Nalazite se na CroRIS probnoj okolini. Ovdje evidentirani podaci neće biti pohranjeni u Informacijskom sustavu znanosti RH. Ako je ovo greška, CroRIS produkcijskoj okolini moguće je pristupi putem poveznice www.croris.hr
izvor podataka: crosbi !

Cardiorespiratory complications in patients with osteogenesis imperfecta (CROSBI ID 256401)

Prilog u časopisu | stručni rad | međunarodna recenzija

Turkalj, Mirjana ; Miranović, Vesna ; Lulić-Jurjević, Rajka ; Gjergja Juraški, Romana ; Primorac, Dragan Cardiorespiratory complications in patients with osteogenesis imperfecta // Paediatria Croatica, 61 (2017), 1; 106-112

Podaci o odgovornosti

Turkalj, Mirjana ; Miranović, Vesna ; Lulić-Jurjević, Rajka ; Gjergja Juraški, Romana ; Primorac, Dragan

engleski

Cardiorespiratory complications in patients with osteogenesis imperfecta

Osteogenesis Imperfecta (OI) is the hereditary connective tissue disorder, usually caused by dominant mutations of genes coding for collagen type I alpha chains, COL1A1/A2. Although, skeletal manifestations of OI are the most readily notable, cardiopulmonary disorders among patients with OI are increasingly recognized as life-threatening but treatable disorders. Unfortunately, the majority of patients with moderate to severe types of OI die from or with cardio-pulmonary complications. The lungs as well as the heart is often an unrecognizable and neglected organ in patients with OI. In the monitoring of patients with OI the biggest attention is focused on monitoring the deformity of the long bone and spine, and indirectly the deformities of the chest wall which have consequences on development of lungs and the airways diseases. Lung disorder is frequently ignored until breathing problems become severe. An important component in patients with OI have is obstructive lung disease, sleep disordered breathing, acute as well as chronic infection often connected with resultant bronchiectasis, as well. In addition to respiratory complications, some patients with OI have serious cardiovascular problems, including severe mitral valve prolapse, aortic valve insufficiency and dilation of the aorta, which require cardiac surgery. The diagnosis and management of the lungs and cardiovascular complications in some patients with OI it is quite difficult. In all patients with OI is it important to recognize and monitor the respiratory and cardiovascular manifestation to further and prevent progression of any complications.

osteogenesis imperfecta, pulmonary disease, pulmonary funtion, lung infections, cardiovascular manifestation, prevention

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

nije evidentirano

Podaci o izdanju

61 (1)

2017.

106-112

objavljeno

1330-1403

1846-405X

Povezanost rada

Kliničke medicinske znanosti

Indeksiranost