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Background: Arthropathy is a common complication in patients with hemophilia. We examined the prevalence of this skeletal complication in patients with hemophilia who were registered at a Comprehensive Hemophilia Center in Shiraz, Southern Iran. Materials and Methods: In this cross-sectional study, an orthopedic specialist visited 448 patients and conducted screenings for skeletal complications. The assessment included evaluating the type of hemophilia, disease severity, treatment modality, the presence of inhibitors, and the identification of skeletal complications. Results: Ninety patients with hemophilia A, with a mean age (SD) of 31.6 (14.4) years, and 10 patients with hemophilia B, with a mean age of 30.5 (20.6) years, were assessed. The most frequently affected joints were the knee and ankle joints. In the univariate analysis, patients with severe disease were more likely to exhibit synovitis, a target joint, and bone disease compared to patients with non-severe disease. Additionally, a history of treated or active hepatitis and an annual bleeding rate showed significant associations with the target joint. In the multivariable logistic regression analysis, disease severity (OR 14.43, 95% CI 1.6–129.6) and a higher age at diagnosis (OR 1.06, 95% CI 1.00–1.13) increased the likelihood of developing osteoporosis. A history of hepatitis (OR 3.67, 95% CI 1.28–10.48) was identified as an independent risk factor for the target joint. Conclusion: Skeletal complications are a common occurrence in hemophilia. Regular consultations with orthopedic specialists, focusing on bleeding control and hepatitis prevention, are essential for reducing the impact of this debilitating complication. [ABSTRACT FROM AUTHOR]

Background: The overall incidence of breast cancer is different all over the world and even within a nation. The present study aims to investigate the stratum-specific incidence trends of breast cancer in southern Iran. Methods: In this retrospective cohort study, the data of Fars Population-Based Cancer Registry was used during 2001–2018. New cancer cases with ICD-O-3 codes C50.0 to C50.9 were categorized based on age group, morphology, and topography. Age-specific incidence rates of breast cancer were calculated during 2001–2018. Annual overall and truncated age-standardized incidence rates and their 95% Confidence Intervals (CIs) were also calculated. Afterward, the Annual Percentage Changes (APCs) of the age-specific and age-standardized incidence rates of breast cancer during 2001–2018 were calculated using Joinpoint regression software. Results: An increasing trend was observed in the incidence of breast cancer among women during 2001–2018 (APC of age-standardized incidence rates: 9.5 (95% CI: 7.5, 11.5)).However, the trend was increasing less during the recent years. The APC of age-standardized rates decreased from 15.03 (95% CI: 10.4, 19.8) in 2007 to 6.15(95% CI: 4.0, 8.4) in 2018. The most common morphology of breast cancer was invasive ductal carcinoma (77.3% in females and 75.1% in males) and its trend was similar to the general trend of different types of breast cancer. The most common site of breast cancer was the upper outer quadrant. Most breast cancer cases were female and males accounted for 2.45% of the cases. Among females, 40–55 was the most prevalent age group. Conclusion: The incidence of breast cancer among women living in southern Iran showed an increasing trend from 2001 to 2018. However, the rate of increase exhibited a milder slope during the more recent years. Based on the higher prevalence of breast cancer in the 40–55 age group observed in the present study, it offers valuable insight into the potential reduction of the breast cancer screening age from 50 to 40 years for healthy Iranian women. However, before implementing such a policy change, it is crucial to conduct additional studies that specifically examine the cost-effectiveness, as well as the potential benefits and risks associated with this alteration. [ABSTRACT FROM AUTHOR]

Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of the study: Our aim was to investigate the efficacy and side effects of the Sinopharm vaccine in patients with hemoglobinopathies in Iran and the frequency of breakthrough infection after a full course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 ß-thalassemia major, 118 ß-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of the China Sinopharm vaccine and received the second dose of the vaccine 28 days apart. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzymelinked immunosorbent assay (Pishtazteb ELISA commercial kit), including a surrogate virus neutralization test (sVNT), for detection of SARS-CoV-2 immunoglobulins (IgA, IgM, IgG), total neutralizing antibody (NAb). Results: The mean age of patients was 35.0 ± 8.5 (from 18 to 70) years, and 55.6% were positive for the antibody. Overall, 48.2% of the studied population had at least one side effect after vaccination. The most frequent side effects were fever and chills, dizziness, and body pain. A total of 90 (20.7%) vaccinated patients developed breakthrough infections after two doses of Sinopharm vaccination. Disease severity was recorded, and it was classified as mild in 77.8%, moderate in 13.6%, and severe in 7.4% of patients. One 28-year-old woman with ß-thalassemia major died eight days after diagnosing a breakthrough SARS-CoV-2 infection. Conclusion: No safety concerns were identified in patients who received two doses of the Sinopharm vaccine. Its efficacy was not optimal due to the lack of effect on new variations of the virus. However, our data show that it seems to be protective against the severity of COVID-19 infection in patients with hemoglobinopathies. The frequency of breakthrough infections after two doses of Sinopharm vaccination supports the evolving dynamic of SARS-CoV-2 variants requiring special challenge since such infection may represent a risk for vulnerable patients. [ABSTRACT FROM AUTHOR]

This study aimed to compare the cost-effectiveness of these two regimens in hemophilia A patients, under-12-years-old in southern Iran. A cost-effectiveness study comparing prophylaxis versus on-demand was conducted on 34 hemophilia patients (24 and 10 patients were on the prophylaxis and on-demand regimens respectively) in 2017. The Markov model was used to estimate the economic and clinical outcomes. The costs were collected from the societal perspective, and the utility criterion was the 'quality adjusted life year (QALY)' indicator. The required data were collected using a researcher-made cost checklist, the EQ5D standard questionnaire and Hemophilia Joint Health Score. The probabilistic sensitivity analysis (PSA) was performed to determine the robustness of the results. The means of costs, joint health score and QALY in the prophylaxis regimen were $478,963.1 purchasing power parity (PPP), 96.67, and 11.98 respectively, and in the on-demand regimen were $521,797.2 PPP, 93.46 and 10.99 respectively. The PSA confirmed the robustness of the model's results. The results of the scatter plots and acceptability curves showed that the prophylaxis regimen in 97% of the simulations for the thresholds below $20950 PPP was more cost-effective than on-demand regimen. Prophylaxis regimen showed the lower costs and higher effectiveness and utility in comparison with the on-demand regimen. It is recommended that prophylaxis should be considered as the standard care for treatment of hemophilic patients. [ABSTRACT FROM AUTHOR]

Over the past 20 years, hydroxyurea (HU) has emerged as an effective therapeutic agent in thalassemic patients to improve anemia and decrease the transfusion dependency. We evaluated long-term safety and clinical response to HU in patients with non-transfusion-dependent β-thalassemia (NTDT). In this retrospective study, medical records of 181 patients with NTDT were evaluated during October to December 2020 in Southern Iran. No requirement to blood transfusion was considered as sustained transfusion independence response. All patients were regularly examined and monitored for the occurrence of any adverse event (AE) of HU. The mean duration of HU consumption ± SD was 18.2 ± 4.0 (8–22) years. Overall, 149 patients (82.3%) had sustained transfusion independence response. β-globin gene mutations and XmnI polymorphisms were not significantly associated with clinical response (P > 0.05). Mild and transient AEs were reported in 60 patients (33%) with no requirement to drug interruption. Hydroxyurea with the dose of 8–15 mg/kg can be used as a safe and effective treatment in NTDT patients. It was well tolerated in long term without any serious complication or secondary malignancy. No relationship between XmnI or β-globin gene mutations with HU response was observed in this geographic area of the world. [ABSTRACT FROM AUTHOR]

