Pulmonary hypertension, interstitial lung fibrosis, and lung iron deposition in thalassaemia major.
Although restrictive lung disease is the predominant abnormality of pulmonary function in patients with thalassaemia major (TM), its aetiology and its association with pulmonary hypertension (PH) detected in some patients with TM remains unknown. We report a patient with TM, iron overload, frequent pulmonary infections, and progressive severe precapillary PH over the previous 5 years. A severe restrictive pattern and interstitial lung fibrosis were revealed by pulmonary function tests and high resolution computed tomography, respectively. This presentation suggests that interstitial fibrosis may complicate lung involvement in TM and can significantly contribute to the development of PH.
Zakynthinos
E
E
Department of Critical Care and Pulmonary Services, University of Athens Medical School, 'Evangelismos' Hospital, Athens, Greece.
Vassilakopoulos
T
T
Kaltsas
P
P
Malagari
E
E
Daniil
Z
Z
Roussos
C
C
Zakynthinos
S G
SG
D000328:Adult; D006328:Cardiac Catheterization; D004562:Electrocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology*; D007501:Iron / Q000378:metabolism*; D008297:Male; D011658:Pulmonary Fibrosis / Q000209:etiology*; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D014463:Ultrasonography; D018497:Ventricular Dysfunction, Right / Q000209:etiology; D017086:beta-Thalassemia / Q000150:complications* / Q000378:metabolism
D007501:Iron