The disease presents with orbital pain, edema on the eyelids, erythema, and fever. In this case, a child with hereditary angioedema type 2 who presented as mimicking a complication of acute sinusitis is discussed. [ncbi.nlm.nih.gov]

She presented multiple times with angioedema involving head and neck region and required multiple paediatric Intensive care unit admissions with potential risk of air way compromise. [adc.bmj.com]

A diagnosis of HAE may be made based on clinical presentation and supporting laboratory studies, and imaging studies are rarely helpful. [clinicaladvisor.com]

Case presentation We present the case of a 41 year-old male who presented to the allergy/immunology clinic after having an episode of facial, lip, and tongue swelling following tooth extraction. [hoajonline.com]

• Swelling

  On physical examination, the peripheral swelling during an acute attack is typically asymmetric and not well circumscribed. Facial edema and oral mucuous membrane swelling may be present. [clinicaladvisor.com]

  We herein describe a case of 25-year-old male who presented with swelling over face since one day. There was history of similar episodes since two years with gradual subsidence of swelling without any treatment. [e-ijd.org]

  Swelling in your belly can cause: Extreme pain Nausea Vomiting Diarrhea You may notice warning signs before swelling begins. [webmd.com]

  Abstract BACKGROUND: Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance characterized by sudden attacks of peripheral swelling. [ncbi.nlm.nih.gov]

• Pain

  GI edema manifests as severe abdominal pain and cramping (mimicking acute abdomen), while laryngeal edema may cause breathing difficulties and even asphyxiation. [symptoma.com]

  The patient had no symptoms until 3 months ago when she started to have daily abdominal pain below the umbilicus. The pain lasts a whole day and she has night symptoms as well. There is no consistent relief provided by pain medications. [allergycases.blogspot.com]

  Abdominal associated with angioedema may manifest as severe acute-onset abdominal pain or as moderately severe chronic recurrent abdominal pain. Two medications are currently FDA-approved for the treatment of these patients. [ncbi.nlm.nih.gov]

  HAE is a potentially lethal disease and should be considered if repeating edema of various body parts, painful swelling and tightening of the skin are present. [signavitae.com]

• Fatigue

  Common warning signs that can signal the onset of an HAE attack include: Sudden mood changes Irritability or aggressiveness Anxiety Extreme fatigue Rash or tingling sensation of the skin where the swelling begins Nausea Understanding what triggers your [berinert.com]

  Dagen & Craig (2010) documented that “the most common identifiable prodromal symptoms include unusual fatigue, rash on arms or legs and muscle aches” (p. 3). [haecanada.org]

  […] possibly associated with an increased incidence of autoimmune disease Prognosis variable after the attack occurs, they may recur throughout the patient's life attack frequency can be reduced with appropriate therapy Presentation Symptoms prodromal symptoms fatigue [medbullets.com]

  Some symptoms of hereditary angioedema include: Swelling of the skin (most common symptom) Swelling of the hands and feet Fatigue Headache Muscle aches Skin tingling Abdominal pain (sometimes severe) Nausea and vomiting Hoarseness Shortness of breath [rxlist.com]

• Malaise

  Fatigue, malaise, irritability, hyperactivity, mood changes, and nausea are other preceding factors [ 21, 24 ]. The severity of HAE is variable and usually unpredictable. [intechopen.com]

• Collapse

  In at least some cases, these symptoms can lead to hypovolemic shock, ascites, and, in rare cases, circulatory collapse, tetany, hemorrhagic stools, dysuria, and intussusception of the colon. 38, 53, 54 Age at initial symptoms In about one-third of HAE [dovepress.com]

• Hoarseness

  A 34-year-old female patient presented to the hospital complained of swollen and painful legs, flatulence, palpebral and labial edema, dyspnea, dysuria, frequent herpetic infections of mouth and nose, subfebrile body temperature and was hoarse. [signavitae.com]

  Some symptoms of hereditary angioedema include: Swelling of the skin (most common symptom) Swelling of the hands and feet Fatigue Headache Muscle aches Skin tingling Abdominal pain (sometimes severe) Nausea and vomiting Hoarseness Shortness of breath [rxlist.com]

  These may include: Extreme fatigue Muscle ache Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as [webmd.com]

