IRANIAN JOURNAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY
Year:2017 | Volume:7 | Issue:1|Papers Count:8

Background: Pediatric patients on chemotherapy are vulnerable to invasive fungal infection especially Candida spices. Resistance to antifungal agents has increased in Candida spp., especially in non-albicans species. This study aims to assess the susceptibility of Candida spp. strains isolated from children with acute leukemia less than 18 years. Materials and Methods: This prospective cross-sectional study was conducted during March 2011 to March 2012. Participants were 188 children aging from 1 month to 18 years, who had acute leukemia, were admitted in Amir Oncology Hospital affiliated to Shiraz University of Medical Science, Shiraz, Iran. Identification of Candida strains was performed using germ tube and chlamydospore production tests on an Application Programming Interface (API) 20 C AUX yeast identification system. Susceptibility testing for 7 antifungal agents was performed by the agar-based E-test method. Fungal cultures were carried out from nose, oropharynx, stool, and urine specimens. Results: A total of 229 yeasts were isolated. C. albicans was the most common species found, followed by C. krusei, C. parapsilosis, C. glabrata, and other Candida species. Candida glabrata was the most highly resistant of the yeasts isolated, being 100% resistant to fluconazole and itraconazole, 88% to posaconazole, and 75% to amphotericin B and ketoconazole. Conclusion: In this study, caspofungin was the most effective antifungal agent against the colonized Candida spp. found, followed by conventional amphotericin B. Knowledge about susceptibility patterns of colonized Candida spp. can be of help to clinicians managing pediatric patients on chemotherapy.

Background: Malignant neoplasms remain the second leading cause of death in children after accidents. The aim of this study was to assess the epidemiology of childhood cancer in children admitted to Dr sheikh hospital, Iran. Materials and Methods: This descriptive cross-sectional study had been done in Dr Sheikh Hospital in Mashhad University of medical science on 1764 children younger than 14 years old from 2006up to 2014 with cancer that has been pathologically confirmed. All information about the age, sex, type of cancers, and the residence of patients were collected and recorded from their medical records. Results: regarding gender, 1055 of cases were male and 709 female. The mean age of patients was 5. 8± 4. 2 years old while 30% were in age group of 3-6 years. Results showed that leukemia (56. 4%), Lymphomas (10. 3%), renal tumor (9. 3%), malignant bone tumor (4. 4%), and CNS tumor (4. 1%) were the most prevalent malignancies in children admitted to Dr Sheikh hospital in Mashhad, Iran. The most common form of leukemia was ALL (86. 9%). during the course of thestudy, the lowest and highest age standardized incidence rate was 114 (2006) and 142 (2014) cases out of each 1000, 000 person, respectively. Conclusion: Distribution of childhood cancer in terms of year revealed the increase of malignant tumor prevalence about 2. 5% from 2006 to 2014. Incidence of childhood cancers in Dr Sheikh hospital in Northeast of Iran was similar to neighboring province and other countries.

Background: In the process of cancer treatment, mothers of children with cancer experience burden of care as a result of these conditions. Early detection of burden of care has an important role in promoting health care. The aim of this study was to evaluate burden of care in mothers of children with a malignancy. Materials and Methods: This cross sectional study was done on 70 mothers of children with cancer in Oncology Department of Shahid Sadoughi Hospital, Yazd, Iran, during December 2015-March 2016. The required data were collected demographic information and burden of care questionnaire. Statistical analysis was performed using T-test, ANOVA, Pearson and Spearman correlation coefficient. Results: In this study, the mean age of mothers and children was 47. 5 ± 8. 54 and 5. 3 ± 3. 2 years old, respectively. Moreover, 12. 9٪ of mothers had low burden of care and 71. 4٪ and 15. 7٪ of them experienced moderate to severe burden of care, respectively. An inverse relation (R=-0. 322)was found between emotional burden of care and education as well as age of mothers(p<0. 05). There was significant difference between burden of care in mothers who received aid from government institutions and those who didn’ t receive help from state institutions(p<0. 05). Moreover, significant difference was seen between burden of care in mothers with special disease and mothers without special disease, and between mothers whose children had surgery and those didn’ t have surgery. Moreover, burden of care was observed in mothers who take care of affected child lonely and mothers who were not alone (p<0. 05). Conclusion: The result of this study showed that the majority of mothers of children with a malignancy had moderate to severe burden of care. Therefore, effective interventions are recommended to medical team members to reduce burden of care in mothers.

Background: Cardiac dysfunction is one of the major causes of morbidity and mortality in thalassemia patients. This study aimed to compare the effect of Brain Natriuretic Peptides (BNP) and Calcitonin Gene-Related Peptide (CGRP) with echocardiographic findings in early diagnosis of cardiac disease in major thalassemia patients. Materials and Methods: This study was performed on 80 patients among 500 major thalassemia patients and 80 healthy people. Those with metabolic, endocrine disorder, hypertension, heart failure, and valvular disease excluded from the study. These two groups were matched based on age and sex. Essential heart findings were analyzed using Mylab 60. After blood sampling, levels of CGRP and BNP were measured by use of ELISA kit from extracted plasma. Mann-Whitney test, independent t-test, and Pearson correlation were used to analyze data and P< 0. 05 was considered significant. Results: The results showed that mean age of all participants was 17. 581± 5. 344 years when distributed between case and control as 18. 21 ± 5. 14 and 16. 95 ± 5. 49 respectively without significant difference. Means of weight, length, hemoglobin, systolic and diastolic pressure were lower in patients group. The majority of echocardiography findings of left and right heart were higher in case group. The average of CGRP and BNP level were more in case group (p<0. 05). A positive correlation was observed between BNP (R=0. 229, P=0. 041) and right MPI. There was also correlation between PEP(R=0. 0238, P=0. 035), PEP/ET(P=0. 005) of right heart and peak A velocity of left heart (R=0. 245, P=0. 03) with CGRP. Conclusion: Findings of this survey showed that systolic and diastolic function of left heart would be changed in patients with major beta thalassemia. Therefore, monitoring BNP and CGRP in symptom free thalassemia patients as well as serial echocardiography is recommended.

