Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies.

INTRODUCTION: A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. AIM: Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. METHODS: A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. RESULTS: In our cohort (N = 449; 215 male, 234 female), the higher prevalence of mucocutaneous bleeds in females strongly predicted ensuing gynaecological bleeding (hazard ratio = 12.8, 95% CI 1.68-97.6, P = 0.014). Menorrhagia was the most prevalent type of bleeding (46.4% of patients), and was the presentation symptom in 12% of cases. Replacement therapies administered were also analysed. For surgical procedures (n = 50), a receiver operator characteristic analysis showed that the minimal first dose of rFVIIa to avoid postsurgical bleeding during the first 24 hours was 22 µg kg(-1) , and no less than two administrations. Prophylaxis was reported in 25 women with excellent or effective outcomes when performed with a total weekly rFVIIa dose of 90 µg kg(-1) (divided as three doses). CONCLUSION: Women with FVII deficiency have a bleeding disorder mainly characterized by mucocutaneous bleeds, which predicts an increased risk of ensuing gynaecological bleeding. Systematic replacement therapy or long-term prophylaxis with rFVIIa may reduce the impact of menorrhagia on the reproductive system, iron loss and may avoid unnecessary hysterectomies.

Social determinants and health-related dimensions of quality of life in adult patients with haemophilia.

The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with health-related dimensions, in Tabriz, Iran. The survey instrument was a self-report 36 items questionnaire, 'A36 Hemofilia - QoL', which is a disease-specific questionnaire for the assessment of the health-related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score was 71.88 (±26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.

Human papilloma virus in head and neck squamous cell cancer.

BACKGROUND: Epidemiologic and molecular evidences have established a strong link between high risk types of Human Papilloma Virus and a subgroup of Head and Neck Squamous Cell Carcinomas (HNSCC). We evaluated the frequency of HPV positivity in HNSCC and its relationship to demographic and some risk factor variables in an open case- control study. METHODS: Fourteen recently diagnosed patients with squamous cell cancer of oropharynx, hypopharynx and larynx aged 18-50 years were examined from 2008-2010 in Tabriz, Iran. HPV DNA was extracted from paraffin-embedded blocks of each patient's sample for PCR evaluation. Saliva samples of 94 control cancer-free subjects were collected for DNA analysis. Multivariable logistic regression method was used to calculate odds ratio for case-control comparisons. RESULTS: High risk HPV was detected in 6(42.8%) patients, and 6(5.3%) control subjects which was statistically significant (p<0.0001). HPV-18 was the most frequent type both in the cases and controls. HPV-16 DNA was detected in two patients of the case group, but it was not detected in any of the controls. The relation between demographic and risk factor variables was not statistically significant. CONCLUSION: HPV infection has a significant impact on HNSCC. Despite HPV-16 stronger impact, HPV-18 is more likely to cause malignant degeneration in such cancers amongst some communities. It is vital to introduce and conduct immunization schedules in health care systems to protect communities to some extent.

Evaluation of Copper, Zinc, Cu/Zn, and VEGF in Patients with AML in Iran.

BACKGROUND: Copper and zinc are the elements with numerous physiological activities. Copper (Cu) has an important role in angiogenesis and acts by increasing Vascular Endothelial Growth Factor (VEGF). Serum levels of copper will be increased in cancer incidence, progression and recurrence. The aim of this study was to measure blood levels of copper, zinc, and the ratio of Cu /Zn, as well as VEGF levels before and after treatment of acute myeloid leukemia. METHODS: Thirty patients who were recently diagnosed with Acute Myeloblastic Leukemia (AML) in Shahid Ghazi Tabatabai oncology hospital enrolled in this clinical trial. On the first day, blood samples were taken for copper, zinc, and VEGF assay and flowcytometry. Treatment protocol was (7×3) regimen. Blood samples were collected for evaluation of copper, zinc, and VEGF. They were sent to Biochemistry Laboratory in medicine faculty for analysis. RESULTS: Amongst 30 AML patients, 14 (46.7%) were female and 16 (53.3%) were male. Patients of various ages ranged from 16 to 53 years, with a median age of 9.1±9.35 years. The mean serum level of copper, zinc, and mean Cu/Zn ratio before and after treatment showed significant difference (p<0.05) There was also significant difference between the mean VEGF level before and after treatment (p<0.05). CONCLUSION: This study reveals that there is no significant relationship between copper, zinc serum levels, their ratio, and VEGF in AML patients. We hypothesize that increased serum copper is associated with increase of VEGF levels which can indicate the impact of copper in malignancies including AML.