Isolated angioedema: A review of classification and update on management.

OBJECTIVE: To review the various types of angioedema including diagnosis and treatment. DATA SOURCES: PubMed search of articles in the English language of various types of angioedema. STUDY SELECTIONS: Articles on the subject matter were selected and reviewed. RESULTS: Herein, a case-based approach is presented for discussing the major types of angioedema, including the following: hereditary angioedema types I and II and normal complement, acquired angioedema, angiotensin-converting enzyme-induced angioedema, and histaminergic and nonhistaminergic angioedema. Emerging treatments of hereditary angioedema including targets of prekallikrein, DNA vector technology replacing C1-INH protein, and CRIPSR technology targeting prekallikrein among many others are explored. In addition, other causes and mimickers of angioedema are briefly reviewed. Finally, a novel algorithm is proposed to help guide the treating physician through the workup and management of patients with suspected idiopathic angioedema unresponsive to conventional therapy with antihistamines. CONCLUSION: Over the years, many strides have been made in both understanding the pathophysiology of various types of angioedema and expansion of treatment options. It is important for clinicians to be aware of current and emerging treatment options. We provide a novel practical algorithm to guide clinicians in challenging cases of idiopathic angioedema refractory to antihistamines.

Cardiovascular diseases in congenital prekallikrein deficiency: comparison with other chance-associated morbidities.

: To compare the prevalence of cardiovascular diseases with other chance-associated morbidities in patients with congenital prekallikrein deficiency. Patients with prekallikrein deficiency were gathered from two time unlimited PubMed searches and from personal files. Inclusion criteria were prekallikrein level less than 15% of normal; correction of aPTT on long incubation times; prolonged aPTT corrected by normal plasma or serum; normal prothrombin time and normal FXII and FXI. Acquired forms were excluded. Out of 106 patients, we have found that 45 patients had at least one chance-associated defect or morbidity at the time of the diagnosis of the clotting defect. Twenty-nine of these 45 patients had cardiovascular disorders. Other comorbidities were found in a much smaller proportion. Congenital prekallikrein deficiency is frequently associated with cardiovascular conditions, namely hypertension, coronary disease, ischemic stroke, venous thrombosis. The significance of these findings is critically discussed as association between two diseases does not necessarily indicate the existence of a causative relation between the two. However, the findings presented here clearly indicate the possibility that such a relation might indeed exist.

Severe prekallikrein deficiency due to a homozygous Trp499Stop nonsense mutation.

Prekallikrein deficiency is a rare autosomal recessive disease not considered to be associated with a tendency for bleeding, despite marked prolongation of activated partial thromboplastin time. Currently, six kinds of mutations in the prekallikrein gene are known to be associated with prekallikrein deficiency. In this report, we describe a patient with idiopathic thrombocytopenic purpura who was recognized to have severe prekallikrein deficiency. Molecular analysis of the patient's prekallikrein gene showed a homozygous Trp499Stop nonsense mutation that has not been reported previously. The mutant allele is predicted to encode a truncated protein lacking half of the catalytic domain of prekallikrein, suggesting that the truncated protein causes prekallikrein deficiency in the patient.

Comportamiento de algunos paramteros determinantes de efectos adversos inducidos por inmunoglobulinas intravenosas / Performance of some determinant parameters of intravenous immnuglobulin-induced adverse effects

Los analisis de laboratorio de las inmunoglobulinas intravenosas ofrecen informacion sobre 3 aspectos fundamentales: seguridad, estabilidad y eficacia. En este estudio se analizan las variables que pueden determinar reacciones adversas, entre las mas importantes se pueden citar los niveles de inmunoglobulina A, inmunoglobulina E, activadores de la precalicreina, agregados de inmunoglobulina G, isoaglutinina y activacion espontanea del complemento. Los bajos niveles de contaminantes estan de acuerdo con el pequeno porcentaje de reacciones secundarias encontradas con su uso clinico

Comportamiento de algunos parámteros determinantes de efectos adversos inducidos por inmunoglobulinas intravenosas / Performance of some determinant parameters of intravenous immnuglobulin-induced adverse effects

Los análisis de laboratorio de las inmunoglobulinas intravenosas ofrecen información sobre 3 aspectos fundamentales: seguridad, estabilidad y eficacia. En este estudio se analizan las variables que pueden determinar reacciones adversas, entre las mas importantes se pueden citar los niveles de inmunoglobulina A, inmunoglobulina E, activadores de la precalicreína, agregados de inmunoglobulina G, isoaglutinina y activación espontánea del complemento. Los bajos niveles de contaminantes están de acuerdo con el pequeño porcentaje de reacciones secundarias encontradas con su uso clínico (AU)