Yazar "Ozdemir, Hamiyet Hekimci" seçeneğine göre listele
Listeleniyor 1 - 9 / 9
Sayfa Başına Sonuç
Sıralama seçenekleri
Öğe Aplastic Anemia Frequency and Management in Pediatric Liver Transplantations Due to Non-A-E Hepatitis(Aves, 2021) Tasci, Ezgi Kiran; Karakoyun, Miray; Karadas, Nihal Ozdemir; Ozdemir, Hamiyet Hekimci; Karapinar, Deniz Yilmaz; Karaca, Can; Cetin, FundaBackground: Hepatitis-associated aplastic anemia (HAAA) is a rare complication that presented with bone marrow failure after acute hepatitis. HAAA usually occurs in adolescent men within 1-6 months following hepatitis. Most of HAAA's etiology has non-A-E viral hepatitis. Methods: Our retrospective study included patients with acute fulminant hepatitis who had been treated in Ege University Pediatric Gastroenterology, Hepatology and Nutrition Department and Izmir Kent Hospital Clinical, laboratory, and epidemiological data of the patients were collected from the files. Results: In this study, 499 children underwent liver transplantation (LT) in two pediatric transplantation centers. Sixty-eight (13.6%) out of 499 patients, underwent liver transplantation due to fulminant hepatic failure (FHF). Therefore, a total of 64 patients (34 girls, 30 boys) with a diagnosis of FHF have included in the study. Thirty-two (50.0%) of 64 FHF were due to non-A-E hepatitis and 4 out of the 64 patients (6.2%) with FHF developed HAAA. All of the patients received prednisolone as immunosuppression treatment after LT. Three patients were also given Tacrolimus and I received an additional mycophenolate mofetil. One of the patients was given prednisolone and cyclosporine treatment without tacrolimus. Bone marrow transplantation was performed in 1 patient (25.0%). Two of the patients received immunosuppressive treatment including rabbit-derived anti-thymocyte globulin, cyclosporine, and initially prednisolone. Conclusion: In children who underwent liver transplantation for non-A-E FHF are at high risk to develop aplastic anemia. The clinicians should be alert after orthotropic liver transplantation patient could develop aplastic anemia and early treatment with immunosuppressive therapies result in a more successful outcome.Öğe Clinicopathological characteristics, genetics and prognosis of patients with myeloid sarcoma: a single-center study(Bmj Publishing Group, 2022) Demir, Derya; Hekimgi, Mine; Karaca, Emin; Ulusoy, Yusuf; Ozdemir, Hamiyet Hekimci; Saydam, Guray; Durmaz, BurakAim Myeloid sarcoma (MS) is a rare tumour comprising myeloid blasts occurring at an anatomical site other than the bone marrow. We sought to investigate both paediatric and adult patients with MS diagnosed at our institution and determine possible correlations among their clinicopathological, phenotypic, molecular and prognostic features. Methods This study retrospectively evaluated the data of 45 patients diagnosed with MS at Ege University Faculty of Medicine Hospital, Turkey, over a 17-year period. Results The male-to-female ratio was 1.5:1, and the median age was 39.12 years. The most commonly involved sites were the skin, lymph nodes, soft tissues and bone. Immunohistochemically, CD68-KP1 was the most commonly expressed marker, followed by CD33, myeloperoxidase, CD117, lysozyme, CD68-PGM1 and CD34. Of the patients, 26 (57.7%) presented with de novo MS, 7 (15.5%) had simultaneous acute myeloid leukaemia and 12 (26.8%) had a previous history of haematological disorders. Kaplan-Meier survival analysis revealed that the 2-year and 5-year overall survival (OS) rates were 46.4% and 39.8%, respectively; the median OS duration was 11 months. Increasing age had a negative prognostic relationship with survival (p = 0.04). Chromosomal abnormalities were detected in approximately 6/10 (60%) of paediatric patients and 6/9 (66.7%) of adult patients. t(8;21)(q22;q22) translocation was identified in 20% of paediatric patients. Conclusions MS diagnosis is usually challenging; an expanded immunohistochemical panel should be used for an accurate diagnosis. Although MS generally has a poor prognosis, increasing age appears to be associated with a worse outcome.