Acute hypoxic respiratory failure as the first manifestation of systemic-onset juvenile rheumatoid arthritis in a child.

Systemic onset juvenile rheumatoid arthritis is the most common rheumatologic disorder of childhood. Pleuropulmonary manifestations are rare in children in this multiorgan disease, and are usually not severe. The diagnosis of systemic onset juvenile rheumatoid arthritis is made by exclusion, in the presence of clinical findings constellation. We present the case of an 8-year-old girl who developed acute hypoxic respiratory failure as the first manifestation of systemic onset juvenile rheumatoid arthritis, then severe respiratory relapse 16 months later. Clinical and radiological improvement were achieved at both times after high dose pulse methylprednisolone therapy.

Chest CT in bronchopulmonary dysplasia: clinical and radiological correlations.

BACKGROUND: Chest CT is very sensitive in assessing pulmonary damage in bronchopulmonary dysplasia (BPD) and radiological findings in BPD are well described. Validated CT scores are available to assess BPD, as available in other pulmonary diseases such as cystic fibrosis. AIM: To investigate whether there is a correlation between radiological pulmonary lesions and relevant BPD clinical data (gestational age, type and duration of mechanical ventilation, and severity of BPD) and assess the usefulness of a CT score in evaluating clinical severity. MATERIALS AND METHODS: Retrospective study of 19 premature infants with BPD born between 1998 and 2007 who underwent at least one chest CT during their first year of life. A total of 29 CT were blindly evaluated by two radiologists for the presence or absence of pulmonary parenchymal abnormalities described in BPD (areas of decreased attenuation, presence of bullae/emphysema, bronchial wall thickening, bronchiectasis, linear, and subpleural opacities). This score was then compared with the most relevant clinical data. RESULTS: All CT scans showed abnormalities. The most frequent lesion was bronchial wall thickening observed in all patients, followed by linear (89.5%) and subpleural (89.5%) opacities. Areas of decreased attenuation were found in 68.4%. Bullae/emphysema and bronchiectasis were the less frequent item described (26.3% and 21.1%, respectively). The presence of areas of decreased attenuation significantly correlated with BPD severity (P = 0.03). However, there was no significant correlation between the CT score and clinical data. CONCLUSIONS: This study demonstrates the potential usefulness of chest CT score to assess the severity of BPD. Areas of decreased attenuation seem the most sensitive item to predict BPD severity. More patients are needed to validate this approach and to evaluate the long-term usefulness of CT scan.

Generalised infantile myofibromatosis with intracranial involvement: imaging findings in a newborn.

Infantile myofibromatosis is a disorder of infancy and early childhood characterised by single or multiple nodular lesions localised to bone, muscle, viscera, subcutaneous tissue or central nervous system. These pseudotumours contain smooth muscle and fibroblasts. The prognosis depends on the amount, as well as location, of the lesions. We present the radiological findings on plain films, US and MRI of a newborn who presented at birth with multiple organ involvement, including the CNS. To our knowledge, the comprehensive imaging features of such severe and multifocal involvement have not previously been reported.