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Samenvatting leerpad B pneumo 2

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leerpad B - verdiepend 4

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  • April 7, 2023
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  • 2022/2023
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LEERPAD B: PATHOLOGIE – PNEUMO 2
MUCOVISCIDOSE (CYSTISCHE FIBROSE, TAAISLIJMZIEKTE)


OORZAAK
 Gezond gen codeert voor transmembraaneiwit dat als regelbaar chloorkanaal optreedt:
normaal volgen watermoleculen chloorionen via osmose

 Gevolgen:
– Geen watersecretie
– Indikking mucus

* CFTR-gen = cystic fibrosis transmembrane regulator




ADEMHALINGSSTELSEL
 Gecompliceerde mucociliaire lift
 Hoesten en opgeven taaie slijmen
 Recidiverende luchtweginfecties
 Clubbing
 Progressieve longdestructie
 Overlijden


SPIJSVERTERINGSSTELSEL
 Pancreas:
– Exocriene insufficiëntie door obstructie ductus pancreaticus
§ Steatorree door tekort lipasen in spijsverteringskanaal
§ Vetoplosbare vitaminen-deficiëntie (A, D, E, K)
§ Malabsorbtie en vermagering
§ Meconium ileus
– Endocriene insufficiëntie door destructie eilandjes van Langerhans
§ Diabetes type II

 Lever:
– Levercirrose
– Portale hypertensie
– Slokdarmvarices → hematemesis




1

, DIAGNOSE
 Zweettest
– Cave: normaal CFTR-gen zorgt ervoor dat merocriene cellen chloor reabsorberen →
gemuteerd CFTR-gen leidt ertoe dat er hoge concentraties chloor opgestapeld
worden in ducti
– Verhoogde concentratie chloor in zweet is dus indicatief voor mucoviscidose
– Medicijn pilocarpine in de huid leidt lokaal tot zweten → zweettest

 Genetische test
– Ter bevestiging zweettest
– 500 mutaties CFTR-gen gekend
– Dorothine Andersen (1901-1963) → ‘Andersen syndroom’ John-Hopkins University in
Baltimore


BEHANDELING
 Ademhalingskinesitherapie
 Behandeling infecties
 Toediening pancreasenzymen
 Toediening vitaminesupplementen
 Longtransplantatie



LONGEMBOLIE


OORZAAK
 Klonter van bloed, weefsel of gas in de arteria pulmonalis (zie slide 28 les 1)
– Door diepe veneuze trombose (triade van Virchow: bloeddebiet↓, stollingsgraad↑,
vaattrauma)
– Door rechter voorkamerfibrillatie



MASSIEF LONGEMBOOL OF ZADELEMBOOL
 Cardiovasculaire collaps




KLASSIEK MIDDELGROOT EMBOOL
 Longinfarct




2

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