Title Primjena plazmafereze u liječenju bolesnika s trombotičkom trombocitopeničnom purpurom
Title (english) Application of plasmapheresis in the treatment of patients with thrombitic thrombocytopenic purpure
Author Ivana Hošnjak
Mentor Hrvoje Hećimović (mentor)
Committee member Pero Lučin (predsjednik povjerenstva)
Committee member Hrvoje Hećimović (član povjerenstva)
Committee member Gordana Blagojević Zagorac (član povjerenstva)
Granter University North (University centre Varaždin) (Department of Nursing) Koprivnica
Defense date and country 2021-10-12, Croatia
Scientific / art field, discipline and subdiscipline BIOMEDICINE AND HEALTHCARE Clinical Medical Sciences Nursing
Abstract Trombotička trombocitopenična purpura (TTP) rijetka je bolest koju
karakterizira mikroangiopatska hemolitička anemija i teška trombocitopenija
uzrokovana stvaranjem mikro tromba. Može biti kongenitalna, odnosno
urođena, kao rezultat mutacije u proteinu ADAMTS13 (A Dezintegrin i
metaloproteaza s Thrombo Spondin tip 1), cijepajući multimere von
Willebrandova faktora (VWF) ili stečena razvojem autoantitijela usmjerenih
protiv ADAMTS13. Izvorni opis TTP-a 1924. godine od strane Moschowitza
detaljno opisuje pentadu znakova i simptoma, koji uključuju anemiju,
trombocitopeniju, vrućicu, neurološki poremećaj i oštećenje bubrega. Na
dijagnozu TTP-a treba sumnjati u prisutnosti gore opisanih kliničkih značajki.
S obzirom na kliničku hitnost, liječenje treba započeti empirijski prije nego što
se dijagnoza potvrdi laboratorijskim ispitivanjem. Za utvrđivanje dijagnoze
TTP-a potrebna je nedostatna aktivnost ADAMTS13 od <10%. Dalje
istraživanje anti-ADAMTS13 IgG inhibitornih autoantitijela potrebno je da bi se
dokumentirao mehanizam nedostatka ADAMTS13. Zlatni standard u liječenju
TTP-a je terapijska izmjena plazme, odnosno plazmafereza. To je invazivna
terapijska metoda koja odvaja plazmu od krvnih stanica. Patogena antitijela ili
druge velike molekule se uklanjaju, a plazma se zamjenjuje humanim
albuminom i / ili svježe smrznutom plazmom. Medicinska sestra ima iznimno
važnu ulogu u liječenju bolesnika s trombotičkom trombocitopeničnom
purpurom, posebno u liječenju primjenom plazmafereze. Asistira liječniku
tijekom plasiranja dijaliznog centralnog venskog katetera, odgovorna je za
osiguravanje plazme ili albumina iz transfuzijske službe, te pripremu uređaja i
vantjelesnog kruga za plazmaferezu. Isto tako, intervencije medicinske sestre
uključuju procjenu i pripremu bolesnika za postupak plazmafereze te praćenje
eventualne pojave komplikacija.
Abstract (english) Thrombotic thrombocytopenic purpura (TTP) is a rare disease
characterized by microangiopathic hemolytic anemia and severe
thrombocytopenia caused by microthrombus formation. It can be congenitalas
a result of a mutation in the ADAMTS13 protein (A disintegrant and
metalloprotease with Thrombo Spondin type 1), cleaving von Willebrand factor
(VWF) multimers, or acquired by the development of autoantibodies directed
against ADAMTS13. The original description of TTP in 1924 by Moschowitz
describes in detail the pentad of signs and symptoms, which include anemia,
thrombocytopenia, fever, neurological disorder, and kidney damage. The
diagnosis of TTP should be suspected in the presence of the clinical features
described above. Due to clinical urgency, treatment should be initiated
empirically before the diagnosis is confirmed by laboratory examination.
Insufficient ADAMTS13 activity of <10% is required to diagnose TTP. Further
investigation of anti-ADAMTS13 IgG inhibitory autoantibodies is needed to
document the mechanism of ADAMTS13 deficiency. The gold standard in the
treatment of TTP is the therapeutic exchange of plasma, or plasmapheresis. It
is an invasive therapeutic method that separates plasma from blood cells.
Pathogenic antibodies or other large molecules are removed and plasma is
replaced by human albumin and / or fresh frozen plasma. The nurse has an
extremely important role in the treatment of patients with thrombotic
thrombocytopenic purpura, especially in the treatment using plasmapheresis.
She assists the physician during the placement of a dialysis central venous
catheter, is responsible for providing plasma or albumin from the transfusion
service, and preparing the device and extracorporeal circuit for
plasmapheresis. Also, nurse interventions include assessing and preparing
patients for plasmapheresis and monitoring for complications.
Keywords
trombotička trombocitopenična purpura
ADAMTS13
dijagnza
plazmafereza
Keywords (english)
thrombotic thrombocytopenic purpura
ADAMTS13
diagnosis
plasmapheresis
Language croatian
URN:NBN urn:nbn:hr:122:172442
Study programme Title: Nursing Study programme type: university Study level: graduate Academic / professional title: magistar/magistra sestrinstva (magistar/magistra sestrinstva)
Type of resource Text
File origin Born digital
Access conditions Open access
Terms of use
Created on 2021-11-02 11:50:15