Red Blood Cell Immunization and Contributing Factors in 685 Thalassemia Patients.

Background: An analysis of red blood cell alloimmunization in patients with thalassemia can help to devise specific strategies to decrease the alloimmunization rate. This study explored the frequency and specificity of alloantibodies and autoantibodies against red blood cell (RBC) antigens in patients with thalassemia referring to the Iranian Blood Transfusion Organization (IBTO) Immunohematology Reference Laboratory (IRL) in Tehran. Materials and Methods: This study first examined the laboratory records of 23,113 patients suffering from different diseases referring to IBTO's IRL for pretransfusion testing in the 2008-2015 period. ABO and Rh(D) typing and antibody screening tests were performed for all 23,113 patient records and 685 (2.97%) beta-thalassemia patients with positive pre-transfusion test results (antibody screening and/or DAT) were selected for further investigation. Results: The antibody screening test was positive in 640 out of 685 thalassemic patients (93.4%). DAT was performed for 529 patients, 226 (33%) of which showed positive results. Meanwhile, 161 out of 685 beta-thalassemia patients (23.5%) had positive auto control test results, reflecting the possible presence of allo- and/or autoantibodies. The most common antigen-specific alloantibodies were directed against K and E RBC antigens with a frequency of 25% (Anti-K) and 11.91% (Anti-E), respectively. The development of two antibodies (double antibodies) in one patient was observed in 80 individuals (11.46%). Conclusion: Age, gender, history of pregnancy, and splenectomy were not contributing factors to the antibody presence in the patient population under study. Extended red blood cell phenotyping should be considered as an essential procedure for expected multi-transfused thalassemia patients before blood transfusion. Considering the high frequency of anti-K and anti-E observed in this study, it is recommended that thalassemia patients in Iran are tested through phenotyping of RBC units for K and E antigens before transfusion.

The First Comprehensive Study of H-Deficient Phenotypes in Iran.

BACKGROUND: The lack of correct blood grouping practices can lead to missing of the rare Bombay Oh phenotype and subjecting patients to the risk of severe hemolytic transfusion reaction. In the absence of blood donor registry, transfusion management of patients is a challenge. We performed this study in order to estimate the prevalence of the Bombay blood group (Oh) in Iran and to determine whether consanguinity plays a role in the prevalence of Oh group. METHODS: This is a descriptive study in the Immunohematology Reference Laboratory of the Iranian Blood Transfusion Organization (IBTO) Tehran, Iran, over a period of 7 years. All donor blood samples showing blood group O and a strong initial reaction with blood group O RBC control cells were tested with anti-H lectin. Also blood samples from blood group O patients were tested with anti-H lectin if all cells on both antibody screening tests and antibody identification panels were reactive with negative auto control test. Specialized tests like adsorption/elution technique and inhibition assay for determination of secretor status were performed on Oh cases. Any history of consanguineous marriages were recorded. All variables were categorical variables, and percentage and proportions were calculated manually. RESULTS: Analysis of the results of over 7 million first-time blood donors in Iran showed that the most common ABO blood group was O, with 2,520,000 (36%) subjects. 56 Oh individuals' (donors and patients) phenotypes (0.0008%) were detected. Consanguinity was observed in 50 cases (89%). CONCLUSIONS: This study shows that the prevalence of Bombay blood group in the general population of Iran is relatively high (0.0008%) and associated with consanguineous marriage. Thus, consanguinity is still an important risk factor present.

First report of the rare RhCE-depleted D--phenotype in sixteen people of Iranian origin.

