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Erschienen in: European Journal of Pediatrics 4/2023

03.02.2023 | RESEARCH

Mortality predictive factors in congenital hepatic hemangioma: a case–control study

verfasst von: Carlos Delgado-Miguel, Paloma Triana, Miriam Miguel-Ferrero, Mercedes Díaz, Loreto Hierro, Paloma Jara, Juan Carlos López-Gutiérrez, Francisco Hernández Oliveros

Erschienen in: European Journal of Pediatrics | Ausgabe 4/2023

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Abstract

Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case–control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data.
Conclusions: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality.
What is Known:
• Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening.
What is New:
• Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.
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Metadaten
Titel
Mortality predictive factors in congenital hepatic hemangioma: a case–control study
verfasst von
Carlos Delgado-Miguel
Paloma Triana
Miriam Miguel-Ferrero
Mercedes Díaz
Loreto Hierro
Paloma Jara
Juan Carlos López-Gutiérrez
Francisco Hernández Oliveros
Publikationsdatum
03.02.2023
Verlag
Springer Berlin Heidelberg
Erschienen in
European Journal of Pediatrics / Ausgabe 4/2023
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-023-04849-w

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