Anesthesia for Urgent Surgery in a Patient with Angioneurotic Oedema: A Case Report

Hereditary angioneurotic oedema is an autosomal dominant disease associated with serum deficiency of functional C1-inhibitor. It is characterized by periodic swelling of subcutaneous tissues, abdominal viscera and upper airways. Lethal acute episodes of oedema can occur during anaesthesia and surgery. It is essential to prepare such patients before surgery. This article describes a case and the various preventive measures used to avoid acute episodes during anaesthesia for urgent surgery for mesenteric ischemia. In emergency situations where C1 inhibitor concentrate is not available, fresh frozen plasma (FFP) can be used as an alternative, as it also contains C1 inhibitor, corticosteroids, antihistamines, and epinephrine can be useful adjuncts but typically are not efficacious in aborting acute attacks. Prophylactic management involves long-term use of attenuated androgens or antifibrinolytic agents (Tranexamic acid).Their various indications are discussed.

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

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20190314 ◽

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.

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Hereditary Angioedema: Management of Laryngeal Attacks

American Journal of Rhinology and Allergy ◽

10.2500/ajra.2011.25.3670 ◽

2011 ◽

Vol 25 (6) ◽

pp. 379-382 ◽

Cited By ~ 7

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.

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Clinical Application of Prothrombin Complex Concentrate in Blood Management in Patients

Critical Care Nurse ◽

10.4037/ccn2017333 ◽

2017 ◽

Vol 37 (2) ◽

pp. 49-56

Author(s):

Sherri Ozawa ◽

Tiffany Nelson

Keyword(s):

Vitamin K ◽

Prothrombin Complex Concentrate ◽

International Normalized Ratio ◽

Fresh Frozen Plasma ◽

Drug Discontinuation ◽

Clotting Factors ◽

Urgent Surgery ◽

Anticoagulant Reversal ◽

Fresh Frozen ◽

Frozen Plasma

Management of patients receiving anticoagulants is a major factor in achieving better outcomes. Anticoagulant therapy may need to be discontinued or rapidly reversed before urgent surgery or invasive procedures. In these situations, treatment with concentrated vitamin K, fresh frozen plasma, and/or clotting factors can achieve more rapid anticoagulant reversal than can drug discontinuation alone. Activated prothrombin complex concentrate is used to treat hemophiliac patients with acquired factor VIII inhibitors. Nonactivated prothrombin complex concentrates are used for anticoagulant reversal. The concentrates are effective within minutes of dosing, providing a nearly immediate decrease in the international normalized ratio. The concentrates are lyophilized powders that can be quickly reconstituted, do not require ABO blood typing before use, and contain 25 times the concentration of vitamin K–dependent clotting factors compared with fresh frozen plasma. Studies suggest that the concentrates are associated with better clinical end points than is fresh frozen plasma.

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Prothrombin Complex Concentrate Versus Fresh Frozen Plasma for Vitamin K Antagonist Reversal in Acutely Bleeding Patients: A Retrospective Study

Blood ◽

10.1182/blood.v128.22.3830.3830 ◽

2016 ◽

Vol 128 (22) ◽

pp. 3830-3830

Author(s):

Martin H. Ellis ◽

Orly Avnery ◽

Leticia Aizenberg ◽

Muhamad Mahamid

Keyword(s):

