Oral Food Challenge for FPIES in Practice-A Survey: Report from the Work Group on FPIES Within the Adverse Reactions to Foods Committee, FAED IS, AAAAI.

BACKGROUND: Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food allergy diagnosed via history and/or an oral food challenge (OFC). OBJECTIVE: To determine allergists' approach to FPIES OFCs. METHODS: A web-based survey was e-mailed to 1100 randomly selected American Academy of Allergy, Asthma and Immunology members. RESULTS: A total of 132 individuals responded (12% response rate). A total of 95.5% (n = 105) of respondents perform OFCs in their practice, but only 58.7% (n = 71) perform FPIES OFCs. The median number of FPIES OFCs in children was reported as 3 per year (range, 0-76); all but 1 respondent (2.5%) had not performed any FPIES OFCs in adults. The most common FPIES OFC foods were cow's milk, rice, lightly cooked egg, oat, soy, baked milk, and baked egg. The decision to offer FPIES OFCs was based on the severity of past reactions, the patient and family's desire, and the patient's age. FPIES OFCs were most commonly performed in an outpatient setting, with placement of peripheral intravenous access depending on the severity of past reactions and with a serving appropriate for age divided into 3 equal portions administered over 30 minutes. There was significant variability in the approach to conducting FPIES OFCs. Most respondents (87.4%, n = 127) indicated that specific guidelines for performing FPIES OFCs would be helpful. CONCLUSIONS: Our study highlights the discordance in allergists' practices performing OFCs for IgE-mediated food allergy compared with FPIES. The lack of universal agreement on the optimal way to perform OFCs in FPIES demonstrates the need for future studies to develop a standardized protocol for FPIES OFCs.

Food Allergy Immunotherapy with Adjuvants.

Treatment of immunoglobulin E-mediated food allergy has traditionally been limited to allergen avoidance and emergency treatment after accidental ingestion. In recent years, significant progress has been made with oral, sublingual, and epicutaneous immunotherapy in the treatment of food allergy; however, these emerging treatment options have important limitations in efficacy, durability of effect, and safety. These limitations have already spurred interest in the development of adjuvant therapies to increase efficacy, produce more durable immunologic changes, and/or reduce treatment-related adverse reactions.

Disseminated Nannizziopsis Infection in an Adolescent With a STAT1 Mutation.

An adolescent with failure to thrive developed cuboid bone osteomyelitis and brain abscesses. Mold isolated from both locations was identified by universal genetic sequencing as Nannizziopsis spp, which is typically a pathogen of reptiles. The patient was subsequently diagnosed with a STAT1 mutation and was successfully treated.

Inherited and acquired clinical phenotypes associated with neuroendocrine tumors.

PURPOSE OF REVIEW: Overview of neuroendocrine neoplasms in the context of their associations with primary and secondary immunodeficiency states. RECENT FINDINGS: Malignancies of neuroendocrine origin are well known to be associated with hereditary syndromes, including multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, neurofibromatosis type 1, and tuberous sclerosis. This review includes the X-linked form of hyper-IgM syndrome (XHIGM), due to mutations in the CD40Ligand gene (CD40LG), as an additional inherited disorder with susceptibility to such malignancies, and discusses neuroendocrine tumors (NETs) arising in other immunocompromised states. Of all primary immune deficiency diseases, NETs appear to be unique to XHIGM patients. Outcomes for XHIGM patients with NETs is poor, and the mechanism behind this association remains unclear. In secondary immune deficiency states, NET occurrences were primarily in patients with HIV or AIDS, the autoimmune disease systemic lupus erythematosus and solid organ transplant recipients. Gastroenteropancreatic NETs were most frequent in XHIGM patients, whereas nongastroenteropancreatic-NETs, like Merkel cell carcinoma and small-cell lung carcinoma, affected HIV/AIDS patients. Possible mechanisms as to the nature of these associations are discussed, including chronic infections and inflammation, and CD40-CD40L interactions. Many questions remain, and further studies are needed to clarify the predisposition of patients with XHIGM to the development of NETs. Given that many of these patients present late in their disease state and have poor outcomes, it is imperative to keep a high index of suspicion at the advent of early signs and symptoms. Regular monitoring with laboratory or imaging studies, including tumor markers, may be warranted, for which further studies are needed. SUMMARY: Of all primary immunodeficiency diseases, NETs appear to be unique to XHIGM, and the mechanism behind this association remains unclear. Outcome for XHIGM patients with NETs is poor, and it is imperative to keep a high index of suspicion at the advent of early signs and symptoms.