Association between Transfusion-Related Iron Overload and Liver Fibrosis in Survivors of Pediatric Leukemia: A Cross-Sectional Study.

Purpose: Patients who receive frequent blood transfusions are at an elevated risk of developing hepatic fibrosis due to iron overload in the liver. In this study, we evaluated the effectiveness of transient elastography (TE) (FibroScan) for assessing liver fibrosis in patients with pediatric cancer.

Methods: We enrolled 106 consecutive cases of acute leukemia in individuals under 21 years of age.

The efficacy of L. in reducing neutrophil recovery time in childhood cancer with febrile neutropenia: a randomized, double-blind, placebo-controlled trial.

Introduction: Febrile neutropenia is a serious complication of cancer chemotherapy that can result in delays in treatment. This study evaluates the efficacy of L. at neutrophil recovery time in children with chemotherapy-associated febrile neutropenia.

Cardioprotective effects of deferoxamine in acute and subacute cardiotoxicities of doxorubicin: a randomized clinical trial.

Background: Cardiotoxicity is a major concern following doxorubicin (DOX) use in the treatment of malignancies. We aimed to investigate whether deferoxamine (DFO) can prevent acute cardiotoxicity in children with cancer who were treated with DOX as part of their chemotherapy.

Results: Sixty-two newly-diagnosed pediatric cancer patients aged 2-18 years with DOX as part of their treatment regimens were assigned to three groups: group 1 (no intervention, n = 21), group II (Deferoxamine (DFO) 10 times DOX dose, n = 20), and group III (DFO 50 mg/kg, n = 21).

Prognosis and Overall Survival of Thalassemia Patients using Semi-Parametric Mixture Cure-Rate Model: a 30-Year Retrospective Cohort Study.

Background: Understanding the determinants of long-term overall survival (OS) of thalassemia patients (TPs) is the mainstay of care.

Methods: As a retrospective survey, we assessed the data of 769 TPs who had regular follow-up and blood transfusion for at least 30 years from 1990 - 2019. We utilized semi-parametric proportional hazards mixture cure-rate regression to discover the factors with a significant effect on short- and long-term OS separately.

An evaluation of ascitic calprotectin for diagnosis of ascitic fluid infection in children with cirrhosis.

Background: The most common infection in children with the hepatic disease with or without cirrhotic ascites is spontaneous bacterial peritonitis (SBP), which occurs in the absence of an evident intra-abdominal source of infection. The present study aims to assess the value of calprotectin in ascitic fluid in the diagnosis of ascitic fluid infection in children with liver cirrhosis.

Materials And Methods: In this cross-section study, 80 children with underlying liver disease who attended the Hepatology and Emergency Department in Shiraz University Hospitals were studied.

Study of the Serum Immunoglobulin and Cell-Mediated Immunity in Patients with Congenital Severe Hemophilia.

Background: It has been shown that a close relationship exists between the immune system and coagulation cascade. Hemophilia A is an X-linked, recessive bleeding disorder caused by deficiency of functional plasma clotting factor VIII that is classically treated with factor VIII replacement therapy. Despite this, some patients produce inhibitors or antibodies against epitopes of infused factor VIII, indicating the activation of the adaptive immune system.

Feasibility and Therapeutic Potential of 177Lu-Fibroblast Activation Protein Inhibitor-46 for Patients With Relapsed or Refractory Cancers: A Preliminary Study.

Introduction: Fibroblast activation protein (FAP) is a member of the serine protease family and has a high expression in the stroma of approximately 90% of epithelial malignancies. The present investigation aimed to assess the feasibility, safety, and dosimetry data of 177Lu-FAPI-46 in diverse malignancies.

Patients And Methods: Patients with advanced cancers with nonoperable tumors, or tumors refractory to conventional therapies, were enrolled.

Extramedullary manifestations in acute lymphoblastic leukemia in children: a systematic review and guideline-based approach of treatment.

Objective: Acute lymphoblastic leukemia (ALL) may present with signs and symptoms related to extramedullary involvement, therefore, leads to delayed diagnosis of ALL in children. This study aims to consider the extramedullary manifestations of ALL in children and their proper treatment.

Method: The databases were searched for all relevant subjects including "acute lymphoblastic leukemia", "clinical presentation", "unusual presentation", "childhood acute lymphoblastic leukemia", "presenting features of ALL", "extramedullary presentation", and "atypical presentation" from April 1968 to June 2020.

A novel frame-shift deletion in FANCF gene causing autosomal recessive Fanconi anemia: a case report.

Background: Fanconi anemia (FA) is a heterogeneous genetic disorder characterized by congenital anomalies, early-onset bone marrow failure, and a high predisposition to cancers. Up to know, different genes involved in the DNA repair pathway, mainly FANCA genes, have been identified to be affected in patients with FA.

Case Presentation: Here, we report clinical, laboratory and genetic findings in a 3.

Successful treatment of refractory metastatic neuroblastoma with panobinostat in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine therapy.

Introduction: Neuroblastoma commonly required multimodal therapy containing surgery, chemotherapy, radiotherapy, and immunotherapy.

Case Report: In our case, who had refractory metastatic neuroblastoma, we use histone deacetylase inhibitor (panobinostat) in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine (MIBG) therapy.

Management And Outcome: This approach leads to successfully treat the patient.

Gynecomastia as a late complication of childhood cancer and its treatment that can affect the quality of life of male survivors.

Childhood cancer is relatively rare, and nowadays it is curable in more than 80% of children. Childhood cancer therapy is directed not only at improving survival but recently, we also concentrate on reducing late effects. We want children who have a diagnosis of cancer to survive and have an excellent quality of life.

