Impact of education on awareness towards reproductive health in women with beta-thalassemia major

Objectives: This study aimed to investigate the effect of nursing educational programs on the awareness of females with beta-thalassemia major [beta-TM] regarding their disease and reproductive health

Methods: Thirty-nine consecutive female patients with beta-TM [age range between 20 and 32 years] participated in this interventional pre-post study. All patients attended the Thalassemia Clinic of Dastgheib hospital, a referral governmental center in Shiraz, Southern Iran. Awareness of patients regarding reproductive health was evaluated by a designed questionnaire. The intervention consisted of nursing educational program regarding important issues related to reproductive health of female patients with beta-TM. Knowledge of patients was revaluated and compared with their knowledge before education. Data were analyzed using SPSS software v.21 using Paired t-test, Pearson correlation test and Mann-Whitney test

Results: Total awareness score significantly increased after education [mean +/- standard deviation [SD]: 16.12 +/- 1.67 vs. 13.69 +/- 2.35, P < 0.001]. Increased knowledge of patients after the intervention was not significantly associated with educational level or with age of the patients [P = 0.058 and P = 0.395, respectively]

Conclusions: An educational program can be helpful in increasing awareness of females with beta-TM regarding their disease and reproductive health issues resulting in increased life expectancy and quality of life

Effect of educational-spiritual intervention on the burnout of the parents of school age children with Cancer: a randomized controlled clinical trial

Background: Parents of children with cancer experience high levels of stress and discomfort. Religious beliefs are important sources of comfort and support for many cancer patients and their families. The present study aimed to assess the effect of educational-spiritual intervention on burnout of the parents of the children with cancer

Methods: In this randomized clinical trial, 135 parents of children with cancer were randomly assigned into intervention and control groups. Data were collected through SMBQ [Shirom and Melamed Burnout Questionnaire] from both groups, before, immediately after and one month after the intervention. Educational-spiritual programs were held for six weeks, one session every week. The data were analyzed by SPSS using independent t-test, and repeated measure ANOVA

Results: The results showed that the mean burnout score before the intervention in the intervention group was 4.28+/-0.61 and in the control group it was 4.23+/-0.50; most of the parents reported moderate to high burnout. But, there was a significant difference between the intervention and control groups immediately after and one month after the intervention [t=10.16, P<0.0001]. The mean burnout score in the intervention group was less than the control group. Results also showed that there was a significant difference between the two groups in terms of parental burnout in three times of measurements [F=58.62, P<0.0001]

Conclusion: This study indicated that educational-spiritual intervention was effective on reduction of the burnout of the parents of the children with cancer. Due to high burnout of most of the parents, offering such a program could be beneficial for them. More studies in this regard are recommended

effect of yoga on the quality of life in the children and adolescents with haemophilia

The problems caused by haemophilia lead to impairments of the quality of life in patients with haemophilia. This study aimed to investigate the effects of yoga on quality of life in the children and adolescents with haemophilia in Shiraz, Iran. This semi-experimental study with pre- and post-test design was performed on 27 boys between 8 and 16 years old who suffered from haemophilia. The patients were divided into two groups. The number of bleedings, absences from school, and referrals to the clinic was evaluated. The demographic data were collected through interviews and using the patients' records in the haemophilia center. Besides, the quality of life was assessed through the Haemo-QoL questionnaire. Then, the yoga intervention was performed for 14 weeks and the data were collected in three stages. The collected data were entered into the SPSS statistical software, version 18 and were analyzed using non-parametric Friedman test. After the intervention, significant differences were observed in the mean scores of quality of life dimensions and the number of bleedings, school absences, and referrals to the haemophilia clinic [P<0.001]. Thus, yoga may improve the haemophilia children's and adolescents' perception of quality of life without the risk of injury. This intervention also seemed to be effective in reducing the number of bleedings, referrals to the haemophilia clinic, and absences from school

Effect of coping strategies training on its use by thalassemia major adolescents: a randomized controlled clinical trial

Thalassemia is a chronic disease with serious clinical and psychological challenges. The incidence of thalassemia in a family member may cause a psychological crisis in all family members and in this case coping strategies are required. This clinical trial study aimed to determine the impact of training coping strategies on their use by major thalassemic adolescents referred to Dastgheib Hospital in Shiraz. In this randomized clinical trial, 87 adolescents with major thalassemia who were randomly assigned to two groups of experiment and control were enrolled. Then the experiment group attended six sessions of coping strategies training program in isolation, each lasting for one and half hour. In order to examine the coping strategies, Jalowice's coping strategy questionnaire was used in three periods including pre-intervention period and one month and two months after the intervention. The collected data were analyzed using independent t-test and Chi-square. Mean scores of problem-focused coping strategies in the experiment group increased in one month and two months after the intervention from 45 +/- 12.7 to 54.8 +/- 7.3 and 55.7 +/- 7.2, respectively. Also, the difference in mean scores of problem-focused coping strategies was significant in the two groups [P<0.001]. Furthermore, the difference in mean scores of emotion-focused coping strategies was significantly different between the two groups in two months after the intervention [P=0.007]. Based on the obtained results, teaching coping strategies has improved the use of problem-focused coping strategies and also effective coping with stress and disease problems in patients with thalassemia major. Therefore, it is recommended that authorities should consider this as a part of treatment program

