Ultrasonography in the diagnosis and monitoring of ascites in acute abdominal attacks of hereditary angioneurotic oedema

Farkas, Henriette; Harmat, G.; Kaposi, Pál Novák; Karádi, I; Fekete, B.; Füst, George; Fáy, Kálmán; Vass, Aniko

doi:10.1097/00042737-200110000-00016

Cited by 53 publications

(44 citation statements)

References 24 publications

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“…Abdominal ultrasound or CT scan may be performed to help exclude acute surgical abdominal disease 29, 30, 39, 40, 41, 42, 43, 44. Abdominal ultrasound may be a sensitive, rapid, and noninvasive differential diagnostic modality in patients with known C1‐INH‐HAE to help differentiate acute appendicitis and monitor response to event intervention with C1‐INH‐HAE therapeutic agents 43, 45, 46. Clinical and ultrasound response to specific C1‐INH‐HAE therapeutic medications helps differentiate C1‐INH‐HAE from non‐C1‐INH‐HAE‐related abdominal events.…”

Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

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BackgroundThe consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE.MethodsDuring an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting.ResultsThe symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center.ConclusionsThe pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.

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Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

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“…In a study of 22 acute abdominal HAE attacks severe enough to warrant hospital admission, abdominal ultrasound revealed thickening of the intestinal wall in 80% of the cases and the presence of free peritoneal fluid in all cases. Subsequent treatment of the HAE attack resulted in regression of all ultrasound abnormalities [41]. In another study of 11 acute abdominal HAE attacks, abdominal ultrasound performed during the attack revealed ascites and intestinal wall swelling in 7 of 11 patients, helping to confirm the diagnosis [42].…”

Section: Hereditary C1-inh Deficiency (Hae Types I and Ii)mentioning

confidence: 99%

Hereditary and Acquired Complement Component 1 Esterase Inhibitor Deficiency: A Review for the Hematologist

Cicardi

Johnston

2012

Acta Haematol

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Hereditary angioedema (HAE), a rare autosomal dominant disorder, was first described in the late 19th century. The disease remained poorly understood and without therapeutic options until the latter half of the 20th century. Advances in the understanding of immunologic and hematologic pathways have shed light on HAE, a disease characterized by painful and unpredictable recurrent attacks of nonpitting edema without urticaria. Recognition that a deficiency of complement component 1 (C1) esterase inhibitor leads to overproduction of vasoactive kinins that cause angioedema paved the way for the development of early treatments. Increased understanding of the role of bradykinin in hereditary and acquired forms of C1 esterase inhibitor deficiency has led to the development of more targeted treatments for this painful, debilitating and potentially life-threatening disease.

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“…Attacks affecting the upper airways, however, can lead to obstruction and suffocation, and the manifestations of gastrointestinal edema (ie, abdominal attacks) can include intractable abdominal pain, vomiting, nausea, diarrhea, and intestinal obstruction, and potentially can lead to hypovolemic shock. [2][3][4]9,26,27 During an attack, the activation of the complement and contact cascades and the inadequate response by C1-esterase inhibitor cause an overproduction of bradykinin (Fig. 2).…”

Section: Clinical Presentationmentioning

confidence: 99%

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower

Busse

Aygören‐Pürsün

et al. 2011

World Allergy Organization Journal

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Abstract:Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-esterase inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.

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Ultrasonography in the diagnosis and monitoring of ascites in acute abdominal attacks of hereditary angioneurotic oedema

Abstract: Transitory ascites demonstrated by abdominal US is a clue to the diagnosis of an acute abdominal attack of HAE. The possibility of HAE should always be considered whenever unexplained abdominal pain recurs with or without ascites.

Cited by 53 publications

References 24 publications

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Hereditary and Acquired Complement Component 1 Esterase Inhibitor Deficiency: A Review for the Hematologist

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

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