[Persistence of pulmonary lesions in a 6-year-old boy with type I Gaucher's disease treated by alglucerase since the age of 20 months].
BACKGROUND
Gaucher's disease is a serious disorder which becomes curable by bone marrow transplantation. Enzyme replacement therapy has permitted improvement in quality of life and has been shown to decrease the disease's progression.


METHODS
A 6-year-old boy was treated by alglucerase (Ceredase) since the age of 20 months. He presented at that time a failure to thrive, pancytopenia, hepatosplenomegaly and bone involvement. The lung X-rays revealed a diffuse reticulonodular pattern. After 4 years and 5 months, there was an improvement of growth and hepatosplenomegaly, associated with hematological parameters and bone changes normalization. However, an important interstitial syndrome without clinical signs persisted.


CONCLUSIONS
In our patient it is impossible to note the difference between pulmonary disease from fibrotic origin and pulmonary involvement by Gaucher's cells accumulation.
Versteegh
C
C
Service d'hématologie, hôpital universitaire des enfants Reine-Fabiola, ULB, Belgique.
Avni
F
F
Cuvelier
P
P
Ferster
A
A
D002648:Child; D005776:Gaucher Disease / Q000150:complications / Q000188:drug therapy*; D005962:Glucosylceramidase / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D013902:Radiography, Thoracic; D013997:Time Factors; D014057:Tomography, X-Ray Computed
C070292:alglucerase; D005962:Glucosylceramidase