Birt-Hogg-Dubé Syndrome presenting with chronic progressive dyspnea.

David Reilly, Lila Pourzand, Chidinma Chima-Melton

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Article in Respiratory medicine case reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed

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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

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0 citing papers in PubMed, 0 citations in OpenAlex.

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4 · The record

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5 · Who and what money

Authors and funding

3 authors at 1 institution in 1 country.

David ReillyUniversity of California Los Angeles, Department of Radiology, 200 UCLA Med Plaza, Los Angeles, CA, USA.

Lila PourzandUniversity of California Los Angeles, Department of Radiology, 200 UCLA Med Plaza, Los Angeles, CA, USA.

Chidinma Chima-MeltonUniversity of California Los Angeles, Department of Pulmonary & Critical Care Medicine, 200 UCLA Med Plaza, Los Angeles, CA, USA.

University of California, Los Angeles · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Birt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant disease which manifests with cutaneous hamartomas, lung cysts and renal carcinomas. A wide spectrum of phenotypic expression and few visible manifestations makes BHDS a likely under-recognized entity. Diffuse cystic lung disease (DCLD) is the typical pulmonary manifestation of BHDS, which in the absence of other specific findings carries a broad differential diagnosis. Unlike many other causes of DCLD, BHDS is not known to present with symptomatic pulmonary dysfunction. We report a typical case of BHDS with an atypical presentation - chronic progressive dyspnea. The unusual presentation provides an opportunity to discuss the differential for DCLD and highlights the importance of maintaining an index of suspicion for BHDS even when symptoms appear inconsistent with the diagnosis. Also examined is the management of BHDS patients and their immediate relatives, and recommendations for the treatment of obstructive sleep apnea (OSA) with continuous positive airway pressure (CPAP) given the potential risk of pneumothorax in this group.

Indexed as

BackgroundBirt Hogg DubeCystic lung diseaseDyspneaObstructive sleep apneaRenal cell carcinoma

Identifiers

PMID33912385

DOI10.1016/j.rmcr.2021.101407

PMCPMC8065275

OpenAlexW3143276706

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LicenceCC BY-NC-ND

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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.