Publications by authors named "R C Nuss"

Lung tissue from human patients and murine models of sickle cell disease pulmonary hypertension (SCD-PH) show perivascular regions with excessive iron accumulation. The iron accumulation arises from chronic hemolysis and extravasation of hemoglobin (Hb) into the lung adventitial spaces, where it is linked to nitric oxide depletion, oxidative stress, inflammation, and tissue hypoxia, which collectively drive SCD-PH. Here, we tested the hypothesis that intrapulmonary delivery of hemopexin (Hpx) to the deep lung is effective at scavenging heme-iron and attenuating the progression of SCD-PH.

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Introduction: Tertiary syphilis, a late form of the disease with neurologic, cutaneous, and cardiovascular manifestations, is rarely seen in the United States in modern times. Also called the "great imitator," syphilis tends to mimic other disease processes, which can lead to a delay in proper diagnosis and treatment.

Case: We present the case of a 59-year-old woman who presented for evaluation of possible squamous cell carcinoma of the vulva.

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Objective: To explore the role of multidisciplinary velopharyngeal dysfunction (VPD) assessment in diagnosing 22q11.2 deletion syndrome (22q) in children.

Design: Retrospective cohort study.

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Sickle cell disease (SCD) is a group of complex genetic disorders of hemoglobin with multisystem manifestations. The scope of this clinical report is such that in-depth recommendations for management of all complications is not possible. Rather, the authors present an overview focused on the practical management of children and adolescents with SCD and the complications that are of particular relevance to pediatric primary care providers.

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