RESUMO
Background: Adenosine deaminase deficiency 2 (DADA2) is an autoinflammatory disorder, caused by the CECR1 gene mutation. The major clinical manifestations include recurrent vasculitis, neurological disorders such as stroke, hematologic abnormalities, and immunodeficiency. As reported in previous studies, DADA2 may be manifested by ischemic or hemorrhagic strokes. This disorder also includes various hematological manifestations (pure red cell aplasia, pancytopenia, hemolytic anemia, and pancytopenia with bone marrow involvement). Case Presentation. In this case report, we present the clinical and immunological findings of two unrelated patients with DADA2. The first patient was a 7-year-old female who experienced recurrent neurological symptoms such as vertigo, tinnitus, hearing loss, and right-sided hemiparesis. Her brain magnetic resonance imaging (MRI) revealed a left-sided stroke, and she responded well to antitumor necrosis factor alpha agents and plasmapheresis. The second patient was a 6-year-old female who had recurrent fever and bicytopenia, aphthous lesions, cervical lymphadenopathy, and elevated liver enzymes. We also discussed the strategies used to manage the clinical manifestations in these two DADA2 patients. Conclusion: In this case report, we discussed two cases with DADA2 deficiency and their respective manifestations. The first case showed neurological symptoms while the second case had hematological symptoms. Although there is no established treatment for DADA2 due to its rarity, steroids are commonly used to treat this disorder. Antitumor necrosis factor is also effective in controlling the symptoms, especially the neurological ones. In cases where there is no appropriate response to these treatments, hematopoietic stem cell transplantation can be beneficial.
RESUMO
We conducted a hospital-based case-control study to explore the association between proximity to various land use types and childhood leukemia and lymphoma. This research involved 428 cases of childhood leukemia and lymphoma (2016-2021), along with a control group of 428 children aged 1-15 in Tehran. We analyzed the risk of childhood cancer associated with land use by employing logistic regression adjusted for confounding factors such as parental smoking and family history. The odds ratio (OR) for children with leukemia and lymphoma residing within 100 m of the nearest highway was 1.87 (95% CI = 1.00-3.49) and 1.71 (95% CI = 1.00-2.93), respectively, in comparison to those living at a distance of 1000 m or more from a highway. The OR for leukemia with exposure to petrol stations within 100 m was 2.15 (95% CI = 1.00-4.63), and for lymphoma it was 1.09 (95% CI = 0.47-2.50). A significant association was observed near power lines (OR = 3.05; 95% CI = 0.97-9.55) within < 100 m for leukemia. However, no significant association was observed between power lines and the incidence of childhood lymphoma. There was no association between bus stations, major road class 2, and the incidence of childhood leukemia and lymphoma. In conclusion, our results suggest a possible association between the incidence of childhood leukemia and proximity to different urban land uses (i.e., highways and petrol stations). This study is the first step in understanding how urban land use affects childhood leukemia and lymphoma in Tehran. However, comprehensive studies considering individual-level data and specific pollutants are essential for a more nuanced understanding of these associations.
Assuntos
Leucemia , Linfoma , Humanos , Criança , Irã (Geográfico)/epidemiologia , Masculino , Leucemia/epidemiologia , Leucemia/etiologia , Feminino , Linfoma/epidemiologia , Linfoma/etiologia , Linfoma/induzido quimicamente , Pré-Escolar , Adolescente , Estudos de Casos e Controles , Lactente , Exposição Ambiental/efeitos adversos , Fatores de Risco , Razão de Chances , IncidênciaRESUMO
Introduction: Liver dysfunction is one of the most common disorders in patients with acute lymphoblastic leukemia (ALL). In recent studies, silymarin has been observed to have hepatic protective effects. Therefore, in this study, the effect of oral silymarin on the hepatic functions of patients with ALL was investigated. Methods: In the present double-blind clinical trial study, 121 patients with ALL over 5 years of age were divided into two groups after obtaining informed consent. The subjects were randomly divided into a silymarin-treatment group and a placebo group. In the silymarin-treatment group, patients received 70 mg oral capsules of silymarin twice daily or syrup of silymarin three times a day (each 5 ml of syrup contains 50 mg of silymarin). Patients were examined once a month for 9 months to receive capsules and measure the levels of alanine aminotransferase (ALT), aspartate transferase (AST), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), bilirubin, albumin, and cholesterol. Results: Comparison of changes before and after treatment in the two groups showed that receiving oral silymarin resulted in a slight significant decrease in the levels of ALT, AST, GGT, and bilirubin (p < 0.05), but had no effect on ALP, albumin, and cholesterol (p > 0.05). Discussion: The results of the present study showed that in pediatric patients with ALL, silymarin intake improves liver function. The very strong antioxidant effect of silymarin may explain its protective effect on the liver. Clinical Trial Registration: IRCT20150119020715N10.
