Emotional Processes and Stress in Children Affected by Hereditary Angioedema With C1-inhibitor Deficiency: A Multicenter, Prospective Study

Orphanet J Rare Dis. 2018 Jul 13;13(1):115. doi: 10.1186/s13023-018-0871-x.


Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent edema of unpredictable frequency and severity. Stress, anxiety, and low mood are among the triggering factors most frequently reported. Impaired regulation and processing of emotions, also known as alexithymia, may influence outcomes. The aim of this study was to confirm the presence of alexithymia and stress in children with C1-INH-HAE, to determine whether they are also present in children affected by other chronic diseases, and to investigate their relationship with C1-INH-HAE severity. Data from children with C1-INH-HAE (n = 28) from four reference centers in Italy were compared with data from children with type 1 diabetes (T1D; n = 23) and rheumatoid arthritis (RA; n = 25). Alexithymia was assessed using the Alexithymia Questionnaire for Children scale; perceived stress was assessed using the Coddington Life Event Scale for Children (CLES-C).

Results: Mean age (standard deviation [SD]) in the C1-INH-HAE, T1D, and RA groups was 11.8 (3.3), 11.7 (2.9), and 11.1 (2.6) years, respectively. Mean C1-INH-HAE severity score was 5.9 (2.1), indicating moderate disease. Alexithymia scores were similar among disease groups and suggestive of difficulties in identifying and describing emotions; CLES-C scores tended to be worse in C1-INH-HAE children. C1-INH-HAE severity was found to correlate significantly and positively with alexithymia (p = 0.046), but not with perceived stress. Alexithymia correlated positively with perceived stress.

Conclusions: Alexithymia is common in children with chronic diseases. In C1-INH-HAE, it may result in increased perceived stress and act as a trigger of edema attacks. Comprehensive management of C1-INH-HAE children should consider psychological factors.

Keywords: Alexithymia; C1-inhibitor deficiency; Children; Hereditary angioedema; Psychological factor; Stress.

MeSH terms

  • Adolescent
  • Angioedemas, Hereditary / metabolism*
  • Angioedemas, Hereditary / psychology*
  • Anxiety / metabolism
  • Child
  • Complement C1 Inhibitor Protein / metabolism*
  • Disease Progression
  • Emotions / physiology
  • Female
  • Humans
  • Male
  • Prospective Studies
  • Surveys and Questionnaires


  • Complement C1 Inhibitor Protein