Visual asymmetries for relative depth judgments in a three-dimensional space.

Our ability to process information about an object's location in depth varies along the horizontal and vertical axes. These variations reflect functional specialisation of the cerebral hemispheres as well as the ventral/dorsal visual streams for processing stimuli located in near and far space. Prior research has demonstrated visual field superiorities for processing near space in the lower and right hemispaces and for far space in the upper and left hemispaces.

Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis.

Purpose: Acute Hemorrhagic Leukoencephalitis (AHLE) is a rare demyelinating disorder of acute onset, rapid deterioration and significant morbidity and mortality. Most often described as a post-infectious complication of an upper respiratory illness, its precise pathophysiology remains unclear. We describe two pediatric patients with AHLE with partial complement factor I (FI) deficiency whose successful treatment included the interleukin-1 (IL-1) receptor antagonist, anakinra, implicating a role for FI and IL-1 in this disorder.

Recurrent fever syndromes in patients after recovery from Kawasaki syndrome.

The recurrence of fever in a child with a history of Kawasaki syndrome (KS) poses a dilemma for clinicians who must consider the possibility of recurrent KS. In this report we present the cases of 4 patients who presented with classical symptoms of KS, were successfully treated with intravenous immunoglobulin, and later experienced a reappearance of inflammatory symptoms in a pattern consistent with a recurrent fever syndrome. The association of these syndromes within the same patient suggests that some patients may have a genetic propensity toward altered immune responses and autoinflammatory syndromes.

Gerardo: asthma and cultural beliefs in a Latino family.

Gerardo is an 8-year-old Latino boy who saw his primary care pediatrician with a second asthma exacerbation this year. His frustration with his illness was immediately apparent when he said, "I hate having to go to the nurse's office to take my albuterol!" His mother expressed concern that her son frequently refused to take his prevention medication for asthma, montelukast, each morning. When questioned about compliance with his inhaled steroid, his mother hesitated and then admitted that she discontinued the controller medication because she is afraid to "poison his body with so many chemicals.

Mast cells infiltrate the esophageal smooth muscle in patients with eosinophilic esophagitis, express TGF-β1, and increase esophageal smooth muscle contraction.

Background: Increased numbers of mast cells are present in the esophageal epithelium in patients with eosinophilic esophagitis (EE). However, mast cell infiltration into the esophageal lamina propria (LP) and smooth muscle (SM) and the effects of their products on SM function has not been determined.

Objective: We investigated mast cell localization and characterization in esophageal SM, the functional significance of mast cell TGF-β1 expression to contraction of human esophageal smooth muscle (HESM) cells in vitro, and the effect of topical corticosteroids on the number of tryptase-positive (MC(T)) and chymase-positive (MC(C)) mast cells in patients with EE.

Oral viscous budesonide is effective in children with eosinophilic esophagitis in a randomized, placebo-controlled trial.

Background & Aims: Eosinophilic esophagitis (EoE) is caused by immunologic reactions to ingested/inhaled allergens. The diagnosis is considered if >or=15 eosinophils per high-powered field (eos/hpf) are detected in mucosal biopsies. Placebo-controlled studies have not been conducted to evaluate the safety and efficacy of oral viscous budesonide (OVB).

Hodgkin's and non-Hodgkin's lymphomas occurring in two brothers with Wiskott-Aldrich syndrome and review of the literature.

Approximately 13% of patients with Wiskott-Aldrich syndrome (WAS), a primary immune deficiency, develop malignant tumors, the predominant form being non-Hodgkin's lymphoma. Previously, only 4 cases of Hodgkin's lymphoma have been reported in WAS patients. Herein, we review the literature of WAS-related lymphomas and report 2 brothers with WAS who both developed lymphomas; one developed Epstein-Barr virus (EBV)-driven diffuse large B-cell lymphoma, and one developed EBV-negative classical Hodgkin's lymphoma.

A symptom scoring tool for identifying pediatric patients with eosinophilic esophagitis and correlating symptoms with inflammation.

Background: Eosinophilic esophagitis (EE) is an increasingly recognized allergic disease entity that is difficult to distinguish clinically from other causes of esophagitis, especially gastroesophageal reflux disease (GERD). To our knowledge, there are no prospectively analyzed or validated symptom scoring tools for pediatric patients with EE and no prospective evaluation correlating symptoms with tissue inflammation.

Objectives: To prospectively analyze a symptom scoring tool's ability to distinguish pediatric patients with EE from those with GERD and from control patients with and without allergies and to correlate symptoms with tissue inflammation.

The two emergencies of Kawasaki syndrome and the implications for the developing world.

Kawasaki syndrome (KS) is the most common cause of acquired pediatric heart disease in the developed world. There have been 2 distinctive patterns for the emergence of KS that are likely related to several factors including exposure to the causative agent(s) and host genetics. In Europe and North America where we presume the genetic susceptibility seems to be low, KS has existed in the pediatric population for more than a century and is associated with relatively low incidence.

Oral viscous budesonide: a potential new therapy for eosinophilic esophagitis in children.

Background: Eosinophilic esophagitis (EE) is a disorder characterized typically by pan-esophageal eosinophilia. We evaluate a palatable, long-acting topical corticosteroid preparation for the treatment of EE.

Study Design: This is a retrospective analysis of symptoms, endoscopic and histologic findings, efficacy, and safety of treatment in children with EE receiving oral viscous budesonide.

Delayed diagnosis by physicians contributes to the development of coronary artery aneurysms in children with Kawasaki syndrome.

