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BACKGROUND: About half of all children with rheumatic diseases need continuous medical care during adolescence and adulthood. A good transition into adult rheumatology is essential. Guidelines for a structured transition process have therefore been recommended by the European League Against Rheumatism (EULAR) and the Paediatric Rheumatology European Society (PReS). However, implementation of these guidelines requires resources often not available in a busy clinical practice. AIMS: To assess the current practice of transitional care in Switzerland in relation to EULAR/PReS recommendations and to describe gaps and challenges in following the recommendations. METHODS: All paediatric Swiss rheumatology centres and their collaborating adult centres offering a transition service to adult care were invited to participate in this survey. The responsible paediatric and adult rheumatologist of each centre was interviewed separately using a structured manual addressing the EULAR/PReS transitional care recommendations. RESULTS: All 10 paediatric and 9 out of 10 adult rheumatologists agreed to participate. Centres varied in the number of patients in transition, from n = 0 to n = 111. The following EULAR/PReS recommendations were implemented and applied in most centres: continuity in the healthcare team, consultations focused on adolescents and young adults, joint consultations between the paediatric and adult rheumatologist, and access to the EULAR website. Only rarely did a centre have a written transition policy or evaluate their transitional care programme. The vast majority of the interviewees had no specific training in adolescent health. Most centres rated their transitional care performance as very good. CONCLUSION: Transition in Switzerland is not uniform and consequently the implementation of the EULAR/PReS recommendations is variable in Swiss rheumatology centres. Skills of healthcare professionals, continuity between clinical settings, size of the centres, and hospital focus on the needs of adolescents and young adults may represent key predictors of successful transitional care for patients with chronic rheumatic diseases. Future studies should examine these variables.

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BackgroundPopulation-based studies assessing the impact of pneumococcal conjugate vaccines (PCV) on burden of pneumococcal sepsis in children are lacking. We aimed to assess this burden following introduction of PCV-13 in a nationwide cohort study.MethodsThe Swiss Pediatric Sepsis Study (September 2011 to December 2015) prospectively recruited children ResultsChildren with pneumococcal sepsis (n = 117) accounted for a crude incidence of 2.0 per 100 000 children (95% confidence interval [CI] 1.7–2.4) and 25% of community-acquired sepsis episodes. Case fatality rate was 8%. Forty-two (36%) patients required PICU admission. Children with meningitis (29; 25%) were more often infected by serotypes not included in PCV (69% vs 31%; P < .001). Sixteen (26%) of 62 children up to date with PCV immunization presented with vaccine failure, including 11 infected with serotype 3. In multivariable analyses, children with meningitis (odds ratio [OR] 6.8; 95% CI 2.4–19.3; P < .001) or infected with serotype 3 (OR 2.8; 95% CI 1.1–7.3; P = .04) were more often admitted to PICU. Children infected with serotype 3 had longer LOS (β coefficient 0.2, 95% CI .1–1.1; P = .01).ConclusionsThe incidence of pneumococcal sepsis in children shortly after introduction of PCV-13 remained substantial. Meningitis mostly due to non-vaccine serotypes and disease caused by serotype 3 represented significant predictors of severity.

Background Sepsis and severe focal infections represent a substantial disease burden in children admitted to hospital. We aimed to understand the burden of disease and outcomes in children with life-threatening bacterial infections in Europe. Methods The European Union Childhood Life-threatening Infectious Disease Study (EUCLIDS) was a prospective, multicentre, cohort study done in six countries in Europe. Patients aged 1 month to 18 years with sepsis (or suspected sepsis) or severe focal infections, admitted to 98 participating hospitals in the UK, Austria, Germany, Lithuania, Spain, and the Netherlands were prospectively recruited between July 1, 2012, and Dec 31, 2015. To assess disease burden and outcomes, we collected demographic and clinical data using a secured web-based platform and obtained microbiological data using locally available clinical diagnostic procedures. Findings 2844 patients were recruited and included in the analysis. 1512 (53·2%) of 2841 patients were male and median age was 39·1 months (IQR 12·4–93·9). 1229 (43·2%) patients had sepsis and 1615 (56·8%) had severe focal infections. Patients diagnosed with sepsis had a median age of 27·6 months (IQR 9·0–80·2), whereas those diagnosed with severe focal infections had a median age of 46·5 months (15·8–100·4; pInterpretation Mortality in children admitted to hospital for sepsis or severe focal infections is low in Europe. The disease burden is mainly in children younger than 5 years and is largely due to vaccine-preventable meningococcal and pneumococcal infections. Despite the availability and application of clinical procedures for microbiological diagnosis, the causative organism remained unidentified in approximately 50% of patients.

