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Hyper-immunoglobulin M syndrome is an X-linked primary immunodeficiency disease caused by mutations in the CD40 ligand gene. The CD40 ligand has been recently highlighted as playing a key role in the pathogenesis of primary biliary cholangitis. In the present study, we assessed an extensive set of serum autoantibodies in a series of well-defined patients with hyper-immunoglobulin M syndrome. Serum, liver-related and liver-not-related autoantibodies IgG, IgM and IgA were tested by ELISA and standard indirect immunofluorescence in HEp-2 cells in 13 Tunisian patients (8 males and 5 females, aged 1–12 years) with hyper-immunoglobulin M syndrome during 1995–2012 and, as controls, 21 age- and gender-matched blood donors. The level of IgM antibody against MIT3 was significantly higher in patients than in controls (35.8 vs 10.7, P=0.002). Half of the hyperimmunoglobulin M syndrome patients were found to be anti-MIT3 IgM positive vs none of the controls (P<0.0001). Twenty-three percent of patients were found to be anti-sp100 antibody positive vs only 0.05% of controls. By immunofluorescence, 92.3% of patients were MIT3 IgM positive vs none of the controls. In conclusion, the IgM class of anti-MIT3 antibodies was shown to be present by both ELISA and immunofluorescence in most of the patients with hyper-immunoglobulin M syndrome. The presence of the hallmark of primary biliary cholangitis, a disease where the CD40 ligand is a key player, in an immunodeficiency disease caused by mutations in the CD40 ligand gene is very intriguing and opens new scenarios in understanding the immune pathogenesis of primary biliary cholangitis.

Mehdi Trifa,1,2 Dmitry Tumin,1,3 Hina Walia,1 Kathleen L Lemanek,4 Joseph D Tobias,1,3 Tarun Bhalla1,3 1Department of Anesthesiology and Pain Medicine, Nationwide Children’s Hospital, Columbus, OH, USA; 2Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia; 3Department of Anesthesiology and Pain Medicine, The Ohio State University College of Medicine, Columbus, OH, USA; 4Department of Pediatric Psychology and Neuropsychology, Nationwide Children’s Hospital, Columbus, OH, USA Background: The use of complementary and alternative medicine (CAM) therapies has increased in children, especially in those with chronic health conditions. However, this increase may not translate into acceptance of CAM in the perioperative setting. We surveyed caregivers of patients undergoing surgery to determine their knowledge and acceptance of hypnotherapy, acupuncture, and music therapy as alternatives to standard medication in the perioperative period. Materials and methods: An anonymous, 12-question survey was administered to caregivers of children undergoing procedures under general anesthesia. Caregivers reported their knowledge about hypnotherapy, music therapy, and acupuncture and interest in one of these methods during the perioperative period. CAM acceptance was defined as interest in one or more CAM methods.Results: Data from 164 caregivers were analyzed. The majority of caregivers were 20–40 years of age (68%) and mothers of the patient (82%). Caregivers were most familiar with acupuncture (70%), followed by music therapy (60%) and hypnotherapy (38%). Overall CAM acceptance was 51%. The acceptance of specific CAM modalities was highest for music therapy (50%), followed by hypnotherapy (17%) and acupuncture (13%). In multivariable logistic regression, familiarity with music therapy was associated with greater odds of CAM acceptance (odds ratio=3.36; 95% CI: 1.46, 7.74; P=0.004).Conclusion: Overall CAM acceptance among caregivers of child