We describe here a three year-old girl with classic clinical and histological features of juvenile hyaline fibromatosis. We found a history of similar skin findings in her eldest sister, in whom the disorder took a rapidly progressive and fatal course in the second year of life, suggesting either a very severe form of juvenile hyaline fibromatosis, or the possibility of infantile systemic hyalinosis. The similarities and differences between these two described types of hyalinoses have been reviewed in reference to the present report.

No abstract

BackgroundMenACYW-TT is an investigational quadrivalent meningococcal conjugate vaccine that contains tetanus toxoid as carrier protein. The vaccine is intended for global use in individuals 6 weeks of age and older. We evaluated the safety and immunogenicity of MenACYW-TT compared with a licensed quadrivalent conjugate meningococcal vaccine (MenACWY-CRM [Menveo®]) in US children 2–9 years of age.MethodsIn a modified double-blind Phase III study (NCT03077438), 1000 children were randomized to receive one dose of either MenACYW-TT vaccine or MenACWY-CRM vaccine. Serum bactericidal assays with human (hSBA) and baby rabbit (rSBA) complement were used to measure antibodies against representative meningococcal serogroup strains at baseline and 30 days after vaccination. Safety data were collected up to 6 months post-vaccination.ResultsNon-inferiority of immune responses for all four serogroups, based on percentages of participants achieving hSBA vaccine seroresponse, was demonstrated for MenACYW-TT compared with MenACWY-CRM at Day 30 compared with baseline. The proportions of individuals with hSBA titers ≥ 1:8 following MenACYW-TT administration were higher than those after MenACWY-CRM administration for all four serogroups (A: 86.4% vs 79.3%; C: 97.8% vs 67.1%; W: 94.8% vs 86.3%; Y: 98.5% vs 90.8%). Similar results were observed in two age substrata (2 to 5 years and 6 to 9 years). Percentages of participants with post-vaccination rSBA titers ≥ 1:128 were comparable between both groups. The safety profiles of MenACYW-TT and MenACWY-CRM were comparable. Reactogenicity at the MenACYW-TT injection site was lower than at the MenACWY-CRM injection site. There were no immediate adverse events (AEs), no AEs leading to study discontinuation, and no vaccine-related serious adverse events reported in the study.ConclusionMenACYW-TT vaccine was well tolerated and demonstrated a non-inferior immune response compared with that for the licensed MenACWY-CRM vaccine when administered as a single dose to meningococcal vaccine-naïve children.Disclosures All authors: No reported disclosures.

BackgroundThe MenACYW-TT conjugate vaccine is a quadrivalent meningococcal vaccine that contains tetanus toxoid as carrier protein. The vaccine is intended for global use in all age groups (i.e., individuals 6 weeks of age and older). This Phase III study evaluated the immune lot consistency, and safety and immunogenicity of the vaccine when compared with a licensed quadrivalent meningococcal conjugate vaccine in individuals 10–55 years of age.MethodsA randomized, modified double-blind, multi-center study (NCT02842853) was conducted in the United States. The study evaluated 3344 meningococcal vaccine naïve adolescents and adults, who were randomly assigned to receive either a single dose of one of the three lots of MenACYW-TT conjugate vaccine or single dose of Menactra® [MenACWY-D]. Serum bactericidal assay with human complement (hSBA) and baby rabbit complement (rSBA) was used to measure antibodies against serogroups A, C, W and Y at baseline before vaccination (Day 0) and 30 days post-vaccination. Safety data were collected up to 6 months post-vaccination. Herein we report the performance of MenACYW-TT in adolescents 10 through 17 years of age (n = 1504).ResultsImmune equivalence was demonstrated across all 3 lots of MenACYW-TT conjugate vaccine based on geometric mean titers (GMTs) for all serogroups. Non-inferiority of immune responses, based on percentages of participants achieving hSBA vaccine seroresponse, was demonstrated between MenACYW-TT and MenACWY-D for all four serogroups at Day 30 compared with baseline. The proportions of individuals (10–17 years) with hSBA ≥ 1:8 following MenACYW-TT administration were higher than those after MenACWY-D administration for all four serogroups (A: 96.2% vs. 89.0%; C: 98.5% vs. 74.7%; W: 98.3% vs. 93.7%; Y: 99.1% vs. 94.3%). A similar trend was observed for post vaccination GMTs in adolescent participants. Reactogenicity profiles were comparable across study groups. Most unsolicited adverse events were of grade 1 or grade 2 intensity. No vaccine-related serious adverse events were reported.ConclusionMenACYW-TT vaccine was well tolerated and demonstrated a non-inferior immune response compared with the licensed MenACWY-D vaccine when administered as a single dose to meningococcal vaccine naïve adolescents.Disclosures All authors: No reported disclosures.

