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.