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Pregled bibliografske jedinice broj: 1106524

Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report


Tagasovski, Igor; Crkvenac Gregorek, Andrea; Huljev Frković, Sanda; Perkov, Dražen; Vrkić Kirhmajer, Majda
Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report // Cardiologia Croatica 16(1-2)
Virtualni kongres: Medical Publishing, d.o.o., 2021. str. 73-73 doi:10.15836/ccar2021.73 (poster, domaća recenzija, sažetak, ostalo)


CROSBI ID: 1106524 Za ispravke kontaktirajte CROSBI podršku putem web obrasca

Naslov
Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report
(Klippel-Trenaunay syndrome – a promising role of sirolimus?
 Case report)

Autori
Tagasovski, Igor ; Crkvenac Gregorek, Andrea ; Huljev Frković, Sanda ; Perkov, Dražen ; Vrkić Kirhmajer, Majda

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, ostalo

Izvornik
Cardiologia Croatica 16(1-2) / - : Medical Publishing, d.o.o., 2021, 73-73

Skup
13. kongres Hrvatskoga kardiološkog društva s međunarodnim sudjelovanjem

Mjesto i datum
Virtualni kongres, 10.-13.12.2020. ; 21.-24.01.2021

Vrsta sudjelovanja
Poster

Vrsta recenzije
Domaća recenzija

Ključne riječi
Klippel-Trenaunay syndrome ; congenital malformation ; sirolimus

Sažetak
Introduction: Klippel-Trenaunay Syndrome (KTS) is rare syndrome characterized by a presence of capillary and venous malformation, limb overgrowth, with or without lymphatic anomalies1 . KTS is related to mutations in the PIK3CA gene. Complications of KTS include clotting disorder, bleeding, lymphedema, soft tissue infection and pain. Treatment is individualized ; it may include endovascular or surgical procedures and supportive care (management of coagulopathy, infection, pain). The use of mammalian target of rapamycin (mTOR) inhibitor sirolimus shows promising results in complex vascular malformations2, 3. Case report: We present a 45 years old patient, currently living out of Croatia. Vascular malformation of the left arm was noticed a few months after his birth. During his childhood an angiography of the left arm lead to confirmation of KTS without further specific treatment. During the last 15 years he was undergoing regular hematology controls and treatment of consumptive coagulopathy as complication of KTS. In 2010 at age 35, he suffered a fracture on the left arm, but he was declared inoperable on basis of vascular malformation which extended the healing process. From 2018 further proliferation of vascular malformation was noticed on his left hemithorax. In 2020 he was presented to multidisciplinary vascular team, MR angiography was performed Unfortunately, due to extreme extension of vascular malformation with soft tissue hypertrophy and osseous deformation the patient is not suitable for any surgical or endovascular procedure. We suggested a treatment with sirolimus which is delayed due to patient’s temporary relocation. Genetic panel testing covering PIK3CA gene is in progress. Conclusion: By presenting this case, our objective is to increase the awareness of KTS, related complications and the role of sirolimus which can improve the prognosis of vascular anomalies. Additionally, we wish to emphasize the importance of multidisciplinary vs specialty focused approach in management patients with KTS.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti



POVEZANOST RADA


Ustanove:
Medicinski fakultet, Zagreb,
Klinički bolnički centar Zagreb

Poveznice na cjeloviti tekst rada:

doi hrcak.srce.hr doi.org

Citiraj ovu publikaciju:

Tagasovski, Igor; Crkvenac Gregorek, Andrea; Huljev Frković, Sanda; Perkov, Dražen; Vrkić Kirhmajer, Majda
Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report // Cardiologia Croatica 16(1-2)
Virtualni kongres: Medical Publishing, d.o.o., 2021. str. 73-73 doi:10.15836/ccar2021.73 (poster, domaća recenzija, sažetak, ostalo)
Tagasovski, I., Crkvenac Gregorek, A., Huljev Frković, S., Perkov, D. & Vrkić Kirhmajer, M. (2021) Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report. U: Cardiologia Croatica 16(1-2) doi:10.15836/ccar2021.73.
@article{article, author = {Tagasovski, Igor and Crkvenac Gregorek, Andrea and Huljev Frkovi\'{c}, Sanda and Perkov, Dra\v{z}en and Vrki\'{c} Kirhmajer, Majda}, year = {2021}, pages = {73-73}, DOI = {10.15836/ccar2021.73}, keywords = {Klippel-Trenaunay syndrome, congenital malformation, sirolimus}, doi = {10.15836/ccar2021.73}, title = {Klippel-Trenaunay syndrome – a promising role of sirolimus? Case report}, keyword = {Klippel-Trenaunay syndrome, congenital malformation, sirolimus}, publisher = {Medical Publishing, d.o.o.}, publisherplace = {Virtualni kongres} }
@article{article, author = {Tagasovski, Igor and Crkvenac Gregorek, Andrea and Huljev Frkovi\'{c}, Sanda and Perkov, Dra\v{z}en and Vrki\'{c} Kirhmajer, Majda}, year = {2021}, pages = {73-73}, DOI = {10.15836/ccar2021.73}, keywords = {Klippel-Trenaunay syndrome, congenital malformation, sirolimus}, doi = {10.15836/ccar2021.73}, title = {Klippel-Trenaunay syndrome – a promising role of sirolimus?
 Case report}, keyword = {Klippel-Trenaunay syndrome, congenital malformation, sirolimus}, publisher = {Medical Publishing, d.o.o.}, publisherplace = {Virtualni kongres} }

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