  Voice changes - high-pitched, hoarse, rough or with a resonant, 'barky' cough or unable to speak. [patient.info]

• Stridor

  […] swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea, vomiting, and/or diarrhea Physical exam skin erythema marginatum may be seen upper airway voice changes or horseness stridor [medbullets.com]

  Pruritus, urticaria, and bronchospasm do not occur, but laryngeal edema may be present, causing stridor (and sometimes death). Swelling resolves within about 1 to 3 days of onset. [merckmanuals.com]

  Edema of the throat (pharynx) or voice-box (larynx) can result in pain, difficulty swallowing (dysphagia), difficulty speaking (dysphonia), noisy respiration (stridor), and potentially life-threatening asphyxiation. [rarediseases.org]

  Symptoms/signs of respiratory distress - eg, stridor, dyspnoea, fear of suffocation, anxiety/agitation; the patient may grasp his/her throat with the thumb and index fingers (the universal choking sign). [patient.info]

  Ominous signs suggesting airway involvement include dysphagia, voice change, and stridor. There will generally be a family history of angio-oedema. However, 5% of adults carry the mutation for the C1-INH gene while remaining asymptomatic. [academic.oup.com]

• Dyspnea

  A 34-year-old female patient presented to the hospital complained of swollen and painful legs, flatulence, palpebral and labial edema, dyspnea, dysuria, frequent herpetic infections of mouth and nose, subfebrile body temperature and was hoarse. [signavitae.com]

  Her medical history showed sudden onsets of facial swelling, vomiting, abdominal pain and dyspnea several times a year since infancy. [omicsonline.org]

  Other symptoms that can appear are edema of glottis and/or pharynx, dyspnea, dysphagia, abdominal distention, vomiting, diarrhea, and constipation, so far our patient has not had digestive symptoms. [medwave.cl]

• Pleural Effusion

  effusions rarely may be seen Abdominal ultrasonography or computed tomography scanning: Edematous thickening of the intestinal wall, a fluid layer around the bowel, and large amounts of free peritoneal fluid may be found See Workup for more detail. [emedicine.medscape.com]

• Abdominal Pain

  Abdominal associated with angioedema may manifest as severe acute-onset abdominal pain or as moderately severe chronic recurrent abdominal pain. Two medications are currently FDA-approved for the treatment of these patients. [ncbi.nlm.nih.gov]

  The patient had no symptoms until 3 months ago when she started to have daily abdominal pain below the umbilicus. The pain lasts a whole day and she has night symptoms as well. There is no consistent relief provided by pain medications. [allergycases.blogspot.com]

  It is characterized by swelling of limbs, face, airway and abdominal pains caused by trauma, cold and stress.The mortality rate is up to 35% due to laryngeal oedema therefore spinal anesthesia was preferred. [journals.lww.com]

  Brief Summary: Development of a new MS-based biomarker for the early and sensitive diagnosis of hereditary angioedema disease type 1 from the blood C1 Esterase Inhibitor Deficiency Angio-Oedema of Lips Angio-Oedema of Tongue Abdominal Pain Laryngeal Edema [clinicaltrials.gov]

• Vomiting

  Her medical history showed sudden onsets of facial swelling, vomiting, abdominal pain and dyspnea several times a year since infancy. [omicsonline.org]

  Pain irradiated to the right lower quadrant and was associated with five episodes of vomiting. Computed tomography showed thickening of the duodenal wall with liquid in the subphrenic space. [ncbi.nlm.nih.gov]

  Symptoms include abdominal pain (can be severe or sudden in onset), nausea and vomiting, diarrhoea, constipation and abdominal distention. Large fluid shifts with ascites or hypovolaemic shock may occur. [patient.info]

  […] pruritus affecting the skin swelling of the extremities, face, and genitals can affect any area upper airway swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea, vomiting [medbullets.com]

• Nausea

  […] urticaria or pruritus affecting the skin swelling of the extremities, face, and genitals can affect any area upper airway swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea [medbullets.com]

  Although they have few side effects involving most commonly nausea, vomiting, and diarrhea, which are dose‐dependent, they are not as effective as androgens. [intechopen.com]