Background: In operating rooms, blood product wastage occurs with various reasons especially over-demand ordering during elective surgeries. Consequently, it imposes a heavy financial burden on health system. Therefore, managing blood consumption in the operating rooms is of special importance. Surgeons and anesthesiologists play a key role in blood transfusion practice in operating rooms. Objective: To investigate surgeons´ and anesthesiologists´ perspectives in regard to the effective strategies for better management of blood transfusion practice in operating rooms. Method: In this qualitative cross-sectional study, from January to March 2020, 60 surgeons and anesthesiologists were participated. All of them were working in surgical departments of the general hospitals affiliated with Shiraz University of Medical Sciences, in Shiraz, Southern Iran. Their viewpoints regarding the improvement of blood consumption management in operating rooms were recorded through interviews and several focus group sessions. Results: After content analysis, the most important recommendations with the highest scores were regular training programs for surgical team (23.3%), collaboration of surgeons, anesthesiologists, and managers of the surgical departments by arrangement of monthly meetings to estimate the number of required blood bags more accurately (23.3%), establishing a well-organized electronic requesting and registration system (13.3%), promoting the ordering process by a comprehensive preoperative evaluation (11.6%), and updating transfusion protocols based on the standards and monitoring the adherence to these standards (10%). Conclusion: Implementing the proposed policies, which are based on the invaluable experiences of related experts would be effective in improving blood consumption management in operating rooms. [ABSTRACT FROM AUTHOR]

Background: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation. We aimed to describe the clinical and laboratory findings of HLH patients secondary to Visceral leishmaniasis (VL) and their treatment outcome during a 4-year follow-up period compared to primary HLH.Method: Forty children with primary HLH confirmed by genetic study and 20 children with HLH secondary to VL confirmed by a blood or bone marrow polymerase chain reaction from 2014 to 2018 in Shiraz, Fars province, Southern Iran, were enrolled.Results: The median age at diagnosis was 11.5 months (range 1-170), and 56.7% were male. Fever and splenomegaly were the most frequent clinical presentations. 93.3% of the subjects had an HScore > 169, which had a good correlation with HLH-2004 criteria (r = 0.371, P = 0.004). Patients with primary HLH experienced more thrombocytopenia (P = 0.012) and higher alanine transaminase (P = 0.016), while patients with VL-associated HLH had higher ferritin (P = 0.034) and erythrocyte sedimentation rate (P = 0.011). Central nervous system (CNS) involvement occurred in 38.3% of patients. The mortality rate was higher in patients with CNS disease (61% vs. 35%, P = 0.051). The 3-yr overall survival rate was 35.9%. (24% in primary HLH and 100% in VL-associated HLH, P < 0.001). In Cox regression analysis, platelet count < 100,000/ μ l (hazard ratio 4.472, 95% confidence interval 1.324-15.107, P = 0.016) correlated with increased mortality in patients with primary HLH.Conclusion: VL is a potential source of secondary HLH in regions with high endemicity. Treatment of the underlying disease in VL-associated HLH is sufficient in most cases, with no need to start etoposide-based chemotherapy. [ABSTRACT FROM AUTHOR]

Background and objective: Saving blood products is an important public health issue especially in developing countries with limited financial resources. We aimed to suggest a new hypothetical model to make a change in the current blood transfusion policy in the newborn intensive care unit (NICU) to reduce wastage of blood supplies as well as the risk of exposure to multiple donors. Methods: In this cross-sectional study, all transfused neonates (n = 70) who were admitted to NICU of Nemazee Hospital, a tertiary referral hospital in Southern Iran, were evaluated between March and June 2019. Based on the information of neonates' transfusion during this study period and determined transfusion indices, a specific pediatric pack was suggested and the related total costs per transfusion, as well as the donor-exposure rate of the hypothetical and the current transfusion method, were compared. Results: Considering the mean number of transfusions per neonate: 4 and mean volume of transfused packed red cells: 20 ml per transfusion, the cost-analysis of pediatric and the adult pack was presented. Arithmetically, we proved a higher total cost per transfusion for using adult pack comparing to pediatric pack. Additionally, using a pediatric pack set leads to a 24% reduction in RBCs wastage per transfusion and a 68.13% reduction in donor-exposure rate. Conclusions: The assignment of a dedicated pediatric pack for neonates will be able to improve the cost-effectiveness by a substantial reduction in donor-exposure rate and blood wastage. This finding should be taken into consideration to generate economic growth and make improvements in child health status. [ABSTRACT FROM AUTHOR]

Oxidative stress is a major mechanism contributing to the progression of β-thalassemia. To assess the effect of vitamin E and N-acetyl cysteine (NAC) as antioxidant agents on total oxidative stress (TOS) status and total antioxidant capacity (TAC) in patients with transfusion-dependent β-thalassemia (TDT). In this open-label randomized controlled trial, from May to August 2019, 78 eligible patients with TDT over the age of 18 were enrolled. All patients were registered at the Thalassemia Clinic of Shiraz University of Medical Sciences in Southern Iran. Patients were randomly allocated to the NAC group (10 mg/kg/day, orally), vitamin E group (10 U/kg/day, orally), and control group. The duration of the study was 3 months. The mean age of the participants was 28.5 ± 5.1 (range: 18–41) years. At the end of the study, TOS significantly decreased only in the vitamin E group (mean difference (MD), 95% confidence interval (CI): 0.27 (0.03–0.50), P = 0.026). TAC significantly decreased in both supplemented groups at the 3rd month of treatment (NAC group: MD (95% CI): 0.11 (0.04–0.18), P = 0.002 and vitamin E group: 0.09 (0.01–0.16), P = 0.022 respectively). Hemoglobin did not significantly change at the end of the study in each group (P > 0.05). Mild transient adverse events occurred in 4 patients of the NAC group and 5 patients of the vitamin E group with no need to discontinue the treatment. Vitamin E can be a safe and effective supplement in improving oxidative stress in patients with TDT. Moreover, it seems that a longer duration of using antioxidant supplements needs to make clinical hematologic improvement in TDT patients. [ABSTRACT FROM AUTHOR]

Background: The requests for blood products in elective surgeries exceed actual use, leading to financial wastage and loss of shelf-life. In this study, we assessed the blood transfusion indices in elective surgeries performed in the operating rooms. Methods: In this cross-sectional study, from January to June 2017, a total of 970 adult patients who underwent elective surgeries in the operating rooms of Nemazee hospital, a general referral hospital in southern Iran, were investigated. Demographic, clinical, and laboratory data, such as hemoglobin (Hb), hematocrit (Hct), platelets, prothrombin time (PT), and partial thromboplastin time (PTT) were gathered from medical records. Blood utilization was evaluated using the following indices: cross-match to transfusion ratio (C/T ratio), transfusion probability (T%), transfusion index (TI), and Maximum Surgical Blood Order Schedule (MSBOS). Results: The overall C/T, T%, and TI ratios were 2.49, 46.6%, and 0.83 for all procedures, and the highest and lowest ratios pertained to the thoracic and cardiac surgeries, respectively. The C/T ratio was ≥2.5 for all surgical procedures except for cardiac surgeries. T% was <30 for thoracic and orthopedics surgeries and ≥30 for other surgical procedures. In all surgical procedures, TI was less than 0.5, except for cardiac surgeries. Also, the MSBOS was about 3 units for cardiac surgeries and ranged from 0.5 to 1 units in other surgeries. Conclusion: The results of this study showed a high quality blood transfusion practice in cardiac surgeries, possibly due to more focus on this critical ward. Assessing difficulties in the process of reservation, utilization, and preparation of standard protocols and policies are required to improve the blood utilization practice in operating rooms. [ABSTRACT FROM AUTHOR]

Hydroxyurea (HU) activates the γ-globin gene, resulting in increased Hb F synthesis. The SOX6 gene is a member of the Sox (Sry-type HMG box) family of transcription factors, characterized by minor groove binding domain. The DNA binding domain of this gene is encoded by exon 14. We assessed the relationship between response to HU and exon 14 of the SOX6 gene sequence variations in patients with non transfusion-dependent thalassemia (NTDT). One hundred NTDT patients from southern Iran underwent HU therapy randomly participated in this cross-sectional study between February 2013 and October 2014. Based on response to HU therapy, the patients were divided into two groups: good and poor responder. Sequence variations of exon 14 of the SOX6 gene was assayed by the Sanger sequencing technique. From all evaluated single nucleotide polymorphisms (SNPs) as above, we found no significant association between sequence variations of exon 14 of the SOX6 gene and response to HU therapy (p > 0.05). It seems that no SNPs in exon 14 of the SOX6 gene is associated with response to HU in NTDT patients, but more studies are needed for further evaluation. [ABSTRACT FROM AUTHOR]

