Research About EGID
What is an Eosinophil?
The eosinophil is a specialized cell of the immune system. This pro-inflammatory white blood cell generally has a nucleus with two lobes (bilobed) and cytoplasm filled with approximately 200 large granules containing enzymes and proteins with different (known and unknown) functions.
Eosinophils are formed exclusively in the bone marrow, where they spend about 8 days in the process of maturation before moving into the blood vessels. They travel through the vessels for 8 to 12 hours before they finally arrive at destination tissues, where they remain for 1 to 2 weeks. Interleukin 5 (IL-5) appears to be the major growth factor for this type of cell.
The functions of the eosinophil are varied, some of which are very similar to other white blood cells. They are implicated in numerous inflammatory processes, especially allergic disorders. In addition, eosinophils may have a physiological role in organ formation (e. g. postgestational mammary gland development).
Eosinophilic functions include moving to inflamed areas, trapping substances, killing cells, antiparasitic and bactericidal activity, participating in immediate allergic reactions, and modulating inflammatory responses.
Eosinophils can be either helpful or harmful. At one extreme, such as in the illness erythema toxicum, eosinophils play the role of a beneficial modulatory element or an innocent bystander. At the other extreme, represented by conditions like Loeffler’s disease and idiopathic hypereosinophilic syndrome, eosinophils are linked with permanent pathologic changes.
What is an Eosinophilic Disorder?
Eosinophils are a normal cellular component of the blood and also of certain tissues, including spleen, lymph nodes, thymus, and the submucosal areas of the gastrointestinal, respiratory, and genitourinary tracts. Counts of 0 to 450 eosinophils per cubic millimeter of blood are considered within normal limits. Eosinophilic disorders occur when eosinophils are found in above-normal amounts in various parts of the body.
When the body wants to attack a substance, such as an allergy-triggering food or airborne allergen, eosinophils respond by moving into the area and releasing a variety of toxins. However, when the body produces too many eosinophils, they can cause chronic inflammation resulting in tissue damage. Eosinophilic disorders are diagnosed according to the location where the levels of eosinophils are elevated:
Eosinophilic esophagitis (esophagus)
Eosinophilic gastritis (stomach)
Eosinophilic enteritis (small intestine)
Eosinophilic colitis (large intestine)
Hypereosinophilic syndrome (blood and any organ)
There are many disorders where eosinophils have been found elevated in the blood or different tissues. General categories of disease, each with examples of those that have increased levels of eosinophils, range from allergic disorders to endocrine disorders.
Range of Disorders
Allergic disorders are classically characterized by the presence of eosinophils. Allergic rhinoconjunctivitis (hay fever) has increased levels of eosinophils in the nasal mucosa. Asthma, after an exacerbation, shows increased numbers of eosinophils in the lung.
Any drug/medicine has the potential to cause a reaction. Some of these reactions are allergic in nature, and eosinophils might be elevated in blood or in tissues where the drug is concentrated.
Parasitic infections (helminthiasis − worms), fungal infections, and some other types of infections are associated with increased numbers of eosinophils.
Hematologic disorders with increased levels of eosinophils include hypereosinophilic syndrome, leukemias, lymphomas, tumors, mastocytosis, and atheroembolic disease.
Immunologic Disorders and Reactions
Hyper-IgE syndrome, Omenn’s syndrome, thymomas, and transplant rejections are only a few types of conditions with increased numbers of eosinophils.
Hypoadrenalism has been associated with increases in the levels of eosinophils in the blood.
Specific Organ Involvement
There are certain conditions where eosinophils have been found to be increased or pathologically present.
Skin and Subcutaneous Disorders
Atopic dermatitis (eczema), bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis, drug-induced lesions, urticaria, eosinophilic panniculitis, angioedema with eosinophilia, Kimura’s disease, Shulman’s syndrome, Well’s syndrome, eosinophilic ulcer of the oral mucosa, eosinophilic pustular folliculitis, and recurrent cutaneous necrotizing eosinophilic vasculitis.
Drug / toxin-induced eosinophilic lung disease, Loeffler’s syndrome, allergic bronchopulmonary aspergillosis, eosinophilic pneumonia, Churg-Strauss syndrome, eosinophilic granuloma, and pleural eosinophilia.
