Lung transplantation for cystic fibrosis: ten years of experience.

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.

Malignancies following lung transplantation.

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.

Evolving techniques and perspectives in lung transplantation.

Lung transplantation is currently a suitable option for patients with end-stage lung disease. Since the early 1980s the surgical technique and immunosuppressive protocols have been progressively modified to improve results and favor long-term survival. The original heart-lung transplantation under cardiopulmonary bypass is now rarely performed and single or bilateral lung transplantation is the procedure of choice. Bilateral transplantation is performed with two single lung transplants performed in sequence. Extracorporeal support is rarely employed and in most cases it is instituted through the femoral approach. Also, the surgical approach has been modified and the original clam shell incision has been replaced by two small anterior thoracotomies. The use of marginal donors has been increasingly proposed to enlarge the number of organs potentially available for transplantation. Immunosuppressive protocols have evolved to patient-specific regimens that can be quickly modified if required by the clinical status. Induction is now more aggressive and also rescue protocols for obliterative bronchiolitis can contribute to improved outcomes. Overall, lung transplantation is now performed with encouraging long-term results.

Lung transplantation for cystic fibrosis.

BACKGROUND: Lung transplantation is a robust therapeutic option to treat patients with cystic fibrosis. PATIENTS AND METHODS: Since 1996, 109 patients with cystic fibrosis were accepted onto our waiting list with 58 bilateral sequential lung transplants performed in 56 patients and two patients retransplanted for obliterative bronchiolitis syndrome. RESULTS: Preoperative mean FEV(1) was 0.64 L/s, mean PaO(2) with supplemental oxygen was 56 mm Hg, and the mean 6-minute walking test was 320 m. Transplantation was performed through a "clam shell incision" in the first 29 patients and via bilateral anterolateral thoracotomies without sternal division in the remaining patients. Cardiopulmonary bypass was required in 14 patients. In 21 patients the donor lungs had to be trimmed by wedge resections with mechanical staplers and bovine pericardium buttressing to fit the recipient chest size. Eleven patients were extubated in the operating room immediately after the procedure. Hospital mortality of 13.8% was related to infection (n = 5), primary graft failure (n = 2), and myocardial infarction (n = 1). Acute rejection episodes occurred 1.6 times per patient/year; lower respiratory tract infections occurred 1.4 times per patient in the first year after transplantation. The mean FEV(1) increased to 82% at 1 year after operation. The 5-year survival rate was 61%. A cyclosporine-based immunosuppressive regimen was initially employed in all patients; 24 were subsequently switched to tacrolimus because of central nervous system toxicity, cyclosporine-related myopathy, or renal failure, obliterative bronchiolitis syndrome, gingival hyperplasia, or hypertrichosis. Ten patients were subsequently switched to sirolimus. Freedom from bronchiolitis obliterans at 5 years was 60%. CONCLUSIONS: Our results confirm that bilateral sequential lung transplantation is a robust therapeutic option for patients with cystic fibrosis.

Endoscopic treatment of lung cancer invading the airway before induction chemotherapy and surgical resection.

OBJECTIVE: Many patients with advanced lung cancer invading the airway require only palliation; however, induction chemotherapy and surgery may sometimes be considered. Preliminary endoscopic palliation may improve quality of life and functional status, allows better evaluation of tumor extension and contributes to prevent infectious complications. We reviewed our experience with preliminary laser treatment, induction chemotherapy and surgical resection in patients with lung cancer invading the airway. METHODS: Twenty-one patients with stage IIIA and IIIB lung cancer presenting with an 80% unilateral airway obstruction were treated with laser resection, induction chemotherapy and surgery. Spirometry, arterial blood gas analysis, quality of life (QLQ-C30 score) and performance status were recorded before and after laser treatment and after chemotherapy. Complications during chemotherapy, surgical morbidity and mortality, and survival were also recorded. RESULTS: No complications were observed after endoscopic treatment. FEV(1) significantly improved from 1.4+/-0.4 l/s to 2.2+/-0.7 l/s, as well as FVC (from 2+/-0.5 to 3.1+/-0.8 l), and remained stable after chemotherapy. The QLQ-C30 score significantly improved after laser treatment (from 45+/-4.8 to 31+/-2.5) as well as the Karnofsky status (from 76+/-5 to 90). One patient developed pneumonia during induction chemotherapy. Three patients were not operated on. We performed five pneumonectomies (one right tracheal sleeve pneumonectomy) and 13 lobectomies (five associated to a bronchial sleeve resection). One patient (5.5%) died after the operation. Four patients experienced minor postoperative complications. Three-year survival after the operation was 52%. CONCLUSIONS: Preliminary endoscopic palliation of lung cancer invading the airway is feasible, improves evaluation and staging, helps to reduce the incidence of complications during induction chemotherapy without increasing surgical morbidity and mortality.

[Diagnostic delay in post-traumatic diaphragmatic ruptures]. / Ritardo diagnostico delle rotture post-traumatiche del diaframma.

Traumatic ruptures of the diaphragm are associated with closed chest and abdominal traumas, often the outcome of road accidents, with an incidence varying from 1 to 5% depending on the series. Diagnosis is frequently late if the possibility of breakages in two stages is also considered. This delay in diagnosis is encountered in a variable percentage of cases, between 9.5 and 60%. The clinical case of a post-traumatic ruptures of the diaphragm diagnosed 26 years after a car accident is described.