Background: Children with acute lymphoblastic leukemia (ALL) are prone to neurotoxicity and consequently neurocognitive function impairment mainly due to undergoing different treatment modalities. In the current investigation, neurocognitive function of children with ALL was compared to that of healthy children. Materials and Methods: In this cross-sectional study, 155 ALL and 155 age-matched healthy children in Shiraz, Southern Iran, were included and evaluated using Continuous Performance Test (CPT). Results: Mean age of the patients was 9.9± 2.4 years. The number of wrong responses and duration of response did not lead to significant difference between healthy and affected children. In the age group less than 12 years old, the frequency of no-response was higher in the case group compared to control group both in boys and girls (P = 0.012, P = 0.006 respectively). In addition, in male patients younger than 12 years old, the number of correct responses was significantly less than that of controls (P = 0.010). Patients underwent concurrent radiotherapy and chemotherapy needed significantly more time for responding compared to patients in whom chemotherapy were discontinued and were in remission (P=0.001). Conclusion: Based on the results, ALL children younger than 12 years old showed some defects in cognitive function. Moreover, it was more prominent in young boys compared to young girls. Regardless of the type of treatment regimens, early detection of neurocognitive disorders should be warranted in this high-risk population with more focus on boys and younger children. Psychological support and appropriate interventions can help improve cognitive function, reduce the disruption of education, and enhance the social and family relationships. [ABSTRACT FROM AUTHOR]

Recently, resveratrol showed induction of γ-globin mRNA synthesis in human erythroid precursors and reducing oxidative stress in red cells of thalassemia patients in many in vitro studies. We aimed to investigate the efficacy and safety of resveratrol, for the first time, in non-transfusion-dependent beta-thalassemia intermedia (B-TI) in Southern Iran. In this double-blind randomized clinical trial, 54 patients with B-TI were investigated during 6 months between October 2016 and March 2017. Patients were randomly allocated into three groups by simple randomization method. Group 1 (hydroxyurea (HU) and placebo, 18 patients), group 2 (resveratrol/piperine and placebo, 16 patients), and group 3(HU and resveratrol/piperine, 20 patients). Primary end point was considered as change in hemoglobin (Hb) levels and need for blood transfusion. Drug safety was considered as a secondary end point. Mean age of the patients was 28.2 ± 5.6 (18-42) years. Response rate was not significantly different among the three groups (P > 0.05). Higher percentages of adverse events were detected in groups 2 (31.3%) and 3 (25%) compared to group 1 (5.6%). However, the difference was not statistically significant (P > 0.05). All reported adverse events were gastrointestinal symptoms. Resveratrol showed a similar efficacy with HU in the small population of non-transfusion B-TI patients during a 6-month follow-up. Complications, mostly gastrointestinal, were observed more frequently in resveratrol groups compared to the HU group. Although it was not statistically significant, more attention should be given to safety and efficacy of resveratrol as an oral HbF-augmenting agent. [ABSTRACT FROM AUTHOR]

Background: Folate and vitamin B12 have a number of biologic roles that make them important in hematological disorders and malignancy. In the present study, we have assessed serum folate and vitamin B12 levels and their associated variables in patients with leukemia, lymphoma, and solid tumors. Methods: This cross-section study investigated 98 patients (57 with leukemia, 16 lymphoma, and 25 solid tumors) between April 2015 and March 2016 in Southern Iran. Complete blood counts and serum levels of folate, vitamin B12, lactate dehydrogenase, and homocysteine were measured. Clinical characteristics of the patients were also gathered from their medical records. Results: Patients had the following mean serum levels: serum folate (12.27±6.69 ng/ml), vitamin B12 (331.81±183.22 pg/ml), and homocysteine (11.74±26.67 µmole/L). Vitamin B12 showed a significant negative correlation with homocysteine levels (r= -0.223, P=0.043). Overall, there were 21(21.9%) vitamin B12 and 9 (9.8%) folate deficient patients. Vitamin B12 patients had a longer remission time (time from off therapy until study entry) of 18±16.97 months compared to those with normal vitamin B12 levels (8.81±8.08 months, P=025). However, logistic regression analysis showed that only mean corpuscular volume had a significant correlation with vitamin B12 deficiency (B= -0.105, odds ratio=0.9, 95% CI: 0.819-0.990, P=0.03). None of the variables showed significant correlation with folate deficiency (P>0.05). Conclusion: Vitamin B12 and folate deficiency are frequently seen in survivors of childhood malignancy, mainly due to the effects of chemotherapy. These vitamins have important roles in hematopoiesis, as well as development and maintenance of the nervous system; therefore, timely detection of their deficiencies is of utmost importance. It is highly recommended to check the serum levels of these vitamins in children who successfully survive their cancer treatments. [ABSTRACT FROM AUTHOR]

Background: Reduced survival and impaired quality of life of the children affected by cancers is one of the most important health problems. In this study, 5-year survival of children affected with Central nervous system (CNS) tumors and its related factors were evaluated. Materials and Methods: Participants in this historical cohort study consisted of 161children with mean age of diagnosis 72 ± 51 months (median:60 months, range from 1 month to 17 years) who were diagnosed with CNS tumors from 1999 to 2005. All patients had referred to Oncology hospitals of Shiraz University of Medical Sciences, Shiraz, Iran. Data were extracted by checklist from their medical records. Result: Five-year overall survival (OS) and disease free survival (DFS) of the patients were 59% (standard error: 5%) and 51.7% (standard error: 5%). Moreover, 10-year OS was calculated as 47% (standard error: 7%). Based on tumor histology, OS was 70% for low grade tumors and 52% for high grade tumors (P=0.202). Based on the results, gender (girls had longer survival than boys), recurrence, neurologic deficit and age of diagnosis (60-119 months had longer survival), were determined as the influencing factors on OS rate (HR (95% CI) =0.48 (0.24-0.98), P=0.044, 0.48(0.25-0.93) P=0.031, 0.42 (0.18-0.95), P=0.039, and 0.32 (0.11-0.88), P=0.029, respectively). Moreover, tumor location in diencephalon was determined as poor prognostic factors ((HR (95% CI) =10 (1.9-57), P=0.007). Conclusion: Aforementioned prognostic factors should be taken into account by oncologists to make better decisions in the management of the patients with CNS tumors. It seems that survival is a multifactorial event and besides these prognostic factors, it might be also related to different clinical settings, ethnicity and type of treatment. Further studies with more focus on different treatment modalities are suggested. [ABSTRACT FROM AUTHOR]

Background: Use of complementary and alternative medicine (CAM) among cancer patients is not exactly defined. Objectives: The aim of this study was to determine the use of CAM and associated factors among Iranian cancer patients, reasons behind this use, satisfaction and information about complementary and alternative therapy. Methods: To assess CAM usage by adult cancer patients, a questionnaire was designed and distributed among the patients admitted in Amir Oncology hospital affiliated to the Shiraz University of Medical Sciences in Shiraz, South of Iran. The questionnaire consisted of 29 items including patients' socio-demographic characteristics and questions about CAM use. Results: A total number of 36 cancer patients participated in this study. The majority of respondents (94.4%) used or were using at least one CAM product or practice till the study date. Altogether, the most common type of CAM used among cancer patients was praying (86.1%). The most popular herbs in herbals subgroup were Mint and Garlic with 41.7%. Multivitamins and vitamin C were more favored than other vitamins and minerals (33.3%). No major side effects were reported regarding CAM use. The most reliable source of information about complementary medicine was reported to be physicians. Conclusions: Iranian cancer patients frequently use CAM in addition to medical treatments. Therefore, it is important for oncologists to be aware of CAM use, its effects and adverse reactions to guide their patients. [ABSTRACT FROM AUTHOR]

