal care

49
Q

in ce clase de mutatii apare insuficienta pancreatica exocrina?

A

la toti din clasele I-III si la 50% din clasele IV-V

50
Q

cum se pune dg de insuficienta pancreatica exocrina?

A

analiza elastazei pancreatica in materii fecale >200microg/g

51
Q

care este tratamentul pentru cei cu insuf pancreatica exocrina?

A

substitutie cu enzime pancreatice (PERT)
sunt enzime derivate din pancreasul porcin

52
Q

afectare hepatica din FC

A

NAFLD=non alcoholic fatty liver disease

53
Q

cum apare ciroza hepatica din FC

A

apare prin obstructia ductelor biliare intrahepatice

54
Q

cum se trateaza ciroza hepatica din FC?

A

acid ursodeoxicolic
vaccinare anti hepatita A si B
contraindicatie de AINS
la fel ca orice ciroza hepatica, se poate ajunge la transplant

55
Q

Sindromul pseudo-Barterr

A

este o alcaloza metabolica hipocloremica, hipopotasemica, hiponatremica
pot apare convulsii

56
Q

Cum se monitorizeaza la 3 luni?

A

examen clinic, functie pulmonara, culturi secretii pulmonare

57
Q

cum se monitorizeaza anual?

A

imagistic (rx+ct)
laborator
eco abd
test de toleranta la glucoza
osteodensitometrie
screening psihologic
pletismografie

58
Q

Care este tratamentul pentru cls III?

A

IVACAFTOR (Kalydeco)

59
Q

la cine este CI Ivacaftorul?

A

la cei cu insuficienta hepatica severa

60
Q

Care este tratamentul pentru homozigot F508del?

A

Iva+lumacaftor (orkambi)

61
Q

Tratament homo/heterozigoti F508del

A

iva+tezacaftor+elexacaftor (potentiator+ 2 corectori)

62
Q
A

Pedi (14 decks)

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