BACKGROUND AND OBJECTIVES: In transfusion medicine, it may be a challenge to acquire compatible blood for patients who have clinically important alloantibodies to high-prevalence antigens. The aim of this study was to study prevalence of rare D-- phenotype in samples from patients and their relatives referred to the Immunohematology reference laboratory of the Iranian Blood Transfusion Organization and the detection and identification of the phenotype and associated antibodies, particularly in an antenatal setting. This is the first report of the cases evaluated by the IBTO and family studies of the D-- proposita in Iran and possibly the first attempted comprehensive study in the current transfusion-related literatures. MATERIALS AND METHODS: This retrospective cross-sectional study was carried out on 6720 pregnant women and individuals with difficult positive pretransfusion testing referred for ABO/Rh(D) typing and antibody screening during a period of 8 years from 2008 to December 2016 in the Immunohematology Reference Laboratory of the Iranian Blood Transfusion Organization, Tehran, Iran. RESULTS: During 2008 to December 2016, 16 persons from ten families were detected to have rare D-- phenotype. Anti-Rh17 and anti-c were identified in plasma of the 11 persons, including 10 females with a history of multiple unsuccessful pregnancy and the total number of 24 abortions and one male with history of blood transfusion vs. 5 individuals, including an unmarried single woman, 1 person with a history of first-time pregnancy and 3 persons with a history of multiple pregnancy, who showed no alloimmunization. Based on these collective findings, we interpreted these results as being confirmed as D-- phenotype (0.23%). CONCLUSION: Irrespective of Rh (D) group a serological antibody screening test is recommended to be required in a National prenatal testing guideline.

Comparison of the Effects of Video Modeling and Clinician Modeling on Naming Deficiency in Aphasic Patients.

BACKGROUND: Aphasia is the most frequent disorder that could occur following a stroke. Aphasia has a negative impact on the patient's communication ability through language. One of the common consequences of aphasia is naming deficits that can lead to communication disorders. Therefore, the treatment of aphasia is necessary. The aim of the current study was to investigate the effect of video modeling and clinician modeling on naming skills of patients with chronic aphasia. MATERIALS AND METHODS: The design of this prospective single subject study was ABA that performed on four patients with chronic aphasia. participated. This study was administered during three phases including the baseline (three sessions); the intervention (nine sessions); and a follow-up phase (three sessions). The outcome measure was taken in three phases including baseline, intervention, and follow-up. For each patient, the naming score for items modeled by the clinician, the naming score for items modeled video modeling by other, the naming score for self-video modeling, and the reaction time score were recorded. RESULTS: A total of three patients complete the study and one of them refused to continue treatment. The naming score of all modeling types increased in all patients. In the other words, the intervention helped the patients be improved in naming. Also, the results of the reaction time indicated that the video modeling, as well as clinician modeling, could decrease the response time that means the intervention could increase the speed of retrieval processes. CONCLUSION: In our study, all three types of modeling could improve the naming scores in patients with chronic aphasia. Additionally, the findings demonstrate that the clinician and video modeling might increase mental processing for naming verbally.

Effects of multimodal communication program on patients with chronic aphasia: a single-subject A-B-A design study.

BACKGROUND AND AIM: Aphasia as a common consequence of stroke, is an acquired neurologic communication disorder that can affect symbol language processing. Different types of intervention approaches have been introduced. Multimodal Communication Program (MCP) is a new augmentative alternative communication approach in chronic aphasia. The aim of this study was to investigate the effect of MCP on communication skills of patients with chronic aphasia. METHODS: This prospective, single subject, A-B-A design study was done during 2016 in Semnan, Iran. Participants were two patients with severe aphasia with a single left-hemisphere stroke. Three phases, including baseline, intervention and follow-up were administered. The patients received nine-hour intervention, over 10 working days. RESULTS: Three different scores were calculated for each patient: verbal efforts, the frequency of each modality and the accuracy of switching between modalities and the reaction time. The frequency of verbal modality increased for both patients. They could switch between modalities more successfully than before the intervention. The results for the reaction time, however were challenging. The onset reaction time decreased for patient 1, and increased during switching between modalities, and patient 2 showed the opposite. CONCLUSION: The MCP can improve the communication skills in patients with chronic post stroke aphasia. However, some factors, such as reduction of the patients' reaction time is probably related to the amount of allocated resources during intervention. TRIAL REGISTRATION: The trial was registered at IRCT center with ID: IRCT2016032325194N3. FUNDING: The study was financed by Semnan University of Medical Sciences (Grant no.: A-10-333-3).