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This study aimed to investigate clinical symptoms in patients with congenital factor V (FV) deficiency and the relationship between phenotype and factor activity level. Thirteen patients with congenital FV deficiency were investigated and the factor activity level and first clinical presentations were studied for each patient. The most common first signs and symptoms were post-surgery, post-partum, post-circumcision, and post-traumatic bleeding (30.76%), followed by easy bruising in 23.10% of the patients. The median age at the onset of clinical signs was 18 (range: 1-53) years. Patients were categorized into two groups of major and minor bleeding based on their first clinical bleeding symptoms. There was not a significant difference between the two groups with regard to factor activity level, age at diagnosis, prothrombin time, partial thromboplastin time, and international normalized ratio (p>0.05). There is a discrepancy between plasma FV activity level and the severity of clinical presentations. [ABSTRACT FROM AUTHOR]

Background: Total bone mass acquired during childhood is known to be the most important determinant and base of lifelong skeletal health. The present study investigated the prevalence of low bone mass for chronological age (LBM) in lumbar and femoral areas of leukemic children in the south of Iran and evaluated the association of anthropometric, clinical and laboratory data of these children with low bone mass. Materials and Methods: This cross sectional study was conducted on 106 patients with proven diagnosis of acute leukemia who aged 4-18 years old. Anthropometric data, physical activity, sun exposure, pubertal stage and mineral biochemical parameters were assessed. Bone mineral density was measured by Dual-energy X-ray absorptiometry. The data were analyzed using SPSS (version21). Results: The prevalence of low bone mass for chronological age in leukemic children of southern Iran was 28.3% and 27% in lumbar and femoral regions, respectively and it was not sex dependent. This prevalence was associated with duration of disease, serum calcium, serum level of vitamin D, Body mass index (BMI), and radiotherapy. Among these factors, serum calcium had independent predictive effects. Conclusion: The present investigation revealed high prevalence of low bone mass in Iranian leukemic children. The most important associated factors with bone mineral density were radiotherapy, BMI, duration of illness, and serum calcium level. Further studies are recommended on bone mineral density in leukemic children, especially in Asian countries, with attention to patients' fractures to find out the important risk factors for future osteoporosis. [ABSTRACT FROM AUTHOR]

Introduction: Zinc and copper have a main role as nutrients in the growth and development of neonates. Aim: To evaluate the relationship of birth weight, feeding, and Gestational Age (GA) with serum copper and zinc in preterm neonates. Materials and Methods: This cross-sectional study has evaluated 107 preterm infants with Birth Weight (BWT) less than 2500 g that were hospitalised in neonatal intensive care unit for at least 10 days from January 2014 to March 2016 in Shiraz, Iran. Zinc and copper serum levels were compared in very low BWT (≤1500 g) and low BWT (1500-2500 g) in newborns and were investigated in regard to the type of nutrition, BWT, and GA. Zinc and copper levels were estimated using Flame-Atomic Absorption Spectrophotometry. Multiple linear regression analysis and Pearson's correlation coefficient (r) test were used. Results: The mean plasma zinc levels in low BWT and very low BWT groups were 83.9±17.1 µg/dL and 48.2±10.4 µg/dL, respectively (p<0.001). The mean serum copper levels in low BWT and very low BWT groups were 70.48±15.4 µg/ dL and 82.7±12.03 µg/dL respectively (p<0.001). There was a significant inverse correlation of serum copper with BWT (r=-0.525, p-value<0.001) and GA (r=-0.572, p-value<0.001). However, there was a positive correlation of the zinc level with BWT (r=0.758, p=0.001) and GA (r=0.741, p-value=0.001). Conclusion: Serum zinc is directly related to GA and LBW. Consequently, supplementation with zinc in premature neonates and LBW is necessary, but not copper. [ABSTRACT FROM AUTHOR]

Objectives: This study was conducted to assess bone mineral density (BMD) and bone mineral content (BMC) of patients with hemoglobin H (HbH) disease.Methods: BMD and BMC were measured by dual energy X-ray absorptiometry of the lumbar spines and femur neck in 21 patients with Hb H disease over the age of 10 years. An association of BMD with sex, age, hemoglobin, calcium, phosphorus, and serum ferritin level was also evaluated.Results: Prevalence of BMD below the expected range for age in the lumbar spine and femur neck region in patients with HbH disease were 33.3 and 14.3 %, respectively. Lumbar BMD was significantly lower in the patients compared to healthy individuals (median (min-max) 0.725 (0.595-0.924) vs. 1.061 (0.645-1.238), P < 0.001)). There was no significant relationship between BMD in the lumbar and femur neck with any of the evaluated variables (P value >0.05).Conclusion: Data regarding bone density in HbH disease is limited; osteoporosis as a common complication of β-thalassemia intermedia syndrome should be considered even in HbH which shows its prevalence is less than β-thalassemia intermedia. [ABSTRACT FROM AUTHOR]

Background: Abdominal tumors are still a diagnostic problem in children. This study was conducted to assess the presentation and types of childhood intra abdominal tumors in children in Shiraz. Materials and Methods: Participants of this historical cohort study consisted of 298 children aged between 1.5 months to 15 years old who were diagnosed with abdominal mass between March 2003 and March 2013. All patients referred to pediatric surgery wards of Namazi hospital affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. Data extracted by checklist included patient's demographic data, type of malignancy, signs and symptoms at the time of presentation and duration of signs and symptoms. Results: The majority of tumors were retroperitoneal followed by intra-peritoneal and pelvic masses. Wilms tumor was the most common tumor constituting 34% of all cases followed by neuroblastoma (24.7%), and non-Hodgkin lymphoma (12.7%). The most common signs and symptoms were abdominal pain (39.5%), abdominal distention (34.2%), palpable mass (27.5%), weight loss (19.4%), fever (16.7%), and vomiting (10.7%). Conclusion: Better understanding of signs and symptoms of abdominal masses can facilitate early diagnosis and proper treatment of malignant abdominal tumors in children. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the efficacy and safety of deferasirox in patients from Iran. Methods: This was a retrospective, observational study in regularly transfused, iron-overloaded patients who received deferasirox 20-38 mg/kg/day for up to 12 months. Changes in serum ferritin were assessed as follows: from baseline to 3 months with deferasirox doses of 20-24 mg/kg/day; from 3 to 6 months with doses of 25-29 mg/kg/day; and from 6 to 12 months with doses of 30-38 mg/kg/day. The safety of deferasirox was evaluated monthly. Patients' satisfaction with treatment was assessed after 9 months. Results: One hundred and nineteen patients were included. Overall mean serum ferritin levels were significantly decreased from baseline after 12 months of deferasirox therapy (2510 ± 1210 to 1665 ± 1240 ng/ml; P < 0.002). A significant decrease was observed once doses were increased to ≥30 mg/kg/day (P = 0.0003). Most adverse events were mild and observed at the dose of 20-24 mg/kg. Only one patient discontinued treatment. Around 90% of patients were satisfied with therapy. Conclusion: This is the first study evaluating deferasirox in heavily iron-overloaded patients from Iran and confirms that deferasirox is effective and well tolerated; however, dose increases to ≥30 mg/kg/day should be considered if efficacy is insufficient. [ABSTRACT FROM AUTHOR]

