A 13-month-old girl presented with failure to thrive, repeated chest infection, and fatty offensive diarrhea. Hemoglobin level 8.8gm/dl, platelate count 150/mm3, white blood cell count 3.500/mm3, with absolute neutrophil count (ANC)SOO/uL

(E). Shwachman-Diamond syndrome (505) is an autosomal recessive disorder classically characterized by neutrOpenia, pancreatic insufficiency, and short stature with skeletal abnormalities. The initial symptoms are usually steatorrhea and failure to thrive because of malabsorption, which usually develops by 4 mo of age, although the gastrointestinal symptoms may be subtle in some patients and go unrecognized. Virtually all patients with 505 have neutropenia, with the ANC periodically <1000/uL