First Report of Known Rare Rhnull Phenotype Individuals in Iran.

Rhnull phenotype is a rare blood group with a frequency of approximately 1 in 6 million individuals, transmitted via an autosomal recessive mode. It is characterized by the weak (Rhmod) or lack (Rhnull) of expression of all Rh antigens on the red cells. The clinical significance of its assessment is that such patients with Rhnull syndrome are associated with chronic hemolytic anemia of varying degrees. Another clinical importance is that such subjects readily form alloantibodies when exposed to Rh antigens.We report herein a rare Rhnull phenotype in a sibling, which was detected as a part of the difficult sample work-up for red cell antibody screening and identification.

Health-related quality of life in Iranian adult men with severe hemophilia.

: Health-related quality of life (HRQOL) assessment should be part of the regular clinical assessment of persons with hemophilia. This study assessed quality of life of severe hemophilia patients (Type A) in Tehran comprehensive hemophilia care center. This cross-sectional study was done in 2016 and 84 men aged over 20 years with severe hemophilia (Type A) were assessed. All patients have been treated over 10 years at the comprehensive hemophilia care center, Iran. The HRQOL assessment includes the A36 Hemophilia-QOL questionnaire, sociodemographical and clinical characteristics. The overall HRQOL mean (±SD) in the present sample was 69.1 ±â€Š26.0 (A36Hemofilia-QoL Global Index). The mean (±SD) scores according the domains were: physical health (15.0 ±â€Š5.7), daily activities (6.7 ±â€Š3.8), joint damage (4.2 ±â€Š2.9), pain (3.0 ±â€Š1.9), social activities and relationships (10.7 ±â€Š5.8) and emotional function (9.2 ±â€Š5.0) classified moderate-to-poor state. In contrast with treatment satisfaction (4.8 ±â€Š1.6), treatment difficulties (9.5 ±â€Š3.4) and mental health (6.2 ±â€Š3.3), those were all in moderate-to-good state. Patients with higher education levels had better quality of life in the area of anxiety (P = 0.034), mental health (P = 0.007), social activities and personal relationships (P = 0.002). In general, poor quality of life of patients was 13.1%, 42.9% weak-to-moderate, 38.1% moderate-to-good and 6% good. It was shown that over 50% of patients had low-to-moderate HRQOL in the domains, such as physical health, daily activities, joint damage, pain, social activities and personal relationships. Efforts are necessary to improve their HRQOL.

A Rare Case of Vasculitis Patched Necrosis of Cecum due to Behçet's Disease.

Isolated cecal necrosis is a rare form of acute ischemic colitis and a rare cause of surgical abdomen. Behçet's disease is a multisystemic autoimmune condition which can induce vasculitis. This can result in cecal necrosis while disease involves colon vessels. We describe a patient with complicated Behçet's disease and ischemic colitis admitted to our hospital. Patient was a 62-year-old female with more accompanying diseases. Histopathologic findings confirmed the diagnosis of ischemic colitis and regarding patient's vision problem and skin lesions, rheumatologic tests were performed which were positive for HLAB5 and HLAB51 suggestive of Behçet's disease; the patient was improved with surgery. Abdominal pain can indicate a disease with vascular involvement like Behçet's disease, especially in the presence of other clinical findings suggestive of the disease including blurred vision and skin lesions. An abdominal computerized tomography (CT) scan is very diagnostic in the same patients.

Utility of the CT Scan in Diagnosing Midgut Volvulus in Patients with Chronic Abdominal Pain.

Symptomatic intestinal malrotation first presenting in the adults is rare. Midgut volvulus is the most common complication of malrotation in the adults. Because of more differential diagnosis, Computed Tomography (CT) scan can play an important role in the evaluation of patients with this abnormality. The whirl pattern around the superior mesenteric artery found on CT scan in patients with midgut volvulus is pathognomonic and diagnostic. We describe a case of intestinal malrotation complicated by midgut volvulus in an adult patient. The preoperative CT findings were pathognomonic.