Background: General health is related not only to physical illness but also to mental and social conditions including personality traits and the quality of life. The main objective of the current study was to investigate the relationship between five main personality factors, quality of life, and mental health in patients suffering f rom thalassemia. Materials and Methods: The current study was a correlative one. The convenient sampling method was used to select 40 people suffering from thalassemia major and 40 people suffering from thalassemia minor (age: 15-18 years old). In order to gather the required data, questionnaires of general health (GHQ), quality of life, and NEO five-factor inventory (NEO-FFI) were used. The acquired data were analyzed using step-wise regression u sing SPSS (version 20). Results: Multiple regression analysis indicated that personality traits and components of quality of life can predicte variables for the variance and mental health. . For example, 13 percent of the variance for the general health variable was explained based on the linear combination of the two variables of personality and quality of life. Neuroticism explained 26 percent of the variance for the variable of anxiety and sleep disorder and conscientiousness accounted for 36 percent of that variance. Mental health explained 20 percent of the variance for the component of social action and mental health explains 19 percent, social relations explains 23 percent, and environmental health explained 26 percent of the variance for the v ariable of depression Conclusion: The results showed that there was a relationship between components of quality of life and personality and the components of general health.

Background: The most common polymorphisms identified in the Methylenetetrahydrofolate reductase (MTHFR) gene, C677T and A1298C lead to defective activity of this enzyme and increase the risk of venous and arterial thrombosis. There are limited investigations regarding the effects of thrombogenic polymorphisms on the clinical phenotypes of rare hereditary hemorrhagic disorders like Glanzmann's thrombasthenia (GT) and the exact correlation between MTHFR polymorphisms and GT is not well established. This calls for further studies in populations with a large number of such patients. So, this study was performed to question whether coinheritance of MTHFR polymorphisms and GT can modulate the clinical phenotype of GT. Material and Methods: In the present case-control study which performed at Pathology and Stem Cell Research Center at Kerman University of Medical Sciences, 65 patients with GT and 100 normal voluntary blood donors as the control group were evaluated. The mean (SD) age of patients and the control group were 2. 33± 1. 54 years (range 0-5 years) and 2. 6± 1. 72, respectively. The detection of MTHFR C677T and A1298C polymorphisms was carried out using a Polymerase Chain Reaction-Restriction Fragment Length Polymorphism (PCR-RFLP) method. In accord with the Glanzmann's Thrombasthenia Italian Team (GLATIT) Protocol, the clinical severity of bleeding in patients with GT was determined. Two tests of descriptive statistics (i. e. frequencies) and Chi-square, using the SPSS version 19, were employed to analyze the data. Results: Based on results, there were no significant statistical differences in the prevalence of the MTHFR C677T polymorphism (P=0. 703) or the MTHFR A1298C polymorphism (P=0. 187) between patients and the control group. In addition, no association between the severity of bleeding and these polymorphisms was found (P=0. 385). Conclusions: It was concluded that the thrombogenic mutations of MTHFR do not solely modulate the severity of clinical symptoms in patients with GT.

This study presented a mini review on folic acid deficiency and recommendations for its supplementation in thalassemia intermedia (TI). TI is a clinical condition which lies between thalassemia major and thalassemia minor. Although TI patients may not need regular blood transfusion, precise diagnosis and management are critical for the prevention of clinical complications and quality of life improvement. Blood transfusion, iron chelation, and modulation of HbF are the main management strategies used for TI patients. High red blood cells turnover and nutritional deficiency in thalassemic patients lead to some vitamins and minerals deficiency as well as folic acid deficiency. Folic acid deficiency is more prevalent in TI patients compared with thalassemia major because of fewer blood transfusion which leads to higher red cells turnover. Therefore, Daily folic acid supplementation (1 mg/day) is recommended in these patients but the annual evaluation of vitamin B12 deficiency is also recommended in these patients for the prevention of its deficiency and complications.

Pleuropulmonary blastoma (PPB) is a rare, malignant intrathoracic tumor that occurs mostly in children before 5 years old. It consists about 15% of all primary pediatric pulmonary tumors and shows various mesenchymal components. Its presentations are nonspecific and common symptoms include fever, cough, chest pain, respiratory distress, and pulmonary infection. PPB should be considered in the differential diagnosis of solid and cystic lesions of thorax in children. Surgery is the main treatment of PPB that followed by chemotherapy. Radiotherapy has controversial effects on PPB. For recurrent tumors, bone marrow transplant is recommended. Two cases of this rare tumor is reported to increase the awareness about this entity and considering it in differential diagnosis of solid and cystic lesions of thorax in infants and children. The first case initially was treated with vincristine, actinomycin-D, and cyclophosphamide (VAC) regimen. Following relapse, it was scheduled with ifosfamide, vincristine, actinomycin-D and doxorubicin (IVADo) regimen and radiotherapy. Then, autologous stem cell rescue (ASCR) was recommended; but the patient was expired due to progressive disease before considering it. The second case was first treated with ifosfamide and doxorubicin alternate with ifosfamide and etoposide (IDo/ IE regimen). Following relapse, it was scheduled with ifosfamide, carboplatin and etoposide (ICE) chemotherapy regimen and he felt better.