Spontaneous resolution of severe idiopathic T cell lymphopenia.

Potential etiologies of T-B+NK+ SCID include both hematopoietic defects and thymic aplasia. The management of patients with this phenotype, identified by newborn screen, may be unclear in the absence of a genetic diagnosis. We report an infant with lymphocyte flow cytometry consistent with T-B+NK+ SCID and reduced proliferative response to phytohemagglutinin. The patient had no genetic diagnosis after targeted panel and exome sequencing. The decision to trend laboratory values rather than move immediately to hematopoietic cell transplant was made given the absence of a genetic defect and the finding of a normal thymus on ultrasound. During the course of evaluation for transplant, the patient unexpectedly had normalization of T cell number and function. This case demonstrates a role for mediastinal ultrasound and the utility of trending laboratory values in patients with severe T cell lymphopenia but no genetic diagnosis, given the small but important possibility of spontaneous resolution.

Frequent Versus Infrequent Bathing in Pediatric Atopic Dermatitis: A Randomized Clinical Trial.

BACKGROUND: Studies evaluating bathing frequency in pediatric atopic dermatitis (AD) are limited. Parents of children with AD often receive conflicting information, leading to frustration and confusion. OBJECTIVE: To evaluate efficacy of twice-daily soaking baths, followed by immediate application of an occlusive moisturizer (ie, soak-and-seal [SS]), versus twice-weekly SS baths, in the acute management of pediatric AD. METHODS: We conducted a randomized, single-blind, crossover-controlled trial comparing frequent versus infrequent SS baths, in children 6 months to 11 years of age with moderate-to-severe AD. Children were randomized 1:1 into 2 groups: group 1 underwent twice-weekly SS baths, for 10 minutes or less, over 2 weeks ("dry method" [DM]) followed by twice-daily SS baths, for 15 to 20 minutes, over 2 weeks ("wet method" [WM]). Group 2 did the inverse. Patients received the same moisturizer, cleanser, and low-potency topical corticosteroid (TCS). Primary outcome was AD severity evaluated using the SCORing Atopic Dermatitis (SCORAD) index. Caregiver assessment of AD severity (Atopic Dermatitis Quickscore [ADQ]), quality of life, Staphylococcal aureus colonization, skin hydration, moisturizer, and TCS usage were assessed. RESULTS: Of the 63 children screened, 42 fulfilled inclusion criteria and were randomized. Forty (95%) completed the study. WM decreased SCORAD by 21.2 compared with DM (95% confidence interval [CI], 14.9-27.6; P < .0001). Secondary analysis showed a greater than 30% SCORAD improvement for WM versus DM (McNemar's χ2 = 8.83, df = 1, P = .0030). SCORAD correlated with ADQ (r = 0.66), and ADQ also showed significant improvement with WM decreasing ADQ by 5.8 (95% CI, 1.8-9.7). No other secondary endpoints showed significance. CONCLUSIONS: As an acute treatment intervention, WM is superior to DM at improving disease severity in moderate-to-severe pediatric AD.

Bathing Frequency Recommendations for Children with Atopic Dermatitis: Results of Three Observational Pilot Surveys.

The results from three online surveys of dermatologists, allergists and immunologists, and primary care physicians (PCPs) regarding routine bathing frequency recommendations for children with atopic dermatitis (AD) are presented. The results suggest that PCPs approach bathing frequency differently than specialists, with PCPs recommending daily bathing less than 50% of the time and specialists recommending daily bathing more than 50% of the time. Because there is lack of consensus, studies are needed to evaluate whether bathing frequency makes a clinical difference in the treatment of pediatric AD.

Role of bacterial superantigens in atopic dermatitis : implications for future therapeutic strategies.

The role of staphylococcal superantigens in the pathophysiology of atopic dermatitis (AD) has been the focus of intense interest during the past decade. Although the increased prevalence of Staphylococcus aureus and its bacterial toxins in AD skin is well established, exploitation of the known mechanisms of superantigens in this disease for the development of novel therapies remains an active area of research. With the emergence of multi-drug resistant S. aureus, the need for a better understanding of the pathophysiology of bacterial superantigens in AD has become increasingly important. This review examines the mechanisms of S. aureus colonization and infection, of which the most important are defective skin barrier function, increased S. aureus adherence, and the decreased innate immune responses found in AD skin. The contribution of superantigens to the pathophysiology of AD is then discussed. Important immunologic mechanisms in this context include the role of superantigens in promoting T helper-2 skin inflammation, IgE production, T-regulatory cell subversion, expansion and migration of skin-homing T cells, and IgE anti-superantigen production. Lastly, these findings are discussed with reference to current therapeutic approaches, of which the most important include anti-inflammatory and antimicrobial medications, and future strategies, which are expected to consist of immune-modulators and synthetic antibacterials.

Staphylococcal enterotoxin B inhibits regulatory T cells by inducing glucocorticoid-induced TNF receptor-related protein ligand on monocytes.

