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[Pneumocystis carinii pneumonia in patients with a severe combined immunodeficiency]
M J Smit1, R de Groot, J J van Dongen
1Afd. Kindergeneeskunde, Academisch Ziekenhuis Rotterdam/Sophia Kinderziekenhuis.
Abstract:
We describe three patients with Pneumocystis carinii pneumonia as the initial presentation of severe combined immunodeficiency disease. The pneumonia in the first patient was treated successfully with trimethoprim/sulphamethoxazole (Tmp/Smz). The second patient died despite therapy with Tmp/Smz and pentamidine. The third patient failed to respond to therapy with Tmp/Smz and pentamidine. He was subsequently treated with trimetrexate and leucovorin. Treatment with the new folic acid antagonist trimetrexate resulted in complete recovery. The case histories of these children serve to illustrate the clinical symptoms and new therapeutic modalities of P. carinii pneumonia in patients with immunodeficiency disease.
Insights
Severe combined immunodeficiency disease can present as Pneumocystis pneumonia. Trimetrexate offers a new effective treatment for Pneumocystis pneumonia in immunocompromised patients.
Area of Science:
- Immunology
- Infectious Diseases
- Pediatrics
Background:
- Severe combined immunodeficiency (SCID) is a group of rare genetic disorders characterized by profound defects in both cellular and humoral immunity.
- Pneumocystis carinii pneumonia (PCP) is a common opportunistic infection in individuals with compromised immune systems, including those with SCID.
- Early diagnosis and treatment of PCP in SCID patients are crucial for survival.
Observation:
- Three pediatric cases of SCID presenting initially with PCP are detailed.
- The first patient responded to trimethoprim/sulphamethoxazole (Tmp/Smz).
- The second patient succumbed to PCP despite treatment with Tmp/Smz and pentamidine.
Findings:
- The third patient, unresponsive to Tmp/Smz and pentamidine, achieved complete recovery with trimetrexate and leucovorin.
- Trimetrexate, a novel folic acid antagonist, demonstrated significant efficacy in this refractory PCP case.
- This highlights varying responses to standard PCP therapies in SCID.
Implications:
- These cases underscore the importance of considering SCID in infants presenting with PCP.
- Trimetrexate represents a promising therapeutic option for PCP in SCID patients refractory to conventional treatments.
- Further research into optimal PCP management strategies for SCID is warranted.