Fanconi Anemia: Challenges in Diagnosis and Management-A Case Series Report.

Fanconi anemia (FA) is a rare inherited disorder characterized by congenital abnormalities, progressive bone marrow failure, and a predisposition to malignancies. Detecting FA can be challenging, as it involves identifying increased chromosomal sensitivity to DNA cross-linking agents and detecting causative genetic variants via genome sequencing. We report two cases of siblings with FA, both confirmed to have the FANCD2 variant through whole-exome sequencing (WES).

Examining the relationship between land use and childhood leukemia and lymphoma in Tehran.

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.

Effect of oral silymarin on liver function in pediatric acute lymphoblastic leukemia in the maintenance phase: a double-blind randomized clinical trial.

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.

Correlation of Magnesium Level with Cardiac and Hepatic Hemosiderosis Measured by T2*MRI Technique and Age in Patients with Thalassemia Major.

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). In a descriptive cross-sectional study, a total of 62 patients with β-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.

The Effect and Safety of Olanzapine on Nausea and Vomiting in Children Receiving Moderately Emetogenic Chemotherapy.

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.

Health anxiety and the negative interpretation of children's bodily symptoms in mothers of cancer patients.

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.

Polyneuropathy Associated with Age of Starting the Transfusion and Serum Ferritin Level in Iranian Patients with Thalassemia Major and Intermedia.

Considering the importance of managing patients with β-thalassemia and the importance of early detection of disease complications, we examined the rate of sensorimotor neuropathy in patients with β-thalassemia and the risk factors related to it. This cross-sectional study included 44 blood transfusion-dependent β-thalassemia patients aged 5 years and older. Nerve conduction studies (NCSs) were performed via standard procedures for both motor and sensory nerves.

Magnesium Supplementation May Not Be Protective against Carboplatin-Induced Nephrotoxicity But May Be Beneficial for Children Suffering Malignancies: A Randomized Clinical Trial.

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 ( = 18) was treated with 250 mg/day magnesium oxide supplementation (MOS) and compared with a matched placebo-treated group ( = 18).

Comparison of the effect of ferrous sulfate and ferrous gluconate on prophylaxis of iron deficiency in toddlers 6-24 months old: A randomized clinical trial.

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.

Kidney Failure in Children with Wilms Tumor: A Study Based on Urine Analysis and Ultrasound.

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.

Correlation between Myocardial Iron Overload Detected by CMRT2* and Left Ventricular Function Assessed by Tissue Doppler Imaging in Patients with Thalassemia Major.

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*.

Evaluating Aprepitant single-dose plus granisetron and dexamethasone in children receiving highly emetogenic chemotherapy for the prevention of chemotherapy-induced nausea and vomiting: A triple-blinded randomized clinical trial.

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.

Evaluation of the Relationship Among Pulmonary Artery Pressure with Serum Ferritin Level and CMR T2* in Thalassemia Major.

Background: Pulmonary hypertension is one of the most common cardiac complications among patients with β-thalassemia major (β-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 β-TM.

Method: This cross-sectional study was performed on 50 patients older than 7 years old.

Sirolimus versus cyclosporine for the treatment of pediatric chronic immune thrombocytopenia: A randomized blinded trial.

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.

Correlation of Cardiac and Liver Iron Level with T2*MRI and Vitamin D3 Serum Level in Patients with Thalassemia Major.

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.

Alteration in Expression of miR-32 and FBXW7 Tumor Suppressor in Plasma Samples of Patients with T-cell Acute Lymphoblastic Leukemia.

Background: T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive and malignant neoplasm that arises from the hematopoietic T-cell precursors. Inactivation of gene is frequently observed in T-cell acute lymphoblastic leukemia, suggesting a significant tumor-suppressive role for 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 in patients with T-ALL.

A 1-year randomized trial of deferasirox alone versus deferasirox and deferoxamine combination for the treatment of iron overload in thalassemia major.

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.

Pantoprazole reduces serum ferritin in patients with thalassemia major and intermedia: A randomized, controlled study.

Aim: Complications due to iron overload exert a problematic situation in patients with thalassemia. Proton pump inhibitors (PPIs) like pantoprazole are effective agents to reduce acid gastric acid secretion and perhaps to interrupt iron absorption in conditions with increased iron absorption. Our purpose was to study effects of pantoprazole addition to chelators on iron levels in patients with thalassemia major and intermedia.

Comparative evaluation of the safety and efficacy of recombinant FVIII in severe hemophilia A patients.

Objective: This study compared the safety and efficacy of Safacto versus xyntha in patients with severe hemophilia A.

Methods: Thirty-three male patients with severe hemophilia A were randomly divided into two groups. Seventeen patients received Safacto and 16 patients received Xyntha for four consecutive times.

A 24-Week Treatment of Pediatric Hemangioma with Oral Propranolol.

Hemangioma is a benign vascular tumor that shouldbe treated in problematic situations.Propranolol efficacy, target dose, range of age, duration of treatment and complications arenot conclusive for treatment of pediatric hemangioma. Our goal was to study efficacy and safety of propranolol for hemangioma treating in children.

A randomized, controlled study evaluating effects of amlodipine addition to chelators to reduce iron loading in patients with thalassemia major.

Aim: Cardiomyopathy due to iron overload can be fatal in patients with thalassemia major. Calcium channel blockers seem to be effective to reduce iron loading. Our goal was to study effects of amlodipine addition to chelators on iron loading in patients with thalassemia major.

Comparison between IV immune globulin (IVIG) and anti-D globulin for treatment of immune thrombocytopenia: a randomized open-label study.

To compare the effect of IV immune globulin (IVIG) and anti-D globulin (anti-D) for treatment of immune thrombocytopenia (ITP) in children. A randomized, open-label, single-center clinical trial was carried out in Amir-Kabir Hospital (Arak, Iran). The study was performed on 60 children with acute and chronic ITP, aged from 1 to 15 years.