BACKGROUND: Pulmonary alveolar proteinosis (PAP) is characterized by an abnormal accumulation of periodic acid-schiff-positive lipoproteinaceous material in the alveoli. Early diagnosis allows setting up of therapeutic lung lavages, which reduces the need for oxygen supplementation and weight gain. OBJECTIVE: To provide a description of radiological features by CT at the onset of primary PAP in children. MATERIALS AND METHODS: The clinical and radiological data of 24 patients, including 16 boys and 8 girls (median age: 12 months), diagnosed with a primary form of PAP between April 1992 and May 2012 in a tertiary referral hospital, were retrospectively reviewed. CT images were examined for the presence of alveolar and interstitial elementary lesions. Correlation between clinical and radiological findings was assessed. RESULTS: The types of elementary lesions detected were: ground-glass opacities (n = 24), intralobular lines (n = 24), thickened interlobular septa (n = 22), thickened fissures (n = 21), airspace consolidation (n = 16), hyperinflation (n = 16), cystic lesions (n = 2) and micronodules (n = 1). A crazy-paving pattern was found in 92% of cases. Consolidation and hyperinflation were especially detected in younger children (median age, 8 months, P < 0.01). A density dependent gradient was found. The distribution of the lesions was symmetrical. There was no correlation between radiological and clinical data of severity of the disease. CONCLUSION: CT findings are suggestive of diagnosis of PAP in immunocompetent children with chronic respiratory failure.
BACKGROUND:Pulmonary alveolar proteinosis (PAP) is characterized by an abnormal accumulation of periodic acid-schiff-positive lipoproteinaceous material in the alveoli. Early diagnosis allows setting up of therapeutic lung lavages, which reduces the need for oxygen supplementation and weight gain. OBJECTIVE: To provide a description of radiological features by CT at the onset of primary PAP in children. MATERIALS AND METHODS: The clinical and radiological data of 24 patients, including 16 boys and 8 girls (median age: 12 months), diagnosed with a primary form of PAP between April 1992 and May 2012 in a tertiary referral hospital, were retrospectively reviewed. CT images were examined for the presence of alveolar and interstitial elementary lesions. Correlation between clinical and radiological findings was assessed. RESULTS: The types of elementary lesions detected were: ground-glass opacities (n = 24), intralobular lines (n = 24), thickened interlobular septa (n = 22), thickened fissures (n = 21), airspace consolidation (n = 16), hyperinflation (n = 16), cystic lesions (n = 2) and micronodules (n = 1). A crazy-paving pattern was found in 92% of cases. Consolidation and hyperinflation were especially detected in younger children (median age, 8 months, P < 0.01). A density dependent gradient was found. The distribution of the lesions was symmetrical. There was no correlation between radiological and clinical data of severity of the disease. CONCLUSION: CT findings are suggestive of diagnosis of PAP in immunocompetent children with chronic respiratory failure.
Authors: David M Hansell; Alexander A Bankier; Heber MacMahon; Theresa C McLoud; Nestor L Müller; Jacques Remy Journal: Radiology Date: 2008-01-14 Impact factor: 11.105
Authors: M Mechri; R Epaud; S Emond; A Coulomb; F Jaubert; A Tarrant; D Feldmann; F Flamein; A Clement; J de Blic; R Abou Taam; F Brunelle; H Ducou le Pointe Journal: Pediatr Pulmonol Date: 2010-10
Authors: Seung Young Jin; Hye Ri Yun; Yun Jung Choi; Jun Dong Park; Jin Tae Kim; Chang Hyun Kang; Young Sik Park; Young Hun Choi; Woo Sun Kim; Dong In Suh Journal: Korean J Pediatr Date: 2017-07-31