PULMONARY ALVEOLAR PROTEINOSIS - EXPERIENCE WITH 8 PEDIATRIC CASES AND A REVIEW

We report eight pediatric cases of pulmonary alveolar proteinosis (PAP ) that illustrate the poly-morphic nature of this disease: two cases w ith severe neonatal onset, three cases with progressive respiratory di stress 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 deficien cy, myelodysplasia). The respective roles of a macrophagic dysfunction and of an anomaly of the surfactant are discussed according to the va rious clinical presentations of pediatric PAP. We performed eight unil tateral pulmonary lavages under endoscopy and selective ventilation fo r two patients under 7 kg in weight. These interventions led to progre ssive discontinuation of oxygen therapy in one case, and temporarily s tabilized the disease for the second. Subsequent recurrence in this se cond patient was treated by massive lavage under extracorporeal oxygen ation. A third infant was successfully transplanted with no recurrence within 3 years. Ambroxol was administered in one case. The three olde st children of our series remained asymptomatic, whereas three of the younger patients died. In the light of this experience, we propose tha t 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.