Congenital lobar emphysema

A hereditary autosomal dominant form of emphysema that presents in infancy and is characterized by hyperinflation of the affected lobe; compression of surrounding normal lung tissue, and hypoplasia of the bronchial tissue. OMIM: 130710

Also Known As:

Networked: 35 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Congenital Cystic Adenomatoid Malformation of Lung (Cystic Adenomatoid Malformation of Lung)
2.	Pneumothorax
3.	Congenital Heart Defects (Congenital Heart Defect)
4.	Hamartoma
5.	Pulmonary Echinococcosis

Experts

1.	Cinel, Güzin: 1 article (04/2022)
2.	Gençoğlu, Murat Yasin: 1 article (04/2022)
3.	Ocak, Ece: 1 article (04/2022)
4.	Polat, Sanem Eryılmaz: 1 article (04/2022)
5.	Soydaş, Sule Selin Akyan: 1 article (04/2022)
6.	Tabakçı, Satı Özkan: 1 article (04/2022)
7.	Tuğcu, Gökçen Dilşa: 1 article (04/2022)
8.	Uytun, Salih: 1 article (04/2022)
9.	Rajagopalan, Muthukumar: 1 article (10/2021)
10.	Bhende, Vishal V: 1 article (09/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital lobar emphysema:

1.	Anesthetics (Anesthetic Agents)IBA 08/01/2016 - "Congenital Lobar Emphysema: An Approach of Anesthetic Management." 07/01/2015 - "The purpose of this retrospective study was to present the anesthetic management of children with congenital lobar emphysema (CLE) receiving treatment in our center. " 07/01/2015 - "Anesthetic Management in Children with Congenital Lobar Emphysema." 07/01/2015 - "Anesthetic management of congenital lobar emphysema in a neonate." 01/01/2015 - "Congenital lobar emphysema: A modified approach to anesthetic management."
2.	4- Acetamido- 4'- isothiocyanatostilbene- 2,2'- disulfonic Acid (SITS)IBA 05/01/2015 - "Four patients who had bronchiectasis [left lower lobe (LLL)], congenital lobar emphysema [left upper lobe (LUL)], aspergilloma [right upper lobe (RUL)], and hamartoma (RUL) were planned to undergo 2.5 cm SITS lobectomy. "
3.	Radioisotopes (Radionuclides)IBA 01/01/1989 - "An infant with congenital lobar emphysema of the left upper lobe presented with unusually severe mediastinal shift and underwent computed tomography (CT) and radionuclide V/Q scans. "
4.	ElastinIBA 01/01/2017 - "A diagnosis of congenital lobar emphysema should prompt further screening for congenital heart disease and genetic deletion, and further research is needed to investigate the role of elastin gene mutation in the development of the neonatal lung."
5.	antineoplaston A10 (A 10)IBA 06/23/1977 - "Congenital lobar emphysema is reported occurring in a 10 day old, low birth weight infant. "
6.	9 alpha,11 alpha,15 alpha- trihydroxy- 16- phenoxy- 17,18,19,20- tetranorprosta- 4,5,13- trienoic acid (TPT)IBA 05/01/2007 - "Congenital lobar emphysema (CLE) and tension pneumothorax (TPT) are rarely reported in dogs. "
7.	PS-6 (PS 6)IBA 04/01/2009 - "The distribution of diagnoses was as follows: bronchogenic cyst(s) (BC) - 5 patients; pulmonary sequestration (PS) - 6 patients; congenital lobar emphysema (CLE) - 4 patients; congenital cystic adenomatoid malformation (CCAM) Type I - 3 patients, and PS and CCAM coexistence - 1 patient. "

Therapies and Procedures

1.	Thoracotomy 10/01/2021 - "Emergency Thoracotomy for Congenital Lobar Emphysema - Anesthesiology Concerns." 05/01/2006 - "One symptomatic infant underwent thoracotomy for congenital lobar emphysema. " 01/01/2020 - "Congenital lobar emphysema: Thoracotomy versus minimally invasive surgery." 01/01/2023 - "The most common indication for thoracotomy was pulmonary hydatid cyst (17; 25%) followed by congenital lobar emphysema (11; 16.2%). "
2.	Conservative Treatment 04/01/2022 - "Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery." 07/01/2009 - "Conservative treatment of children with congenital lobar emphysema is an attractive option, whenever justifiable on medical grounds. " 07/01/2009 - "Considering that formerly asserted hypotheses postulating benefits of surgical treatment cannot unambiguously be corroborated from cases in literature, we could show that conservative treatment in patients with congenital lobar emphysema is appropriate in mildly to moderately symptomatic children. " 07/01/2009 - "For decades, the well-established standard recommended treatment for patients with congenital lobar emphysema (CLE) and respiratory distress has been lobectomy of the affected lobe or lobes, whereas indications for conservative management have been controversially discussed. " 01/01/1997 - "Although the possibility of conservative management in congenital lobar emphysema has been reported recently, we believe that surgery is the treatment of choice in patients who have persistent or progressive, severe respiratory distress in spite of medical treatment."
3.	Therapeutics 06/01/1975 - "[Differentiated surgical therapy in congenital lobar emphysema (author's transl)]." 01/01/1962 - "[On the clinical aspects and therapy of congenital lobar emphysema]."
4.	Interactive Ventilatory Support 01/01/2023 - "Neurally adjusted ventilatory assist for congenital lobar emphysema in an infant."
5.	Continuous Positive Airway Pressure 05/21/1977 - "Continuous positive airway pressure in congenital lobar emphysema."