We reviewed 127 patients who were operated upon for adult presentation Chiari malformation and made six conclusions: (a) The clinical examination remains crucial in the diagnosis. (b) The surgical anatomy is highly varied. (c) Syrinxes can be missed on preoperative contrast studies. (d) By a conservative grading system, we determined that 46% of the patients improved during long term follow-up. One-quarter deteriorated over the long run in spite of any treatment. (e) The overall results did not differ whether the treatment was plugging of the central canal plus decompression or decompression alone. (f) In patients with progression, plugging of the central canal obtained superior results. A review of the literature shows that the natural history of this complex disease process has not been established. This history is needed to identify the course of what may be several important factors that lead to the pathological condition in this disease.
ObjectiveTo summarise the available information on efficacy and safety of immunomodulatory agents in SARS-CoV-2 infection.MethodsAs part of a European League Against Rheumatism (EULAR) taskforce, a systematic literature search was conducted from January 2019 to 11 December 2020. Two reviewers independently identified eligible studies according to the Population, Intervention, Comparator and Outcome framework and extracted data on efficacy and safety of immunomodulatory agents used therapeutically in SARS-CoV-2 infection at any stage. The risk of bias was assessed with validated tools.ResultsOf the 60 372 records, 401 articles were eligible for inclusion. Studies were at variable risk of bias. Randomised controlled trials (RCTs) were available for the following drugs: hydroxychloroquine (n=12), glucocorticoids (n=6), tocilizumab (n=4), convalescent plasma (n=4), interferon beta (n=2), intravenous immunoglobulins (IVIg) (n=2) and n=1 each for anakinra, baricitinib, colchicine, leflunomide, ruxolitinib, interferon kappa and vilobelimab. Glucocorticoids were able to reduce mortality in specific subsets of patients, while conflicting data were available about tocilizumab. Hydroxychloroquine was not beneficial at any disease stage, one RCT with anakinra was negative, one RCT with baricitinib+remdesivir was positive, and individual trials on some other compounds provided interesting, although preliminary, results.ConclusionAlthough there is emerging evidence about immunomodulatory therapies for the management of COVID-19, conclusive data are scarce with some conflicting data. Since glucocorticoids seem to improve survival in some subsets of patients, RCTs comparing glucocorticoids alone versus glucocorticoids plus anticytokine/immunomodulatory treatment are warranted. This systematic literature review informed the initiative to formulate EULAR ‘points to consider’ on COVID-19 pathophysiology and immunomodulatory treatment from the rheumatology perspective.
Early in the coronavirus-2019 (COVID-19) containment strategy, people with end-stage renal disease (ESRD) were identified as extremely clinically vulnerable and subsequently asked to ‘shield’ at home where possible. The aim of this study was to investigate how these restrictions and the transition to an increased reliance on telemedicine within clinical care of people living with kidney disease impacted the physical activity (PA), wellbeing and quality of life (QoL) of adults dialysing at home (HHD) or receiving in-centre haemodialysis (ICHD) in the UK. Individual semistructured telephone interviews were conducted with adults receiving HHD (n = 10) or ICHD (n = 10), were transcribed verbatim and, subsequently, thematically analysed. As result of the COVID-19 restrictions, PA, wellbeing and QoL of people with ESRD were found to have been hindered. However, widespread support for the continued use of telemedicine was strongly advocated and promoted independence and satisfaction in patient care. These findings highlight the need for more proactive care of people with ESRD if asked to shield again, as well as increased awareness of safe and appropriate PA resources to help with home-based PA and emotional wellbeing.
Recent studies have shown major gene effects for obesity in randomly ascertained families. To investigate the familial aggregation of a specific subset of obesity, which is particularly prone to medical complications, families with morbid obesity were studied. This condition occurs in 1%-2%of the population and is defined as 45.5 kg (100 pounds) or more over ideal weight. First-degree relatives of 221 morbidly obese probands (1560 adults) were identified, and height and weight (current and greatest) were obtained from each family member. Morbid obesity occurred in the family members of the probands 8 times more often than in the general population. Of the morbidly obese probands, 48% had one or more first-degree relatives who were also morbidly obese compared to a 6% population estimate. By the ages of 20-24, 12% of the morbidly obese probands were already 45.5 kg or more overweight, and 45% were 22.7 kg (50 pounds) or more overweight. There was little difference in the prevalence of familial morbid obesity by the gender of the probands: 47% of the male probands and 48% of the female probands had another morbidly obese relative, while 67% and 53% of the early onset (before age 25) male and female probands, respectively, had one or more firstdegree relatives who were also morbidly obese. In addition to the extreme degree of familial aggregation, the prevalence of morbid obesity in parent-offspring sets was calculated within the morbidly obese families. Morbidly obese families who have one or two morbidly obese parents have a 2.6 times
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