Clinical and paraclinical charateristics of pulmonary agenesis and hypoplasia in children treated at national pediatric hospital

Objectives: To provide better understanding of pulmonary agenesis and hypoplasia. Subjects and methods: A retrospective and prospective descriptive study on all patients with pulmonary agenesis and hypoplasia admitted to Respiratory Department, National Pediatric Hospital from January 2011 to December 2016. | Journal of military pharmaco-medicine no4-2018 CLINICAL AND PARACLINICAL CHARATERISTICS OF PULMONARY AGENESIS AND HYPOPLASIA IN CHILDREN TREATED AT NATIONAL PEDIATRIC HOSPITAL Nguyen Thi Mai Hoan*; Dao Minh Tuan*; Mai Xuan Khan** SUMMARY Objectives: To provide better understanding of pulmonary agenesis and hypoplasia. Subjects and methods: A retrospective and prospective descriptive study on all patients with pulmonary agenesis and hypoplasia admitted to Respiratory Department, National Pediatric Hospital from January 2011 to December 2016. Results and conclusion: In the 6-year-period study, there were a total of 21 patients with pulmonary agenesis and hypoplasia () admitted to Respiratory Department. 14 of them had pulmonary agenesis and 6 had pulmonary hypoplasia. 80% of patients were younger than 6 months old, the earliest diagnosis was during the fetal period whereas the latest was a 12-year-old patient and high female-to-male ratio. The most common signs and symptoms were coughing, wheezing, dyspnea, decreased vesicular breath sound and recurrent pneumonia. Radiologic imaging: Hemithorax white-out, unilateral pulmonary artery agenesis or pulmonary artery atresia. * Keywords: Pulmonary agenesis; Pulmonary hypoplasia; Children. INTRODUCTION Pulmonary agenesis and hypoplasia are characterised by the incomplete development of airways and lung parenchyma. The severity of this disorder depends on the stages of lung development during the fetal period and the presence of other associated anatomical abnormalites. Pulmonary agenesis and hypoplasia occured in the embryonic stage (about 4 weeks of gestational age) when the 'primitive' lung is formed. The reason is unknown but genetic factors, environment and chemicals might be the causes [6]. The most typical characteristics consist of the incomplete development of the lung parenchyma, which still has bronchial stump or carina as in aplasia and is usually diagnosed at 23 to 30 weeks of gestational age. The .

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