PMID- 25872948
OWN - NLM
STAT- MEDLINE
DCOM- 20150824
LR  - 20151119
IS  - 1534-4436 (Electronic)
IS  - 1081-1206 (Linking)
VI  - 114
IP  - 6
DP  - 2015 Jun
TI  - Clinical manifestations, diagnosis, and treatment of hereditary angioedema: 
      survey data from 94 physicians in Japan.
PG  - 492-8
LID - S1081-1206(15)00161-1 [pii]
LID - 10.1016/j.anai.2015.03.010 [doi]
AB  - BACKGROUND: Hereditary angioedema (HAE) is a rare and potentially 
      life-threatening condition that results from mutations in the C1 inhibitor 
      (C1-INH). Awareness of HAE among physicians in Japan is increasing, but 
      real-world data are lacking. OBJECTIVE: To explore the clinical manifestations, 
      diagnosis, quality of life (QOL), and treatment of Japanese patients with HAE. 
      METHODS: A 14-point survey was developed and sent to 387 physicians in Japan 
      (March to May 2014) to gather clinical data on their HAE patients' family 
      history, severity and frequency of attacks, QOL, and therapy use. RESULTS: Data 
      on 171 HAE patients were collected from 94 physicians (24.3% response rate). Of 
      the patients, 76.6% had a family history of angioedema (AE), and 11.7% had 
      experienced a death in the family due to an AE attack. HAE type I occurred in 99 
      patients (57.9%), HAE type II occurred in 9 patients (5.3%), HAE with normal 
      C1-INH occurred in 3 patients (1.8%), and an additional 60 patients were 
      unclassified. Mean time from initial symptoms to diagnosis was 13.8 years. 
      Attacks that required airway management and abdominal surgery with uncertain 
      diagnosis were observed in 9.5% and 2.9% of patients, respectively. In the past 
      year, 21.0% of patients presented with more than 10 attacks, 21.1% were admitted 
      to the hospital for more than 1 day, and 28.7% were absent from work or school. 
      On-demand C1-INH concentrate and prophylactic tranexamic acid were used in 
      approximately half of the patients (47.4% and 39.2%, respectively). CONCLUSION: 
      HAE is a severe condition characterized by recurrent AE attacks. In Japan, 
      delayed patient diagnosis and limited use of HAE-specific therapies exacerbate 
      the burden on HAE patients.
CI  - Copyright (c) 2015 American College of Allergy, Asthma & Immunology. Published by 
      Elsevier Inc. All rights reserved.
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. Electronic address: yasu@juntendo.ac.jp.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20150411
PL  - United States
TA  - Ann Allergy Asthma Immunol
JT  - Annals of allergy, asthma & immunology : official publication of the American 
      College of Allergy, Asthma, & Immunology
JID - 9503580
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Androgens)
RN  - 0 (Antifibrinolytic Agents)
RN  - 0 (Complement C1 Inhibitor Protein)
RN  - 0 (Histamine Antagonists)
RN  - 6T84R30KC1 (Tranexamic Acid)
RN  - N29QWW3BUO (Danazol)
SB  - IM
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Adult
MH  - Androgens/therapeutic use
MH  - Angioedemas, Hereditary/*diagnosis/*drug therapy
MH  - Antifibrinolytic Agents/therapeutic use
MH  - Complement C1 Inhibitor Protein/*genetics
MH  - Danazol/therapeutic use
MH  - *Delayed Diagnosis
MH  - Female
MH  - Genetic Testing
MH  - Health Knowledge, Attitudes, Practice
MH  - Histamine Antagonists/therapeutic use
MH  - Humans
MH  - Japan
MH  - Male
MH  - *Practice Patterns, Physicians'
MH  - Quality of Life
MH  - Surveys and Questionnaires
MH  - Tranexamic Acid/therapeutic use
EDAT- 2015/04/16 06:00
MHDA- 2015/08/25 06:00
CRDT- 2015/04/16 06:00
PHST- 2014/12/01 00:00 [received]
PHST- 2015/03/04 00:00 [revised]
PHST- 2015/03/15 00:00 [accepted]
PHST- 2015/04/16 06:00 [entrez]
PHST- 2015/04/16 06:00 [pubmed]
PHST- 2015/08/25 06:00 [medline]
AID - S1081-1206(15)00161-1 [pii]
AID - 10.1016/j.anai.2015.03.010 [doi]
PST - ppublish
SO  - Ann Allergy Asthma Immunol. 2015 Jun;114(6):492-8. doi: 
      10.1016/j.anai.2015.03.010. Epub 2015 Apr 11.