Unsuspected Pneumocystis carinii pneumonia at presentation of severe primary immunodeficiency.
BACKGROUND
Pneumocystis carinii is an important pathogen in immunodeficiency but may be an unrecognised cause of respiratory compromise.


OBJECTIVE
To ascertain the incidence of P carinii pneumonia (PCP) at presentation of severe combined immunodeficiency (SCID), whether it had been diagnosed, and the effect of treatment on outcome.


METHODS
The supraregional paediatric bone marrow transplant unit for primary immunodeficiencies at Newcastle General Hospital.


METHODS
Retrospective case note review of infants referred with a diagnosis of SCID from 1992 to 1998.


RESULTS
Ten of 50 infants had PCP at presentation; only one was diagnosed before transfer. Eight were diagnosed by bronchoalveolar lavage and two by lung biopsy. In only one was P carinii identified in nasopharyngeal secretions. Five required ventilation for respiratory failure but all were successfully treated with co-trimoxazole and methylprednisolone with or without nebulised budesonide. Nine survived to bone marrow transplantation and four are long term survivors after bone marrow transplantation; no deaths were related to PCP.


CONCLUSIONS
PCP is a common presenting feature of SCID but is rarely recognised. Bronchoalveolar lavage or lung biopsy are needed for diagnosis. Treatment with co-trimoxazole is highly successful.
Berrington
J E
JE
Paediatric Immunology and Infectious Diseases Unit, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK.
Flood
T J
TJ
Abinun
M
M
Galloway
A
A
Cant
A J
AJ
D006801:Humans; D007223:Infant; D008297:Male; D009894:Opportunistic Infections / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011020:Pneumonia, Pneumocystis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012189:Retrospective Studies; D016511:Severe Combined Immunodeficiency / Q000150:complications*