  […] tissues occur throughout the body without the presence of urticaria Typically involves arms, legs, hands, trunk, face, mouth, larynx, airway, genitals, and tongue Gastrointestinal tract often involved, with recurrent episodes of cramping, abdominal pain, nausea [arupconsult.com]

  The most common adverse reactions reported in patients treated with Ruconest were headache, nausea and diarrhea. [aetna.com]

• Diarrhea

  Swelling in your belly can cause: Extreme pain Nausea Vomiting Diarrhea You may notice warning signs before swelling begins. [webmd.com]

  […] affecting the skin swelling of the extremities, face, and genitals can affect any area upper airway swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea, vomiting, and/or diarrhea [medbullets.com]

  Attacks of acute pain, nausea, and diarrhea were accompanied by severe, mysterious swelling. It was excruciating and unrelenting. At times, my abdomen would be so distended I appeared pregnant. [globalgenes.org]

  The most common adverse reactions included subsequent HAE attack, abdominal pain, diarrhea, headache, muscle spasms, nausea, pain, and vomiting. [link.springer.com]

• Dysphagia

  Clinical features Recurrent episodes of angio-oedema and/or abdominal pain - may involve: Laryngeal oedema - can be fatal: Symptoms and signs of laryngeal oedema Throat - sore, tight, itchy, lump, 'something stuck', or dysphagia. [patient.info]

  Edema of the throat (pharynx) or voice-box (larynx) can result in pain, difficulty swallowing (dysphagia), difficulty speaking (dysphonia), noisy respiration (stridor), and potentially life-threatening asphyxiation. [rarediseases.org]

  Other symptoms that can appear are edema of glottis and/or pharynx, dyspnea, dysphagia, abdominal distention, vomiting, diarrhea, and constipation, so far our patient has not had digestive symptoms. [medwave.cl]

  Ominous signs suggesting airway involvement include dysphagia, voice change, and stridor. There will generally be a family history of angio-oedema. However, 5% of adults carry the mutation for the C1-INH gene while remaining asymptomatic. [academic.oup.com]

  Isolated dysphagia or edema of the gastrointestinal tract are rare. Angioedema of the upper respiratory tract can result in acute respiratory distress, airway obstruction and, rarely, death. [aacijournal.biomedcentral.com]

• Hypotension

  If hypotension is present, elevation of the legs, commencement of basic life support, if indicated, and administration of i.v. crystalloid at rapid infusion rates are the first steps in treatment. [academic.oup.com]

  It leads to an increase in vascular permeability by binding to the B2 receptor on the vascular endothelial cells, and this in turn causes the development of edema, ascites, and hypotension [ 25, 26 ]. [intechopen.com]

  Allergy attacks are rapid in onset, there is generally a recognisable allergen, and will be associated with a superficial itch, a generalised rash with raised wheals, respiratory wheeze, abdominal pain and possibly hypotension. [rcemlearning.co.uk]

  Antifibrinolytics also may have efficacy for HAE, but these agents have been associated with a variety of adverse effects, including nausea and diarrhea, postural hypotension, fatigue, enhanced thrombosis, retinal changes, and teratogenicity. 8, 22, 23 [the-hospitalist.org]

  When urticaria, generalized pruritus, or hypotension is present, the mast cell system is usually involved. In these cases, internal and external triggers (ie, foods, drugs, insects) should be sought. [mdmag.com]

• Lip Swelling

  Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing. [hoajonline.com]

  Causes of Swollen Lips Swelling of the lips can be caused by a variety of different conditions. [medicinenet.com]

  Facial swellings, mainly lip swellings, are relatively more frequent. The tongue is considerably more often affected: Recurrent tongue swelling is observed in many patients and is a cardinal symptom of the condition. [aacijournal.biomedcentral.com]

• Myalgia

  Antifibrinolytic agents are reserved for patients who cannot tolerate anabolic steroids or other forms of prophylactic therapy. 12,13 Side effects associated with these agents include myalgias, fatigue, and coagulation disorders. [clevelandclinicmeded.com]

  Adverse events were reported by nearly 80% of patients on danazol therapy and included weight gain, menstrual irregularities, female virilization, acne, headache, depression, and myalgia. [link.springer.com]

