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1.
Cancer Genet ; 282-283: 27-34, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38183785

RESUMEN

The current genomic abnormalities provide prognostic value in pediatric Acute Lymphoblastic Leukemia (ALL). Furthermore, Copy Number Alteration (CNA) has recently been used to improve the genetic risk stratification of patients. This study aimed to evaluate CNA profiles in BCR-ABL1-negative pediatric B-ALL patients and correlate the data with Minimal Residual Disease (MRD) results after induction therapy. We examined 82 bone marrow samples from pediatric BCR-ABL1-negative B-ALL using the MLPA method for the most common CNAs, including IKZF1, CDKN2A/B, PAX5, RB1, BTG1, ETV6, EBF1, JAK2, and PAR1 region. Subsequently, patients were followed-up by multiparameter Flow Cytometry for MRD (MFC-MRD) assessment on days 15 and 33 after induction. Data showed that 58.5 % of patients carried at least one gene deletion, whereas 41.7 % of them carried more than one gene deletion simultaneously. The most frequent gene deletions were CDKN2A/B, ETV6, and IKZF1 (30.5 %, 14.6 %, and 14.6 %, respectively), while the PAR1 region showed predominantly duplication (30.5 %). CDKN2A/B and IKZF1 were related to positive MRD results on day 15 (p = 0.003 and p = 0.007, respectively). The simultaneous presence of more than one deletion was significantly associated with high induction failure (p = 0.001). Also, according to the CNA profile criteria, the CNA with poor risk (CNA-PR) profile was statistically associated with older age and positive MRD results on day 15 (p = 0.014 and p = 0.013, respectively). According to our results, the combined use of CNAs with MRD results on day 15 can predict induction failure and be helpful in ameliorating B-ALL risk stratification and treatment approaches.


Asunto(s)
Leucemia-Linfoma Linfoblástico de Células Precursoras B , Leucemia-Linfoma Linfoblástico de Células Precursoras , Niño , Humanos , Variaciones en el Número de Copia de ADN/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras B/genética , Neoplasia Residual/genética , Receptor PAR-1/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Pronóstico , Factores de Transcripción/genética
2.
Pediatr Hematol Oncol ; 41(4): 251-259, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38088332

RESUMEN

Patients with beta-thalassemia major require lifelong and frequent red blood cell transfusions for survival, impacting their quality of life and life expectancy. This treatment approach poses risks of organ damage, iron overload, and increased transfusion-transmitted diseases. N-acetylcysteine (NAC) has been studied for its potential antioxidant effects on hemoglobin stability, aiming to reduce the burden of red blood cell transfusions. To explore this possibility further, we conducted a quasi-experimental study involving 35 individuals with thalassemia major over six months All subjects were already receiving iron chelators and blood transfusions. They were given a daily oral dose of 10 mg/kg NAC for three months. After three months of treatment with NAC, the serum levels of ferritin and liver enzymes (SGOT and SGPT) did not show significant changes (p = 0.35, p = 0.352, and p = 0.686, respectively). However, the red blood cell transfusion burden was significantly reduced in all patients after NAC therapy (p = 0.029), with no corresponding decrease in serum hemoglobin levels (p = 0.931), indicating maintained hemoglobin concentration despite reduced transfusion volume. The study indicates that NAC can effectively decrease the burden of red blood cell transfusions without significant toxicity in these patients. This finding suggests the potential for NAC as a cost-effective and manageable treatment option for these patients. A larger clinical trial with more robust statistical methods could further confirm these results and pave the way for using NAC as a valuable therapeutic agent for managing beta-thalassemia major patients.


Asunto(s)
Sobrecarga de Hierro , Talasemia beta , Humanos , Transfusión de Eritrocitos , Acetilcisteína/uso terapéutico , Calidad de Vida , Sobrecarga de Hierro/tratamiento farmacológico , Sobrecarga de Hierro/etiología , Hemoglobinas/análisis
3.
Radiol Case Rep ; 17(10): 4053, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36068872

RESUMEN

[This corrects the article DOI: 10.1016/j.radcr.2020.10.055.].