Maternal red blood cell alloantibodies identified in blood samples obtained from Iranian pregnant women: the first population study in Iran.

BACKGROUND: The objective was to determine the frequency of occurrence of alloantibodies among pregnant women in Iran. STUDY DESIGN AND METHODS: This was a prospective cross-sectional study, which was carried out in the immunohematology reference laboratory of the Iranian Blood Transfusion Organization in Tehran, Iran, in 2008 to 2015. Screening and identification of red blood cell (RBC) alloantibodies was done on the sera of 7340 pregnant females using the standard tube method and gel column agglutination technique. RESULTS: Alloantibodies were identified in the serum of 332 of the 7340 (4.5%) pregnant women. A total of 410 antibodies were detected in 332 positive maternal serum samples with no previous history of blood transfusion. Anti-D was the most common antibody accounting for 70.5% of all the antibodies formed in D- women. The incidence of specific alloimmunization other than Rh group was 14.4%. CONCLUSION: We concluded that the alloimmunization rate was high in comparison with wide pattern in previous studies. In Iran, like other developing countries, alloimmunization screening tests are performed only to detect anti-D in pregnant D- women. This high rate of alloimmunization, quite possibly, is due to the fact that the majority of blood samples came from pregnant women known to have previous obstetric problems. However, we suggest that RBC antibody screening tests should be extended to all D+ women.

The Main Causes of Bacterial Colonization in Endotracheal Tubes and Tracheal Secretions in Neonates Admitted to the Neonatal Intensive Care Unit.

BACKGROUND: Ventilator-associated pneumonia (VAP) is the second most common nosocomial infection in neonates, admitted to neonatal intensive care units (NICUs). The aim of this study was to identify the main causes of bacterial colonization in endotracheal tubes and tracheal secretions of neonates hospitalized in the NICUs of our university hospitals. MATERIALS AND METHODS: This cross sectional study was conducted during 2015-2016. Thirty-nine neonates, who were under mechanical ventilation in the NICUs of Baqiyatallah and Najmiyeh hospitals of Tehran, Iran, were assessed. The patients were selected using the census sampling method. Gestational age, birth weight, duration of mechanical ventilation, length of hospital stay, tracheal discharge culture, endotracheal tube culture, blood culture, and chest radiography were evaluated. RESULTS: In a total of 39 neonates (50.3% males and 49.7% females) with the mean age of 1.17±1.12 days, bacterial growth was reported in 6 (15.3%) cases. The antibiotic study of tracheal secretion and endotracheal tube cultures showed that 2.6% of patients were resistant to cephalosporins, aminoglycosides, nitrofurantoin, and carbapenem. Moreover, 12.8% were also resistant to fluoroquinolones, besides these antibiotics. CONCLUSION: Tracheal secretion culture is a simple and proper approach for the diagnosis of VAP. Besides reducing the costs, this method can play a significant role in decreasing the incidence of infections.

Central Nervous System Toxoplasmosis in Relapsed Hodgkin's Lymphoma: A Case Report.

INTRODUCTION: Patients with immunosuppression have an increased incidence of toxoplasmosis characterized by involvement of the central nervous system. Only a few cases of toxoplasmosis associated with immunosuppressive agents have been reported. Such cases have been reported in immune suppressed patients outside the Iran, but a search of the literature has not revealed any previous reports from this country. CASE PRESENTATION: We described a 17- year -old male, a known case of Hodgkin's lymphoma with the diagnosis of central nervous system (CNS) toxoplasmosis. CONCLUSIONS: As a conclusion, CNS toxoplasmosis should be considered in the differential diagnosis of immunosuppressed patients who present with neurological manifestations.

Continuous Positive Airway Pressure Compliance in Patients with Obstructive Sleep Apnea.