Hypothyroidism in beta-thalassemia intermedia patients with and without hydroxyurea

Hydroxyurea [HU] has been successfully used in patients with beta-thalassemia intermedia [beta-TI]. We aimed to evaluate the effect of the long-term use of HU on thyroid function in patients with beta-TI. Seventy-five patients with beta-TI aged?11 years and taking HU were randomly selected during 2010 in southern Iran. Thirtyone patients with beta-TI without HU were considered as a control group. Serum levels of thyroid stimulating hormone [TSH] and T4 were measured. The mean age of the participants was 22.7 +/- 5.1 years [age range=12-41 years]. Serum ferritin level had no significant correlation with HU consumption [P>0.05]. Overall, we detected 10 [9.4%] patients with hypothyroidism. We found that the use of HU at a dose of 8-15 mg/kg/day has no significant association with thyroid function in beta-TI patients. However, due to the small sample size in our study, documentation of this finding needs further studies with higher numbers of patients

quality of life of children with hemophilia in Shiraz, Iran

Hemophilia might impact the quality of life [QOL] in children and adolescent. This study aimed to assess the quality of life in children with hemophilia and identify the factors that predict their QOL. It was a cross-sectional study. A consensus sample of twenty-seven male children aged 8-16 years old with hemophilia participated in this study during 2011. The Haemo-QoL questionnaire version age group II [8-12 years old] or age group III [13-16 years] was used to measure the quality of life in children with hemophilia. Data were analyzed in SPSS using Spearman's rho, Pearson correlation coefficients, and multiple regression analysis. The total mean score on the Haemo-QoL was 54.1 +/- 7.3. Haemo-QoL correlated with the number of bleeding event and school absence, family income, mothers' education level, severity of hemophilia, person who performs his/her infusions, insight to health status and insight to suffering from hemophilia [P0.05]. QOL was predicated by family income [fiN-0.69; explained 73% of variance] and infusion of the clotting factor replacement by parents [P=-0.33; explained 81% of variance]. The QOL of a sample of male children with hemophilia was impaired. Moreover, number of bleeding event, number of school absences, mother s' education level, severity of hemophilia, insight to health status and insight to suffering from hemophilia were associated with Haemo- QOL. In addition, family income and parent- administration of the product were the factors which predict the QOL in children with hemophilia

Bacterial contamination of expressed breast milk in neonatal intensive care unit

The milks expressed from the mothers' breast might be infected during squeeze, storage and/or transmission. The infection level has been reported as different in various studies up to 97 percent. The main purpose of this study is to determine the infection level and its relevant organisms as well as to specify drug allergy of the expressed milks from the mothers with their infant admitted to NICU ward. In this study, among the expressed milks from 80 mothers, were cultured each in an amount of 0.5-1cc and antibiotic discs selected for every strain was placed. The results indicate that 85 percent of samples were infected and dominant microorganisms were firstly Klebsiella [13.7%] and then S. epidermidis [12.5%]. In addition, 95% of Gram negative bacteria strains were susceptible to imipenem. The most effective antibiotic on isolated staphylococci was ceftizoxime [46.6% resistance]. The colony count in 32.4% gram negative bacteria and in 66.7% gram positive bacteria was between 10[4] to 10[5]CFU/ml and the remaining was above 10[5]CFU/ml [p=0.02]. Furthermore, there was no significant relationship between bacterial infection of the expressed milks with the site of milk expressing [house or hospital], mode of expressing[by pump or hand], storage duration and the mother's demographic characteristics including age and/or literacy. The studies show that infection prevalence in the milk samples was 85%; the most common infection factor was Klebsiella and then S. epidermidis that is indicative of high prevalence of hospital infection [nosocomial infection] in the infants ward

Diffuse hepatic calcifications in a transfusion- dependent patient with beta-thalassemia: a case report

Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusiondependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain. Computed tomography scan of the abdomen revealed diffuse hepatic calcification in the right, left, and caudate lobes of the liver. Her medical history disclosed hypoparathyroidism as well as chronic hepatitis C virus infection, which was successfully treated but led to early micronodular cirrhosis on liver biopsy. Other studies done to search for the cause of hepatic calcification failed to reveal any abnormalities. We suspect that hypoparathyroidism caused liver calcification, and should be, therefore, considered in the differential diagnosis of hepatic calcification if other causative factors have been ruled out

Survival rate of childhood leukemia in Shiraz, southern Iran

Leukemia is the most prevalent type of cancer in children. The aim of this study Was to estimate the 5-year survival rates of Acute Lymphoblastic Leukemia [ALL] and Acute Myeloblastic Leukemia [AML] as well as factors influencing them. This is a nonrandomized retrospective study conducted on 280 patients with ALL and AML. They were all below 15 years old children admitted to Shahid Faghihi hospital, Shiraz, Iran from 2004 to 2008.The survival rates were estimated by applying the Kaplan-Meier method. In addition, the log rank test was used to estimate the statistical significance of differences in the survival probability. Cox regression model was applied to conduct multivariate analysis for adjusting confounding variable. All analyses were performed in SPSS statistical software [version 16]. P-values less than 0.05 were considered as statistically significant. The mean [ +/- standard deviation] of the observation period was 28.2 +/- 16.1 months. In this period, 50 [24.7%] patients [47 ALL and 15 AML] passed away. The cumulative rate of survival in this study was 53.3 +/- 0.1 percent. This probability was 56.6 +/- 0.1% and 44.2 +/- 0.1% for ALL and AML patients, respectively, which indicates no statistically significant difference between them [P=0.8]. According to Cox model, there was a significant relationship among the variables of platelet count and relapse with the survival rate. Platelet count was identified as a positive prognostic factor of the survival rate in ALL patients. However, on the base of our results and other studies, incidence of relapse and the number of relapses are significant factors of survival rates of leukemia

effect of educational intervention on decreasing mothers' expressed breast milk bacterial contamination whose infants are admitted to neonatal intensive care unit

Various reasons accounted for the infection of infants kept at Neonatal Intensive Care Unit [NICU]. Expressed breast milk may be regarded as a source of infection in infants. This study investigated the source of bacterial contamination and the impact of educational interventions on the contamination of mothers' expressed breast milk [EBM] level whose infants are hospitalized at NICU. Fifteen mothers used to express breast milking their infants admitted at NICU, involved in this study was conducted during October 2011-March 2012. Samples taken from hands, breast, pumps, breast milk, and milk storage containers and therefore 244 samples were prepared by sterile cotton swab and cultured on Blood Agar and EBM. After presenting enough training to mothers, cultures of the same positions were carried out again. Only those samples proved infected that number of their bacterial colonies exceeded 104 cfu / ml or even there was a growth of pathogenic organism. Before intervention 80% of mothers had infected by at least one sample that reduced to 36% after the intervention. Before intervention 25.4% of samples were contaminated; however after intervention, it reduced to 8.2%. The main source of contamination was milk containers and pumps; moreover, Pseudomonas, E-coli, and Klebsiella were among the most common bacteria of samples' contamination. The possibility of EBM contamination was relatively high but educational interventions might reduce the risk of prevalence

Nicolau syndrome following intramuscular penicillin injection

Nicolau Syndrome [NS] is a rare but severe localized adverse reaction at the site of intramuscular drug injection. The typical presentation is intense pain around the injection site soon after injection, followed by erythema, purplish network discolouration of the skin, haemorrhagic patch, and finally tissue necrosis. Here in, we report a 9 years old boy, the third Nicolau Syndrome [NS] reported from Iran after a single intramuscular injection of penicillin

Major causes of hospital admission in beta thalassemia major patients in southern Iran

Beta thalassemia major is a prevalent hereditary disease in Mediterranean region especially Iran. Early blood transfusion is necessary for most of the patients and frequent transfusion can cause various medical problems for the patients. The aim of this study was to find major causes of hospital admission in beta thalassemia major patients to reach the accurate preventive and therapeutic plans for these patients. Four hundred twenty six patients were admitted to the Nemazee Hospital [the main University referral Hospital Center affiliated to Shiraz University of Medical Sciences in Pars Province, southern Iran] during 3 years period [January 2007 to January 2010]. A questionnaire was filled containing age, gender, hemoglobin level, frequency of blood transfusions, deferoxamine injection, cause of hospital admission and hospital course. The mean age of patients was 11.28 years. The mean serum ferritin level was 1820 +/- 749 Hg/lit. Two hundred fifty five [59.75%] patients were male and 171 [40.25%] patients were female. The top five most prevalent causes of hospital admission were splenectomy [21.8%], infections [19.9%], congestive heart failure [19.0%], diabetes mellitus [13.4%], and Liver biopsy [11.5%]. [P=0.0002]. Results of this study revealed that infections and complications due to iron overload are major causes of hospital admission in beta thalassemia major patients

