Interstitial lung disease in a child heterozygous for the 1549C-->GAA (121ins2) mutation of surfactant protein B.

bacteria, an altered immune response, an altered histiocytic response or intrinsically abnormal macrophages [5]. The most common site of infection in females is the urinary tract and it is associated with infection with Gram-negative organisms, mainly Escherichia coli and Klebsiella sp. The extragenitourinary sites that are involved are the skin and the pulmonary, renal and gastrointestinal tract. Other microorganisms associated with malakoplakia are Rhodococci equi, Mycobacteria sp., Pasteurella multocida, Proteus mirabilis, Pseudomonas aeruginosa, Yersinia sp., Staphylococcus aurues and fungal infection [6–9]. Thus, in cases such as ours where no positive microbiology is obtained, antibiotics covering Gram-positive and -negative organisms should perhaps be used for at least 2 weeks. So far, less than 40 cases of pulmonary malakoplakia have been reported and there is therefore little evidence for definite treatment.