Öğe Congenital Neutropenia Patient With Hypomorphic Biallelic CSF3R Mutation Responding to GCSF(Lippincott Williams & Wilkins, 2019) Karapinar, Deniz Yilmaz; Akinci, Burcu; Yasar, Akkiz Sahin; Ozdemir, Hamiyet Hekimci; Sivis, Zuhal Onder; Onay, Huseyin; Özkınay, FerdaCongenital neutropenia (CN) is a rare disorder, and the most common gene responsible for CN is ELANE. Furthermore, the mutations of HAX1, G6PC3, and JAGN1 genes may cause CN. These patients generally find great benefit from subcutaneous administration of Granulocyte Colony Stimulating Factor (GCSF). In recent years, Biallelic Colony Stimulating Factor 3 Receptor (CSF3R) mutations have been described as an underlying defect of CN in several children. In contrast to the previous group, the patients who have a CSF3R mutation do not respond to GCSF treatment. Here, we present a CN patient with hypomorphic biallelic CSF3R mutation responding to GCSF.Öğe Current childhood chronic myeloid leukemia management under tyrosine kinase inhibitor treatment(Springer Japan Kk, 2022) Karadas, Nihal; Goktepe, Serife Sebnem Onen; Bas, Ilke; Ece, Dilek; Ozdemir, Hamiyet Hekimci; Balkan, Can; Kavakli, KaanChronic myeloid leukemia (CML) is very rare during childhood. Tyrosine kinase inhibitors (TKI) provide very good results in terms of survival. The medical records of 15 chronic phase (CP)-CML patients in a university hospital pediatric hematology department between 1997 and 2022 were reviewed retrospectively. Complete hematological response was documented in all patients between 20 and 68 (median 30) days of treatment. Major molecular response was achieved in seven patients within 6 months. Median follow-up for the study group was 79 (range 3-330) months and overall survival was 100%. Three patients (2 blastic transformation, 1 therapy resistant) underwent bone marrow transplantation (BMT) and one with blastic transformation is scheduled to undergo BMT. TKI were discontinued in three patients after a median of 86 (range 73-177) months. The complete molecular remission maintenance period before discontinuation of TKI was 81 (range 62-122) months. While no molecular relapse was seen before the last follow-up, the median overall follow-up period was 152 (range 131-300) months. In conclusion, recent advances have led to a very good prognosis for children with CP-CML. With TKI treatment, most patients continue their lives without disease progression. Additionally, in selected patients TKI can be discontinued without molecular relapse.Öğe Homozygous c.130-131 ins A (pW44X) mutation in the HAX1 gene as the most common cause of congenital neutropenia in Turkey: Report from the Turkish Severe Congenital Neutropenia Registry(Wiley, 2019) Karapinar, Deniz Yilmaz; Patiroglu, Turkan; Metin, Ayse; Caliskan, Umran; Celkan, Tiraje; Yilmaz, Baris; Karakas, Zeynep; Karapinar, Tuba H.; Akinci, Burcu; Özkınay, Ferda; Onay, Huseyin; Yesilipek, Mehmet Akif; Akar, Himmet Haluk; Tuysuz, Gulen; Tokgoz, Huseyin; Ozdemir, Gul Nihal; Kiykim, Ayca Aslan; Karaman, Serap; Kilinc, Yurdanur; Oymak, Yesim; Kupesiz, Alphan; Olcay, Lale; Yildirim, Zuhal Keskin; Aydogan, Gonul; Gokce, Muge; Ileri, Talia; Aral, Yusuf Ziya; Bay, Ali; Atabay, Berna; Kaya, Zuhre; Soker, Murat; Karadas, Nihal Ozdemir; Ozbek, Ugur; Selcuk, Bilge Ozsait; Ozdemir, Hamiyet Hekimci; Uygun, Vedat; Karasu, Gulsun Tezcan; Yilmaz, SebnemBackground Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation is the most frequently observed genetic defect in the registries from North America and Western Europe. However, in eastern countries where consanguineous marriages are common, autosomal recessive forms might be more frequent. Method Two hundred and sixteen patients with severe congenital neutropenia from 28 different pediatric centers in Turkey were registered. Results The most frequently observed mutation was HAX1 mutation (n = 78, 36.1%). A heterozygous ELANE mutation was detected in 29 patients (13.4%) in our cohort. Biallelic mutations of G6PC3 (n = 9, 4.3%), CSF3R (n = 6, 2.9%), and JAGN1 (n = 2, 1%) were also observed. Granulocyte colony-stimulating factor treatment was