Correspondence

Case 49-1993: Cystic Fibrosis

N Engl J Med 1994; 330:1319May 5, 1994

Article

To the Editor:

The clinicians involved in Case 49-1993 (Dec. 9 issue),1 which describes a patient with chronic pulmonary disease that started in infancy, do a disservice to pediatricians in not remembering that common things are common. The patient had chronic respiratory disease and failure to thrive, with progression to bronchiectasis and pulmonary failure. I realize that the clinicopathological exercises are intended to stretch our minds, but they should also teach us to think logically. The diagnosis of cystic fibrosis appears nowhere in the Case Record.1 Although there were findings that militated against this diagnosis, it at least deserved to be discussed and ruled out. Surely it is near the top of the list of causes of bronchiectasis in childhood.

Neil R.M. Buist, M.B., Ch.B.
Oregon Health Sciences University, Portland, OR 97201-3042

1 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 49-1993). N Engl J Med 1993;329:1797-1805
   Full Text | Web of Science | Medline

Author/Editor Response

Dr. Colin, the discussant of Case 49-1993, replies:

To the Editor: Dr. Buist's letter is appreciated, and his comment is unquestionably correct. Since the course of the disease of this patient was distinctly not suggestive of cystic fibrosis, the discussion of bronchiectasis was limited to conditions relevant to the case -- traction bronchiectasis and allergic bronchopulmonary dysplasia.

The original presentation contained a slide of the causes of bronchiectasis in adolescents, but because of irrelevance to the case, it was not discussed in detail. This slide included congenital anatomical disorders, exemplified by tracheobronchomegaly (Mounier-Kuhn syndrome); congenital diseases, such as cystic fibrosis and various conditions of congenital immune deficiencies, among many others; infectious disorders, such as pertussis and measles; and chronic bronchitis, exemplified by tuberculosis or foreign-body aspiration.

For a focused discussion and for considerations of space, it appeared to me that a table including the detailed differential diagnosis would be beyond the scope of the published case. If, as Dr. Buist points out, the clinicopathological exercises are a wider teaching tool, then the information probably should have been incorporated into the body of the discussion. Dr. Buist's point is well taken.

Andrew A. Colin, M.D.
Children's Hospital, Boston, MA 02115