BACKGROUND: Obstructive sleep apnea (OSA) is a common condition in adults. In most cases, first-line therapy includes treatment with positive airway pressure devices. However, because of discomfort, continuous positive airway pressure (CPAP) compliance is often poor. To determine the willingness of patients to use CPAP device, the relationship of demographic and polysomnographic variables with tolerance and the willingness to use CPAP, was evaluated. MATERIALS AND METHODS: In this cross-sectional study, 120 OSA patients who were treated with CPAP in Baqiyatallah Hospital, Tehran, Iran, were selected by convenience sampling. Polysomnographic variables, willingness to use CPAP for short and long periods of time and possible complications were evaluated. RESULTS: One hundred-twenty cases with a mean age of 53±10.3 years were assessed. The mean Epworth Sleepiness Scale (ESS) score was 11.9 ± 6.2 in CPAP users versus 11.8±6.1 in patients who did not use CPAP. The willingness to use CPAP for short-term was significantly different between the two groups (P=0.008). The average minimum oxygen saturation rate of patients was 75.21% in CPAP users versus 71.63% in non CPAP users. Also, the average desaturation index was higher in CPAP users (54.5 vs. 44.98). The mean ESS was 14.03 ± 6.19 in those who accepted long-term treatment versus 8.85 ± 4.89 (P=0.003). Skin wounds and rhinitis were reported in 4.1% and 4.1% of patients, respectively. CONCLUSION: It is concluded that high CPAP compliance rates are achievable through comprehensive CPAP therapy.

A Review of the Clinical Implications of Breast Cancer Biology.

BACKGROUND: Histologically similar tumors may have different prognoses and responses to treatment. These differences are due to molecular differences. Hence, in this review, the biological interaction of breast cancer in several different areas is discussed. In addition, the performance and clinical application of the most widely-recognized biomarkers, metastasis, and recurrences from a biological perspective and current global advances in these areas are addressed. OBJECTIVE: This review provides the performance and clinical application of the most widely-recognized biomarkers, metastasis, and recurrences from the biological perspective and current global advances in these areas. METHODS: PubMed, Scopus, and Google Scholar were searched comprehensively with combinations of the following keywords: "breast cancer," "biological markers," and "clinical." The definition of breast cancer, diagnostic methods, biological markers, and available treatment approaches were extracted from the literature. RESULTS: Estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor-2 (HER-2), and Ki-67 are the most well-known biological markers that have important roles in prognosis and response to therapeutic methods. Some studies showed the response of ER-positive and PR-negative tumors to anti-estrogenic treatment to be lower than ER-positive and PR-positive tumors. Patients with high expression of HER-2 and Ki-67 had a poor prognosis. In addition, recent investigations indicated the roles of new biomarkers, such as VEGF, IGF, P53 and P21, which are associated with many factors, such as age, race, and histological features. CONCLUSION: The objective of scientists, from establishing a relationship between cancer biology infrastructures with clinical manifestations, is to find new ways of prevention and progression inhibition and then possible introduction of less dangerous and better treatments to resolve this dilemma of human society.

Antioxidant and Anti-Inflammatory Effects of Coenzyme Q10 on L-Arginine-Induced Acute Pancreatitis in Rat.

This study was aimed at evaluating the protective effect of coenzyme Q10 on L-arginine-induced acute pancreatitis in rats regarding biomarkers and morphologic changes. Thirty-two male Sprague-Dawley rats were divided into 4 equal groups. Control group received intraperitoneal normal saline, while in sham and experimental groups 1 and 2 pancreatitis was induced with L-arginine. E1 and E2 groups were treated with a single dose of 100 and 200 mg/kg Q10, respectively. Serum lipase and amylase, along with pancreas IL-10, IL-1ß, and TNF-α, were measured. For evaluation of oxidative stress, pancreatic superoxide dismutase (SOD), glutathione (GSH), malondialdehyde (MDA), and myeloperoxidase (MPO) were assessed. Histopathological examination for morphologic investigation was conducted. Serum amylase and lipase, as well as TNF-α and IL-1ß cytokines, reverted with administration of Q10 in consistence with dosage. In contrast, Q10 assisted in boosting of IL-10 with higher dosage (200 mg/kg). A similar pattern for oxidative stress markers was noticed. Both MDA and MPO levels declined with increased dosage, contrary to elevation of SOD and GSH. Histopathology was in favor of protective effects of Q10. Our findings proved the amelioration of pancreatic injury by Q10, which suggest the anti-inflammatory and antioxidant property of Q10 and its potential therapeutic role.

