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A recently developed preparation of C1 inhibitor concentrate was evaluated in patients with hereditary angioedema in two trials. In the acute-attack treatment trial, the time to relief of an acute attack of angioedema was significantly shorter with the C1 inhibitor than with placebo. In the prophylaxis trial, the attack rate over a 12-week period was significantly lower with the C1 inhibitor than with placebo. Hereditary angioedema due to C1 inhibitor deficiency is an autosomal dominant disorder characterized by recurrent episodes of angioedema that typically involve the extremities, abdomen, external genitalia, face, or oropharynx. 1 Abdominal attacks of angioedema, which are caused by local mucosal swelling, are often associated with severe abdominal pain, nausea, and vomiting. Such attacks frequently lead to hospitalization and occasionally to unnecessary exploratory surgery. 2 Laryngeal attacks are associated with a substantial risk of death. 2 Two forms of hereditary angioedema have been defined: type I (accounting for 85% of cases) is characterized by low antigenic and functional levels of C1 inhibitor, whereas type . . .

Cystic fibrosis (CF) is a disease which predisposes individuals to recurrent infective exacerbations of suppurative lung disease; however, empyema is a rare complication in these patients. Empyemas secondary to Staphylococcus aureus and Burkholderia cepacia have been described in patients with CF. We report the case of pleural empyema with mixed S. aureus and Pseudomonas aeruginosa infection in a 34-year-old woman with CF, which was managed with ultrasound-guided pigtail catheter insertion, fibrinolysis, and antibiotic therapy. Physicians should be aware of this unusual complication in CF patients, especially those receiving an immunosuppressive therapy.

Aspirin is one of the world’s most commonly used medications and its use benefits many diverse conditions. Adverse reactions, however, are relatively common as well. Hypersensitivity to aspirin can be manifested as acute asthma, urticaria and/or angioedema, or a systemic anaphylactoid reaction. We report 3 cases in whom aspirin was indicated for secondary prophylaxis of myocardial infarction but in whom a remote history of an untoward reaction to it prevented its initial use. These patients all underwent further evaluation of their pulmonary and allergic history and all 3 were challenged with aspirin. Two patients were found not to be sensitive and started on aspirin, the other had a classic asthmatic reaction to the drug and was successfully desensitized to aspirin allowing for its use.

Paraneoplastic pemphigus (PNP) is an autoimmune disease associated with leukemia and non-Hodgkin's lymphoma. A patient with stage IVB poorly differentiated lymphocytic lymphoma developed characteristic upper and lower airway involvement with profound mucocutaneous erosion and tracheobronchial epithelial desquamation. Immunofluorescence testing confirmed autoantibody deposition along the basement membrane of bronchial epithelium. Disruption of the cellular adhesion mechanisms, including desmosomes, hemidesmosomes, and possibly the integrin subunits, is presumed to have led to disruption and desquamation of the tracheobronchial epithelial barrier, severe obstruction of the airways and hypoxia, and possibly bacterial superinfection. As far as can be determined, the feature of airflow obstruction occurring in association with PNP has not been described. Physicians should be aware that these complications of PNP may rapidly lead to hypoxic respiratory failure and death.

Paraneoplastic pemphigus (PNP) is an autoimmune disease associated with leukemia and non-Hodgkin's lymphoma. A patient with stage IVB poorly differentiated lymphocytic lymphoma developed characteristic upper and lower airway involvement with profound mucocutaneous erosion and tracheobronchial epithelial desquamation. Immunofluorescence testing confirmed autoantibody deposition along the basement membrane of bronchial epithelium. Disruption of the cellular adhesion mechanisms, including desmosomes, hemidesmosomes, and possibly the integrin subunits, is presumed to have led to disruption and desquamation of the tracheobronchial epithelial barrier, severe obstruction of the airways and hypoxia, and possibly bacterial superinfection. As far as can be determined, the feature of airflow obstruction occurring in association with PNP has not been described. Physicians should be aware that these complications of PNP may rapidly lead to hypoxic respiratory failure and death.

About 2 million Americans are allergic to the venom of stinging insects, and many of these individuals are at risk for life-threatening allergic reactions. About 50 deaths each year in the U.S. are attributed to allergic reactions to insect stings, though experts believe that this may be an underestimate. An allergic reaction occurs when the immune system overreacts to an allergen. In stinging insect allergy, the allergen is venom from a sting. Before considering an allergic reaction, remember that a normal sting reaction will result in pain, swelling and redness confined to the sting site. Wash the sting area with soap and water. An analgesic such as acetaminophen or ibuprofen may be useful. If stung on the hand, remove any rings from your fingers immediately in case swelling occurs. For those experiencing a severe sting reaction, medical attention is required immediately as fatal reactions have occurred in as quickly as 10 minutes. Dial 911. If you have suffered such a reaction you should consult with an allergist. You may be a candidate for allergy shots (venom immunotherapy) which are a highly effective way for you to be desensitized to the sting reaction. You should always carry self-injectable epinephrine (EpiPen) and MedicAlert identification.

Abstract The goal of this study was to characterize the receptors and coupling mechanisms mediating muscarinic inhibition of adenylyl cyclase activity in bovine tracheal smooth muscle. In radioligand binding experiments, methoctramine and AF-DX 116 competed for approximately 85% of the 3H-quinuclidynyl benzilate (3H-QNB) binding sites on intact cells with high affinities (-log KI of 7.73 +/- 0.16 and 6.67 +/- 0.31, respectively) characteristic of binding to M2 receptors. The antagonist 4-diphenylacetoxy-N-methylpiperidine (4-DAMP) bound the receptors on intact cells with an affinity (-log KI = 7.76 +/- 0.21) characteristic of binding at M2 receptors. In experiments measuring 3′,5′-cyclic adenosine monophosphate (cAMP) accumulation, methoctramine, AF-DX 116, and 4-DAMP antagonized the inhibitory effect of carbachol on isoproterenol-stimulated cAMP accumulation with potencies consistent with mediation by M2 muscarinic receptors (-log Kb of 8.01 +/- 0.22 to 7.58 +/- 0.25 for methoctramine; 7.43 +/- 0.36 to 7.02 +/- 0.30 for AF-DX 116; and 7.60 +/- 0.21 for 4-DAMP). In other experiments, 24 +/- 3% of the inhibitory effect of carbachol was not reversed by 60 min exposure to atropine. Moreover, pertussis toxin (10, 250, and 1,000 ng/ml) decreased only a portion of the inhibitory effect of carbachol (8 +/- 19%, 32 +/- 10%, and 33 +/- 8%, respectively) on cAMP accumulation. These findings indicated that M2 receptors were coupled to adenylyl cyclase in trachealis cells, but that coupling mechanisms in addition to those of pertussis toxin-sensitive guanine nucleotide binding proteins were involved. Since the inhibitory effect of carbachol (10(-8) M) on isoproterenol-stimulated cAMP accumulation was decreased from 20 +/- 4% to -1 +/- 5% (n = 6) by okadaic acid (1 microM), protein phosphatases may regulate the processes coupling muscarinic receptors to adenylyl cyclase.