2018
DOI: 10.1111/all.13543
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Plasminogen gene mutation with normal C1 inhibitor hereditary angioedema: Three additional French families

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Cited by 43 publications
(48 citation statements)
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“…Together with the report from Belbezier et al. , the data indicate that the majority of HAE‐PLG patients (ranging between 78–87 %) experience angioedema attacks of the tongue (Table ), and it was suggested that this was characteristic of HAE‐PLG, distinguishing it from HAE‐C1‐INH (12 %) and HAE‐FXII (33 %) . In our study, the percentage of patients who experienced swelling of the tongue was 50 %, similar to a report by Yakushiji et al.…”
Section: Discussionsupporting
confidence: 90%
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“…Together with the report from Belbezier et al. , the data indicate that the majority of HAE‐PLG patients (ranging between 78–87 %) experience angioedema attacks of the tongue (Table ), and it was suggested that this was characteristic of HAE‐PLG, distinguishing it from HAE‐C1‐INH (12 %) and HAE‐FXII (33 %) . In our study, the percentage of patients who experienced swelling of the tongue was 50 %, similar to a report by Yakushiji et al.…”
Section: Discussionsupporting
confidence: 90%
“…We found the most common symptom for HAE‐PLG to be edema of the lips, experienced by 80 % of our patients, compared to 50 to 62 % in the literature (Table ). The rarest symptoms for HAE‐PLG in our patients include edema of the abdomen (10 %) and extremities (0 %).…”
Section: Discussionmentioning
confidence: 55%
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“…Secondly, there is a growing body of biochemical evidence for a role of plasmin as activator of the contact system [6,10,11] More convincingly, there good clinical experience with tranexamic acid as a maintenance therapy in both forms of HAE [7,12,13], and even in patients with (H)AE of unknown origin [14]. This may be explained by our biochemical findings that show that tranexamic acid blocks FXII activation by plasmin [6].The identification of a mutation in the plasminogen (PLG) gene in families with HAE from multiple countries gives rise to the idea that plasmin is an active contributor to bradykinin production [15][16][17]. However, the majority of patients respond well to tranexamic acid.…”
mentioning
confidence: 85%
“…Treatment experiences are still limited; however, case reports and studies suggest that C1INH concentrates, icatibant, and ecallantide might be beneficial for acute attacks while progestins, danazol, and tranexamic acid can be used for prophylaxis [25, 30, 32, 51, 75, 128-133]. …”
Section: Therapiesmentioning
confidence: 99%