Roya Dolatkhah

Effects of music therapy on pain and anxiety in patients undergoing bone marrow biopsy and aspiration.

Bone marrow biopsy and aspiration are commonly used for diagnosing, treating, and following up after treatment for blood disorders and solid tumors. For adults, the infiltration of local anesthesia at the biopsy site has been used as the principal form of analgesia for bone marrow biopsy and aspiration. Pain relief during these procedures is often incomplete, especially during aspiration of the bone marrow, and pain is likely to contribute to patient anxiety. Researchers at the Tabriz Hematology and Oncology Center in Iran conducted a study to quantify and evaluate the effectiveness of music therapy interventions on pain and anxiety control for 100 patients undergoing bone marrow biopsy and aspiration. Participants in the study were randomly assigned to one of two groups: one group listened to music during the procedure, and the other did not. Patients completed the Spielberger State-Trait Anxiety Inventory both before and after the procedure and reported pain severity by using a visual analog scale. Results showed that participants who listened to music had lower state anxiety and pain levels than those who did not listen to music.

Colorectal Cancer in Iran: Molecular Epidemiology and Screening Strategies

Purpose. The increasing incidence of colorectal cancer (CRC) in the past three decades in Iran has made it a major public health burden. This study aimed to report its epidemiologic features, molecular genetic aspects, survival, heredity, and screening pattern in Iran. Methods. A comprehensive literature review was conducted to identify the relevant published articles. We used medical subject headings, including colorectal cancer, molecular genetics, KRAS and BRAF mutations, screening, survival, epidemiologic study, and Iran. Results. Age standardized incidence rate of Iranian CRCs was 11.6 and 10.5 for men and women, respectively. Overall five-year survival rate was 41%, and the proportion of CRC among the younger age group was higher than that of western countries. Depending on ethnicity, geographical region, dietary, and genetic predisposition, mutation genes were considerably diverse and distinct among CRCs across Iran. The high occurrence of CRC in records of relatives of CRC patients showed that family history of CRC was more common among young CRCs. Conclusion. Appropriate screening strategies for CRC which is amenable to early detection through screening, especially in relatives of CRCs, should be considered as the first step in CRC screening programs.

Increased colorectal cancer incidence in Iran: a systematic review and meta-analysis.

BackgroundColorectal cancer is the third most common cancer in Iran. The increasing trend of colorectal cancer incidence in Iran and the close relationship with the geographical location are the underlying reasons for this study.MethodsData source: Eleven databases, including MEDLINE, EMBASE, SCOPUS, and four other databases, for articles in Persian were searched from April 2014 to October 2014. Additional data were obtained from an online survey of the Central Library of Tabriz Faculty of Medicine. Study eligibility criteria: In this systematic review and meta-analysis, we included studies reporting different measures of incidence, age-standardized incidence rates, and crude incidence rates. All rates (per 100,000 person-years) were standardized to the world standard population. Study appraisal and synthesis methods: A preliminary review of the title and abstracts of these articles was used to exclude any that were clearly irrelevant. The full text review determined whether the article was relevant to our topic. All the potentially relevant manuscripts were reviewed by two other investigators (S.D., M.G.). A total of 39 studies (10 Persian and 29 English articles) from different provinces and diverse areas of Iran, were analyzed in this study using comprehensive meta-analysis software. For accuracy studies, we used estimated rates for males and females with 95 % confidence intervals.ResultsAge-standardized incidence rates were obtained based on the random effects model and were 8.16 (95 % CI: 6.64 to 9.68) and 6.17 (95 % CI: 5.01 to 7.32) for males and females, respectively. The random crude rates were 5.58 (95 % CI: 4.22 to 6.94) for males and 4.01 (95 % CI: 3.06 to 4.97) for females.ConclusionsColorectal cancer incidence rates rise due to individual and environmental risk factors as well as improvement in the registry system and increase in access to health services. A more executed organized and structured system for collecting cancer data, in all cities and rural areas of the country, is an essential priority.

