A Case Report of a Girl with Progressive Pulmonary Emphysema who Died at the Age of 16 years

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  • １６歳で死亡に至った進行性肺気腫女児例―診断と治療上の問題点―
  • 診断と治療上の問題点

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Abstract

Pulmonary emphysema and bronchiolitis obliterans (BO) are rare during childhood, and there are various etiologies and pathological findings.A 9-year-old girl who was brought to our department with a chief complaint of exertional dyspnea was clinically diagnosed with BO based on clinical course, chest CT and pulmonary perfusion scintigraphy.In addition to medication, oxygen and physical therapies, multidisciplinary care was provided by collaboration of different departments to improve her quality of life.However, repeated exacerbations progressively worsened her pulmonary function, and at the age of 14 years, she was placed on the waiting list for brain-dead donor lung transplantation.At the age of 16 years, her condition rapidly deteriorated due to pneumonia caused. by Candida and Pseudomonas, and despite intensive care, she died.Autopsy showed marked pulmonary emphysema, but no typical histopathological findings associated with BO at the bronchiolar level, and she was accordingly diagnosed with pulmonary emphysema.<BR>To the reason we raise the timing of pathological study, the site of tissue sampling and the effects of steroid therapy.We also consider the peripheral airway lesion caused by meconium aspiration syndrome of newborn.<BR>In the future, it will be necessary to clarify the pathology of childhood-onset progressive bilateral pulmonary emphysema.

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