Tıp Fakültesi / Faculty of Medicine

Permanent URI for this collectionhttps://hdl.handle.net/11727/1403

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Author: search.filters.author.Ozdemir, Binnaz HandanSubject: search.filters.subject.Diagnosis

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    Hepatic Angiosarcoma and Liver Transplant: A Report of 2 Cases With Diagnostic Difficulties
    (2014) Terzi, Aysen; Deniz, Emine Ebru; Haberal, Nihan; Moray, Gokhan; Ozdemir, Binnaz Handan; https://orcid.org/0000-0002-1225-1320; https://orcid.org/0000-0001-9852-9911; https://orcid.org/0000-0003-2498-7287; https://orcid.org/0000-0002-7528-3557; 24635809; F-7546-2013; AAK-4587-2021; AAE-1041-2021; X-8540-2019
    Angiosarcoma is a rare primary malignant mesenchymal tumor of the liver. The prognosis of hepatic angiosarcoma is poor with an average life expectancy of 6 months after diagnosis. Diagnosing hepatic angiosarcoma is challenging because of nondiagnostic liver biopsy or specious history and radiologic presentation. We report 2 cases with hepatic angiosarcoma which were diagnosed histopathologically in the native liver after liver transplant. One of 2 patients was lost to follow-up, and another patient died of relapsing hepatic angiosarcoma 18 months after the liver transplant.
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    Expert opinion on the recognition, diagnosis and management of children and adults with Fabry disease: a multidisciplinary Turkey perspective
    (2022) Ezgu, Fatih; Alpsoy, Erkan; Bicik Bahcebasi, Zerrin; Kasapcopur, Ozgur; Palamar, Melis; Onay, Huseyin; Ozdemir, Binnaz Handan; Topcuoglu, Mehmet Akif; Tufekcioglu, Omac; 0000-0003-3478-9292; 35236382
    This consensus statement by a panel of Fabry experts aimed to identify areas of consensus on conceptual, clinical and therapeutic aspects of Fabry disease (FD) and to provide guidance to healthcare providers on best practice in the management of pediatric and adult patients with FD. This consensus statement indicated the clinical heterogeneity of FD as well as a large number of pathogenic variants in the GLA gene, emphasizing a need for an individualized approach to patient care. The experts reached consensus on the critical role of a high index of suspicion in symptomatic patients and screening of certain at-risk groups to reveal timely and accurate diagnosis of FD along with an increased awareness of the treating physician about the different kinds of pathogenic variants and their clinical implications. The experts emphasized the crucial role of timely recognition of FD with minimal delay from symptom onset to definite diagnosis in better management of FD patients, given the likelihood of changing the disease's natural history, improving the patients' quality of life and the prognosis after enzyme replacement therapy (ERT) administered through a coordinated, multidisciplinary care approach. In this regard, this consensus document is expected to increase awareness among physicians about unique characteristics of FD to assist clinicians in recognizing FD with a well-established clinical suspicion consistent with pathogenic variants and gender-based heterogeneous clinical manifestations of FD and in translating this information into their clinical practice for best practice in the management of patients with FD.