| Autor: |
Fahad Alsohime, Mohamad-Hani Temsah, Rawan M. Alotaibi, Reham M. Alhalabi, Sarah AlEnezy, Aly Abdelrahman Yousef, Abdullah Mohammed Alzaydi, Hussam Sameer Inany, Ayman Al-Eyadhy, Mohammed Almazyad, Ali Alharbi, Abdulaziz Abdullah Alsoqati, Abdurahman Andijani, Mohammed Abu Ghazal, Kamal El Masri, Maher Doussouki, Raheel Farooq Butt, Saleh Alshehri, Mohammed Alsatrawi, Jaramia Macarambon, Gamal M. Hasan, Abdulrahman Alsultan |
| Jazyk: |
angličtina |
| Rok vydání: |
2023 |
| Předmět: |
|
| Zdroj: |
Frontiers in Pediatrics, Vol 11 (2023) |
| Druh dokumentu: |
article |
| ISSN: |
2296-2360 |
| DOI: |
10.3389/fped.2023.1152409 |
| Popis: |
ObjectivesWe aimed to describe Familial Hemophagocytic Lymphohistiocytosis (F-HLH) patients' clinical features, intensive care courses, and outcomes.MethodsMulti-center retrospective cohort study of pediatric patients diagnosed with F-HLH from 2015 to 2020 in five tertiary centers in Saudi Arabia. Patients were classified as F-HLH based on their genetic confirmation of known mutation or on their clinical criteria, which include a constellation of abnormalities, early disease onset, recurrent HLH in the absence of other causes, or a family history of HLH.ResultsFifty-eight patients (28 male, 30 female), with a mean age of 21.0 ± 33.9 months, were included. The most common principal diagnosis was hematological or immune dysfunction (39.7%), followed by cardiovascular dysfunction in 13 (22.4%) patients. Fever was the most common clinical presentation in 27.6%, followed by convulsions (13.8%) and bleeding (13.8%). There were 20 patients (34.5%) who had splenomegaly, and more than 70% of patients had hyperferritinemia >500 mg/dl, hypertriglyceridemia >150 mg/dl and hemophagocytosis in bone marrow biopsy. Compared to deceased patients 18 (31%), survivors had significantly lower PT (p = 041), bilirubin level of |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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