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Browsing by Author "0000-0001-5740-9729"
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Item Infantile hepatic hemangioendothelioma: Clinical presentation and treatment(AVES, 2007-09) Sevinir, Betül; Özkan, Tanju; Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Onkoloji Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Gastroenteroloji Anabilim Dalı.; 0000-0001-5740-9729; AAH-1570-2021; 6603199915; 7004474005Bachground/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.Item Pediatric gaucher experience in South Marmara region of Turkey(Aves, 2011-10) Erdemir, Gülin; Özkan, Tanju; Özgür, Taner; Yazıcı, Zeynep; Özdemir, Özlem; Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Gastroenteroloji Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Radyoloji Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Nöroloji Anabilim Dalı.; 0000-0001-5740-9729; AAG-8381-2021; AAI-2303-2021; 36015044400; 7004474005; 36087775800; 6701668723; 26647804400Background/aims: The aim was to represent the clinical characteristics of six children with Gaucher disease and to describe the results of three years' enzyme replacement therapy. Material and Methods: The data of six Gaucher patients treated with imiglucerase for more than three years were collected. Age, gender, ant hropometric measurements, physical examination findings, ophthalmological evaluations, blood counts, liver function tests, liver and spleen sizes, and bone mineral density of the patients were recorded. Clinical presentations, progressions and therapeutic achievements were evaluated. Results: At the time of diagnosis, all patients were clinically type 1 Gaucher disease. Bone lesions, thrombocytopenia and hepatosplenomegaly were found in all patients at diagnosis. After three years of therapy, normalization of blood cell counts, liver and spleen sizes, bone mineral density, and growth was achieved in all patients. Two patients developed neurological symptoms on enzyme replacement therapy, and the diagnosis in these patients was changed to Gaucher type 3. We observed progression of vertebral bone lesions in three patients despite treatment. Conclusions: The results of enzyme replacement therapy are satisfying, but the possibility of deterioration in clinical findings despite therapy should be kept in mind.