Thrombosis in pediatric malignancy: a review and future perspectives with focus on management.

: Venous thromboembolism (VTE) result in significant morbidity and mortality in children with cancer. The cause of VTE in children with cancer is multifactorial and includes genetic predisposition (thrombophilia), disease-related factors, and treatment-related factors including use of central venous catheter (CVC), surgery, and chemotherapy. This review aims to examine current knowledge regarding the incidence, risk factors, clinical manifestation, evaluation, prevention, and management of thromboembolic events in children with cancer.

Effect of ursodeoxycholic acid and vitamin E in the prevention of liver injury from methotrexate in pediatric leukemia.

Background/aims: Ursodeoxycholic acid (UDCA) and antioxidants such as vitamin E are considered to have a protective role in preventing chemotherapy-induced liver damage. The aim of this study was to assess the efficacy of these agents for hepatoprotection in pediatric patients with B-cell acute lymphoblastic leukemia (ALL), who were treated with methotrexate in their maintenance phase of treatment.

Materials And Methods: Eighty children with B-cell ALL were randomly divided into four groups.

Acute Progressive Visual Loss in a Case of Acute Myeloid Leukemia: Challenges in the Utility of Molecular Tests in Early Diagnose of Cytomegalovirus Retinitis.

Cytomegalovirus (CMV) retinitis is one of the rare but debilitating presentations of the CMV infection in children with leukemia. Herein, we report a 12-year-old boy with acute myeloid leukemia complicated by rapid progressive visual loss during relapse of leukemia. The definite diagnosis of CMV retinitis was made after vitreous aspiration.

Neurological Crises after Discontinuation of Nitisinone (NTBC) Treatment in Tyrosinemia.

Objective: Tyrosinemia type 1 is a hereditary disorder with liver, kidney and nervous system involvement. Neurological crises can occur in tyrosinemic patients without treatment or when treatment stops. Here we report three children that developed diaphragmatic paralysis after discontinuation of nitisinone.

Colonic basidiobolomycosis with liver involvement masquerading as gastrointestinal lymphoma: a case report and literature review.

Basidiobolomycosis is an unusual fungal skin infection that rarely involves the gastrointestinal tract. This study reported a 5-year-old boy with gastrointestinal basidiobolomycosis that had been misdiagnosed as gastrointestinal lymphoma. He was treated by surgical resection and a combination of posaconazole and amphotericin B deoxycholate with an acceptable response and no recurrence.

Esthesioneuroblastoma Presenting with Bilateral Proptosis and Blindness in a Child: A Case Report.

Esthesioneuroblastoma is a rare malignant tumour of the olfactory epithelium. The most common symptom is related to unilateral nasal obstruction. It rarely presents with bilateral proptosis and blindness.

Acquired Vitamin K Deficiency as Unusual Cause of Bleeding Tendency in Adults: A Case Report of a Nonhospitalized Student Presenting with Severe Menorrhagia.

We report a rare case of acquired vitamin K deficiency presenting with severe menorrhagia and without any gynecological problem. Partial thromboplastin time (59.2 seconds) and prothrombin time (33.

Evaluation of Metabolic Syndrome and Related Factors in Children Affected by Acute Lymphoblastic Leukemia.

Introduction: Obesity is among the medical problems in survivors of childhood acute lymphoblastic leukemia (ALL). These patients are at risk of metabolic syndrome (MS). The present study aimed to follow the patients with ALL regarding the incidence of MS.

Hodgkin Lymphoma in a Patient With IFAP Syndrome: A Case Report and Review of Literature.

The IFAP syndrome is a rare X-linked recessive inheritance disorder with defect of the MBTPS2 gene defined by the triad of follicular ichthyosis, alopecia, and photophobia. A total of 40 cases has been reported, but no correlation with Hodgkin lymphoma has been reported yet. A 3.

Soft Palate Ulcer: An Unusual Presentation of a Posttransplant Lymphoproliferative Disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a serious complication of solid organ transplantation. It has several clinical manifestations, including infectious mononucleosis-like syndrome, lymphadenopathy, and other organ involvement. Soft palate ulcer is a rare manifestation of PTLD.

Rigler Sign in a Child With Posttransplant Lymphoproliferative Disease: A Sign That Should Not Be Missed.

Rigler sign is a double wall sign suggesting pneumoperitoneum and intestinal perforation, and it needs emergency surgical treatment. Early diagnosis of intestinal perforation by clinical symptoms, presence of Rigler sign in abdominal radiography, and then early surgical treatment can reduce mortality. Here, we report a patient with Crigler-Najjar syndrome who underwent liver transplant and then developed posttransplant lymphoproliferative disease and received chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab.

DIAGNOSTIC ACCURACY OF UREA BREATH TEST FOR HELICOBACTER PYLORI INFECTION IN CHILDREN WITH DYSPEPSIA IN COMPARISON TO HISTOPATHOLOGY.

Background: Helicobacter pylori infection is the gram negative bacillus with the close association with chronic antral gastritis.

Objective: In this study, we evaluate the accuracy of urea breath test (UBT) with carbon isotope 13 in comparison with histopathology of gastric antrum for detection of H. pylori infection in children with dyspepsia.

Effect of Ursodeoxycholic Acid on Indirect Hyperbilirubinemia in Neonates Treated With Phototherapy.

Background: Hyperbilirubinemia is a common neonatal problem. The present study aimed to investigate the effect of ursodeoxycholic acid in reducing indirect hyperbilirubinemia of infants under phototherapy.

Methods: This double-blind randomized clinical trial was conducted on neonates with jaundice, who had received phototherapy in the hospitals affiliated with the Shiraz University of Medical Sciences in 2013.