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Öğe Group 3 innate lymhoid cells are absent in DOCK8-defective HIES patients(Amer Assoc Immunologists, 2018) Eken, Ahmet; Okus, Fatma Zehra; Patiroglu, Turkan; Erdem, Serife; Karakukcu, Musa; Cansever, Murat; Donmez-Altuntas, Hamiyet[Abstract Not Available]Öğe Group 3 innate lymhoid cells are absent in dock8-defective hıes patients(Amer assoc ımmunologısts, 9650 rockvılle pıke, bethesda, md 20814 usa, 2018) Eken, Ahmet; Okus, Fatma Zehra; Patiroglu, Turkan; Erdem, SerifeÖğe Inherited prothrombotic risk factors in children with hereditary angioedema(2019) Cansever, Murat; Ozcan, Alper; Ozkul, Yusuf; Patiroglu, TurkanAim: Hereditary angioedema is characterized with recurrent mucocutaneous angioedema, abdominal pain, edema of larynx and extremities. Dermal vascular thrombosis and systemic coagulation may occur in patients with hereditary angioedema due to inhibition of activated factor XII, thrombin and plasmin. Aim of this study was to screen patients with HAE for prothrombotic genetic risk factors before treatment.Material and Methods: Ten patients with hereditary angioedema who were followed up at our clinic were included in our study. The type and frequency of attack, use of prophylaxis and family history of hereditary angioedema were questioned and prothrombotic risk factors were studied.Results: Among the 10 included patients, five of them were male (50%) and five were female (50%). Four patients had abdominal edema (40%), four patients had edema of hands, feet and face (40%). One patient (10%) had heterozygous factor V G1691A mutation, another one had also heterozygous protrombin G20210A mutation. The heterozygous methylene tetrahydrofolate reductase (MTHFR) mutation were identified in seven patients (70%) and homozygous MTHFR mutation were found two patients (20%).Conclusion: In patients with hereditary angioedema, evaluation of protrombotic risk factors was crucial to estimate attack frequency-severity and treatment related thrombosis risk.Keywords: Hereditary angioedema; prothrombotic risk factors; C1 inhibitor; thrombosis.Öğe Multiple hepatic epithelioid hemangioendothelioma in an adolescent girl mimicking liver metastasis(2018) Yilmaz, Ebru; Yikilmaz, Ali; Gul Kirkas, Ozlem; Ozcan, Alper; Deniz, Kemal; Patiroglu, Turkan; Unal, Ekrem; Karakukcu, MusaÖğe A pilot study about subcutaneous administration of 10% immunoglobulin in patients with primary immune deficiencies: Single center experience(2017) Cansever, Murat; Bektas Kut, Fulya; Patiroglu, TurkanAim: In this study, we aimed to see the safety, protectivity and adverse events of rapid infusion subcutaneous immunoglobulin (SCIG) therapy in our patients for one year duration. Material and methods: 10 patients diagnosed with primary immune deficiency and receiving regular intravenous immunoglobulin (IVIG) were randomly included to the study, then their IVIG replacement therapy changed as rapidly infused SCIG in same monthly dose. Patients were evaluated in different times for following aspects; serum IgG levels, frequency of infections, side effects, local reactions, and improvement of life quality. IgG levels of patients were measured at the beginning, 3, 6, and 12 months of SCIG replacement treatment. Results: Local reactions were high at the beginning, then decreased with recurrent infusions. Any severe systemic reactions were not observed in patients. Less infection rate was seen in four patients who were not receiving IVIG regularly before with good compliance in all patients. Infection frequency remained same in 4patients. Increased levels of IgG were achieved eight of the patients at end of the 6months and their levels remained as stable at the end of a year. Conclusion: Our study showed that rapid SCIG therapy in same monthly dose with IVIG is as effective as IVIG for preventing infections without any worse systemic reactionsÖğe Retrospective evaluation of patients with humoral immune deficiency(2018) Cansever, Murat; Patiroglu, TurkanAim: Patients, who were being followed up for the last 10 years by Pediatric Immunology unit of the Faculty of Medicine at Erciyes University, were evaluated retrospectively in order to contribute toward the early diagnosis and the treatment of patients with humoral immunodeficiency. Material and Methods: 412 patients with humoral immunodeficiency were evaluated retrospectively. Patients’ age, gender, consanguineous marriages, family history of immune deficiency or similar diseases were studied at the time of complaints and diagnosis. Results: Humoral immune deficiency was found among 412 out of 536 (76.8%) patients, who were being followed up because of primary immune deficiency. Although the average age of patients at the time of diagnosis was 48.9±4.6 months, the average age was 28.6±33.4 months at the start of complaints. Transient hypogammaglobinemia of infancy was observed the most among patients (50.5%), followed by IgA deficiency (28.4% ), hypogammaglobinemia (6.8%), IgA +IgM deficiency (3.6%), IgG subclass deficiency (3.2%), Common variable immunodeficiency (2.4%), Bruton disease (1.2%), and hyper IgM syndrome (0.5%) respectively. IVIG replacements were given regularly to patients who were diagnosed with either hypogammaglobinemia, CVID, Bruton disease and hyper IgM. Conclusions: In patients with recurrent infections, we need to certainly consider immune deficiency diseases first. Also, we need to be aware of the fact that humoral immune deficiency would relatively be diagnosable and treatable in most of these diseases. Finally,we need to educate the public about the role of marriages among relatives in order to decrease the frequency of these diseases.
