Judith M. Morton

A review of lung transplant donor acceptability criteria

Abstract (A consensus report from The Pulmonary Council of the International Society for Heart and Lung Transplantation)

Regression of Pulmonary Lymphangioleiomyomatosis (PLAM)-associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment

Pulmonary lymphangioleiomyomatosis (PLAM) is an indication for lung transplantation (LTx). Angiomyolipomas occur in approximately 50% to 60% of patients with PLAM. We describe a patient presenting with hemoptysis post-LTx for PLAM. Computed tomography (CT) scan demonstrated no pulmonary abnormality, but identified a retroperitoneal mass confirmed as angiomyolipoma by CT-guided core biopsy. Based on experimental work that rapamycin may inhibit angiomyolipoma cells, we commenced the patient on low-dose rapamycin. She had no adverse reactions and follow-up CT scan after 7 months demonstrated almost complete resolution of the tumor. This suggests a role for rapamycin in routine post-LTx immunosuppression for PLAM.

Lung transplantation for chronic obstructive pulmonary disease at St Vincent's Hospital

Background: Lung transplantation (LTx) offers selected patients with end‐stage chronic obstructive pulmonary disease (COPD) an improved quality of life and possibly enhanced survival.

Lung transplantation in patients with cystic fibrosis.

Cystic fibrosis is one of the most common indications for lung transplantation worldwide and certainly the most common indication for all pediatric lung transplants and for bilateral lung transplantation irrespective of age. Outcomes are outstanding when compared with other indications for lung transplantation, and an increasing number of centers now report mean survival of greater than 10 years posttransplant. Hence it is important to concentrate on the broad panoply of potential systemic complications of cystic fibrosis and address proactively issues that may be associated with adverse outcomes. Optimum management of infectious, nutritional, diabetic, renal, bone, and gut complications is critical to long-term success so that recipients may realize their full potential. Timing of referral for consideration of active listing should allow sufficient time for the patient and lung transplant team to develop a productive working relationship based on best available evidence and mutual trust, which will culminate in a long-term successful outcome.

Physical activity participation by adults with cystic fibrosis: An observational study.

Studies in children with cystic fibrosis (CF) suggest greater physical activity (PA) is associated with a slower rate of decline in respiratory function. In adults with CF, objectively measured PA time and its relationship to long‐term clinical outcomes of respiratory function and need for hospitalization are unknown.

Validation of a multi-sensor armband during free-living activity in adults with cystic fibrosis ☆

BACKGROUND The SenseWear Armband (SWA) provides simple and non-invasive measures of energy expenditure (EE) during physical activity, however its accuracy in adults with cystic fibrosis (CF) during free living physical activities has not been established. METHODS 26 CF adults (mean FEV1 63% predicted; 11 males) completed a series of standardised static and active tasks with simultaneous analysis of EE via the SWA and indirect calorimetry (IC). RESULTS Mean difference and limits of agreement between EE values from the SWA and IC across all activities were -0.02METs (95% CI -1.1 to 1.1). There was moderate agreement between the two measures (ICC 0.4; 95% CI: 0 to 0.7; p=0.03). For individual activity tasks ICC ranged from 0.1 to 0.6. CONCLUSION Overall, the SWA demonstrated good agreement with IC for EE estimates in CF adults during a series of free-living activities, however accuracy was variable when assessing EE for specific activities of shorter duration.

Trough levels are inadequate for monitoring tacrolimus pharmacokinetics in lung transplantation