4.
Radiol Case Rep ; 17(9): 3449, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35909922

RESUMEN

[This corrects the article DOI: 10.1016/j.radcr.2020.10.055.].

5.
Cancer Inform ; 21: 11769351221084812, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35342287

RESUMEN

Objective: In this survey, a protocol-based Chemotherapy Prescription Decision Support System (CPDSS) was designed and evaluated to reduce medication errors in the chemotherapy process of children with ALL. Methods: The CPDSS algorithm was extracted by the software development team based on the protocol used by doctors to treat children with ALL. The ASP.Net MVC and SQL Server 2016 programming languages were used to develop the system. A 3-step evaluation (technical, retrospective, and user satisfaction) was performed on CPDSS designed at 2 children's hospitals in Tehran. The data were analyzed using descriptive statistics. At the technical evaluation step, users provided recommendations included in the system. Results: In the retrospective CPDSS evaluation step, 1281 prescribed doses of the drugs related to 30 patients were entered into the system. CPDSS detected 735 cases of protocol deviations and 57 (95%, CI = 1.25-2.55) errors in prescribed chemotherapy for children with ALL. In the user satisfaction evaluation, the users approved two dimensions of the user interface and functionality of the system. Conclusions: With the provision of alerts, the CPDSS can help increase compliance with chemotherapy protocols and decrease the chemotherapy prescribing errors that can improve patient safety.

6.
J Med Life ; 14(3): 298-309, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34377194

RESUMEN

Accessing the veins for blood delivery, sampling or nutrition is a critical factor in the process of care and management of pediatric patients. In this regard, the peripherally inserted central catheter (PICC) is one of the main alternatives which could be applied effectively as traditional central venous devices in neonates and adults. Due to their essential role in providing safe central venous entry, PICCs could be applied extensively in patients who are critically ill. The main aims of the present study are to review approximately all relevant publications concerning PICC procedures, any possible complications, and the most appropriate decision for preventing these complications due to their high mortality rate. We carried out a comprehensive search on PubMed, HubMed, EMBASE, MEDLINE, Science Direct, Scopus, MEDLINE, and EMBASE databases for identifying the most relevant publications related to potential complications following the application and insertion of PICCs in hospitalized children and infants. Through appropriate care of catheters, the rate of possible infectious, mechanical and thrombotic complications would decrease considerably compared to those patients who received traditional central venous catheters. However, the process of vascular access in neonatal and children is very challenging. Any delay or denying treatment due to the lack of vascular access is intolerable. In this regard, anesthesiologists must achieve extra knowledge of various vascular devices.


Asunto(s)
Cateterismo Venoso Central , Cateterismo Periférico , Catéteres Venosos Centrales , Adulto , Cateterismo Venoso Central/efectos adversos , Cateterismo Periférico/efectos adversos , Catéteres Venosos Centrales/efectos adversos , Niño , Enfermedad Crítica , Humanos , Lactante , Recién Nacido , Estudios Retrospectivos
7.
Transfus Apher Sci ; 60(6): 103212, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34257016

