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In a randomized trial, 72 patients with hereditary angioedema presenting with acute attacks of angioedema were randomly assigned to receive either ecallantide, a recombinant plasma kallikrein inhibitor, or placebo. The median treatment outcome score, a composite patient-reported outcome measure, was significantly better at 4 hours in the ecallantide group than in the placebo group. Hereditary angioedema is an autosomal dominant disorder with an estimated prevalence ranging from 1 case in 10,000 to 1 case in 50,000 persons. 1 – 3 Patients with hereditary angioedema have intermittent acute attacks of edema involving the larynx, oropharynx, face, gastrointestinal mucosa, extremities, or genitalia. 4 Attacks occur unpredictably and may persist for 2 to 5 days. 5 , 6 Gastrointestinal attacks can cause incapacitating colic, vomiting, and diarrhea and can result in unnecessary abdominal surgery. Laryngeal attacks may be life-threatening because of the potential for airway obstruction. 5 , 7 Attacks do not typically respond to antihistamines, corticosteroids, or epinephrine. Hereditary angioedema is caused by . . .

Two randomized trials evaluated the effect of the bradykinin-receptor antagonist icatibant in patients with hereditary angioedema presenting with acute attacks. The primary end point in each trial was the median time to clinically significant relief of symptoms. In one trial, the primary end point was reached significantly faster with icatibant than with tranexamic acid. In the other trial, the primary end point was not reached significantly faster with icatibant than with placebo. Hereditary angioedema is an autosomal dominant disorder caused by a deficiency of C1 esterase inhibitor, which has a regulatory role in the classic complement pathway and in the coagulation, fibrinolytic, and kallikrein–kinin (contact-system) cascades. Reduced activity of C1 esterase inhibitor may result in an elevated plasma level of bradykinin, 1 , 2 the key mediator of symptoms in hereditary angioedema. 3 , 4 Patients with hereditary angioedema present with acute attacks of subcutaneous and submucosal edema that can affect the upper airways, face, extremities, genitals, and gastrointestinal tract. 5 – 7 Pharyngolaryngeal edema, which is potentially life-threatening because of the risk of upper-airway obstruction, can also . . .

Introduction Hereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency, resulting in periodic attacks of acute edema that can be life-threatening if they occur in the laryngeal region. We assessed the efficacy of C1-INH concentrate in the emergency treatment of rarely occurring acute laryngeal HAE attacks in a prospective, open-label clinical study. Methods Acute laryngeal attacks were each treated with C1-INH concentrate (Berinert) at a single dose of 20 U/kg body weight. Efficacy endpoints included time to onset of symptom relief and time to complete resolution of all symptoms, each based on the patient's assessment. Results All 39 laryngeal attacks in 16 patients were treated successfully. The median time to onset of symptom relief was 15 min. The median time to complete resolution of all symptoms was 8.25 h. No treatment-related serious adverse events occurred, and the treatment was well tolerated. The administration of C1-INH concentrate was not associated with any viral infections. Conclusion C1-INH concentrate is an effective and safe emergency treatment for providing reliable and rapid relief from the potentially life-threatening symptoms of laryngeal HAE attacks.

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 . . .

Time to onset of symptom relief in hereditary angioedema (HAE) is a common primary end point in clinical studies but it has never been validated by correlation with the course of HAE symptoms. This study was designed as a retrospective validation of the primary end point for a placebo-controlled phase II/III study in patients with HAE. Ninety-eight abdominal attacks were treated with 10 or 20 U/kg of a highly purified C1 esterase inhibitor (C1-INH) concentrate or placebo. The primary end point was the time to onset of symptom relief, as determined by the patients. Patients assessed the intensity of the symptoms of pain, nausea, vomiting, cramps, and diarrhea over time. By Spearman rank correlation, the primary end point was compared with the time to first reduction of (1) any symptom intensity, (2) the sum of symptom intensity scores, and (3) the intensity of the last symptom present at baseline. The C1-INH, 20 U/kg, and placebo groups were compared by one-sided two-sample Wilcoxon tests. The time to first reduction in intensity of the last symptom present at baseline had the highest correlation with the primary end point (r = 0.77). The time to onset of symptom relief and the time to the first reduction in intensity of the last symptom were significantly shorter for the C1-INH, 20 U/kg, group compared with placebo (p = 0.009 and p = 0.0036, respectively). The association with the intensity of single symptoms confirmed that the time to onset of symptom relief is an appropriate end point for assessing the efficacy of C1-INH therapy.

