JOURNAL ARTICLE

[Hereditary deficiency of C1-esterase inhibitor presenting with recurrent abdominal pain]

J O Steiss, P Mayser, L Gortner, G Alzen
Klinische Pädiatrie 2002, 214 (1): 20-1
11823949
Hereditary deficiency of C1-esterase inhibitor (C1-INH) which clinically manifests as hereditary angioedema is a rare disorder. In previously not diagnosed cases, a fatality rate of up to 30 % has been reported. The diagnosis of C1-esterase inhibitor deficiency should not be missed in patients presenting with angioedema in the face, stem or extremities. We report the case of a 17-year-old girl with recurrent abdominal pain and swelling of the hands.

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