RESUMO
PURPOSE: Fear of progression (FoP) is a substantial concern for family caregivers of cancer survivors and is related to a number of adverse outcomes, including increased mental distress and worse quality of life. Previous research has revealed that health anxiety (HA) contributes to fear of relapse, but cognitive factors underlying establishing and maintaining FoP in mothers of cancer patients have not been examined. In this study, we were looking to investigate this association. METHODS: We used the computerized interpretation bias (IB) assessment to investigate the biased interpretation of ambiguous bodily information and its association with FoP through HA among 69 mothers of cancer patients and 42 mothers of healthy kids. RESULTS: Mothers of cancer patients interpreted more negatively ambiguous bodily symptoms than mothers of healthy kids. Moreover, they had higher levels of HA and FoP and lower quality of life than the healthy group. Also, among mothers of cancer patients, the relationship between negative IB and FoP is mediated by their HA. CONCLUSIONS: The findings of this study imply that negative IB may contribute to increased HA, which in turn contributes to higher levels of FoP among the mothers of cancer patients, which may reduce the quality of life of their children. IMPLICATIONS FOR CANCER SURVIVORS: From these findings, we propose that changing HA through modification of IB might lower the FoP in mothers of kids with cancer and improve the mother and child's quality of life.
Assuntos
Sobreviventes de Câncer , Neoplasias , Feminino , Criança , Humanos , Mães/psicologia , Qualidade de Vida/psicologia , Inquéritos e Questionários , Ansiedade/etiologia , Ansiedade/psicologia , Neoplasias/psicologiaRESUMO
Background: Given the association of hypomagnesemia with cardiac arrhythmia, the aim of this study was to investigate the relationship between serum magnesium levels with age and T2* magnetic resonance imaging (MRI) findings of the heart and liver in patients with thalassemia major (TM). Materials and Methods: In a descriptive cross-sectional study, a total of 62 patients with ß-thalassemia major aged 11-48 years were selected at the Amir-Kabir Hospital, Arak, Iran. Serum magnesium, ferritin, and iron levels of patients were measured, and the rate of cardiac and hepatic hemosiderosis of patients was extracted according to the routine T2*MRI method. Results: The mean age of the patients at diagnosis was 32.6 years. The comparison of TM patients with and without hepatic/cardiac hemosiderosis demonstrated that mean levels of serum ferritin, serum iron, and age were significantly higher in TM patients with cardiac hemosiderosis than in hepatic/cardiac non-hemosiderosis (P < 0.05); however, there was no significant difference in mean levels of serum magnesium in TM patients with and without hepatic/cardiac hemosiderosis (P = 0.279). Interestingly, the correlation of age with serum magnesium levels in TM patients revealed a statistically significant and moderate inverse correlation (r = -0.56, P = 0.013). Conclusion: Hypomagnesemia may occur in a time-dependent manner. It is recommended that, in addition to cardiac and hepatic T2*MRI, serum magnesium levels be measured by using magnesium replacement if necessary.
