A Distinct Instance of Palindromic Rheumatism Disguised as Polymyalgia Rheumatica.

In this case report, we highlight a rare case of palindromic rheumatism (PR) presenting as polymyalgia rheumatica (PMR). Many challenges and complexities are associated with diagnosing and treating PR. Literature reviews showed only a few case reports of this unique presentation. PR has a distinct presentation that often goes unnoticed and is misinterpreted by medical professionals. A more thorough clinical approach is required to identify and treat this condition. We hope sharing such uncommon cases will help the medical community better understand PR and develop improved diagnostic and therapeutic options. This case also demonstrates the need for further research to better understand the pathogenesis of this uncommon condition.

A Systematic Review of the Role of Runt-Related Transcription Factor 1 (RUNX1) in the Pathogenesis of Hematological Malignancies in Patients With Inherited Bone Marrow Failure Syndromes.

Somatic runt-related transcription factor 1 (RUNX1) mutations are the most common mutations in various hematological malignancies, such as myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Mono-allelic RUNX1 mutations in germline cells may cause familial platelet disorder (FPD), an inherited bone marrow failure syndrome (IBMFS) associated with an increased lifetime risk of AML. It is suspected that additional RUNX1 mutations may play a role in the pathogenesis of hematological malignancies in IBMFS. This review aims to study the role of RUNX1 mutations in the pathogenesis of hematological malignancies in patients with IBMFS. A PubMed database search was conducted using the following medical subject heading (MeSH) terms: "inherited bone marrow failure syndromes," "hematological neoplasms," "gene expression regulation, leukemic," "RUNX1 protein, human," "RUNX1 protein, mouse," and "Neutropenia, Severe Congenital, Autosomal recessive." Three studies published in 2020 were identified as meeting our inclusion and exclusion criteria. Leukemic progression in severe congenital neutropenia was used as a disease model to evaluate the clinical, molecular, and mechanistic basis of RUNX1 mutations identified in hematological malignancies. Studies in mice and genetically reprogrammed or induced pluripotent stem cells (iPSCs) have shown that isolated RUNX1 mutations are weakly leukemogenic and only initiate hyperproduction of immature hematopoietic cells when in combination with granulocyte colony-stimulating factor 3 receptor (GCSF3R) mutations. Despite this, whole-exome sequencing (WES) performed on leukemogenic transformed cells revealed that all AML cells had an additional mutation in the CXXC finger protein 4 (CXXC4) gene that caused hyperproduction of the ten-eleven translocation (TET2) protein. This protein causes inflammation in cells with RUNX1 mutations. This process is thought to be critical for clonal myeloid malignant transformation (CMMT) of leukemogenic cells. In conclusion, the combinations of GCSF3R and RUNX1 mutations have a prominent effect on myeloid differentiation resulting in the hyperproduction of myeloblasts. In other studies, it has been noted that the mutations in GCSF3R and RUNX1 genes are not sufficient for the full transformation of leukemogenic cells to AML, and an additional clonal mutation in the CXXC4 gene is essential for full transformation to occur. These data have implicitly demonstrated that RUNX1 mutations are critical in the pathogenesis of various hematological malignancies, and further investigations into the role of RUNX1 are paramount for the development of new cancer treatments.

Worse Outcomes Associated With Liver Transplants: An Increasing Trend.

