Background-A key immunological feature of food allergy (FA) is the presence of a T-helper-2 (Th2)-type cytokine bias. Ligation of the invariant natural killer T cell (iNKT) T cell receptor (TCR) by sphingolipids (SL) presented via the CD1d molecule leads to copious secretion of Th2-type cytokines. Major food allergens (e.g. milk, egg) are the richest dietary source of SL (food-SL). Nonetheless, the role of iNKTs in FA is unknown.

Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency associated with an increased susceptibility

BackgroundWheat allergy is among the most common food allergy in children, but few publications are available assessing the risk of anaphylaxis due to wheat.MethodsIn this study, we report the case of near-fatal anaphylaxis to wheat in a patient undergoing an oral food challenge (OFC) after the ingestion of a low dose (256 mg) of wheat. Moreover, for the first time, we analyzed the risk of anaphylaxis during an OFC to wheat in 93 children, compared to other more commonly challenged foods such as milk, egg, peanuts, and soy in more than 1000 patients.ResultsThis study, which includes a large number of OFCs to wheat, shows that wheat is an independent risk factor that is associated with anaphylaxis requiring epinephrine administration (Odds Ratio [OR] = 2.4) and anaphylaxis requiring epinephrine administration to low dose antigen (OR = 8.02). Other risk factors for anaphylaxis, anaphylaxis requiring epinephrine administration, and anaphylaxis to low dose antigen was a history of a prior reaction not involving only the skin (OR = 1.8, 1.9 and 1.8 respectively). None of the clinical variables available prior to performing the OFC could predict which children among those undergoing OFCs to wheat would develop anaphylaxis or anaphylaxis for low dose antigen.ConclusionThis study shows that wheat is an independent risk factor that is associated with anaphylaxis requiring epinephrine administration and anaphylaxis requiring epinephrine administration to low dose antigen.

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<P>Adverse food reactions or food allergy can be the source of stress for many families. Some studies have assessed the quality of life for children with food allergy. One study showed that parents of children with food allergy report that family activities and general health perception are negatively affected. Cohen et al, using principles established in measuring disease-specific, health-related quality of life instruments, found that multiple domains are affected in families with food allergy including school, camp, social activities involving food, vacations, children in the care of others, restaurant meals, and being near other children who are eating. Parents also reported anxiety and frustration over nutritional issues, worry over not being able to help their child with a reaction, and whether their child eventually will overcome the allergy. Managing food allergy is an ongoing process involving cooperation between the parent and physician. Determining the etiology of food reactions can better guide therapies.</P> <H4>ABOUT THE AUTHORS</H4> <P>Lisa R. Forbes MD; Rushani W. Saltzman MD; and Jonathan M. Spergel, MD, PhD, are with The Children’s Hospital of Philadelphia, Allergy Section, Division of Allergy and Immunology, Philadelphia, PA.</P> <P>Address correspondence to: Jonathan M. Spergel, MD, PhD, 3550 Market Street, 3rd Floor, Philadelphia PA 19104; or e-mail <a href="mailto:spergel@email.chop.edu">spergel@email.chop.edu</a>.</P> <P>Dr. Forbes, Dr. Saltzman, and Dr. Spergel have disclosed no relevant financial relationships.</P> <H4>EDUCATIONAL OBJECTIVES</H4> <OL> <LI>Discuss the mechanisms behind food-related reactions.</LI> <LI>Describe a systematic approach to the evaluation of food allergy.</LI> <LI>Explain the relationship between IgE-mediated food allergy and atopic dermatitis.</LI> </OL>

This study confirms the efficacy and safety of CM-OIT as well as the role of iNKT cells in CM allergy.

Pneumocystis jirovecii is a leading cause of opportunistic infections among the immune compromised. During the 1980s, attention focused on patients with HIV, however, with the advent of anti-retroviral therapy, we wished to revisit the question of underlying diseases associated with Pneumocystis pneumonia in children. We identified 80 cases from 1986 to 2006 and performed a retrospective chart review to identify clinical characteristics for each of the cases. HIV was the single most common associated underlying condition seen in this cohort, accounting for 39% of the cases overall, however, it was seen in just 15% of the cases since 1998. Transplant recipients and oncology patients together comprised another 39% of the cases, with 9% of cases attributed to primary immune deficiency and another 9% of cases associated with less well-recognized causes of susceptibility. This study documents the ongoing need for vigilance to diagnose Pneumocystis pneumonia in less well-recognized clinical scenarios.

Two infants are described who presented with 22q11.2 deletion and a T(-)B(-)NK(+) immune phenotype. For both infants, the initial diagnosis was athymia secondary to complete DiGeorge anomaly. The first infant underwent thymus transplantation but 6 months after transplantation had circulating thymus donor T cells; the patient did not develop recipient naïve T cells. Genetic analyses revealed that both patients had Artemis deficiency, a rare form of severe combined immunodeficiency (SCID). Both infants have subsequently undergone bone marrow transplantation. These cases illustrate the importance and paradox of differentiating SCID from complete DiGeorge anomaly because hematopoietic stem cell transplantation (HSCT) is the preferred treatment for SCID but is ineffective for complete DiGeorge anomaly. However, if the thymus is completely absent, donor stem cells from a HSCT would not be able to be educated.