PedAM
Pediatric Disease Annotations & Medicines
| Disease | idiopathic hypereosinophilic syndrome |
| Phenotype | C0206141|idiopathic hypereosinophilic syndrome |
| Sentences | 6 |
| PubMedID- 24404401 | idiopathic hypereosinophilic syndrome (ihes) is a group of disorders characterized by the overproduction of eosinophils resulting in inflammatory damage to multiple organs, including skin, heart, lung, gastrointestinal tract, and nervous system.1 signs and symptoms associated with lung involvement are relatively common, occurring in approximately 40% of ihes patients.2,3 however, despite frequent lung involvement, acute respiratory distress syndrome (ards) is rarely seen in conjunction with ihes, with only a few cases described in the literature to date.4,5 this is the first case report of ihes presented with ards in korea. |
| PubMedID- 24009732 | idiopathic hypereosinophilic syndrome (hes) refers to the pathological presence of more than 1.5×109/l of nonreactive eosinophils in peripheral blood lasting for more than 6 months with organ involvement [1]. |
| PubMedID- 21687339 | idiopathic hypereosinophilic syndrome is diagnosed on the basis of clinical and pathologic criteria and is characterized by blood eosinophilia that is neither secondary nor clonal in nature.12 the diagnosis requires documentation of both target end-organ damage and an absolute eosinophil count of 1.5 × 109/l or higher for at least 6 months.13 by definition, testing must demonstrate a lack of any known molecular or cytogenetic markers, and bone marrow histology must not display evidence of abnormal populations of mast cells, monocytosis, trilineage myeloproliferation or dysplasia.14 specific therapy for hypereosinophilic syndrome includes high-dose steroids as first-line treatment, with hydroxyurea added if remission is not achieved. |
| PubMedID- 25671117 | A first study was conducted on the treatment of idiopathic hypereosinophilic syndrome [33] and churg-strauss syndrome, in which a significant reduction was demonstrated in the administration of systemic corticosteroids with a good disease control [34]. |
| PubMedID- 25914812 | Case 2 had been previously diagnosed with idiopathic hypereosinophilic syndrome when her case was published in 2010 17, but a pathology review in 2013 demonstrated that she in fact had igg4-rd. |
| PubMedID- 26435854 | idiopathic hypereosinophilic syndrome (hes) is defined as an unexplained eosinophil count greater than 1,500 cells/ml, persisting longer than 6 months, and resulting in eosinophil-mediated single- or multiple-organ damage [1, 2]. |
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