Neurofibromatosis 1 (NF1) is an autosomal-dominant disorder with various clinical expressivity and complications. Arterial hypertension may be present in patients with NF1 and is secondary to vasculopathy or pheochromocytoma. We herein describe two children (17 and 4 years old) diagnosed late with NF1 after severe arterial hypertension had been discovered due to pheochromocytoma and middle aortic syndrome. Routine measurement of arterial pressure is mandatory in children with NF1, in order to diagnose the complications of this disease.
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http://dx.doi.org/10.2147/TCRM.S173237 | DOI Listing |
Biochem Biophys Res Commun
January 2025
Department of Ultrasonography, Fuwai Yunnan Hospital, Chinese Academy of Medical, Sciences/Affiliated Cardiovascular Hospital of Kunming Medical University, Kunming, 650102, China. Electronic address:
Pulmonary arterial hypertension (PAH) is a syndrome characterized by increased pulmonary vascular resistance and elevated pulmonary artery pressure, ultimately leading to right heart failure and even death. Increasing evidence implicates the fat mass and obesity-associated protein (FTO) in various metabolic and inflammatory pathways; however, its role in pulmonary endothelial function and PAH remains largely unexplored. In this study, we examined the effects of endothelial cell-specific FTO knockout on PAH development.
View Article and Find Full Text PDFAnn Intern Med
January 2025
Renal-Electrolyte Division, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania (M.C.-P., R.B.M., C.M.P.).
Background: Prior studies indicate that 1% to 4% of Epstein-Barr virus (EBV)-seronegative recipients of EBV-seropositive donor (EBV D+/R-) kidneys develop posttransplant lymphoproliferative disorder (PTLD). However, these estimates are based on limited data that lack granularity.
Objective: To determine the associations between pretransplant EBV D+/R- and recipient EBV-seropositive status (R+) and the outcomes of PTLD and graft and patient survival among adult kidney transplant recipients.
Ann Intern Med
January 2025
Department of Epidemiology and Welch Center for Prevention, Epidemiology, and Clinical Research, Johns Hopkins Bloomberg School of Public Health, Baltimore; and Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland (T.M.B.).
Background: Guidelines emphasize quiet settings for blood pressure (BP) measurement.
Objective: To determine the effect of noise and public environment on BP readings.
Design: Randomized crossover trial of adults in Baltimore, Maryland.
Circulation
January 2025
Canonsburg, PA (T.H., S. Hackley).
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