Anesthetic management of a patient with acquired angioedema submitted to broncofibroscopy: a case report

Abstract Acquired angioedema with C1 inhibitor deficiency (AAE-C1INH) is a very rare condition of bradykinin-mediated angioedema. One of its major complications is potentially life-threatening, laryngeal edema. We report a 53-year-old woman with AAE-C1INH proposed for an elective broncofibroscopy. The direct stimulation caused by broncofibroscopy poses a high risk of angioedema, thus presenting an anesthetic challenge. Due to the risk of death, it is essential to adopt preventive measures. Short-term prophylaxis was performed, and the acute treatment was readily available. A well-structured multidisciplinary periprocedural plan makes it possible to safely approach the airway, in a remote area of the hospital.

Prospects for plasma protein products in China / 中国输血杂志

Plasma protein products, essential drugs for various clinical diseases, are therapeutic biological products extracted from healthy human plasma. The research and development of new plasma protein products, led by United States and European, has been widely deepened and enhanced. Therefore, accelerating the development of new plasma protein products in China is of great significance. This review summarizes the research and development of plasma protein products that have been marketed abroad but have not produced in China, as well as analyzes the difficulties and prospects of the development of plasma protein products in China.

Study on separation and purification process of C1 esterase inhibitor / 中国输血杂志

【Objective】 To study the technology of separating and purifying C1 esterase inhibitor (C1-INH) by using the waste washing liquid as raw materia during the preparation of human prothrombin complex (PCC) l. 【Methods】 C1-INH was isolated and purified by a two-step method of polyethylene glycol (PEG) 4000 precipitation and cation chromatography. The pH of raw materials and the concentration of PEG4000 were adjusted to investigate the optimal conditions of PEG4000 precipitation method. After PEG was precipitated and centrifuged, the supernatant is treated as the loading solution for cation exchange chromatography, using Fractogel EMD SE HiCap(M) gel and CM Sepharose FF gel for ion exchange chromatography. The most suitable gel and separation conditions were selected by comparing the C1-INH antigen yield, activity yield and specific activity. 【Results】 Under the condition of pH 6.1, when the mass fraction of PEG4000 was 14%, the recovery rate of C1 esterase inhibitor was close to 70%, and the removal rate of ceruloplasmin was more than 95% after stirring for 10 minutes. As fractogel EMD SE HiCap(M) gel was used for cation exchange chromatography, when the eluent salt concentration was 0.25 M sodium chloride, the activity yield of C1 esterase inhibitor was greater than 80%, and the specific activity was greater than 5 IU/mg. 【Conclusions】 Using the waste washing liquid as the raw material during the preparation of PCC, the C1 esterase inhibitor with high specific activity can be prepared through PEG precipitation and purification by Fractogel EMD SE HiCap(M) ion exchange chromatography.

Hereditary angioedema: a disease seldom diagnosed by pediatricians

Abstract Objectives: To describe the hereditary angioedema to improve awareness of this condition and reduce diagnostic delay. Data sources: Relevant articles in the MEDLINE database through PubMed. Data synthesis: Hereditary angioedema is rare and has an autosomal dominant pattern of inheritance. Its onset occurs mainly in childhood, but there is an important delay in the diagnosis. In the most frequent phenotype, there is a quantitative and/or functional deficiency in the C1esterase inhibitor protein, which regulates the activation of the complement, contact and fibrinolysis systems with greater formation of bradykinin, the main mediator of angioedema. There is a third type, the hereditary angioedema with a normal C1 inhibitor level, which is rare in children. Clinical manifestations are characterized by recurrent angioedema attacks, mainly in the extremities, abdomen and upper airways, which can progress to asphyxia and death. The main triggers are mechanical trauma, infections and stress. The diagnosis is attained by patient clinical picture and decreased serum levels of C4 and C1esterase inhibitor or its function. In hereditary angioedema with a normal C1 inhibitor, there is no change in these parameters, thus requiring a genetic study. Treatment is based on the use of attack medications and long and short-term prophylaxis. Conclusions: Hereditary angioedema is little known by pediatricians due to the significant delay in diagnosis of this condition, whose onset usually begins in childhood. The presence of recurrent angioedema that does not respond to treatment with antihistamines, corticosteroids and adrenaline should increase the diagnostic suspicion.

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

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.

Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases)

Hereditary angioedema (HAE) is a rare autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). The disease is characterized by subcutaneous and submucosal edema in the absence of urticaria due to the accumulation of bradykinin. This descriptive study aimed to evaluate the clinical characteristics of patients with a confirmed diagnosis of HAE referred to our Outpatient Clinic between December 2009 and November 2017. Fifty-one patients (38 F, 13 M) with a mean age of 32 years (range: 7-70 y) were included. Family history of HAE was reported in 70% (36/51) of the cases; 33/46 patients became symptomatic by 18 years of age. The median time between onset of symptoms and diagnosis was 13 years (3 mo-50 y). The most frequent triggering factors for attacks were stress (74.4%), trauma (56.4%), and hormonal variations (56%). The main symptoms were subcutaneous edema in 93.5% (43/46) of patients, gastrointestinal symptoms in 84.8% (39/46), and obstruction in the upper airways in 34.8% (16/46). Hospitalization occurred in 65.2%, of whom 13.3% had to be transferred to the Intensive Care Unit. Prophylactic treatment was instituted in 87% (40/46) of patients, and 56.5% (26/46) required additional treatment to control attacks. Owing to our data collection over a period of 8 years, a significant number of patients were identified by this HAE reference center. Despite early recognition and prophylactic treatment, a high percentage of patients were hospitalized. HAE is still diagnosed late, reinforcing the need for more reference centers specialized in diagnosis and educational projects for health professionals.

Angioedema hereditario, una enfermedad de fácil confusión en su diagnóstico / Hereditary angioedema, a disease of easily diagnosis confusion

El angioedema hereditario es una enfermedad genética rara, autosómica dominante, con una aparición de 1:10 000 a 1:50 000 habitantes, que presenta como síntoma principal episodios de edema definido, doloroso, deformante con ausencia de prurito, generalmente localizado en las extremidades. El diagnóstico se realiza basado en anamnesis, examen físico y determinación de C4, C1 - INH, cuyos niveles séricos deben encontrarse bajos. Se presenta el caso de un paciente masculino de 33 años que asiste a consulta con un edema deformante en el rostro y en las extremidades superiores, refiere que en su familia un abuelo presentó un cuadro semejante y sus dos hijos tienen estos síntomas. Los exámenes realizados reflejaron: C3: 87 mg/dL, C4: 10 mg/dL y actividad funcional de C1-INH: 33 por ciento; el resto de los estudios hormonales e inmunológicos mostraron valores dentro de los rangos normales. Se diagnosticó como angioedema hereditario tipo II y se impuso tratamiento profiláctico con Danazol (200 mg); el paciente lleva cuatro meses sin episodios(AU)

Hereditary angioedema is a rare genetic autosomal dominant disease with an appearance from 1:10 000 to 1:50 000, which main symptom is defined episodes of painful deforming edema with absence of pruritus, usually located in extremities. Diagnosis is based on anamnesis, physical examination and determination of C4, C1-INH, where low serum must be found. We present a male patient aged 33, who attends a consultation with disfiguring edema in the face and upper extremities reporting that in his family a grandfather presented a similar symptom as well as his two sons. The tests made reflected: C3: 87 mg / dl, C4: 10 mg / dl and functional activity of C1-INH: 33 percernt; other hormonal and immunological studies showed values within normal ranges. He was diagnosed as hereditary angioedema type II and received prophylactic treatment with Danazol (200 mg); the patient has now been four months without episodes(AU)

Angioedema due to acquired type of C1 esterase inhibitor deficiency / 대한내과학회지

No abstract available.

A case of hereditary angioedema not manifestated classical autosomal dominant trait / 알레르기

32 year-old male patient has experienced the repeated swelling of the skin on the eyelid, both hand, foot, lower and upper extrimities and testicle spontaneously without any trauma since 17 years old. This happening has subsided with or without treatment after 2-3 days. His great grandfather had suffered from the same events but grandfather or father or even his two brothers and one sister didn't have any swellings like him. Two days after this attack, he visited emergency room, his serum complement level was decreased such as C1q 9.7mg/dl (10-20mg/dl), C3 52mg/dl (55-120mg/dl), C4 4mg/dl (20-50mg/dl) measured by single radial immunodiffusion (SRID), but two weeks after full recovery, C1q 11.2mg/dl, C3 79mg/dl, C4 5mg/dl. The level of C1 esterase inhibitor was decreased upto 8.1 mg/dl (> 11 mg/dl) measured by nephlemeter(Mitshibishi Co., Japan). Now he is just under the close observation without androgen treatment because the attack of HAE(hereditary angioedema) happens much less and less severe after adolescence.