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      Congenital lobar emphysema: diagnosis and treatment options

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          Although congenital lobar emphysema is a rare lung disease, it can cause severe respiratory distress in the newborn. Lobectomy can be difficult because of the hyperinflated lobe and limited space to carry out surgery. During the past two decades, conservative treatment options have increased for patients with mild and moderate disease.

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          Most cited references 88

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          Genetic control of branching morphogenesis.

          The genetic programs that direct formation of the treelike branching structures of two animal organs have begun to be elucidated. In both the developing Drosophila tracheal (respiratory) system and mammalian lung, a fibroblast growth factor (FGF) signaling pathway is reiteratively used to pattern successive rounds of branching. The initial pattern of signaling appears to be established by early, more global embryonic patterning systems. The FGF pathway is then modified at each stage of branching by genetic feedback controls and other signals to give distinct branching outcomes. The reiterative use of a signaling pathway by both insects and mammals suggests a general scheme for patterning branching morphogenesis.
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            Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation.

            Congenital lung abnormalities are being detected more frequently at routine high-resolution prenatal ultrasonography. The most commonly encountered anomalies include lung agenesis-hypoplasia complex (pulmonary underdevelopment), congenital pulmonary airway malformations, congenital lobar overinflation, bronchial atresia, bronchogenic cysts, congenital high airway obstruction syndrome, scimitar syndrome, and bronchopulmonary sequestration. Recognizing the antenatal and postnatal imaging features of these abnormalities is necessary for optimal prenatal counseling and appropriate peri- and postnatal management. Supplemental material available at © RSNA, 2010.
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              Congenital malformations of the lung.

              Congenital malformations of the lung are a frequent cause of abnormal chest radiographs in neonates and young children as well as a common cause for recurrent pneumonia in older patients. Based on the mode of presentation and clinical findings along with an understanding of lung embryology, one can develop a thorough differential diagnosis that allows for the evaluation of the lesion in question. Appropriate management of the patient can then be undertaken with a minimum of diagnostic studies and without any delay in treatment.

                Author and article information

                Int J Chron Obstruct Pulmon Dis
                Int J Chron Obstruct Pulmon Dis
                International Journal of Chronic Obstructive Pulmonary Disease
                01 May 2019
                : 14
                : 921-928
                [1 ]Department of Thoracic Surgery, Erciyes University , Kayseri, Turkey
                [2 ]Department of Pediatrics, Division of Pediatric Pulmonology, Erciyes University , Kayseri, Turkey
                Author notes
                Correspondence: Mehmet KoseSchool of Medicine, Department of Pediatrics, Division of Pediatric Pulmonology, Erciyes University , Kayseri38039, TurkeyTel +9 035 220 766 662 5468Email mhmtkose@
                © 2019 Demir et al.

                This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (

                Page count
                Figures: 3, Tables: 2, References: 92, Pages: 8

                Respiratory medicine

                lobectomy, overinflation, congenital lobar emphysema


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