AIM:Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS:Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO2 (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

During their life, cystic fibrosis (CF) patients may require thoracic surgical procedures for a number of reasons before undergoing lung transplantation. In the past, this has been considered to be a contraindication to lung transplantation. However, a meticulous surgical technique and careful intraoperative management allows one to perform the transplantation safely. Herein we have reported our experience with CF patients undergoing lung transplantation after previous surgical treatment for pneumothorax or bronchiectasis.

Lung transplantation (LT) represents the only available therapy for selected patients affected by end-stage pulmonary disease. Cardiopulmonary bypass (CPBP) is used, when required, during single and sequential double lung transplantation; however, it increases the risk of bleeding, early graft dysfunction, failure, and other potential side effects. We report our experience with 145 patients who underwent lung transplantations, among whom 34 required intraoperative CPBP. The indications for LT among these 34 patients were cystic fibrosis (n = 22), chronic obstructive pulmonary disease (n = 3), bronchiectasis (n = 2), primary pulmonary hypertension (n = 1), fibrosis (n = 2), pulmonary microlithiasis (n = 1), and retransplantation for obliterative bronchilitis (n = 3). CPBP was planned in 12 cases (group I) and unplanned in 22 (group II). The main reason for planning CPBP was primary and secondary pulmonary hypertension (mean pulmonary artery pressure >or=25 mm Hg). Acute right ventricular failure, hemodynamic instability, arterial desaturation, and increased pulmonary artery pressure were mandatory for unplanned CPBP. Among the 34 CPBP patients, the 30-day mortality rate was 35% (12/34) including 9 (70%) in group II (unplanned CPBP). The leading cause of death was multiorgan failure. The 1-year survival rates were 67% and 36%, and the 3-year survival rates were 47% and 18% for groups I and II, respectively. In conclusion, even if it represents a useful tool in the management of critical events, the use of unscheduled CPBP during LT procedures is associated with an increased postoperative morbidity and mortality.

C. difficile (C. d.) is the main cause of antibiotic-associated diarrhea and colitis. It is shown in literature a high asymptomatic carriage rate of C. d. in patients with cystic fibrosis (CF), though C. d.-related colitis is an uncommon complication in these patients, despite the use of multiple high-dose antibiotic regimes and the frequency of hospital admissions. Lung transplantation with the associated immunosuppression and aggressive antibiotic therapy may increase the risk of the clinical manifestation of C. d. In this paper, we describe three cases of severe C. d. colitis in patients with CF following lung transplantation and illustrate our experience in the diagnosis and management of these patients.

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective.

Lung transplantation (LT) is the only effective form of therapy for cystic fibrosis (CF) associated with end-stage pulmonary failure. In Italy, the management of CF is regulated by national law, which has instituted regional centers for care and follow-up of all CF patients. LT has been performed since 1992 in only nine LT certified centers. The structured national organization has led to a unified database for LT for CF. As of December 2006, 197 bilateral LT (96 male and 94 female patients; 7 retransplants) have been performed. Of these, four had also liver or heart and liver transplantation, and three are long-term survivors. Overall median survival is 7 years. Mean age at transplantation is 26.5 years, and the mortality on the waiting list is 33.6%. Patients listed for transplant either received a suitable donor within a mean of 10 months or died within a mean of 5.5 months. The most frequent cause of death is bronchiolitis obliterans syndrome (BOS). Our nationwide database indicates the excellent results obtained by LT in FC. Still, mortality on the waiting list remains a challenge and long-term outcome is limited by BOS.

Background: There is limited information about treatment burden (& its monitory cost) in advanced CF lung disease prior to & after successful lung transplantation (LTx).The co-location of the Adult CF Centre & the Lung Transplant Service at TPCH provided an opportunity to assess the treatment requirements and costs.Methods: Length of stay, hospitalisation episodes, OPD visits, and cost of hospitalbased treatment were assessed (total/medication) utilising transition II & hospital pharmacy databases for patients who underwent LTx from 1996 to 2005.Sixmonthly blocks were assessed for 24 months prior to & after LTx.Results: 17 patients were assessed (mean age±sd, gender%).All had advanced lung disease (mean FEV1±SD) six months prior to LTx, (0.7±0.3 litres, 19.2±6.7% predicted, BMI 20.8±3.2 kg/m 2 ).Mean FEV1 was significantly improved post-LTx and remained stable over 24 months.Length of stay increased over the 24 months pre-LTx (F = 2.7, p < 0.001) and decreased over the 24 months post-LTx.The first six months post-LTx had equivalent hospital admission days as the six months pre-LTx (p = 0.45).Similar patterns were seen for hospital episodes and OPD attendances.Medication & total hospital-based costs tended to increase in the 24 months prior to LTx, although the medication/total cost ratio was stable.Medication & total costs were significantly higher in the first six months following LTx, compared with the equivalent period pre-LTx, however, decreased to pre-LTx levels following the first six months.Conclusions: Following recovery from LTx (six months post-LTx) costs returned to pre-LTx levels & hospital-based interventions (admissions, OPD attendances) fell to levels well below that seen prior to LTx.

