Abstract

A 21-month-old female with a history of failure to thrive after the age of 6 months and recurrent, slowly resolving bronchopulmonary infections involving the left lung and leading to respiratory distress requiring oxygen administration, was referred to the Giannina Gaslini Institute (Genova, Italy) for further evaluation. On admission, in December 2004, the patient was in mediocre general conditions. Her height and weight were 56 cm and 10.0 kg (both 25th percentile), respectively. Her respiratory rate was 33 breaths·min−1. Physical examination demonstrated decreased percussion and auscultatory sounds over the left hemithorax. Screening on immunological disorders revealed that B- and T-cell functions were normal. Sweat test and cystic fibrosis transmembrane regulator gene mutations were negative. Results of the blood and microbiological tests performed on admission are shown in table 1⇓. View this table: Table 1— Results of blood and microbiological tests performed on admission A chest radiograph was then carried out as shown in figure 1⇓. Spiral computed tomography (CT) was then performed (fig. 2⇓), followed by echocardiography (fig. 3⇓). The patient underwent cardiac catheterisation and angiography (fig. 4⇓). Fig. 1— Chest radiograph on presentation. Fig. 2— Spiral computed tomography images of the chest at different levels. a, b and c) lung windows. d, e and f) soft tissue windows. Fig. 3— Echocardiograph obtained on admission. Fig. 4— Angiography obtained during cardiac catheterisation. a) Pulmonary circulation. b) Systemic circulation. BEFORE TURNING THE PAGE INTERPRET THE RADIOGRAPH, COMPUTED TOMOGRAPHY SCANS, ECHOCARDIOGRAPHY AND ANGIOGRAPHY, SUGGEST A DIAGNOSIS AND A POSSIBLE CAUSE ### Chest radiography The chest radiograph showed asymmetry of the two lung fields, with widening of the left area of the mediastinum and a leftward mediastinal shift and possible hypoplasia of the left lung (fig. 1⇑). ### Spiral computed tomography Besides defining the …

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