Purpose Immune globulins for IgG supplementation have been produced for over 35 years with essentially no differentiating features regarding their specific antibody composition. Furthermore, the compositions of plasma donor pools used for IG manufacturing are not standardized. While all immune globulin products meet the specifications set by the US FDA for antibodies to pathogens like measles and polio, they have variable levels of antibodies to other important viruses and infectious pathogens, particularly respiratory syncytial virus (RSV). Methods An IVIG was developed that satisfies the requirements for treating patients with primary immune deficiency disease (PIDD) and also has standardized elevated levels of RSV neutralizing antibodies (RI-002). Plasma donors who have naturally occurring high circulating levels of neutralizing anti-RSV antibody were selected as the source for manufacturing IVIG to treat patients with PIDD to prevent serious bacterial infections. While the introduction of the monoclonal antibody Palivizumab has had a dramatic impact in diminishing the burden of RSV disease in the pediatric population, it does not meet the standards for replacing the deficient immune compartments of patients with PIDD. Results Fifty-nine patients with PIDD at 9 different sites across the US were enrolled in this study and received regular infusions of RI-002 over the course of 1 year. Conclusions There were zero serious bacterial infections, thus meeting the primary endpoint for this trial. The secondary endpoints including days missed from work due to infection, unscheduled visits to the physician, and days of hospitalization due to infection compared favorably to published reports of other IVIG products.

Gastroesophageal reflux disease (GERD) is a major problem in primary care. More than 25% of the population experience GERD symptoms, and nonerosive forms of the disease are common. Conservative management is recommended. This review and case study presents conservative treatment options from the updated guidelines for the adult patient with GERD.

Take Heather Scoular. She is the bubbly branch head of the Kitsilano branch of the Vancouver Public Library who entertained me with lurid tales from the circulation desk. Besides the usual squares of toilet paper, shopping lists and bus transfers, staff at Kitsilano have discovered personal diaries, unrequited love letters, a squashed peach, a baggie of pot, unused pantyliners and the ubiquitous slice of uncooked bacon. When I asked what was the most memorable bookmark ever left behind, Scoular burst into throaty laughter and admitted, \"It was a little bit more than I care to know about our patrons\" -- some nude underwater pictures. She explained that her branch usually posts forgotten photos on a little board for patrons to view, but in this case, discretion prevailed. The skinny dipper's name was traced in the computer, a note was tagged to his file, and his birthday-suit snapshots joined the car keys, Pokemon cards and more mundane items in lost and found. Librarians at VPL's Central branch get close to 6,300 visitors a day, so perhaps it's not surprising that they have found everything from birth certificates to tax returns, in addition to their share of risque photos. One candid set of Kodak moments, starring a male patron and his Full Monty crew of capsized boaters, landed in the lap of Alex Campbell. She is a soft-spoken, tenacious librarian who managed to ply a reluctant confession from the bashful bookworm over the telephone. Initially, the patron denied ownership but caved in when Campbell confided that \"they were from the camping trip -- and that they'd leave his photos in a plain brown envelope at the desk for him to pick up.\" For some library patrons, cash still rules, even when it comes to marking pages. Fortunately, Campbell and her colleague, Susan Everall, do what every Good Samaritan librarian does: try to give that currency back. They once tracked down a big tipper who accidentally left an impressive thousand dollar bill in a book. Burnaby librarian Linnea Gibbs recalls an incident five years ago when staff discovered $800 US stuffed between the pages of Harlequins donated by some anonymous romantic. Finding this donor proved impossible, so the money was put into the library's coffers. Based on the testimonials of hundreds of other librarians across North America, few readers are quite that generous. Five, 10 and 20- dollar bills are much more commonplace, though occasionally 50s and 100s do turn up.

[Vanny] says that as a child she was sick with a cold \"and that slick stuff slid down my throat as sublimely as the cool strokes of my mother's hand on my feverish forehead slid down into my soul.\" Obviously, her mom did not raise a Jell-O connoisseur, because she divulges, \"the flavour doesn't matter [as long as it's red] and it must be buried in whipped Pet Milk. Not whipped cream, not Cool- Whip, not any of those squirt-from-a-can mutations.\" Vanny grieves her mother's death, but says she still sees \"her beautiful face every time I see Bill Cosby hustling Jell-O.\" Pass the Pet Milk, please. The one and only time she prepared Pasta with Cocks Combs Sauce was enough. Based on a 16th century Catherine d'Medici recipe, this eccentric dish calls for cocks combs, unborn eggs, chicken livers and ground veal. Like [Umberto Menghi], [Karen Barnaby] also caters to chocolate aficionados and includes herself in the majority of women who crave sweets. In an attempt to eschew sugar, Barnaby sometimes slurps Splenda-sweetened Jell-O instead of shortcakes or puddings. Color Photo: Peter Battistoni, Vancouver Sun / Comfort Food ; Color Photo: Peter Battistoni, Vancouver Sun / Comfort Food ; Color Photo: Peter Battistoni, Vancouver Sun / Kraft Dinner ; Color Photo: Peter Battistoni, Vancouver Sun / Pancakes ;

The next day, as I entered the alley, the bird and I did a repeat performance. This time, I fended it off with a stick, to the amusement of several on-lookers, both human and crow. Humiliated, I vowed to find out why the crows were targeting me. Mobbing behaviour (intimidation, chasing, dive-bombing, and attacking) occurs in breeding season, March to July. Parent crows and their helpers (called \"nanny crows\" and believed to be the returning male yearlings from last year's nest) target potential predators that may threaten babies or \"simps.\" A crow, whose nest was between her house and Balaclava Park, dive- bombed her, dug its claws into her scalp, and drew blood. Hysterical, Konnert managed to pull it off and run to her car. She didn't require stitches or rabies shots, but she did take two days off work to recover from headaches.