Latent class analysis of hospitalization, ICU admission, and mortality among COVID-19 patients in Iran.

Identifying COVID-19 patients' clinical phenotypes based on characteristics and comorbidities, as well as their differences, helps in terms of clinical care and potential crises. Our goal is to use Latent Class Analysis (LCA) to identify COVID-19 patient profiles based on demographics, symptoms, and comorbidities, and evaluate their correlation with ICU admission, hospitalization duration, and mortality in a cross-sectional study. We included hospitalized patients with positive SARS-CoV-2 tests in two referral hospitals in the south of Iran between January 2020 and July 2021.

Abnormal frequency of the memory B cell subsets and plasmablasts in patients with congenital severe hemophilia A: correlation with "Inhibitor" formation.

Background: Development of antibodies against infused Factor VIII (FVIII) or "inhibitors" represents a major challenge following FVIII replacement therapy in patients with hemophilia A (HA). Recent studies have shown that certain cellular compartments of the immune system contribute to the production of such antibodies. Herein, we determined the frequency of class-switched CD19IgDCD27/non-class-switched CD19IgDCD27 memory B cell subsets and CD19CD27CD38 plasmablasts in patients with severe HA and their association with the development of inhibitors in these patients.

Severe acute respiratory syndrome coronavirus-2 Alpha variant (B.1.1.7), original wild-type severe acute respiratory syndrome coronavirus 2, and cytomegalovirus co-infection in a young adult with acute lymphoblastic leukemia, case report, and review of the possible cytomegalovirus reactivation mechanisms.

Background: Like other viral infections, severe acute respiratory syndrome coronavirus-2 infection could affect different human body systems, including host immune responses. Three years after its pandemic, we learn more about this novel coronavirus. As we expected, different co-infections with various organisms, such as viruses, bacteria, and even fungi, have been reported.

Cost-utility analysis of factor VIII diet therapies prepared using blood plasma vs. recombinant technique for patients with hemophilia A.

Background: Hemophilia is known as one of the most common coagulation disorders whose treatment costs are particularly high in developing countries, and about 90% of them are related to factor VIII (FVIII) and direct medical costs (DMCs). Thus, the present study aimed to analyze cost-utility of two FVIII diet therapies prepared using blood plasma and recombinant technique.

Methods: This study was an economic evaluation fulfilled through a cost-utility approach.

An immunocompetent patient with a nonsense mutation in NHEJ1 gene.

Background: DNA double-strand breaks (DSBs) are among the most deleterious types of DNA damage. DSBs are repaired by homologous recombination or non-homologous end-joining (NHEJ). NHEJ, which is central to the process of V(D)J recombination is the principle pathway for DSB repair in higher eukaryotes.

Expression of antiapoptotic proteins livin and survivin in pediatric AML patients, as prognostic markers.

Objective: Survivin and livin are highly expressed in various malignancies and their expression levels may be related to unfavorable prognosis. The aim was to investigate the relationships of these two markers with some prognostic factors and with survival of the children with acute myeloid leukemia (AML).

Methods: Livin and survivin expression was investigated quantitatively by immunohistochemistry staining technique in 43 primary formalin-fixed, paraffin-embedded bone marrow blocks in pediatric age group (<18 years).

A novel splice site mutation in WAS gene in patient with Wiskott-Aldrich syndrome and chronic colitis: a case report.

Background: Wiskott-Aldrich syndrome is an X-linked recessive immunodeficiency due to mutations in Wiskott-Aldrich syndrome (WAS) gene. WAS gene is encoded for a multifunctional protein with key roles in actin polymerization, signaling pathways, and cytoskeletal rearrangement. Therefore, the impaired protein or its absence cause phenotypic spectrum of the disease.

Congenital Orbital Rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common malignant tumor of the orbit in children, but it is rarely present at birth. We report a large congenital orbital RMS with intracranial extension in a newborn baby. A newborn baby girl was referred to our hospital due to severe right-eye exophthalmia.

Evaluation of bone mineral density in children with sickle-cell anemia and its associated factors in the south of Iran: a case-control study.

Sickle-cell anemia is a hereditary hemoglobin disorder among children. We showed that the low bone mass is prevalent among these children, and it has a negative association with hemoglobin. In spite of using supplementary 200 IU/day vitamin D, 59.

A case report of novel mutation in PRF1 gene, which causes familial autosomal recessive hemophagocytic lymphohistiocytosis.

Background: Hemophagocytic Lymphohistiocytosis (HLH) is a life-threatening immunodeficiency and multi-organ disease that affects people of all ages and ethnic groups. Common symptoms and signs of this disease are high fever, hepatosplenomegaly, and cytopenias. Familial form of HLH disease, which is an autosomal recessive hematological disorder is due to disease-causing mutations in several genes essential for NK and T-cell granule-mediated cytotoxic function.