RESUMEN

BACKGROUND: Prophylaxis the current standard care for patients with severe hemophilia should be planned to optimize the replacement therapy and minimize bleeding. We report our single-center experience of tailored prophylaxis in children affected by hemophilia A (HA) and hemophilia B (HB). METHODS: This study was conducted on 55 patients, under 15 years, with HA (PWHA, n: 46) and HB (PWHB, n: 9) between 2015 and 2019. According to the phenotype, three prophylaxis regimens: 25-50 unit/kg once, twice, or three-times a week for PWHA, and two: 30-50 unit/kg once or twice a week for PWHB were administered. Following the occurrence of > 3 joint bleeding, or > 4 soft tissue bleeding, or one spontaneous major bleeding in the last 3 months, the prophylaxis regimen is changed. Annualized bleeding rate (ABR), annualized joint bleeding rate (AJBR), target joints development, inhibitor development, and hemophilia joint health score (HJHS) also were assessed. RESULTS: A mean ± SD of 2520 ± 1045 IU/kg/yr coagulation factor (F) VIII was used to reduce ABR and AJBR from 1.02 ± 1.11 and 0.8 ± 1.3 (in the first year of the study) to 0.27 ± 0.44 (P < 0.001) and 0.19 ± 0.38 (P = 0.004) (at the end of the study) in PWHA, respectively. Furthermore, in PWHB, in the first year of the study, with using 2168 ± 1216 IU/kg coagulation FIX, ABR and AJBR were 0.19 ± 0.39 and 0.06 ± 0.1. At the end of the study, ABR and AJBR were 0.02 ± 0.05 (p = 0.156) and 0.01 ± 0.03 (p = 0.361), respectively. During the study period, the mean number of the target joints and mean HJHS were 0.25 ± 0.57 and 7.6 ± 2.1 for PWHA and 0 and 6.3 ± 1.8 for PWHB, respectively. Finally, 5 PWHA (11 %) did not need dose-escalation in their prophylaxis regimen, whereas 31 (67 %) and 10 (21 %) PWHA needed two and three infusions a week, respectively. In PWHB, 7 (78 %) and 2 (22 %) were adjusted to receive a once and twice weekly regimen, respectively. CONCLUSION: Our results suggest that tailored prophylaxis is an effective strategy to reduce the rate of bleeding and optimize the replacement therapy in children with hemophilia.


Asunto(s)
Hemofilia A/tratamiento farmacológico , Niño , Preescolar , Femenino , Humanos , Irán , Masculino , Estudios Prospectivos , Factores de Tiempo
8.
Clin Appl Thromb Hemost ; 27: 1076029621996813, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33764796

RESUMEN

Congenital combined bleeding disorders (CBDs) are extremely rare disorders which mainly occur in regions with a high rate of consanguineous marriage. These disorders can present with a variety of symptoms ranging from mucocutaneous bleeding to life-threatening episodes. This study aims to evaluate the prevalence and clinical course of Iranian patients with congenital CBDs. This study is conducted on 450 patients with CBDs who were referred to the Iranian Comprehensive Hemophilia Care Center (ICHCC) between 2010 and 2020. All these patients were diagnosed through evaluation of past medical history and coagulation laboratory investigation. Out of 450 patients, 33 were entered in this study. Having excluded cases with factor (F) V and FVIII deficiency, as well as those with hereditary combined Vitamin K dependent clotting factor deficiency (VKCFD), We found the most common CBDs to be FV-FVII deficiency (n: 6, 18.1%), together with FVII and FX deficiency (n: 6, 18.1%). The most common reason for referral of these patients to ICHCC was postoperative bleeding (14.3%). The mean of The International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) and condensed MCMDM-1VWD bleeding assessment tool were 9.6 ± 4.79 and 9.1 ± 4.87, respectively (P < 0.005). In 10 females of reproductive age, the mean of Pictorial Bleeding Assessment Chart (PBAC) score was 649.3 ± 554. Among all patients, 23 (69.7%) received on-demand replacement therapy, whereas 5 patients (15.1%) received prophylaxis. In Iran, the coinheritance of bleeding disorders is surprisingly higher than expected. Moreover, patients with congenital CBDs may experience serious bleeding manifestations.


Asunto(s)
Hemorragia/congénito , Adolescente , Adulto , Femenino , Humanos , Irán , Masculino , Adulto Joven
9.
Asia Pac J Oncol Nurs ; 8(2): 218-223, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33688572

RESUMEN

OBJECTIVE: Hematopoietic stem cell transplantation (HSCT) is known as one of the most advanced and modern treatments in the world for various diseases which do not respond well to other therapies. Evaluating outcomes of these patients, especially in newly developed centers, can crucially help in developing and improving the quality of these centers. METHODS: In a retrospective analytical cohort study, we statistically analyzed all patients treated with HSCT in the Bone Marrow Transplant Unit of the Ali-Asghar Pediatric Hospital affiliated to Iran University of Medical Sciences. The demographic information as well as all information concerning each patient's transplant process was extracted and statistically analyzed using SPSS Version 23. RESULTS: The mean neutrophilic and platelet engraftment days were, respectively, 16 (range = 12-21) and 22 (range = 15-34) days after HSCT, while the neutrophilic engraftment occurred significantly earlier in allogeneic transplants compared to the autologous ones (P = 0.020). The total event-free survival (EFS) rate of the patients based on the median follow-up of 12 months was 11.50% ± 53.60%. Based on the total follow-up period, the estimated total EFS rate of the patients was calculated as 35.20% ± 13.50%. The estimated EFS rate was found to be better in patients who had undergone allogeneic transplantation than those who received an autologous transplant (P = 0.780). CONCLUSIONS: The HSCT results at our center are comparable to those at other centers in Iran. We argue that the facility can provide adequate therapy to patients requiring HSCT, on the proviso that some organizational limitations are addressed.

