Hereditary C1 inhibitor deficiency
ICD-10 D84.1 · ICD-11 4A00.14

Treatment of Hereditary C1 Inhibitor Deficiency in Children Under 12 Years

Acute attacks of hereditary C1 inhibitor deficiency in children under 12 years require an age-specific treatment approach. When preferred first-line options are not accessible, a structured second-line pathway applies.

This protocol is specific to patients under 12 years of age. In this age group, C1 inhibitor or icatibant are the recommended treatments for acute attacks. When these are not available, an alternative approach is indicated.

Treatment Overview

A plasma-based therapy is used as a second-line option when first-choice treatments cannot be administered.

Complete product selection criteria, clinical decision points, and the full treatment algorithm are available in the structured protocol.

References

  • We recommend C1 inhibitor or icatibant be used for the treatment of attacks in children under the age of 12
  • When C1-INH and icatibant are not available, SDP is preferred over FFP, but both are considered second-line treatment.

DOI: 10.1111/all.15214

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