Bone mineral density in children with acute leukemia and its associated factors in Iran: a case-control study.

Unlabelled: Acute leukemia is the most common malignancy in children. We showed that low bone mass is prevalent among children with leukemia, especially in femur. Serum calcium, exercise, chemotherapy protocol, and radiotherapy are the main contributing factors.

Hydroxyurea Treatment in Transfusion-Dependent β-Thalassemia Patients.

Background: β-Thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. Hemoglobin (Hb) F induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance.

Objectives: We aimed to investigate the efficacy and safety of Hydroxyurea (HU) in diminishing transfusion requirements of patients with β-thalassemia major in Southern Iran.

Diffuse hepatic calcifications in a transfusion-dependent patient with Beta-thalassemia: a case report.

Hepatic calcification is usually associated with infectious, vascular, or neoplastic processes in the liver. We report the first case of beta-thalassemia major with isolated diffuse hepatic calcification in a 23 year old woman, who had been transfusion-dependent since the age of 6 months. She was referred to our center with a chief complaint of abdominal pain.

Family planning practices in families with children affected by β-thalassemia major in Southern Iran.

Preventing the birth of children with β-thalassemia major (β-TM) is an important health issue. We investigated family planning practices and related factors among families with affected children. We selected a total of 569 parents from the parents of patients with β-TM who were registered at thalassemia referral clinics in southern Iran.

Iranian experience of deferasirox (Exjade(®)) in transfusion-dependent patients with iron overload: what is the most effective dose based on serum ferritin levels?

Background: The aim of this study was to evaluate the efficacy and safety of deferasirox in patients from Iran.

Methods: This was a retrospective, observational study in regularly transfused, iron-overloaded patients who received deferasirox 20-38 mg/kg/day for up to 12 months. Changes in serum ferritin were assessed as follows: from baseline to 3 months with deferasirox doses of 20-24 mg/kg/day; from 3 to 6 months with doses of 25-29 mg/kg/day; and from 6 to 12 months with doses of 30-38 mg/kg/day.

Frequency and distribution of asymptomatic brain lesions in patients with β-thalassemia intermedia.

We aimed to determine the frequency of asymptomatic brain lesions in a group of patients with β-thalassemia intermedia (β-TI) and to evaluate correlation of asymptomatic brain lesions with splenectomy, thrombocytosis, blood transfusions, and clinical parameters. Ninety five neurologically intact patients with β-TI were randomly enrolled in this cross-sectional study. Diffusion-weighted imaging brain MRI was performed in every patient to detect cerebral white matter lesions (WML).

Thyroid function and stress hormones in children with stress hyperglycemia.

The purpose of the study is to determine the prevalence of stress hyperglycemia and to investigate how thyroid and stress hormones alter during stress hyperglycemia in children admitted to pediatric emergency wards. A prospective cross-sectional study was conducted in children, less than 19 years old, who were admitted to pediatric emergency wards of Nemazee and Dastgheib Hospitals, Shiraz, Southern Iran. Those patients taking steroids, beta-agonists or intravenously administered glucose before venipuncture, and patients with diabetes mellitus (DM) or thyroid diseases were excluded.

Expression of UBE2Q2, a putative member of the ubiquitin-conjugating enzyme family in pediatric acute lymphoblastic leukemia.

Background: Acute lymphoblastic leukemia (ALL) is a cancer of the white blood cells most commonly found in childhood with a peak incidence at 2-5 years of age. The ubiquitin degradation pathway facilitates degradation of damaged proteins and regulates the growth and stress response. This pathway is activated in various cancers, including ALL.

Are laboratory tests necessary in making the diagnosis of limb pains typical for growing pains in children?

Background: The present study was conducted to determine if performing laboratory tests are necessary to exclude other possible diagnoses in a child with limb pains typical for growing pains according to history and physical examination.

Methods: A matched case-control study was carried out on children with limb pains who attended the Motahary clinic, Shiraz, Iran, in a 2 year period. The inclusion criteria were limb pains, which were recurrent and lasted for more than 3 months.

Hyperlipidemia in Iranian liver transplant recipients: prevalence and risk factors.

Background: Hyperlipidemia is a metabolic complication after liver transplantation (LT). The aim of this study was to investigate the prevalence and risk factors for developing hyperlipidemia in patients who underwent LT in the Shiraz Organ Transplantation Center.

Methods: Our patients were 170 liver recipients who underwent LT from 1994 to 2006 in the Organ Transplantation Center of the Shiraz University of Medical Sciences.