Inherited coagulation disorders in the northwestern region of Iran

According to the global survey carried out by World Federation of Haemophilia, Iran with 3463 haemophilic patients was ranked as the second in the eastern Mediterranean region next to the Egypt having 4141 cases of haemophilia. Of this 58 patients are human immunodeficiency virus (HIV) positive and 3198 are infected with hepatitis C virus. The prevalence rate of disease was estimated to be about 5.4 per 100 000 people [1]. The aim of this study was to document the epidemiological features, disease severity and complications associated with inherited coagulation disorders as focused in the northwestern region of Iran. Medical history of all cases (174 persons) diagnosed with inherited coagulation disorders were studied in Haematology–Oncology Department, Tabriz University of Medical Sciences between 1994 and 2004. This department is the unique adult centre in East Azerbaijan (the northwestern part of the country) providing clinical services for adult haemophilic patients. All subjects were diagnosed based on the criteria of the Iranian Registry of Coagulation Disorders and confirmed with haemophilia, von Willebrand disease (VWD) or other coagulation factors deficiency. Disorders were classified as mild, moderate or severe according to the level of plasma clotting factor: 5–25%, 1–5% and <1% respectively [2]. Data collected includes age, clinical characteristics, resident status and type of disorders. Standard enzyme immunoassays were applied for the diagnosis of hepatitis B virus, hepatitis C virus (HCV) and HIV. Rates of prevalence, 95% confidence interval (CI) and descriptive statistics were calculated to document the epidemiological features of the disorders in the region. Total population by age groups was obtained from the Demographic Centre for the East Azerbaijan, and used as denominator in computing prevalence rates. The basic characteristics for the subjects of the study are presented in Table 1. The age of the patients in all groups of disorders ranged between 10 and 66 years as averaged 26.7 with standard deviation of 10. Nearly half of the subjects were located in Tabriz, one of the major cities of Iran, which represents the proportionally normal distribution of the study sample in the population of the region. No female haemophilic patient was identified among the subjects within the 10 years of study from 1994 to 2004. The majority of the subjects (69%) suffered from haemophilia A, followed by 11% haemophilia B and 10% VWD. Other types of disorders were included in the remaining 10%. Table 2 shows the prevalence of inherited coagulation disorders by age groups. Total prevalence of disorders was estimated 5.7 per 100 000 populations (95% CI: 4.8–6.5) in the area. The highest prevalence of disorders was shown to fall in 20–29 years of age group (9.6 per 100 000 people, 95% CI: 7.6–12.0) while the lowest rate was observed among those with 40–49 years of age (2.7 per 100 000 people, 95% CI: 1.4–4.9). The prevalence rate of disorders in those with more than 49 years of age was 2.8 per 100 000 people (95% CI: 1.7–4.5) as described in Table 2. Correlation between age group and the prevalence of inherited coagulation disorders was shown to be significant. Table 3 describes the infectious status of patients. As of this data, only two patients were tested positive for Hbs-Ag when compared with 74 who were negative among haemophilia A patients. Meanwhile, 41% was infected with HCV. Eight haemophilia B subjects (44%) were Hbs-Ag negative, and seven cases (39%) had positive result of HCV. A total of Correspondence: Dr Jamal Eivazi Ziaei, Shahid Ghazi Department of Oncology and Hematology, Tabriz University of Medical Sciences, Tabriz 51665-139, Iran. E-mail: jeziaei@yahoo.com

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.

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

A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency.

Upper gastrointestinal bleedings in patients with hereditary coagulation disorders in Northwest of Iran: prevalence of Helicobacter pylori infection.