Background and aim Since individuals in the early stages of liver cirrhosis are typically asymptomatic, the prevalence of liver cirrhosis may be underestimated. Liver cirrhosis has a significant morbidity and mortality rate, with 1.03 million deaths worldwide each year. For end-stage liver disease, liver transplantation is a potential therapeutic option. The goal of our research was to examine the current trend in liver transplants using data from a national database. Methods Using the International Classification of Diseases (ICD)-9 codes, we identified individuals who had a liver transplant during the index hospital admission in the Nationwide Inpatient Sample from 2007 to 2011. This national sample of patients is from the United States. We looked at the yearly trend in liver transplants and related outcomes, such as duration of hospitalization (DOH), hospital expenses, and mortality in the hospital. In order to find determinants of mortality, we used a multivariate analysis. Results There were 25,331 patients hospitalized (weighted for national estimate). Between 2007 and 2011, the number of transplants grew by 1.2%. The majority of transplant recipients were Caucasian (57%), with an average age of 54 years, had a private healthcare plan (53%), and had average earnings in the upper quartile by zip code (26%). Patients with a higher Charlson Comorbidity Index (79% had a score of four) were more likely to be admitted to a southern hospital (33%), an academic hospital (>99%), and a large capacity hospital (90%). Seventy percent of liver transplant recipients received cadaver donors. Hepatitis C was the most prevalent reason for transplant (30%), followed by hepatocellular carcinoma (HCC) (29%) and alcoholic liver disease (25%). In 2011, compared to 2007, there was an upward rise in fatality (from 3.8% to 5.1%), average hospital expenditures (from $335,504 to $498,369), and DOH (from 17.4 to 22.7 days). The cost of hospitalization was two billion dollars per year. The independent variables related to an increased mortality on multivariate analysis were African American race (OR: 2.0, 95%, CI: 1.2-3.2; p=0.005) and large capacity hospitals (OR: 2.5, 95% CI: 1.6-4.1; p=0.0002). Predictors linked to lower mortality included private healthcare coverage (vs. Medicare: OR: 0.7, 95%, CI: 0.51-0.97; p=0.03), academic hospital (OR: 0.6, 95% CI: 0.4-0.8; p=0.005), cadaver donor (OR: 0.6, 95% CI: 0.5-0.8; p=0.002), HCC (OR: 0.6, 95% CI: 0.4-0.9; p=0.01), and non-alcoholic steatohepatitis (NASH) cirrhosis (OR: 0.4, 95% CI: 0.2-0.9; p=0.02). Conclusion Our study found an increasing trend in worse outcomes (increased mortality, average hospital costs, and average DOH) after a liver transplant. Patients of the African American race and large capacity hospitals were associated with a higher risk of death, whereas private healthcare plans, academic hospitals, cadaver donors, HCC, and NASH cirrhosis were associated with a lower risk.

Acute Limb Ischemia Complicated by Heparin-Induced Thrombocytopenia in an Asymptomatic COVID-19 Patient.

Acute limb ischemia (ALI) is the sudden decrease in limb perfusion caused by embolism secondary to many blood stasis conditions. Treatment commences with intravenous (IV) unfractionated heparin infusion. Individuals can have an immune-mediated reaction to heparin products which results in heparin-induced thrombocytopenia (HIT). Coronavirus disease 2019 (COVID-19) has added to the difficulty of treating patients with ALI due to increasing the likelihood of HIT via the virus's ability to manipulate the coagulation parameters. We present a case of ALI complicated by HIT in a 49-year-old male with a confirmed asymptomatic COVID-19. The patient initially presented with progressive pain, coldness, and tingling in the right foot. He was treated with a tissue plasminogen activator (TPA) and a heparin drip. The occlusion persisted despite medical intervention leading to right below-knee amputation. The patient returned to the emergency department (ED) 13 days later with massive intracranial hemorrhage and subsequently expired. This case study demonstrates the significance of COVID-19 diagnostic testing due to the possibility of developing blood clots and potential complications, including HIT.

Elucidating the Intriguing Association Between Systemic Lupus Erythematosus and Cardiovascular Disease.

