Article
Unsuspected Pneumocystis carinii
pneumonia at presentation of severe primary immunodeficiency
Janet E Berringtona, Terence J Flooda, Mario Abinuna, Angela Gallowayb, Andrew J Canta
a Paediatric
Immunology and Infectious Diseases Unit, Newcastle General Hospital,
Westgate Road, Newcastle upon Tyne NE4 6BE, UK, b Newcastle Public Health Laboratory, Newcastle
upon Tyne, UK
Correspondence to: Dr Cant email: a.j.cant{at}ncl.ac.uk
Accepted 10
September 1999
BACKGROUND
Pneumocystis
carinii is an important pathogen in immunodeficiency but may be
an unrecognised cause of respiratory compromise.
OBJECTIVESTo
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.
SETTINGThe
supraregional paediatric bone marrow transplant unit for primary
immunodeficiencies at Newcastle General Hospital.
METHODSRetrospective
case note review of infants referred with a diagnosis of SCID from 1992 to 1998.
RESULTSTen 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.
CONCLUSIONSPCP 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.
Keywords: Pneumocystis carinii; pneumonia; severe combined immunodeficiency
© 2000 by Archives of Disease in Childhood
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Thompson, N., Saglani, S., Bush, A.
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Gennery, A R, Cant, A J
(2001). Diagnosis of severe combined immunodeficiency. J. Clin. Pathol.
54: 191-195
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