  Side-effects include myalgia, muscle weakness, hypotension, fatigue, and elevation of serum creatine kinase (CK). Liver and renal function, serum CK, and ocular pressures (risk of glaucoma) need to be monitored on treatment (Table 2 ). [academic.oup.com]

  The most frequent adverse effects of the 17α‐alkylated androgens are menstrual irregularities, changes in libido, hirsutism, acne, changes in mood, weight gain, myalgia, erythrocytosis, increased blood pressure, and abnormalities in lipid profiles [ 51 [intechopen.com]

• Swelling of Hand

  Figure 1: Hand swelling (left hand). She was referred to our obstetric outpatient ward for the care of pregnancy. At 18 weeks of pregnancy, she had an episode of abdominal pain and GI tract edema was suspected. [omicsonline.org]

• Hunger

  Attacks are frequently accompanied by prodromal symptoms, including nonspecific complaints of irritability, aggressiveness, fatigue, or hunger[ 15 ]. [link.springer.com]

• Facial Swelling

  Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing. [hoajonline.com]

  Her medical history showed sudden onsets of facial swelling, vomiting, abdominal pain and dyspnea several times a year since infancy. [omicsonline.org]

  severe abdominal pain, facial swelling, laryngeal swelling, nausea and vomiting, painful facial distortion); and Medications known to cause angioedema (i.e. [aetna.com]

  In 2009, FDA approved Berinert, to treat acute abdominal attacks and facial swelling associated with HAE in adults and adolescents. It is a protein product derived from human plasma, and is manufactured by CSL Behring, Inc. [rarediseases.org]

  In this context one or more episodes of isolated angioedema may occur, mostly as a facial swelling. These are reactive swellings, i.e. swellings with a recognizable trigger. They do not occur without a trigger. [aacijournal.biomedcentral.com]

• Facial Edema

  In several occasions he has experienced palpebral and facial edema, the first times repeating every 30 or 45 days, with no apparent cause. [medwave.cl]

  Facial edema and oral mucuous membrane swelling may be present. The abdomen may be distended and tender to palpation, and bowel sounds are often diminished. Abdominal attacks often lead to unnecessary imaging and surgical exploration. [clinicaladvisor.com]

• Urinary Retention

  Rare symptoms include urinary retention due to mucosal edema of the bladder or urethra. [ijdvl.com]

  The genitourinary tract may also be involved, causing acute urinary retention, often with hematuria. Patients also often experience headaches, although the reason for this remains unclear. Angioedema itself is not known to cause a febrile illness. [mdmag.com]

• Tingling

  The rash is characterized by red rings that appear on the trunk and appendages as part of a prodrome that may also include a tingling sensation in the area where swelling will occur. 7 Reproduced with permission from the US Hereditary Angioedema Association [jaoa.org]

  Common warning signs that can signal the onset of an HAE attack include: Sudden mood changes Irritability or aggressiveness Anxiety Extreme fatigue Rash or tingling sensation of the skin where the swelling begins Nausea Understanding what triggers your [berinert.com]

  […] children) Most frequent cause of death is airway obstruction secondary to laryngeal edema Presence of autoimmune diseases (especially glomerulonephritis ) is higher in these patients Typical and predictable course Many attacks, preceded by prodrome (tingling [arupconsult.com]

  Some symptoms of hereditary angioedema include: Swelling of the skin (most common symptom) Swelling of the hands and feet Fatigue Headache Muscle aches Skin tingling Abdominal pain (sometimes severe) Nausea and vomiting Hoarseness Shortness of breath [rxlist.com]

  You may also experience skin tingling or tightness. [health.ucsd.edu]

• Headache

  Upper respiratory infection, sinusitis, rash, and headache were the most commonly reported adverse reactions[ 34 ]. [link.springer.com]

  Some symptoms of hereditary angioedema include: Swelling of the skin (most common symptom) Swelling of the hands and feet Fatigue Headache Muscle aches Skin tingling Abdominal pain (sometimes severe) Nausea and vomiting Hoarseness Shortness of breath [rxlist.com]

  According to the product prescribing information, the most common adverse reactions observed by at least 5% of subjects are upper respiratory tract infection, sinusitis, rash, and headache. [clevelandclinicmeded.com]

  These may include: Extreme fatigue Muscle ache Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as [webmd.com]

