[Novel therapies for idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown cause. The clinical course is unpredictable, but the disease is usually progressive with a median survival of 2-5 years as a result of advanced respiratory failure. The current hypothesis of the disease mechanism is recurrent injury to the respiratory epithelium which leads to an uncontrolled wound healing process resulting in fibrosis rather than repair. Despite better understanding of the pathogenesis, there is no effective therapy for the disease. In this review, we present the positive results of recently published clinical trials regarding therapy for IPF, with emphasis on pirfenidone and nintedanib.

[Pathology of organ transplantation: experience of the Rabin Medical Center].

Solid organ transplantation is currently the treatment of choice for renal, heart, and pancreas insufficiency and selected bowel diseases. Thanks to advances in medical technology, the lifespan of transplanted organs is currently about 10 years. To prevent graft rejection, patients need to take immunosuppressive drugs, usually for the rest of their Lives. Pathologists play a crucial role in organ transplantation. They are responsible for recognizing allograft rejection, both acute and chronic, differentiating rejection from drug toxicity, and identifying recurrent disease. In addition, pathologists identify new diseases in the graft, opportunistic infections in the transplanted organ or other organs, and the development of malignant tumors, which are more common in immunocompromised patients. Accordingly, transplant pathologists require a wide range of knowledge in many complex laboratory techniques, such as immunofluorescence, electron microscopy, immunohistochemical analysis, and molecular pathology. These tests are performed in dedicated Laboratories in departments of pathology. TranspLant pathology is an inseparable part of the field of transplantation medicine and greatly assists clinicians in the diagnosis of disease processes in transplanted organs and in the selection of appropriate treatment.

[Tracheobronchial adenoid cystic carcinoma: seven case reports].

BACKGROUND: Adenoid cystic carcinoma of the respiratory tract is a rare form of malignant neoplasm that arises from salivary glands. A surgical resection with or without radiotherapy is commonly recommended. For inoperable patients, radiotherapy and endobronchial treatment, which include laser and stent insertion, are important therapeutic modalities. AIM: To summarize our experience in adenocystic carcinoma of the respiratory tract. METHODS: Seven patients with adenoid cystic carcinoma were diagnosed and treated in the Pulmonary Institute at Rabin Medical Center, a tertiary care hospital in central Israel in 2000-2006. RESULTS: Three patients were men and four were women, with a presenting age at diagnosis ranging from 31 to 79 years. All the patients were nonsmokers. Major symptoms at presentation included dry cough, dyspnea and hemoptysis. Only two patients were operable at time of presentation and 4 patients had local and distant metastases. In 6 patients the tumor was presented in the trachea with marked obstruction of the tracheal lumen. In 4 patients coexisting lesions in the main bronchus were also found. Two patients with non-metastatic disease underwent surgical resection with external radiation. Multiple laser therapy and brachytherapy were performed in 4 patients. Two patients with severe airway stenosis that was resistant to radiotherapy were treated with laser and intratracheal stent replacement. CONCLUSIONS: Endobronchial therapy that includes brachytherapy, laser and stent insertion, is a useful therapeutic modality for the treatment of patients with adenoid cystic carcinoma of the respiratory tract who are resistant to radiotherapy.

[Ocular manifestation of bronchogenic carcinoma: simultaneous occurrence of diffuse uveal melanocytic proliferation and uveal metastases].

Systemic cancer may affect the eye and orbit as metastatic disease or paraneoplastic retinal degeneration. This is a case report of a 55-year-old man with unilateral visual loss as the presenting symptom of metastatic adenocarcinoma of lung in one eye and diffuse uveal melanocytic proliferation in the other. Fundoscopy, B-scan ultrasonography, fluorescein angiography and the workup for inflammatory, infectious and neoplastic diagnoses are presented. In heavy smokers, bronchogenic carcinoma should be highly suspected.

Improved survival following lung transplantation with long-term use of bilevel positive pressure ventilation in cystic fibrosis.

Bilevel positive airway pressure ventilation (BIPAP) has been used in cystic fibrosis (CF) patients as a bridge to transplantation. Our aim was to evaluate the effect of BIPAP use before transplantation on post-transplantation morbidity and mortality. We performed a retrospective study at a tertiary care center. Twelve CF patients (9 males; mean age = 26 years) were assessed. Group 1 consisted of eight patients that did not use BIPAP before lung transplantation. Group 2 comprised four patients who used BIPAP for 3-15 months while awaiting transplantation. Patients were evaluated before and two to ten years after transplantation. All eight patients who did not use BIPAP died two months to ten years after transplantation. All four BIPAP users are alive with no evidence of bronchiolitis obliterans two to eight years after lung transplantation. We demonstrated a significant improvement in acid-base balance (p < 0.01) and body mass index (p < 0.05) and a tendency toward improvement in the work of breathing and number of hospitalizations. We conclude that improvement in nutritional status and respiratory muscle strength before lung transplantation in BIPAP users may prevent post lung transplantation infection and acute rejection rate, which in turn may reduce chronic rejection (bronchiolitis obliterans) and improve long-term survival after lung transplantation.