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Başlık: Infantile hepatic hemangioendothelioma: Clinical presentation and treatment
Diğer Başlıklar: İnfantil karaciğer hemanjioendotelyoması: Klinik ve tedavi
Yazarlar: Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Onkoloji Anabilim Dalı.
Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Gastroenteroloji Anabilim Dalı.
0000-0001-5740-9729
Sevinir, Betül
Özkan, Tanju
AAH-1570-2021
6603199915
7004474005
Anahtar kelimeler: High dose interferon-alpha treatment
Kasabach-merritt syndrome
Infantile hepatic hemangioendothelioma
Gastroenterology & hepatology
Kasabach merritt phenomenon
Therapy
Hemangiomas
Management
Infancy
Alpha
Yayın Tarihi: Eyl-2007
Yayıncı: AVES
Atıf: Sevinir, B. ve Özkan, T. B. (2007). "Infantile hepatic hemangioendothelioma: Clinical presentation and treatment". Turkish Journal of Gastroenterology, 18(3), 182-187.
Özet: Bachground/aims: Hepatic hemangioendotheliomas are rare tumors in childhood. We report our 10-years' experience in a tertiary health center. Methods: This retrospective analysis included eight patients with infantile hepatic hemangioendothelioma. Results: The median age at diagnosis was 24 days (age range: 1 to 70 days) and the female/male ratio was 5/3. The main symptoms were abdominal distention and respiratory distress. Cutaneous hemangiomas were present in four cases. Three infants had Kasabach-Merritt syndrome. Four cases had single hepatic tumors while the others had multiple. The tumor size ranged from 2 cm to 10 cm in diameter. These lesions were located equally in the right and left hepatic lobes, and three babies had bilobar involvement. Most of the multifocal hepatic tumors were associated with skin hemangiomas. Treatment options were assessed individually. Systemic prednisolone therapy (29 mg/kg/d) was commenced in six patients. Five patients responded to corticosteroids. One boy with Kasabach-Merritt syndrome did not respond to this therapy. Interferon-alpha (1 million units (MU)/m(2)/day) was started, and the daily dose of the drug was increased up to 10 MU/m(2), administered 3 times per week, until clinical improvement was achieved. The response was very good and we observed only constitutional adverse symptoms. Two cases were operated; one died from intraoperative bleeding. Other patients were alive and well for 11 to 66 months. Overall survival was 87% in our series. Conclusions: The treatment approaches depend on the centers experience. A multidisciplinary approach is required for the best treatment option.
URI: https://turkjgastroenterol.org/en/infantile-hepatic-hemangioendothelioma-clinical-presentation-and-treatment-1621485
http://hdl.handle.net/11452/29194
ISSN: 1300-4948
2148-5607
Koleksiyonlarda Görünür:PubMed
Scopus
TrDizin
Web of Science

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