Erdheim Chester disease (ECD) is a type of histiocytosis characterized by a variable clinical presentation. The treatment of ECD is complex and mainly unknown. We aim to conduct a literature review of the treatment of ECD and consolidate the knowledge about the most recent and updated treatment for ECD. To conduct the systematic review, we used the preferred reporting items for systematic reviews and meta-analysis (PRISMA) protocol. To analyze the bias, we used the Cochrane collaboration risk-of-bias tool to assess the bias. We included observational studies and clinical trials on humans, which were written in English. Papers not fulfilling the objective of our study were excluded. Overall, the drug showed efficacy in the clinical trials, showing prolonged improvement and high rates of response rate. Overall, the drug was not well tolerated, and patients had a long list of side effects. Nevertheless, the drug seems to be a good option for second-line treatment for patients with ECD and BRAFV600 mutation.
Ataxia is a constellation of symptoms that involves a lack of coordination, imbalance, and difficulty walking. Hereditary ataxia occurs when a person is born with defective genes, and this degenerative disorder may progress for several years. There is no effective cure for ataxia, so we need to search for new treatments. Recently, interest in riluzole in the treatment of ataxia has emerged. We conducted this systematic review to analyze the safety and efficacy of riluzole for treating hereditary ataxia in recent clinical trials. We conducted a systematic review using PubMed and Google Scholar as databases in search of this relationship. We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Meta-analysis of Observational Studies in Epidemiology (MOOSE) protocols to conduct this study. For inclusion criteria, we included full-text clinical trials on humans written in English and found three clinical trials. We excluded case reports, literature reviews, systematic reviews, and meta-analyses for this analysis. We aimed to evaluate the Scale for the Assessment and Rating of Ataxia (SARA) score, the International Cooperative Ataxia Rating Scale (ICARS) score, and the safety of the medication. Two out of the three clinical trials showed statistically significant clinical improvement in the ICARS and SARA scores, while the other trial did not show improvement in the clinical or radiological outcomes. The drug was safe in all clinical trials. Overall, the results of this analysis of riluzole for the treatment of hereditary ataxia are encouraging. Further clinical trials are needed to investigate the efficacy of riluzole on hereditary ataxia.
The objective is to study the demographic and geographical factors that increase the risk of colorectal cancer (CRC) in inpatients with ulcerative colitis (UC) and evaluate the mortality risk and hospitalization outcomes in terms of length of stay (LOS) and cost of care in patients with CRC in UC. We conducted a cross-sectional study using the nationwide inpatient sample (NIS, 2019). We included 78,835 inpatients (age 15-65 years) hospitalized on emergency-based admissions with a primary diagnosis of UC. The study sample was divided by the presence of CRC. Categorical and continuous data were analyzed using Pearson’s chi-square test and independent-sample t-test respectively. Independent binomial logistic regression models were used to evaluate the odds ratio (OR) of predictors associated with CRC in patients with UC compared to non-CRC. The prevalence of CRC in inpatients with UC was 0.2%, and the mean age for admission of patients with UC with CRC was 49.6 years (SD ± 10.29). A directly proportionate relationship exists between increasing age and the risk of CRC in UC inpatients with 10 times higher odds seen in 51-65 years of age (OR 10.0, 95% CI 5.11-19.61). Males (OR 2.15, 95% CI 1.49-3.08) and Hispanics (OR 1.69, 95% CI 1.04-2.74) are at higher odds for CRC compared to their counterparts. Acquired immunodeficiency syndrome (AIDS) was associated with increased odds (OR 6.23, 95% CI 2.48-15.68) for CRC in UC inpatients. There existed an increased association for CRC in UC inpatients with complicated hypertension, and alcohol and drug abuse but was statistically non-significant. As per the adjusted regression model, CRC in UC inpatients increased the risk of in-hospital mortality (OR 41.09, 95% CI 19.49-86.58). CRC was more prevalent in middle-aged Caucasian males with UC and those with chronic comorbidities including complicated diabetes and hypertension, alcohol abuse, and AIDS. Patients with UC and AIDS were found to have greater odds of developing CRC. A high index of clinical suspicion is needed in the management of these patient groups as the inpatient mortality risk was higher in UC inpatients with CRC.