Author/Editor     Holc, Iztok; Krajnc, Ivan; Pahor, Artur; Mlakar, Boštjan
Title     Sindrom CREST - prikaz primera
Translated title     CREST syndrome - case report
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 68, št. 7-8
Publication year     1999
Volume     str. 433-5
Language     slo
Abstract     Background. We present a patient with CREST syndrome, which represents a form of systemic sclerosis. She satisfied all clinical and X ray criteria for CREST syndrome (calcinosis, Raynaudphenomenon, oesophageal dysfunction, sclerodactyly, teleangiectases), internal organs were also involved. She had positive antinuclear, anticentromere and antimitochondric antibodies. The patient essential clinical problem was cardiac failure with pulmonary oedema. Cause of the condition was dilatatiue cardiomyopathy and pericarditis. Suddenly she developed respiratory effort and cardiac arrest. Extensive pulmonary embolism caused the death. Conclusions. The autopsy confirmed extenstve pulmmonary embolism as essential cause of death, and dilatative cardiomyopaty and pericarditis.
Summary     Izhodišča. Opisan je primer bolnice s sindromom CREST, ki je omejena oblika sistemske skleroze. Bolnica je iz- polnjevala vsa klinična in rentgenološka merila za omenjeni sindrom (kalcinacije, Raynaudovfenomen, spremembe požiralnika, sklerodaktilija, teleangiektazije), prizadeti pa so bili tudi notranji organi. Dokazali smo tudi antinuklearna, anticentromerna in antimitohondrijska protitelesa. Pri bolnici je bil osnovni klinični problem srčno popuščanje z zastojem, ob dilatativni kardiomiopatiji inperikarditisu. Pri bolnici je prišlo do nenadnega zastoja dihanja, srčne odpovedi in hitre smrti. Zaključki. Obdukcija je potrdila obsežno pljučno embolijo kot osnovni vzrok smrti, dilatativno kardiomtopatijo in perikarditis.
Descriptors     CREST SYNDROME
CALCINOSIS
CARDIOMYOPATHY, CONGESTIVE
ANTIBODIES, ANTINUCLEAR
SLOVENIA
PERICARDITIS
PULMONARY EMBOLISM