Journal of investigative medicine : the official publication of the American Federation for Clinical Research
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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease. Clinical manifestations include intravascular hemolysis, renal dysfunction, fatigue, jaundice, pulmonary hypertension, and so on. Renal injury, as a clinical feature of PNH, is difficult to diagnose and is one of the causes of death in patients with PNH. This article reviews the progress in research on PNH combined with renal injury,to improve clinicians' understanding of renal injury in PNH patients, define and judge staging in a timely and accurate manner, enable patients to receive timely and appropriate treatment, and reduce mortality.
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Review
EXPRESS: State of the Art: Alternative Overlap Syndrome - Asthma and Obstructive Sleep Apnea.
In the general population, Bronchial Asthma (BA) and Obstructive Sleep Apnea (OSA) are amongst the most prevalent chronic respiratory disorders. Significant epidemiologic connections and complex pathogenetic pathways link these disorders via complex interactions at genetic, epigenetic and environmental levels. The coexistence of BA and OSA in an individual likely represents a distinct syndrome, i.e., a collection of clinical manifestations attributable to several mechanisms and pathobiological signatures. ⋯ Even longitudinal epidemiological evaluations in BA cohorts developing over time OSA, or OSA cohorts developing BA during follow-up cannot exclude time factors or causal influence of other known or unknown mediators. As such, a lot of pathophysiological interactions described here have suggestive evidence, biological plausibility, potential or actual directionality. By showcasing existing evidence and current knowledge gaps, the hope is that deliberate, focused and collaborative efforts in the near-future will be geared towards opportunities to shine light on the unknowns, and accelerate discovery in this field of health, clinical care, education, research and scholarly endeavors.
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Advances in Human Immunodeficiency Virus (HIV) treatment including combination antiretroviral therapy (cART) have transformed HIV into a chronic condition. Kidney diseases cause morbidity and mortality in patients living with HIV (PLWH), though cART has permitted kidney transplants with acceptable post-transplant graft and patient survival. Risk of allograft rejection remains high, which may be related to interactions between cART, specifically protease inhibitors (PI), and immunosuppressants prescribed post-transplant. ⋯ Two studies found significant patient survival benefit to non-PI-based therapy. For each outcome measure, remaining data suggested improved outcomes with non-PI-based therapies without achieving statistical significance. The results demonstrate superior outcomes in PLWH taking non-PI-based cART, though the paucity of significant results suggests that PLWH who require PI-based cART for virological control may continue their regimen safely post-kidney transplant.
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As more states legalize cannabinoid products for recreational use and medicinal purposes, the prevalence of cannabinoid hyperemesis syndrome has become increasingly common. Yet, it remains unrecognized to many healthcare providers along with the most efficacious treatments. Cannabinoid hyperemesis syndrome most often presents with episodic vomiting secondary to chronic daily cannabis use over several months to years. ⋯ Long-term management and prevention of further attacks are aided by tricyclic antidepressants such as amitriptyline with a dose range of 50-200 mg/d. Once a patient is in remission, amitriptyline can be tapered slowly. As cannabis becomes more widely available and accepted in the continental United States, so must education on the diagnosis of cannabinoid hyperemesis syndrome and treatment strategies.
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The FK506-binding protein (FKBP5) plays significant roles in mediating stress responses by interacting with glucocorticoids, participating in adipogenesis, and influencing various cellular pathways throughout the body. In this review, we described the potential role of FKBP5 in the pathogenesis of two common chronic liver diseases, metabolic dysfunction-associated steatotic liver disease (MASLD), and alcohol-associated liver disease (ALD). We provided an overview of the FK-binding protein family and elucidated their roles in cellular stress responses, metabolic diseases, and adipogenesis. We explored how FKBP5 may mechanistically influence the pathogenesis of MASLD and ALD and provided insights for further investigation into the role of FKBP5 in these two diseases.