given to 174 patients (80.6%). Two patients died with infectious complications, and five patients developed myelodysplastic syndrome/acute myeloblastic leukemia. The mean (+/- mean standard error) follow-up period was 129.7 +/- 76.3 months, and overall survival was 96.8% (CI, 94.4-99.1%) at the age of 15 years. In Turkey, severe congenital neutropenia mostly resulted from the p W44X mutation in the HAX1 gene. Conclusion In Turkey, mutation analysis should be started with HAX1, and if this is negative, ELANE and G6PC3 should be checked. Because of the very high percentage of consanguineous marriage, rare mutations should be tested in patients with a negative mutation screen.Öğe Immunodeficiency in a Child with Alstrom Syndrome(Springer India, 2018) Ozdemir, Taha Resid; Karaca, Neslihan Edeer; Marshall, Jan Davis; Kutukculer, Necil; Aksu, Guzide; Ozgul, Riza Koksal; Ozanturk, Aysegul; Isik, Esra; Akgun, Bilcag; Ozdemir, Hamiyet Hekimci; Darcan, Sukran; Özkınay, Ferda; Cogulu, OzgurÖğe Influence of Paroxysmal Nocturnal Hemoglobinuria Clone Positivity on Outcome of Childhood Acquired Aplastic Anemia: A Multicenter Center Study(Amer Soc Hematology, 2018) Cangul, Sule Unal; Karapinar, Deniz Yilmaz; Erdem, Arzu Yazal; Yarali, Husniye Nese; Ozdemir, Hamiyet Hekimci; Gumruk, Fatma; Cakmakli, Hasan Fatih; Ince, Elif Unal; Ozdemir, Gul Nihal Nihal; Gokce, Muge; Celkan, Tiraje; Bahadir, Aysenur; Bayhan, Turan; Oren, Hale; Gulen, Huseyin; Kupesiz, Funda Tayfun; Cetin, Mualla; Ozbek, NamikÖğe Management of a Patient With Congenital Biallelic CSF3R Mutation With GM-CSF(Lippincott Williams & Wilkins, 2020) Karapinar, Deniz Yilmaz; Ozdemir, Hamiyet Hekimci; Akinci, Burcu; Yasar, Akkiz Sahin; Sivis, Zuhal Onder; Onay, Huseyin; Özkınay, FerdaSevere Congenital Neutropenia (SCN) is a rare inherited disease characterized by an absolute neutrophil count (ANC) lower than 500/mu L. Genetic heterogeneity and biallelic CSF3R mutation has rarely been identified as an underlying genetic defect in SCN. the majority of SCN patients respond to granulocyte colony stimulating factor treatment; however, in patients with inherited CSF3R mutation, ANC cannot generally be increased with granulocyte colony stimulating factor treatment. in such cases, granulocyte macrophage colony stimulating factor presents as an effective treatment option. Herein, we report a case of a 5-year-old SCN girl with homozygous c610-611 del ins AG (p.Q204R) mutation in the CSF3R gene, who was successfully treated with granulocyte macrophage colony stimulating factor.Öğe Therapeutic plasma exchange in critically ill children: A single center experience(Wiley, 2024) Ozkaya, Pinar Yazici; Koc, Gulizar; Ersayoglu, Irem; Cebeci, Kuebra; Ozdemir, Hamiyet Hekimci; Karadas, Nihal; Karapinar, Deniz YilmazIntroduction: Therapeutic plasma exchange (TPE) is used in a wide spectrum of diseases in critically ill pediatric patients. We aim to review the indications, complications, safety, and outcomes of critically ill children who received TPE. Methods: All of the TPE procedures performed in a pediatric intensive care unit providing tertiary care during 19 years (January 2013-January 2023) were evaluated retrospectively. A total of 154 patients underwent 486 TPE sessions. Results: Median age was 6 years (2-12.5) and 35 children had a body weight of <10 kg (22.7%). Number of organ failure was 4 (2-6). Liver diseases were the most common indication for TPE (31.2%) followed by sepsis with multiorgan dysfunction syndrome (27.3%). Overall survival rate was 72.7%. The highest mortality was observed in hemophagocytic lymphohistiocytosis group. Non-survivors had significantly higher number of organ failure (p < 0.001), higher PRISM score (p < 0.001), and higher PELOD score on admission (p < 0.001). Adverse events were observed in 68 (13.9%) sessions. Hypotension (7.8%) and hypocalcemia (5.1%) were the most frequent adverse events. Conclusion: TPE is safe for critically ill pediatric patients with experienced staff. Survival rate may vary depending on the underlying disease. Survival decreases with the increase in the number of failed organs.