RESUMO
ABSTRACT Introduction: This study was performed to evaluate the degree of 3-day chemotherapy-induced nausea and vomiting (CINV) in children with cancer who received highly emetogenic chemotherapy (HEC) to ascertain the efficacy of aprepitant single-dose on dayL 1 plus granisetron and dexamethasone (DEX). Methods: This clinical trial study was conducted on 120 patients in the age range of 5 to 18 years old who received chemotherapy. Patients were divided into two groups; Group A received aprepitant at 125 mg/kg on day 1 orally, followed by 80 mg/kg daily on days 2 and 3 and Group B received a single dose of aprepitant 125 mg/kg on day 1 orally and placebo on days 2 and 3. All groups received granisetron 3 mg/m2 on day 1 and DEX on days 1 to 3. The primary and secondary endpoints were to evaluate the proportion of patients with acute, delayed and overall CINV within each group. Results: There were no significant differences between the two groups for vomiting, nausea or the use of rescue therapy. The number of patients without vomiting on day 1 was similar in both groups (96.5% vs. 98.3%, respectively; p = 0.848). Conclusion: According to the results of this study, a single dose of aprepitant 125 mg/kg was as effective as administering three doses of aprepitant on 3 days. Therefore, the use of a single dose of aprepitant in combination with other standard treatment regimens to prevent CINV in children who received HEC was safe and efficacious and can be beneficial.
Assuntos
Humanos , Pré-Escolar , Criança , Adolescente , Vômito , Dexametasona , Granisetron , Aprepitanto , NáuseaRESUMO
Background: In order to improve the complete recovery of nausea and vomiting, we conducted a study with the aim of preventing acute and delayed nausea and vomiting in children undergoing moderate emetogenic chemotherapy. Materials and Methods: A clinical trial study was done on 130 children received chemotherapy. Patients received olanzapine and placebo. All groups received granisetron along with dexamethasone (DEX). The severity of chemotherapy-induced nausea and vomiting (CINV) induced by chemotherapy was compared in two groups. Results: The severity of nausea on the first, second, third, and fourth days was not significantly different (P > .05) in two groups. The number of patients without vomiting was significantly different during the first 24 hours after chemotherapy between patients in the two groups (82.3% vs 64.5%; P = .016). Conclusion: This study showed that olanzapine, which acts as an inhibitor of neurotransmitters, had a favorable efficacy in controlling acute and delayed CINV. More studies with large sample size are needed to compare the effect of olanzapine with other agents including aprepitant and palonosetron in the prevention of CINV.
RESUMO
Considering the importance of managing patients with ß-thalassemia and the importance of early detection of disease complications, we examined the rate of sensorimotor neuropathy in patients with ß-thalassemia and the risk factors related to it. This cross-sectional study included 44 blood transfusion-dependent ß-thalassemia patients aged 5 years and older. Nerve conduction studies (NCSs) were performed via standard procedures for both motor and sensory nerves. Neuropathy was observed in 14 patients (31.8%). NCS results for sensorimotor nerves in patients were within normal range. In motor NCS results, increased ulnar nerve amplitude was observed in patients with increasing age, and peroneal nerve delay in patients with an increase in serum ferritin level (p < 0.05). In sensory NCS results, delayed ulnar and sural nerves latencies were found in patients with an increase in serum ferritin level (p < 0.05). We provide data that sensorimotor neuropathy exists in thalassemia patients. It seems that with the increase of serum ferritin level and the age of patients, neuropathy becomes more obvious, while other factors such as gender, body mass index, and the number of transfusions may not be associated with neuropathy.
Assuntos
Doenças do Sistema Nervoso Periférico , Polineuropatias , Talassemia beta , Humanos , Talassemia beta/complicações , Talassemia beta/terapia , Irã (Geográfico)/epidemiologia , Estudos Transversais , Polineuropatias/diagnóstico , Polineuropatias/epidemiologia , Polineuropatias/etiologia , Transfusão de Sangue , FerritinasRESUMO
Background: Magnesium oxide may be effective in renal insufficiency prevention after carboplatin therapy. We have evaluated magnesium oxide impression on the serum creatinine (Cr) and blood urea nitrogen (BUN) levels plus glomerular filtration rate (GFR) in cancerous children. Materials and Methods: A group of children with different cancers (n = 18) was treated with 250 mg/day magnesium oxide supplementation (MOS) and compared with a matched placebo-treated group (n = 18). After 2 weeks, carboplatin chemotherapy started. We compared serum Cr, BUN, and GFR values before and 3 and 7 days post intervention. Results: Serum Cr and BUN were increased significantly 3 and 7 days after intervention in both the groups. Serum Cr and BUN were not statistically different between the MOS and placebo groups before the intervention and 3 or 7 days after carboplatin administration (P > 0.05). Three days after the intervention, the GFR reduced from 101.38 ± 14.67 to 90.11 ± 10.52 mL/min/1.73 m2 in the MOS group. Furthermore, in the placebo group, 3 days after the intervention, the GFR was reduced from 97.5 ± 9.71 to 92.33 ± 10.61 mL/min/1.73 m2. Further, in the MOS group, after 7 days of the intervention, the GFR was reduced to 84.11 ± 12.47 mL/min/1.73 m2. In the placebo group, after 7 days of the intervention, the GFR was diminished to 85.38 ± 10.66 mL/min/1.73 m2 (P = 0.371). Conclusion: The current study suggests that magnesium supplementation does not prevent carboplatin-induced nephrotoxicity in children with malignancies. Anyway, we propose magnesium oxide supplementation for this group of pediatrics because magnesium is an essential element for cell and tissue growth, maintenance, and metabolism.