Gastroesophageal reflux, parasitic infections, fungal infections, Helicobacter pylori infections, inflammatory bowel disease (ulcerative colitis and Crohn’s disease), food allergic disorders, protein-induced enteropathy and protein-induced enterocolitis, allergic colitis, celiac disease, pemphigus vegetans (MR) and primary eosinophilic esophagitis, gastroenteritis, and colitis. Rare tumors (leiomyomatosis), connective tissue disorders, and vasculitic disorders.
Organizing chronic subdural hematoma membranes, central nervous system infections, ventriculoperitoneal shunts, and drug-induced adverse reactions.
Secondary to systemic disorders such as the hypereosinophilic syndrome or the Churg-Strauss syndrome, heart damage has been reported. Certain congenital heart conditions (septal defects, aortic stenosis) are associated with increased levels of eosinophils in the blood.
Eosinophiluria (eosinophils in the urine) is associated with infections or interstitial nephritis and eosinophilic cystitis.
Psychological Effects of Eosinophilic Disorders
Many parents have questions about the psychological effects that an eosinophilic disorder can have on their child. Here are answers, provided by the experts at the Cincinnati Center for Eosinophilic Disorders at Cincinnati Children’s, to some frequently asked questions. Expand the following categories for specific examples:
My child is on a restricted / elemental diet. Should our family stop eating in front of our child?
Some families choose not to eat in front of their child because they feel doing so will increase discomfort or frustration. However, doing so may actually increase anxiety, contributing to the child feeling even more different from peers. It is often best to maintain normal mealtime routines with your family. Most children are highly adaptable to new situations. Although eating in front of your child may temporarily increase frustration level, this should subside as he or she adjusts to the new situation. By allowing your child the opportunity to gain practice with refraining from food while others are eating, your child will learn to increase confidence and comfort level as he or she faces social situations where others inevitably eat (e.g., school lunch; parties).
My child is having temper tantrums at mealtime because of a restricted diet. What should I do?
Children need time to adjust to a restricted or elemental diet. When maintaining normal mealtime routines, children, particularly young children, often act out in an attempt to express frustration or persuade parents to allow them to consume the foods they desire. Many parents find that problem behaviors will decrease when tantrums or other negative behaviors are ignored and positive mealtime behaviors such as sitting in a chair, helping to prepare or serve the meal, and staying calm are praised and rewarded. To increase a child’s motivation to cooperate, a behavioral reward chart can be made and children can be rewarded with stickers or special time with their parents after meeting mealtime goals.
Because my child is sick, I am not sure how to discipline him or her. When my child becomes upset he or she seems to have an increase in physical symptoms. What do you suggest?
Many parents feel uncomfortable disciplining their child who is medically compromised. However, it is best to continue disciplinary strategies that are effective in decreasing problem behaviors. Children often thrive on structure, predictability, and routine, particularly when they are struggling with the uncertainty inherent in coping with a chronic illness. Children often demonstrate positive adjustment when their environment and family have stayed consistent with life prior to diagnosis. Without discipline, problem behaviors will only increase. An effective way to discipline is to ignore problem behaviors as much as possible and use positive reinforcement, praise, and incentives when the child engages in desired behaviors.
My child is being teased by peers for being “different.” What can I do to help?
Be supportive of your child’s feelings. Continue to offer your child praise for his or her special and unique qualities. Explain that many children have misconceptions regarding medical problems. Encourage your child to be involved with children from a number of different environments. You may wish to work with school staff to educate children about your child’s diagnosis. An outgoing, friendly peer could be paired with your child to increase social networks and comfort level around peers.
How can I tell if my child is depressed?
Some children have difficulty coping and adjusting to life with a chronic medical condition. Signs of depression in children and teens include loss of interest in previously enjoyable activities, feelings of sadness or hopelessness, negative self-comments, withdrawing from friends or family, changes in sleeping patterns or appetite, thoughts of dying or wishing they were never born, school refusal, and lack of energy.
