Multimodality thoracic imaging of juvenile systemic sclerosis: emphasis on clinical correlation and high-resolution CT of pulmonary fibrosis.

OBJECTIVE. Juvenile systemic sclerosis is a rare multisystem autoimmune disorder characterized by vasculopathy and multiorgan fibrosis. Cardiopulmonary complications are the leading cause of morbidity and mortality. Although pulmonary fibrosis is the complication that is most common and well described, cardiovascular and esophageal involvement may also be observed. In this article, common thoracic findings in juvenile systemic sclerosis will be discussed. We will focus on chest CT, including CT findings of pulmonary fibrosis and associated grading methods, as well as cardiac MRI and esophageal imaging. CONCLUSION. Radiologists play a pivotal role in the initial diagnosis and follow-up evaluation of pediatric patients with systemic sclerosis. Treatment decisions and prognostic assessment are directly related to imaging findings along with clinical evaluation.

Vertical expandable prosthetic titanium rib (VEPTR): a review of indications, normal radiographic appearance and complications.

Vertical expandable prosthetic titanium rib (VEPTR) is increasingly used in the treatment of thoracic insufficiency, idiopathic and neuromuscular scoliosis and chest wall defects in children. In contrast to spinal fusion surgery, the VEPTR allows for growth while stabilizing the deformity. We illustrate the common indications and normal radiographic appearance of the three common configurations of VEPTR (cradle-to-cradle assembly, cradle with lumbar extension assembly, cradle-to-ala hook assembly). There is a relatively high rate of reported complications with VEPTR in the literature. We discuss the potential complications of VEPTR, including infection, rib fracture, dislodged hardware and neurological injury, with an emphasis on imaging diagnosis.

Lipoid pneumonia due to Mexican folk remedies: cultural barriers to diagnosis.

OBJECTIVE: To describe 2 cases of lipoid pneumonia in Mexican American infants after administration of vegetable- or animal-derived oils and the cultural barriers to diagnosis. Various folk remedies have been documented in the international medical literature that involve the oral or nasal administration of vegetable- or animal-derived oils to children for the treatment of common ailments, including nasal stuffiness, constipation, and colic. Lipoid pneumonia is a known complication of such practices in Mexico, India, Saudi Arabia, and other countries. METHODS: Case reports of 2 Mexican American infants with respiratory distress and interviews with 30 immigrant families of Mexican origin. RESULTS: In both cases, language and cultural barriers resulted in a delayed diagnosis of lipoid pneumonia. Interviews with immigrant families confirmed that oil administration to children is a common traditional therapy in Mexican cultures. CONCLUSIONS: These findings underscore the need for primary care providers to be aware of the traditional practice of oil administration to infants in many cultures, its pathophysiological consequences, the potential cultural barriers to timely diagnosis, and the opportunity to prevent cases of lipoid pneumonia through anticipatory guidance.

Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment.

Cystic fibrosis (CF) is a multiorgan disease caused by mutation of the CF transmembrane conductance regulator (CFTR) gene. Obstructive lung disease is the predominant cause of morbidity and mortality; thus, most efforts to improve outcomes are directed toward slowing or halting lung-disease progression. Current therapies, such as mucolytics, airway clearance techniques, bronchodilators, and antibiotics, aim to suppress airway inflammation and the processes that stimulate it, namely, retention and infection of mucus plaques at the airway surface. New approaches to therapy that aim to ameliorate specific CFTR mutations or mutational classes by restoring normal expression or function are being investigated. Because of its sensitivity in detecting changes associated with early airway obstruction and regional lung disease, high-resolution CT (HRCT) complements pulmonary function testing in defining disease natural history and measuring response to both conventional and experimental therapies. In this review, perspectives on the genetics and microbiology of CF provide a context for understanding the increasing importance of HRCT and other imaging techniques in assessing CF therapies.