Background: Beta-thalassemia is commonly associated with lipid abnormalities. The aim of this study was to search for links between these lipid alterations and different types of β-thalassemia mutations. Methods: The study, conducted from 2009 to 2010, included 100 patients with thalassemia major (TM) and 100 with thalassemia intermedia (TI). The control group was selected from 100 age- and sex-matched healthy individuals with normal hematologic indices. Serum lipid profiles, including total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C) and triglycerides (TG), were determined and their relationship with different covariates, including different β-globin gene mutations, was analyzed. Results: Patients with TI had significantly lower values for TC, LDL-C, HDL-C, and LDL-C/HDL-C ratio compared with TM patients and controls (P < .001). TG was greater in TM compared with TI patients (P = .001) and healthy individuals (P = .007). Hemoglobin was positively associated with TC (P < .001), LDL-C (P = .004), and HDL-C (P = .01) in TM patients. Splenectomy correlated with greater TC (P = .006) and LDL-C (P = .01) in TI patients, but only with greater LDL-C in TM patients (P = .02). The average amounts of TC and LDL-C were lower in persons with the β0/β0 mutation compared with the β+/β+ group. Conclusion: Lower amounts of TG, TC, LDL-C, and HDL-C were seen in TI patients compared with TM patients and healthy individuals. The severity of the genotype (ie, β0 type mutations compared with β+ type mutations) affected the degree of reduction in serum lipids. [ABSTRACT FROM AUTHOR]

Introduction. We conducted this study to measure hemodialysis adequacy and determine to what extent some Kidney Disease Outcomes Quality Initiative (KDOQI) goals are achieved in our patients. Materials and Methods. In a cross-sectional study in Fars province, Iran, we assessed 632 patients on hemodialysis in 15 dialysis centers. The Kt/V was calculated, and data on serum levels of albumin, cholesterol, and triglyceride, hemoglobin level, blood pressure, body weight, and body mass index were collected. The values were compared with the KDOQI recommended target values. Results. The mean age of the patients was 54.36 ± 16.34 years. The mean Kt/V was 0.97 ± 0.42, which was significantly higher in those who received 3 dialysis sessions per week than those on 2 dialysis sessions per week (P = .03). Only 32.1% of all patients achieved the Kt/V goal. Seventy-four percent of the patients had a serum albumin equal or greater than 4 g/dL. Hemoglobin levels were between 4.6 g/dL and 16.8 g/dL, and half of the patients had attainment of the hemoglobin target. Cholesterol target was reached in 40% of patients. Only 43 patients (6.8%) attained all targets recommended by the KDOQI guidelines. Conclusions. The target values, except for serum albumin, were not reached in our patients. We conclude that it is necessary to provide essential equipment of dialysis centers such as dialysis machine and trained staffs, and also to raise awareness of KDOQI goals among patients and physicians. [ABSTRACT FROM AUTHOR]

Objective Despite premarital screening, prenatal diagnosis and the option for voluntary termination of pregnancy, some Iranian couples continue a pregnancy with a fetus affected with beta-thalassaemia major (β-TM). We examined the reasons for this decision, to evaluate the sociocultural challenges these couples face. Methods A retrospective evaluation of medical records of all fetuses aborted was conducted to identify those with β-TM, to determine the frequency of β-TM births and to establish the number of couples with β-thalassaemia who declined prenatal diagnosis or a termination of pregnancy in this period. We investigated the reasons for declining these options. Results The birth prevalence of β-TM decreased from 39.38 to 2.68 in 100,000 live births from 2005 to 2010. Terminations of pregnancy for affected fetuses increased from 67 in 2005 to 135 in 2010. We identified eight couples as β-thalassaemia carriers who declined prenatal diagnosis or a termination of pregnancy. All but one couple already had a child with β-TM. The reasons for declining prenatal diagnosis were strong religious beliefs, superstition and faith in a supernatural solution in six couples. Economic and cultural factors, and previous termination of pregnancy were also mentioned as reasons by the other two couples. Conclusion Although most of the couples had a β-TM child and related socioeconomic problems, their reasons for refusing prenatal diagnosis or termination remain a challenge for the healthcare system in Iran. Couple screening and educational programmes have effectively decreased the rates of refusal in at-risk couples in recent years. [ABSTRACT FROM AUTHOR]

Background: Ocular involvement may occur via several mechanisms in patients with transfusion-dependent β-thalassemia (TDT) mainly chronic anemia, iron overload and iron chelator toxicity. We aimed to evaluate the frequency of abnormal ocular findings and their relationship with hematologic parameters in TDT patients.Methods: In this cross-sectional study from January 2018 to January 2019, a total of 79 patients with TDT over the age of 18 who were on iron-chelation therapy (ICT) were consecutively investigated. All patients were registered at the Thalassemia Comprehensive Center affiliated with Shiraz University of Medical Sciences, Shiraz, Southern Iran. Complete ophthalmic examination was performed by an expert ophthalmologist. Clinical and hematologic parameters were collected from the patients´ medical records.Results: The mean age ± standard deviation (SD) of the patients was 28.4 ± 5.6 years (range: 18-43). Twenty-four patients (30.4%) were male and 29 (36.7%) were splenectomized. The mean ± SD of the best-corrected visual acuity (VA) was 0.960 ± 0.086 decimal, (range: 0.6-1), 0.016 ± 0.046 logMar, (range: 0-0.2). The frequency of patients with VA > 0.1 logMar was 3 (3.8%). The mean intraocular pressure (IOP) was 14.88 ± 3.34 (6-25) mmHg. Fundus abnormalities were observed in 8 patients (10.1%), consisting of increased cup-disk ratio (3.8%), vessel tortuosity (2.5%), retinal pigment epithelium degeneration (2.5%), myelinated nerve fiber layer (1.3%), and internal limiting membrane wrinkling (1.3%). No significant association was observed between fundus abnormalities, VA, or IOP with hematologic parameters (P > 0.05). TDT patients with diabetes mellitus had significantly higher IOP (P = 0.010) but similar frequency of fundus abnormalities with non-diabetic patients (P > 0.05).Conclusions: The frequency of ocular abnormalities in our patients was lower than the previous reports. The frequency of fundus abnormalities were similar in diabetic and non-diabetic thalassemia patients indicating close monitoring and proper management of the disease and comorbidities in these patients. [ABSTRACT FROM AUTHOR]

Background: Surveillance of current changes in the epidemiology of Invasive Fungal Diseases (IFDs) as an important component of the antifungal stewardship programs (ASP), requires careful regular monitoring, especially in high-risk settings such as oncology centers. This study aimed to examine Candida colonization status and corresponding current changes in children with malignancy during repeated admissions and also investigate the possible epidemiological shifts after the implementation of ASP.Methods: In this prospective observational study, all eligible patients younger than 18 years were recruited during 2016-2017 at Amir Medical Oncology Center (AMOC) in Shiraz, Iran. Totally, 136 patients were enrolled and 482 samples were collected from different sites (oral/nasal discharges, urine and stool). Weekly regular sampling was carried out during hospitalization. Candida colonization status and epidemiological changes were monitored during repeated admissions. Samples were cultivated on Sabouraud Dextrose agar medium and identified by Polymerase Chain Reaction -Restriction Fragment Length Polymorphism (PCR-RFLP).Results: Estimated Candida colonization incidence was 59.9% (82/136) in our patients. Candida colonization was found to be higher in oral cavity and rectum than that in nasal cavity. Among those long-term follow ups and repetitive hospitalizations, a significant number of patients exhibited changes in their colonization patterns (37.7%). Candida colonization did not reveal any significant relationship with age, sex, oncologic diseases and degree of neutropenia. C. albicans (72.0%) was found as the most common Candida species in colonized patients, followed by C. krusei, C. kefyr, C. glabrata and C. parapsilosis.Conclusion: Given the high incidence of Candida infections in children with cancers, close monitoring of epidemiologic changes is essential for judicious management, based on local surveillance data and improvement of overall quality of care in high risk patients. [ABSTRACT FROM AUTHOR]

