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Arch Dis Child. 2000 Feb;82(2):144-7.

Unsuspected Pneumocystis carinii pneumonia at presentation of severe primary immunodeficiency.

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1
Paediatric Immunology and Infectious Diseases Unit, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK.

Abstract

BACKGROUND:

Pneumocystis carinii is an important pathogen in immunodeficiency but may be an unrecognised cause of respiratory compromise.

OBJECTIVES:

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.

SETTING:

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.

PMID:
10648370
PMCID:
PMC1718210
[Indexed for MEDLINE]
Free PMC Article
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