The Relation of Pre and Postnatal Depression and Anxiety with Exclusive Breastfeeding.

INTRODUCTION: Studies on the relationship between prenatal psychosocial risk factors and breastfeeding are disjointed. Maternal health status has a great influence on breastfeeding, growth, and general health of the neonates. The aim of this study was to ascertain the relation between maternal mental health and breastfeeding status of mothers in Kazerun (Fars province, Iran). METHODS: This cross-sectional study was conducted on a total of 92 pregnant women recruited from clinics in Kazerun in 2015. Participants were divided into two groups subject to exclusive breastfeeding. Spielberger Anxiety Inventory, Beck Depression Inventory and Edinburgh Postnatal Depression Scale (EPDS) were used for data collection. Data analysis was performed by the SPSS version 16 using descriptive statistics, Chi-square, and Fisher's exact test. RESULTS: The average of state anxiety score according to Spielberger Anxiety Inventory was 1.97±0.51 in the exclusive breastfeeding group versus 1.77±0.66 in the non-exclusive breastfeeding group (p = 0.229). The mean trait anxiety score according to Spielberger Anxiety Inventory was 1.98±0.44 in the exclusive breastfeeding group versus 2.22±0.44 in the non-exclusive breastfeeding group (p=0.132). An average of Beck Depression Inventory score was 1.35±0.84 totally (1.33±0.81 in the exclusive breastfeeding group vs. 1.33±1.00, P=0.584). An average of EPDS score was 1.65±1.11, 1.64±1.11, and 1.44±0.88 in total, in the exclusive breastfeeding and non-exclusive breastfeeding groups, respectively (p=0.604). CONCLUSIONS: Screening anxiety and depression before and during pregnancy appeared to be necessary.

Depression and Associated Factors in Patients with Implantable Cardioverter-Defibrillators.

Background: Psychological problems such as depression constitute a dilemma that patients with the implantable cardioverter-defibrillator (ICD) usually encounter and may impact their quality of life. The purpose of this study was to evaluate the prevalence of depression among adults with the ICD and the relationship between depression and associated factors. Methods: Totally, 115 individuals with the ICD participated in this cross-sectional study in Tehran, Iran, and depression and other related risk factors were evaluated in them. Two questionnaires, one for demographic characteristics and the Beck Depression Inventory, were applied for data acquisition. The data were analyzed, and the factors associated with depression in the patients with the ICD were assessed. Results: The mean age of the study population was 59.85 ± 11.46 years. Males comprised 88 (76.5%) and females 27 (23.5%) of the patients. The multivariate analysis on the 115 patients revealed that frequency of ICD shocks (OR = 1.08, 95%CI: 1.02 - 1.10), male sex (OR = 2.28, 95%CI: 1.027 - 5.07), more hospital admissions (OR = 1.19, 95%CI: 1.11 - 1.25), smoking cessation (OR = 9.8, 95%CI: 4.48 - 20.07), presence of ICD shocks (OR = 4.5, 95%CI: 2.45 - 7.38), dyslipidemia (OR = 2.8, 95%CI: 1.22 - 4.95), and family history of depression (OR = 6.4, 95%CI: 3.0 - 13.46) were significantly and independently associated with the Beck score classifications. Conclusion: These findings suggest that a poor psychosocial outcome in patients with the ICD may occur as a result of a variety of associated factors, most of which are predictable and preventable.