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ANSWER

The diagnosis of CEP is certainly very strongly suggested by the characteristic radiology elevated IgE and BAL results. In adults the diagnosis is generally made based on radiological appearance alone.

The gold standard for diagnosis is a lung biopsy. Although this is a safe procedure in experienced hands we felt sufficiently sure of the diagnosis to make it without a lung biopsy. This point was discussed in detail in rounds and there were some differences of opinion. One center said that it is reasonable not to biopsy in the adult population but that this criteria could not be applied to the pediatric population. Biopsy following recurrence may show an altered disease pattern. Another center said that the only role of the biopsy would be to exclude causes of disease, which were excluded with the investigations already performed. It was argued that a biopsy would not alter management in the patient and for this reason it was felt to be an unnecessary procedure for the child. If there is recurrence of the disease biopsy could be considered at a later date.

WHAT MANAGEMENT WOULD YOU RECOMMEND FOR THIS PATIENT?


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