The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency.

The majority of angioedema cases encountered in clinical practice are histamine-mediated (allergic); however, some cases are bradykinin-related (non-allergic) and do not respond to standard anti-allergy medications. Among bradykinin-related angioedema, hereditary angioedema (HAE) is a rare, but chronic and debilitating condition. The majority of HAE is caused by deficiency (type 1) or abnormal function (type 2) of the naturally occurring protein, C1-inhibitor (C1-INH)-a major inhibitor of proteases in the contact (kallikrein-bradykinin cascade), fibrinolytic pathway, and complement systems. Failure to recognize HAE and initiate appropriate intervention can lead to years of pain, disability, impaired quality of life (QoL) and, in cases of laryngeal involvement, it can be life-threatening. HAE must be considered in the differential diagnosis of non-urticarial angioedema, particularly for patients with a history of recurrent angioedema attacks, family history of HAE, symptom onset in childhood/adolescence, prodromal signs/symptoms before swellings, recurrent/painful abdominal symptoms, and upper airway edema. Management strategies for HAE include on-demand treatment for acute attacks, short-term prophylaxis prior to attack-triggering events/procedures, and long-term or routine prophylaxis for attack prevention. Patients should be evaluated at least annually to assess need for routine prophylaxis. HAE specific medications like plasma-derived and recombinant C1-INH products, kallikrein inhibitors, and bradykinin B2 receptor antagonists, have improved management of HAE. While the introduction of intravenous C1-INH represented a major breakthrough in routine HAE prophylaxis, some patients fail to achieve adequate control and others have psychological barriers or experience complications related to intravenous administration. Subcutaneous (SC) C1-INH, SC monoclonal antibody (mAb)-based therapies, and an oral kallikrein inhibitor offer effective alternatives for HAE attack prevention and may facilitate self-administration. HAE management should be individualized, with QoL improvement being a key goal. This can be achieved with broader availability of existing options for routine prophylaxis, including greater global availability of C1-INH(SC), mAb-based therapy, oral treatments, and multiple on-demand therapies.

Trends in asthma prevalence, hospitalization risk, and inhaled corticosteroid use among alaska native and nonnative medicaid recipients younger than 20 years.

BACKGROUND: Few trend data on asthma prevalence exist for U.S. indigenous populations, and none exist for Alaska Natives. OBJECTIVE: To document the epidemiologic features of asthma in Alaska Natives and nonnatives stratified by urban (Anchorage) and rural (non-Anchorage) residence. METHODS: We conducted a retrospective review of Alaskans younger than 20 years enrolled in Medicaid during 1999 to 2002. Asthma was defined as a claim for International Classification of Diseases, Ninth Revision, codes 493.0x to 493.9x plus asthma-associated medication during the same calendar year. RESULTS: Among 117,080 Medicaid enrollees, the 4-year asthma prevalence was 3.1% and was 40% to 90% greater for urban residents regardless of race. Yearly prevalence increased from 1.0% to 2.2% (P < .001), with increases in all subgroups. Of 4 predominantly Alaska Native census areas, the area with resident pediatricians and previous participation in asthma research had a 4-year asthma prevalence 5- to 11-fold higher than the other areas. Among persons with asthma, yearly hospitalization risk decreased (from 9.3% to 6.8%; P = .02) concurrent with an increase in the yearly use of inhaled corticosteroids (from 50% to 64%; P < .001). Urban Alaska Natives had the greatest decrease in hospitalization risk and the greatest increase in inhaled corticosteroid use. CONCLUSIONS: Relatively dramatic demographic differences and temporal trends in asthma prevalence occurred in the absence of known differences or changes in risk factor prevalences. This suggests a role for differences in the use of asthma as a diagnosis for respiratory illness. Failure to diagnose and thus treat asthma may affect outcomes because decreases in hospitalization risk were temporally associated with increases in inhaled corticosteroid use.