10.
Blood Coagul Fibrinolysis ; 32(3): 200-203, 2021 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-33560003

RESUMEN

Coronavirus disease 2019 (COVID-19) is a new medical challenge for all individuals, especially for those with underlying disorders, such as congenital bleeding disorders (CBDs). Therefore, the pandemic might significantly change the behaviour of patients with CBDs and results in some challenges. In the present study, we assessed the main challenges of COVID-19 infection to patients with CBDs. Data were collected from medical files and interviews of patients with CBDs who had COVID-19 infection. Follow-ups were performed on patients who had active severe acute respiratory syndrome coronavirus 2 infection between April and October 2020. All patients were interviewed by an expert in order to collect the pertinent data. Some questions were about patients' preventive behaviors and feelings prior to infection, and some were about the consequences of infection on patients' replacement therapy and bleeding management. Among 25 patients, infection and death of loved ones (n: 7, 28%), and their own (n: 5, 20%) or family members' (n: 1, 4%) infection, and the resulting economic burden (n: 2, 8%) were main concerns. Six patients experienced depression during the pandemic. The pandemic caused all severely affected patients but one (n: 11, 92%) to abandon replacement therapy. However, two received on-demand therapy after exacerbation of their bleeding. Only one (25%) of four patients on prophylaxis received in-home therapy, whereas the others (75%) abandoned prophylaxis. It seems that COVID-19 infection has great consequences on the lives of patients with CBDs, causing some to take dangerous actions, such as abandonment of their treatment. Healthcare systems, and healthcare providers, should have an appropriate strategy for management of patients with CBDs that prevents infection and provides timely replacement therapy.


Asunto(s)
Trastornos de la Coagulación Sanguínea Heredados/complicaciones , COVID-19/complicaciones , Adolescente , Adulto , Trastornos de la Coagulación Sanguínea Heredados/terapia , COVID-19/sangre , COVID-19/fisiopatología , COVID-19/psicología , Bases de Datos Factuales , Depresión/complicaciones , Femenino , Estudios de Seguimiento , Hemorragia/prevención & control , Humanos , Masculino , Persona de Mediana Edad , Encuestas y Cuestionarios
11.
Radiol Case Rep ; 16(1): 166-170, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33250947

RESUMEN

Botryoid Wilms tumors are intrapelvic polypoid masses that are uncommon feature of Wilms tumor. The authors reported a rare case of bilateral botryoid Wilms tumor in a 19-month-old boy who admitted to hospital with acute renal failure secondary to hydronephrosis caused by bilateral botryoid Wilms tumor with ureteral extension. The patient was successfully treated with chemotherapy and bilateral nephron sparing surgery in 2 separate operations. During 15 months follow-up, no evidence of recurrence or metastasis was found.