RESUMO
BACKGROUND: Synbiotics are supplements containing probiotics and prebiotics and potentially have a stronger effect in modulating the gut microbiota than probiotics or prebiotics alone. The aim of this study was to determine the effects of LactoCare synbiotic administration on chemotherapy-induced diarrhea (CID), nausea, vomiting, and constipation in children with acute lymphoblastic leukemia (ALL) who receiving maintenance chemotherapy. METHODS: This double-blind clinical trial was performed on 113 children with ALL. The patients were randomly assigned into two groups to receive either 5 × 109 CFU LactoCare synbiotic administration or placebo (58 patients in the LactoCare-treatment group and 55 patients in the placebo group), twice a day for 7 days. The number of times CID, vomiting, nausea, and constipation were recorded in the first week after the beginning of receiving LactoCare and the placebo. RESULTS: In the LactoCare-treatment group, CID was present in 3.7% and 1.8% of patients on the first and second days, respectively, and no CID was observed on the third to seventh days (p < .05). While in the placebo group, the rate of patients with CID on the second, third, and fourth days was 11.5%, 13.5%, and 11.5%, respectively, and less than 10% on the first, fifth, sixth, and seventh days. It was observed that the rate of constipation in the LactoCare-treatment group was significantly lower than in the placebo group. The difference between the groups was about 14% on the third day, which increased to about 20% on the sixth day (p < .05). CONCLUSION: The use of synbiotic supplements in this study reduced CID in patients. This study supports the concept that the use of synbiotic supplements will be an easy and effective way to reduce CID in ALL patients.
Assuntos
Antineoplásicos , Leucemia-Linfoma Linfoblástico de Células Precursoras , Simbióticos , Humanos , Criança , Vômito/induzido quimicamente , Constipação Intestinal , Náusea/induzido quimicamente , Diarreia , Antineoplásicos/efeitos adversos , Método Duplo-CegoRESUMO
INTRODUCTION: This study was performed to evaluate the degree of 3-day chemotherapy-induced nausea and vomiting (CINV) in children with cancer who received highly emetogenic chemotherapy (HEC) to ascertain the efficacy of aprepitant single-dose on dayL 1 plus granisetron and dexamethasone (DEX). METHODS: This clinical trial study was conducted on 120 patients in the age range of 5 to 18 years old who received chemotherapy. Patients were divided into two groups; Group A received aprepitant at 125 mg/kg on day 1 orally, followed by 80 mg/kg daily on days 2 and 3 and Group B received a single dose of aprepitant 125 mg/kg on day 1 orally and placebo on days 2 and 3. All groups received granisetron 3 mg/m2 on day 1 and DEX on days 1 to 3. The primary and secondary endpoints were to evaluate the proportion of patients with acute, delayed and overall CINV within each group. RESULTS: There were no significant differences between the two groups for vomiting, nausea or the use of rescue therapy. The number of patients without vomiting on day 1 was similar in both groups (96.5% vs. 98.3%, respectively; p = 0.848). CONCLUSION: According to the results of this study, a single dose of aprepitant 125 mg/kg was as effective as administering three doses of aprepitant on 3 days. Therefore, the use of a single dose of aprepitant in combination with other standard treatment regimens to prevent CINV in children who received HEC was safe and efficacious and can be beneficial.
