We describe a monogenic defect leading to a specific PC deficiency in human subjects, expanding our knowledge about the pathogenesis of antibody deficiencies.

A new cyclodimerization of ruthenium carbenes and alkynes is reported. The cyclodimerization produces substituted cyclopentadienes and consumes the carbene complex. Cyclodimerization of alkynes is predominant at low alkene concentrations and was found to be competitive with enyne metathesis. The presence of the ortho alkyl ether in the ruthenium carbene complex appears to be a critical determinant for the formation of cyclopentadienes. At higher alkene concentration, the observed cyclodimerization can be suppressed, favoring enyne metathesis.

Certain dienynes give cyclorearrangement by tandem cyclopropanation/ring-closing alkene metathesis, triggered by either a ruthenium carbene or noncarbene ruthenium(II) precatalyst. The process represents a variation of enyne metathesis where presumed cyclopropyl carbene intermediates undergo a consecutive ring-closing metathesis. A mechanistic proposal is offered, and sequential use of catalysts provided a tandem ring-closing enyne/alkene metathesis product.

IntroductionAnaphylaxis is a life threatening systemic inflammatory process that share mediators involved in the coagulation cascade. Platelet activating factor, known to increase platelet aggregation, has also been implicated as an important mediator of anaphylaxis. Although other inflammatory reactions are associated with an increased risk of thrombosis, anaphylaxis is currently not reported as one of them. Furthermore the role platelets may have in the perianaphylaxis period is not well understood. We here in present a retrospective case series of three patients that had platelet aberrations suggestive of PAF involvement and clinically significant thrombosis in close relationship with anaphylaxis.ObjectiveTo investigate platelet response before and after anaphylaxis and indirect observation evidence of platelet activating factors involvement with possible increased risk of thrombosis.MethodsA retrospective investigation into medical records including medication administrations times, laboratory, and radiology results. Platelet levels pre‐ and post‐ anaphylaxis were statistically analyzed.ResultsCase 1, a 44 year old man had an anaphylactic reaction shortly after envenomation and subsequently suffered an acute infarction with thrombus in a cerebral artery. Case 2 is a 49 year old man with idiopathic anaphylaxis who developed a deep vein thrombosis after a protracted anaphylaxis event. Case 3 involved an 18 year old female with acute myeloid leukemia was found to have a thrombus in the celiac trunk following anaphylaxis. A paired two‐tailed Wilcoxon test on the subjects pre and post anaphylactic platelet levels resulted in a overall P < 0.0001.Conclusions and Clinical RelevanceThese three cases illustrate the potential role platelets may have in anaphylaxis and possible increased secondary risk for the development of thrombosis. Larger studies are required to determine incidence and risk factors for blood clots following anaphylaxis in order to provide management or screening recommendations.

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New benzimidazolidinone cyclophanes were synthesized through a double Stevens rearrangement employed as a ring expansion technique. Para-substituted heterophanes underwent efficient rearrangement. Meta-substituted heterophanes were also prepared. Structure analyses of the new cyclophanes are also provided. [reaction: see text]

Objective: To evaluate the outcome of immunoglobulin (IG) replacement therapy in adults with rhinosinusitis and primary humoral immunodeficiency disorders (PID).Study Design: Retrospective cohort study. Methods: Retrospective chart review of adult (18 years and older) patients who were diagnosed with PID and had the diagnosis of recurrent acute rhinosinusitis (RARS) and chronic rhinosinusitis (CRS) and who are on IG replacement therapy. Demographic data, associated conditions, and duration of treatment were reviewed. Number of yearly sinus infections, sinus CT Lund-Mackay (LM) score, and need for functional endoscopic sinus surgery (FESS) were reviewed before and after starting IG therapy.Results: Fifty-eight patients were included. Average age was 52 years (18-79 years). About 74% were female. Thirty patients (51.7%) had common variable immunodeficiency (CVID), 18 (31.1%) had hypogammaglobulinemia, and 10 (17.2%) had specific antibody deficiency (SAD). About 79% of patients had allergic rhinitis and 74% had asthma. Pretreatment LM score was 7.6 (AE2.2, range of 0-24) compared to posttreatment score of 3.5 (AE1.3, range of 0-16) (P = .01). Eleven patients (19%) had FESS pretreatment compared to only two patients (3.4%) requiring ESS after starting treatment on IG therapy (P = .004). Prior to starting IG therapy, five patients (8.6%) had more than 10 sinus infections per year, 33 patients (56.9%) had 5 to 10 infections, and 20 patients (34.5%) had 1 to 5 infections per year. After starting on IG, 34 patients (58.6%) had no infections reported anymore (P < .001), and 24 patients (41.4%) had only 1 to 5 infections reported per year Conclusion: IG therapy is a promising treatment option for recurrent rhinosinusitis in patients with PID.

The methylene-free ring synthesis of 1,3-cyclohexadienes has been expanded to include a diverse set of functional group-rich terminal and internal alkynes. [reaction: see text]