12.
J Med Life ; 13(2): 151-155, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32742506

RESUMEN

Thalassemia represents a heterogeneous group of inherited diseases characterized by the lack or reduced production of hemoglobin ß-chains. Many patients with thalassemia require splenectomy. What should be considered in the evaluation and management of candidates for splenectomy is to cover vaccination against infections such as pneumococci and the implementation of antibiotic prophylaxis. This study aimed to investigate the effect of the antibiotic type on the outcome of acute post-splenectomy infection in patients with thalassemia. This investigation is a retrospective cohort study. One hundred fifty medical records of hemoglobinopathy patients who underwent splenectomy were collected from the Ali-Asghar Hospital, Tehran, Iran. SPSS v. 20 and SAS v. 1.9 were used to analyze the data. A total of 150 patients that were vaccinated against post-splenectomy infections and were under antibiotic prophylaxis underwent splenectomy. The most commonly prescribed drugs were ceftriaxone or cefotaxime (132 cases, 88%), followed by ceftriaxone plus clindamycin (5.3%), ceftriaxone plus amikacin (3.3%), clindamycin (1.3%), vancomycin plus amikacin (0.7%), and others (1.3%). In terms of treatment outcomes, 143 cases (95.3%) were treated with the same antibiotics, and 4 (2.7%) experienced a changed antibiotic regimen with vancomycin. The results show that perceptions of treatment for fever in splenectomized children need to be changed, and most of them do not require hospitalization and initiation of broad-spectrum antibiotics such as vancomycin for initially refractory cases, and can only be treated with daily intravenous ceftriaxone.


Asunto(s)
Antibacterianos/uso terapéutico , Fiebre/tratamiento farmacológico , Esplenectomía , Adolescente , Adulto , Amicacina/administración & dosificación , Amicacina/uso terapéutico , Ceftriaxona/administración & dosificación , Ceftriaxona/uso terapéutico , Niño , Preescolar , Femenino , Humanos , Irán , Masculino , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
14.
Sci Rep ; 10(1): 11717, 2020 07 16.
Artículo en Inglés | MEDLINE | ID: mdl-32678199

RESUMEN

Atopic dermatitis (AD) is a chronic disease affecting 10-30% of children and 2-10% of adults worldwide. It is manifested by the pruritus eczema lesions on the skin. Immune thrombocytopenic purpura (ITP) is the most common cause of acute onset of thrombocytopenia in childhood. The aim of this study was association of primary immune thrombocytopenic purpura and atopia among children. This case control study was performed on patients with acute and chronic ITP who were confirmed by a hematologist. The control group was also selected from the siblings of the patients who were healthy and almost matched by age and sex with the patient group. Data were entered into a questionnaire under the SPSS-20 program, and demographic data were analyzed descriptively. In the present study, 120 patients were enrolled, 60 of whom were in the patient group and 60 in the control group. Mean age was 95 and 98 months for patients and control. This study showed a significant association of ITP with allergic rhinitis (P = 0.02), atopic dermatitis (P = 0.004), itching (P = 0.042), and dry skin (P = 0.015). However, no significant relationship was found between ITP and asthma (P-value = 0.18). This study does not reveal the causality between atopy and ITP but clearly shows the association between atopy and ITP disease, so the prevalence of atopy in ITP patients is higher than the normal population. According to the results of this study, it is necessary to investigate the cause of atopy and ITP and to find other immunological and possibly genetic commonalities.


Asunto(s)
Asma/epidemiología , Dermatitis Atópica/epidemiología , Prurito/epidemiología , Púrpura Trombocitopénica Idiopática/epidemiología , Rinitis Alérgica/epidemiología , Enfermedad Aguda/epidemiología , Estudios de Casos y Controles , Niño , Enfermedad Crónica/epidemiología , Comorbilidad , Femenino , Humanos , Incidencia , Masculino , Prevalencia , Púrpura Trombocitopénica Idiopática/complicaciones , Encuestas y Cuestionarios , Trombocitopenia/etiología
15.
Leuk Res Rep ; 14: 100209, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32566479

RESUMEN

The most common childhood malignancy is acute leukemia. Approximately 15- 20% of it, is Acute myeloid leukemia (AML). The general symptoms of this malignancy include fatigue, weakness, fever, paleness and bleeding disorders. There are two methods of classifying for AML: The French-American-British (FAB) and the World Health Organization (WHO) classification.The M4 eos subtype, also called myelomonocytic leukemia, is one subtype of AML with eosinophilia. The most common cytogenetic variations in this leukemia include inv (16) (p13q22) or the variant t (16; 16) (p13; q22). In this report, we present the first AML-M4 eos case with a new translocation that has not yet been reported.

