English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

Von Recklinghausen’s disease (Neurofibromatosis Type 1) represents a complex clinical syndrome. Not every feature is completely disclosured. Few reports in the last decade showed growing cardiac involvement, but not totally understood. Cardiac abnormalities have potential long-term hemodynamic consequences, for these reasons close cardiological assessment are needed for these patients. Anecdotic cases for pulmonary involvement are described, but there may be an unrecognized association between von Recklinghausen disease and pulmonary hypertension above all about the way of clinical presentation. In the comprehensive clinical classification of pulmonary hypertension this systemic disorder was put in group 5 (pulmonary hypertension) with unclear and/or multifactorial mechanisms [1]. In this case we describe a patient with pulmonary hypertension, diagnosed in the clinical setting of neurofibromatosis type 1, presented with severe pericardial effusion. The contemporary presence of NF1, pulmonary hypertension and pericardial effusion in the same clinical setting gives the possibility to discuss about potential physio pathological mechanisms not deeply clear.

Clinical Case of Rare Acute and Chronic Cardio-Pulmonary Involvement in Neurofibromatosis Type-1 (Von Recklinghausen’s Disease)