Summary Background Sepsis is a leading cause of childhood mortality worldwide. We assessed population-based incidence and outcomes of blood culture-proven bacterial sepsis in children in Switzerland. Methods We did a multicentre, prospective, cohort study at ten paediatric hospitals in Switzerland. We included neonates and children younger than 17 years with blood culture-proven bacterial sepsis. Children were eligible if they met criteria for systemic inflammatory response syndrome—according to 2005 paediatric consensus definition— at the time of blood culture sampling. Incidence was calculated by dividing the number of annual sepsis episodes in the study for the years 2012–15 by the end-of-year resident paediatric population in Switzerland. The primary outcome was in-hospital mortality in the first 30 days after sepsis onset. Findings Between Sept 1, 2011, and Dec 31, 2015, we enrolled 1096 children to our study. Of 1181 episodes of blood culture-proven bacterial sepsis, 382 (32%) occurred in 379 previously healthy children, 402 (34%) in 391 neonates, and 397 (34%) in 341 children with comorbidities. Incidence was 25·1 cases per 100 000 (95% CI 23·8–26·4) in children and 146·0 cases per 100 000 (133·2–159·6) in neonates. Central line-associated bloodstream infections and primary bloodstream infections accounted for 569 (48%) of 1181 episodes, and organ dysfunction was present in 455 (39%) of 1181 episodes. Escherichia coli (242 of 1181 [20%]), Staphylococcus aureus (177 of 1181 [15%]), coagulase-negative staphylococci (135 of 1181 [11%]), and Streptococcus pneumoniae (118 of 1181 [10%]) were the most prevalent pathogens in our study, accounting for 57% of episodes. The overall case-fatality ratio was 7% (82 of 1181 episodes; 95% CI 5·6–8·6), and it was higher in neonates (11%, 45 of 402 episodes; 8·4–14·8; adjusted odds ratio [OR] 4·41, 95% CI 1·75–11·1) and children with comorbidities (7%, 27 of 397 episodes; 4·6–9·9; OR 4·97, 1·84–13·4) compared with previously healthy children (3%, ten of 382 episodes; 1·3–4·9). The case-fatality ratio was 1% (five of 726 episodes [95% CI 0·3–1·7]) for children without organ dysfunction, which increased to 17% (77 of 455 episodes [13·7–20·8]) when organ dysfunction was present (adjusted OR 4·84, 95% CI 1·40–16·7). Interpretation The burden of blood culture-proven bacterial sepsis on child health remains considerable. We recorded key differences in predominant organisms, severity, and outcome between neonates, previously healthy children, and children with comorbidities. Although for most episodes of blood culture-proven bacterial sepsis, no organ dysfunction was seen, presence of organ dysfunction was strongly associated with mortality. Funding Swiss National Science Foundation, Swiss Society of Intensive Care, Bangerter Foundation, Vinetum and Borer Foundation, and Foundation for the Health of Children and Adolescents.

