Search

It seems that the patients' age, severity of disease, and additional complications should dictate the type of treatment for patients with pulmonary alveolar proteinosis (PAP). Researchers presented the medical histories and treatment plans of eight patients with different forms of PAP, proposed theories to explain the development of PAP, and suggested guidelines for treating patients with different PAP disease forms. Six of the 8 patients had family histories of PAP or were children of parents possibly related by blood. One young infant (4 months) treated with lavage, or a washing out of the lungs, fully recovered while another young infant (6 months) treated with lavage showed marginal improvement but died 5 months later. A heart-lung transplantation was successful in one 16-month old patient. One young infant with PAP and a defective immune system died. All three of the older children remained stable with minimal drug intervention., 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 uniltateral 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., ABBREVIATIONS. PAP, pulmonary alveolar proteinosis; PAS, periodic acid Schiff; BAL, bronchoalveolar lavage; OLB, open lung biopsy; SCID, severe combined immune deficiency; PC, Pneumocystis carinii; CT, computed tomography; LFT, lung function [...]