Naslov
Primary pulmonary hypertension in a boy-plexogenic pulmonary arteriopathy

Autori
Malčić, Ivan ; Jelašić, Dražen ; Rojnić Putarek, Nataša ; Jelušić, Marija

Izvornik
Paediatria Croatica (1330-1403) 45 (2001); 77-80

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Primary pulmonary hypertension; plexogenic pulmonary arteriopathy; childhood

Sažetak
Primary pulmonary hypertension (PPH) is a rare disease, with rapid detoriation leading to death, in the mayority of cases within 2.6 years following the diagnosis.(1) It usually affects young people predominantly in females. The aetiology of PPH is unknown, although familial disease accounts for roughly 10% of case, which suggests a genetic predisposition. (2) There are three pathohystologic types of PPH: plexogenic pulmonary arteriopathy, thromboembolic hypertension and venooclusive pulmonary hypertension. We present a boy who was treated for PPH diagnosed through a pulmonary biopsy, with pulmonary vasodilators, diuretics, intermittent infusion of PGE1 during the hypertension crises, antithrombotic agents and countinuous oxygen supplementation in the last year of life. He died six years after the diagnosis, due to right ventricular failure. The diagnosis was confirmed on autopsy. It was of the plexogenic pulmonary arteriopathy type.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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