Research article
Isao Ohsawa1†,
Seiji Nagamachi1†,
Hiyori Suzuki1†,
Daisuke Honda1†,
Nobuyuki Sato1†,
Hiroyuki Ohi2†,
Satoshi Horikoshi1† and
Yasuhiko Tomino1*†
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BMC Gastroenterology 2013, 13:123 doi:10.1186/1471-230X-13-123
Published: 2 August 2013
Abstract
Background
The diagnosis of hereditary angioedema (HAE) is often delayed due to the low awareness of this condition. In patients with undiagnosed HAE, abdominal symptoms often create the risk of unnecessary surgical operation and/or drug therapy. To explore the cause of misdiagnosis, we compared the laboratory findings of HAE patients under normal conditions with those during abdominal attacks.
Methods
Patient medical histories were analyzed and laboratory data at the first consultation with no symptoms and no medication were compared with those at visits to the emergency department during severe attacks.
Results
Fourteen HAE patients were enrolled. Initial HAE symptoms occurred at 20.2 ± 9.4 years of age. The correct diagnosis of HAE was made 22.7 ± 14.2 years after the initial symptoms. A common site of angioedema was the extremities. Half of the patients experienced a life-threatening laryngeal attack and/or severe abdominal pain. In the patients with severe abdominal pain, significant leukocytosis with neutrophilia along with increased levels of hematocrit were observed while levels of C-reactive protein (CRP) remained low. All severe attacks were alleviated with an infusion of C1-inhibitor concentrate.
Conclusions
Consideration of the likelihood of a HAE attack is important when patients present with acute abdominal pain and leukocytosis without elevation of CRP.
Keywords:
Hereditary angioedema; C1-inhibitor; C1-inhibitor concentrate; Acute abdomen; Leukocytosis; Hemoconcentration
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