Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review.

We report eight pediatric cases of pulmonary alveolar proteinosis (PAP) that illustrate the polymorphic nature of this disease: two cases with severe neonatal onset, three cases with progressive respiratory distress in patients under 1 year old, and three cases in older children with mild symptoms. Consanguineous parents or affected siblings were identified or suspected in four families. Three patients suffered from associated immune or blood disorders (severe combined immune deficiency, myelodysplasia). The respective roles of a macrophagic dysfunction and of an anomaly of the surfactant are discussed according to the various clinical presentations of pediatric PAP. We performed eight unilateral pulmonary lavages under endoscopy and selective ventilation for two patients under 7 kg in weight. These interventions led to progressive discontinuation of oxygen therapy in one case, and temporarily stabilized the disease for the second. Subsequent recurrence in this second patient was treated by massive lavage under extracorporeal oxygenation. A third infant was successfully transplanted with no recurrence within 3 years. Ambroxol was administered in one case. The three oldest children of our series remained asymptomatic, whereas three of the younger patients died. In the light of this experience, we propose that the treatment administered should be determined according to the age of the patient, the degree of respiratory deficiency, and the nature of any associated pathology.

Bronchiectasis in a child after acrolein inhalation.

Acrolein is an unsaturated aldehyde produced by combustion of many organic compounds. Massive exposure may lead to severe pulmonary disease and possibly death. We report a case of intoxication in a 2-year-old boy; an 18-month follow-up showed development of obstructive bronchiolar disease with diffuse bronchiectasis.

Imaging of lung vascularization and perfusion in children.

Imaging techniques to evaluate lung perfusion are continuously evolving. Chest film is still the first step. Spiral CT has revolutionized chest imaging in children, MRI is still progressing and thanks to it, multiplanar abilities will probably take a great importance in the future. The accessibility of the machine is still a limiting factor. Angiography is now restricted to interventional procedures.

[What is new in pediatric pneumology?]. / Quoi de neuf en pneumologie pédiatrique?

Have been selected for this review: 1) the recent and impressive development of high-resolution and spiral CT scan in pediatric thoracic imaging; 2) the emerging of new and promising therapies for asthma (long acting inhaled beta 2-agonists, leukotriene antagonists, anti-IgE monoclonal antibodies); 3) the multifactorial origin of asthma in childhood; 4) the development of thoracoscopic surgery, a minimal-invasive approach beneficial in numerous circumstances.

Metastatic pulmonary calcifications after cardiac surgery in children.

Six children in whom pulmonary calcifications developed after open heart surgery are described. Asymptomatic alveolar lesions appeared within the first 3 weeks after surgery, during or after intensive care. They were first diagnosed as possible infection or edema and secondarily identified as calcifications in the lungs. In five patients, the pulmonary calcifications partially decreased or disappeared, and the prognosis did not seem to be altered. In one patient, the pulmonary calcifications were associated with cardiac calcifications leading to death. The mechanism of these pulmonary calcifications is thought to be predominantly or exclusively metastatic and partially iatrogenic. Data indicated two major causative factors: therapy with massive doses of calcium and postsurgical acute renal failure. Radiographic findings of persistent alveolar lesions of increasing density in patients undergoing antibiotic therapy after open heart surgery are suggestive of the diagnosis of metastatic pulmonary calcifications. Scintigraphy with bone agents or computed tomography is recommended for early confirmation and prevention of therapeutic errors, especially after cardiac transplantation.

Magnetic resonance imaging for diagnosis of Shwachman's syndrome.

Shwachman's syndrome is a rare disease characterized by the association of chronic diarrhea due to exocrine pancreatic insufficiency, metaphyseal dysostosis, and neutropenia. The diagnosis requires demonstration of lipomatosis, or fatty replacement of the pancreas, which is the typical pathological feature of the disease. Magnetic resonance imaging (MRI) was performed in 13 patients with exocrine pancreatic insufficiency, 7 with Shwachman's syndrome, 2 with Pearson's syndrome, 1 with normal sweat test later diagnosed as cystic fibrosis, and 3 without identified syndrome, and in 7 control children. Ultrasonography in the patients did not differentiate between atrophy and lipomatosis and could not be performed in 3. MRI visualized the pancreas in all. The same image was noted in all patients with Shwachman's syndrome with a normal-sized or enlarged pancreas, a hyperintense signal on T1- and T2-weighted image, and a null signal on short time inversion recovery (STIR)-weighted image, characteristic of fat. In all other patients, the findings were very different: The pancreas was a small structure surrounded with fat. In 1 patient without identified syndrome, the pancreas appeared to be partially replaced with fat. MRI is an excellent imaging technique to correlate the nature of a tissue and its radiological representation, especially fat, which gives a very typical signal. In our brief series of patients with Shwachman's syndrome, MRI had 100% positive predictive value in demonstrating lipomatosis. In atypical cases of pancreatic insufficiency in which some of the clinical features of Shwachman's syndrome are absent. MRI is an invaluable aid in the diagnostic procedure.

Computed tomography with normal chest radiograph in tuberculous infection.

Children with primary tuberculosis infection without disease must be identified and treated preventively to avoid an increase in the incidence of tuberculosis in children. However, the recognition of infected cases without disease is often difficult. In particular, minimal active disease may be present in many cases but unrecognised on chest radiography. Computed tomography was therefore performed in 15 children with tuberculous infection and a normal chest radiograph to measure the size of their mediastinal lymph nodes. Ten control children without tuberculosis were also evaluated. When compared with controls it was found that nine of 15 (60%) infected children had enlarged lymph nodes. Adenopathies were more frequent in infected children less than 4 years old than in those over 8 years old. The demonstration of unrecognised active disease in many infected children raises the question of the adequate treatment for these children. It is proposed that a two drug regimen would be more appropriate than isoniazid alone in these cases.