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The adenosine triphosphate (ATP)-binding cassette subfamily A member 3 (ABCA3) is a transmembrane glycoprotein that uses energy of ATP hydrolysis to transport phospholipids into the lamellar bodies of type 2 alveolar epithelial cells (AEC) and regulates lung surfactant homeostasis. More than 200 mutations have already been described in ABCA3, located on chromosome 16 [1, 2]. Patients present with a great heterogeneity of phenotypes, from lethal neonatal respiratory distress syndrome (RDS) to childhood and rarely adult interstitial lung disease (ILD) [3, 4]. ABCA3 mutations-related lung disease inheritance is autosomal recessive, as it requires two disease-causing (bi-allelic) mutations, one from each parent., Children with ABCA3 mutations may survive beyond infancy and reach adulthood. Genetic mechanisms should always be examined in adult patients with childhood onset ILD and molecular analysis should be performed accordingly in specialised referral centres. http://bit.ly/2LzMNOE

Objectives: We present a modified wedge resection of the bronchus, as an alternative bronchoplastic technique for lung resection, in cases of patients with or without adequate pulmonary reserve to undergo a pneumonectomy, in order to preserve lung tissue. Methods: Seventeen patients underwent a major lung resection with wedge resection of the bronchus for non-small cell lung cancer (NSCLC) in our department, from March 1995 to October 1999. A right-sided NSCLC were diagnosed in 17 males, with a mean age 62.5 ^ 6.6 (range 51‐72) years. Further workup was free of metastatic disease. All patients underwent a right posterolateral thoracotomy, under general anesthesia with a double lumen endotracheal tube. Twelve right upper lobectomies, four right upper and middle lobectomies and one carinal resection were performed. The wedge resection of the bronchus carried out longitudinally, along the bronchial tree, and the bronchial defect was reapproximated transversely, in a single-layer, with interrupted non-absorbable suture. The frozen section of the distal margin of the resected bronchus was negative for malignancy in all patients. Extended mediastinal lymph node dissection followed each lung resection. Results: The pathology report showed 12 squamous-cell carcinomas, three adenocarcinomas, one adenosquamous carcinoma and one neuroendocrine carcinoma. The differentiation of the carcinomas was well in two cases, moderate in ten and poor in five. The pTNM stage was IB in four patients (23.5%), IIA in one (5.9%), IIB in eight (47.1%) and IIIA in four (23.5%). The median disease-free distal margin of the bronchus was 5 mm (range 2‐15 mm). The average postoperative hospital stay was 15 days (range 12‐28 days). The morbidity and mortality rate was 11.8 and 5.9%, respectively. Postoperative follow-up was every 6 months. The average survival is 20.0 ^ 15.2 months (range 1‐54 months). There are 12 patients alive, and their follow-up is negative for locoregional recurrence or distant metastasis. The survival study showed no significantly statistic relation to the histologic type, cancer differentiation, pTNM stage, and disease-free distal margin of resection larger or less than 0.5 cm (Kaplan‐Meier study log rank method). Conclusions: The wedge resection of the bronchus as a bronchoplastic procedure is an easy, fast and safe technique of reparation of the bronchial tree. It presents not only a low rate of morbidity and mortality, but also a satisfactory survival. q 2001 Elsevier Science B.V. All rights reserved.

Objective: In the staging of lung cancer, pleural effusion that is malignant on cytologic examination is regarded as T4 disease, and curative resection cannot be performed. We conducted this study to determine whether cancer cells can be present in the pleural cavity with no pleural effusion, to investigate the factors contributing to that occurrence, and to evaluate its prognostic significance. Methods: Eighty-five patients (77 males, eight females) with a median age 60:1 ^ 7:9 years (31‐74 years) underwent a major lung resection, due to lung cancer in our department. From January 1998 to December 1999, 30 pneumonectomies, seven bilobectomies, 46 lobectomies and two wedge-resections were performed. Chest wall resection was performed in four patients. After performing a posterolateral thoracotomy and lung resection with extended mediastinal lymph node dissection, the pleural cavity was filled with 1 l physiologic saline solution (PSS) and the fluid was shaken. The lavage fluid was suctioned off (S1). Immediately after the lavage, the pleural cavity was refilled with 3 l PSS. The surgeon washed out the pleural cavity by hand for 1 min and the fluid was suctioned off. Finally, the pleural cavity was refilled with 1 l PSS and a new lavage fluid was suctioned off (S2). A cytologic examination was carried out for each sample. Results: The pathology report showed 39 adenocarcinomas, 33 squamous-cell, two adenosquamous, four large-cell, two neuroendocrine and five undifferentiated carcinomas. S1 was positive in eight patients (9.4%), while S2 was positive in four patients (4.7%). The correlation of positive pleural lavage and infiltrated lymph nodes demonstrated a statistically significant relation between presence of N2 disease and positive S2 sample (Pa 0:049). No significant correlation existed between positive lavage sample (S1 or S2) and TNM stage, level of T, extent of tumor invasion, kind of operation, histological type or differentiation of the cancer (Chi square test). The mean follow-up is 11:3 ^ 6:2 months (4‐22 months). There are 78 patients alive. A significance difference in survival was identified in-patients with positive S1 (Pa 0:0081), and positive S2 (Pa 0:0251) (Kaplan‐Meier). Conclusion: The cytologic results of lavage were positive for malignant cells in eight of 85 patients (9.4%). The existence of cancer cells in the pleural cavity can be the result of their exfoliation or surgical manipulations. The mechanical irrigation subdivides the percentage of positive samples. Our study supports that the positive findings on pleural lavage cytology is an essential prognostic factor. q 2001 Elsevier Science B.V. All rights reserved.