Background: Wilms tumor (WT) is the most common malignant renal tumor in children. This study investigated the clinical features, pathological findings, and outcomes of children with malignant renal tumors in Southern Iran. Factors associated with recurrence and mortality were assessed., Methods: Electronic files of children with malignant renal tumors from 2009 to 2023 were reviewed. The 5-year overall survival (OS) and event-free survival (EFS) were reported., Results: Eighty-three patients (44 males) with a median age of 40 months (range: 3-122) were included. WT was the most common pathological variant (94%). Anaplasia was found in 17.3% of patients. Upfront chemotherapy followed by nephrectomy was performed in 54.2% of the patients. Ten patients (12%) experienced relapse, and five patients (6%) died during the 14-year follow-up. The 5-year OS and EFS were 90.75% (95% CI, 78.64-96.16%) and 81.9% (95% CI, 70.10-89.38%), respectively, and were comparable between the two treatment strategies (upfront chemotherapy vs. upfront nephrectomy). Metastasis and residual disease were associated with relapse, whereas tumor recurrence was the only predictive factor of survival., Conclusion: WT is a curable disease with excellent outcomes if diagnosed and treated promptly. The timing of nephrectomy does not affect OS and EFS. Patients with low-stage tumors and those with complete surgical excision are at a lower risk of tumor recurrence. Relapse is the primary risk factor for death., Competing Interests: Declarations Ethics approval and consent to participate The Ethics Committee of Shiraz University of Medical Sciences approved the study with the code number IR.SUMS.REC.1402.235. The participants were called and consented that their data be used in the study. Informed consent was obtained from all participants or their legal guardians (in children younger than 16 years old) to use their data in the study”. Consent for publication Not applicable. Competing interests The authors declare no competing interests., (© 2024. The Author(s).)

Background: Gastrointestinal cancers account for a significant number of deaths and new cancer cases worldwide. Our aim is to investigate stratum-specific incidence trends of esophageal cancer in southern Iran considering age, gender, and morphological (adenocarcinoma/squamous cell carcinoma) subtypes., Method: We used data on esophageal cancer incidence in the catchment area of Shiraz Population-Based Cancer Registry. New cancer cases with ICD-O-3 codes of C15.0 to C15.9 were retrieved and prepared. New annual cases were counted for categories defied based on age group, gender, and morphology. Average annual percentage change (AAPC) and its 95% CI was estimated for each trend using joinpoint regression., Results: The total number of esophageal cancer was higher in males than females. It is an increasing rate in esophageal adenocarcinoma in females and this increase was more prominent in older ages. Also, our study showed that SCC has a decreasing trend in females and a steady trend in males., Discussion: Taken together, the increasing trend in adenocarcinoma in females in our study can be related to the increasing rate of obesity and smoking in females in recent years in our region. The incidence trend of different morphologic types of esophageal cancer is changing. This can be a consequence of alterations in the incidence trend of different risk factors. It is necessary to conduct more studies evaluating these risk factors., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Using maps and spatial analysis are technologies to evaluate the magnitude and spatial distribution of disease in epidemiology investigations. We aimed to conduct a Bayesian spatial analysis on epidemiologic data of transfusion-dependent β-thalassemia (TDT) patients., Methods: In this cross-sectional study, data of all TDT patients diagnosed during 1955-2018 in all counties of Fars Province were obtained from data registry of the Organization of Special Diseases of Shiraz University of Medical Sciences in Shiraz, Fars Province, Iran. Besag, York, and Mollie's (BYM) model was used for mapping., Results: The estimated relative risk ranged from 0.23 to 1.66 for TDT patients. The highest and lowest relative risks of TDT were observed in Larestan located in Southern and Abadeh in Northern Fars Province respectively., Conclusions: Determining the accurate geographical distribution of a chronic disease such as β-thalassemia can be an essential prerequisite in allocation of regional health system resources., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2020. Published by Elsevier Ltd.)

Despite the significant improvements in the management of thalassemia, there are growing concerns regarding their long-term complications. We showed that low bone mass is one of the most prevalent complications among these patients. Insufficient physical activity and hypogonadism are the main possible associated factors followed by DM and insufficient sun exposure., Purpose: Despite the significant improvements in the management of transfusion-dependent thalassemia (TDT), there are growing concerns regarding their long-term complications., Methods: This cross-sectional study included 615 TDT patients who were registered and followed in a comprehensive thalassemia clinic in Southern Iran. We measured serum biochemical tests and bone mineral density in all patients. We recorded physical activity and sun exposure subjectively, and an endocrinologist visited and examined all the patients. A group of age- and gender-matched healthy volunteers participated in the study as the control group., Results: The mean age of the studied population was 28.4 ± 7.7 years, and 55.8% were female. The prevalence of vitamin D deficiency was 45.6% and 54.4% in TDT patients and the control group. A portion of TDT patients suffered from different endocrinopathies, which included hypogonadism (49.8%), diabetes mellitus (17.2%), hypoparathyroidism (14.6%), and hypothyroidism (6.3%). The prevalence of low bone mass in patients with TDT was 48.3 and 74.6% in the femoral and lumbar bones, respectively. Low physical activity, insufficient sun exposure, diabetes mellitus, and hypogonadism were associated with low bone mass., Conclusion: Low bone mass is highly prevalent among TDT patients in Southern Iran. Insufficient physical activity and hypogonadism are the main possible associated factors, followed by DM and insufficient sun exposure.

Background: Coronavirus disease 2019 (COVID-19) outbreak is a global and challenging disease that is accompany with mortality and morbidity., Aim of Study: We evaluated the prevalence and the impact of comorbidities in thalassemia Iranian patients affected by COVID-19.  Methods: A multicenter, retrospective, cross-sectional study was conducted across all comprehensive thalassemia centers in Iran, from January to June 15th, 2020., Results: Forty-three confirmed COVID-19 thalassemia patients (32 TDT, and 11 NTDT) were detected. The mean age of patients was 35.3 ± 11.5 years (range 9 - 67); 21 females and 22 males. Overall, 78.1% of TDT and 90.9% of NTDT patients were complicated with at least one comorbidity (P: 0.656). The overall mortality rate of thalassemia patients with COVID-19 was 18.6% while 27.3% was in NTDT patients compared to 15.6% in TDT patients (P:0.401). The dead group had a non-significant higher frequency of endocrinopathies compared to the recovered group (62.5% versus 45.7% P:0.457). Ten female thalassemia patients with positive COVID-19 had hypogonadism, six patients were receiving hormone replacement therapy and all of them recovered (zero death) compared to two deaths from 4 patients who were not receiving hormone replacement therapy (P:0.133). Furthermore, the prevalence of COVID-19 in NTDT patients was significantly higher than the general population (45 per 10,000 versus 22.29 per 10,000 respectively, P:0.018) while the prevalence of TDT was almost similar to the normal population (P:0.539). The mortality rate of COVID-19 was 4.71% in the normal Iranian population compared to 18.6% in β-thalassemias (P: <0.001) at the same date., Conclusions: It is important to acknowledge that β-thalassemia patients, especially young adults/adults, have a chronic condition which may contribute to increase susceptibility to SARS-CoV-2 infection. A higher susceptibility to the infection was observed in patients with NTDT and in untreated hypogonadal female thalassemic patients. However, to confirm these data, more accurate designed studies are needed.

Background: Data on the prevalence and type of endocrine disorders in β-thalassemia intermedia (β-TI) patients are scarce. This multicenter study was designed to determine the prevalence of endocrine complications and the associated risk factors in a large group of β-TI patients., Methods: In this cross-sectional multicenter study, 726 β-TI patients, aged 2.5-80 years, registered at 12 thalassemic centers, from nine countries, were enrolled during 2017. In a subgroup of 522 patients (mean age 30.8 ± 12.1; range: 2.5-80 years) from Qatar, Iran, Oman, Cyprus, and Jordan detailed data were available., Results: Overall, the most prevalent complications were osteopenia/osteoporosis (22.3%), hypogonadism (10.1%), and primary hypothyroidism (5.3%). In the subgroup multivariate analysis, older age was a risk factor for osteoporosis (Odds ratio: 7.870, 95% CI: 4.729-13.099, P < 0.001), hypogonadism (Odds ratio: 6.310, 95% CI: 2.944-13.521, P < 0.001), and non-insulin-dependent diabetes mellitus (NIDDM; Odds ratio: 17.67, 95% CI: 2.217-140.968, P = 0.007). Splenectomy was a risk factor for osteoporosis (Odds ratio: 1.736, 95% CI: 1.012-2.977, P = 0.045). Hydroxyurea was identified as a "protective factor" for NIDDM (Odds ratio: 0.259, 95% CI: 0.074-0.902, P = 0.034)., Conclusions: To the best of our knowledge, this is the largest cohort of β-TI patients with endocrine disorders evaluated in extremely heterogenic thalassemic populations for age, clinical, hematological, and molecular composition. The study demonstrates that endocrine complications are less common in patients with β-TI compared with β-TM patients. However, regular monitoring with timely diagnosis and proper management is crucial to prevent endocrine complications in β-TI patients.

