J 2025

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

GASPARELLA, Paolo; Emir Q HAXHIJA; Rune ANDERSEN; Maria BAREA; Eulalia BASELGA et al.

Základní údaje

Originální název

The VASCERN-VASCA diagnostic and management pathways for kaposiform hemangioendothelioma

Autoři

GASPARELLA, Paolo; Emir Q HAXHIJA; Rune ANDERSEN; Maria BAREA; Eulalia BASELGA; Miguel Bejarano SERRANO; Sigurd BERGER; Annouk Anne BISDORFF; Olivia BOCCARA; Petra BORGARDS; Maria BOM-SUCESSO; Laurence M BOON; Anca Maria CIMPEAN; Andrea DIOCIAIUTI; Veronika DVORAKOVA; May El HACHEM; Sofia FRISK; Nader GHAFFARPOUR; Annegret HOLM; Alan D IRVINE; Mikkel KALTOFT; Friedrich G KAPP; Olga KOSKOVA; Kristiina KYRKLUND; Miguel MADUREIRA; Darius PALIONIS; Przemyslaw PRZEWRATIL; Bitten SCHONEWOLF-GREULICH; Maria-Corina STANCIULESCU; Jaroslav ŠTĚRBA; Jukka TOLONEN; Birute VAISNYTE; Van Der Vleuten CARINE; Dariusz WYRZYKOWSKI; Leo Schultze KOOL a Miikka VIKKULA

Vydání

European journal of pediatrics, New York, Springer, 2025, 0340-6199

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30230 Other clinical medicine subjects

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

Impakt faktor

Impact factor: 2.600 v roce 2024

Organizační jednotka

CZECRIN IV

UT WoS

001637522100002

Klíčová slova česky

Kaposiform hemangioendothelioma; Kasabach-Merrit phenomenon; Vascular tumour; Diagnosis; Treatment; Algorithm; Sirolimus; Patient pathway

Klíčová slova anglicky

Kaposiform hemangioendothelioma; Kasabach-Merrit phenomenon; Vascular tumour; Diagnosis; Treatment; Algorithm; Sirolimus; Patient pathway

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 22. 1. 2026 12:26, Bc. Hana Vladíková, BBA

Anotace

V originále

The European Reference Network (ERN) on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe in order to improve and provide high-level healthcare to all patients with rare vascular diseases and provide accessible cross-border healthcare to patients with rare vascular diseases. Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour with local aggressiveness, often associated with a life-threatening coagulopathy known as the Kasabach-Merritt phenomenon (KMP). The Vascular Anomalies Working Group (VASCA-WG) developed diagnostic and therapeutic pathways for the management of KHE using the nominal group technique (NGT), a well-established, structured, multi-stage, facilitated group meeting technique used to generate consensus statements. The pathways were developed following two face-to-face meetings and several web meetings to facilitate discussion.Conclusion: VASCA has produced this expert opinion, which reflects the strategies developed by experts and patient representatives on how to approach patients with Kaposiform Hemangiothelioma in a practical manner; here, we present an algorithmic flow chart of this work.What is Known:center dot Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, often associated with a life threatening coagulopathy, and can be challenging to manage.What is New:center dot The diagnostic and therapeutic pathway for KHE, developed by a multidisciplinary group of experts (VASCA-VASCERN Group), offers concrete offers concrete help in making decisions about managing this condition.

Návaznosti

90249, velká výzkumná infrastruktura
Název: CZECRIN IV