Lung transplantation represents the only therapeutic option for patients affected by end-stage cystic fibrosis (CF). We performed 76 lung transplantations in 73 patients from 1996-2007. The mean time on the waiting list was 10+/-6 months. The median follow-up after the transplantation was 69.3 months. Twenty-one transplants (27.6%) were performed under cardiopulmonary bypass. Perioperative mortality, excluding retransplants, was 16.4% (12 patients) and the causes of death were sepsis, primary graft failure, and myocardial infarction. The overall survival was 74.5%+/-5%, 62.9%+/-5%, 54.1%+/-6%, and 43.4%+/-6% at 1, 3, 5, and 10 years, respectively. The accurate selection of potential recipients and the correct timing of referral and transplantation are factors that play crucial roles to obtain satisfactory results in term of improvement of quality of life and long-term survival.

S23predictive variables, respectively directly and inversely related to CFRD development (pseudo R 2 for the model: 0.581, p < 0.001).Glycated hemoglobin and baseline glucose concentrations were directly related with outcome at univariate analysis whereas c-peptide concentrations were inversely related.In contrast, no relationship emerged between insulin-sensitivity indexes and outcome.Anthropometric (weight, height z scores, BMI) and pulmonary function indexes were also unrelated.Conclusions: Defects in insulin secretion are determinants of subsequent CFRD development.The evaluation of insulin concentrations during OGTT may be helpful in CFRD prediction.

Background. Lymphoma is a serious complication following solid organ transplantation. Endobronchial involvement of non-Hodgkin's lymphoma (NHL) is rare usually occurring in the setting of widely disseminated disease. Only a few cases of isolated endobronchial NHL have been reported. They were mostly confined to the main bronchi.Methods. Herein we have described a case of an obstructive endobronchial NHL lesion in a young patient, which developed 6 years after bilateral lung transplantation for cystic fibrosis.Results. Successful treatment was obtained with endoscopic resection of the lesion followed by chemotherapy.Conclusions. In rare cases NHL may present as an isolated endobronchial mass in the main bronchi. Because in these cases the prognosis seems to be better, early diagnosis is essential. Rigid bronchoscopy offers the opportunity to safely obtain large amounts of tissue for complete histological diagnosis and to de-obstruct the airway to relieve the symptoms.

During the last 2 decades, long-term survival after lung transplantation has significantly improved. However, among the complications related to the continuous administration of immunosuppressive drugs, malignancy plays an important role. We retrospectively revisited our series of patients to report our experience. From January 1991 we performed 134 lung transplantations in 128 recipients (mean age, 33.4 +/- 13.5 years). In all patients the first-line immunosuppressive regimen was based on a calcineurin inhibitor (cyclosporine or tacrolimus), an antimetabolic agent (azathioprine), and steroids. Five patients (4.2%) developed malignancy and the mean time of occurrence after the transplantation was 46.4+/-23 months. The mean age was 41 +/- 16 years (P = not significant [ns]). The tumors were as follows: laryngeal cancer (radiotherapy), colon cancer (surgery plus adjuvant chemotherapy), gastric cancer (surgery plus adjuvant chemotherapy), endobronchial non-Hodgkin lymphoma (NHL) (endoscopic resection plus chemoradiotherapy), and cutaneous and visceral Kaposi's sarcoma (KS) (chemotherapy). All patients have reduced the dose of immunosuppressive drugs; in 1 of them, tacrolimus was changed to rapamycin. Two patients died because of neoplastic dissemination, another 1 due to obliterans bronchiolitis. The 2 patients with NHL and KS are alive at 6 and 9 months, respectively, without signs of recurrence. Malignancies after lung transplantation represent an important problem. A multidisciplinary approach is mandatory to obtain satisfactory results in terms of improved quality of life and long-term survival.

Invasive fungal infections are a significant cause of morbidity and mortality for patients undergoing solid organ transplantation. Our aim was to evaluate the incidence of invasive fungal infections in solid organ recipients within a dedicated intensive care unit (ICU).Materials and Methods. From May 2002 to May 2005, 278 patients undergoing solid organ transplantation (105 liver, 142 kidney, 20 lung, 2 combined liver-kidney, 9 combined pancreas-kidney) were admitted to our posttransplant intensive care unit. We retrospectively analyzed data obtained from the ICU stay. Fungal infection was defined by positivity of normally sterile biological samples and by elevated positivity of normally non sterile biological samples. We did not consider superficial fungal infections and asymptornatic colonizations.Results. Forty-six patients (16.5%) developed a fungal infection; at least one mycotic agent was isolated from each patient. Candida albicans was the most common pathogen, isolated from 71% of infected patients (33 of 46). Infected patients showed a mortality rate of 35%, while that for non infected recipients was 3.5%. Total length of ICU stay was the most significant risk factor among infected patients (30.26 days vs 5.04 days P <.0001). Mean time between transplantation and first positive samples was 6.17 days (SD 8.88).Conclusion. Fungal infections in solid organ transplant patients are a major issue because of their associated morbidity and mortality. Candida albicans was the most common pathogen and total lenght of ICU stay was the most important risk factor.

The incidence of multilobular cirrhosis (MC) with portal hypertension in CF is 5-15%. Hepatosplenomegaly is the first clinical sign of Cystic Fibrosis Liver Disease (CFLD). Complications due to portal hypertension (ascites, hypersplenism, oesophageal varices) dominate the clinical picture and OLT is the only viable therapy. From 1996 to 2001 six CF patients (5 males; mean age 18,5 years) underwent OLT. Mean follow-up after OLT is 4,6 years (range 1-6 years). All patients (pts) excpet one had an improvement of lung function with a reduction of infections.

Meningitis is a rare complication in CF. We illustrate a case report of a CF newborn patient with an episode of FM meningitis after surgery for meconium ileus.