RESUMO
Background: Curcumin present in turmeric has been considered due to its cancer-preventive features, antioxidant and anti-inflammatory properties. This double-blind, randomized, controlled clinical trial with a reasonable sample size and longer intervention period was conducted to investigate how oral curcumin affected cardiac and hepatic T2*MRI and liver enzymes in patients with ß-thalassemia major. Method: This clinical trial study was conducted on 171 patients over 5 years old. The subjects were randomly divided into a curcumin-treatment group and a placebo group to receive either curcumin capsules twice daily or placebo for 6 months. Patients were examined once a month for 6 months to receive capsules and measure the levels of alanine aminotransferase (ALT), aspartate transferase (AST), alkaline phosphatase (ALP), direct and total bilirubin, ferritin and cardiac and hepatic T2*MRI. Result: There was a significant decrease in levels of AST, ALT, ALP, and bilirubin (direct and total) in the curcumin group compared with the placebo group by the end of the study (p < 0.05). The levels of serum ferritin remained unchanged in both groups at the end of the follow-up period (p > 0.05). No significant differences were observed between the curcumin and placebo groups at baseline values or at the end of the study of cardiac and hepatic T2*MRI and serum magnesium. Conclusion: Administration of curcumin has some beneficial effects on liver function by reducing liver enzymes in patients with beta-thalassemia major.
RESUMO
Background: Renal insufficiency is one of the inevitable complications in patients with Wilms tumor (WT). The purpose of this study was to assess the renal function in children with WT at baseline and every 3 months to 2 years. Materials and Methods: In a descriptive-analytical study from 2018 to 2020, 48 children with WT were included in the study. Urine creatinine (UCr), serum calcium (SCr), blood pressure (BP), estimated glomerular filtration rate (eGFR), and urinary protein (UPro) were evaluated at baseline and every 3 months during the study. Spot UCa/UCr and spot UPro/UCr ratio were calculated. Kidney ultrasonography was used in all patients. Independent Sample t-test and Chi-square tests were utilized to compare age and sex, respectively. Results: The mean age of patients at follow-up was 7.3 years. There was no significant difference in mean UCr, SCr, eGFR, 24-h UPro, UCa/UCr ratio, and spot UPro/UCr ratio at baseline and end of study (P baseline> 0.05, P end of study> 0.05). Analysis of kidney size showed a statistical association with tumor stage (P < 0.05). Comparison of the kidney size in patients showed that there is a statistically significant difference (P < 0.0001) at baseline and end of the study. Conclusion: This study showed that as WT progressed, the size of the kidneys increases without any renal insufficiency. Therefore, it seems that urinalysis of patients with WT along with sonography is necessary to determine renal insufficiency and the use of ultrasound alone to determine kidney insufficiency is not recommended.
RESUMO
Background: Iron overload and cardiac dysfunctions are common complications in patients with thalassemia major (TM). Different imaging methods can be used to detect ventricular dysfunction in these patients. In this study, we aim to understand the value of tissue Doppler imaging (TDI) in the detection of myocardial dysfunction in patients with TM who have been diagnosed with iron overload using cardiovascular magnetic resonance CMRT2*. Methods: In this cross-sectional study, fifty patients with TM diagnosed with iron overload who had no clinical signs and symptoms of cardiac dysfunction were chosen as a case group. The control group included fifty sex- and age-matched healthy participants without a history of cardiac and hematological diseases. TDI, pulsed wave Doppler (PWD), and standard echocardiography were performed to study the left ventricular function, and cardiac iron overload assessed by CMRT2*. Then, the patients with TM were divided into two subgroups and compared with each other. Group 1a includes individuals with T2* value <20 ms and group 1b T2* value >20 ms. Results: There was no significant difference between the standard echocardiography results and PWD parameters of the case and control groups; however, CMRT2* findings and TDI parameters were different between the case and control groups. CMRT2* findings also were not correlated with PWD parameters. In group 1a, CMRT2* findings were negatively correlated with age, E', A', early deceleration time, and isovolumetric relaxation time and positively correlated with E/E' ratio. Finally, PWD and TDI parameters were significantly different between the two subgroups. Conclusion: TDI can detect ventricular systolic and diastolic dysfunctions in earlier stages among patients with iron overload. It seems that TDI could detect abnormalities more accurately, and it is better to consider subclinical cardiac dysfunction in patients with even CMRT2* value of more than 20 ms and reevaluate them in future.
