The American journal of the medical sciences
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Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. It is a leading cause of chronic kidney disease and progresses to end-stage kidney disease in up to 40% of patients about 20 years after diagnosis. Additionally, IgAN is associated with significant mortality. ⋯ Recent advances in understanding the underlying pathophysiology of the disease have led to the proposal of a four-hit hypothesis supporting an autoimmune process. To date, there is no disease-specific treatment but, with a better understanding of the disease pathogenesis, new therapeutic approaches are currently being tested in clinical trials. In this review, we examine the multiple facets and most recent advances of this interesting disease.
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Review Case Reports
Abnormally short erythrocyte lifespan in three patients with primary myelofibrosis despite successful control of splenomegaly.
Splenectomy is an elective operation for refractory anemia in patients with primary myelofibrosis (PMF). We found that 3/3 patients with PMF in our department continued to have very shortened erythrocyte (RBC) lifespans (35 days, 66 days, and 37 days, respectively) after treatment-alleviated splenomegaly. These outcomes suggest that intravascular hemolysis predominantly independent of hypersplenism may underlie, at least to some extent, peripheral hemolysis in patients with PMF. More cases studies are needed to elucidate the role of splenomegaly in PMF-associated anemia.
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Review
Association Between the Anti-Aging Gene Klotho and Selected Rheumatologic Autoimmune Diseases.
Klotho long recognized for its role in anti-aging, is potentially implicated in the pathogenesis of rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Aging of the immune system coincides with the inability of the body to recognize self-antigens, which often leads to autoimmune responses. The role of Klotho in these autoimmune diseases should be of high interest; however, few articles have been published exploring the role of Klotho in the pathogenesis, organ involvement, or clinical manifestation of rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Herein, we discuss information gathered from peer-reviewed publications to describe the emerging role of Kl in these select rheumatologic autoimmune diseases.
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Meta Analysis
The Association of "Loss of Smell" to COVID-19: A Systematic Review and Meta-Nnalysis.
The presence of olfactory dysfunction or "loss of smell" has been reported as an atypical symptom in patients with coronavirus disease 2019 (COVID-19). We performed a systematic review and meta-analysis of the available literature to evaluate the prevalence of "loss of smell" in COVID-19 as well as its utility for prognosticating the disease severity. ⋯ Olfactory dysfunction or "loss of smell" is a prevalent symptom in COVID-19 patients. Moreover, COVID-19 patients with "loss of smell" appear to have a milder course of the disease.
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Review Case Reports
Hemophagocytic Lymphohistiocytosis Secondary to Ehrlichia Chaffeensis in Adults: A Case Series From Oklahoma.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation that occurs as either a familial disorder or as a sporadic condition in association with a variety of triggers. Infections are the most common cause of HLH in adults and should be searched for as early treatment usually results in a favorable outcome. ⋯ Treatment for secondary HLH is aimed at reducing hypercytokinemia and eradicating inflammatory and infected cells. It is important to promptly initiate doxycycline when tick-borne diseases are being entertained as a possible trigger, as the antibiotic is effective, safe and inexpensive.