Deletion of tissue factor pathway inhibitor isoform beta or gamma, but not alpha, improves clotting in hemophilic mice.

Background: Tissue factor pathway inhibitor (TFPI) regulates tissue factor-triggered coagulation. Humans and mice express transcripts encoding for multidistributed (endothelial, platelet, and plasma) 3-Kunitz domain TFPIα and endothelial membrane-anchored 2-Kunitz TFPIβ. Mice express a third transcript, γ, that encodes plasma lipoprotein-associated 2-Kunitz TFPI.

Red blood cell distribution width as a potential inflammatory marker in pediatric osteomyelitis.

Background: Red cell distribution width (RDW) has been used in the differential diagnosis of anemia and revealed to be a potential marker of inflammation.

Method: We conducted a retrospective study of acute-phase reactant changes in correlation with RDW among pediatric patients with osteomyelitis.

Results: We identified 82 patients whose mean RDW increased on average by 1% during antibiotic therapy (mean 13.

Treatment and outcomes of symptomatic hyperammonemia following asparaginase therapy in children with acute lymphoblastic leukemia.

Hyperammonemia has been reported following asparaginase administration, consistent with the mechanisms of asparaginase, which catabolizes asparagine to aspartic acid and ammonia, and secondarily converts glutamine to glutamate and ammonia. However, there are only a few reports on the treatment of these patients, which varies widely from watchful waiting to treatment with lactulose, protein restriction, sodium benzoate, and phenylbutyrate to dialysis. While many patients with reported asparaginase-induced hyperammonemia (AIH) are asymptomatic, some have severe complications and even fatal outcomes despite medical intervention.

Peripheral blood mononuclear cell tissue factor (F3 gene) transcript levels and circulating extracellular vesicles are elevated in severe coronavirus 2019 (COVID-19) disease.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with excessive coagulation, thrombosis, and mortality.

Objective: To provide insight into mechanisms that contribute to excessive coagulation in coronavirus 2019 (COVID-19) disease.

Patients/methods: Blood from COVID-19 patients was investigated for coagulation-related gene expression and functional activities.

ALK-positive histiocytosis: a new clinicopathologic spectrum highlighting neurologic involvement and responses to ALK inhibition.

ALK-positive histiocytosis is a rare subtype of histiocytic neoplasm first described in 2008 in 3 infants with multisystemic disease involving the liver and hematopoietic system. This entity has subsequently been documented in case reports and series to occupy a wider clinicopathologic spectrum with recurrent KIF5B-ALK fusions. The full clinicopathologic and molecular spectra of ALK-positive histiocytosis remain, however, poorly characterized.

Evaluation of Pediatric Hematology Referrals at a Tertiary University Hospital in West Texas.

One in 40 pediatric office visits in the United States result in referral to subspecialty care, mostly for secondary opinion. The aim of this study was to evaluate the necessity of pediatric hematology referrals from Eastern New Mexico and West Texas to a tertiary university hospital. Retrospective data was obtained from chart review based on referrals made to the Southwest Cancer Center in Lubbock, TX for abnormal complete blood count or coagulation tests.

Screening Awareness of HPV-Related Oropharyngeal Cancers and Attitudes and Concerns towards HPV Vaccination Among Parents : HPV and Oropharyngeal Cancer.

Baseline population opinions on human papillomavirus (HPV) and HPV vaccination must be understood before physicians can address knowledge gaps in that population and encourage timely vaccination. To determine the opinions of parents of children age 9 to 18 on HPV-related oropharyngeal cancers (OPC); the associations with education level, socioeconomic status, and having a family member/friend with OPC; and the main concerns against having a vaccination., An anonymous survey was created and administered.

A novel mutation at the gene in a newborn with transient diabetes mellitus.

Neonatal diabetes mellitus is a monogenic disease that can present with hyperglycemia, dehydration, failure to thrive, and ketoacidosis within the first six months of life. Neonatal diabetes mellitus can be transient or permanent. Here, we describe a 10-week-old infant with transient neonatal diabetes mellitus who presented with diabetic ketoacidosis and was found to have heterozygous a de novo mutation, p.

How to Treat Cytomegalovirus-induced Hemophagocytic Lymphohistiocytosis in a Child With Leukemia.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by a hyperinflammatory state due to an aberrant activation of the immune cells. It can be familial or secondary to malignancy, autoimmune or metabolic diseases. Most HLH cases are triggered by infection.

SLCO1B1 Polymorphisms are Associated With Drug Intolerance in Childhood Leukemia Maintenance Therapy.

Background: Therapy discontinuations and toxicities occur because of significant interindividual variations in 6-mercaptopurine (6-MP) and methotrexate (MTX) response during maintenance therapy of childhood acute lymphoblastic leukemia (ALL). 6-MP/MTX intolerance in some of the patients cannot be explained by thiopurine S-methyl transferase (TPMT) gene variants. In this study, we aimed to investigate candidate pharmacogenetic determinants of 6-MP and MTX intolerance in Turkish ALL children.

Venlafaxine intoxication in an adolescent presenting with severe lactic acidosis.

Venlafaxine is a selective serotonin noradrenaline reuptake inhibitor and commonly prescribed antidepressant in adults. Most patients overdosing with venlafaxine develop only mild symptoms. Severe toxicity is reported with the most common symptoms being CNS depression, serotonin toxicity, seizure, or cardiac conduction abnormalities.

Idiopathic Pulmonary Hemosiderosis With Allergic Asthma Diagnosis in a Pediatric Patient.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder with unknown pathogenesis that usually presents in the first decade of life. As a result of diffuse alveolar hemorrhage, respiratory symptoms such as cough attacks, hemoptysis, dyspnea, and recurrent and refractory iron-deficiency anemia (IDA) are observed. We present an 8-year-old girl who was followed up with recurrent IDA and allergic asthma and later diagnosed with IPH.