• Irritability

  Common warning signs that can signal the onset of an HAE attack include: Sudden mood changes Irritability or aggressiveness Anxiety Extreme fatigue Rash or tingling sensation of the skin where the swelling begins Nausea Understanding what triggers your [berinert.com]

  Due to the nature of the disease, swelling can also occur in the intestines, causing various types of episodic and unpredictable abdominal pain, which may be confused with irritable bowel syndrome, inflammatory bowel disease, gynecological conditions [health.ucsd.edu]

  Firazyy is a 10 amino acid peptide which causes local irritation, but otherwise has not been associated with serious side effects thus far. It is approved for self-administration subcutaneously. [clinicaladvisor.com]

  Attacks are frequently accompanied by prodromal symptoms, including nonspecific complaints of irritability, aggressiveness, fatigue, or hunger[ 15 ]. [link.springer.com]

• Ataxia

  遺伝性運動失調症 hereditary central nervous system demyelinating disease 遺伝性中枢神経系脱髄疾患 hereditary cerebellar ataxia 遺伝性小脳性運動失調 hereditary chorea 遺伝性舞踏病 hereditary coproporphyria 遺伝性コプロポルフィリン症 hereditary corneal dystrophy 遺伝性角膜ジストロフィー hereditary deafness 遺伝性難聴 [jpeds.or.jp]

Workup for AAE included evaluation for both lymphoproliferative and autoimmune disease. [hoajonline.com]

Patients that have a parent with hereditary angioedema have a good possibility of inheriting the disease (about 50%), so the family genetics history is part of the diagnostic workup if hereditary angioedema is suspected. [rxlist.com]

The most reliable and cost-effective screening test for HAE is a serum C4 level (see Workup). Treatment of HAE consists of prophylaxis, management of acute attacks, and prophylactic therapy in situations where attacks may occur. [emedicine.medscape.com]

In ACID, levels of C1q are also reduced; these patients require further workup to rule out an undiagnosed malignancy or an autoimmune process. [the-hospitalist.org]

• Neutrophilia

  […] attacks may be nonspecific with diffuse abdominal tenderness on palpation, hyper- or hypoactive bowel sounds, and shifting dullness if ascites is present. 51 During an acute abdominal episode, patients may present with significant leukocytosis with neutrophilia [dovepress.com]

• C4 Decreased

  Decreased levels suggest hereditary angioedema Confirm with repeat testing of C1-INH and C4 If family history present, consider functional testing for typing Functional C1-inhibitor activity Assists with determination of hereditary angioedema type Consider [arupconsult.com]

• Pleural Effusion

  effusions rarely may be seen Abdominal ultrasonography or computed tomography scanning: Edematous thickening of the intestinal wall, a fluid layer around the bowel, and large amounts of free peritoneal fluid may be found See Workup for more detail. [emedicine.medscape.com]

[…] of breathing if treatment is delayed. [symptoma.com]

HAE treatment consists of prophylactic treatment, treatment of acute attacks and prophylactic treatment prior to certain medical interventions (dental procedures). [signavitae.com]

Patients usually begin to respond within 30-60 minutes of treatment. [clinicaladvisor.com]

It is likely that HAE treatment will change dramatically in near future. [allergycases.blogspot.com]

Cost of treatment is an issue [ 10 ]. [patient.info]

What is the prognosis for a person with hereditary angioedema (HAE)? There is no known cure for hereditary angioedema. The prognosis for an individual with hereditary angioedema ranges from good to fair. [rxlist.com]

HAE can be a debilitating disease in the absence of appropriate therapy, which is why an early diagnosis carries a very good prognosis and minimal impairment of daily life. [symptoma.com]

Nagamachi Seiji, Ohsawa Isao, Suzuki Hiyori, Sato Nobuyuki, Inoshita Hiroyuki, Hisada Atsuko, Honda Daisuke, Shimamoto Mamiko, Shimizu Yoshio, Horikoshi Satoshi, Tomino Yasuhiko BMC NEPHROLOGY 15 May 2014 [Refereed] ASSOCIATION BETWEEN THE WORSE RENAL PROGNOSIS [researchmap.jp]

With appropriate therapy and monitoring for adverse effects associated with therapy, HAE is not associated with any long-term complications and carries a good prognosis. [clinicaladvisor.com]

