Syndromes predisposing to leukemia are a major cause of inherited cytopenias in children.

Prolonged cytopenias are a non-specific sign with a wide differential diagnosis. Among inherited disorders, cytopenias predisposing to leukemia require a timely and accurate diagnosis to ensure appropriate medical management, including adequate monitoring and stem cell transplantation prior to the development of leukemia. We aimed to define the types and prevalences of the genetic causes leading to persistent cytopenias in children.

Tramadol Treatment for Chemotherapy-induced Mucositis Pain in Children.

Mucositis, a painful and debilitating condition, is a common side effect of chemotherapy. The role of tramadol in the treatment of mucositis in pediatric patients has not yet been determined. In this retrospective study, we evaluate whether tramadol as single agent achieved a reduction of pain intensity among oncologic children admitted for mucositis.

Neutrophil Elastase Activity as a Surrogate Marker for Neutrophil Extracellular Trap Formation following Hematopoietic Stem Cell Transplantation.

Impaired neutrophil extracellular trap (NET) formation compromises the host defense after engraftment following hematopoietic stem cell transplantation (HSCT) despite adequate neutrophil counts. The aims of the present study were to determine reference ranges for the activity of key enzymes of NET formation-neutrophil elastase (NE) and myeloperoxidase (MPO)-in a healthy population and to unravel the recovery dynamics of NET formation over time following HSCT, along with NE and MPO enzymatic activities. Reference ranges of NE and MPO activity were derived from 50 healthy volunteers.

Neutrophil extracellular traps in pediatric inflammatory bowel disease.

Neutrophil extracellular traps (NETs) are fibers composed of chromatin and neutrophil proteins released by activated neutrophils. NETs trap and kill microbes, activate dendritic and T cells, and are implicated in autoimmune and vascular diseases. The pathogenesis of inflammatory bowel disease (IBD) is multifactorial and characterized by chronic active mucosal inflammation with controversial contribution of neutrophils.

Significant correlation between peripheral blood CD34+ cell count in children prior to aphaeresis and CD34+ cell yield following aphaeresis: A single-center experience.

Numerous adults' studies demonstrated that preaphaeresis CD34+ cells significantly correlate with the number of CD34+ cells collected by the aphaeresis procedure. Equivalent studies in children are scarce. We studied retrospectively 92 aphaeresis procedures performed following chemotherapy (44) or in steady state (48) in 60 pediatric patients (40 males, 20 females), median age of 7.

Lessons From an Outbreak of Varicella Infection in Pediatric Hemato-oncology Patients.

Background: Immunocompromised patients exposed to varicella may experience significant morbidity and a 7% mortality rate. Management and outcome of an outbreak of varicella infection among hospitalized pediatric hemato-oncology patients using the guidelines of the American Academy of Pediatrics Committee on Infectious Diseases are presented.

Methods: This retrospective study describes an outbreak of varicella infection between February 2011 and June 2011.

Reduced Neutrophil Elastase Activity and Neutrophil Extracellular Traps in Pediatric Acute Myeloid Leukemia May Increase the Rate of Infections.

Data on the production of neutrophil extracellular traps (NETs) in leukemia patients are scant. Phagocytosis, hydrogen peroxide, neutrophil elastase and myeloperoxidase enzymatic activity as well as NETs formation were studied in 10 pediatric acute lymphoblastic leukemia and 7 pediatric acute myeloid leukemia (AML) patients after induction chemotherapy. Median neutrophil elastase activity and NETs formation were lower in AML versus acute lymphoblastic leukemia (41% vs.

Chronic granulomatous disease: Clinical, functional, molecular, and genetic studies. The Israeli experience with 84 patients.

Chronic granulomatous disease (CGD) is an innate immunodeficiency with a genetic defect of the nicotinamide adenosine dinucleotide phosphate, reduced, oxidase components. This leads to decreased reactive oxygen species (ROS) production, which renders patients susceptible to life-threatening infections. Over the course of 30 years, we diagnosed CGD in 84 patients from 61 families using functional, molecular, and genetic studies.

Combined plerixafor and granulocyte colony-stimulating factor for harvesting high-dose hematopoietic stem cells: Possible niche for plerixafor use in pediatric patients.

PB is a source of HSC, especially for autologous HCT in solid tumors. However, there is a risk of failing to achieve the target number of SC after mobilization with growth factors alone in patients who were heavily pretreated with chemotherapy or those in need for tandem transplants. SC were harvested from seven pediatric patients with solid tumors who were in need of autologous HCT following combination GCSF and plerixafor.

