Purpose: We aimed to prospectively interpret the cardiac repolarization changes with 12-lead electrocardiography(ECG) in children with cancer who were treated with anthracycline drugs.Materials and methods: A total of 53 patients with cancer treated with anthracycline were enrolled in the study.During 6-month follow-up, standard 12-lead ECG was performed at basal, 1st, 4th, and 24th hours after thefirst dose of anthracycline treatment, at the time of 120 mg/m2 cumulative anthracycline dose and 240 mg/m2of cumulative anthracycline dose in the same patients, respectively. P dispersion (PWd), QT dispersion (QTd),corrected QT dispersion (QTcd), Tp-e interval, Tp-e/QT, and Tp-e/QTc ratio were obtained from 12-lead ECG.The patients were classified into three groups according to increasing cumulative anthracycline doses: Group 1:first dose (n=53), Group 2: 120 mg/m2 (n=53), Group 3: 240 mg/m2 (n=53).Results: The median age was 48 months (range 9-192 months). While PWd, QTd, QTcd, and Tp-e interval weresignificantly increased during first 24 hours of the first dose (p<0.001, p=0.005, p=0.041, p=0.016, respectively),Tp-e/QT and Tp-e/QTc ratios were significantly altered during first 24 hours of 120 mg/m2 cumulative dose ofanthracycline treatment (p<0.001). Any changes in 12-lead ECG were not significantly at 240 mg/m2 cumulativedose. However, it was detected that all variables were affected according to each increased anthracyclinecumulative dose despite it was not statistically significant.Conclusions: ECG parameters such as PWd, QTd, QTcd, Tp-e interval, Tp-e/QT, and Tp-e/QTc ratios areuseful for detecting subclinical cardiac abnormality and acute anthracycline toxicity during both uses of singledose anthracycline and increased anthracycline doses. These parameters may also predict arrhythmias inpatients with cancer.
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Objective: Infections are an important cause of morbidity and mortality for patients with congenital neutropenia. In the present study, we report on the incidence, type, localization of documented infections, as well as the clinical features and long-term outcome in patients with congenital neutropenia in our clinic. Method: We performed a retrospective chart review of children with neutropenia seen at our hospital from 2000-2018. The data of 15 patients with congenital neutropenia were included in this study. Clinical and laboratory data were analyzed retrospectively using patients’ files and an electronic data system. Results: The median age at diagnosis was 34 months (range, four months- 150 months) and the median follow-up time was 48 months (range, 13-179 months). The leading causes of hospital admission before the establishment of the diagnosis were upper respiratory tract infection in six, pneumonia in four, gingival stomatitis in three and soft tissue infection in two patients. We reached the documented 74 hospitalization episodes and the most common reasons for hospitalization were pneumonia (35%), fever (21%), stomatitis (16%), cutaneous and deep soft tissue infections (12%). Conclusion: The management of infectious complications in children with congenital neutropenia is crucial. Early diagnosis is essential to prevent infections and permanent organ damage. Congenital neutropenia should be suspected in patients with a history of frequent upper respiratory tract infection, and necessary investigations should be performed accordingly. However, it should be kept in mind that the clinical findings of the patients may vary despite having the same mutation.
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Tuba Hilkay KARAPINAR ,
Ersin DURGUN ,
Yeşim OYMAK , Nesrin GÜLEZ,
Yılmaz AY ,
Ferah GENEL ,
Salih GÖZMEN ,
Sultan AYDIN KÖKER , Erkin SERDAROĞLU,
Ersin TORET ,
Canan VERGİN
Background and objectives. Autoimmune cytopenias are a group of heterogeneous disorders characterized by immune-mediated destruction of one or more hematopoietic lineage cells. The differential diagnosis of children with autoimmune cytopenias requires much time and laboratory investigations. The aim of the present study was to evaluate the clinical course and significance of autoimmune cytopenias due to immunodeficiency or autoimmune diseases in children at a single children’s hospital. Method. Between February 1997 and September 2015, chronic/refractory autoimmune cytopenias patient data were evaluated retrospectively. Twenty-three patients were assessed in this study. Results. The median duration of following was 2.6 years (4 months-18.5 years). The median age of diagnosis was 3.1 years (6 months-16 years). A total of 13 patients (56.5%) had single-lineage and 10 (46.5%) had multilineage cytopenias. The most frequent single-lineage cytopenia was thrombocytopenia, followed by anemia. In 22 of the patients, cytopenias was detected before the primary diseases. All of the patients were treated with corticosteroids or intravenous immune globulin as first-line treatment. Ten patients (43.5%) needed second or further-line immunosuppressive therapies that patients diagnosed as systemic lupus erythematosus, hypogammaglobulinemia, or common variable immunodeficiency. A total of 8 patients (34.7%) recovered from autoimmune cytopenias after the treatment of primer disease. Cytopenias were continued in 14 patients. Conclusion. Cytopenia may be the first finding of an immunodeficiency or autoimmune disease and primary disease may be diagnosed in the clinical course. Taking the new targeted treatment options into consideration; early diagnosis is likely to become more important in the near-future in order to begin the treatment for the underlying disease as early as possible.
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İlknur ÇAĞLAR ,
İlker DEVRİM ,
Mine DÜZGÖL ,
Ahu AKSAY ,
Özgür CARTI ,
Bengü DEMİRAĞ ,
Salih GÖZMEN ,
Yılmaz AY ,
Tuba Hilkay KARAPINAR ,
Nuri BAYRAM ,
Yeşim OYMAK ,
Canan VERGİN
Objective: There are limited data focusing on incidence and manifestations of cytomegalovirus (CMV) inchildren with acute lymphoblastic leukemia (ALL) apart from bone marrow transplant recipients. In thisstudy, we aimed to review our experience regarding the manifestations, treatment, and outcome of cytomegalovirus infection in pediatric ALL patients.Methods: We retrospectively reviewed the clinical characteristics of patients with ALL that were diagnosed with CMV disease while they were on standard chemotherapy.Results: Fourteen patients were included in the std. Fever was the most common symptom (64%). Eyes,lungs and liver were the most commonly involved organs in CMV disease. Lymphopenia was found in mostof the patients. At the time of diagnosis, 50% of the patients were on maintenance phase of chemotherapy. All patients were treated with intravenous ganciclovir. Two patients died because of concomitantinfections, and two children with retinitis had permanent visual sequelae while others had a completerecovery.Conclusion: In children with ALL, CMV is an important pathogen with serious consequences includingretinitis which may be asymptomatic and result in complete visual loss. Not only during intense chemotherapy but also in maintenance phase CMV disease may occur. Especially when prolonged (>7 days) febrile neutropenia and lymphopenia is present, CMV must be kept in mind in the differential diagnosis.
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Type II Griscelli Syndrome (GS) is caused by a mutation in the RAB27A gene and usually manifests with silvery-gray hair, immune deficiency and the development of hemophagocytic lymphohistiocytosis (HLH). A hematopoietic stem cell transplantation is the curative treatment for HLH and reduced-intensity conditioning prevents the morbidity/mortality in the transplantation related to myeloablative conditioning. We report on a 21-month old boy with cerebral involvement of HLH related to GS.
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