Browsing by Author "Albayrak, Meryem"

Filter results by typing the first few letters
Now showing 1 - 2 of 2
  • No Thumbnail Available
    Item
    Assessment of clinical and laboratory presentations of familial hemophagocytic lymphohistiocytosis patients with homozygous W374X mutation
    (Pergamon-Elsevier, 2010-08) Balta, Günay; Okur, Hamza; Ünal, Şule; Yaralı, Neşe; Ünal, Selma; Türker, Meral; Güler, Elif; Ertem, Mehmet; Albayrak, Meryem; Patıroğlu, Türkan; Gürgey, Aytemiz; Güneş, Adalet Meral; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 24072843300
    Homozygous W374X mutation was identified in unrelated 13 patients (6M/7F) from consanguineous families, 62% of which had history of deceased sibling. Haplotype analysis provided evidence for the probable existence of a founder effect. Age at disease onset ranged from 1 day to 5.5 months (median 2 months). Hepatic dysfunction was observed in 69%, ascite 62%, hypertriglyceridemia 77%, each hyperferritinemia and hypofibrinogenemia 85%, CNS involvement 46% of patients while birth weights were in normal range. Those with very high ferritin (>20,000 ng/ml) had extremely low fibrinogen levels. Two-thirds of patients receiving HLH protocol died within 20 days of therapy.
  • Thumbnail Image
    Publication
    Thrombolysis with systemic recombinant tissue plasminogen activator in children: A multicenter retrospective study
    (Galenos Yayıncılık, 2021-08-18) Zengin, Emine; Sarper, Nazan; Erdem, Arzu Yazal; Al, Işık Odaman; Evim, Melike Sezgin; Yaralı, Neşe; Belen, Burcu; Akçay, Arzu; Yıldırım, Aysen Türedi; Karapınar, Tuba Hilkay; Güneş, Adalet Meral; Gelen, Sema Aylan; Oren, Hale; Olcay, Lale; Baytan, Birol; Gülen, Hüseyin; Öztürk, Gülyüz; Orhan, Mehmet Fatih; Oymak, Yeşim; Akpınar, Sibel; Tüfekci, Özlem; Albayrak, Meryem; Günen, Burçak Tatlı; Canpolat, Aylin; Özbek, Namık; SEZGİN EVİM, MELİKE; MERAL GÜNEŞ, ADALET; Baytan, Birol; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Hematoloji Bilim Dalı.; 0000-0002-4792-269X; 0000-0002-0686-7129; 0000-0002-9375-2855; JGX-6145-2023 ; AAH-1452-2021 ; DVW-8108-2022
    Objective: This study aimed to evaluate systemic thrombolysis experiences with recombinant tissue plasminogen activator (rtPA). Materials and Methods: Retrospective data were collected from 13 Turkish pediatric hematology centers. The dose and duration of rtPA treatment, concomitant anticoagulant treatment, complete clot resolution (CCR), partial clot resolution (PCR), and bleeding complications were evaluated. Low-dose (LD) rtPA treatment was defined as 0.01-0.06 mg/kg/h and high-dose (HD) rtPA as 0.1-0.5 mg/kg/h. Results: Between 2005 and 2019, 55 thrombotic episodes of 54 pediatric patients with a median age of 5 years (range: 1 day to 17.75 years) were evaluated. These patients had intracardiac thrombosis (n=16), deep vein thrombosis (DVT) (n=15), non-stroke arterial thrombosis (n=14), pulmonary thromboembolism (PE) (n=6), and stroke (n=4). The duration from thrombus detection to rtPA initiation was a median of 12 h (range: 2-504 h) and it was significantly longer in cases of DVT and PE compared to stroke, non-stroke arterial thrombosis, and intracardiac thrombosis (p=0.024). In 63.6% of the episodes, heparin was initiated before rtPA treatment. LD and HD rtPA were administered in 22 and 33 of the episodes, respectively. Concomitant anticoagulation was used in 90% and 36% of the episodes with LD and HD rtPA, respectively (p=0.0001). Median total duration of LD and HD rtPA infusions was 30 h (range: 2-120 h) and 18 h (2-120 h), respectively (p=0.044). Non-fatal major and minor bleeding rates were 12.5% and 16.7% for LD and 3.2% and 25.8% for HD rtPA, respectively. At the end of the rtPA infusions, CCR and PCR were achieved in 32.7% and 49.0% of the episodes, respectively. The most successful site for thrombolysis was intracardiac thrombosis. HD versus LD rtPA administration was not correlated with CCR/PCR or bleeding (p>0.05). Conclusion: Systemic thrombolytic therapy may save lives and organs effectively if it is used at the right indications and the right times in children with high-risk thrombosis by experienced hematologists with close monitoring of recanalization and bleeding.