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Objectives: This study aims to analyze the growth parameters in children with familial Mediterranean fever (FMF) according to disease characteristics including genotype and disease severity by a recently validated tool in relatively more patients. Patients and methods: This retrospective study included 126 patients with FMF (70 males, 56 females; mean age 7.3±3.6 years; range, 4.1 to 18 years). MEditerranean FeVer (MEFV) gene analysis was performed with a molecular diagnostics tool by using a next-generation sequencing platform. Disease severity was determined for the first visit by the validated tool in children, international severity scoring system for FMF. Growth parameters including weight and height were investigated after standard deviation (SD) scores were calculated by anthropometric references in Turkish children. Results: Median follow-up duration was 74.7 months (range, 7.5 to 169 months). Ninety-three patients (73.8%) had at least one M694V mutation in MEFV gene. Six patients (4.8%) had severe disease, 58 (46%) had intermediate severity, and 62 (49.2%) had mild disease. Mean height SD score was significantly lower at last visit than before colchicine treatment. Initial and last height and weight SD scores were lower in patients with at least one M694V mutation than those without. However, the difference was statistically significant for only initial height SD score. We also found statistically significant lower initial height, final height, and weight SD scores in patients with intermediate severity-severe disease activity than mild disease. Conclusion: We advise physicians to score disease severity prospectively and pay attention to patients with intermediate severity-severe disease to avoid growth disturbances.

Congenital Ewing sarcoma is extremely rare. Here we present a newborn baby born with a mass on the left shoulder. Immunohistochemical staining showed congenital Ewing sarcoma. Chemotherapy and then surgical operation were planned, however the patient died before initiation of chemotherapy on the 30th day of life.

Background: Patients with congenital heart disease (CHD) are more prone to thromboembolism. Aspirin is the most commonly used medication around the world to prevent thrombosis in cardiovascular diseases. Objectives: This study aimed to define the frequency of aspirin resistance in pediatric patients with CHD and to evaluate the correlation of clinical and laboratory parameters with aspirin resistance. Methods: The study population consisted of 103 patients using aspirin, including 53 cases of cyanotic CHD and 50 cases of non-cyanotic CHD. Platelet aggregation was measured by the AggreGuide A-100 ADP Assay. Results: The prevalence of aspirin resistance was 36.9% in children with CHD. Although aspirin resistance in cyanotic CHD patients (41.5%) was higher than in non-cyanotic patients (32%), the difference was not statistically significant (P = 0.414). There was no significant association between aspirin responsiveness or resistance and the patient’s sex, age, duration of aspirin use, and concomitant medication use. Comparison of the laboratory data of aspirin-responsive and -resistant patients showed no significant difference between these groups, except for albumin (P = 0.032) and serum fibrinogen (P = 0.0001) levels. The fibrinogen level and thromboembolism history were independent risk factors for aspirin resistance. Also, there was a significant correlation between platelet aggregation in peripheral blood smears and aspirin resistance (P = 0.0001). Conclusions: Our findings suggest that measurement of the serum fibrinogen level and platelet aggregation in blood smears may be the first step to predict aspirin resistance.