Knowledge of HAE lacking among pediatricians in Brazil: Study

Raising awareness about disease would improve diagnosis, treatment

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by Andrea Lobo |

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Pediatricians in Brazil show, in general, a low level of knowledge of hereditary angioedema (HAE), according to a recent study.

Even though those who were board-certified specialists in allergy and immunology were more knowledgeable than those who weren’t, their level of knowledge was still considered unsatisfactory.

“Based on the present study, actions are needed to raise awareness about HAE in order to reduce time for diagnosis and initiation of a proper treatment, improving patients’ quality of life, and reducing disease mortality,” the researchers wrote in “Are pediatricians familiar with hereditary angioedema?,” which was published in the World Allergy Organization Journal.

A feature of HAE are recurrent episodes of localized swelling attacks that affect the deeper skin layers. It’s commonly caused by genetic mutations that lead to overproducing bradykinin, a signaling molecule that regulates blood pressure by promoting blood vessel widening. When bradykinin is activated in the deeper skin layers, fluid flows from the bloodstream into nearby tissues, causing swelling.

No studies regarding the prevalence of HAE have been made in Brazil. The disease is still unknown to many healthcare professionals and is often underdiagnosed.

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Need for growing awareness about HAE in Brazil

To evaluate Brazilian pediatricians’ level of knowledge about HAE, researchers emailed an electronic questionnaire to 17,145 pediatricians who were members of the Brazilian Society of Pediatrics between June and July 2021. It included 12 questions on symptoms, diagnosis, and treatment in children and adolescents. It was developed by members of the Brazilian Group for the Study of Hereditary Angioedema, which has much experience with the disease.

Of the total number contacted, 455 (2.6%) responded to the questionnaire. From these, 55 (12.1%) were board-certified in allergy and immunology. The remaining 400 (87.9%) weren’t.

Most were women (80.9%) under the age of 50 (55.7%), had graduated from medical school more than 10 years previously (62.9%), and lived in the southeast region of Brazil (55.6%). Around a fifth held an MSc/PhD degree.

When comparing both groups, no significant differences were found regarding the physicians’ age, gender, geographical region, time since graduation from medical school, type of practice, and information received about HAE during medical school or later.

The self-assessment of HAE knowledge was higher among board-certified pediatricians than nonspecialists, with 69.1% of specialists evaluating their knowledge as great/good versus 5.5% of nonspecialists. Significantly more specialists cared for someone with HAE than nonspecialists (76.3% vs. 30.7%).

The median number of correct answers to questions about HAE was seven out of 12 (58.3%) for specialists, and three out of 12 (25%) for nonspecialists, a statistically significant difference.

Compared with nonspecialists, a significantly higher proportion of pediatricians who were specialists in allergy/immunology responded correctly to questions about clinical manifestations (70.9% vs. 24.2%) and diagnosis (94.5% vs. 71.7%). A similar trend was seen with questions about treatment (26.3% vs. 3.1%).

Researchers saw a significantly higher number of correct answers among specialists when they examined the median number of correct answers among specialists versus nonspecialists who graduated less than 10 years before living in the north, northeast, or southeast regions of Brazil. The same was seen when comparing specialists with nonspecialists who graduated 10 years earlier or more and were living in the southeast or southern regions of the country.

“The highest median number of correct answers among [specialist] pediatricians with 10 or more years since graduation from medical school living in the south and southeast regions of Brazil may be explained by greater concentration of reference centers and pioneering in the care of children with HAE, allied to academic excellence,” the researchers wrote. “More recently an increase in the presence of specialist and reference centers in the north and northeast regions, in addition to the south and southeast, has been observed.”

The findings in general indicate a low level of knowledge on HAE, even among pediatricians with a specialization in allergy and immunology, considering the total percentage of correct answers was close to 50%.

Increasing awareness may lead to improvements in diagnosing and treating HAE, the researchers wrote.