scholarly journals An anesthetic experience of hereditary angioedema type I patient undertook total laparoscopic hysterectomy - A case report -

2022 ◽  
Author(s):  
Yun-Sic Bang ◽  
Jaeho Cho ◽  
Chunghyun Park
Keyword(s):  
Hereditary Angioedema ◽  
Fatal Case ◽  
Upper Airway ◽  
Laparoscopic Hysterectomy ◽  
Fresh Frozen Plasma ◽  
Total Laparoscopic Hysterectomy ◽  
Type I ◽  
Autosomal Dominant Disorder ◽  
Airway Edema ◽  
Fresh Frozen

Background: Hereditary angioedema (HAE) is an autosomal dominant disorder. The characteristic of HAE is recurrent angioedema episodes due to low C1 esterase inhibitor (C1-INH) level. HAE symptoms, especially those affecting oropharynx or larynx may develop respiratory distress syndrome due to impaired airway, which can be potentially fatal. Case: We report a clinical case of a 57 year-old woman, with type I HAE, scheduled for total laparoscopic hysterectomy under general endotracheal anesthesia, which was done successfully without inducing airway edema. Danazol, which increases liver synthesis of C1-INH, was administered and fresh frozen plasma (FFP), which contained C1-INH, was transfused after induction.Conclusions: For HAE patients, the greatest concern is that general anesthesia can induces upper airway edema by direct mucosal irritation by the endotracheal tube. The perioperative management should include both prophylactic increase of C1-INH production and on-demand administration of C1-INH or FFP.

Short-term prophylaxis for children and adolescents with hereditary angioedema

2021 ◽  
Vol 42 (3) ◽  
pp. 205-213
Author(s):  
Oyindamola Ajewole ◽  
Mosopefoluwa Lanlokun ◽  
Stevenson Dimanche ◽  
Timothy Craig
Keyword(s):  
Tranexamic Acid ◽  
Hereditary Angioedema ◽  
Upper Airway ◽  
Fresh Frozen Plasma ◽  
Published Data ◽  
C1 Inhibitor ◽  
Short Term ◽  
Fresh Frozen ◽  
Short Term Prophylaxis ◽  
Frozen Plasma

Background: Hereditary Angioedema (HAE) is a rare, autosomal dominant, life threatening disease, secondary to the deficiency of C1-inhibitor, dysfunction of C1-inhibitor or inadequate control of the contact pathway. Presentation includes recurrent swelling of the skin, upper airway and the abdomen. Trauma can precipitate attacks, which in the airway can lead to asphyxia. For this reason, short term prophylaxis (STP) may be indicated before medical, surgical and dental procedures. The goal of the manuscript is to review short term prophylaxis for children of all ages. Methods: We searched the following search words: children, pediatric, adolescent, plasma derived C1-inhibitor, recombinant C1-inhibitor, surgery, medical procedures, prophylaxis, dental, Hereditary Angioedema, tranexamic acid, androgens, fresh frozen plasma, short term prophylaxis, lanadelumab, subcutaneous C1-inhibitor in Google Scholar and in PubMed to develop our results. Results: STP should be discussed at every visit. Plans should be individualized based upon the procedure, therapies available and shared decision making with patient/parent. For high risk procedures plasma derived C1-inhibitor should be used at 20 units/kg just prior to the procedure. Alternative agents for STP include recombinant C1-inhibitor, fresh frozen plasma, androgens, or tranexamic acid. In all cases, with or without the use of STP, 2 doses of on-demand therapy should be available in case of an attack. Conclusion: Herein, we review the published data on STP for pediatric patients with HAE and discuss first-line options, and off label use of medications, as well as review the guidelines pertaining to short term prophylaxis.

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 Fresh frozen plasma for on-demand hereditary angioedema treatment in South Africa and Iran

2019 ◽  
Vol 12 (9) ◽  
pp. 100049 ◽  
Author(s):  
Nicole Wentzel ◽  
Angelica Panieri ◽  
Maryam Ayazi ◽  
Sipho Duncan Ntshalintshali ◽  
Zahra Pourpak ◽  
...  
Keyword(s):  
South Africa ◽  
Hereditary Angioedema ◽  
Fresh Frozen Plasma ◽  
On Demand ◽  
Fresh Frozen ◽  
Frozen Plasma

Hereditary Angioedema: Management of Laryngeal Attacks

2011 ◽  
Vol 25 (6) ◽  
pp. 379-382 ◽  
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw
Keyword(s):  
Hereditary Angioedema ◽  
Treatment Options ◽  
The United States ◽  
Fresh Frozen Plasma ◽  
C1 Inhibitor ◽  
Acute Therapy ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma ◽  
Future Management

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.

Fresh frozen plasma for the treatment of hereditary angioedema

2007 ◽  
Vol 98 (4) ◽  
pp. 383-388 ◽  
Author(s):  
Michael Prematta ◽  
Joshua G. Gibbs ◽  
Ellen L. Pratt ◽  
Tracy R. Stoughton ◽  
Timothy J. Craig
Keyword(s):  
Hereditary Angioedema ◽  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Frozen Plasma

scholarly journals Spontaneous Sublingual Haematoma in a 90-year Old Patient: A Complication of Direct Oral Anticoagulants

2020 ◽  
Vol 6 (2) ◽  
pp. 111-114
Author(s):  
Johannes Daniël Cnossen ◽  
Jeannette Fenna Schoonderbeek ◽  
Maaike Muller
Keyword(s):  
Rare Complication ◽  
Oral Anticoagulants ◽  
Direct Oral Anticoagulants ◽  
Upper Airway ◽  
Chronic Atrial Fibrillation ◽  
Fresh Frozen Plasma ◽  
Nasal Intubation ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma

AbstractSublingual haematoma is a rare complication of anticoagulants and can be life-threatening. As the number of prescribed anticoagulants is increasing, the incidence of complications of these drugs will continue to increase. A report of a sublingual haematoma in an elderly patient with chronic atrial fibrillation treated with edoxban (Lixiana ©, Daiichi Sankyo Europe GmbH, München, Germany) is reported. A 90-year male presented at the emergency department with an obstructed upper airway due to a sublingual haematoma. The patient received tranexamic acid, prothrombin complex, and fresh frozen plasma. After fiberoptic nasal intubation, the patient was monitored in the intensive care unit. After four days, the patient was extubated, and after six days, the swelling resolved completely. Complications of anticoagulants are rare but can be life-threatening. Recognition of an endangered airway and reversing the effects of the anticoagulant are essential. Surgical evacuation of the haematoma could be considered but is not necessary.

scholarly journals Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

2021 ◽  
Vol 16 (1) ◽  
pp. 75-80
Author(s):  
Jeong Wook Park ◽  
Jinyoung Seo ◽  
Sang Hun Kim ◽  
Ki Tae Jung
Keyword(s):  
Kidney Transplantation ◽  
Disseminated Intravascular Coagulation ◽  
Hereditary Angioedema ◽  
Treatment Options ◽  
Deceased Donor ◽  
Fresh Frozen Plasma ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Intravascular Coagulation ◽  
Fresh Frozen

Background: Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC).Case: A 42-year-old man with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient’s condition worsened despite intensive care and he died due to shock.Conclusions: The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

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