(AUC) monitoring may improve efficacy and decrease toxicity. Limited sampling strategies (LSS) have shown that concentration 2 hours post dose (C2) is the best single point surrogate marker for AUC. Aim: 1. To investigate AUC monitoring of CsA in lung transplantation (LTx) and compare absorption in Cystic Fibrosis (CF) versus non-CF patients. 2. To assess LSS strategies in LTx. Method: CsA levels were measured hourly over an eight-hour interval and analysed by HPLC to calculate AUC-8. Subjects: 33 patients underwent AUC. The CF group (n 10) was younger (32.3 9.3 vs 54.5 6.7 yr, p 0.001) and further post transplant (162 144 vs 58 57 weeks, p 0.05). Creatinine was similar between CF and non-CF (0.15 vs 0.14 mmol/L). (All results expressed as mean SD) Results: Despite similar C0 levels (215 106 vs 223 133 g/L, p 0.9), AUC-8 of CF was less than non-CF patients (3185 1031 vs 4717 1999 g.hr/L, p 0.03). Peak CsA concentration was lower in CF patients (768 407 g/L vs 1415 452 g/L, p 0.001) and occurred later (2hr vs 1hr, p 0.03) than in non-CF. In the group as a whole C2 had the best correlation as a single point with AUC-8 (r 0.89), however for the CF subgroup the correlation was unacceptably lower (r 0.6). Adding C0 to the model improved the correlation significantly up to r 0.95 overall, and for CF up to r 0.90. Conclusion: The optimal LSS in LTx, particularly in patients with CF has yet to be established. There are significant differences in the pharmokinetics of CsA in CF compared to other LTx recipients supporting the use of AUC monitoring as a tool to optimise CsA dosing and thereby improve outcomes.

Prevalence and impact of urinary incontinence in men with cystic fibrosis.

OBJECTIVES To determine the prevalence and impact of urinary incontinence (UI) in men with cystic fibrosis (CF). DESIGN Prospective observational study. SETTING Adult CF clinics at tertiary referral centres. PARTICIPANTS Men with CF (n=80) and age-matched men without lung disease (n=80). INTERVENTIONS Validated questionnaires to identify the prevalence and impact of UI. MAIN OUTCOME MEASURES Prevalence of UI and relationship to disease specific factors, relationship of UI with anxiety and depression. RESULTS The prevalence of UI was higher in men with CF (15%) compared to controls (10%) (p=0.339). Men with CF and UI had higher scores for anxiety than those without UI (mean 9.1 (SD 4.8) vs 4.7 (4.1), p=0.003), with similar findings for depression (6.8 (4.6) vs 2.8 (3.4), p=0.002) using the Hospital Anxiety and Depression Scale. CONCLUSIONS Incontinence is more prevalent in adult men with CF than age matched controls, and may have an adverse effect on mental health. The mechanisms involved are still unclear and may differ from those reported in women.

Intravenous ribavirin : effective therapy for respiratory syncytial virus (RSV) infection after lung transplantation

ents were CMVMM. CMVMM patients surviving 30 days were excluded (n 2). Results: Group 1(n 19) received IV ganciclovir 5mg/kg 3x week for 10 weeks. Group 2(n 7) received IV ganciclovir 5mg/kg BD for 14 days then IV ganciclovir 5mg/kg or oral ganciclovir at 1gm TDS 3x week for a further 10 weeks plus IV CMVIG @ 3x10units on days 1,2,3,7,14,28,56,84. Ref: CMV and Rejection Status Table Conclusion: Prophylatic CMV-IG resulted in a significant reduction in the incidence of rejection within the first 6 months post LTx. However it did not reduce the development of initial CMV disease. Further followup is required to determine long term outcomes, especially recurrence of CMV disease and prevalence of BOS.

Exercise after lung transplant

Lung transplantation has evolved into the standard treatment for selected patients with severe end-stage pulmonary and pulmonary–vascular disease. In the two decades since the first successful heart–lung transplant in 1981, there has been a substantial improvement in early mortality. Many heart–lung and bilateral lung transplant recipients achieve near normal lung function. Despite a significant improvement in maximal exercise capacity after lung transplantation with VO2 peak increasing to the range of 40 to 60% predicted, this remains well below normal values. Ventilatory limitation to exercise does not appear to contribute to impaired exercise capacity in those subjects with normal graft function and generally reflects the presence of complications in the allograft (for example, bronchiolitis obliterans). The evidence points toward a pretransplant injury resulting in impairment of skeletal muscle oxidative capacity with subsequent further injury after transplantation, particularly related to immunosuppressant drugs such as corticosteroids and cyclosporin A, resulting in significant peripheral limitation to exercise.