Portosinusoidal Vascular Disorder: A Heretofore Unrecognized Manifestation of Sickle Cell Disease?

Portosinusoidal vascular disorder (PSVD) is a recently proposed histopathologic entity that encompasses a spectrum of often subtle hepatic microvascular lesions and related microarchitectural abnormalities. Clinical manifestations may arise years after histologic diagnosis and include extrahepatic portal vein thrombosis and portal hypertension. While the histopathologic features of PSVD have been associated with numerous clinical conditions, most notably prothrombotic/vasculopathic disorders, PSVD has not yet been described in sickle cell disease. This gap is striking given the central role of microvascular dysfunction in sickle cell disease and well-described patterns of hepatic injury and dysfunction in this population. This case series is the first to explore the prevalence and pathogenesis of PSVD in sickle cell disease. Forty-one diagnostically adequate liver biopsies from patients with sickle cell disease were identified across the archives of 5 tertiary medical centers. All biopsies exhibited at least 1 histopathologic feature associated with PSVD (mean 3.8 features/case). Overall, 90.2% of patients met the criteria for a diagnosis of PSVD based on the presence of specific histopathologic and/or clinical findings. Immunohistochemical stains for von Willebrand factor, CD34, and glutamine synthetase were performed on 36 cases (87.8%). Aberrant (centrilobular sinusoidal) CD34 and von Willebrand factor staining was present in 97.2% and 86.1% of cases, respectively. Glutamine synthetase reactivity was at least mildly decreased in zone 3 hepatocytes in 52.8% of cases. We posit that chronic erythrocyte sickling results in dysfunction and remodeling of the portal microvasculature, culminating in regression of zone 3 hepatocytes. The presence of PSVD may explain, at least in part, the hepatic dysfunction observed in this patient population. These patients may also benefit from extended clinical surveillance for portal hypertension and other complications. While subtle and prone to overdiagnosis, the features of PSVD should be carefully considered when interpreting liver biopsies from patients with sickle cell disease.

All Together Now: Standardization of Nomenclature for Neuroendocrine Neoplasms across Multiple Organs.

Neuroendocrine neoplasms (NENs) span virtually all organ systems and exhibit a broad spectrum of behavior, from indolent to highly aggressive. Historically, nomenclature and grading practices have varied widely across, and even within, organ systems. However, certain core features are recapitulated across anatomic sites, including characteristic morphology and the crucial role of proliferative activity in prognostication. A recent emphasis on unifying themes has driven an increasingly standardized approach to NEN classification, as delineated in the World Health Organization's Classification of Tumours series. Here, we review recent developments in NEN classification, with a focus on NENs of the pancreas and lungs.

ECHO-Chicago Geriatrics: using telementoring to "geriatricize" the primary care workforce.

BACKGROUND: The primary care workforce is under-prepared to care for the growing older adult population. Extension for Community Healthcare Outcomes (ECHO) is a continuing education intervention that connects subspecialists and community health care providers (HCPs) via videoconferencing technology for didactic and case-discussion sessions. Methods: We asked participants to complete 8 to 12 educational telementoring sessions. These sessions were conducted between February 2016 and October 2017. Pre/post surveys of self-efficacy and frequency of practice behaviors were collected from eligible participants. Results: 121 HCPs from multiple sites participated. Of these, 89 (non-trainee status) were eligible to complete surveys. Sixty-two participants (69.7%) completed pre/post surveys. Participants were queried regarding 15 geriatrics competencies. Self-efficacy significantly increased across all competencies after series participation (p < 0.05). Frequency of 8 out of 11 queried geriatrics-centered practice behaviors also increased significantly (p < 0.05). Discussion: ECHO-Chicago Geriatrics offers a novel strategy for improving community HCPs' geriatrics self-efficacy and frequency of geriatrics-centered practice behaviors.