Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

Objective: The aim of this study was to determine the clinical course and treatment outcomes of patients with hereditary angioedema (HAE) after infection with coronavirus disease 2019 (COVID-19). Materials and Methods: Thirty-nine patients with HAE were included in this study. These patients were regularly followed up over phone calls since the first COVID-19 case was seen in our country. Patients were asked to visit the hospital if there was a history of contact with a confirmed COVID-19 patient or if the patient developed clinical symptoms of COVID-19.Results: There were 21 (54%) patients with type I HAE, and 18 (46%) with type II HAE. All patients received treatment for angioedema attacks (C1-inhibitor [C1-INH], icatibant), and seven (20%) received long-term prophylaxis (danazol). Treatment for attacks was continued for all patients during the pandemic. Patients taking danazol were switched to long-term prophylaxis using the C1-INH concentrate. Eleven (28%) patients with HAE developed COVID-19 during this study. Only one patient had severe COVID-19. Six patients (54.5%) were diagnosed with type II HAE, and five (45.5%) were diagnosed with type I HAE. The most common COVID-19 symptoms were fever (7/11; 64%) and myalgia (6/11; 55%). Mild angioedema attacks were experienced by 36% (4/11) of the HAE patients diagnosed with COVID-19. Icatibant was used in all patients.Conclusion: Agents used for HAE block the kallikrein-kinin system and may be useful in the treatment of COVID-19. Considering their beneficial effects on COVID-19, it is recommended that HAE patients should continue the use of agents blocking the kallikrein-kinin system. Keywords: COVID-19, hereditary angioedema, kallikrein-kinin system, bradykinin, C1-INH

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Hereditary Angioedema due to C1 Inhibitor Deficiency: C1-INH Replacement Therapy

Reviews in Health Care ◽

10.7175/rhc.v5i2.913 ◽

2014 ◽

Vol 5 (2) ◽

pp. 55-66

Author(s):

Mauro Cancian

Keyword(s):

Replacement Therapy ◽

Hereditary Angioedema ◽

Rare Condition ◽

Type I ◽

Therapeutic Approaches ◽

C1 Inhibitor Deficiency ◽

Deep Tissue ◽

Gene Encoding ◽

The Face

Hereditary angioedema (HAE) is a rare condition affecting about 1 in 50.000 individuals and caused by a mutation in the gene encoding the C1-esterase inhibitor (C1-INH), which is involved in the control of complement, clotting, fibrinolytic and kinin pathways. HAE is characterized by plasma outflow from blood vessels, leading to fluid collecting (edema) in the deep tissue layers of the face, larynx, abdomen, and extremities. Three different types of HAE have been identified: in type I the mutation leads to the lack of production of C1-INH, in type II the mutation leads to the production of dysfunctional C1-INH, while type III is extremely rare and still not fully understood. Therapeutic approaches for HAE include on-demand treatments to stop angioedema attacks and prophylactic treatment to prevent attacks both by pre-procedural (short-term) and routine (long-term) prophylaxis. Aim of the present review is to present an overview of C1-INH replacement therapy with the plasma-derived concentrate of C1-INH Berinert® (CSL Behring GmbH) in the treatment of type I and II HAE.

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Successful use of lanadelumab in an elderly patient with type II hereditary angioedema

10.22541/au.163352939.93914485/v1 ◽

2021 ◽

Author(s):

Tasha S. Hellu ◽

Samuel L. Weiss ◽

Derek M. Smith

Keyword(s):

Hereditary Angioedema ◽

Genetic Disorder ◽

Poor Quality ◽

Type I ◽

Type Ii ◽

C1 Esterase Inhibitor ◽

Submucosal Edema ◽

Esterase Inhibitor

Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurring episodes of subcutaneous and/or submucosal edema without urticaria due to an excess of bradykinin (1, 2). HAE is classified into 2 main types (1). Type I HAE is caused by deficiency of C1 esterase inhibitor, accounting for 85% of cases (1). Type II HAE occurs in only 15% of cases and is marked by normal to elevated levels of C1 esterase inhibitor but with a reduction in activity (1). An angioedema attack can range in severity depending on the location and degree of edema (2). Furthermore, patients with HAE are often diagnosed with anxiety and depression secondary to their poor quality of life (3). Thus, long-term prophylaxis of attacks can be crucial to reduce the physical and psychological implications. For long-term prophylaxis, lanadelumab, a subcutaneously delivered monoclonal antibody inhibitor of plasma kallikrein, has been proven to decrease the frequency of HAE attacks without significant side effects (4). However, data is limited, specifically regarding patients with type II HAE and patients >/= 65 years (4).

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LONG-TERM EFFICACY AND SAFETY OF SUBCUTANEOUS C1-INHIBITOR IN ELDERLY PATIENTS WITH HEREDITARY ANGIOEDEMA TYPE I/II

Annals of Allergy Asthma & Immunology ◽

10.1016/j.anai.2018.09.100 ◽

2018 ◽

Vol 121 (5) ◽

pp. S32

Author(s):

J. Bernstein ◽

L. Schwartz ◽

H. Feuersenger ◽

I. Pragst ◽

J. Chiao ◽

...

Keyword(s):

Elderly Patients ◽

Hereditary Angioedema ◽

Type I ◽

Efficacy And Safety ◽

C1 Inhibitor ◽

Term Efficacy

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Comparative Study of effectiveness of mobilization with movement (MWM) and End range mobilization (ERM) techniques in frozen shoulder

Research in Pharmacy and Health Sciences ◽

10.32463/rphs.2018.v04i04.22 ◽

2018 ◽

Vol 4 (4) ◽

pp. 519-522

Author(s):

Jeyakumar S ◽

Jagatheesan Alagesan ◽

T.S. Muthukumar

Keyword(s):

Range Of Motion ◽

Frozen Shoulder ◽

Type I ◽

Mean Values ◽

Functional Activities ◽

Group A ◽

The Mean ◽

Group B

Background: Frozen shoulder is disorder of the connective tissue that limits the normal Range of motion of the shoulder in diabetes, frozen shoulder is thought to be caused by changes to the collagen in the shoulder joint as a result of long term Hypoglycemia. Mobilization is a therapeutic movement of the joint. The goal is to restore normal joint motion and rhythm. The use of mobilization with movement for peripheral joints was developed by mulligan. This technique combines a sustained application of manual technique “gliding” force to the joint with concurrent physiologic motion of joint, either actively or passively. This study aims to find out the effects of mobilization with movement and end range mobilization in frozen shoulder in Type I diabetics. Materials and Methods: 30 subjects both male and female, suffering with shoulder pain and clinically diagnosed with frozen shoulder was recruited for the study and divided into two groups with 15 patients each based on convenient sampling method. Group A patients received mobilization with movement and Group B patients received end range mobilization for three weeks. The outcome measurements were SPADI, Functional hand to back scale, abduction range of motion using goniometer and VAS. Results: The mean values of all parameters showed significant differences in group A as compared to group B in terms of decreased pain, increased abduction range and other outcome measures. Conclusion: Based on the results it has been concluded that treating the type 1 diabetic patient with frozen shoulder, mobilization with movement exercise shows better results than end range mobilization in reducing pain and increase functional activities and mobility in frozen shoulder.

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