Objective: Deferasirox is a once-daily oral iron-chelation agent approved by the US Food and Drug Administration in November 2005. The authors aimed to evaluate efficacy, safety, and satisfaction of patients regarding twice-daily dose of deferasirox in patients with thalassemia who are resistant to once-daily regimen., Methods: In this historical cohort multicenter study, 34 patients with beta-thalassemia major resistant or intolerant to once-daily dose of deferasirox (35 mg/kg/d) were investigated in 2016. Patients were registered at 3 thalassemia referral centers in Shiraz, southern Iran and Tehran, the capital of Iran. All patients were followed for 1 year and monitored by regular physical examination, laboratory data, serum ferritin levels, and heart and liver T2 magnetic resonance imaging., Results: Mean age of thalassemia patients was 25.6±8.1 (8 to 40) years, including 22 female individuals and 12 male individuals. Serum ferritin levels significantly decreased during the study period (2021±955 at baseline vs. 1228±894 at the end of the study, P<0.001). Liver T2 magnetic resonance imaging of the patients demonstrated a significant improvement during the study. 73.3% of patients showed normal values at the end of study compared with 28.1% at the baseline (P<0.001). Drug side effects were reported only in 2 patients (5.8%) including 1 patient with abdominal pain and 1 with leukopenia and thrombocytopenia., Conclusions: It seems that deferasirox can be used with increased dose and twice daily with acceptable efficacy in unresponsive or intolerant thalassemia patients to once-daily dose. Close monitoring of the patients is necessary to detect and manage any possible adverse events.

Objectives: The ISTH bleeding assessment tool (ISTH-BAT) is developed for standardization of bleeding symptoms in bleeding disorders. The aim of this study is to apply this bleeding score for FXIII deficient patients and its relation to the frequency and severity of symptoms., Methods: In this cross-sectional study, 63 patients with severe FXIII deficiency were evaluated for the assessment of bleeding score according to the standard ISTH-BAT questionnaire. All patients were registered at two major thrombosis and hemostasis centers in Iran affiliated to Zahedan University of medical sciences (50 patients) and Shiraz University of medical sciences (13 patients)., Results: Significant correlations between the bleeding score and number of symptoms (r = 0.668, P < 0.001) and with a number of severe symptoms (r = 0.938, P < 0.001) were detected. There was no significant relationship between the mean bleeding score and CNS bleeding (P = 0.390)., Conclusion: The ISTH-BAT score is an acceptable bleeding assessment tool for standardization and evaluation of patients with FXIII deficiency., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Recently the low-dose tailoring method of primary prophylaxis has been introduced for previously untreated patients with hemophilia A., Objective: To evaluate the efficacy and safety of low-dose tailoring method of primary prophylaxis in previously untreated patients with severe hemophilia A., Materials and Methods: In this pre-post interventional study, 33 patients with severe hemophilia A who were previously untreated and affiliated to universities in the capital city and southern Iran were evaluated during 2014 to 2015. Modified primary continuous prophylaxis was used for patients with age below 3 years old, after first and before the second episodes of obvious clinical bleeding in large joints or large soft tissue hematoma or large amount of bleeding. Prophylaxis was started by 25 IU/kg once per week and increased to twice or 3 times a week according to defined bleeding events., Results: The median age at diagnosis and age of starting prophylaxis were: 4 months (at birth, 22 mo) and 12 months (1 to 35 mo), respectively. Mean annual bleeding rate of patients after prophylaxis was 1.08±2.21 episodes per year. None of the patients had inhibitors before therapy. Inhibitor was present in 5 patients (15.1%) after prophylaxis., Conclusions: It seems that modified primary continuous prophylaxis has been successful in reducing bleeding episodes in children with severe hemophilia A and in the texture of lower cost it can improve quality of life of these patients, especially in developing countries where financial resources are limited.

Introduction: Hypothyroidism is one the most complication due to iron overload in patients with β-thalassemia major (TM). On the other hand these patients are prone to Hepatitis C virus (HCV) infection that can cause  thyroid dysfunction by itself or as the side effect of treatment with interferon (INF) or IFN plus ribavirin. The aim of this study is to evaluate the association of hypothyroidism with HCV positivity and serum ferritin levels in patients with TM., Methods: In this cross-sectional study, 201 randomly selected patients with TM who were registered at the Thalassemia Clinic of a tertiary hospital in Shiraz, southern Iran were investigated. Thyroid function tests and serologic screening assays for HCV seropositivity (HCV Ab and HCV-RNA) were conducted for all patients., Results: Frequency of hypothyroidism was 22.9% including 19.9% subclinical hypothyroidism, 2% primary overt hypothyroidism and 1% central hypothyroidism. Eighty six patients (42.8%) were HCV Ab positive and 60 patients (29.9%) were HCV RNA positive. No significant relationship was found between hypothyroidism and HCV positivity or receiving IFN-α (P>0.05). Hypothyroidism showed a borderline significant association with high serum ferritin levels in TM patients (P=0.055)., Conclusion: Our results showed no significant association between hypothyroidism and HCV infection in TM patients. It seems that the main mechanism of hypothyroidism in our patients is iron overload; however, for better evaluation a larger multicenter study is recommended.  Also due to the importance of consequences of HCV infection, more careful pre-transfusional screening of blood should be considered in TM patients.

Along with increasing childhood cancer survival, there is increasing concern about its chronic complications. We showed that 20.5 and 45.9% of children with solid tumors in southern Iran had low bone mass for chronological age in lumbar and femoral area, which was associated with serum ferritin and hemoglobin. 52.4% of these children had vitamin D deficiency, as well., Purpose/introduction: Along with increasing the childhood cancer survival, there is increasing concern about the chronic complications of the disease and the related therapies. This study aims to compare the vitamin D status and bone mineral apparent density (BMAD) of these children with healthy ones and assess some possible associated factors., Method: This case-control study enrolled 50 children with solid tumors and their age- and sex-matched controls. Dual-energy X-ray absorptiometry was used to assess bone mineral density. Body mass index, puberty, physical activity, sun exposure, and biochemical data were assessed., Results: 52.4% of children with solid tumors had vitamin D deficiency, and there was no significant difference between the prevalence of vitamin D deficiency in patients and controls (P = 0.285). The prevalence of low bone mass for chronological age in lumbar area was 20.5 and 12.5% in patients and controls, respectively (P = 0.399). Lumbar spine BMD was associated with hemoglobin level (r = 0.468, P = 0.049), while low bone mass in femoral neck was associated with serum ferritin (859 ± 1037 in low bone mass vs. 178 ± 264 in without low bone mass, P = 0.039)., Conclusion: Vitamin D deficiency and low bone mass are prevalent among Iranian children with solid tumors. Future studies are warranted to investigate the best strategies to prevent and treat vitamin D deficiency and low bone mass in children surviving cancer.

We evaluated the correlation between thrombin generation (TG) parameters with bleeding symptoms and disease severity in patients with hemophilia. In this cross-sectional study, 59 patients with hemophilia without inhibitors and regardless of their severity were randomly selected from southern Iran and TG assays were conducted. Bleeding score (BS) was calculated by performing a clinical evaluation using Tosetto questionnaire. Only lag time showed a statistically significant correlation with BS (rs = .316,P= .016). All TG parameters except peak showed association with disease severity (P< .05). Endogenous thrombin potential showed a significant correlation with factor activity level (rs = .459,P< .001). Both lag time and start tail showed significant negative correlations with factor activity level (rs = -0.488,P< .001 andrs = - .289,P< .026, respectively). Although most of the TG parameters evaluated were not significantly correlated with the BS of patients with hemophilia, the majority of TG parameters were significantly associated with factor activity level and disease severity., (© The Author(s) 2014.)

