PMID- 25249065
OWN - NLM
STAT- MEDLINE
DCOM- 20150723
LR  - 20220419
IS  - 1440-1592 (Electronic)
IS  - 1323-8930 (Linking)
VI  - 63
IP  - 4
DP  - 2014 Dec
TI  - Clinical and laboratory characteristics that differentiate hereditary angioedema 
      in 72 patients with angioedema.
PG  - 595-602
LID - 10.2332/allergolint.14-OA-0700 [doi]
AB  - BACKGROUND: Hereditary angioedema (HAE) is a rare but life-threatening condition 
      that results from mutations in C1-inhibitor (C1-INH). Since distinguishing HAE 
      from other causes of angioedema (AE) is a critical problem in emergencies, the 
      objective of the present study was to clarify the differences between HAE and 
      other forms of AE. METHODS: Seventy-two patients with AE were enrolled in this 
      study. The medical history and laboratory data of patients with HAE at the first 
      visit were compared to those with other types of AE. RESULTS: Subjects included 
      23 patients with HAE, 33 with mast cell-mediated AE, 5 with drug-induced AE and 
      11 with idiopathic AE. The average age of HAE onset (19.5 +/- 8.0 years old) was 
      significantly lower than in other groups. A family history of AE was noted in 
      82.6% of HAE patients, which was significantly higher than other groups. Swelling 
      affecting the extremities and gastrointestinal (GI) tract was observed in the 
      majority (60 to 80%) of HAE patients. Life threatening laryngeal edema was 
      observed in 30.4% of HAE patients. In 95.6% of HAE patients serum levels of C4 
      were less than the lower limit of the normal range. In our subjects, the 
      sensitivity and specificity of low C4 for HAE were 95.6% and 93.8%, respectively. 
      CONCLUSIONS: Early onset of AE, positive family history, recurrent AE in the 
      extremities and GI tract, and suffocation are distinctive characteristics of HAE. 
      A low serum level of C4 is a useful marker for making a differential diagnosis of 
      HAE.
FAU - Ohsawa, Isao
AU  - Ohsawa I
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Honda, Daisuke
AU  - Honda D
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Nagamachi, Seiji
AU  - Nagamachi S
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Hisada, Atsuko
AU  - Hisada A
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Shimamoto, Mamiko
AU  - Shimamoto M
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Inoshita, Hiroyuki
AU  - Inoshita H
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Mano, Satoshi
AU  - Mano S
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
FAU - Tomino, Yasuhiko
AU  - Tomino Y
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Tokyo, Japan.
LA  - eng
PT  - Journal Article
PL  - England
TA  - Allergol Int
JT  - Allergology international : official journal of the Japanese Society of 
      Allergology
JID - 9616296
RN  - 0 (Biomarkers)
RN  - 0 (Complement C1 Inhibitor Protein)
RN  - 0 (Complement C4)
RN  - 0 (Fibrin Fibrinogen Degradation Products)
RN  - 0 (fibrin fragment D)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age of Onset
MH  - Aged
MH  - Algorithms
MH  - Angioedema/blood/*diagnosis
MH  - Angioedemas, Hereditary/blood/*diagnosis
MH  - Biomarkers/blood/metabolism
MH  - Child
MH  - Complement C1 Inhibitor Protein
MH  - Complement C4
MH  - Diagnosis, Differential
MH  - Female
MH  - Fibrin Fibrinogen Degradation Products
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Mucous Membrane/immunology/pathology
MH  - Subcutaneous Tissue/immunology/pathology
MH  - Young Adult
EDAT- 2014/09/25 06:00
MHDA- 2015/07/24 06:00
CRDT- 2014/09/25 06:00
PHST- 2014/02/17 00:00 [received]
PHST- 2014/05/19 00:00 [accepted]
PHST- 2014/09/25 06:00 [entrez]
PHST- 2014/09/25 06:00 [pubmed]
PHST- 2015/07/24 06:00 [medline]
AID - S1323-8930(15)30072-1 [pii]
AID - 10.2332/allergolint.14-OA-0700 [doi]
PST - ppublish
SO  - Allergol Int. 2014 Dec;63(4):595-602. doi: 10.2332/allergolint.14-OA-0700.