Systemic lupus erythematosus (SLE) patients have demonstrated a higher risk of developing cardiovascular disease (CVD), resulting in it being one of the leading causes of death in SLE patients. SLE itself acts as a sole risk factor influencing the prevalence and progression of CVD. However, conventional risk factors, such as age, hypertension, smoking, and obesity, play a crucial role as well. Therefore, this systematic review attempts to unravel the association of CVD in SLE patients while evaluating the role of conventional risk factors. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to search the PubMed database starting from March 2021 systematically. Original studies that evaluated the prevalence and progression of CVD in SLE patients were extracted by two reviewers independently. Quality in Prognostic Studies (QUIPS) tool was used to assess the risk of bias. Most studies have a moderate to low risk of bias. Among 3,653 studies identified by our search, 10 studies were included in the review. Strong epidemiologic evidence of SLE patients having an increased relative risk of CVD compared to controls was found. Traditional CVD risk factors, such as age, hypertension, obesity, and smoking, influence the prevalence of CVD among SLE patients. Several SLE-specific factors such disease activity, duration, and certain medications also acted as influencing factors. However, the relative risk of CVD was still higher in SLE patients after adjustment of certain risk factors. One study found that the odds of having a Coronary Artery Calcification (CAC) score greater than zero in women with SLE aged less than or equal to 45 years was 12.6 times higher than women in the Coronary Artery Risk Development in Young Adults (CARDIA) cohort (95% CI 5.2 to 30.7) (participants of CARDIA cohort acted as control). This finding was made after age, hypertension, total cholesterol levels, and aspirin use were adjusted, and the study was restricted to women. Although conventional risk factors increase CVD prevalence, SLE itself also dramatically increases the prevalence of CVD. Therefore, we recommend that SLE should be treated as a "CVD risk equivalent." SLE patients should be managed more extensively with greater emphasis given to cardiac health for better clinical outcomes.

D-Dimer: A Potential Solution to Problems of Cancer Screening, Surveillance, and Prognosis Assessment.

Research has established a direct link between the plasma level of D-dimer and underlying malignancy. D-dimer has a strong association with the detection and prognosis of several cancers. For these reasons, this literature review aimed to evaluate the usefulness of elevated D-dimer levels in the initial screening of cancer, cancer recurrence surveillance, and for use as a cancer prognostic tool. A search of PubMed up to February 1, 2021, was carried out by reviewers. This literature review includes studies investigating the relationship between pretreatment plasma D-dimer levels and cancer. From the findings, pretreatment D-dimer levels can assist with cancer screening and prognosis assessment. Pretreatment plasma D-dimer levels can function as an effective cancer recurrence control. Elevated pre-treatment plasma D-dimer concentration is valuable in facilitating cancer screening, predicting an augmented risk of cancer recurrence, and anticipating a worse cancer prognosis.

Unraveling the Role of Hypothyroidism in Non-alcoholic Fatty Liver Disease Pathogenesis: Correlations, Conflicts, and the Current Stand.

Non-alcoholic fatty liver disease (NAFLD) has become one of the most common causes of chronic liver diseases globally. Because thyroid hormones play a crucial role in lipid metabolism, thyroid dysfunction has been implicated in NAFLD pathogenesis in the past decade, with hypothyroidism-induced NAFLD being regarded as a distinct disease entity. However, there has been no common consensus yet, and several studies have found contradictory results. Hence, we conducted this systematic review to represent the current view on the role of hypothyroidism (HT) and individual thyroid function parameters such as thyroid-stimulating hormone (TSH), thyroxine (T4), triiodothyronine (T3), thyroid peroxidase antibody (TPOAb), and thyroglobulin antibody (TGAb) in NAFLD pathogenesis. We searched PubMed, PubMed Central, and Semantic Scholar databases from inception until January 2021 to identify relevant observational (case-control, cross-sectional, and longitudinal) studies. A total of 699 articles were recognized through our database search. After applying the eligibility criteria and performing quality assessment, 10 studies involving 42,227 participants were included in the final systematic review. Each of these studies assessed different thyroid function parameters, and NAFLD was found to be associated with HT in two studies, elevated TSH in three studies, suppressed T4 in three studies, elevated T3 in one study, and elevated TPOAb in one study. There was also a wide heterogeneity in HT definition, study population characteristics, and study design among these studies, making a direct comparison difficult. Because the recognition of HT-induced NAFLD has possible diagnostic, therapeutic, and prognostic implications, we recommend that comprehensive, long-term prospective studies be carried out to determine if HT or thyroid function parameters are causally associated with NAFLD.