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Food Protein-Induced Enterocolitis Syndrome (FPIES)
What is food protein-induced enterocolitis syndrome (FPIES)?
FPIES is a type of food allergy that affects the gastrointestinal system. Patients’ blood tests often
look similar to those typical of infection. Untreated FPIES has progressed to acidemia and shock;
shock is estimated to occur in 15% to 20% of patients. FPIES often resolves with age. A recent
study of patients with FPIES triggered by milk showed that 50% recovered by 12 months of age,
75% by 18 months, 89% by 24 months, and 94% by 30 months. The mean age at presentation is
5.5 months.
What causes FPIES?
The pathophysiology of FPIES is not well understood. Reports of several immunologic
alterations suggest the involvement of antigen-specific T cells and their production of
proinflammatory cytokines that regulate the permeability of the intestinal barrier. It is also
thought that humoral immune responses are involved.
What are the symptoms of FPIES?
 Profound vomiting (approximately 1 to 6 hours after ingestion of the trigger food)
 Diarrhea (approximately 2 to 10 hours after ingestion of the trigger food)
 Dehydration
 Lethargy
 Fever
 Changes to blood pressure
 Pallor or cyanosis
 Failure to thrive
 High rate of atopic disease (approximately 40% to 60%, described by case series of patients)
What are the most common triggers of FPIES?
In infancy, reactions are most commonly secondary to cow’s milk-based or soy-based formulas.
Some experts believe that infants may have a reaction to the protein in breast milk.
Other common triggers include:
 Barley
 Green beans
 Lentils
 Milk
 Oats
 Peanuts
 Peas
 Poultry
 Rice
 Soy
 Squash
 Sweet potatoes
However, any food can trigger FPIES. Rice is the most common solid food trigger. In adults,
crustacean shellfish and fish hypersensitivity may trigger a similar syndrome in adults.
How is FPIES diagnosed?
FPIES often is misdiagnosed as sepsis, a metabolic disorder, anaphylaxis, necrotizing
enterocolitis, severe gastroesophageal reflux disease, or ileus. FPIES usually is not detectable via
standard allergy tests. However, a few reports do exist of children with FPIES with detectable
IgE. These children had a more prolonged course of FPIES and some progressed to a typical
IgE-mediated sensitivity.
Complicating matters further, often a delay of 2 to 3 hours occurs between eating a trigger food
and development of symptoms. Diagnosis usually is based on medical history and exclusion of
other possible diagnoses. Atopy patch tests often are used for FPIES patients, but it is not a
validated test for diagnosis. The most definitive test is a medically supervised oral food
challenge, but it often is not necessary for diagnosis.
Endoscopic evaluation shows diffuse colitis with variable ileal involvement. The colonic mucosa
may demonstrate mild friability to severe spontaneous hemorrhage and minute ulcers similar to
those seen in ulcerative colitis. Some patients have had a crypt abscess. Jejunal biopsies reveal
flattened villi, edema, and an increased number of lymphocytes, eosinophils, and mast cells.
Leukocystosis with a left shift is described in patients presenting with FPIES. This is likely
secondary to the secretion of various cytokines and chemokines during the inflammatory
reaction. Eosinophils found in intestinal biopsies suggest a possible continuum of eosinophilic
gastrointestinal diseases with similar underlying pathophysiology.
How is FPIES treated?
If the FPIES reaction is severe, fluid resuscitation, epinephrine to stabilize blood pressure, and
steroids to quell the immune reaction often are necessary.
References and recommended readings
Bajowala SS, Bird JA, Clark A, et al; The FPIES Foundation Board of Directors and Medical
Advisory Board. About food protein-induced enterocolitis syndrome. FPIES Foundation Web
site. http://fpiesfoundation.org/about-fpies-3/. Accessed December 10, 2013.
Caubet JC, Nowak-Wegrzyn A. Current understanding of the immune mechanisms of food
protein-induced enterocolitis syndrome. Expert Rev Clin Immunol. 2011;7(3):317-327.
doi:10.1586/eci.11.13.
Contributed by Elaine Koontz, RD, LD/N
Review Date 12/13
G-1966