RESUMO
BACKGROUND: Iron deficiency anemia (IDA) is one of the most common anemias, especially in children 4-23 months. Therefore, prophylaxis is necessary to improve iron status as well as reduce IDA in Toddlers. The aim of this study was to compare the efficacy of daily supplementation with ferrous gluconate (FG) and ferrous sulfate (FS) on iron status in toddlers. MATERIALS AND METHODS: A total of 120 healthy toddlers were divided randomly into 2 groups at the Amir-Kabir Hospital, Arak, Iran and received FS and FG from March 2020 to December 2020. Iron status was evaluated at baseline and after 6 months of supplementation. The statistical significance of the differences in iron status between FS and FG groups was calculated using Student's t-test and the Pearson' s Chi-square test for qualitative variables. SPSS software (version 16, Chicago, IL, USA) was used for statistical analysis. RESULTS: Comparison of iron status of FS and FG groups toddlers at baseline and after 6 months of supplementation showed that there was a significant difference in hemoglobin (Hb) (10.46 vs. 12.45, P = 0.001) and ferritin level (28.08 vs. 59.63, P = 0.001). CONCLUSIONS: Although prophylaxis with FG led to a higher Hb and ferritin levels, our study recommended that both FG and FS supplements were effective for prophylactic use in the prevention of IDA. However, FG was more effective than FS because FG group that received FG supplementation indicated a higher Hb and ferritin levels in comparison to the FS group that received FS supplementation.
RESUMO
BACKGROUND: Pulmonary hypertension is one of the most common cardiac complications among patients with ß-thalassemia major (ß-TM). The aim of this study is to investigate the correlation of pulmonary artery pressure (PAP), serum ferritin level, and cardiac T2* MRI in patients with ß-TM. METHOD: This cross-sectional study was performed on 50 patients older than 7 years old. Echocardiography, electrocardiography, and cardiac T2* MRI were performed on all patients and their serum ferritin levels were measured. Based on the echocardiographic the patients were divided into two groups of PAP>30 and <30 mmHg. RESULTS: 40% had PAP higher than 30 mmHg, 32% had abnormal T2* MRI, and 36% had serum ferritin levels higher than 1500ng/dl. there were significant negative correlations between CMRI *T2 and PAP (-0.36) and the P-pulmonary (-0.29). the serum ferritin level was positively correlated with PAP (0.44) and the P pulmonary in ECG (0.30). in the patients with PAP>30, the means of age, serum ferritin level, and P-pulmonary were significantly higher than patients with PAP<30 (P=0.001). In the patients with PAP>30mmHg, CMRI *T2 had higher accuracy (80%), sensitivity (65%), and specificity (90%) compared to the measurement of serum ferritin levels. CONCLUSION: The PAP was positively correlated with serum ferritin levels and negatively with T2MRI. The accuracy of 80% for T2MRI as a method to diagnose or even anticipate PH among patients with TM suggested that this method is a useful and accurate method of PH diagnosis and can be used as an alternative among clinicians.
RESUMO
INTRODUCTION: Chronic immune thrombocytopenia (ITP) of childhood is still a problem. For treating ITP, several immunosuppressive medications can be considered with various response rates. Our goal was to compare effects of sirolimus and cyclosporine on children with chronic ITP. METHODS: This randomized and blinded trial was carried out on 67 children over 5 years old with chronic ITP. Patients were assigned 1:1 to cyclosporine and sirolimus for 6 months. Platelet count was assessed and compared between 2 study groups at different intervals. The clinical trial registry number was IRCT20180501039499N1. RESULTS: Sixty-one children completed the 6-month treatment. Mean age was 9.3 years with an excess of females. Compared to baseline values, both drugs caused a significant increase in number of platelets over the course of treatment; sirolimus group: 15,800/mcL vs 96,566/mcL, (P < 0.001), cyclosporine group: 14,400/mcL vs 111,266/mcL, P < 0.001,). In addition, differences of platelet number were statistically significant at some treatment intervals (3rd and 6th month, P < 0.05). A quicker response was observed in children receiving cyclosporine. Both drugs had similar rate of response which occurred in 50% of included patients. Finally, sirolimus had a better safety profile. CONCLUSIONS: Our study showed that cyclosporine and sirolimus had an equal rate of response in treating chronic ITP of children. At the same time, the two medications showed significant differences in their side effects.
