Intestinal mucus accumulation in a child with acutemyeloblastic leukemia

Intestinal mucus accumulation is a very rare situation observed in some solid tumors, intestinal inflammation, mucosal hyperplasia, elevated intestinal pressure, and various other diseases. However, it has never been described in acute myeloblastic leukemia. The pathogenesis of intestinal mucus accumulation is still not clear. Here, we report a 14-year-old girl with acute myeloblastic leukemia and febrile neutropenia in addition to typhlitis. She was also immobilized due to joint contractures of the lower extremities and had intestinal mucus accumulation, which was, at first, misdiagnosed as intestinal parasitosis. We speculate that typhlitis, immobilization and decreased intestinal motility due to usage of antiemetic drugs might have been the potential etiologic factors in this case. However, its impact on prognosis of the primary disease is unknown

Acute massive myelofibrosis with acutelymphoblastic leukemia

Acute myelofibrosis is characterized by pancytopenia of sudden onset, megakaryocytic hyperplasia, extensive bone marrow fibrosis, and the absence of organomegaly. Acute myelofibrosis in patients with acute lymphoblastic leukemia is extremely rare. We report a 4-year-old boy who was diagnosed as having acute massive myelofibrosis and acute lymphoblastic leukemia. Performing bone marrow aspiration in this patient was difficult (a “dry tap”), and the diagnosis was established by means of a bone marrow biopsy and immunohistopathologic analysis. The prognostic significance of acute myelofibrosis in patients with acute lymphoblastic leukemia is not clear

Posterior reversible leukoencephalopathy syndrome in children with hematologic disorders

Objective: Posterior reversible leukoencephalopathy syndrome (PRES) is characterized by headache, altered mental status, cortical blindness, and seizures associated with neuroradiological findings. It involves predominantly white matter of the parieto-occipital lobes. Several medications and disorders play a role in the etiology of PRES. In this study, we aimed to show how the prognosis of PRES in hematological diseases of childhood might be according to the etiological factors.Materials and Methods: Here, we report PRES in six patients, aged 4 to 14 years, with diagnoses of leukemia and aplastic anemia. Results: Suggested causes in our patients were chemotherapeutics, hypertension, infection and antimicrobial drug administration, tumor lysis syndrome, acute renal failure and hemodialysis, immunosuppressive drug administration, and hypomagnesemia. One of the patients died of sepsis, renal failure and pulmonary hemorrhage and another died of relapse after total recovery from PRES. The other four patients are under follow-up without problems. Conclusion: We suggest that PRES can recover fully with early diagnosis and treatment whereas it can show poor prognosis depending on the etiology

Children with Behçet Disease-associated Thrombosis: A Single-Center Experience

Behçet disease (BD) is a systemic vasculitis that can be complicated with thrombosis, which is an important cause of mortality and morbidity. The course of BD is more severe, and the diagnosis is usually delayed. In children, thrombosis associated with BD is very rare. In this study, we aimed to evaluate the characteristics of children with BD complicated with thrombosis. Forty-six patients with BD who were followed-up at a pediatric rheumatology department between January 2012 and September 2019 were evaluated retrospectively. Thrombosis was detected in 10 patients (21.7%), and it was the first sign of BD in 7 patients. Four patients had cerebral sinus venous thrombosis, 4 patients had deep-vein thrombosis, 1 patient had renal vein thrombosis, 1 had pulmonary artery thrombosis, and 1 had intracardiac thrombosis. None of the patients had arterial thrombosis. All patients had received anticoagulant therapy with immunosuppressive treatment. Any complication due to anticoagulant therapy was not detected. One patient had recurrent thrombosis, and none of the patients died during follow-up. Vasculitic diseases such as BD may cause a predisposition to thrombosis, and thrombosis might be the first sign of BD. Therefore, in children presenting with unprovoked thrombosis, BD should also be investigated. © 2021 Lippincott Williams and Wilkins. All rights reserved

Evaluation of Leukemia and Solid Tumors in Refugee Children in Turkey: A Tertiary Center Experience

Cancer care is progressively became as a significant worldwide challenge. Wars can cause destructions and delays in cancer diagnosis and treatment of displaced people. Cancer cure rates need to be improved in indefensible populations such as refugees. In this study, we purposed to highlight the clinical peculiarities and outcomes of refugee children with cancer in our hospital. Our purpose was to present our findings and contribute to improve the health care for these children. Seventy one refugee pediatric patients admitted to the oncology and hematology units of our hospital between April 2011 and January 2019 were included in this study. The demographic characteristics of the patients at the initial diagnosis, their countries of origin, living conditions, histopathological diagnoses, treatments, relapse, and mortality data were analyzed retrospectively from the patient files. The median age of patients was 6.5±4.5 years, and the male-to-female ratio was 39/32. While 44 patients (61.9%) presented with complaints and had primary diagnoses in our hospital, the remaining 27 patients (38.1%) were diagnosed in their country and applied to our hospital for treatment. Our mean follow-up period was 18.2±18.8 months (1-90 months). As a result, 44 patients (62%) were alive and 22 (31%) were dead. The survival rate without relapse in the second year was 83.6%. Two and fiveyear survival rates were 77.5% vs. 58.1% respectively. Compared to Turkish children, lower survival rates were found in refugee children. In addition to cancer-specific factors such as tumor type and stage, some problems such as shelter, communication, adherence to treatment, and difficulties supplying medicine may be responsible for lower survival rates in refugee children. Further studies are needed to improve the survival rates of patients