Background To analyze the clinical presentation and complications of varicella zoster virus (VZV) infection in children with rheumatic diseases treated with immunosuppressive medication such as biological disease-modifying antirheumatic drugs (bDMARDs) and/or conventional disease-modifying antirheumatic drugs (cDMARDs), and to analyze the therapeutic approach to VZV infections with respect to the concomitant immunosuppressive treatment. Methods Retrospective multicenter study using the Swiss Pediatric Rheumatology registry. Children with rheumatic diseases followed in a Swiss center for pediatric rheumatology and treated with cDMARD and/or bDMARD with a clinical diagnosis of varicella or herpes zoster between January 2004 and December 2013 were included. Results Twenty-two patients were identified, of whom 20 were treated for juvenile idiopathic arthritis, 1 for a polyglandular autoimmune syndrome type III, and 1 for uveitis. Of these 22 patients, 16 had varicella and 6 had herpes zoster. Median age at VZV disease was 7.6 years (range 2 to 17 years), with 6.3 years (range 2 to 17 years) for those with varicella and 11.6 years (range 5 to 16 years) for those with herpes zoster. The median interval between start of immunosuppression and VZV disease was 14.1 months (range 1 to 63 months). Two patients had received varicella vaccine (1 dose each) prior to start of immunosuppression. Concomitant immunosuppressive therapy was methotrexate (MTX) monotherapy (n = 9) or bDMARD monotherapy (n = 2), or a combination of bDMARD with prednisone, MTX or Leflunomide (n = 11). Four patients experienced VZV related complications: cellulitis in 1 patient treated with MTX, and cellulitis, sepsis and cerebellitis in 3 patients treated with biological agents and MTX combination therapy. Six children were admitted to hospital (range of duration: 4 to 9 days) and 12 were treated with valaciclovir or aciclovir. Conclusion The clinical course of varicella and herpes zoster in children under immunosuppression is variable, with 4 (18 %) of 22 children showing a complicated course. Thorough assessment of VZV disease and vaccination history and correct VZV vaccination according to national guidelines at diagnosis of a rheumatic autoimmune disease is essential to minimize VZV complications during a later immunosuppressive treatment.

The incidence and outcome of group B streptococcal (GBS) sepsis were assessed prospectively between September 2011 and February 2015 in all tertiary care pediatric hospitals of Switzerland. We describe a low incidence of GBS early-onset sepsis (0.12/1000 livebirths) and a predominance of GBS late-onset sepsis (0.36/1000 livebirths), a pattern that has not been reported in other countries.

Improving information technology security demands greater efforts from both the public and private sectors, as well as better coordination and cooperation between them. US initiatives include creating a series of industry-specific Information Sharing and Analysis Centers to encourage government/industry collaboration on information security. Private sector firms also need stronger incentives to invest adequately in IT security. Moving from trust management to risk management, with widely available and well-functioning markets for insurance covering IT security risks, could go a long way toward strengthening such incentives. Although the insurance industry has developed new forms of cyber insurance, coverage is limited, and few businesses now purchase these policies. Problems include lack of underwriting standards, little experience on which to base premiums, numerous exclusions and limits on liability, and an underdeveloped reinsurance market. Possible ways for government to spur insurance market expansio...

Background and aims Neonatal infection is a major cause of morbidity and mortality. The ongoing Swiss Paediatric Sepsis Study prospectively evaluates the epidemiology of blood culture-proven sepsis in newborns and children in Switzerland. Methods Newborn infants admitted to nine Swiss neonatal intensive care units (NICUs) and presenting with culture-proven sepsis between 9.2011–2.2014 were prospectively enrolled. Early-onset sepsis (EOS) was defined as infection occurring. Results We identified 189 episodes of blood culture-proven sepsis in 186 patients. Thirty seven episodes were classified as EOS and 152 episodes were classified as LOS. Median gestational age at birth was 34 weeks for EOS, and 28 weeks for LOS. Mortality was 14% in EOS and 9% in LOS. Forty six percent of patients required mechanical ventilation during the sepsis episode, and 4% required catecholamine treatment for arterial hypotension. Group B Streptococcus (GBS) and Escherichia coli were the most frequently isolated pathogens in EOS, accounting for 35% and 16% of episodes. Coagulase-negative staphylococci were the leading pathogens in LOS (36%), followed by Staphylococcus aureus (18%), Escherichia coli (16%), and GBS (11%). The proportion of hospital-acquired LOS due to Coagulase-negative staphylococci, Staphylococcus aureus and Escherichia coli varied between 10–68%, 0–45% and 0–30% in different NICUs. Conclusions This national study confirms that neonatal sepsis continues to cause high morbidity and significant mortality. GBS is the most common cause of EOS. There are important differences in the aetiology of LOS in different institutions.

