Naslov
Value of contemporary myocard bioptate specimens analysis in the diagnostic flow diagram for primary cardiomyopathies in children

Autori
Malčić, Ivan ; Jelašić, Dražen ; Kniewald, Hrvoje ; Jelušić, Marija ; Rojnić-Putarek, Nataša ; Dilber, Daniel

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Cardiology in the Young 2003 ; 12(suppl 1): (CC) / - , 2003

Skup
38th Congress of Association for European Paediatric Cardiology, Amstredam 2003

Mjesto i datum
Amsterdam, Nizozemska, 2003

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
: Primary cardiomyopathies; Endomyocardial biopsy; Children

Sažetak
The purpose of this work was to show the importance of myocard bioptate analysis using different methods in diagnosis of primary cardiomyopathies (CMP) in children. According to the guidelines of the Task Force on Cardiomyopathies of the WHO and the ISF of Cardiology, we identified 135 infants children and adolescents with CMP, 55 female (40, 7%) and 80 male (59, 3%), giving an average occurrence for all CMP of 43.32 on 10, 000 patients examined in our outpatient paediatric cardiology clinics. Dilated CMP was found in 52 patients (38, 5%), hypertrophic CMP 43 patients (31.9%), and restrictive CMP in 6 patients (4.4%). In nine patients (6, 6%) were impossible to classify the cardiomyopathy. The majority of dilated CMP diagnosis were made before the age of 3 years (RR 1.9, 95% CI 1.4- 2.47). There were no statistically significant differences in the incidences of dilated and hypertrophic CMP (Z 0.923, p 0.1779), but we encountered significantly lower occurrence of restrictive CMP (Z 6.044, p<0, 001). The biopsy of endocard and myocard was done in 22 patients, (12 male and 10 female), age 1 to 17 (average 6.3 ys). The specimens were examined by light microscope (Dallas criteria) in all patient, in 13 cases direct immunofluorescency and immunohystochemical methods were used, two were examined hystochemically, one by the microscopy in polarised light and 7 by electron microscope. Five bioptates were analysed by PCR method (DNA and RNA of cardiotrophic viruses). Four of ten children with dilated cardiomyopathy had myonecrosis as a consequence of acute myocarditis, and six of them developed signs of late inflammatory processes: immunoglobulin and complement deposits due to chronic immunologic myocarditis. In 4 patients rebiopsy proved complete healing (corticosteroide therapy). In 5 patients the hypertrophic CMP diagnosis was confirmed hystologicaly, one was additionally analysed by electron microscope to rule out the possibility of mytochondriopathy. The finding correlated with the pathology of neuromuscular system (scapulo- peroneal amiotrophy). Pathohystological findings of a bioptate specimens in patients with restrictive cardiomyopathy, showed primary amiloidosis of the heart in one case, fibroelestosis in one another, in 3 children inflammation, and in one case heart tumor (fibroma) was found.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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