Hospital Admission ◽

Fresh Frozen Plasma ◽

Vitamin K Antagonist ◽

Red Cell ◽

Major Hemorrhage ◽

Urgent Surgery ◽

Fresh Frozen ◽

Clinical Benefits ◽

Red Cell Transfusion ◽

Frozen Plasma

Abstract Introduction Vitamin K antagonist (VKA) drugs require immediate reversal in VKA-treated patients with major bleeding or requiring urgent surgery. 4-factor prothrombin complex concentrates (PCCs)are approved for urgent VKA reversalbecause they reverse the international normalized ratio (INR) more rapidly than fresh frozen plasma (FFP). Studies comparing the clinical benefits of PCC and FFP have focused on VKA reversal prior to urgent surgery. Few data comparing laboratory and clinical outcomes of patients receiving PCC or FFP for major hemorrhage have been published, and these pertain to intracranial hemorrhage (ICH) only. Given the complexity of performing randomized studies in this setting, observational studies are relevant to inform on this issue. AIMS To compare the effects of PCC versus FFP on patient outcomes in VKA-associated major hemorrhage. The primary outcomes were the rate of INR reversal and blood product utilization. The secondary outcome was duration of intensive care and total hospital admission. Methods We performed a retrospective, single-center study of consecutive unselected patients receiving a 4-factor PCC for VKA reversal because of hemorrhage between January 2012 and April 2015 compared to consecutive unselected patients treated with FFP for the same indication from January 2010-December 2011, a period prior to introduction of PCC at the Meir Medical Center. Patients were identified by review of clinical and blood bank electronic medical records. We analyzed patient demographics, indication for VKA, underlying illnesses, aspirin use, site and severity of hemorrhage, INR pre- and post reversal, rate of INR reversal, transfusion requirements and duration of hospitalization, treatment. Results 56 patients received PCC and were compared to 56 patients treated with FFP. In the PCC group 17 patients had ICH and 25 had gastrointestinal hemorrhage compared to 17 and 31 patients respectively in the FFP group. Patients were adjusted for age, sex, presence of renal failure, active cancer, aspirin use, site of hemorrhage, pre-treatment INR and hemoglobin concentration and hemorrhagic shock at presentation. Outcomes: Median time to INR of ≤1.3 was 0.5 (range 0.5-1.5) vs 15.5 (range 5-96) hours for PCC vs FFP respectively, P<0.001). Packed red cell transfusion did not differ between the groups: median =1(range 0-13) in the PCC group and median= 2(range=2-10) in the FFP group (P=0.3), but more FFP was transfused in the FFP vs PCC group median =0(range 0-8) in the PCC group and median= 4(range=2-8) in the FFP group (P<0.001). Duration of hospital admission was longer in the FFP (median=7 days, range 1-93) vs PCC patients (median =6 days, range=1-35) (P=0.04). Conclusion This is the first observational study comparing PCC and FFP for VKA-related hemorrhage to our knowledge and the first study of PCC versus FFP for extracranial VKA-related hemorrhage. PCC was more effective than FFP in for reversal of the INR and was associated with less overall FFP use but not packed red cell transfusion. Similarly hospital admission duration was shorter among the PCC patients. Larger studies are required to determine whether PCC confers other clinical benefits over FFP for VKA reversal in acutely bleeding patients. Disclosures Ellis: Boehringer Ingelheim: Speakers Bureau; Bayer: Speakers Bureau; Pfizer: Speakers Bureau.

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Peri- and Postpartum Management of Patients With Factor XI Deficiency

Clinical and Applied Thrombosis/Hemostasis ◽

10.1177/1076029619880262 ◽

2019 ◽

Vol 25 ◽

pp. 107602961988026 ◽

Cited By ~ 2

Author(s):

Gloria F. Gerber ◽

Kelsey A. Klute ◽

John Chapin ◽

James Bussel ◽

Maria T. DeSancho

Keyword(s):

Pregnant Women ◽

Medical Center ◽

Academic Medical Center ◽

Fresh Frozen Plasma ◽

Neuraxial Anesthesia ◽

Factor Xi ◽

Antifibrinolytic Agents ◽

Factor Xi Deficiency ◽

Severe Deficiency ◽

Fresh Frozen

Factor XI (FXI) deficiency is an uncommon autosomal disorder with variable bleeding phenotype, making peripartum management challenging. We describe our experience in pregnant women with FXI deficiency and identify strategies to minimize the use of hemostatic agents and increase utilization of neuraxial anesthesia. Electronic records of 28 pregnant women with FXI deficiency seen by a hematology service in an academic medical center from January 2006 to August 2018 were reviewed. Data on bleeding, obstetric history, peripartum management, and FXI activity were collected. Partial FXI deficiency was defined as >20 IU/dL and severe <20 IU/dL. Median FXI activity was 42 IU/dL (range <1-73 IU/dL), and median activated partial thromboplastin time was 32.2 seconds (range: 27.8-75 seconds). There were 64 pregnancies: 53 (83%) live births and 11 (17%) pregnancy losses. Postpartum hemorrhage occurred in 9 (17%) pregnancies. Antifibrinolytic agents and fresh frozen plasma were used only in women with severe deficiency (42% with bleeding and 17% with no bleeding phenotype, respectively). Neuraxial anesthesia was successfully administered in 32 (59%) deliveries. Most women with FXI deficiency have uncomplicated pregnancies and deliveries with minimal hemostatic support. Neuraxial anesthesia can be safely administered in most women.

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Treatment of rare factor deficiencies in 2016

Hematology ◽

10.1182/asheducation-2016.1.663 ◽

2016 ◽

Vol 2016 (1) ◽

pp. 663-669 ◽

Cited By ~ 20

Author(s):

Flora Peyvandi ◽

Marzia Menegatti

Keyword(s):

Clinical Symptoms ◽

Therapeutic Option ◽

Fresh Frozen Plasma ◽

Factor V ◽

Bleeding Disorders ◽

Antifibrinolytic Agents ◽

Specific Product ◽

Wide Range ◽

Fresh Frozen ◽

Hemostatic Factors

Abstract Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K–dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population. Patients affected with RBDs may present a wide range of clinical symptoms, varying from mucocutaneous bleeding, common to all types of RBDs to the most life-threatening symptoms such as central nervous system and gastrointestinal bleeding. Treatment of these disorders is mainly based on the replacement of the deficient factor, using specific plasma-derived or recombinant products. In countries where these facilities are not available, bleedings could be managed using cryoprecipitate, fresh frozen plasma (FFP), or virus-inactivated plasma. Minor bleedings could be managed using antifibrinolytic agents. Recently, 2 novel drugs, recombinant FXIIIA and a plasma-derived FX, have been added to the list of available specific hemostatic factors; only prothrombin and FV deficiencies still remain without a specific product. Novel no-replacement therapies, such as monoclonal antibody anti–tissue factor pathway inhibitor, RNA interference, and a bispecific antibody that is an FVIIIa mimetic, enhancing thrombin generation through different mechanisms, were developed for patients with hemophilia and may in the future be a good therapeutic option also in RBDs.

