Mouy R, Ropert J C, Donadieu J, Hubert P, de Blic J, Revillon Y, Brunelle F, Schollet Martin S, Descamps B, Debré M
Département de pédiatrie, Inserm U25, hôpital des Enfants-Malades, Paris, France.
Arch Pediatr. 1995 Sep;2(9):861-4. doi: 10.1016/0929-693x(96)81264-4.
Pulmonary aspergillosis is now the main cause of death in chronic granulomatous disease (CGD); it may occur before the age of one year and then often reveals CGD.
A male newborn was referred to hospital at 27 days of age for fever (39 degrees C), hemodynamic failure and biological inflammation syndrome caused by pulmonary infection. Chest CT scan revealed multiple and bilateral intraparenchymatous nodules. An open lung biopsy showed histiocystic granuloma with multinucleated giant cells. Culture of tracheal, bronchoalveolar lavage samples and lung biopsy grew positive for Aspergillus fumigatus. Impaired chemiluminescence production by neutrophils was detected, enabling the diagnosis of CGD. It was later confirmed by the study of neutrophils functions. The child recovered after 12 months of parenteral amphotericin B therapy.
A febrile multifocal pneumopathy occurring in infancy should lead to consider the possibility of CGD which may be confirmed by the chemiluminescence test.
