The findings suggest that NOX-derived ROS results in increased mito-ROS. Whereas short-term increase in mito-ROS was counteracted by MnSOD, long-term increase in ROS resulted in nitrotyrosine-mediated inactivation of MnSOD, leading to unchecked increase in mito-ROS and loss of Δψm followed by inhibition of endothelial function and proliferation.
Systemic pseudohypoaldosteronism (PHA) type I is a rare genetic disorder resulting from mutations in the subunits of the epithelial sodium channel that manifests as severe salt wasting, hyperkalemia, and metabolic acidosis in infancy. In this article we report a patient with systemic PHA type I presenting with severe dehydration due to salt wasting at 6 days of life. She was found to have a known mutation in the SCNN1A gene and subsequently required treatment with sodium supplementation. We also review the clinical presentation, differential diagnosis, and treatment of systemic PHA type I and summarize data from 27 cases with follow-up data.
A 67-year-old woman with morbid obesity and severe obstructive sleep apnea presented to the emergency department with 2 days of productive cough, fever, shortness of breath and loose stools. A chest x-ray showed extensive bilateral infiltrates, and she was quickly intubated for acute hypoxic respiratory failure. A urine legionella antigen test was positive, and she was admitted to the intensive care unit with a diagnosis of severe legionella pneumonia. She improved over the next week with a course of levofloxacin. Once improved, the state health department visited her home and interviewed her husband and determined that she had not been cleaning her continuous positive pressure mask, tubing and humidifier appropriately. They concluded that the legionella likely came from her continuous positive airway pressure equipment. Cases of legionella pneumonia have frequently been reported through infected water supplies and even respiratory equipment. It is crucial that the mask and tubing used with these devices are cleaned appropriately to minimize the risk of infection.
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