BACKGROUND: Superantigens inhibit naturally occurring CD4+CD25+ regulatory T-cell (nTreg) activity, yet the mechanism for this is unknown. OBJECTIVE: We tested the hypothesis that staphylococcal enterotoxin B (SEB), a prototypic superantigen, inhibits the ability of nTregs to suppress T-effector cell (Teff) proliferation by an antigen-presenting cell-dependent cell contact mechanism and the induction of glucocorticoid-induced TNF receptor-related protein ligand (GITR-L) on monocytes. METHODS: Cell proliferation assays were performed on immunomagnetic-bead separated CD4+CD25+, CD4+CD25- T cells and CD14+ monocytes using anti-CD3, SEB, or a neutralizing anti-GITR-L antibody for stimulation. A cytokine permeable membrane insert separating cells in culture was used to evaluate the role of cell contact. Proliferation was measured by [3H]-thymidine incorporation or a carboxyfluorescein diacetate succinimidyl ester assay. GITR-L expression was determined by RT-PCR and immunostaining. RESULTS: Antigen-presenting cell contact was required for proliferation of nTregs and loss of their ability to suppress Teff proliferation as confirmed by carboxyfluorescein diacetate succinimidyl ester. At baseline, monocytes expressed no GITR-L, but after SEB stimulation, GITR-L gene and protein expression increased significantly over a period of 24 hours (P<.05). When GITR-L activity was neutralized with blocking antibody, as opposed to isotype control, nTregs suppressed Teff proliferation despite the presence of SEB (P<.05). Likewise, loss of GITR-L at 48 hours was associated with restoration of nTreg activity (P

Factors that regulate naturally occurring T regulatory cell-mediated suppression.

T regulatory (Treg) cells play a key role in the modulation of allergic and autoimmune responses. This review will discuss the factors that regulate naturally occurring Treg (nTreg) cell-mediated suppression. The involvement of cytokines, costimulatory molecules, and ligands on antigen-presenting cells in the inhibition of nTreg cell-mediated suppression in vitro is summarized. Understanding the events that control suppressive function of nTreg cells will allow manipulation of these cells to inhibit or enhance their functions in the development of novel therapies for autoimmune and allergic diseases.

Asthma adherence and the athlete.

Although much has been written on the topic of adherence (also known as compliance) in asthma, little is known about adherence in the asthmatic athlete. There are shared reasons for poor adherence to treatment among athletes of varying ages and levels of competition. The Shared Decision-Making model is considered to be the optimal approach to enhancing adherence among all patients with chronic disease but is particularly well applied to athletes with asthma. This article discusses athlete-related, physician-related, and treatment-related barriers to adherence, as well as how to overcome these barriers to promote optimal disease management. Special considerations for the elite or professional athlete are also addressed.

A pilot survey of beta2-agonist inhaler availability for children with asthma during organized sporting events.

BACKGROUND: One of the main stimuli that cause asthma exacerbations in children is physical activity, and exercise-induced bronchospasm (EIB) occurs in most asthmatic patients. OBJECTIVE: To investigate the level of preparedness for a potential EIB event in suburban children involved in recreational sports. METHODS: A cross-sectional study, focusing on children 12 years and younger, who participated in Little League baseball or soccer in Lower Merion Township, PA, was conducted by clinical survey. The survey was performed during a face-to-face encounter with the parent or caretaker of the child. RESULTS: Five hundred seventy-nine children 12 years and younger were surveyed. The parents or caretakers of 80 reported a history of asthma, indicating a prevalence of 14%. Only 18 (22%) of these children with asthma were reported to have a rescue medicine readily available. The 95% confidence interval was 14.22% to 33.47%, indicating that we can be 95% certain that the true population with rescue medication lies between 14% and 33%. CONCLUSIONS: Despite having been diagnosed as having asthma, more than 75% of children surveyed were unprepared for an EIB event. Measures should be explored to improve the quality of care of asthmatic children involved in community sports. We also propose that it may be beneficial for first-aid kits to include a beta2-agonist bronchodilator.

Cura operatoria de hernia inguino escrotal: epidemiología y factores asociados: Hospital Dr. Egidio Montesinos El Tocuyo, enero 1991-julio 1998 / Operatory parish of hernia inguino escrotal: epidemiology and associated factors. Hospital Dr. Egidio Montesinos, El Tocuyo, january 1991-july 1998

La hernia inguinal es un padecimiento muy frecuente en nuestro medio. Con el objeto de analizar 279 hernioplastias en 7 años, se realizó un estudio retrospectivo, con un muestreo no probabilístico de clase accidental. Se estudiaron 259 pacientes que ingresaron al Hospital Dr. Egidio Montesinos de El Tocuyo por presentar hernias inguinales en el período comprendido entre enero 1991-julio 1998. El 80,31 por ciento pertenecían al sexo masculino con una mayor proporción en el grupo etario de > 60 años (17.37 por ciento). El 69.51 por ciento presentó hernia inguinal indirecta siendo la localización derecha la predominante (53.28 por ciento). La hernia inguinal derecha predominó en el sexo masculino y la izquierda en el femenino. El 1.54 por ciento presentó complicaciones incarceración (1.15 por ciento) y el estrangulamiento (0.38 por ciento), donde el 98.45 por ciento de las intervenciones fueron electivas y 1.55 por ciento emergencias. La anestesia general inhalatoria y la local fueron las más utilizadas en el acto quirúrgico. Los hallazgos de la intervención fueron: epiplón mayor (30,4 por ciento), lipoma preherniario (10,4 por ciento) y asas delgadas (5,9 por ciento). Las patologías asociadas encontradas son: hernia umbilical (53.1 por ciento) e hidrocele (31.25 por ciento). El tiempo de permanencia hospitalaria post operatoria fue < 24 horas (56.29 por ciento). Las historias clínicas se llenan en forma deficiente y hay un subregistro de intervenciones quirúrgicas que no se reportan en la institución