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- Title
A complication of lysinuric protein intolerance: Intermittent haemophagocytic lymphohistiocytosis.
- Authors
Akyol, Şefika; Yılmaz, Şebnem; Tüfekçi, Özlem; Arslan, Nur; Ören, Hale
- Abstract
Therefore, the patient's current diagnosis of LPI, the presence of impaired metabolic control, and clinical, laboratory and bone marrow findings were all evaluated together and a diagnosis of LPI-related HLH was considered. The diagnosis of lysinuric protein intolerance (LPI) or other metabolic disorders causing haemophagocytic lymphohistiocytosis (HLH) should be considered in every patient presenting with symptoms of HLH, even in adults. Recognition of chronic intermittent HLH findings, initiation of effective treatment and ensuring metabolic control under appropriate conditions in patients with the diagnosis of LPI are of clinical importance.
- Subjects
HEMOPHAGOCYTIC lymphohistiocytosis; AMINO acid metabolism disorders; MACROPHAGE activation syndrome
- Publication
Journal of Paediatrics & Child Health, 2022, Vol 58, Issue 12, p2300
- ISSN
1034-4810
- Publication type
Article
- DOI
10.1111/jpc.16158