A man in his 40s presented with a 2-year history of a few small painful nodules on both ears. The nodules were painful on touch, especially while the patient was sleeping in a lateral position. There was no history of trauma. However, he used to wear a cap, which created pressure and friction on the upper part of both ears. On examination, the free border of the helix of both the ears showed a few dull red, dome-shaped, firm nodules 2 to 3 mm in size with central crusting (Figure, A). They were tender on palpation. No cervical lymphadenopathy was observed. One of the nodules on the right ear was excised; histopathologic features of the nodule are shown in the Figure, B. It showed a sharply defined, centrally depressed ulcer covered by a hyperkeratotic parakeratotic scale, occasional bacterial colonies, and plasma. The adjacent epidermis showed hyperplasia. The base of ulcer showed eosinophilic degeneration of collagen and solar elastosis. In addition, there were a few proliferating blood vessels and mild lymphomononuclear infiltrate. Underlying cartilage was not seen in the section. On serial sectioning, cartilage was seen and found to be normal.A B

Background Booster doses of meningococcal conjugate vaccines may induce long-term protection against invasive meningococcal disease. MenACYW-TT [MenQuadfi®] is a quadrivalent meningococcal conjugate vaccine, licensed for use in ages 2 years and older in USA. The vaccine is also licensed in ages 12 months and older in EU and other countries. Methods A phase IIIb study (NCT04084769) was conducted to evaluate the persistence of immune response in adults and adolescents primed 3-6 years earlier with either MenACYW-TT or MCV4-CRM (Menveo®) and, safety and immunogenicity of MenACYW-TT when administered as a booster dose with or without concomitant administration with MenB vaccines (Bexsero® and Trumenba®). Serum bactericidal assays with human complement (hSBA) and baby rabbit complement (rSBA) were used to measure antibodies against vaccine serogroups at baseline (Day 0 [D0]), D06 (in a subset) and 30 days post-vaccination (D30). Safety data were collected up to 6 months post-vaccination. Results At D0, the GMTs were higher in subjects primed with MenACYW-TT vs MCV4-CRM for serogroups C, Y and W, and were comparable for serogroup A. At D0, all hSBA GMTs were higher than those observed pre-priming dose, suggesting persistence of immunity. Sufficiency of hSBA seroresponse ( >75%) was demonstrated following administration of MenACYW-TT booster dose regardless of the priming vaccine administered 3-6 years earlier. Vaccine seroresponse in a subset of participants at D06 ranged from 77.8% (95%CI 62.9%; 88.8%) for serogroup A to 97.8% (88.5%; 99.9%) for serogroup W suggesting a quick onset of immune response post-booster. Post-vaccination (D30) hSBA GMTs were comparable for serogroups A, Y and W regardless of the nature of the priming vaccine and were higher for serogroup C in subjects primed with MenACYW-TT vaccine. The MenACYW-TT booster dose was well-tolerated and had similar safety profiles regardless of the priming vaccine. The safety profiles were comparable regardless of the MenB vaccine co-administered with MenACYW-TT vaccine. Conclusion MenACYW-TT used as priming vaccine was able to demonstrate persistence of immune response 3-6 years later. MenACYW-TT elicits robust booster responses in adults and adolescents primed with MenACYW-TT or MCV4-CRM Disclosures Betzana Zambrano, MD, Sanofi Pasteur (Employee) Germán Áñez, MD, Sanofi Pasteur (Other Financial or Material Support, Former employee) Sue Jiayuan, MSc, Sanofi Pasteur (Independent Contractor) Judy Pan, PhD, Sanofi Pasteur (Employee) Habiba Arroum, MD, Sanofi Pasteur (Employee) Kucku Varghese, PhD, Sanofi Pasteur (Employee) Emilia Jordanov, MD, Sanofi Pasteur (Employee, Shareholder) Mandeep S. Dhingra, MD, Sanofi Pasteur (Employee, Shareholder)

Background & objectives: Periodontitis is a destructive inflammatory disease of the supporting tissues of the teeth that occurs in response to a predominantly Gram-negative bacterial infection originating from dental plaque. Periodontitis presents with increased systemic inflammation and is known to contribute to rise in serum lipid levels. The aim of present study was to determine influence of periodontal therapy on serum lipid levels. Methods: A total of 105 consecutive subjects were studied. Group I (n=35) included subjects with chronic generalised periodontitis who were not given periodontal therapy during study period and served as control group. Group II (n=70) comprised of subjects with chronic generalized periodontitis who were rendered needful periodontal therapy. Serum levels of triglycerides, total cholesterol, HDL & LDL cholesterol were measured at day 0 (baseline) and reassessed on day 60. Results: In the treatment group, serum triglycerides (Pre=127.81±59.32 & Post = 121.20 ± 58.94 mg/dL, p < 0.001)), total cholesterol (Pre=176.33±38.31 & Post=171.39±31.19 mg/dL, p=0.045) and mean LDLcholesterol levels (Pre=91.91±28.54 & Post=83.94±26.00 mg/dL, p < 0.001) showed a significant decline from the pre-treatment values. HDL-cholesterol levels did not change significantly in both groups. Other lipid levels were not significantly altered in the control group. Conclusions: Patients of chronic generalised periodontitis who were offered periodontal therapy showed improvement in the various lipid parameters except HDL-cholesterol, which was not significantly altered. Chronic periodontitis in otherwise healthy individuals may therefore, be contributing to the systemic inflammatory burden in these patients and adversely altering the lipid profile.

No abstract