PMID- 29093383
OWN - NLM
STAT- MEDLINE
DCOM- 20180716
LR  - 20181202
IS  - 1349-7235 (Electronic)
IS  - 0918-2918 (Print)
IS  - 0918-2918 (Linking)
VI  - 57
IP  - 3
DP  - 2018 Feb 1
TI  - Clinical Features of Hereditary and Mast Cell-mediated Angioedema Focusing on the 
      Differential Diagnosis in Japanese Patients.
PG  - 319-324
LID - 10.2169/internalmedicine.8624-16 [doi]
AB  - Objective The present study was designed to identify the clinical characteristics 
      that permit the differential diagnosis of hereditary angioedema (HAE) and mast 
      cell-mediated angioedema (Mast-AE) during the first consultation. Methods The 
      medical histories and laboratory data of 46 patients with HAE and 41 patients 
      with Mast-AE were compared. Results The average age of onset in the HAE group 
      (19.8+/-9.0 years) was significantly lower than that in the Mast-AE group 
      (35.2+/-12.0 years). The incidence of familial angioedema (AE) in the HAE group 
      (73.9%) was significantly higher than that in the Mast-AE group (9.7%). The 
      frequency of history of AE in the extremities, larynx, or gastrointestinal tract 
      was significantly higher in the HAE group. The frequency of AE episodes of the 
      lips and eyelids was significantly lower in the HAE group. The serum C4 
      concentration and CH50 titer were lower than the normal limit in 91.3% and 45.6% 
      of the patients in the HAE group, respectively; in Mast-AE group the serum C4 
      concentration and CH50 titer were significantly lower than the normal limit in 
      4.8% and 0% of the patients, the difference between the two groups was 
      statistically significant. A C1-inhibitor (C1-INH) activity level of <50% was 
      observed in all of the HAE patients, but none of the Mast-AE patients. The mean 
      serum IgE titer in the HAE group (120.8+/-130.5 IU/mL) was significantly lower than 
      that in the Mast-AE group (262.2+/-314.9 IU/mL). Conclusion The parameters within 
      the patients' medical histories, such as the age at the onset of AE, a family 
      history of AE, and the locations of past AE episodes are critical for the 
      successful diagnosis of the disease. Measurements of the C4 and C1-INH activity 
      are very useful for differential diagnosis of HAE from Mast-AE.
FAU - Ohsawa, Isao
AU  - Ohsawa I
AD  - Nephrology Unit, Saiyu Soka Hospital, Japan.
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Honda, Daisuke
AU  - Honda D
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Hisada, Atsuko
AU  - Hisada A
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
AD  - Department of Nephrology, Ikegami General Hospital, Japan.
FAU - Inoshita, Hiroyuki
AU  - Inoshita H
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Onda-Tsueshita, Kisara
AU  - Onda-Tsueshita K
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Mano, Satoshi
AU  - Mano S
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Sato, Nobuyuki
AU  - Sato N
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
AD  - Deprtment of Internal Medicine, Niigata Rosai Hospital, Japan.
FAU - Nakamura, Yuya
AU  - Nakamura Y
AD  - Nephrology Unit, Saiyu Soka Hospital, Japan.
FAU - Shimizu, Tatsuo
AU  - Shimizu T
AD  - Nephrology Unit, Saiyu Soka Hospital, Japan.
AD  - Department of Internal Medicine, Saiyu Kawaguchi Clinic, Japan.
FAU - Gotoh, Hiromichi
AU  - Gotoh H
AD  - Nephrology Unit, Saiyu Soka Hospital, Japan.
FAU - Goto, Yoshikazu
AU  - Goto Y
AD  - Nephrology Unit, Saiyu Soka Hospital, Japan.
FAU - Suzuki, Yusuke
AU  - Suzuki Y
AD  - Division of Nephrology, Department of Internal Medicine, Juntendo University 
      Faculty of Medicine, Japan.
FAU - Tomino, Yasuhiko
AU  - Tomino Y
AD  - Medical Corporation SHOWAKAI, Japan.
LA  - eng
PT  - Journal Article
DEP - 20171101
PL  - Japan
TA  - Intern Med
JT  - Internal medicine (Tokyo, Japan)
JID - 9204241
RN  - 0 (Biomarkers)
SB  - IM
MH  - Adult
MH  - Angioedema/blood/*diagnosis/etiology
MH  - Angioedemas, Hereditary/blood/diagnosis
MH  - Biomarkers/blood
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Japan
MH  - Male
MH  - Mast Cells/*metabolism
MH  - Middle Aged
PMC - PMC5827308
OTO - NOTNLM
OT  - C1-inhibitor
OT  - C4
OT  - IgE
OT  - hereditary angioedema
OT  - mast cell
EDAT- 2017/11/03 06:00
MHDA- 2018/07/17 06:00
PMCR- 2018/02/01
CRDT- 2017/11/03 06:00
PHST- 2017/11/03 06:00 [pubmed]
PHST- 2018/07/17 06:00 [medline]
PHST- 2017/11/03 06:00 [entrez]
PHST- 2018/02/01 00:00 [pmc-release]
AID - 10.2169/internalmedicine.8624-16 [doi]
PST - ppublish
SO  - Intern Med. 2018 Feb 1;57(3):319-324. doi: 10.2169/internalmedicine.8624-16. Epub 
      2017 Nov 1.