Document Detail


Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center.
MedLine Citation:
PMID:  12687596     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Congenital lobar emphysema (CLE) is characterized by overdistension and air-trapping in the affected lobe, and is one of the causes of infantile respiratory distress. In this report, we review our 27 years of experience with 30 CLE patients. Patients' medical records were evaluated with regard to age, clinical presentation, diagnostic methods, associated diseases, treatment, histopathologic findings, and final clinical and laboratory findings at the end of a long-term period. The mean age of 30 patients (18 male) at diagnosis was 4.9 +/- 6.7 months (range, 2 days-2.5 years). Tachypnea, dyspnea, cough, cyanosis, wheezing, hoarseness, and decreased breath sounds on the affected side were the main symptoms and clinical findings. On chest X-rays, emphysema was seen in all patients; shift/herniation to the opposite lung, atelectasis, and pneumothorax were observed in 16, 5, and 2 cases, respectively. Computerized tomography of the thorax was performed in 16 cases and revealed emphysema at affected lobe/lobes in all, a shift/herniation to the opposite side in 12 cases, and atelectasis of neighbor lobe/lobes in 7 cases. All 8 patients who had perfusion scintigraphy showed reduced perfusion in the affected lobe. Narrowed and flaccid bronchi were detected in one patient by using flexible bronchoscopy. Blood gas analysis was performed in 11 patients, and hypoxia and hypercarbia were revealed in 9 and 7 of these patients, respectively. The most common affected lobe was the left upper lobe (57%), followed by the right upper lobe (30%) and right middle lobe (27%). Two lobes were involved in 4 patients. Associated abnormalities were observed in 5 patients. Twenty-one patients underwent lobectomy; 9 were followed conservatively. Ages at diagnosis were significantly younger in surgically treated patients. Emphysema was detected in all pathological specimens, with an additional bronchial cartilage deficiency in 2 patients. In the surgically treated group, 2 patients died and 2 patients were lost to follow-up. In the conservatively treated group, one patient was lost to follow-up. Mean follow-up duration of all patients was 63.2 +/- 56.2 months (range, 1-209 months). At follow-up visits, all patients were doing well. In surgically treated patients, chest X-rays were normal (9 cases), or showed hyperlucency on the operated side (6 cases) or chronic changes in the operation area (2 cases). Hyperexpansion in the affected lobe was found to be reduced in all cases in the conservatively treated group.
Authors:
Uğur Ozçelik; Ayhan Göçmen; Nural Kiper; Deniz Doğru; Embiya Dilber; Ebru Güneş Yalçin
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Pediatric pulmonology     Volume:  35     ISSN:  8755-6863     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:  2003 May 
Date Detail:
Created Date:  2003-04-10     Completed Date:  2003-08-22     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  384-91     Citation Subset:  IM    
Copyright Information:
Copyright 2003 Wiley-Liss, Inc.
Affiliation:
School of Medicine, Hacettepe University, Ihsan Doğramaci Children's Hospital, Ankara, Turkey. uozcelik@hacettepe.edu.tr
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Child
Child, Preschool
Female
Follow-Up Studies
Hospitals, Pediatric / statistics & numerical data*
Humans
Infant
Infant, Newborn
Male
Outcome Assessment (Health Care) / statistics & numerical data*
Pulmonary Emphysema / congenital*,  diagnosis,  therapy*
Retrospective Studies
Time Factors
Turkey

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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