International consensus on pediatric hereditary angioedema with C1-Inhibitor deficiency

The symptoms of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) often present in childhood but can go unrecognized for years.

Differential diagnosis can be difficult as abdominal pain is common in pediatric C1-INH-HAE but also commonly occurs in the general pediatric population.

Have in mind that before the age of 1 year, C1-INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year.

All neonates/infants with an affected C1-INH-HAE family member should be screened for C1-INH deficiency.

Pediatric patients should always carry a C1-INH-HAE information card, and medicine for emergency use. Plasma-derived C1-INH, recombinant C1-INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients.

It is recommended to follow-up patients in a specialized HAE comprehensive care center.

Treatments for Hereditary Angioedema (HAE) (click to enlarge the image).


International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1-Inhibitor deficiency - Farkas - 2016 - Allergy - Wiley Online Library

One-page list of most allergists in the US, by the American Board of Allergy and Immunology (ABAI)

One-page list of most allergists in the US, by the American Board of Allergy and Immunology. The following physicians are currently reported as Meeting Maintenance of Certification (MOC) Requirements:

Allergists who board-certified more than 10 years ago (or so) and don't have to participate in MOC may not be listed. They are in the "grandfathered-in" group. Please note that participation in the ABAI MOC program is voluntary for diplomates with time-unlimited certificates.

The 12 Best Asthma Blogs of 2016 - according to Healthline - we made the list at number 3

The 12 Best Asthma Blogs of 2016 - according to Healthline: "We’ve carefully selected these blogs because they are actively working to educate, inspire, and empower their readers with frequent updates and high quality information."

We made the list at number 3.

May 2nd is Now "Bronchial Thermoplasty Awareness Day" in Pennsylvania

Asthma is a disease that causes the airways of the lungs to swell and narrow, leading to wheeze, shortness of breath, chest tightness, and cough. Asthma is the most common chronic lung disease, affecting up to 10% of adults and 30% of children. Bronchial Thermoplasty (BT) is one of the treatment options for severe asthma - see below:

Asthma Treatment Options in 6 Steps (click to enlarge the image).

May 2nd is Now "Bronchial Thermoplasty Awareness Day" in Pennsylvania. From the manufacturer's YouTube channel: Bronchial Thermoplasty Day in Pennsylvania:

Here is how the procedure looks like, again from the manufacturer's channel:

Proton pump inhibitors are first-line treatment for EoE, achieving improvement of symptoms in 60% of patients

There are various therapeutic options available for treating patients with eosinophilic esophagitis (EoE):

- dietary modifications
- proton pump inhibitors (PPI)
- topical corticosteroids
- endoscopic esophageal dilation

Proton pump inhibitors are currently considered the first-line treatment for eosinophilic esophagitis, achieving histological remission and improvement of symptoms in 50.5% and 60.8% of patients, respectively.

Regarding topical corticosteroids in eosinophilic esophagitis, budesonide and fluticasone propionate both decrease eosinophil densities in the esophageal mucosa and relieve symptoms. However, owing to differences in drug delivery, viscous budesonide seems to be the best pharmacological therapy for eosinophilic esophagitis.

Results for dietary modifications have been mixed depending on the type of diet prescribed:

- amino acid-based elemental diets are the most effective in inducing histological remission of eosinophilic esophagitis (90.8% but they have drawbacks limiting implementation in clinical practice

- allergy testing-based food elimination provides a suboptimal remission rate of 45.5%, although this is lower in adults than in children (32.2% vs 47.9%, respectively)

- empirical 6-food elimination diets were shown to be the best diet-based therapy, with a remission rate of 72%

Eosinophilic Esophagitis (click here to enlarge the image).


Eosinophilic Esophagitis: An Evidence-Based Approach to Therapy. González-Cervera J, Lucendo AJ. J Investig Allergol Clin Immunol. 2016;26(1):8-18; quiz 2p following 18.

Image source: Eosinophilic esophagitis, Wikipedia, GNU Free Documentation License.
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