A proposed role for neutrophil extracellular traps in cancer immunoediting.

Upon activation, neutrophils release fibers composed of chromatin and neutrophil proteins termed neutrophil extracellular traps (NETs). NETs trap and kill microbes, activate dendritic cells and T cells, and are implicated in autoimmune and vascular diseases. Given the growing interest in the role of neutrophils in cancer immunoediting and the diverse function of NETs, we searched for NETs release by tumor-associated neutrophils (TANs).

Lessons learned from phagocytic function studies in a large cohort of patients with recurrent infections.

Background: There is a paucity of data on the relationship between demographic characteristics, specific clinical manifestations, and neutrophil dysfunction, guiding physicians to decide which clinical signs and symptoms are a code for an underlying phagocytic disorder.

Methods: The data over a 21-year period of all adult and pediatric patients referred to our Laboratory for Leukocyte Functions with recurrent pyogenic infections were analyzed. Neutrophil function studies included chemotaxis, superoxide production (SOP), bactericidal activity (BA), and specific studies in case of suspected primary phagocytic disorder (PPD).

Pediatric chronic rhinosinusitis histopathology: differences and similarities with the adult form.

Objective: To compare the histopathology and immunohistochemistry of pediatric and adult chronic rhinosinusitis (CRS).

Study Design: Cross-sectional study.

Setting: University-affiliated hospital.

Blood sampling through peripheral venous catheters is reliable for selected basic analytes in children.

Objective: The goal was to determine the interchangeability of peripheral venous catheter (PVC) and venipuncture blood sampling (BS).

Methods: Paired blood samples from hospitalized children were obtained through venipuncture and from existing PVCs, following discard of 2 mL of blood. Comparisons of 9 complete blood count indices (white and red blood cell counts, hemoglobin and hematocrit levels, mean corpuscular volume, mean corpuscular hemoglobin level, red blood cell distribution width, platelet count, and mean platelet volume) and 5 basic chemical analysis indices (sodium, potassium, glucose, chloride, and urea levels) were performed, and hemolysis was documented.

Gastric emptying time of oral contrast material in children and adolescents undergoing abdominal computed tomography.

Objectives: : Considering the hazards of pulmonary aspiration of oral contrast material (OCM) during general anesthesia, we investigated the gastric emptying time (GET) of OCM in children and adolescents undergoing abdominal computed tomography (CT).

Patients And Methods: : Included in the study were 101 consecutive patients ages 3.1 to 17.

The effect of aerobic exercise on neutrophil functions.

Purpose: Intense exercise bouts are associated with a reduction of immune function and increased susceptibility to infections. Neutrophils act as a first line of defense to eliminate infectious agents and are also involved in muscle tissue inflammatory response to exercise. Intensive exercise suppresses several neutrophil functions including chemotaxis.

Transfusion-related acute lung injury following intravenous anti-D administration in an adolescent.

Transfusion-related acute lung injury (TRALI) is associated with administration of all plasma containing blood products. We present a 14-year-old adolescent diagnosed with idiopathic thrombocytopenic purpura who developed acute respiratory insufficiency compatible with TRALI within 5 hr following intravenous anti-D. Full blown noncardiogenic pulmonary edema was noted after 9 hr.

Widespread use of soy-based formula without clinical indications.

Objectives: In view of reports of the growing popularity of soy-based formula for infants, we examined soy consumption and its possible overuse during early infancy in central Israel.

Methods: Mothers of 1,803 infants aged 2, 4, 6 and 12 months attending well-baby clinics participated in a telephone survey covering background data, rate, duration, and pattern of soy-based formula use and the reasons for its initiation. The reasons were grouped into those based on the recommendations of the medical personnel and those based on mothers' initiative, and evaluated according to infants' age at soy-based formula initiation (0 to 1, 2 to 4 and 5 to 12 months).

Breast-feeding patterns in Central Israel.

Background: The rate of breast-feeding in Israel has increased over the last two decades but is still lower than rates in other developed countries that have taken an active role in promoting breast-feeding.

Objective: To determine breast-feeding patterns and the association between sociodemographic characteristics and breast-feeding in the Tel Aviv district.

Methods: The mothers of infants aged 2, 4, 6 and 12 months, attending 59 well-baby clinics in the Tel Aviv district, were interviewed by telephone.