[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]
BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna
SEPTEMBER 20, 2018
Lege Artis Medicinae - 2018;28(08-09)
BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna
SEPTEMBER 20, 2018
Lege Artis Medicinae - 2018;28(08-09)
[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion between experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]
Lege Artis Medicinae
[Those familiar with psychology are no strangers to the here-and-now attention exercises Wilhelm Reich asked his clients to allow the present moment to be observed. The here-and-now focus later become the focus of neo- and postreichian movements and a number of other psychotherapeutic movements too. Among the analysts Jung, Horney and Fromm showed a interest in the present-focused Zen Buddhist practices...]
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Clinical Oncology
[The interstitial lung diseases (ILD) are a heterogeneous group of disorders that are classifi ed together because of similar clinical, radiographic or pathologic manifestation. The diffuse interstitial lung diseases are divided into those that are associated with known causes and that are idiopathic. The treatment choices and prognosis vary among the different causes and types of ILD, so the early and correct diagnosis is important. In oncology ILD could be a rather severe adverse effect of treatment with radiationor chemotherapy.]
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[More than half of the cases of primary pulmonary hypertension belongs to the thromboembolic form which can successfully be treated by pulmonary thromboendarterectomy. The aim of the study was to demonstrate the selection of surgical candidates and the results of first operations. 22 patients were studied in the last 2 years in whom pulmonary systolic pressure was over 50 mm Hgby Doppler-echocardiography, and no cause could be identified and in whom transesophageal echocardiography was performed. Per fusion lung scan was carried out in 14, venous Doppler in 10 and pulmonary angiography in 7 cases. Out of 22 patients with suspected PPH an atrial septal defect (ASD) was found in 4 cases, chronic thromboembolic pulmonary hypertension (CTH-PH) in 10 patients and major vessel disease suitable for surgery was established in 6 of them. Acute pulmonary embolism was documented in the history of 2 and deep vein thrombosis in 6 out of the 10 cases. Patent foramen ovale was revealed in 5 of the 10 patients 2 of which led to fatal paradoxic embolism. ASD was closed in 3 cases. Two patients had successful pulmonary thromboendarterectomy followed by complete hemodynamic recovery. CTh-PH should be considered in any patient with effort dyspnea or signs of right ventricular overload for which no cardiac cause can be found, as successful surgical treatment may be possible. A diagnostic algorithm is proposed for the selection of surgical candidates. ]
Lege Artis Medicinae
[During the last decade lung transplantation became an accepted treatment option for endstage lung disease of different origin. Technical problems of the early period have been solved completely during the course of time and the operation has found its place as a routine procedure. The shortage of donor lungs has stimulated the development of new operative techniques. Parallel to achievements in surgical treatment, significant progress in immunosuppressive therapy was obtained. Long-term survival following lung transplantation however remains limited by the onset of bronchiolitis obliterans syndrome. Since Vienna serves as a leading centre for lung transplantation in Middle-Europe, between 05/03/1996 and 17/01/2003 21 Hungarian patients underwent lung transplantation here. In close co-operation with the Vienna Lung Transplant Group, the Hungarian specialists prepare for introduction of lung transplantation in Hungary.]
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