Amaç: Langerhans hücreli histiositoz (LHH), tüm organ ve sistemleri etkileyebilen ve çok çeşitli klinik bulgulara neden olabilen nadir bir hastalıktır. Tedavi ve prognoz organ tutulumu bölgesine ve riskine göre değişkenlik gösterir. Bu çalışmada, kliniğimizde LHH tanısıyla takip edilen hastaları değerlendirmeyi amaçladık. Gereç ve Yöntemler: Çalışmada Ocak 2000 ile Aralık 2019 arasında LCH tanısı alan 24 hastanın verileri geriye dönük olarak incelendi. Hastalar tanı anındaki yaş, cinsiyet, başvuru semptomları, organ tutulumu, tedaviler, takip süresi ve tetavi yanıtları açısından değerlendirildi. Bulgular: Hastaların tanı yaşı ortalaması 4,7±4,6 (0,6-16,6) yıl ve ortalama takip süresi 91,6±67,8 (12,1-240) ay idi. Tanı anındaki en sık görülen bulgu cilt lezyonları iken, en sık görülen organ tutulumları kemik ve ciltti. Bunu sırasıyla hipofiz, karaciğer, lenf nodu, akciğer, dalak, periodontal bölge, tiroid, orbital bölge, mastoid, pankreas ve kemik iliği izledi. Hastalara risk gruplarına göre LCH-III çalışma protokolüne uygun olarak tedavi uygulandı. Progresyon gösteren 6 hastanın 5’ine kladribin tedavisi, 1 hastaya hematopoetik kök hücre nakli (HKHN) uygulandı ve hepsinde tam yanıt elde edildi. Sonuç: LHH’de tutulan organ ve sistemlere, hastalığın yaygınlığına göre kemoterapi, radyoterapi ve cerrahi tedavi seçenekleri farklı kombinasyonlarda uygulanmaktadır. Yanıtsızlık veya progresyon durumlarında bizim serimizde olduğu gibi kladribin ve HKHN tedavileri başarılı bir şekilde uygulanmaktadır.
Objective: Iron is an element, which is found in the structure of antioxidant enzymes and has an important role in the inactivation of reactive oxygen species. Disruption of oxidant-antioxidant balance may be playing a role in the pathogenesis of iron deficiency anemia (IDA). Dynamic thiol-disulfide homeostasis (DTDH) and serum ischemia-modified albumin (IMA) levels are important indicators of pro-oxidant/antioxidant status. In this study, we aimed to evaluate DTDH parameters and serum IMA levels in children with newly diagnosed IDA, who did not receive iron therapy. Material and Methods: Fifty patients diagnosed with IDA and 33 healthy age- and sex-matched control patients were included in the study. DTDH parameters and IMA levels of the patients and control groups were measured. The same parameters were also compared in patients with Hb<7 g/dl (profound IDA) (n:14/50, 28%) and Hb≥7 g/dl (mild-moderate IDA) (n: 36/50, 72%) in the IDA group. The relationship between DTDH parameters in these groups were investigated. Results: Native thiol, total thiol, native thiol/total thiol levels, constituting antioxidant capacity indicators, were found to be significantly lower in IDA patients; while oxidant disulfide, disulfide/native thiol, disulfide/total thiol, and IMA levels were found to be statistically higher compared to those in the control group (p<0.050). When DTDH parameters and IMA levels were examined; there was a positive correlation between antioxidant parameters and a negative correlation between oxidative parameters with hemoglobin and ferritin levels (p<0.050). Also, oxidative parameters were found to be much higher in profound IDA group than in the group with Hb>7 g/dl (p<0.050). Conclusion: In this study, increase in serum disulfide and IMA levels with the decrease in serum native thiol and total thiol levels indicated oxidative stress in IDA patients before treatment, compared to the control group. Evaluation of these indicators in children is important in predicting the toxicity due to IDA.
Background. Heavy menstrual bleeding (HMB) is a common condition in adolescents. However, bleeding disorders are known to be one of the causes of HMB in adolescent girls, so they should be considered. Simple methods that can be used in primary health care are needed to determine whether patients have bleeding disorders. The aim of this study was to evaluate the bleeding score of patients admitted with HMB and to determine the diagnostic value of patients who were symptomatic but whose initial hemostatic tests were normal. Methods. A total of 113 adolescents with HMB and 20 healthy adolescent girls were included in the study. The Pediatric Bleeding Questionnaire (PBQ) and the International Society of Thrombosis Haemostasis-Bleeding Assessment Tool (ISTH-BAT) were used for evaluation. Results. Overall, approximately 18% (n= 20) of the adolescents in the study were diagnosed with a bleeding disorder. The cut off value for the `clinically significant bleeding score` was found to be 3.5. Conclusions. The PBQ and ISTH-BAT can help distinguish a significant bleeding history from an otherwise trivial bleeding and can be included in the algorithm for the primary care of adolescents with HMB with suspected bleeding disorders.
Severe acute respiratory syndrome, coronavirus (SARS, COVID-19) has been declared a pandemic by the World Health Organization since March 2020. Patients with active cancer should be considered especially for priority access to the COVID-19 vaccine. Therefore, our study aimed to learn parents’ opinions of cancer-diagnosed patients about the COVID-19 vaccine. Between December 2021 and January 2022, 76 people were willing to answer the questionnaire from the parents of outpatient/inpatient patients at the Pediatric Hematology and Oncology Clinic of Dr. Sami Ulus Obstetrics, Child Health and Diseases Education Research Hospital were included. In our study, 18 parents (23.7%) did not get vaccinated. Among the reasons for not getting vaccinated, the most common answer for “I fear from the vaccine’s side effects.”, “Do you think oncology patients are at risk for COVID-19?”, “Do you think the COVID-19 pandemic affects cancer treatment?” were 90.7%, 89.5% and 21% yes, respectively. Would your opinion of the vaccine be positive if the domestic vaccine was produced?”—67.4% answered yes. Vaccine hesitation and public misinformation put cancer patients at risk. Increasing awareness of the rejection of COVID-19 vaccines is important for public health and the fight against the pandemic. In addition, the doctors’ recommendations for conducting oncology treatment will significantly impact parents’ compliance with the COVID-19 vaccine.
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