pregnancy outcome in patients with minor beta-thalassemia

beta-thalassemia is the most common hereditary disease in Iran and more than 2 million carriers of the beta-thalassemia mutant gene are living in this country. To determine pregnancy outcome of women with beta-thalassemia minor. In this retrospective, case-control study in two universities affiliated hospitals in Shiraz, all pregnancies occurred between 2006 and 2008 were included. Patients were divided in two groups regarding the presence of beta-thalassemia minor. Patients in case and control groups were matched according to maternal age, gestational age and number of previous pregnancies. Cesarean delivery, hypertensive disorders, gestational diabetes mellitus, premature rupture of membranes and preterm labor were recorded in each group and were compared using the lampda 2 or Fisher exact tests. Overall 510 beta-thalassemia minor subjects and 512 healthy controls were studied. Cases with beta-thalassemia minor had significantly higher prevalence of oligohydramnios [p<0.001] and cesarean section delivery [p=0.001]. There was no significant difference regarding Apgar score in 1[st] [p=0.65] and 5[th] minute [p=0.25], IUGR [p=0.073], gestational diabetes mellitus [DM] [p=0.443] and preeclampsia [p=0.116] between two study groups. beta-thalassemia minor does not significantly influence the pregnancy outcome in the negative way

Pet exposure and the symptoms of asthma, allergic rhinitis and eczema in 6-7 years old children

Allergic diseases are frequent in children and their prevalence and severity differ in the different regions of the world. The association between pet ownership in childhood and subsequent asthma and sensitization is very controversial. In our survey conducted with standardized method [International Study of Asthma and Allergies in Childhood], 3200 children 6-7 years old were questioned regarding asthma, allergic rhinitis and eczema. The prevalence of Attacks and shortness of breath with wheezing during last 12 months in the children who had exposure to pets in the first year of life was 34.3% that was less than children who had not exposure [OR=3.06, 95% confidence interval [CI] 1.14-8.21, P=0.021]. Also during the past 12 months the prevalence of night dry coughs, allergic rhinitis symptoms and eczema symptoms in those who had pet exposure in the first year of their life was lower than the children did not have it. However there was no significant difference in some other symptoms of asthma in two groups. Our findings suggest that pet exposure in the first year of life can have a protective effect on asthma, allergic rhinitis and eczema

Non-hodgkin lymphoma in a childe with schimke immuno-osseous dysplasia

Schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterizedsteroid-resistant nephritic syndrome, immunodeficiency, and spondyloepiphy-seal dysplasia. Mutations in SWI/SNF2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 [SMARCAL1] gene are responsible for the disease. The present report describes, for the first time, a Schimke immuno-osseous dysplasia child with SMARCAL1 missense mutation [R561H] and manifestations of intussusception secondary to Epstein-Barr virus-negative non-Hodgkin lymphoma, who expired due to septicemia following chemotherapy. The report emphasizes the necessity of more limited immunosuppressive protocols in Schimke immuno-osseous dysplasia patients with lymphoproliferative disorders

Efficacy of carvedilol in patients with dilated cardiomyopathy due to beta-thalassemia major; a double-blind randomized controlled trial

Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging [TDI] data were collected from each patient. Eight patients received Carvedilol [Group 1] and six received placebo [Group 2]. The mean age of patients in Group1 and 2 were 16 +/- 0.7 years and 17 +/- 3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association [NYHA] classification, Ejection fraction, End diastolic dimension changes, TDI systolic[S], early [Ea] and late [Aa] diastolic waves were not statistically significant in these two Groups [P>0.05]. Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1 +/- 0.37 m/s to 1.8 +/- 0.40 m/s and from 1.34 +/- 0.30 m/s to 2.6 +/- 0.23m/s respectively [P=0.04]. Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients

Frequency of sensory neural hearing loss in major beta-thalassemias in southern Iran

The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major beta- thalassemias transfusion dependent patients in south of Iran. A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of beta- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine [DFO] dose, mean daily doses of DFO [mg/kg] and audiometric variables was recorded. Out of 308 cases, 283 [96.5%] had normal hearing and 10 [3.5%] sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity

Leukemic infiltration of the appendix as an unusal site of extramedulary relapse: report of two cases and review of the literature

The appendix is an unusual site for extramedulary relapse in acute leukemia. The present case report describes two cases of late course acute lymphoblastic leukemia presented with leu-kemic infiltration of the appendix and complete remission of bone marrow. The signs and symptoms of the cases suggest that leukemic involvement of the appendix should be considered in the differential diagnosis of leukemia patients presenting with acute abdomen