Sudden unexpected death due to hereditary angioedema — A case report

2021 ◽  
Vol 6 (2) ◽  
pp. 60-65
Author(s):  
S Narayanan ◽  
S Ramalingam ◽  
R Narendar
Keyword(s):  
Case Report ◽  
Mortality Rate ◽  
Hereditary Angioedema ◽  
Laryngeal Edema ◽  
Inhibitor Protein ◽  
Unexpected Death ◽  
C1 Esterase Inhibitor ◽  
First Case ◽  
C1 Esterase Inhibitor Protein ◽  
Esterase Inhibitor

Angioedema related to deficiency of the C1- esterase inhibitor protein (C1-inh) is characterized by lack of response to therapies, which include antihistamines, steroids and epinephrine. In case of laryngeal edema, mortality rate is an estimated mammoth 30 percent. The first case of such acquired form of angioedema related to the deficiency in C1- esterase inhibitor was published in 1972. In the present case, we entail details of one such case.,

scholarly journals Acquired Form of Angioedema of the Head and Neck Related to a Deficiency in C1-Inhibitor: A Case Report with a Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Bassel Hallak ◽  
Propser Konu ◽  
Florian Lang ◽  
Christian Simon ◽  
Philippe Monnier
Keyword(s):  
Case Report ◽  
Mortality Rate ◽  
Enzyme Inhibitor ◽  
Laryngeal Edema ◽  
Simultaneous Occurrence ◽  
Inhibitor Protein ◽  
C1 Inhibitor ◽  
Review Of The Literature ◽  
First Case ◽  
Causative Factors

Angioedema related to a deficiency in the C1-inhibitor protein is characterized by its lack of response to therapies including antihistamine, steroids, and epinephrine. In the case of laryngeal edema, mortality rate is approximately 30 percent. The first case of the acquired form of angioedema related to a deficiency in C1-inhibitor was published in 1972. In our paper, we present a case of an acquired form of angioedema of the oropharyngeal region secondary to the simultaneous occurrence of two causative factors: neutralization of C1-inhibitor by an autoantibody and the use of an angiotensin convertin enzyme inhibitor.

scholarly journals Hereditary angioedema caused by C1-esterase inhibitor deficiency: a case report with literature-based analysis

2019 ◽  
Vol 8 (2) ◽  
pp. 741
Author(s):  
Sufia Athar ◽  
Noureddine Korichi ◽  
Yousra Shehada Siam
Keyword(s):  
Case Report ◽  
Hereditary Angioedema ◽  
Fresh Frozen Plasma ◽  
Pregnancy And Childbirth ◽  
C1 Esterase Inhibitor ◽  
Close Observation ◽  
Fresh Frozen ◽  
Dominant Disease ◽  
Frozen Plasma ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease caused by a mutation in the C1-inhibitor gene. It is a rare disease that is often worsened during pregnancy and childbirth. HAE, though uncommon but if untreated it may lead to maternal death.  The case report presents the successful management of a 24 years old, G2P1, with hereditary angioedema caused by C1-esterase inhibitor deficiency. This patient was managed with a multidisciplinary approach by an obstetrician, an immunologist, an anaesthesiologist and a pediatrician. She had an uneventful antenatal period, labor was induced. She had precipitate delivery and soon after delivery had a flare up of the disease. It was successfully managed with fresh frozen plasma and close observation. 

scholarly journals Quantification of human C1 esterase inhibitor protein using an automated turbidimetric immunoassay

2018 ◽  
Vol 33 (1) ◽  
pp. e22627 ◽  
Author(s):  
Clare E. Tange ◽  
Amrit Kaur ◽  
Nisha Verma ◽  
Alaco Hickey ◽  
Sofia Grigoriadou ◽  
...  
Keyword(s):  
Inhibitor Protein ◽  
C1 Esterase Inhibitor ◽  
C1 Esterase Inhibitor Protein ◽  
Turbidimetric Immunoassay ◽  
Esterase Inhibitor

Hereditary Angioedema Managed with Low-Dose Danazol and C1 Esterase Inhibitor Concentrate: A Case Report

2006 ◽  
Vol 28 (1) ◽  
pp. 27-31 ◽  
Author(s):  
Alon D. Altman ◽  
Janice McLaughlin ◽  
Robert Schellenberg ◽  
Charles Penner ◽  
Laura Arbour ◽  
...  
Keyword(s):  
Case Report ◽  
Hereditary Angioedema ◽  
Low Dose ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

scholarly journals Hereditary Angioedema: a Challenging Diagnosis for the Gastroenterologist

2016 ◽  
Vol 1 (3) ◽  
pp. 287-292
Author(s):  
Gabriella Gábos ◽  
Dumitru Moldovan ◽  
Daniela Dobru
Keyword(s):  
Quality Of Life ◽  
Differential Diagnosis ◽  
Hereditary Angioedema ◽  
Genetic Disorder ◽  
Laryngeal Edema ◽  
Cutaneous Manifestations ◽  
C1 Esterase Inhibitor ◽  
High Degree ◽  
Esterase Inhibitor

Abstract Hereditary angioedema (HAE) caused by a deficiency of C1 esterase inhibitor enzyme (C1-INH) is a very rare, autosomal dominantly inherited genetic disorder, characterized by recurrent peripheral angioedema, painful abdominal attacks and episodes of laryngeal edema. Abdominal attacks are frequent symptoms in adult HAE patients, occurring in more than 90% of the cases. Angioedema in the bowel or abdomen can occur in the absence of cutaneous manifestations and may be easily misdiagnosed unless the clinician has a high degree of awareness to include HAE in the differential diagnosis. Misdiagnosis is associated with inadequate treatments, including unnecessary surgical procedures. Any patient who presents recurrent episodes of swelling should be evaluated for HAE caused by C1-INH deficiency. New therapies could save lives and dramatically improve their quality of life.

scholarly journals Hereditary angioedema and pregnancy: successful management of recurrent and frequent attacks of angioedema with C1-inhibitor concentrate, danazol and tranexamic acid – a case report

2009 ◽  
Vol 2 (3) ◽  
pp. 123-125 ◽  
Author(s):  
D S Milingos ◽  
P Madhuvrata ◽  
J Dean ◽  
A Shetty ◽  
D M Campbell
Keyword(s):  
Case Report ◽  
Pregnant Woman ◽  
Tranexamic Acid ◽  
Hereditary Angioedema ◽  
C1 Inhibitor ◽  
Successful Management ◽  
C1 Esterase Inhibitor ◽  
Threatening Condition ◽  
Life Threatening ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is a rare but potentially life-threatening condition caused by deficiency of C1 esterase inhibitor. It is characterized by subcutaneous swelling in any part of the skin, gastrointestinal and respiratory tracts. We present the case of a pregnant woman with known HAE that deteriorated during pregnancy with frequent attacks that were managed successfully with danazol, tranexamic acid and regular intravenous administration of C1 esterase inhibitor.

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