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Unsuspected Pneumocystis carinii pneumonia at presentation of severe primary immunodeficiency

Lookup NU author(s): Dr Janet Berrington, Dr Terence Flood, Dr Mario Abinun, Dr Angela Galloway, Professor Andrew Cant

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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 deficiency (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 nasophapyngeal secretions. Five required ventilation for respiratory failure but all were successful treated with co-trimoxazole and methylprednisolone with or without nebulised budesonide. Nine survived to bone narrow 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 needled for diagnosis. Treatment with co-trimoxazole is highly successful.


Publication metadata

Author(s): Berrington JE, Flood TJ, Abinun M, Galloway A, Cant AJ

Publication type: Article

Publication status: Published

Journal: Archives of Disease in Childhood

Year: 2000

Volume: 82

Issue: 2

Pages: 144-147

ISSN (print): 0003-9888

ISSN (electronic): 1468-2044

Publisher: BMJ Publishing Group Ltd

URL: http://adc.bmj.com/content/82/2/144.full.pdf


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