ReviewEuropean respiratory review : an official journal of the European Respiratory Society2018
Pulmonary hypertension associated with neurofibromatosis type 1.
Review in European respiratory review : an official journal of the European Respiratory Society, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
17 citing papers in PubMed, 35 citations in OpenAlex.
- Diagnostic and management dilemmas in pulmonary hypertension.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- When the Skin Tells a Pulmonary Story: A Case of Neurofibromatosis Type 1-Associated Pulmonary Hypertension.Cureus · 2025Article
- Pulmonary Arterial Hypertension in Neurofibromatosis Type 1: A Case with a Novel NF1 Gene Mutation.Internal medicine (Tokyo, Japan) · 2025Article
- Rare genetic interstitial lung diseases: a pictorial essay.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- Therapeutic challenges in transcatheter arterial embolization for an enlarging subcutaneous hematoma in a patient with neurofibromatosis type 1: A case report.Radiology case reports · 2024Article
- Pulmonary Hypertension in Chronic Lung Diseases: What Role Do Radiologists Play?Diagnostics (Basel, Switzerland) · 2023Review
- Neurofibromatosis Type 1: Pediatric Aspects and Review of Genotype-Phenotype Correlations.Cancers · 2023Review
- Neurofibromatosis: New Clinical Challenges in the Era of COVID-19.Biomedicines · 2022Review
- Article
- Bullous lung disease in association with an isolated giant plexiform neuroma: A case report and brief review of pulmonary involvement in neurofibromatosis type 1.African journal of thoracic and critical care medicine · 2021Article
- The Elephant Man Meets Pulmonary Hypertension. A Cautionary Tale.American journal of respiratory and critical care medicine · 2020Article
- Neurofibromatosis-associated pulmonary hypertension: an ominous duo.BMJ case reports · 2020Article
- Neurofibromatosis-Associated Diffuse Lung Disease: A Case Report and Review of the Literature.Cureus · 2020Article
- Pheochromocytoma Multisystem Crisis and Masquerading Disseminated Histoplasmosis in a Neurofibromatosis Type 1 Patient With Bilateral Adrenal Tumors.Academic forensic pathology · 2020Article
- Epidemiology, Pathogenesis, and Clinical Approach in Group 5 Pulmonary Hypertension.Frontiers in medicine · 2020Review
- Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1.Respiratory medicine case reports · 2019Article
- An Uncommon Incidence of Pulmonary Hypertension Associated With Neurofibromatosis Type 1: A Case Report.Journal of investigative medicine high impact case reportsArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease, is a frequent autosomal dominant genetic disorder with a prevalence of 1 in 3000. Pulmonary hypertension (PH) associated with NF1 (PH-NF1) is a rare but severe complication of NF1 and is classified as Group 5 PH, defined as "PH with unclear and/or multifactorial mechanisms". A literature review in PubMed on the association between NF1 and PH identified 18 articles describing 31 cases. PH-NF1 was characterised by a female predominance, an advanced age at diagnosis, an association with parenchymal lung disease in two out of three cases and poor long-term prognosis. NF1 is generally associated with interstitial lung disease but some cases of severe PH without parenchymal lung disease suggest that there could be a specific pulmonary vascular disease. There is no data available on the efficacy of specific pulmonary arterial hypertension treatment in PH-NF1. Therefore, these patients should be evaluated in expert PH centres and referred for lung transplantation at an early stage. As these patients have an increased risk of malignancy, careful assessment of the post-transplant malignancy risk prior to listing for transplantation is necessary. Clinical trials are needed to evaluate promising treatments targeting the RAS-downstream signalling pathways.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.