Are there any parents with children who have idiopathic anaphalyxis?

I have a 9 yr old with idiopathic anaphalyxis. This means she has anaphalactic shock with no known cause. I was hoping someone out there would be going through this and would have some suggestions or ways they deal with this.

2 Answers

  • 1 decade ago
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    Idiopathic Anaphylaxis (IA) is a well-described syndrome of anaphylaxis without any recognised external trigger. These patients present with the same symptoms as patients with other types of anaphylactic reaction. The attacks occur with variable frequency. Fatalities have been reported in patients who have been diagnosed with idiopathic anaphylaxis

    Symptoms & signs do not differ from other forms of anaphylaxis and include:

    • Hypotension & increased pulse rate

    • Wheezing & stridor

    • Hives, angioedema, flushing, & itching

    • Nausea, vomiting, diarrhea, difficulty swallowing

    • Light-headedness and loss of consciousness

    In the series of Ditto et al [3] all of them experienced hives and angioedema, whereas 60% experienced symptoms of upper airway obstruction. Individual patients usually tend to have the same manifestations on repeated episodes. Progression from hives and itching to life-threatening symptoms of wheeze, loss of consciousness, and laryngeal edema may occur in 10 min to hours after onset.

    Classification of Idiopathic Anaphylaxis (IA)

    (Adopted from Roy Patterson, M.D. textbook on Idiopathic Anaphylaxis)

    Disease Symptom

    Generalized (G) Urticaria or angioedema with bronchospasm (asthma)

    Hypotension (reduce blood pressure), syncope

    Angioedema (A) Angioedema with upper airway compromise

    (Laryngeal, pharyngeal, tongue)

    Frequency of episodes More than 6 episodes per year: Frequent (F)

    Less than 6 episodes per year: Infrequent

    Treatment depends on the severity and frequency of the attacks. Steroids seem to be universally effective in IA. If symptoms are not controlled on 60 mg daily by the end of 6 weeks, the diagnosis of IA should be questioned.

    Acute Treatment


    Prednisone 60 mg

    Hydroxyzine 25 mg

    Go to the nearest emergency room

    Programme for IA-F

    Arrange acute treatment

    Prednisone 40 — 60 mg daily for at least 1 week or until symptoms controlled, then decrease to alternate day and the wean by 5 — 10 mg each month.

    If it is proving difficult to wean off steroids ketotifen should be added [4]

    Cetirizine 10 mg daily or Hydroxyzine 25 mg tds

    Follow-up based on response to treatment

    Programme for IA-I

    Arrange acute treatment

    No chronic medicTreatment recommendations for idiopathic anaphylaxis patients depend on the frequency and severity of their exacerbations. Whereas care should be individualized, some simple guidelines should be universally applied (Figure 1). All idiopathic anaphylaxis patients should be educated about idiopathic anaphylaxis and taught how to manage an acute attack. Immediately after the first signs of anaphylaxis, adult patients should inject 0.3 ml of 1:1000 w:v aqueous epinephrine intramuscularly followed by oral doses of both an antihistamine (e.g. diphenhydramine 50 mg or hydroxyzine 25 mg) and 60 mg prednisone. This emergency kit should always be within reach and should be checked occasionally to make sure it has not expired. After use, patients should be transported immediately to the nearest emergency departmentation

    Patients classified with frequent symptoms require maintenance therapy, which should include 40-60 mg of daily prednisone and an antihistamine, such as cetirizine 10 mg, hydroxyzine 25-50 mg, or benadryl 25-50 mg. Prednisone should be given daily for at least 1 week, but if symptoms are not controlled after 6 weeks then the diagnosis may be called into question. When the condition is controlled, prednisone may be decreased to every-other-day dosing and subsequently weaned by 5-10 mg each month. After the prednisone is discontinued, the antihistamines may then be tapered. Although steroids seem to be universally effective in idiopathic anaphylaxis, the natural history of the condition may also lead to remission.[43]

    Those patients who fail steroid tapering (classified as corticosteroid-dependent idiopathic anaphylaxis or malignant idiopathic anaphylaxis) may be tried on alternative medications. Wong et al.[44]studied nine steroid-dependent idiopathic anaphylaxis patients and reported that the addition of ketotifen helped seven of them significantly reduce or discontinue their steroids. Alternative medications that can be used include oral cromolyn, oral albuterol, or montelukast. To avoid burdensome and costly drug regimens, these second-line agents should be discontinued if they do not obviously decrease the patient's prednisone dose.

    Pediatric patients with idiopathic anaphylaxis should be classified and treated in a similar fashion to adults, while taking into account dose adjustments for steroids, epinephrine, and antihistamines.[45] Of the second-line medications, montelukast has been approved for children over the age of 2 years, whereas both cromolyn and albuterol have also been approved for infants.

    Although the literature has placed limited emphasis on the perioperative management of idiopathic anaphylaxis, there are cases of surgically induced exacerbations.[4, 44] A prophylactic regimen of perioperative steroids is aimed at preventing intra and postoperative anaphylaxis. Patients who experience frequent exacerbations before surgery should not undergo elective procedures until their disease is under optimal control. Patients who have had an episode of idiopathic anaphylaxis within the past year but are well controlled should be treated prophylactically with 40-60 mg prednisone per day on the 5 days preceding surgery and then 100 mg intravenous hydrocortisone every 8 h during surgery and through the recovery period.[46]

    All patients diagnosed with idiopathic anaphylaxis should receive a Medic Alert bracelet or other identification that clarifies their diagnosis. They should also be educated about what symptoms to look for and how to administer their emergency epinephrine, prednisone, and antihistamine properly. Physicians should reassure their patients that compliance with the prescribed regimen offers an excellent remission rate, in excess of 80%.[4] Patients also benefit from knowing that it is unlikely that an unforeseen external allergen is responsible for their symptoms.

    Follow to assess response & compliance

  • Anonymous
    4 years ago


    Source(s): Cure Urticaria
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