Assuntos
Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Sirolimo/uso terapêutico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Método Simples-CegoRESUMO
INTRODUCTION: Thalassemia is a hypochromic microcytic anemia, which is characterized by congenital disorders. In thalassemia patients, bone diseases are one of the causes of mortality. Our goal was to investigate the association between vitamin D deficiency and increased iron uptake by cardiac myocytes and hepatocytes. MATERIALS AND METHODS: Forty patients with thalassemia major were studied in Amir Kabir Hospital, Arak, Iran. The information obtained through clinical examination. Serum ferritin level was determined by ELISA and T2*MRI performed for measuring iron content in the heart and the liver. RESULTS: The average age of the patients was 23.8 ± 10.7 years. The mean T2*MRI values were 23.7 ± 7. The vitamin D3 level in 33 patients (82.5% cases) was less than 20 ng/dl, 2 patients (5%) in the range of 20-30 ng/dl, and the others had above 30 ng/dl. Correlation between vitamin D and age was 0.611. Correlation coefficient between heart and liver T2*MRI with ferritin level in patients was 0.437 and 0.335, respectively. CONCLUSION: Due to significant associations, the periodic measurement of vitamin D, as well as PTH, is recommended for patients with thalassemia major.
RESUMO
BACKGROUND: T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive and malignant neoplasm that arises from the hematopoietic T-cell precursors. Inactivation of FBXW7 gene is frequently observed in T-cell acute lymphoblastic leukemia, suggesting a significant tumor-suppressive role for FBXW7 in the pathobiology of this leukemia. Considering the role of microRNAs in cell proliferation and regulation of apoptosis, the aim of this study was to identify novel oncogenic microRNAs that suppress FBXW7 in patients with T-ALL. PATIENTS AND METHODS: The expression levels of two bioinformatically predicted microRNAs - miR-32 and miR-107 were compared in patients with T-ALL and a control group. A total of 80 plasma samples were subjected to RNA extraction, and the microRNA expression profiles were assessed by the RT-qPCR. The expression level of miR-103 was used as the endogenous reference for normalization of quantitative data. RESULTS: The plasma levels of miR-32 and miR-107 in patients with T-ALL were significantly higher (5.65, P < 0.001) and lower (0.432, P = 0.002), respectively. On the other hand, the expression levels of FBXW7 gene were significantly downregulated by -76.9 fold in T-ALL patients (P < 0.001). The results of the ROC curve analysis indicated that overexpression of miR-32 might be used to distinguish T-ALL patients with reasonable sensitivity and specificity. CONCLUSION: miR-32 is considered as a novel oncomir that targets FBXW7 and might have a role in the etiology or progression of T-ALL. Furthermore, miR-32 can potentially serve as a non-invasive biomarker for detection of T-ALL.
RESUMO
AIM: Iron chelators are extensively used to reduce iron overload. Our purpose was to compare effects of deferasirox versus deferasirox and deferoxamine in patients with thalassemia major. METHODS: This randomized and double blind trial was performed on 62 patients. Patients were assigned 1:1 to oral 30 mg/kg deferasirox daily or oral 30 mg/kg deferasirox daily plus SC 50 mg/kg deferoxamine for 5 days a week. Treatment continued for 12 months in both groups. RESULTS: Fifty-five patients completed the 1 year of treatment. Mean age was 24.5 years with an excess of females. Combined therapy caused a significant increase in myocardial T2* from 23.1 ± 7.5 ms at baseline to 27.1 ± 7.0 ms at 12 months (P < 0.05). This difference was statistically significant between 2 groups at 12 months (P = 0.01). Combined therapy and monotherapy had no significant effect on liver T2*. At 12 months, serum ferritin levels were reduced in two groups; however, the difference was significant (737 ± 459 µg/ml vs 1085 ± 919 µg/ml, P < 0.01). CONCLUSION: Our study indicates that combined treatment with deferasirox and deferoxmaine is more effective than deferasirox for reduction of iron over load in patients with thalassemia major.