Background: Alpha-thalassemia as one of the most common monogenetic disorders is widely spread over the Mediterranean, Southeast Asian, and Middle Eastern populations, including Iran. Although beta-thalassemia is much more common than alpha-thalassemia, alpha-thalassemia is still one of the main health problems in Iran with different mutation frequencies in various ethnic groups. So the evaluation of alpha-thalassemia mutations could be helpful to detect carriers as well as prevention strategy in Iranian population., Objectives: The aim of this study was to investigate the spectrum and frequencies of alpha-globin mutations in different ethnic groups of southern Iran., Materials and Methods: Common alpha-globin mutations were evaluated in 4010 Iranian population using a reverse dot blot for all point mutations and gap-polymerase chain reaction., Results: Out of all individuals, 3993 were distinguished as carriers of alpha-thalassemia mutations. Thirteen types of alpha-thalassemia mutations were discovered. Allele of α(3.7) mutation was the most prevalent (43.84%) followed by the α(IVS1/-5NT) allele with the prevalence of 4.91%. The less frequent alleles were Hb ICARIA and α(codon16) with the prevalence of 0.04 and 0.01%, respectively., Conclusion: Our findings are essential for carrier screening, genetic counseling, and prenatal diagnosis in order to decrease the prevalence of α-thalassemia in Iran which is one of the goals of the national screening program.

Combined factor V (FV) and factor VIII (FVIII) deficiency is a rare autosomal recessive bleeding disorder characterized by mild-to-moderate bleeding. Epistaxis, postsurgical bleeding and menorrhagia are the most common symptoms. The aim of this study is to report the phenotype-genotype characterization carried out in patients affected with combined FV and FVIII deficiency from Iran. A cross-sectional study was conducted in Shiraz Hemophilia Center, southern Iran. Twelve cases, seven men and five women coming from eight families were included in our study after taking consent form. Coagulation activity for all patients was measured. All exons and intron-exon junctions of lectin mannose binding protein 1 (LMAN1) gene and multiple coagulation factor deficiency 2 genes were amplified by PCR, and subsequently sequenced by the Sanger method. Patients[Combining Acute Accent] age ranged from 6 to 59 years mean ± SD: 23.8 ± 15.4 years and median: 22 years. No patient presented with severe bleeding symptom. Only one patient had severe FV and FVIII deficiency (both factor levels <1%). Four different type of mutations (duplication, insertion, splice site and nonsense), occurring in different locuses, were identified on LMAN1 gene in 12 Iranian patients. There was a significant correlation between FV and FVIII levels, which is indicative of association with loss of function of LMAN1 gene, and reduced plasma levels of both factors. Our study showed that all of our characterized patients with combined FV and FVIII deficiency present different homozygous mutations on LMAN1 gene introducing a premature stop codon. Larger studies are needed to calculate the correlation between factor levels, genetic and bleeding symptoms.

Intracranial hemorrhage (ICH) is one of the most severe and life-threatening manifestations occurring in the patients with factor XIII (F XIII) deficiency. The aim of this study was to describe the ICH pattern in the patients suffering from F XIII deficiency. In this case series, we investigated 38 patients with severe F XIII deficiency in south of Iran from January to May 2012. ICH pattern, neurologic complications, efficacy of treatment, and incidence of recurrence were reported. The site of ICH was intraparenchymal in 35 patients (92.1 %), subdural in 2 patients (5.2 %), and epidural hemorrhage in 1 patient (2.6 %). Besides, neurologic complications occurred in 21 patients (55.2 %), including locomotor disability in 8, psychological impairment in 7, mental disorders in 5, speech impairment in 4, and visual impairment in 2. Prophylaxis was started with a dose of 10 IU/kg Fibrogammin every 4-6 weeks for all the patients, except for one. All the patients on prophylaxis showed good response without any episodes of recurrence, except for one. The most frequent site of ICH in our patients was intraparenchymal. It seems that long-term prophylactic treatment with a dose of 10 IU/kg Fibrogammin could be effective in the prevention of CNS bleeding in the patients with F XIII deficiency. Moreover, all the patients with severe F XIII deficiency even without severe bleeding symptoms are recommended to undergo prophylactic treatment.

Hemoglobinopathies and thalassemias are the most frequent genetic hereditary disorders with an increasing global health burden, especially in low- and middle-income countries. We aimed to determine the epidemiologic pattern of hemoglobinopathies and thalassemias in individuals referred to the Haematology Research Centre, Shiraz University of Medical Sciences, Shiraz, Iran, which is the most important referral center in Southern Iran during 2006 to 2011. The most frequent abnormality was β-thalassemia (β-thal) minor (24.0%), followed by α-thalassemia (α-thal) trait (10.0%), hemoglobin (Hb) S trait (4.0%) and Hb D-Punjab trait (4.0%). Because this center is a referral center, we detected a higher prevalence compared to the normal population; however, these data could help policymakers and health service providers to better programming for prevention of births affected with Hb disorders.

Preventing the birth of children with β-thalassemia major (β-TM) is an important health issue. We investigated family planning practices and related factors among families with affected children. We selected a total of 569 parents from the parents of patients with β-TM who were registered at thalassemia referral clinics in southern Iran. Information was recorded regarding demographic variables, socioeconomic status and family planning practices. The correlations between family planning practice and related factors were evaluated. Approximately 96.0% of the parents (546) were practicing contraception at the time of the study. Only 12.8% of the families whose first child had β-TM decided to have no more children. The most frequent contraceptive method was tubal ligation (TL) (37.5%) followed by oral contraceptive pills (OCP) (31.5%). Higher education level of the mothers and higher economic status of the families were found to be related with the lower numbers of children with β-TM (p = 0.001). We found a high percentage of safe contraception being used by at-risk couples. It seems that educational programs have been effective in influencing family planning practices. Further attention should be devoted to increasing the knowledge of at-risk couples with a greater focus on parents of low socioeconomic status. Because of cultural factors in Iran, many of these at-risk couples opted to achieve the desired family size, so implementation of a well-organized prenatal diagnostic system seems necessary.

CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (β-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with β-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with β-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with β-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state.

We aimed to determine the frequency of asymptomatic brain lesions in a group of patients with β-thalassemia intermedia (β-TI) and to evaluate correlation of asymptomatic brain lesions with splenectomy, thrombocytosis, blood transfusions, and clinical parameters. Ninety five neurologically intact patients with β-TI were randomly enrolled in this cross-sectional study. Diffusion-weighted imaging brain MRI was performed in every patient to detect cerebral white matter lesions (WML). We found an overall frequency of 15 (15.8 %) for WMLs, 14 (23.7 %) in splenectomized, and 1 (2.8 %) in nonsplenectomized patients. The presence of WML was significantly associated with splenectomy (P = 0.008) and thrombocytosis (P = 0.009). However, after adjustment for splenectomy, thrombocytosis was not significantly associated with the presence of WML (P > 0.05). The number of patients with regular blood transfusions and normal MRI was not significantly higher compared to those with abnormal findings (52.5 % vs. 26.7 %; P = 0.092). In untransfused patients, hydroxyurea (HU) administration was associated with a lower incidence of WML (P < 0.001). Although in univariate analysis either splenectomy or thrombocytosis showed significant correlation with the presence of single or multiple WMLs, thrombocytosis by itself did not significantly contribute in developing asymptomatic brain lesions. The lack of significant correlation between lesions and regular blood transfusions could be related to the treatment with HU in untransfused patients, which increased fetal hemoglobin levels and improved the morphology and the pathological indices of the red blood cells. Larger prospective studies are suggested for the accurate evaluation of the correlation of these factors with developing asymptomatic brain lesions.