Research On Antıoxıdatıve And Cytoprotectıve Effects Of Phenolıc Antıoxıdants Agaınst Xhantıne/Xhanthıne Oxıdase-Induced Cellular Stress And Toxıcıty In Cardıomyocyte Cultures In Vıtro

Hipoksi-yeniden oksijenlenme sürecinde kalpte ksantin oksidaz enziminin aktive olması sebebiyle güçlü oksidan süperoksit anyonu üretimininin arttığı gösterilmiştir. Süperoksit anyonu oluşturucu ksantin/ksantin oksidaz sistemlerin kardiyomiyosit ölümünü tetiklediği ve iskemik kalp hastalıklarına zemin oluşturduğu bilinmektedir. X/XO ın kardiyak etkilerini ve polifenolik bileşiklerin koruyucu özelliklerinin mekanizmalarını daha iyi anlayabilmek için, bu tezde X/XO ile indüklenen oksidatif stres modelinde, iki farklı zeytin yaprağı kuru ekstresinin ve zeytin yaprağı polifenolik bileşiklerinin (oleuropein, kersetin ve hidroksitirozol) hücre canlılığı, reaktif oksijen türevi (ROS) oluşumu ve sağkalım-ölüm süreçlerinde rol oynayan hücre içi sinyal proteinleri düzeyindeki etkilerini H9c2 kardiyomiyositlerde araştırdık. H9c2 hücrelerinin X/XO ile uyarılması ROS oluşumunu arttırdı (DCFDA deneyi) ve hücre canlılığını (MTT deneyi) inhibe etti. Her bir ekstrenin ve polifenolik bileşiklerin farklı konsantrasyonları ile ön inkübasyon, X/XO aracılı ROS oluşumunu ve hücre canlılığını anlamlı olarak inhibe etti. Western blot analizleri, MAPKAPK-2 ve p44/42-MAPK ın fosforile formlarının ve cl-caspase-3 düzeylerinin X/XO muamelesi ile arttığını ve fosforile c-jun ve Hsp27 in düzeylerinin ise azaldığını gösterdi. Estreler, kersetin ve hidroksitirozol X/XO ile indüklenen p-MAPKAPK-2 ve cl-caspase-3 düzeylerini iyileştirdi. Oleuropein, X/XO ile indüklenen p-MAPKAPK-2 düzeylerini inhibe ederken, cl-caspase-3 düzeylerini anlamlı olarak etkilemedi. Hidroksitirozol p-c-Jun düzeylerinde X/XO ile oluşan azalmayı arttırırken, Hsp27 ve p44/42-MAPK (ERK1/2) nin fosforile formlarının indüksiyonuna neden oldu. Bu çalışma sonuç olarak oleuropein, hidroksitirazol ve kersetin içeriği yüksek polifenolik zeytin yaprağı ekstrelerinin, X/XO ile indüklenen oksidatif strese karşı hücre canlılığını iyileştirerek, ROS oluşumunu önleyerek ve sağkalım-ölüm süreçlerinde rol oynayan özgün proteinlerin fosforile formlarının ifadelenme düzeylerini değiştirerek kardiyomiyosit hücrelerini koruduğunu göstermiştir.The superoxide anion generating xanthine/xanthine oxidase (X/XO) system has been shown to induce cardiomyocyte death, which is a hallmark of ischemic heart disease. To better understand the exact mechanism of the action of X/XO and the effects of polyphenols, in this thesis we investigated the molecular action mechanism (s) and involved signaling pathways in the treatment of X/XO-induced oxidative stress with two different olive leaf extract mixtures and olive leaf polyphenolic compounds, oleuropein, quercetin and hydroxytyrosol in cardiomyocyte cell line. The stimulation of H9c2 cells with X/XO evoked ROS generation and inhibited the viability of cells. The preincubation with each extract, oleuropein, quercetin or hydroxytyrosol partially improved the viability and completely inhibited the generation of intracellular ROS in X/XO exposed cells. Western blot analysis showed that X/XO led to an increase in p-MAPKAPK-2, phospho-p44/42-MAPK and cl-caspase-3, but a decrease in the phosphorilation of p-c-Jun and p-Hsp27. Preincubation with each extract, quercetin or hydroxytyrosol partially ameliorated p-MAPKAPK-2 and inhibited cl-caspase-3 induced by X/XO. Oleuropein while inhibited p-MAPKAPK-2, had no effect on cl-caspase-3 in X/XO exposed cells. Hydroxytyrosol also increased down regulation of a transcriptional target p-c-Jun and led to overexpression in a protective stress-sensitive protein phospho-p44/42-MAPK (ERK1/2) and p-Hsp27 in X/XO exposed cells. We concluded from the results of our experiments that olive leaf extracts including high polyphenol content with oleuropein, hydroxytyrosol and quercetin provide cardiomyocyte cell protection activity against X/XO-induced oxidative challenge through recovery and good maintenance of cell viability, inhibiting ROS production and modulation of signaling proteins related with cell survival and death