Evaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assays.

INTRODUCTION: Inherited factor VII (FVII) deficiency is the most common of the rare bleeding disorders and shows a heterogenous distribution of bleeding phenotypes independent of factor activity level. The bleeding score (BS) evaluates the phenotype of patients with rare bleeding disorders. Thromboelastography (TEG) and thrombin generation assays (TGAs) are 2 methods to evaluate global hemostasis, and controversially both tests are useful for identifying different bleeding tendency phenotypes. The purpose of this study was to investigate the use of the BS and global assays (TEG and TGAs) to predict the bleeding phenotype of inherited FVII deficiency. MATERIALS AND METHODS: A total of 27 patients with FVII deficiency were evaluated with the BS and global hemostasis assays. RESULTS: The BS was compatible with disease severity according to the FVII activity level (P<0.05) but the BS and bleeding grade of patients did not show a statistically significant correlation with factor activity level (P>0.05). No significant correlation was observed between the factor activity level and any TEG parameter (P>0.05). The factor activity level was negatively correlated with the lag time of the TGA on the contrary positively correlated with the peak thrombin time of the TGA (P<0.05). CONCLUSIONS: The global assays do not successfully predict the bleeding phenotype. The BS is a more suitable tool than conventional and global assays for predicting the bleeding phenotype.

Assessment of Self-Image With the Offer Self-Image Questionnaire in Adolescents With Hemophilia: A Single-Center Experience.

BACKGROUND: Hemophilia, which is a chronic illness associated with recurrent bleeding, may occur with psychosocial and behavioral problems. AIM: The aim of this study was to evaluate the clinical characteristics and demographic features and changes in the self-image of adolescents with hemophilia. MATERIALS AND METHODS: Data about hemophilia type, the severity of hemophilia, secondary prophylaxis received, and annual bleeding rate (ABR) were recorded from patient files. Hemophilia Joint Health Score (HJHS) and the Offer Self-Image Questionnaire (OSIQ) (as a measure of self-esteem) were applied to hemophilia patients and a healthy control group. RESULTS: Thirty-two hemophilia patients (mean age=16.2±3.06 y) and 35 healthy male individuals (mean age=16.02±1.4 y) were enrolled in the study. Hemophilia patients had lower total OSIQ score than their peers (P=0.007). There was no difference between patients who received and who did not receive secondary prophylaxis (P=0.408) in terms of total OSIQ score. The median total OSIQ score of patients with pathologic HJHS (>0 points) was lower than that of patients with normal HJHS (0 points) (P=0.010). The median of ABR was 6 (range: 0 to 20) in the whole hemophilia group. There were no differences between hemophilia patients with ABR≤4 and >4 (P=0.084). All of the subscale parameters of the OSIQ were lower for hemophilia patients compared with their peers, besides one. The subscale of sexuality attitudes was better for hemophilia patients than for the healthy control group (P=0.028). CONCLUSIONS: Low self-esteem in hemophilia patients indicates the importance of lifelong psychosocial support. Patients with pathologic HJHS are at risk of low-esteem. Using OSIQ with HJHS during follow-up of hemophilia patients may be useful for management.

Assessment of sleep in pediatric cancer patients.

Ince D, Demirag B, Karapinar TH, Oymak Y, Ay Y, Kaygusuz A, Töret E, Vergin C. Assessment of sleep in pediatric cancer patients. Turk J Pediatr 2017; 59: 379-386. The purpose of the study is to describe sleep habits, assess the prevalence of sleep disturbances in pediatric cancer patients and healthy controls, and to compare sleep patterns, sleep problems. One hundred-thirty-five patients and 190 healthy controls were evaluated. Healthy children matched for age, sex, economic status, parental education and family structure constituted the control group. Sleep was evaluated by using the Children`s Sleep Habits Questionnaire (CSHQ). Sleep problems were detected in half of patients. There were no significant differences in total sleep score and subscale scores between patients and controls. Solely the wake-time was found significantly different between patients and controls. Although our results indicated that neither childhood cancer survivors nor patients with cancer during treatment period had more sleep problems than their healthy peers, sleep problems were not uncommon in whole study group. This study underlines the need to screen, assess and manage sleep problems in children with diagnosis of cancer.