Through a Kronlein incision, a firm tumor mass measuring 23 by 22 by 20 millimeters was removed from the upper outer part of the orbit. For six years the development of a swelling in the upper outer part of the orbit had been accompanied by gradually increasing exophthalmos, and recently the exposure of the eyeball had caused a large sloughing ulcer of the lower two-thirds of the cornea. Vision before operation was counting fingers at eighteen inches, while after operation, with normal position and normal motility, vision of 15/200 was recovered. The tumor contained muscles and connective tissue, cartilaginous metaplasia, and glandular structures. Report and patient presented before the Ophthalmological Section of the New York Academy of Medicine.

Our present knowledge regarding the condition is cited and a case seen by the author is reported in detail.

cDNA and genomic cloning has been used to determine the mRNA and amino acid sequence of human plasma lecithin-cholesterol acyltransferase (LCATase; EC 2.3.1.43). The mature protein was found to contain 416 amino acid residues with a hydrophobic leader sequence of 24 amino acids. An unusual feature of the message is that the poly(A) signal AATAAA overlaps the COOH-terminal glutamic acid and stop codons, and the 3' untranslated region is only 23 bases. The protein itself is distinguished by a number of extended sequences of hydrophobic amino acids, one of which contains a hexapeptide identical with the interfacial binding segment of the active site of pancreatic lipase and is similar to the same site of lingual lipase. The cloned cDNA allows the expression of active LCATase by transfected tissue culture cells.

The cases here reported were submitted to vaccine treatment and did well under it. Read before the American Ophthalmological Society July 9th, 1918. With four illustrations.

Blepharochalasis is the name suggested by Fuchs in 1896 for that condition affecting the skin and the subcutaneous connective tissue of the lids, with atrophy. This condition has been reported repeatedly since then, but still must be considered as a comparatively rare affection and up to the present I have not been able to find any cases reported except those of the foreign clinics. The disease is not a new condition, as McKenzie 1 as early as 1854 called attention to a disease of the lids which he thought was a ptosis with hypertrophy following edema, and which was later followed by a relaxation and drooping of the skin of the lids. In nearly every instance in which the affection has been reported it has been given a new name by the individual who reported the case, and this has been most unfortunate, as it has only added confusion in

Pemphigus of the conjunctiva is one of the grave diseases of the eye, and was recognized and diagnosed as such as early as 1800. Before this time it was classified with the urticarias and scabies, involving the face and eyes; many others considered it a xerosis of the conjunctiva. When this condition affects the eyes it is usually bilateral, although often unequal in the degree of severity. There does not seem to be any constant relation between the duration of the skin affection and the development of the eye lesions. Pemphigus may affect the conjunctiva primarily, but this is rather rare, and in some of the cases so reported the lesions of the skin or mucous membranes may have been overlooked. The course of the disease is usually very slow, extending over a period of years. Samelsohn 1 mentions a case that lasted over seven years, with little or no

The three cases here presented are of such a nature that many authors would regard them as gumma of the iris; but as all of these lesions were seen during the secondary stage of syphilis, it would seem that they should be more properly diagnosed and classified as papules or condylomas of the iris. Papular iritis is often called iritis gummosa. This is because papules of the iris were always regarded anatomically as gummas, and it is often impossible to differentiate between these syphilitic formations even with the microscope. I believe that in the very virulent or malignant form of syphilis, it is possible to have true gumma of the iris during the secondary stage. It would seem that it is more a matter of size and the period of its appearance that makes one lesion a papule and the other a gumma. It is my opinion that gummas are