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Öğe The investigation of relationship between joint findings and serum angiogenic and inflammatory factor levels in severe hemophilia A patients(Lippincott Williams & Wilkins, 2014) Karapinar, Tuba H.; Karadas, Nihal; Ozek, Gulcihan; Tufekci, Ozlem; Atabay, Berna; Turker, Meral; Yuksel, Faize; Karapinar, Deniz Y.; Vergin, Canan; Irken, Gulersu; Oren, HaleDespite the use of primary prophylactic Factor VIII replacement in severe hemophilia A patients, bleeding into joints cannot be prevented completely and early diagnosis and treatment of the joint bleedings are important for prevention of permanent joint damage. Recent studies have shown that neoangiogenesis plays important role in development of synovitis after recurrent joint bleedings. This study aimed to investigate the relationship between joint findings and levels of serum angiogenic and inflammatory factors in severe hemophilia A patients.The patient groups consisted of 10 severe hemophilia A patients with acute joint bleeding and 25 severe hemophilia A patients without acute joint bleeding. They were all inhibitor negative. The control group consisted of 22 healthy male children. Complete blood cell count analysis, C-reactive protein (CRP), serum ferritin, lactic acid, and ELISA-based detection of vascular endothelial growth factor (VEGF), intercellular adhesion molecule-1, thrombomodulin, macrophage migration inhibitory factor (Min, and endostatin were performed from peripheral blood of patient and the control groups. CRP and M IF levels were detected significantly higher in hemophilia patients with acute joint bleeding than patients without acute joint bleeding. There was a positive correlation between serum thrombomodulin, VEGF, and MIF levels. In this study, we demonstrated that serum CRP and MIF levels increases in acute bleeding period regardless of the presence of previous joint damage in children with severe hemophilia. CRP elevation may be a useful and rapid marker for acute bleeding in these patients. Blood Coagul Fibrinolysis 25:703-708 2014 (C) Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.Öğe A novel mutation, IVS13+5G > A, in Ellis-van Creveld syndrome associated with haemophagocytic lymphohistiocytosis(Lippincott Williams & Wilkins, 2013) Zeytinoglu, Mert; Aykut, Ayca; Hazan, Filiz; Torrente, Isabella; Akay, Mehmet C.; Karapinar, Deniz Y.; Vergin, Canan; Cogulu, Ozgur; Özkınay, FerdaÖğe Stevens-Johnson syndrome associated with methotrexate treatment for acute lymphoblastic leukemia: a case report(Soc Argentina Pediatria, 2018) Akinci, Burcu; Sivis, Zuhal O.; Sahin, Akkiz; Karapinar, Deniz Y.; Balkan, Can; Kavakli, Kaan; Aydinok, YesimToxic epidermal necrolysis and Stevens-Johnson syndrome are rare mucocutaneous diseases which are associated with a prolonged course and potentially lethal outcome. They are mostly drug induced and mortality rates are very high. Although mostly skin is involved, multiple organ systems such as cardiovascular, pulmonary, gastrointestinal, and urinary systems may be affected. Here, we report a case of Stevens-Johnson Syndrome associated with methotrexate treatment who developed acute cardiac failure and gastrointestinal hemorrhage beside skin findings. He had been treated with intravenous immunglobulin and methylprednisolone succesfully and continued chemotherapy with methotrexate treatment again.Öğe The use of recombinant activated factor VII in the treatment of massive pulmonary hemorrhage in a preterm infant(Lippincott Williams & Wilkins, 2006) Cetin, Hasan; Yalaz, Mehmet; Akisu, Mete; Karapinar, Deniz Y.; Kavakli, Kaan; Kultursay, NigunPulmonary hemorrhage is a rare but well-known complication in preterm infants. We present a case of massive pulmonary hemorrhage in a 9-day-old male infant, successfully treated with intravenous recombinant activated factor VII (rFVIIa) (NovoSeven; Novo Nordisk). The infant was diagnosed with sepsis-related disseminated intravascular coagulation and required ventilator support for respiratory distress syndrome and blood transfusions due to active bleeding from endotracheal tube. After administration of the second dose of rFVIIa (120 mu g/kg per dose, every 2 h), the active bleeding subsided dramatically and a significant improvement in the oxygenation index was seen 8 h after the third dose of rFVIIa treatment. There were also significant improvements in the prothrombin time, International Normalized Ratio, activated partial thromboplastin time and plasma fibrinogen levels after the third dose of rFVIIa treatment. The infant was discharged on day 82 of life and there was no finding of thrombosis during the hospitalization period. At month 18 of follow-up, there was no morbidity related to the pulmonary and central nervous systems. This case suggests that rFVIIa is effective as an alternative therapy in controlling massive pulmonary hemorrhage of preterm infants.