Objective Upper gastrointestinal (UGI) bleeding is one of the most life-threatening complications, in up to 25% of persons with hemophilia (PWH). Recurrent bleeding is common and can be caused by the Helicobacter pylori infection. Our aim was to evaluate the role of H. pylori infection in UGI bleeding in PWH. Material and methods Ninety patients with hereditary bleeding disorders, 30 patients with (group A), and 60 patients without (group B) a history of UGI bleeding episodes were included. The prevalence of H. pylori infection was investigated by stool antigen test, and serum serologic tests including immunoglobulin G and anti-CagA. Results Among 90 patients (81 men, nine women, mean age 31.30±10.72 years), 66 patients with hemophilia A, 10 patients with hemophilia B, six patients with Von Willebrand disease, five patients with platelet function disorders, and three patients with other factor deficiencies were evaluated. About 46.7% of patients in group A, and 23.3% of patients in group B were anti-CagA–positive (P=0.02), whereas 76.7% of patients in group A and 51.7% of patients in group B had H. pylori immunoglobulin G antibodies (P=0.02). H. pylori antigen in stool was positive in 76.7% in group A and 55% in group B (P=0.03). No statistically significant difference was found between type and severity of diseases and risk of UGI. Conclusion H. pylori infection should be considered as an important cause of UGI bleeding in PWH. We would recommend stool antigen test as a new and noninvasive screening test for diagnosis of H. pylori infection in all patients with hereditary hemorrhagic disorders.

SUCCESSFUL MANAGEMENT OF TOTAL KNEE REPLACEMENT IN A HIGH RESPONDER HEMOPHILIA PATIENT WITH A HISTORY OF INHIBITOR

The development of inhibitors against administered clotting factors may render replacement therapy ineffective for some hemophilia patients. Such patients are therefore at the highest risk of developing arthropathy. Elective orthopedic surgery (EOS) in hemophilic patients having such inhibitors remains a rare, expensive, and difficult surgery, whose management represents a significant challenge. We report the case of a 35-year-old man with a severe form of hemophilia A (factor VIII < 1%), who was suffering from repetitive spontaneous hemarthrosis, especially in his knee joints that had consequently become more susceptible to bleeding. The patient had a history of high levels of factor VIII inhibitor (> 5.0 Bethesda Unit [BU]/ml) as shown by the factor VIII inhibitor assay; therefore, we began treatment with factor VIIa for his mild-to-moderate bleeding (90 µg/kg intravenous bolus injections). The interval between injections varied with the severity of the hemorrhage in each bleeding episode. The inhibitor level reduced to 3.1 BU/ml after three months, to 1.6 BU/ml after six months, and disappeared completely after one year of treatment. We administered factor VIII at a dose of 50 IU/kg every eight hours during the first three post-operative days, then continued administration with a dose of 40 IU/kg every 12 hours for another four days, and observed a very good response to treatment with no bleeding. Recombinant activated factor VII (rFVIIa) is not an inhibitor-removal strategy, but an inhibitor-bypassing product. However, in our patient, the treatment of mild-to-moderate bleeding with short-term use of rFVIIa and no exposure to factor VIII caused a gradual reduction in the inhibitor level over a period of 1 year.

Delayed hemolytic transfusion reaction with multiple alloantibody (Anti S, N, K) and a monospecific autoanti-JK b in intermediate β-thalassemia patient in Tabriz

It appears that delayed hemolytic transfusion reactions may occur several days after the administration of donor red cells is true even though they have been shown to be compatible in cross match tests by the antiglobulin technique. A specific case was observed in our center, which confirms the fact. The patient was a 37-year-old male suffering from intermediate β-thalassemia. He had a history of two previous transfusions, with unknown transfusion reaction. In the last transfusion, laboratory data showed: Hb 7.8 g/dL and Hematocrit (Hct) 24.2%. The patient received two units of cross matched, compatible concentrated red blood cells (RBCs). After eight days a severe reaction was observed with clinical evidence of tachycardia, fatigue, fever, back pain, chest discomfort, jaundice, nausea and anorexia. Accordingly delayed hemolytic transfusion reaction was suspected, and anti-RBC antibodies were tested. Laboratory tests revealed the presence of three alloantibodies: Anti-N, anti-S, anti-K, and a monospecific autoanti-JKb.

A novelKRASgene mutation report in sporadic colorectal cancer, from Northwest of Iran

While the role of KRAS gene mutations has been widely accepted for predicting responses to anti‐EGFR therapy in patients with colorectal cancer, although this study was based on observation of a single case it gives hope that some KRAS gene mutation may have favorable prognosis. More studies are required on patients with similar mutation to validate this finding.