Background: A diagnosis of Kawasaki syndrome is based on clinical criteria with nonspecific laboratory findings, and there is a substantial risk of coronary artery aneurysms if treatment with intravenous immunoglobulin is delayed. In this study, we examined the contributions of sociodemographic factors and parent and physician behavior to the development of coronary artery aneurysms in children with Kawasaki syndrome.

Methods: We performed a retrospective, case-control chart review of Kawasaki syndrome patients treated at our institution during an 11-year period (1991-2002).

Distinguishing eosinophilic esophagitis in pediatric patients: clinical, endoscopic, and histologic features of an emerging disorder.

Goals: To determine the clinical, endoscopic, and histologic criteria that distinguish children with eosinophilic esophagitis (EE) from those with non-EE diagnoses.

Background: EE is a disease of escalating incidence. Distinguishing children with EE from those with non-EE diagnosis can be difficult before endoscopy.

Esophageal remodeling in pediatric eosinophilic esophagitis.

Background: Eosinophils are associated with airway remodeling in asthma, but studies have not yet determined whether eosinophilic esophagitis (EE) is associated with esophageal remodeling.

Objective: We performed quantitative immunohistochemical analysis of remodeling changes in esophageal biopsy specimens from children with and without EE to evaluate if there were changes in the esophagus of pediatric patients with EE akin to airway remodeling. In addition, we determined whether the esophagus of patients with EE had increased levels of expression of TGF-beta(1) and its signaling molecule, phosphorylated SMAD2/3 (phospho-SMAD2/3).

Immunodeficiency in childhood.

Primary immunodeficiency disorders (PIDs) continue to illuminate mechanisms of human immunity and hypersensitivity. New discoveries in common variable immunodeficiency, the most enigmatic of PID syndromes, reveal molecular pathways of importance in human antibody production. FOXP3 mutations demonstrate the essential role that T-regulatory cells play in controlling autoantibody formation and disease.

Long-term efficacy of enzyme replacement therapy for adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID).

Adenosine deaminase (ADA)-deficient Severe Combined Immunodeficiency (ADA-deficient SCID) is characterized by impaired lymphocyte development and function resulting from the adenosine metabolism defect. Enzyme replacement therapy with polyethylene glycol-conjugated adenosine deaminase (PEG-ADA) minimizes infectious complications of ADA-deficient patients who have not received bone marrow transplantation. In PEG-ADA therapy, enzymatically active ADA continuously circulates to act as a metabolic sink, detoxifying the adenosine and deoxyadenosine metabolites that accumulate to high levels in the absence of ADA.

Staphylococcus aureus golden pigment impairs neutrophil killing and promotes virulence through its antioxidant activity.

Golden color imparted by carotenoid pigments is the eponymous feature of the human pathogen Staphylococcus aureus. Here we demonstrate a role of this hallmark phenotype in virulence. Compared with the wild-type (WT) bacterium, a S.

Infliximab treatment for refractory Kawasaki syndrome.

Objective: To evaluate the use of tumor necrosis factor (TNF)-alpha blockade for treatment of patients with Kawasaki syndrome (KS) who fail to become afebrile or who experience persistent arthritis after treatment with intravenous gamma globulin (IVIG) and high-dose aspirin.

Study Design: Cases were retrospectively collected from clinicians throughout the United States who had used infliximab, a chimeric murine/human immunoglobulin (Ig)G1 monoclonal antibody that binds specifically to human TNF-alpha-1, for patients with KS who had either persistent arthritis or persistent or recrudescent fever > or =48 hours following infusion of 2 g/kg of IVIG.

Results: Response to therapy with cessation of fever occurred in 13 of 16 patients.

Failure to diagnose Kawasaki disease at the extremes of the pediatric age range.

To learn about physician practices in diagnosing Kawasaki disease, we surveyed general pediatricians and pediatric infectious disease physicians by questionnaire. A high proportion of general pediatricians (>50%) and infectious disease subspecialists (25%) did not consider the diagnosis of Kawasaki disease in children younger than 6 months and older than 8 years. Failure to consider the diagnosis at the extremes of the pediatric age range puts children at risk because coronary artery abnormalities occur more often in young infants and adolescents with Kawasaki disease.

Chronic granulomatous disease caused by a deficiency in p47(phox) mimicking Crohn's disease.

We describe 2 cases of autosomal recessive chronic granulomatous disease (CGD) in 2 sisters presenting with a picture consistent with inflammatory bowel disease. The index case is a 10-year-old girl with a history of refractory Crohn's colitis treated with aggressive immunosuppressive therapy whose course subsequently was complicated by central nervous system aspergillosis. Additional evaluation showed a diagnosis of CGD, an underlying immunodeficiency in which phagocytes fail to produce microbicidal reactive oxygen intermediates because of inherited defects in the reduced form of nicotinamide-adenine phosphate dinucleotide (NADPH) oxidase.

The narratives of Kawasaki disease.

Kawasaki disease is a rash/fever illness of early childhood in which coronary artery aneurysms (CAA), sometimes fatal, may develop in up to 25 percent of untreated children. Because its etiology and pathophysiology are unknown and no diagnostic laboratory test exists, diagnosis is made via a list of clinical signs; however, a significant number of children fail to meet the clinical criteria and go on to develop CAA. We suspected a connection between these missed cases and the continuing difficulty in identifying the etiological agent(s) and mechanisms for CAA.

Rethinking the boundaries of Kawasaki disease: toward a revised case definition.

This paper describes the historical evolution of the Kawasaki disease (KD) case definition and its limitations for identification and treatment of children at risk for coronary artery aneurysms (CAA). The dominant view of pathogenesis is that an unknown agent infects infants and children, who then develop the signs of KD. Some of the infected infants and children then develop CAA, and a few die from myocardial infarction.