16.
J Family Med Prim Care ; 9(2): 590-596, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32318387

RESUMEN

INTRODUCTION: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Anthracyclines are among the most common and effective drugs for the treatment of children ALL. However, long-term consumption and higher doses of these drugs may lead to toxic effects on the heart of children. For this purpose, in the present study, the baseline and posttreatment echocardiography status was evaluated in children with ALL during 7 years. MATERIALS AND METHODS: This retrospective cross-sectional study was performed on 53 children with ALL who were under the age of 18 years. Different factors including risk groups, age, gender, white blood cells, dosage, and duration of treatment, as well as baseline and posttreatment echocardiography findings including EF, E/E/, E//A/, MVE/A were evaluated in all patients. RESULTS: All enrolled patients had not any abnormalities in the baseline echocardiography preventing the beginning of chemotherapy. The results of this study did not show a significant difference in mean baseline echocardiography parameters after treatment including EF̨E/E/˛E//A/˛, MVE/A. In addition, there was no significant difference in mean EF, E//A/˛, MVE/A, and ˛E/E/ before and after treatment among SR, IR, and HR groups. Although there was no significant difference in the mean EF, E//A/, and MVE/A before and after treatment between male and female sex, the mean E/E/ after chemotherapy in girls (8.5 ± 0.7) was significantly higher than the mean before treatment (6.85 ± 1.5) (P < 0.001). It was also higher than the mean in boys (7.04 ± 0.99) (P = 0.019). Although there was no significant relationship between the duration of chemotherapy and the mean of EF̨E/E/˛E//A/˛, MVE/A after treatment, high dose of the drug was found to be significantly associated with a gradual decrease of EF or systolic function of the heart (P < 0.001). There was no significant relationship between drug dose and other parameters of echocardiography including E/ A/, MVE/A, and E/E/ after treatment. CONCLUSION: It appears that chemotherapy should not be delayed by echocardiography. Based on the findings presented herein, increasing the dose of anthracycline can be considered as an effective factor in reducing the systolic function of the heart (EF reduction). On the other hand, chemotherapy in the period of 1 to 5.3 years does not seem to have a significant effect on the mean parameters of EF, E/E/, E//, and MVE/A although another study with higher sample size and follow-up is needed to confirm these results.

17.
Leuk Res Rep ; 13: 100199, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32346509

RESUMEN

In this retrospective cohort study, oral glucose tolerance testing (OGTT) was performed for all enrolled survivors of patients who suffered from acute lymphoblastic leukemia (ALL) at least 9 months after ending chemotherapy, as well as for control group. All demographic, treatment and anthropomorphic data were obtained from medical records. Furthermore, insulin resistance was evaluated according to insulin level, Matsuda and HOMA-IR index. Of 53 subjects, 28 exhibited B-cell ALL, and 25 were healthy children (F/M = 1.12/1). Mean age (±SD) was determined to be 10.77 ± 2.83 years, and meantime off for therapy was also recorded as 3.0 ± 1.6 years. ALL patients were diagnosed at age 1-15 years; none of them received the cranial radiotherapy, and demographic characteristics (age, sex, and sexual maturity) were found to be the same in both groups. Seven patients showed transient hyperglycemia during ALL treatment, however, no significant differences were found in terms of body mass index (BMI) means between ALL patients with and without transient hyperglycemia. Based on the data presented herein, insulin resistance could not be predicted by glucose levels during therapy, cumulative asparaginase, and steroid. Our findings demonstrated that elevated BMI could be a valuable predictor of insulin resistance probability in ALL patients. The presence of transient hyperglycemia does not appear to be associated with the increased risk of glucose intolerance or insulin resistance in the first few years after completion of therapy.

