Liver and lung transplantation in cystic fibrosis: an adult cystic fibrosis centre's experience.

Liver disease develops in one-third of patients with cystic fibrosis (CF). It is rare for liver disease to have its onset after 20 years of age. Lung disease, however, is usually more severe in adulthood. A retrospective analysis was performed on nine patients. Three patients required lung transplantation approximately a decade after liver transplant, and another underwent combined liver and lung transplants. Four additional patients with liver transplants are awaiting assessment for lung transplants. One patient is awaiting combined liver and lung transplants. With increased survival in CF, several patients may require more than single organ transplantation.

Diagnostic utility of endobronchial ultrasound-guided transbronchial needle aspiration compared with transbronchial and endobronchial biopsy for suspected sarcoidosis.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is an accurate and minimally invasive technique that has been shown to have excellent diagnostic yield in the investigation of mediastinal and hilar lymphadenopathy. There is, however, little evidence comparing this procedure to the traditional diagnostic approach of transbronchial lung (TBLB) and endobronchial (EB) biopsies combined with characteristic clinical and radiological features in sarcoidosis. AIM: To compare the diagnostic yield of EBUS-TBNA, TBLB and EB in patients with suspected sarcoidosis. METHODS: Data from 40 consecutive patients with suspected sarcoidosis who underwent combined EBUS-TBNA with TBLB and EB biopsies were recorded. RESULTS: A total of 37 patients was confirmed as sarcoidosis, and three had other diagnoses. There was no difference in diagnostic accuracy rates between EBUS-TBNA and TBLB for all stages of sarcoidosis (84% vs 78%, P= 0.77). Combined EBUS-TBNA and TBLB procedures yielded a diagnostic accuracy of 100%. There was a highly significant difference in diagnostic accuracy between EBUS-TBNA and EB in stage I (80% vs 27%) (P < 0.01) and stage II disease (86% vs 27%) (P < 0.01). Similarly, a highly significant difference in diagnostic accuracy was seen between TBLB and EB (P < 0.01). No adverse events occurred. CONCLUSION: Endobronchial ultrasound-guided transbronchial needle aspiration alone has a high diagnostic yield with a very low complication rate for patients with suspected sarcoidosis.

Use of computed tomography pulmonary angiography in the diagnosis of pulmonary embolismin patients with an intermediate probability ventilation/perfusion scan.

BACKGROUND: Computed tomography pulmonary -angiography (CTPA) plays an increasingly important role in the diagnosis of pulmonary embolism (PE). Although accurate in the detection of large PE, its accuracy in other patient groups is yet to be defined. AIM: To compare CTPA with pulmonary angiography as a second-line investigation in patients with a ventilation/perfusion (VQ) scan indicating an intermediate probability of PE. METHODS: We recruited 25 patients over a 17-month period. Subjects were eligible if they: (i). had clinically suspected PE, (ii). had a VQ scan indicating an intermediate probability of PE and (iii). were referred for pulmonary angiography. Subjects underwent CTPA within 36 h of the VQ scan. CTPA was interpreted without knowledge of the results of the pulmonary angiogram by two of the authors. RESULTS: PE was prevalent (i.e. embolus detected at pulmonary angiography) in seven of 25 subjects (28%). The sensitivity of CTPA was 57% and the specificity was 94%. CONCLUSIONS: In the setting of intermediate-probability VQ scanning, CTPA may be used to clarify the diagnosis of PE. However, a negative CTPA cannot -definitely exclude PE. Conventional pulmonary angiography may be necessary to determine the presence of PE if CTPA is negative.