Talk to your child in a supportive, non-threatening way if you suspect that your child has signs of depression. It is helpful to discuss your concerns with your child’s pediatrician, psychologist, or other mental health professional who can assist with evaluation and treatment.
Besides medication, is there anything to help decrease my child’s pain?
There are many behavioral strategies that can be helpful in decreasing the pain level. Helping your child develop relaxation skills is important in managing pain. Children can use visualization to imagine a peaceful place, such as a family vacation or trip to the beach. Additional strategies include progressive muscle relaxation, deep breathing, and biofeedback. The Division of Psychology at Cincinnati Children’s provides biofeedback services and assists in the development of effective relaxation strategies.
My child seems to be having difficulty coping with having a chronic illness. What can I do to help?
Coping with medical problems varies from child to child. There is often an “adjustment period” when children feel different from others and frustrated with changes in lifestyle. Discuss these issues with your child and normalize thoughts and feelings. Encourage your child to continue with hobbies and interests and use coping strategies when feeling down. If you feel your child is having problems with adjustment it may be helpful to contact your child’s pediatrician, a psychologist, or other mental health professionals to assist with the development of coping strategies.
My child is refusing to attend school
Chronic school refusal is typically a sign of a more significant psychological problem such as anxiety, depression, or difficulty with coping and adjustment. Many children with chronic medical conditions feel different from their peers and are unable to enjoy many of the activities offered at school (e.g., recess, lunch, after-school activities). In addition, children who have missed school due to medical appointments or hospitalizations fall behind academically compared to their peers. This may decrease a child’s self-esteem and ability to cope with having a chronic illness.
Common Questions From Families
Eosinophil gastrointestinal disorder (EGID) can present acutely in association with a viral appearing illness. While the start of symptoms may be sudden, it is likely that the intestinal inflammation was present well before the onset of symptoms. The new insult (i.e., the viral illness) may promote the severity of the symptoms because of the underlying problem. However, it is important to note that EGID is a chronic disorder and usually presents gradually rather than acutely; it also does not go away quickly. It does respond rather rapidly to effective treatment. In your child, the effective treatment appears to be an alteration in the diet. A true remission is only determined by repeat endoscopy and biopsy. Because of the unusual acute presentation in your child, an experienced physician should carefully scrutinize the biopsy slides; one must be sure that the problem is truly EGID and not another process.
The long-term consequences of EGID are currently being studied. However, most patients with EGID do not have a life-threatening disease and can live a long life.
The eosinophils are sometimes elevated in the blood (less than half of the patients) and their levels generally correlate with the severity of tissue disease. The eosinophil levels in the blood often return to normal.
A variety of foods can be associated with EGID. Unlike classic anaphylaxis, there appears to be a broader range of foods identified as culprits. This area is under active research investigation.
Eosinophilic esophagitis does have seasonal variation in some patients, typically with worse symptoms in the spring and summer.
Questions to ask your Doctor
Eosinophilic esophagitis is divided into primary or secondary. Primary eosinophilic esophagitis is further subclassified into idiopathic (unknown cause), atopic (allergic), or familial. Secondary eosinophilic esophagitis could be due to a more generalized eosinophilic disorder, such as eosinophilic gastroenteritis or hypereosinophilic syndrome, or due to noneosinophilic disorders such as drug reactions, infections, gastroesophageal reflux, connective tissue disorders, vasculitis, and leiomyomatosis.
It is important to know if the increased eosinophils were found only in the esophagus, in the stomach, in the duodenum, or in all of them. If the esophagus is affected, it is also essential to know if it was the lower part only, the upper part only or both. This information will help in the decision making (Is it eosinophilic esophagitis? Is it gastroesophageal reflux? Is it eosinophilic gastroenteritis?).
More than 15 eosinophils per high power field (in the microscope) in esophageal biopsies are suggestive of primary eosinophilic esophagitis, while less than that are associated with food allergies or gastroesophageal reflux.
A good percentage of patients with eosinophilic disorders of the gastrointestinal tract have food allergies. This area needs to be thoroughly evaluated by a doctor who is specialized in allergy and immunology.
Many disorders are associated with increased numbers in eosinophils in the gut. A complete history and physical exam plus associated tests should narrow the diagnosis.