18.
J Med Life ; 13(4): 484-489, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33456596

RESUMEN

Wilms' tumor is the most common kidney tumor of childhood. The outcome of this malignant tumor has improved due to the improvement of therapeutic strategies. The most important factor in determining the prognosis of these patients is the histopathology subtype of the tumor; unfavorable histopathology is seen in only 11.5% of the patients, which accounts for 52% of deaths. Therefore, the aim of this study was to determine the outcome of children with Wilms' tumor referred to our hospital over a period of 10 years. This is a retrospective cohort study, and the target population included all patients with Wilms' tumor referred to Ali Asghar Hospital and were treated according to the National Wilms tumor study 4 (NWTS-4) protocol. All patients' data were extracted from the medical records of the department. Overall survival and event-free survival (EFS) were analyzed by the Kaplan Mayer method in the SPSS software, version 23. Fifty-two patients (24 male and 28 female patients) with Wilms' tumor were included. The mean age of the subjects was 40 months. The most common stage among boys and girls was stage II (23.08% and 28.85%, respectively). Our findings revealed that the overall five-year survival of patients was 87±5%; this figure was determined as 100% for boys and 76.8% ± 1.6 for girls (P = 0.018). Our findings show a dramatic improvement in the outcome of children with Wilms's tumor, and our results are comparable with other results from developed countries. Gender may be an independent prognostic factor of children with Wilms' tumor.


Asunto(s)
Países en Desarrollo , Neoplasias Renales/terapia , Tumor de Wilms/terapia , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Lactante , Neoplasias Renales/patología , Masculino , Análisis Multivariante , Estadificación de Neoplasias , Pronóstico , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Resultado del Tratamiento , Tumor de Wilms/patología
19.
J Pediatr Hematol Oncol ; 42(1): 23-26, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31568183

RESUMEN

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


Asunto(s)
Transfusión Sanguínea , Deferasirox/administración & dosificación , Ferritinas/metabolismo , Talasemia beta , Administración Oral , Adolescente , Adulto , Niño , Deferasirox/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Irán , Masculino , Talasemia beta/sangre , Talasemia beta/terapia
20.
Med Arch ; 73(5): 311-315, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31819303

RESUMEN

INTRODUCTION: Acute lymphoblastic leukemia (ALL) is one of the most common cancers in children and accounts for about ⅓ of cancers in children. The annual incidence of ALL is 4 patients per 100,000 children. Their peak age is between 2-5 Year. One of the most important prognostic factors is cytogenetic abnormalities which are very effective in determining treatment policy. AIM: To determine the frequency of cytogenetic findings and its effect on the outcome of children with ALL. MATERIALS AND METHODS: This retrospective cross-sectional analytical study was conducted on children with ALL who their disease was diagnosed between 2001and 2009. Furthermore, 206 patients with ALL were examined by referring to Clinic of Ali Asghar Hospital in Tehran. Data was collected from medical records and analyzed by SPSS16 software. RESULTS: 206 children with ALL were enrolled in the study. The estimated event-free survival rate of all enrolled patients was more than 70%. There was a significant relationship between type of cytogenetic disorder and clinical outcome of patients (P˂0.0001), where the highest mortality was observed in patients with t (9;22) and t (4;11). There was no significant correlation between the sex and age with the clinical outcome of the patient (P = 0.064; p=0.322). There was a statistically significant relationship between mediastinal mass and clinical outcome (P = 0.002), indicating that the presence of cells growth in an involuntary way can be cause of the cancer. A significant association was found between the clinical outcome of patients and radiotherapy (P = 0.043), indicating that radiotherapy is effective in improving cancer. CONCLUSION: The findings demonstrated that the average survival rate without recurrence in children was at level of the European countries. However, the strong chemotherapy weakened the role of many prognostic factors in ALL patients, but some translocations are prognostic factors in predicting death in patients with ALL. Therefore, patients with this factor need to receive more confident treatment policy. Comprehensive studies are required by focusing on more samples because of low number of relapses and deaths in the present study.


Asunto(s)
Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Translocación Genética/genética , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Aberraciones Cromosómicas , Cromosomas Humanos Par 1 , Cromosomas Humanos Par 11 , Cromosomas Humanos Par 12 , Cromosomas Humanos Par 19 , Cromosomas Humanos Par 21 , Cromosomas Humanos Par 22 , Cromosomas Humanos Par 4 , Cromosomas Humanos Par 9 , Estudios Transversales , Diploidia , Síndrome de Down/genética , Femenino , Humanos , Lactante , Irán , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidad , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Pronóstico , Supervivencia sin Progresión , Radioterapia , Estudios Retrospectivos
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