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Coagulopathies in Intensive Care Medicine: Balancing Act between Thrombosis and Bleeding

Journal of Clinical Medicine ◽

10.3390/jcm10225369 ◽

2021 ◽

Vol 10 (22) ◽

pp. 5369

Author(s):

Friederike S. Neuenfeldt ◽

Markus A. Weigand ◽

Dania Fischer

Keyword(s):

Critical Care ◽

Treatment Options ◽

Fresh Frozen Plasma ◽

Clotting Factor ◽

Individual Risk ◽

Antifibrinolytic Agents ◽

Coagulation Management ◽

Fresh Frozen ◽

The Individual ◽

Critical Care Patients

Patient Blood Management advocates an individualized treatment approach, tailored to each patient’s needs, in order to reduce unnecessary exposure to allogeneic blood products. The optimization of hemostasis and minimization of blood loss is of high importance when it comes to critical care patients, as coagulopathies are a common phenomenon among them and may significantly impact morbidity and mortality. Treating coagulopathies is complex as thrombotic and hemorrhagic conditions may coexist and the medications at hand to modulate hemostasis can be powerful. The cornerstones of coagulation management are an appropriate patient evaluation, including the individual risk of bleeding weighed against the risk of thrombosis, a proper diagnostic work-up of the coagulopathy’s etiology, treatment with targeted therapies, and transfusion of blood product components when clinically indicated in a goal-directed manner. In this article, we will outline various reasons for coagulopathy in critical care patients to highlight the aspects that need special consideration. The treatment options outlined in this article include anticoagulation, anticoagulant reversal, clotting factor concentrates, antifibrinolytic agents, desmopressin, fresh frozen plasma, and platelets. This article outlines concepts with the aim of the minimization of complications associated with coagulopathies in critically ill patients. Hereditary coagulopathies will be omitted in this review.

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Short-term prophylaxis for children and adolescents with hereditary angioedema

Allergy and Asthma Proceedings ◽

10.2500/aap.2021.42.210006 ◽

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.

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The Challenges in the Follow-Up and Treatment of Brazilian Children with Hereditary Angioedema

International Archives of Allergy and Immunology ◽

10.1159/000512944 ◽

2021 ◽

pp. 1-7

Author(s):

Joanna Araújo-Simões ◽

Aline Gisele Pena Boanova ◽

Rosemeire Navickas Constantino-Silva ◽

Nyla Thyara Melo Lobão Fragnan ◽

Jorge Andrade Pinto ◽

...

Keyword(s):

Family History ◽

Tranexamic Acid ◽

Hereditary Angioedema ◽

Pediatric Patients ◽

Clinical Manifestations ◽

Fresh Frozen Plasma ◽

Child Therapy ◽

Strong Impact ◽

Upper Airways ◽

Fresh Frozen

Introduction: Hereditary angioedema (HAE) with C1 inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. Although the first symptoms can appear in childhood, the diagnosis’s delay has a strong impact on the patient’s quality of life. We analyzed clinical and laboratory characteristics and the drug therapy of pediatric patients with HAE in Brazil. Methods: Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH. Results: A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients. Conclusions: This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.

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Das Smith-Lemli-Opitz-Syndrom

Kinder- und Jugendmedizin ◽

10.1055/s-0037-1617867 ◽

2005 ◽

Vol 5 (04) ◽

pp. 178-182

Author(s):

Wieland Kiess ◽

Manuela Schulz ◽

Sabine Liebermann ◽

Roland Pfäffle ◽

Peter Bührdel ◽

...

Keyword(s):

Fresh Frozen Plasma ◽

Therapeutische Möglichkeiten ◽

Fresh Frozen ◽

Frozen Plasma

ZusammenfassungDas Smith-Lemli-Opitz-Syndrom wird durch einen Defekt des letzten Schrittes der Cholesterolbiosynthese, den Mangel an 7-Dehydrocholesterolreduktase, verursacht. Die Akkumulation der Metaboliten 7-Dehydrocholesterol und 8-Dehydrocholesterol, die die wichtigsten biochemischen Marker für die Diagnose der Erkrankung darstellen, sowie der Mangel an Cholesterol können zu multiplen kongenitalen Anomalien führen. Die Ursache des Enzymmangels sind Mutationen innerhalb des DHCR7-Gens, welches auf Chromosom 11q13 lokalisiert ist. Therapeutische Möglichkeiten bestehen in der Gabe von Cholesterol und im Notfall Fresh Frozen Plasma (FFP); der therapeutische Nutzen von Statinen befindet sich zurzeit in der klinischen Erprobung.

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