Hence, etiology of AAE was determined to be IgM-monoclonal gammopathy of undetermined significance (MGUS). [hoajonline.com]

Despite the existence of a myriad of etiologies, most cases of angioedema remain idiopathic. Acquired angioedema has a high correlation with malignancies. [mdmag.com]

In instances of high clinical suspicion and recurrent episodic angioedema of uncertain etiology, genetic testing is indicated. [rarediseases.org]

Ongoing controversies regarding etiology, diagnosis, treatment Considerable controversy surrounds the diagnosis and management of HAE type III. [clinicaladvisor.com]

One recent study of fatalities due to HAE identified a significantly higher mortality by asphyxiation in undiagnosed HAE patients. 43 It is important to note that bradykinin-mediated angioedema may result from other etiologies that can be confused with [dovepress.com]

[…] lymphomas) Autoimmune disease Neoplasia Urticarial vasculitis associated with angioedema Idiopathic or cold-induced angioedema Cellulitis Parasitic infection (eg, Trichinella spp) Autoimmune conditions Laryngeal edema – peritonsillar abscess Background Epidemiology [arupconsult.com]

TABLE OF CONTENTS 5 OVERVIEW 6 KEY TAKEAWAYS 7 DISEASE BACKGROUND 7 Subtypes 8 TREATMENT 8 C1-INH concentrates 8 Plasma-derived C1-INH (pdC1-INH) 8 Recombinant C1-INH (rhC1-INH) 8 Kallikrein inhibitor 8 Bradykinin receptor antagonist 10 EPIDEMIOLOGY 14 [gii.co.jp]

[…] angioedema without urticaria or pruritus triggers include physical dental work surgery intubation medical angiotensin-converting enzyme (ACE) inhibitors tamoxifen estrogen-containing medications ( e.g., hormone replacement therapy and oral contraceptives) Epidemiology [medbullets.com]

[…] table for study information Study Type : Observational Estimated Enrollment : 1000 participants Observational Model: Cohort Time Perspective: Prospective Official Title: Biomarker for Hereditary Angioedema Disease Type 1 - An International, Multicenter, Epidemiological [clinicaltrials.gov]

However, neither disease prevalence nor epidemiologic features of HAE has been surveyed in Japan. Methods: A nation-wide prevalence survey of HAE in Japan was conducted in 2009. [ci.nii.ac.jp]

Journal Transfus Apher Sci 29:195-203 (2003) DOI: 10.1016/j.transci.2003.08.012 Reference PMID: 15596403 Authors Davis AE 3rd Title The pathophysiology of hereditary angioedema. [genome.jp]

On the other hand, the role of B1 (B1R) receptors as part of the pathophysiology of FSGS lesions is well established by the adriamycin-induced FSGS model in mice. [clinical-experimental-nephrology.imedpub.com]

1 – low complement 1 esterase inhibitor (C1-INH) level and low function (85%) If C1q is also low, suspect acquired angioedema Type 2 – normal C1-INH level but low function (15%) Type 3 (familial angioedema) – normal C1-INH and normal function (rare) Pathophysiology [arupconsult.com]

In the past decade, there has been substantial advancement in our understanding of HAE pathophysiology. HAE occurs as a result of functional or quantitative C1 esterase inhibitor (C1INH) deficiency. [the-hospitalist.org]

We do not yet fully understand how these genetic alterations contribute to the pathophysiology of nC1-INH-HAE. [rarediseasesjournal.com]

Management of acute attacks and long-term use of drugs to prevent their occurrence is the mainstay of treatment and patients should be trained for self-treatment at home, in order to prevent life-threatening edema of the larynx that can cause cessation [symptoma.com]

Prevention of hereditary anagioedema attacks with a subcutaneous inhibitor. N Engl J Med. 2017;376 (12):1131-1140. [raredr.com]

How can Hereditary Angioedema be prevented? Hereditary Angioedema cannot be prevented, but appropriate management includes measures that reduce attack frequency. [clinicaladvisor.com]

Food and Drug Administration approved lanadelumab, an injectable monoclonal antibody, to prevent attacks of HAE types I and II in patients over age 12. [en.wikipedia.org]

To prevent complications it is important to start early treatment. [medwave.cl]